tag:blogger.com,1999:blog-73455378583735187812024-03-06T12:02:50.507-08:00Understanding Williams SyndromeSarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.comBlogger26125tag:blogger.com,1999:blog-7345537858373518781.post-40022128931595903352022-08-09T18:52:00.000-07:002022-08-09T18:52:13.687-07:00Math Instruction <p> <span style="font-family: Arial; font-size: 16pt; white-space: pre-wrap;">“Breaking the Math Myth for Students with Williams Syndrome”</span></p><span id="docs-internal-guid-8a2781d6-7fff-a70d-de27-f96cdd550203"><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">A summary of a presentation at the Williams Syndrome Association National Convention, 2022</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><br /></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Information in this post is summarized from a presentation with permission from the authors to share:</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">By Robin Pegg, EdDc, MEd, COTA/L, ATP- WSA Educational Consultant</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Dr. Carolyn Mervis, PhD- University of Louisville</span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Research supported by NIH (National Institute of Health) and Williams Syndrome Association</span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Often the math gaps for students with Williams Syndrome is due to language. </span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">In IQ DAS-II tests, children with Williams Syndrome test in mild to moderate intellectual disabilities, but when we look at the subscores, we can see that the </span><span style="font-family: Arial; font-size: 13pt; font-style: italic; font-variant-east-asian: normal; font-variant-numeric: normal; font-weight: 700; vertical-align: baseline; white-space: pre-wrap;">low IQ is due to their spatial disability.</span><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"> The verbal and nonverbal scores tend to be in low to average ranges but the spatial range shows a moderate disability, pulling the overall IQ score down. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="border: none; display: inline-block; height: 360px; overflow: hidden; width: 624px;"><img height="360" src="https://lh6.googleusercontent.com/bRZ6cWjaGNEHUDlYO26rNrWunaAj90gMZKkNGtly9nuKD5_cmSpcPowH_w64eaCcKHpu0uNJ-EqC7KP_5zuwXrkrW3Tqtmnw25a6_UkTT5-xrpen26UmL0ggBKoja_QRROHfa-3r8oFcZQZwawXVmtM" style="margin-left: 0px; margin-top: 0px;" width="624" /></span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">IQ data source: </span><a href="https://williams-syndrome.org/files/paragraphs/pdf/intellectualassessmentanddata.pdf" style="text-decoration-line: none;"><span style="color: #1155cc; font-family: Arial; font-size: 11pt; font-style: italic; font-variant-east-asian: normal; font-variant-numeric: normal; text-decoration-line: underline; text-decoration-skip-ink: none; vertical-align: baseline; white-space: pre-wrap;">Mervis, Intellectual Assessments </span></a></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Williams syndrome brain studies have shown that due to genetic code insufficiency, the neuron pathways in the dorsal stream develop abnormally. This pathway of information in the brain greatly limits a person’s ability to reason spatially. This makes mathematics and many motor planning functions difficult. The same brain study found an increase in neural movement in the ventral stream, the pathway that is used in social, musical and auditory functions. Knowing this brain science, we know that </span><span style="font-family: Arial; font-size: 11pt; font-style: italic; font-variant-east-asian: normal; font-variant-numeric: normal; font-weight: 700; vertical-align: baseline; white-space: pre-wrap;">students with Williams syndrome activate the ventral (social) pathway during learning. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="border: none; display: inline-block; height: 351px; overflow: hidden; width: 624px;"><img height="351" src="https://lh5.googleusercontent.com/6Fk26K7oLG7sG-NDaDpp6PF6_GsCOn6xblbYfZpSpt4ngPZkd_uBmLgPUthFmngdfWO-bI_3fEzoJa9qgDnoNyazTaGuxzWZfIxO5RC2s3smNBYPTEljopib_sE3c0FjzECURVOL_naYv1Z9Z8SLU_M" style="margin-left: 0px; margin-top: 0px;" width="624" /></span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><a href="https://williams-syndrome.org/resource/neural-mechanisms-in-williams-syndrome" style="text-decoration-line: none;"><span style="color: #1155cc; font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; text-decoration-line: underline; text-decoration-skip-ink: none; vertical-align: baseline; white-space: pre-wrap;">Source: Mervis, </span><span style="color: #1155cc; font-family: Arial; font-size: 11pt; font-style: italic; font-variant-east-asian: normal; font-variant-numeric: normal; text-decoration-line: underline; text-decoration-skip-ink: none; vertical-align: baseline; white-space: pre-wrap;">Neural mechanisms (2006)</span></a></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">In the following graph, you can compare a typical developing (TD) child performing math to a child with Williams Syndrome (WS). You can see that students typically activate their visuospatial pathways during a math task but students with WS have higher activity in the social pathway of their brain. Because brain pathways are used differently for students with WS, </span><span style="font-family: Arial; font-size: 13pt; font-style: italic; font-variant-east-asian: normal; font-variant-numeric: normal; font-weight: 700; vertical-align: baseline; white-space: pre-wrap;">we need to approach math in a different way to optimize their learning. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="border: none; display: inline-block; height: 351px; overflow: hidden; width: 624px;"><img height="351" src="https://lh6.googleusercontent.com/usZ9K5EgaAo2mB_Op9K4lsCcGWfXjr38YRKrelA9R28nxDCrxWQIA5J6Vv_wM1TRGL3-irW2AOSk16s3v1ZIO0tEqFHGbd6GdK6A250pMN68ryNA45qDoql43Vtagg08jJRPVg0Ty-tbEQb9zR-V65k" style="margin-left: 0px; margin-top: 0px;" width="624" /></span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="border: none; display: inline-block; height: 377px; overflow: hidden; width: 624px;"><img height="377" src="https://lh4.googleusercontent.com/3QyHv8-_OjquIBngRicTBzxZKVjRNxoZ4shgNQBr-fGee-126R8Kzt-b2gfYO7XcKxHZR010XiYZp9pkZfb_Pz5Sf5OipYN1s9PEmCuGses-3MIwfcE0APi1CJ7CmkjJQtIx9k7Mf5Pk0bNPpDtEtyA" style="margin-left: 0px; margin-top: 0px;" width="624" /></span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Strategies to improve math success in students with Williams syndrome:</span></p><ul style="margin-bottom: 0; margin-top: 0; padding-inline-start: 48px;"><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 13pt; font-style: italic; font-variant-east-asian: normal; font-variant-numeric: normal; font-weight: 700; vertical-align: baseline; white-space: pre-wrap;">Deliberately teach relational language</span><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">. One weakness for students with WS is that they struggle with spatial vocabulary; making it difficult to interpret instructions in math. The following vocabulary should be practiced in multiple settings including in the math classroom and during speech/language therapy. In order to master these terms, students must know both the word and its antonym. Otherwise, they have not truly mastered the concept. </span></p></li><ul style="margin-bottom: 0; margin-top: 0; padding-inline-start: 48px;"><li aria-level="2" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Dimensional terms: ex- small/large</span></p></li><li aria-level="2" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Quantitative terms: ex- more/less</span></p></li><li aria-level="2" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Spatial terms: ex- under/over</span></p></li><li aria-level="2" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Temporal terms: ex- before/after</span></p></li></ul><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Notice in the graph below, that the curve of students that can master relational terms (TRC graph) correlates directly with IQ sub-scores (DAS-II spatial tests). The PPVT graph shows typical developing students for comparison.</span></p></li><ul style="margin-bottom: 0; margin-top: 0; padding-inline-start: 48px;"><li aria-level="2" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><br /></li></ul></ul><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="border: none; display: inline-block; height: 359px; overflow: hidden; width: 624px;"><img height="359" src="https://lh3.googleusercontent.com/sMGmrOFRPibyYvNZk0S9WSTU8Z7mDgbFM56N6c7nLHUvJuT9GjKuS9wHpmqGAr9foMsW6oRSCO9fLThZUgZLrK_nhJwNwk69VRxpPvD4ISSbqd8rXx-KG-jvumYqyc-OPTdAKv7pTIaRA58lQO293V0" style="margin-left: 0px; margin-top: 0px;" width="624" /></span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"> </span><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="border: none; display: inline-block; height: 355px; overflow: hidden; width: 624px;"><img height="355" src="https://lh5.googleusercontent.com/lo9QDQOPvYGM1-XBTzIrKHCmVK-1GN8E_8LIhfXlKVoR83a-PJEZSgr_fSEA7u3t49_jC4TfgcP3iG2laMHwCC0UVHRKmk-HtiGEPDg3TIp94FduytAvxB4stFMePlrlTOnMF0X_rr4EBX9iNdI4tAg" style="margin-left: 0px; margin-top: 0px;" width="624" /></span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="border: none; display: inline-block; height: 355px; overflow: hidden; width: 624px;"><img height="355" src="https://lh4.googleusercontent.com/0T4VR7y46HQaoyxIYCiZ_AjQa2bGP-43ZEfvYiq5X7vyXL0N8HZol2UQmf60QlKhIBFV2uZb7WD25lYhOlCwSY0pXjnlqZvdee42V2hvbwchhZMAZXiwewUTNtd91x4Y5SGnEWuiq7afV5V8UXAFhcI" style="margin-left: 0px; margin-top: 0px;" width="624" /></span></span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 13pt; font-style: italic; font-variant-east-asian: normal; font-variant-numeric: normal; font-weight: 700; vertical-align: baseline; white-space: pre-wrap;">Most students with WS are said to “max out” on their math success by 2-3 grades. This overall lack of success is not due to their ability to do math, it has to do with the shift in teaching styles that occur that turn toward representational math. </span><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"> </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="border: none; display: inline-block; height: 329px; overflow: hidden; width: 624px;"><img height="329" src="https://lh3.googleusercontent.com/FEsjxsbK4ooqlBHpZHw_6bOJ-ta0IWhHQmZPwe8Qgo9bSJVw37SLrjN8TxXg5l17pIMLuLnpF1Z0mV5U1p1PDNpvZ7ON_ZT1fak_4DMs5Uhk2i34Igiqu5EeLLD1CTtZRtQzw1mBGspxiAEE76PbMU4" style="margin-left: 0px; margin-top: 0px;" width="624" /></span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">The CRA approach to math does not work well for students with Williams Syndrome for several reasons:</span></p><ul style="margin-bottom: 0; margin-top: 0; padding-inline-start: 48px;"><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">This teaching is designed as a spiral approach. Students with WS need prolonged practice until mastery. The spiral approach, which leaves a concept and then revisits it later for reinforcement does not give students with WS enough time to master the concept. They do better in a sequential curriculum. </span></p></li><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">“Draw it out” or representational tasks (which are typically introduced in 2-3 grade) require spatial reasoning and are not likely something a student with WS can do successfully… explaining why many have low success advancing past 3rd grade math! </span></p></li><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 13pt; font-style: italic; font-variant-east-asian: normal; font-variant-numeric: normal; font-weight: 700; vertical-align: baseline; white-space: pre-wrap;">Concrete and abstract approaches to math work for students with WS</span><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">. Abstract is best used with accommodations where students have sequences mapped out for the process since they have poor working memory. </span></p></li><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 13pt; font-style: italic; font-variant-east-asian: normal; font-variant-numeric: normal; font-weight: 700; vertical-align: baseline; white-space: pre-wrap;">Work around representational math</span><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">- students with WS have spatial deficits that are not curable. They will never think that way. Instead…</span></p></li><ul style="margin-bottom: 0; margin-top: 0; padding-inline-start: 48px;"><li aria-level="2" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Use manipulatives and technology.</span></p></li><li aria-level="2" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Provide cue cards for the process of solving a problem</span></p></li><li aria-level="2" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Use graph or lined paper on its side to help them line up their place values.</span></p></li><li aria-level="2" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Use concrete method of teaching even in higher grades. </span></p></li></ul></ul><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Math programs that work well for many students with WS:</span></p><ul style="margin-bottom: 0; margin-top: 0; padding-inline-start: 48px;"><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Programs designed for dyscalculia- students with dyscalculia have similar problems to kids with WS. </span></p></li><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">TouchMath</span></p></li><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">NumberRock- uses music to solve problems</span></p></li><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Manipulaties- best if they are designed for low vision students- larger and more tactile work best</span></p></li><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Kidspiration maps- virtual math manipulatives for fractions- </span></p></li><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">EquatIO tool in google- allows students to type their math so they are not limited by their handwriting; can be used for dictation, lines up numbers for the student and suggests equations or gives a limited options in the formula finder which helps students figure out what to use. </span></p></li><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Algebra tiles- makes algebra manipulative *really cool program!</span></p></li><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Desmos graphing calculator</span></p></li><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Geogebra- geometry will be difficult</span></p></li></ul><br /><br /><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 13pt; font-style: italic; font-variant-east-asian: normal; font-variant-numeric: normal; font-weight: 700; vertical-align: baseline; white-space: pre-wrap;">Math goals should NEVER be centered around time, measurement and money because these are representational tasks.</span><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"> </span></p><ul style="margin-bottom: 0; margin-top: 0; padding-inline-start: 48px;"><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Teach students to use technology, digital clocks and alarms for time. </span></p></li><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Mix functional math with grade level mathematics. Students with WS can perform algebra with support!</span></p></li><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Teach students the dollar up method for money. </span></p></li><ul style="margin-bottom: 0; margin-top: 0; padding-inline-start: 48px;"><li aria-level="2" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Counting coins is not a useful skill anymore. Focus on money handling that they will use: calculating tips, adding totals, rounding up to the nearest dollar for payment, counting cash. </span></p></li></ul><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Focus math goals on number sense. Push them to higher tasks on Bloom's taxonomy. </span></p></li><ul style="margin-bottom: 0; margin-top: 0; padding-inline-start: 48px;"><li aria-level="2" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Know when they should use math; why; what situations</span></p></li><li aria-level="2" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Work on when to use money, how to manage money, judgment on when and how to use it</span></p></li><li aria-level="2" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: circle; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Focus on all aspects of number sense</span></p></li></ul></ul><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="border: none; display: inline-block; height: 329px; overflow: hidden; width: 624px;"><img height="329" src="https://lh3.googleusercontent.com/BlMaYw6QX9wk-yBXgjTX5y2PDDAR8wesA7mfbc6GiYWstPJA0jSOLVkOdXyC1qscEH7RL26m6MnSZIXPsFoiIuRxqgajCjJhvyRYQfoC6r0yFJxCFPwhxO5b5NllTpUHZHcF6ks0Jb7w_3AEW6PGD9s" style="margin-left: 0px; margin-top: 0px;" width="624" /></span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="border: none; display: inline-block; height: 321px; overflow: hidden; width: 624px;"><img height="321" src="https://lh6.googleusercontent.com/fY9LVMuGAZhy6qF22kpGY7rNXfkWX0GaigPQxjFjIKUjHsDgqGqmBuLebqGKbi-IKdixsgsQl-UX31RF9bLPWxrHCHOnGfCGSSgw318_QgYY2sFXM1zCU_L0MMIO4kFFsE8bWN721AzHRt7VMWtQt8w" style="margin-left: 0px; margin-top: 0px;" width="624" /></span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">With the right supports and teaching strategies, students with WS have the capacity to learn algebra!</span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="border: none; display: inline-block; height: 364px; overflow: hidden; width: 624px;"><img height="364" src="https://lh3.googleusercontent.com/DvcFmd0rF12xETP_XjrDTBndn7qK0wzGmhWhLyePI97r-yHL-qVL-eStAp0wW4CHaSWJddoiIJi46dhxeoNjX2RsfIapG2vTOktAZzXhK4AvdTN49kWnno-sQBXzASM7qqj2QgqFWLiKx3yuQ1rj1zQ" style="margin-left: 0px; margin-top: 0px;" width="624" /></span></span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Students with WS have poor working memory. This makes solving problems with multiple steps or sequences very difficult. Typically students with WS will lose the ability to solve a problem due to not knowing the next step. They often get stuck and respond by just sitting there, guessing or relying on a teacher for a verbal cue. </span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 13pt; font-style: italic; font-variant-east-asian: normal; font-variant-numeric: normal; font-weight: 700; vertical-align: baseline; white-space: pre-wrap;">Students with WS do well with routines and with cues that help them remember steps</span><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">. Use the following methods to accommodate working memory issues:</span></p><ul style="margin-bottom: 0; margin-top: 0; padding-inline-start: 48px;"><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Use instructional routines to liberate the students to know what to do next. Example- cue cards for the sequence of actions to solve a problem, color code each step with an example problem showing those steps. </span></p></li><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Make cue cards in google slides and print like a book with a sequence on how to solve an algebraic problem. </span></p></li><li aria-level="1" dir="ltr" style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; list-style-type: disc; vertical-align: baseline; white-space: pre;"><p dir="ltr" role="presentation" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">PEMDAS- Pre-algebra instructional routines made for students with WS work as an accommodation (see below). </span></p></li></ul><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="border: none; display: inline-block; height: 411px; overflow: hidden; width: 624px;"><img height="411" src="https://lh6.googleusercontent.com/JPXBWkA_u7yVfULb0yzExDg4o9MPdtTYyIpZBTgZgy8RV5fZwDXKjAap0Y0UVcHeY2Xt79iKrLSiA2LknMWK1xCAkHkaiewlxfgP_MtLgWmyStJSQmmxjnCo7jF9UljgP_nSCn-6-N-qVkm1uFr2Nyw" style="margin-left: 0px; margin-top: 0px;" width="624" /></span></span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Because students with WS think with their social pathway, teacher cues can become restrictive. If a student needs to stay on task, the worst cue a teacher can use is verbal instruction. It pulls their attention away from the task and makes it difficult for them to return to the work. </span><span style="font-family: Arial; font-size: 13pt; font-style: italic; font-variant-east-asian: normal; font-variant-numeric: normal; font-weight: 700; vertical-align: baseline; white-space: pre-wrap;">Use the prompt hierarchy below to prioritize how you can cue a student with WS in the least restrictive manner. </span><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">Use that cue routinely to teach them predictable, on task behaviors (conditioned responses) that lead to their success. </span></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="border: none; display: inline-block; height: 447px; overflow: hidden; width: 572px;"><img height="447" src="https://lh3.googleusercontent.com/O4VTfoGNYVDsAkLBdy1qnqwE96LFudGBrAAKOT7fe-G3DG_SheBkW6HhFNFLYH8sdRrRPNhSYyF4qK7X8kr8OpGmkeXKKRBN4Vg7fcUFdSkpg4MliIvSS7giH2f-b8Ue3zAvv3WTVnmagSB-qSU3lZ8" style="margin-left: 0px; margin-top: 0px;" width="572" /></span></span></p><br /><br /><br /><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;"><span style="border: none; display: inline-block; height: 320px; overflow: hidden; width: 624px;"><img height="320" src="https://lh4.googleusercontent.com/P43omVqPrFVcl5GTX-_YGsNQBRmskbuDBckxL9Gi6PdNtYmYU84rKXQZ9GJ5a1pgH3Pv9O29oi3tpPnffQMuH-XIdW7GSOotg_HkUART2_Po6wF2J5BErV4ICk4STpHaV2x0bBWeMSxiaVbtH5FSk9U" style="margin-left: 0px; margin-top: 0px;" width="624" /></span></span></p><br /><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><a href="https://sites.google.com/williams-syndrome.org/wsa-education-resources/math" style="text-decoration-line: none;"><span style="color: #1155cc; font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; text-decoration-line: underline; text-decoration-skip-ink: none; vertical-align: baseline; white-space: pre-wrap;">Link to virtual math teaching resources</span></a></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><a href="https://sites.google.com/williams-syndrome.org/wsa-education-resources/just-for-teachers" style="text-decoration-line: none;"><span style="color: #1155cc; font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; text-decoration-line: underline; text-decoration-skip-ink: none; vertical-align: baseline; white-space: pre-wrap;">The educational profile of students with WS</span></a></p><p dir="ltr" style="line-height: 1.38; margin-bottom: 0pt; margin-top: 0pt;"><span style="font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; vertical-align: baseline; white-space: pre-wrap;">“Designing </span><a href="https://williams-syndrome.org/resources/convention-workshop/designing-math-instruction" style="text-decoration-line: none;"><span style="color: #1155cc; font-family: Arial; font-size: 11pt; font-variant-east-asian: normal; font-variant-numeric: normal; text-decoration-line: underline; text-decoration-skip-ink: none; vertical-align: baseline; white-space: pre-wrap;">Math Instruction”- webinar for teaching students with WS math</span></a></p><br /></span>Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com0tag:blogger.com,1999:blog-7345537858373518781.post-2721029086154099912016-07-06T10:00:00.000-07:002016-07-09T06:53:37.253-07:00Understanding the Power of the Parent Advocate- 2016 WSA National convention speech<br />
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</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">Welcome
everyone!<span style="mso-spacerun: yes;"> </span>Thank you for coming to my
session on Understanding the Power of the parent advocate.<span style="mso-spacerun: yes;"> </span>My name is Sarah Moonier and like you I am a
parent of a child with Williams syndrome.<span style="mso-spacerun: yes;">
</span>This presentation stems from my experiences navigating through Katie’s
ups and downs and the strategies I’ve learned in my professional life that have
given me a unique perspective in both the doctor’s office and at the IEP table.<o:p></o:p></span></div>
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<span style="font-family: "calibri";">So my story started in a room such as this, at the St. Louis
national convention.<span style="mso-spacerun: yes;"> </span>My daughter was
diagnosed at 5 months and we were lucky to discover that the convention was in
our home town 2 months later.<span style="mso-spacerun: yes;"> </span>I had no
idea what to expect but it was an experience of many mixed emotions as you all
know especially since I was still in a state of shock over it all.<span style="mso-spacerun: yes;"> </span>At the St. Louis convention, we attended as
many medical sessions as we could since those were the primary issues we were
experiencing at the time.<span style="mso-spacerun: yes;"> </span>While I was
there I became very cognizant of the community’s need for straight forward
explanations of their kid’s challenges.<span style="mso-spacerun: yes;">
</span>I saw high emotions and some speakers who struggled to give parents information
that was too scientific.<span style="mso-spacerun: yes;"> </span>This planted an
idea in my head that I could provide that sort of resource.<span style="mso-spacerun: yes;"> </span>By day I’m a human anatomy teacher and I have
a talent of explaining very difficult material in a way that people can
understand so I knew I could do that for this community.<span style="mso-spacerun: yes;"> </span>So that’s how my blog was born. <o:p></o:p></span></div>
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<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhfc9CS02pOPH3SStKFRtyMf5eBOEO1QTKwnavwq5bTSYu-uYZTlgksyIncKEdOFX9V_pgH5_OVPa4p8NF-Uz5OGRR6HbzxVQqYIFr3xRwlWV0R9-b-_7lxEt-Vj6_fWFC42mBMuubfuQgx/s1600/Capture2.PNG" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhfc9CS02pOPH3SStKFRtyMf5eBOEO1QTKwnavwq5bTSYu-uYZTlgksyIncKEdOFX9V_pgH5_OVPa4p8NF-Uz5OGRR6HbzxVQqYIFr3xRwlWV0R9-b-_7lxEt-Vj6_fWFC42mBMuubfuQgx/s640/Capture2.PNG" width="432" /></a></div>
<span style="font-family: "calibri";">Over the 6 years that my blog has existed it has morphed
from purely medical related advice to a lot of educational advice.<span style="mso-spacerun: yes;"> </span>My blog posts tend to evolve with the issues
that my family tackles and I’ve learned that the knowledge and resources that I
create have opened many modes of communication for my daughter’s medical and
educational professionals.<span style="mso-spacerun: yes;"> </span>It’s really
changed the relationships that I have with her care team so that’s what I want
to share with you today.<o:p></o:p></span></div>
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</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">My goal in this
session today is to share with you my advocacy strategy.<span style="mso-spacerun: yes;"> </span>Now, it’s based on my experiences so it’s not
a one size fits all solution of course.<span style="mso-spacerun: yes;">
</span>Every school or hospital has different resources or procedures BUT I
have found this form of advocacy to be very successful and hope you will too.<span style="mso-spacerun: yes;"> </span>So this presentation will be delivered in two
parts.<span style="mso-spacerun: yes;"> </span>First, as an advocate you have to
become an expert in your child.<span style="mso-spacerun: yes;"> </span>You know
your child better than anyone so you are already an expert in that regard but
knowing the whole profile and having access to professional articles and tools
will help you build valuable resources for your child’s team, in turn elevating
your role as a participant in those IEP meetings.<span style="mso-spacerun: yes;"> </span>The second phase then is sharing those
resources with the team.<span style="mso-spacerun: yes;"> </span>Having been on
both sides of an IEP table, I witnessed the whole realm of emotions that come
with them but I’ve also observed the social game and have a unique perspective on
how to navigate that.<span style="mso-spacerun: yes;"> </span><o:p></o:p></span></div>
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</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">So the beginning is
what you are here at the convention to do and that is educate yourself.<span style="mso-spacerun: yes;"> </span>We’re going to focus today on the nervous
system because the issues related to WS that are core problems with learning
are all situated around the nervous system.<span style="mso-spacerun: yes;">
</span>The biggest take away in this portion is that isolated issues are a
myth.<span style="mso-spacerun: yes;"> </span>You may see an ophthalmologist for
vision and a speech therapist for language and a PT for muscles and special
educators for reading and math but all of those issues are not isolated.<span style="mso-spacerun: yes;"> </span>They are all connected.<span style="mso-spacerun: yes;"> </span>If we can understand that and know that big
picture of how they are all related, you can then begin to treat the whole
child.<span style="mso-spacerun: yes;"> </span>Now your therapists and doctors
and educators don’t usually all communicate with one another so that becomes
your role.<span style="mso-spacerun: yes;"> </span>The parent is the
communication pipeline between all these experts so the more you fully
understand that big picture, the better your conversations with those
professionals linking them and treating your child to the fullest extent will
fall into place.<span style="mso-spacerun: yes;"> </span>So here we go…<o:p></o:p></span></div>
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<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgaJr057lUNypd8xTtcGQ-9GXBlJ6usy63UoEsXO62J5t9tXhzvthbtIkjX9ccDEUyUbgPn09XAbRW8RLCi4Ayp-mJ4D5h5JlZw_DSsadvGXRAfQav5jwz0Bp1SYIzdFSn6bdcNgSdkUBy2/s1600/Capture3.PNG" imageanchor="1" style="clear: left; float: left; margin-bottom: 1em; margin-right: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgaJr057lUNypd8xTtcGQ-9GXBlJ6usy63UoEsXO62J5t9tXhzvthbtIkjX9ccDEUyUbgPn09XAbRW8RLCi4Ayp-mJ4D5h5JlZw_DSsadvGXRAfQav5jwz0Bp1SYIzdFSn6bdcNgSdkUBy2/s640/Capture3.PNG" width="434" /></a></div>
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</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">In order to
understand that big picture to treat your whole child you should start with the
basic function of the nervous system.<span style="mso-spacerun: yes;">
</span>When people think of the nervous system they immediately think of the
brain but our nervous system is much more complex than that.<span style="mso-spacerun: yes;"> </span>It actually begins with the sensory organs
which monitor our environment and send messages about stimuli in our
environment so we can navigate it and make decisions that keep us safe and
functional.<span style="mso-spacerun: yes;"> </span>You can probably think of a
dozen issues related to our kids’ sensory organs such as vision, hearing,
texture issues, feeding issues, attention issues… we’ll focus on a couple of
these but I want you to understand that many of your child’s behaviors are
related to this portion of the nervous pathway- gathering that
information.<span style="mso-spacerun: yes;"> </span>The issues can also extend
to how that input is interpreted.<span style="mso-spacerun: yes;"> </span>This
is a fundamental process that requires communication across the brain where we
interpret the senses and then decide on a response.<span style="mso-spacerun: yes;"> </span>WS affects a few of basic pathways in the
brain that cause our kids to interpret their environment and create a motor
output in an atypical way. Understanding this allows us to come up with creative
and effective ways to help them in the classroom.<span style="mso-spacerun: yes;"> </span>The final function is the response from the
muscles.<span style="mso-spacerun: yes;"> </span>Our kids often show muscle tone
issues and movement issues and most of those muscle issues are actually caused
by ineffective communication of the nervous system and those muscles causing
them to become overly weak or overly tense.<span style="mso-spacerun: yes;">
</span>So this is what we’ll do for the next half hour or so, we’ll work
through these three functions and look at ways it affects a kid with WS and how
by looking at the big picture, we can help many issues at once.<o:p></o:p></span></div>
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</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhT7eDru6EqnJ5aOs5Vz9D5peejt-A3FLxe7gQlnFv-B2nv03POi8DuHzOu0NnJYwGGeM0bnGpdFTOFX7tjNAiuys2bmSi8tv8FiTRDT6D54SVK3yceBgoI8ZpBatJ1xemm4t_sptULY5-L/s1600/Capture4.PNG" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhT7eDru6EqnJ5aOs5Vz9D5peejt-A3FLxe7gQlnFv-B2nv03POi8DuHzOu0NnJYwGGeM0bnGpdFTOFX7tjNAiuys2bmSi8tv8FiTRDT6D54SVK3yceBgoI8ZpBatJ1xemm4t_sptULY5-L/s640/Capture4.PNG" width="438" /></a><span style="font-family: "calibri";">So let’s begin with
the senses.<span style="mso-spacerun: yes;"> </span>One common issue for 50% of kids
with WS is hyperopia or far sightedness.<span style="mso-spacerun: yes;">
</span>In hyperopia, the shape of the eye causes the light to focus behind the
eye rather than hit a clear focused stream of light on the receptors of the
retina.<span style="mso-spacerun: yes;"> </span>This causes poor, blurry vision.<o:p></o:p></span></div>
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<span style="font-family: "calibri";"><span style="mso-spacerun: yes;"> </span><o:p></o:p></span></div>
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<span style="font-family: "calibri";">Hyperopia is a condition caused by either a flattened cornea
which is the window to the eye or due to a physically shorter eyeball and can
easily be corrected with glasses or contacts.<span style="mso-spacerun: yes;">
</span>So you might notice your child squinting or like my Katie on the right
there, we noticed that she hated tummy time as an infant and that she tilted
her head far backwards to see things.<span style="mso-spacerun: yes;">
</span>That was our first clue and the tummy time ended up because she couldn’t
see anything!<span style="mso-spacerun: yes;"> </span>She couldn’t pick up her
head and tilt it backwards to see the object in front of her and once we got
her to an ophthalmologist she started tolerating it better and developing
faster.<span style="mso-spacerun: yes;"> </span>If your child has poor eyesight
it will negatively affect their development because it limits the amount of
sensory input the brain receives in order to do the learning.<span style="mso-spacerun: yes;"> </span>So, it’s important to see a pediatric ophthalmologist
for testing. </span></div>
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</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">The second sensory
issue we’re going to explore is hearing.<span style="mso-spacerun: yes;">
</span>61% of our children have frequent middle ear infections, called otitis
media.<span style="mso-spacerun: yes;"> </span>You can see in the picture there,
the middle ear is the area right behind the eardrum where the ossicles or ear
bones are.<span style="mso-spacerun: yes;"> </span>The ossicles vibrate when
sound hits the ear drum and transfers that motion to the cochlea where the
sensory receptors are.<span style="mso-spacerun: yes;"> </span>Frequent ear
infections can cause delays in learning and difficulty with attention.<span style="mso-spacerun: yes;"> </span>Interestingly, our kids tend to produce
excess ear wax or have unusually narrow ear canals.<span style="mso-spacerun: yes;"> </span>My daughter’s ear canals are tilted upwards
into her middle ear which makes it difficult for the body to naturally move
that wax out. <span style="mso-spacerun: yes;"> </span>It builds up and affects
her hearing so we use ear drops, see an ear nose and throat doctor to remove it
and get a yearly pediatric audiologist assessment to make sure her hearing is functioning
properly since all those issues can directly affect her learning.<o:p></o:p></span></div>
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<o:p><span style="font-family: "calibri";"> </span></o:p><br />
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<span style="font-family: "calibri";"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjCWzrY3-hB_yg2lyhacVmzGtXV9LM2K0VsV3M1J9EtcZz5nGi8Sb7Bj2v2gVPLVhgW1ycZCvkDWpREU-eJhZxdlTuFWwJYZjhPI3GQJL8SaniMfS1XK8vMG-0OV_COM_Q7sfpVf7JmNDqP/s1600/Capture5.PNG" imageanchor="1" style="clear: left; float: left; margin-bottom: 1em; margin-right: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjCWzrY3-hB_yg2lyhacVmzGtXV9LM2K0VsV3M1J9EtcZz5nGi8Sb7Bj2v2gVPLVhgW1ycZCvkDWpREU-eJhZxdlTuFWwJYZjhPI3GQJL8SaniMfS1XK8vMG-0OV_COM_Q7sfpVf7JmNDqP/s640/Capture5.PNG" width="430" /></a></span></div>
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<span style="font-family: "calibri";">Another sensory issue that we are all familiar with that
affects 90% of those with WS throughout their life is hyperacusis.<span style="mso-spacerun: yes;"> </span>For my daughter her triggers are the leaf
blowers, vacuums and motorcycles but every kid has their own set of sound
triggers that inflicts terror and pain.<span style="mso-spacerun: yes;">
</span>Scientists aren’t positive about what causes this issue but there are
three different theories.<span style="mso-spacerun: yes;"> </span><o:p></o:p></span></div>
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<span style="mso-no-proof: yes;"><span style="font-family: "calibri";"><span style="mso-spacerun: yes;"> </span>The first theory is that the sound is actually
perceived as motion.<span style="mso-spacerun: yes;"> </span>The inner ear has
two regions, the vestibular apparatus made up of the semicircular canals which
monitor movement of the body and the cochlea which picks up the sound.<span style="mso-spacerun: yes;"> </span>The theory is that the vestibular
semicircular canals confuse the sound as movement, amplifying the sensory input
to the brain.<o:p></o:p></span></span></div>
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<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image013.png"
o:title="" croptop="22118f" cropbottom="21827f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="mso-no-proof: yes;"><span style="font-family: "calibri";"><span style="mso-spacerun: yes;"> </span>The second
theory has to do with muscle tone.<span style="mso-spacerun: yes;"> </span>Think
back to a time when you went to a loud concert or to a pep rally at
school.<span style="mso-spacerun: yes;"> </span>When you leave the venue, you
feel like sound is muffled and you speak a little too loud to people because
you can’t hear yourself.<span style="mso-spacerun: yes;"> </span>But after a
while the sound returns to normal.<span style="mso-spacerun: yes;"> </span>This
demonstrates the function of a tiny muscle in your middle ear called the tensor
tympanii whose job is to contract around the eardrum to dampen very loud sounds
and protect your hearing.<span style="mso-spacerun: yes;"> </span>The theory is
that low tone in this muscle causes it to fail to adjust to loud sounds causing
intense sound reception.<o:p></o:p></span></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<!--[if gte vml 1]><v:shape id="Picture_x0020_12" o:spid="_x0000_s1059"
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<br />
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<div class="separator" style="clear: both; text-align: center;">
<br /></div>
<span style="font-family: "calibri";">The third and final theory has to do with
neurotransmitters.<span style="mso-spacerun: yes;"> </span>Neurotransmitters are
chemicals that allow one neuron or nerve cell to communicate with another.<span style="mso-spacerun: yes;"> </span>We have many of these chemicals in our
body.<span style="mso-spacerun: yes;"> </span>Some neurotransmitters send
messages and others regulate the intensity of that message and block or dampen
signals.<span style="mso-spacerun: yes;"> </span>The theory is that the cochlea,
the area that senses sound has low serotonin which is that dampening
chemical.<span style="mso-spacerun: yes;"> </span>Due to less regulation, the
nerve fires more intensely causing the brain to interpret louder more intense
sound.<o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<span style="font-family: "calibri";">There is no real way to stop hyperacusis from happening but
we can use behavioral approaches to anticipate issues or give our kids coping
skills on how to accommodate their environment.<span style="mso-spacerun: yes;">
</span>Things such as anticipating the sound and avoiding it such as bringing
headphones to the fireworks display or watching them from inside a room.<span style="mso-spacerun: yes;"> </span>Gradual conditioning is also very
effective.<span style="mso-spacerun: yes;"> </span>Katie was very afraid of
animated toys that move and make sounds.<span style="mso-spacerun: yes;">
</span>So we started with just having one in the room turned off so that she
got used to the toy.<span style="mso-spacerun: yes;"> </span>Then we’d watch
youtube videos of them with the sound off so she became used to the
motion.<span style="mso-spacerun: yes;"> </span>We’d play with it when she was
out of the room so she was used to the sound but at a lower intensity and then
eventually exposed her to it.<span style="mso-spacerun: yes;"> </span>She
managed to grow out of it and moved onto other scary sounds like hair dryers
and vacuums!<o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<div class="separator" style="clear: both; text-align: center;">
<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjr1x_ZCdEIVm_a_1_EWlXCHw2vfJA8uA2CjkG_lo6JRYITo-qlDBJyuMq3dMmNzTe2tL3QExgU_-m39ad-jEHbUI5YW11tt4pkGduA4wAoBG01rnZEaitrDshi5ajpqg9uDoZbpGwSB1L7/s1600/Capture7.PNG" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjr1x_ZCdEIVm_a_1_EWlXCHw2vfJA8uA2CjkG_lo6JRYITo-qlDBJyuMq3dMmNzTe2tL3QExgU_-m39ad-jEHbUI5YW11tt4pkGduA4wAoBG01rnZEaitrDshi5ajpqg9uDoZbpGwSB1L7/s640/Capture7.PNG" width="442" /></a></div>
<span style="font-family: "calibri";">So again, these sensory function in our body are all about
gathering information about our surroundings so we can interpret them and come
up with a response so since our kids have these and other atypical sensory
receptions, it can affect all the steps further down the nervous system
pathway.<span style="mso-spacerun: yes;"> </span>Therefore, as we look at the next
two functions we’ll come back to these sensory issues and see how they can
complicate other issues related to physical activity and education and we can
come up with strategies to think of all these steps as we go to give our kids a
leg up.<o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<!--[if gte vml 1]><v:shape id="Picture_x0020_13" o:spid="_x0000_s1058"
type="#_x0000_t75" style='position:absolute;margin-left:-12.65pt;margin-top:562.55pt;
width:178.5pt;height:128.15pt;z-index:251671552;visibility:visible;
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o:title="" cropbottom="43956f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">So our next step
along the nervous pathway is the brain where we’ll take all this sensory information
and interpret our surroundings so we can decide on a proper response.<span style="mso-spacerun: yes;"> </span>This is called integration or association
because we’ll take that information and associate it with memories, experiences
and put all that input together like a puzzle to make sense of what is
happening around us.<span style="mso-spacerun: yes;"> </span>The brain
accomplishes this with two major functional areas- primary areas and
association areas.<span style="mso-spacerun: yes;"> </span>There are primary and
association areas for all our sensory and motor functions and they are kind of
like depot stops along that pathway that put two and two together.<span style="mso-spacerun: yes;"> </span>The primary areas are our basic instincts
they are where we identify the stimulus as a bright vs dim light, the
wavelength the color the amplitude of the sound the pitch the frequency… just
basic awareness.<span style="mso-spacerun: yes;"> </span>The association areas
are where we label that as the sun and associate it with feelings, experiences
and prior knowledge.<span style="mso-spacerun: yes;"> </span><o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<!--[if gte vml 1]><v:shape id="Picture_x0020_14" o:spid="_x0000_s1057"
type="#_x0000_t75" style='position:absolute;margin-left:-7.1pt;margin-top:133pt;
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o:title="" croptop="21451f" cropbottom="21576f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><o:p></o:p><br /></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<span style="font-family: "calibri";">Once we’ve labelled and figured out our surrounds we have
two major streams of information that move that nervous impulses together
toward areas of the brain that will create a response.<span style="mso-spacerun: yes;"> </span>The dorsal stream or where stream is a
nervous pathway that moves upwards towards the motor areas of the brain that
control movement.<span style="mso-spacerun: yes;"> </span>This stream is the
spatial stream where we make sense of where things are relative to one another
and it tends to be a relative weak area for those with WS.<span style="mso-spacerun: yes;"> </span><o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<o:p><span style="font-family: "calibri";"> </span></o:p></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<span style="font-family: "calibri";">The ventral stream moves laterally towards the temporal lobe
where our language centers are.<span style="mso-spacerun: yes;"> </span>This
stream of information illicit a verbal response and is highly associated with
speech, music and facial recognition… a strength for our kids.<span style="mso-spacerun: yes;"> </span>You can see where I’m going here, right?<o:p></o:p></span></div>
<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgYyIqCMydQvD2EiHZB2tE9SOYSEa_jVXAfY3ckX5sgvadkJTgmpC6PeVb4DEY6cmOGrOkZCMN2hjmQB2YsV-jAl719c0dbzsUs1jnnw1riqJOXqOB1QXTGABl6nkciV8-UI2VRBTwqInbT/s1600/Capture8.PNG" imageanchor="1" style="clear: left; float: left; margin-bottom: 1em; margin-right: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgYyIqCMydQvD2EiHZB2tE9SOYSEa_jVXAfY3ckX5sgvadkJTgmpC6PeVb4DEY6cmOGrOkZCMN2hjmQB2YsV-jAl719c0dbzsUs1jnnw1riqJOXqOB1QXTGABl6nkciV8-UI2VRBTwqInbT/s640/Capture8.PNG" width="440" /></a><br />
<div class="MsoNormal" style="margin: 0in -13.5pt 8pt 0in;">
<!--[if gte vml 1]><v:shape
id="Picture_x0020_15" o:spid="_x0000_s1056" type="#_x0000_t75" style='position:absolute;
margin-left:-6.5pt;margin-top:330.7pt;width:178.5pt;height:130.5pt;z-index:251673600;
visibility:visible;mso-wrap-style:square;mso-wrap-distance-left:9pt;
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mso-wrap-distance-bottom:0;mso-position-horizontal:absolute;
mso-position-horizontal-relative:margin;mso-position-vertical:absolute;
mso-position-vertical-relative:margin'>
<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image017.png"
o:title="" croptop="43560f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">So, when thinking
about WS.<span style="mso-spacerun: yes;"> </span>We can use our scientific
knowledge of how our kids think and translate that into ways we can help them
overcome their challenges.<span style="mso-spacerun: yes;"> </span>We know that
kids with WS have visuo-spatial difficulties and this is because the where or
dorsal stream has low amounts of gray matter.<span style="mso-spacerun: yes;">
</span>The gray matter is the area of the brain where we make our decisions and
do the thinking that makes us each unique.<span style="mso-spacerun: yes;">
</span>Since there is less development here it causes somewhat of a traffic jam
of information- they just can’t process it as effectively as the typical
person, resulting in slower activation and decision making.<span style="mso-spacerun: yes;"> </span><o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<!--[if gte vml 1]><v:shape id="Picture_x0020_16" o:spid="_x0000_s1055"
type="#_x0000_t75" style='position:absolute;margin-left:-9.2pt;margin-top:486.65pt;
width:181.5pt;height:131.35pt;z-index:251674624;visibility:visible;
mso-wrap-style:square;mso-wrap-distance-left:9pt;mso-wrap-distance-top:0;
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o:title="" croptop="-1f" cropbottom="43463f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">Since there are
slower neural pathways in this dorsal region our kids tend to have these
difficulties- poor dexterity, slow speeds, difficulty reacting to visual
information and trouble manipulating fine motor skills.<span style="mso-spacerun: yes;"> </span>If you’ve ever watched your child try to
manipulate a coin into a slot, you’ll understand what I mean.<span style="mso-spacerun: yes;"> </span>It took Katie a very long time to accomplish
this skill and still now that she can, she still takes quite a long time to do
it and will attempt a variety of directions before she can.<span style="mso-spacerun: yes;"> </span>It also translates into self-help
issues.<span style="mso-spacerun: yes;"> </span>That same skill is used to
manipulate buttons and zippers or tying shoes for example.<span style="mso-spacerun: yes;"> </span>But knowing these issues you can also create
solutions.<span style="mso-spacerun: yes;"> </span>Until they can handle buttons,
use alternatives.<span style="mso-spacerun: yes;"> </span>Talk to teachers about
using multiple intelligences to teach them these spatial skills by tapping into
those stronger skills on the ventral pathways by using sound, music and verbal
cues.<o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<o:p><span style="font-family: "calibri";"> </span></o:p></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<div class="separator" style="clear: both; text-align: center;">
<br /></div>
<!--[if gte vml 1]><v:shape id="Picture_x0020_17" o:spid="_x0000_s1054"
type="#_x0000_t75" style='position:absolute;margin-left:-7.1pt;margin-top:92.55pt;
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o:title="" croptop="21012f" cropbottom="21922f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgxO_gEhbOGzNausjWbYmuPUZnDIF7UA4II4ULzid9IAGlmqQSFL2yOvd6nQufF1ghNzlVhhM2RgcYn7ghW6fBBSRX3fsMLz0dwymDXI_6LPW-fm0AYF1N5SIGR2s2RO2Fby-z5oZUMJ5I7/s1600/Capture9.PNG" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgxO_gEhbOGzNausjWbYmuPUZnDIF7UA4II4ULzid9IAGlmqQSFL2yOvd6nQufF1ghNzlVhhM2RgcYn7ghW6fBBSRX3fsMLz0dwymDXI_6LPW-fm0AYF1N5SIGR2s2RO2Fby-z5oZUMJ5I7/s640/Capture9.PNG" width="438" /></a><span style="font-family: "calibri";">In addition to the
movement difficulties the integration portion of planning those movements-
called motor planning can be difficult.<span style="mso-spacerun: yes;"> </span>Also
called apraxia, this is all about predicting and planning how to move based on
senses.<span style="mso-spacerun: yes;"> </span>My daughter just recently
underwent surgery to lengthen her heel cords and the PT during her assessment
asks her to push against her hand or resist her from pushing against her foot
in order to assess her strength.<span style="mso-spacerun: yes;"> </span>You can
see my daughter’s wheels turning trying to figure out what she wants her to do.
<span style="mso-spacerun: yes;"> </span>So, we’ve approached it with a variety
of accommodations.<span style="mso-spacerun: yes;"> </span>We sing silly songs
for example, when they want my daughter to move her feet medially to put toes
together we play a game and have her “kiss” her toes and then when she wants
them to move them outwards we play “blah”.<span style="mso-spacerun: yes;">
</span>When she does bridges we sing London bridges or when she does clam
shells we sing the shark song.<span style="mso-spacerun: yes;"> </span>All of
these little modifications help her understand how we want her to move, keeps
her engaged in the activity and improves her memory of the muscle movement
during practice at home or at later sessions.<span style="mso-spacerun: yes;">
</span>I’ve found that when you suggest to people this approach they look at me
like I’m crazy but I just belt out my song all out of tune and go with it.<span style="mso-spacerun: yes;"> </span>Once they see that it works they pick it up
and go with it.<span style="mso-spacerun: yes;"> </span>You just have to break
the ice for them </span><span style="font-family: "wingdings"; mso-ascii-font-family: Calibri; mso-ascii-theme-font: minor-latin; mso-char-type: symbol; mso-hansi-font-family: Calibri; mso-hansi-theme-font: minor-latin; mso-symbol-font-family: Wingdings;"><span style="mso-char-type: symbol; mso-symbol-font-family: Wingdings;">J</span></span><o:p></o:p></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<o:p><span style="font-family: "calibri";"> </span></o:p></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<!--[if gte vml 1]><v:shape id="Picture_x0020_18" o:spid="_x0000_s1053"
type="#_x0000_t75" style='position:absolute;margin-left:0;margin-top:385.45pt;
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o:title="" croptop="42683f" cropbottom="254f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">Now when you combine
the two difficulties- moving and space and planning on how that’s going to
work, you’ll find that nearly all our kids have difficulty with this.<span style="mso-spacerun: yes;"> </span>It’s a hallmark issue with WS and one that
will affect them in so many ways so I feel it’s an important characteristic to
discuss with your child’s therapists and teachers so they understand why your
child will have difficulty in PE or with writing.<span style="mso-spacerun: yes;"> </span>It’s part of their biology.<span style="mso-spacerun: yes;"> </span>Here I have another example with
throwing.<span style="mso-spacerun: yes;"> </span>My daughter has been working
on throwing and catching as a PT goal for about 4 years now and I don’t
anticipate that ever changing.<span style="mso-spacerun: yes;"> </span>She has trouble
seeing that ball coming to her, a sensory issue, she has to process that in her
slower where/dorsal pathway, predict where the ball will land with her motor
planning and then activate the muscles she needs to stand in the right place
and hold her hands out to catch.<span style="mso-spacerun: yes;"> </span>She has
a very slow response which typically results in her moving her hands out to
catch the ball long after it has already whizzed past her.<span style="mso-spacerun: yes;"> </span>So ways we can help?<span style="mso-spacerun: yes;"> </span>Start with slower moving objects.<span style="mso-spacerun: yes;"> </span>We practice with a balloon.<span style="mso-spacerun: yes;"> </span>It moves much more slowly so it gives her
time to process.<span style="mso-spacerun: yes;"> </span>My daughter’s PT also
works on her visual tracking to help with this skill.<span style="mso-spacerun: yes;"> </span>There is a website called </span><a href="http://eyecanlearn.com/"><span style="color: #0563c1; font-family: "calibri";">Eye can learn</span></a><span style="font-family: "calibri";"> that has simple exercises you
can use at home to help your child with visual tracking.<o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<!--[if gte vml 1]><v:shape id="Picture_x0020_19" o:spid="_x0000_s1052"
type="#_x0000_t75" style='position:absolute;margin-left:0;margin-top:0;
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mso-position-vertical:top;mso-position-vertical-relative:margin'>
<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image025.png"
o:title="" cropbottom="43507f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjxOrmEIZ0aftDZwETBJv8uT7JmNjOottIee_kF_mPeHzu6W793G-PZrMPYrLD8S27DmCKDVPJymD9Tb0F9sf4Ga6CRq6cTnecWTTXhzXQlSMh2OHdKAk7Dp4NUMibXuH_NWS_iQAoZbdJb/s1600/Capture10.PNG" imageanchor="1" style="clear: left; float: left; margin-bottom: 1em; margin-right: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjxOrmEIZ0aftDZwETBJv8uT7JmNjOottIee_kF_mPeHzu6W793G-PZrMPYrLD8S27DmCKDVPJymD9Tb0F9sf4Ga6CRq6cTnecWTTXhzXQlSMh2OHdKAk7Dp4NUMibXuH_NWS_iQAoZbdJb/s640/Capture10.PNG" width="436" /></a><span style="font-family: "calibri";">The other way to
overcome these motor deficits is to tap into the strengths.<span style="mso-spacerun: yes;"> </span>Neurologists have long been fascinated with
the WS brain because we have those unique challenges paired with unique
strengths of verbal ability.<span style="mso-spacerun: yes;"> </span>Since the
where stream has a slow “two lane pathway”, the brain builds up a highway
system in the stream that allows the information to move faster resulting in a
strong verbal ventral stream.<span style="mso-spacerun: yes;"> </span>This what
stream gives our kids a strong and sometimes unique pathway and a good working
memory that is in tune with facial recognition.<span style="mso-spacerun: yes;">
</span>It is also activated by music explaining our kid’s affinity to music.<o:p></o:p></span></div>
<br />
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<o:p><span style="font-family: "calibri";"> </span></o:p></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<!--[if gte vml 1]><v:shape id="Picture_x0020_20" o:spid="_x0000_s1051"
type="#_x0000_t75" style='position:absolute;margin-left:0;margin-top:190.85pt;
width:181.5pt;height:136.05pt;z-index:251678720;visibility:visible;
mso-wrap-style:square;mso-wrap-distance-left:9pt;mso-wrap-distance-top:0;
mso-wrap-distance-right:9pt;mso-wrap-distance-bottom:0;
mso-position-horizontal:left;mso-position-horizontal-relative:margin;
mso-position-vertical:absolute;mso-position-vertical-relative:margin'>
<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image025.png"
o:title="" croptop="21498f" cropbottom="21214f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">The reason the
verbal stream is strong has to do with the anatomy of the neurons there. Brain
scan show that there are enlarged and more active regions in this area of the
brain.<span style="mso-spacerun: yes;"> </span>There is greater blood flow to
the hippocampus resulting in work memory strengths and there is more gray area
in the auditory regions the brain, making auditory learning a strength for our
kids.<span style="mso-spacerun: yes;"> </span></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<!--[if gte vml 1]><v:shape id="Picture_x0020_21" o:spid="_x0000_s1050"
type="#_x0000_t75" style='position:absolute;margin-left:0;margin-top:344.9pt;
width:181.5pt;height:128.85pt;z-index:251679744;visibility:visible;
mso-wrap-style:square;mso-wrap-distance-left:9pt;mso-wrap-distance-top:0;
mso-wrap-distance-right:9pt;mso-wrap-distance-bottom:0;
mso-position-horizontal:left;mso-position-horizontal-relative:margin;
mso-position-vertical:absolute;mso-position-vertical-relative:margin'>
<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image025.png"
o:title="" croptop="43924f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">So because our
children’s brains put more stock in this pathway we can see some of those
strengths and use them to overcome the disadvantages they experience in that
where stream.<span style="mso-spacerun: yes;"> </span>Our kids tend to have
strong memory retrieval and this stream is where our auditory areas are found
making them great auditory learners.<span style="mso-spacerun: yes;">
</span>When they are facing tasks that require body movement from anything to
learning their letters, school work to navigating through a maze they should
combine the learning with auditory information.<span style="mso-spacerun: yes;">
</span>This will activate more areas of their brain and keep them more engaged
in the activity.<span style="mso-spacerun: yes;"> </span><o:p></o:p></span></div>
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<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgufKxTlCPPXiPZUkYPwsftT1BStQ0vZf3PZBSLG_CR5fqvJE54wCMKjXL_gip7JL7ymmNQS93GCUP4lObDt_qZWGBmVqsfJYuaGarheX6LtJSt5_7GMyWZFkk2Ffi_XGX5CTsgjGSKakCU/s1600/Capture11.PNG" imageanchor="1" style="clear: left; float: left; margin-bottom: 1em; margin-right: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgufKxTlCPPXiPZUkYPwsftT1BStQ0vZf3PZBSLG_CR5fqvJE54wCMKjXL_gip7JL7ymmNQS93GCUP4lObDt_qZWGBmVqsfJYuaGarheX6LtJSt5_7GMyWZFkk2Ffi_XGX5CTsgjGSKakCU/s640/Capture11.PNG" width="440" /></a><br />
<div class="separator" style="clear: both; text-align: center;">
<br /></div>
<o:p><span style="font-family: "calibri";"> </span></o:p></div>
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<span style="font-family: "calibri";">Our kids learn differently that the typical child because of
the differences in brain matter.<span style="mso-spacerun: yes;"> </span>Very
young children with WS have delay in language development partly because they
learning and develop differently than their peers.<span style="mso-spacerun: yes;"> </span>Usually teachers search for prior knowledge
to build upon but many kids with WS rely more on their working verbal memory to
access learning so it’s important to begin lessons or supplement lessons with
listening skills.<span style="mso-spacerun: yes;"> </span>Request books on
audio, use websites that have a read aloud mode… this explains why so many of
our kids are infatuated with youtube.<span style="mso-spacerun: yes;">
</span>They respond to the sounds and stories they hear there and process that
sound differently like we saw in the hyperacusis section.<span style="mso-spacerun: yes;"> </span><o:p></o:p></span></div>
<br />
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<o:p><span style="font-family: "calibri";"> </span></o:p></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<!--[if gte vml 1]><v:shape id="Picture_x0020_22" o:spid="_x0000_s1049"
type="#_x0000_t75" style='position:absolute;margin-left:0;margin-top:.75pt;
width:180pt;height:128.95pt;z-index:251680768;visibility:visible;
mso-wrap-style:square;mso-wrap-distance-left:9pt;mso-wrap-distance-top:0;
mso-wrap-distance-right:9pt;mso-wrap-distance-bottom:0;
mso-position-horizontal:left;mso-position-horizontal-relative:margin;
mso-position-vertical:absolute;mso-position-vertical-relative:margin'>
<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image029.png"
o:title="" cropbottom="43696f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">There are some
situations where language requires the dorsal where stream and this is where
language becomes difficult for our kids.<span style="mso-spacerun: yes;">
</span>Terms that have to do with opposites and space, size and color,
directions they all require a conceptual understanding of where something is
classified in relation to another concept.<span style="mso-spacerun: yes;">
</span>Many of the speech therapy goals of Katie’s are centered around
understanding these concepts. <o:p></o:p></span></div>
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<o:p><span style="font-family: "calibri";"> </span></o:p></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<!--[if gte vml 1]><v:shape id="Picture_x0020_23" o:spid="_x0000_s1048"
type="#_x0000_t75" style='position:absolute;margin-left:0;margin-top:135.3pt;
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mso-wrap-style:square;mso-wrap-distance-left:9pt;mso-wrap-distance-top:0;
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mso-position-horizontal:left;mso-position-horizontal-relative:margin;
mso-position-vertical:absolute;mso-position-vertical-relative:margin'>
<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image029.png"
o:title="" croptop="20635f" cropbottom="21455f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">So knowing the brain
science, there are strategies you can share with care givers or use at home to
help your children tap into those verbal strengths to overcome their spatial
weaknesses.<span style="mso-spacerun: yes;"> </span>One example, you can teach
verbal reasoning.<span style="mso-spacerun: yes;"> </span>When I was in
elementary school my mom used to coach us before the big game with
visualization techniques of making the goal or imagining the muscle movement to
dive into the pool.<span style="mso-spacerun: yes;"> </span>When you watch the Olympics
you’ll see the gymnasts doing it before a vault visualizing the movements they’ll
take.<span style="mso-spacerun: yes;"> </span>This is a strategy you can teach
your children when working on spatial tasks.<span style="mso-spacerun: yes;">
</span>Talk them through the process and help them accomplish that motor
planning they have trouble doing.<span style="mso-spacerun: yes;"> </span>By
hearing the plan they’ll have more success and gives them the skill where they
can talk through it themselves with practice to plan.<span style="mso-spacerun: yes;"> </span>The same strategy can work with
navigation.<span style="mso-spacerun: yes;"> </span>Say your child has trouble
following instructions to do a task like centers.<span style="mso-spacerun: yes;"> </span>The teacher can verbalize the instructions
before the activity or even use assistive technology on frequent tasks that
help kids follow multi step directions or navigate around their school.<span style="mso-spacerun: yes;"> </span>Provide the use of technology such as gps and
map apps for older kids and provide visual task reminders, signs or symbols to
find locations.<span style="mso-spacerun: yes;"> </span>One problem young kids
have is learning to draw shapes and later how to put those shapes together to make
a picture.<span style="mso-spacerun: yes;"> </span>For example, you’ve probably
seen that classic picture of a bike that was drawn by a person with WS where
their bike looked like a disassembled bike instead.<span style="mso-spacerun: yes;"> </span>Drawing is a very spatial task so you can
help your child by talking them through and drawing faces to learn shapes.<span style="mso-spacerun: yes;"> </span>My daughter started with drawing Mat man
which is a little person drawn with each of the shapes- circle, rectangle,
triangle, square.<span style="mso-spacerun: yes;"> </span>By connecting the
shapes to draw a person, you are tapping into the what stream in order to
accomplish a where task, making our kids more successful at it.<o:p></o:p></span></div>
<o:p><span style="font-family: "calibri";"></span></o:p><br />
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<div class="separator" style="clear: both; text-align: center;">
<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjQfmDUdYHp752aNHOPPGDfy8eZHo0JS498KxBt8ihDU6S5Au6z_Ct4-4NMahhACydpEvjOPk4kDD6aCAf7Pq36ckXY-iGrlL8NV1l9F5SdJnJNKZhqEkSkTULjL57S2dTqOw47rXHYwyn2/s1600/Capture12.PNG" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjQfmDUdYHp752aNHOPPGDfy8eZHo0JS498KxBt8ihDU6S5Au6z_Ct4-4NMahhACydpEvjOPk4kDD6aCAf7Pq36ckXY-iGrlL8NV1l9F5SdJnJNKZhqEkSkTULjL57S2dTqOw47rXHYwyn2/s640/Capture12.PNG" width="438" /></a></div>
<!--[if gte vml 1]><v:shape id="Picture_x0020_24"
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z-index:251658240;visibility:visible;mso-wrap-style:square;
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<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image029.png"
o:title="" croptop="43010f"/>
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</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">So we’ve discussed
the sensory input and the processing, now we move onto the final stage of the
nervous pathway which is how we respond to the environment.<span style="mso-spacerun: yes;"> </span>In this pathway, the brain uses regions
within areas of the cerebellum to coordinated muscle movement in response.<span style="mso-spacerun: yes;"> </span>This subconscious control of muscles keeps
our muscles in the proper tone or a contracted state that prepares it for a
muscular response.<span style="mso-spacerun: yes;"> </span>It adjusts core
muscles for balance and small muscles that contract to steady a movement and
improve fluidity.<span style="mso-spacerun: yes;"> </span>It’s also involved in
motor planning and skilled execution such as typing and driving… many of the
tasks that our kids have difficulty with.<o:p></o:p></span></div>
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</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";"><span style="mso-spacerun: yes;"> </span><span style="mso-spacerun: yes;"> </span><o:p></o:p></span></div>
<br />
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<div class="separator" style="clear: both; text-align: center;">
<br /></div>
<span style="font-family: "calibri";">One of these issues is related to
balance.<span style="mso-spacerun: yes;"> </span>Many kids with WS have awkward
gait and trouble moving their body in a fluid coordinated motion.<span style="mso-spacerun: yes;"> </span>This is due to an enlarged region of the
cerebellum which is used for muscle coordination.<span style="mso-spacerun: yes;"> </span>This area disrupts the body’s ability to
connect their senses to memory and control muscles in response to those
senses.<span style="mso-spacerun: yes;"> </span>This brings us back to those
original sensory issues we discussed at the beginning. <span style="mso-spacerun: yes;"> </span>We’ll start with control of the eye and in
this section we’re going to participate in a little experiment.<span style="mso-spacerun: yes;"> </span>I’d like you to choose something on your body
to adjust a bit.<span style="mso-spacerun: yes;"> </span>Put a ring on a
different finger, roll your socks a strange way, tie your hair back
tighter.<span style="mso-spacerun: yes;"> </span>Keep it that way for a bit
while explain the eye… you’ll see why in a minute!<o:p></o:p></span></div>
<br />
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<o:p><span style="font-family: "calibri";"> </span></o:p><br />
<div class="separator" style="clear: both; text-align: center;">
<span style="font-family: "calibri";"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEi3QcCo2yr0FgbjBgXHBm2gYu-kHCoHpboOZcLcxZOglCDuweSCisHJ_qzA_fyK-mFI9ElIZt9F3HLH9AN5kweenum7y4Vvk7nf1yZjKQVEdvltuQv9pvSZzxq08Jdry8-vM-8ayjPdTL1S/s1600/Capture13.PNG" imageanchor="1" style="clear: left; float: left; margin-bottom: 1em; margin-right: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEi3QcCo2yr0FgbjBgXHBm2gYu-kHCoHpboOZcLcxZOglCDuweSCisHJ_qzA_fyK-mFI9ElIZt9F3HLH9AN5kweenum7y4Vvk7nf1yZjKQVEdvltuQv9pvSZzxq08Jdry8-vM-8ayjPdTL1S/s640/Capture13.PNG" width="442" /></a></span></div>
<br /></div>
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<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<!--[if gte vml 1]><v:shape id="Picture_x0020_26"
o:spid="_x0000_s1045" type="#_x0000_t75" style='position:absolute;left:0;
text-align:left;margin-left:-13.45pt;margin-top:385.55pt;width:179.95pt;
height:132.85pt;z-index:251683840;visibility:visible;mso-wrap-style:square;
mso-wrap-distance-left:9pt;mso-wrap-distance-top:0;mso-wrap-distance-right:9pt;
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mso-position-horizontal-relative:margin;mso-position-vertical:absolute;
mso-position-vertical-relative:margin'>
<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image033.png"
o:title="" croptop="21543f" cropbottom="21660f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">So when we’ve
received that visual information and made what sense that we could of it our
brain wants to send information back to the muscles of the eye in order to gain
new perspective.<span style="mso-spacerun: yes;"> </span>The message travels down
the motor pathways of the oculomotor nerve and to the muscles that surround our
eye to move our eye back and forth.<span style="mso-spacerun: yes;"> </span>That
movement is coordinated between the two eye balls to gain depth
perception.<span style="mso-spacerun: yes;"> </span>It also causes movement
within the eye in muscles that adjust the lens of the eye to better focus light
and get a clearer image.<o:p></o:p></span></div>
<span style="font-family: "calibri" , sans-serif; font-size: 11pt; line-height: 107%;"></span><br />
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</span>
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<o:p><span style="font-family: "calibri";"> </span></o:p></div>
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<span style="font-family: "calibri";">
In some children with WS especially those with hyperopia, the kids have trouble
with that coordination effort resulting in estropia where the eyes aren’t
perfectly aligned and one turns in or out slightly.<span style="mso-spacerun: yes;"> </span>This is a picture of my daughter when we
first noticed her estropia.<span style="mso-spacerun: yes;"> </span>As a result,
the two eyes are no longer looking at the same object and they get double
images which further confuses the brain.<span style="mso-spacerun: yes;">
</span>If you notice this, it’s important to see an ophthalmologist immediately
because if you don’t, the brain will accommodate to its surrounds which leads
to bigger issues.<o:p></o:p></span></div>
<br />
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<o:p><span style="font-family: "calibri";"> </span></o:p></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<!--[if gte vml 1]><v:shape id="Picture_x0020_28"
o:spid="_x0000_s1043" type="#_x0000_t75" style='position:absolute;left:0;
text-align:left;margin-left:0;margin-top:176.45pt;width:183pt;height:132.15pt;
z-index:251685888;visibility:visible;mso-wrap-style:square;
mso-wrap-distance-left:9pt;mso-wrap-distance-top:0;mso-wrap-distance-right:9pt;
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<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image037.png"
o:title="" cropbottom="43375f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">So if estropia
persists the brain must chose only one of those images to focus on since the input
is so confusing, so it uses sensory accommodation and begins to ignore that
weaker eye’s input.<span style="mso-spacerun: yes;"> </span>It’s just like the
little experiment we just did.<span style="mso-spacerun: yes;"> </span>You moved
your ring to a new finger.<span style="mso-spacerun: yes;"> </span>At first it
most likely made you uncomfortable.<span style="mso-spacerun: yes;"> </span>The
feeling was out of the ordinary and your body took note of it but after a while
your body decided that it wasn’t worth your attention and now it probably doesn’t
bother you.<span style="mso-spacerun: yes;"> </span>That’s what your brain does
with the weak eye information.<span style="mso-spacerun: yes;"> </span>The brain
decides its better off without the info so it turns off the input or filters it
essentially making the person accomodatively blind in the weak eye!<span style="mso-spacerun: yes;"> </span>This can cause the loss of depth perception
and overtime can become irreversible if not treated.<o:p></o:p></span></div>
<div class="separator" style="clear: both; text-align: center;">
<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjZEAlgM6MK87Iu-jRDsCJ7BhYf6CSxH1mdNGGZk8J0akuWgyMKMY_uaY-tI79-ccNRP2O0uk08vy7VciZW4-qDE7Z8axDqpqswtyjIyVEoxKJ01tNhT1r3FpzmwJcJexB9teSnVm9xygll/s1600/Capture15.PNG" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjZEAlgM6MK87Iu-jRDsCJ7BhYf6CSxH1mdNGGZk8J0akuWgyMKMY_uaY-tI79-ccNRP2O0uk08vy7VciZW4-qDE7Z8axDqpqswtyjIyVEoxKJ01tNhT1r3FpzmwJcJexB9teSnVm9xygll/s640/Capture15.PNG" width="432" /></a></div>
<o:p><span style="font-family: "calibri";"></span></o:p><br />
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<!--[if gte vml 1]><v:shape id="Picture_x0020_29"
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mso-position-horizontal-relative:margin;mso-position-vertical:absolute;
mso-position-vertical-relative:margin'>
<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image037.png"
o:title="" croptop="21630f" cropbottom="22541f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">There are
essentially two types of estropia.<span style="mso-spacerun: yes;"> </span>This
is my daughter’s estropia a few months after we noticed it.<span style="mso-spacerun: yes;"> </span>You can see how much worse the weaker eye is
from the original photo.<span style="mso-spacerun: yes;"> </span>In this stage,
her brain began to lose interest in that sensory information and the motor
control fails causing the eye to fall in.<span style="mso-spacerun: yes;">
</span>This can become complicated by low muscle tone or hypotonia in the
muscles. <span style="mso-spacerun: yes;"> </span>This means the nervous system
fails to keep the muscle on alert in a slightly contracted state in order to
improve and be ready for contraction.<span style="mso-spacerun: yes;"> </span>In
order to repair this, the ophthalmologist will put the children on a patching regimen
where they cover the strong eye in order to force the brain to pay attention to
the weak eye signal and keep it functional.<span style="mso-spacerun: yes;">
</span>For some children, this will be enough to improve the eye strength.<span style="mso-spacerun: yes;"> </span>Some kids will also get glasses.<span style="mso-spacerun: yes;"> </span>Often if the child has hypotonia and far
sightedness together, the weak muscle tires and causes the eye to fatigue.<span style="mso-spacerun: yes;"> </span>By having glasses, the muscle is not worked
as hard to try and focus the image improving muscle stamina.<o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<!--[if gte vml 1]><v:shape id="Picture_x0020_30" o:spid="_x0000_s1041"
type="#_x0000_t75" style='position:absolute;margin-left:0;margin-top:0;
width:183pt;height:129.65pt;z-index:251687936;visibility:visible;
mso-wrap-style:square;mso-wrap-distance-left:9pt;mso-wrap-distance-top:0;
mso-wrap-distance-right:9pt;mso-wrap-distance-bottom:0;
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<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image037.png"
o:title="" croptop="43791f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEiltYQab46t3Uff4ln-aVFCYCTkTREgQJiIBseN8ZqJ1nA0_UsDKdomOq5XQnE4n-JNPFk1A1R5crH4WU7d7XF0Bg1JOxylReNYleIzdGwKaWS7AAOR_WVpvp66d9LUVUaEqLUTGsZ6Pz23/s1600/Capture14.PNG" imageanchor="1" style="clear: left; float: left; margin-bottom: 1em; margin-right: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEiltYQab46t3Uff4ln-aVFCYCTkTREgQJiIBseN8ZqJ1nA0_UsDKdomOq5XQnE4n-JNPFk1A1R5crH4WU7d7XF0Bg1JOxylReNYleIzdGwKaWS7AAOR_WVpvp66d9LUVUaEqLUTGsZ6Pz23/s640/Capture14.PNG" width="444" /></a><span style="font-family: "calibri";">There are some
situations where patching alone is not enough.<span style="mso-spacerun: yes;">
</span>My Katie had to have surgery on her eye muscles three times.<span style="mso-spacerun: yes;"> </span>In surgery, the surgeon reattaches the
hypotonic muscle in a different place on the eye in order to shorten it and
return alignment.<span style="mso-spacerun: yes;"> </span>It’s an imprecise
surgery so it does have a high likelihood that a repeat surgery is needed but
the recovery is very fast and its’ an outpatient procedure.<span style="mso-spacerun: yes;"> </span>My daughter on her final procedure had a
botox injection where the botox temporarily paralyzes the muscle allowing it to
lengthen and stretch and then when nervous control is restored it is adjusted
to the proper alignment.<span style="mso-spacerun: yes;"> </span>This is was enough
paired with patching and the glasses to correct my daughter’s eye alignment and
her vision is restored.<o:p></o:p></span></div>
<o:p><span style="font-family: "calibri";"></span></o:p><br />
<br />
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<div class="separator" style="clear: both; text-align: center;">
<br /></div>
<!--[if gte vml 1]><v:shape id="Picture_x0020_31"
o:spid="_x0000_s1040" type="#_x0000_t75" style='position:absolute;left:0;
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<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image041.png"
o:title="" cropbottom="43815f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">This muscle problem
can extend to other motor issues.<span style="mso-spacerun: yes;"> </span>Next
we’ll look at speech.<span style="mso-spacerun: yes;"> </span>In order to form
words, there are many muscles that the body must learn to manipulate and
contract in the proper ways to form intelligible words.<span style="mso-spacerun: yes;"> </span>The nervous system must coordinate the
muscles, contract them in the right way, move them in the proper range of
motion, control the speed of the lips and the tongue all independent of one
another.<span style="mso-spacerun: yes;"> </span>This can be a large task!<span style="mso-spacerun: yes;"> </span>This is one reason why our children have speech
delays and often fatigue after a while when speaking.<span style="mso-spacerun: yes;"> </span>Katie is working up her endurance but she’ll
often get some words or sentences out and then revert to babble.<span style="mso-spacerun: yes;"> </span>Her muscles and motor planning just can’t
keep up with her thoughts of what she wants to say.<span style="mso-spacerun: yes;"> </span>She does the same with music although she has
a bigger endurance when she sings, she often begins singing her own version of
the song because it’s a big task to coordinate those muscles and process what
to say next in a quick enough manner.<o:p></o:p></span></div>
<br />
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<o:p><span style="font-family: "calibri";"> </span></o:p><br />
<div class="separator" style="clear: both; text-align: center;">
<span style="font-family: "calibri";"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjPONHi_zRvur-qFPPp1Skr_HLfQebxnH_Y8ddtSdJUqBb4N2iXEEDCaDV4oyF5CxL0HfybSjv1wWMLHpYKvuEguBODlVeGkJiirsyta-XAATaq2gw4MlJX41_Umq_IUnxjamJdpm-gkwEi/s1600/Capture16.PNG" imageanchor="1" style="clear: left; float: left; margin-bottom: 1em; margin-right: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjPONHi_zRvur-qFPPp1Skr_HLfQebxnH_Y8ddtSdJUqBb4N2iXEEDCaDV4oyF5CxL0HfybSjv1wWMLHpYKvuEguBODlVeGkJiirsyta-XAATaq2gw4MlJX41_Umq_IUnxjamJdpm-gkwEi/s640/Capture16.PNG" width="438" /></a></span></div>
<br /></div>
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<!--[if gte vml 1]><v:shape id="Picture_x0020_32"
o:spid="_x0000_s1039" type="#_x0000_t75" style='position:absolute;left:0;
text-align:left;margin-left:0;margin-top:461.5pt;width:177pt;height:132.9pt;
z-index:251689984;visibility:visible;mso-wrap-style:square;
mso-wrap-distance-left:9pt;mso-wrap-distance-top:0;mso-wrap-distance-right:9pt;
mso-wrap-distance-bottom:0;mso-position-horizontal:left;
mso-position-horizontal-relative:margin;mso-position-vertical:absolute;
mso-position-vertical-relative:margin'>
<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image041.png"
o:title="" croptop="21724f" cropbottom="21564f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">So because our kids
have the stronger what or ventral stream, language eventually becomes a
strength but our kids need speech and language therapy in order to learn proper
motor output.<span style="mso-spacerun: yes;"> </span>Many of the language
strengths our kids have often cover up these weaknesses, especially as they age
so it’s not uncommon for them to lose some of their minutes to speech therapy
but there are areas that they still need help on even through adolescence.<span style="mso-spacerun: yes;"> </span>One of those is pragmatics- the art of
conversation where they listen to another person and go back and forth with information.<span style="mso-spacerun: yes;"> </span>Our kids tend to want to talk about what interests
them making friendships more difficult to maintain.<span style="mso-spacerun: yes;"> </span>With continued speech therapy, pragmatics can
be learned and our kids can have better social engagements, which they really
crave and prioritize.<o:p></o:p></span></div>
<br />
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<!--[if gte vml 1]><v:shape id="Picture_x0020_33"
o:spid="_x0000_s1038" type="#_x0000_t75" style='position:absolute;left:0;
text-align:left;margin-left:0;margin-top:0;width:177pt;height:128.05pt;
z-index:251691008;visibility:visible;mso-wrap-style:square;
mso-wrap-distance-left:9pt;mso-wrap-distance-top:0;mso-wrap-distance-right:9pt;
mso-wrap-distance-bottom:0;mso-position-horizontal:left;
mso-position-horizontal-relative:margin;mso-position-vertical:top;
mso-position-vertical-relative:margin'>
<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image041.png"
o:title="" croptop="44104f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">More with
motor.<span style="mso-spacerun: yes;"> </span>These are pictures of my Katie at
a very young age and we are just now seeing the issues related to them.<span style="mso-spacerun: yes;"> </span>Folding in half or siting in a W are a result
of the low muscle tone that our kids tend to have and they can turn into big
problems.<o:p></o:p></span></div>
<o:p><span style="font-family: "calibri";"></span></o:p><br />
<div class="separator" style="clear: both; text-align: center;">
<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEi0qTp3VOhRk2DqdHC_Em_e7Wq2RynuKB2ZY-AgSoDnsviuDlTSXQ5A1mDjdLW6EPOsX0eG4krUg8BjFGG7hZB_NKFturF61fFB4pJvbXD44BdIBPuoYXLLUYErM0vyjFv8JnzFHPDJOHGM/s1600/Capture17.PNG" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEi0qTp3VOhRk2DqdHC_Em_e7Wq2RynuKB2ZY-AgSoDnsviuDlTSXQ5A1mDjdLW6EPOsX0eG4krUg8BjFGG7hZB_NKFturF61fFB4pJvbXD44BdIBPuoYXLLUYErM0vyjFv8JnzFHPDJOHGM/s640/Capture17.PNG" width="428" /></a></div>
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<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<!--[if gte vml 1]><v:shape id="Picture_x0020_34"
o:spid="_x0000_s1037" type="#_x0000_t75" style='position:absolute;left:0;
text-align:left;margin-left:0;margin-top:145.5pt;width:181.5pt;height:127.35pt;
z-index:251692032;visibility:visible;mso-wrap-style:square;
mso-wrap-distance-left:9pt;mso-wrap-distance-top:0;mso-wrap-distance-right:9pt;
mso-wrap-distance-bottom:0;mso-position-horizontal:left;
mso-position-horizontal-relative:margin;mso-position-vertical:absolute;
mso-position-vertical-relative:margin'>
<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image045.png"
o:title="" cropbottom="43838f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">When our children
are young especially they have hypotonia which is related to the motor
planning.<span style="mso-spacerun: yes;"> </span>Weak muscles are less of a
muscle issue and more of a nervous system issue.<span style="mso-spacerun: yes;"> </span>They are due to misinterpretation of the
senses and poor communication between the nerve and the muscle creating relaxed
stretched muscles with higher movement.<span style="mso-spacerun: yes;">
</span>This creates delays in all things motor such as speech, feeding and
movement.<span style="mso-spacerun: yes;"> </span>Muscles, like the eyes are
also sensory organs.<span style="mso-spacerun: yes;"> </span>Many people don’t
realize this but we have tiny little receptors in the muscle that send back
information about where a muscle is contracted and where we are in space.<span style="mso-spacerun: yes;"> </span><o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
<!--[if gte vml 1]><v:shape id="Picture_x0020_35" o:spid="_x0000_s1036"
type="#_x0000_t75" style='position:absolute;margin-left:0;margin-top:316.75pt;
width:181.45pt;height:134.45pt;z-index:251693056;visibility:visible;
mso-wrap-style:square;mso-wrap-distance-left:9pt;mso-wrap-distance-top:0;
mso-wrap-distance-right:9pt;mso-wrap-distance-bottom:0;
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<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image045.png"
o:title="" croptop="21161f" cropbottom="21466f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">After time, the
brain begins to crave the sensory information from the muscle that it’s
missing.<span style="mso-spacerun: yes;"> </span>So, it uses muscles outside the
core in the peripheral areas of the body such as the arm and mainly the legs in
order to gain that sensory information from the proprioceptors.<span style="mso-spacerun: yes;"> </span>Early signs of this are dragging your fingers
against the wall or obstacles for balance and toe walking.<span style="mso-spacerun: yes;"> </span>When we toe walk, more specific information
is gathered from the balls and toes creating heightened awareness of balance
and our surrounding environment.<span style="mso-spacerun: yes;"> </span>It also
activates the vestibular apparatus of the inner ear amplifying information
about balance and helping us to gain more sensory info.<o:p></o:p></span></div>
<br />
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<div class="separator" style="clear: both; text-align: center;">
<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEixcUG8Bel2K5CF2thf42ZT7rUjO_LsBqUSQJV9FHi1_EbLsSFH7bk_bHUSDh3UPIgbhVYm_Kt8Sp6_aUNTXI6oS2JBMIa8WqlhmbTHAC5nkPVKD6W-Kmtnad5CpMeUswX4E4skiNSr1abM/s1600/Capture18.PNG" imageanchor="1" style="clear: left; float: left; margin-bottom: 1em; margin-right: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEixcUG8Bel2K5CF2thf42ZT7rUjO_LsBqUSQJV9FHi1_EbLsSFH7bk_bHUSDh3UPIgbhVYm_Kt8Sp6_aUNTXI6oS2JBMIa8WqlhmbTHAC5nkPVKD6W-Kmtnad5CpMeUswX4E4skiNSr1abM/s640/Capture18.PNG" width="438" /></a></div>
<!--[if gte vml 1]><v:shape id="Picture_x0020_36"
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mso-position-vertical-relative:margin'>
<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image045.png"
o:title="" croptop="43800f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><o:p></o:p><br /></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<span style="font-family: "calibri";">Toe walking can also be linking
with visual issues.<span style="mso-spacerun: yes;"> </span>Some children toe
walk to gain information that they can’t gather with their vision.<span style="mso-spacerun: yes;"> </span>With misaligned eyes, the body may get
inaccurate sense of where they are in the environment as slight forward causing
the body to want to lean forward in order to maintain balance which results in
toe walking.<span style="mso-spacerun: yes;"> </span>An ophthalmologist or optometrist
can often check for this and use prisms to correct the issue.<o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<o:p><span style="font-family: "calibri";"> </span></o:p></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<!--[if gte vml 1]><v:shape id="Picture_x0020_37"
o:spid="_x0000_s1034" type="#_x0000_t75" style='position:absolute;left:0;
text-align:left;margin-left:14.25pt;margin-top:.8pt;width:180.75pt;height:128.95pt;
z-index:251695104;visibility:visible;mso-wrap-style:square;
mso-wrap-distance-left:9pt;mso-wrap-distance-top:0;mso-wrap-distance-right:9pt;
mso-wrap-distance-bottom:0;mso-position-horizontal:absolute;
mso-position-horizontal-relative:margin;mso-position-vertical:absolute;
mso-position-vertical-relative:margin'>
<v:imagedata src="file:///C:/Users/MOONIE~1/AppData/Local/Temp/msohtmlclip1/01/clip_image049.png"
o:title="" cropbottom="43654f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">This overall low
tone can create big changes to the balance of muscle in the legs.<span style="mso-spacerun: yes;"> </span>They become asymmetrical.<span style="mso-spacerun: yes;"> </span>Muscles often work in pairs- when one muscle
such as the calf is in a contracted state, the opposite muscle in the shin
become relaxed.<span style="mso-spacerun: yes;"> </span>So when we have high
amounts of contraction in one muscle and overtime ignore the other, the muscles
are unevenly built creating balance and coordination issues and can lead to
joint issues.<span style="mso-spacerun: yes;"> </span><o:p></o:p></span></div>
<br />
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o:title="" croptop="21751f" cropbottom="21772f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><o:p></o:p><br /></div>
<br />
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<div class="separator" style="clear: both; text-align: center;">
<br /></div>
<span style="font-family: "calibri";">This imbalance to create a
condition called contractures which are something that is common in about 50%
of those with WS including my Katie pictured here.<span style="mso-spacerun: yes;"> </span>Due to toe walking her Achilles tendon became
short to the point that she couldn’t flatten her foot if she wanted to;
reducing her range of motion.<span style="mso-spacerun: yes;"> </span>As a result,
she ended up requiring surgery; lots of stretching and increased amount of PT
in order to increase range of motion and create better balance in her state of
contractions.<o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<o:p><span style="font-family: "calibri";"> </span></o:p><br />
<div class="separator" style="clear: both; text-align: center;">
<span style="font-family: "calibri";"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgq91swzinvmx7Zru5YTAlNoPcFxuN6eEvjJYGlvY1wNhLUEcVbFzxD_1gX_gWF3H5BcvYcXwcF6ZbpUxbQBGWJqUEuqUS1UVglzlcQjtLeztuvKITLzNmQEmaDPKGUSvA6E9PY9IHzjUX1/s1600/Capture19.PNG" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgq91swzinvmx7Zru5YTAlNoPcFxuN6eEvjJYGlvY1wNhLUEcVbFzxD_1gX_gWF3H5BcvYcXwcF6ZbpUxbQBGWJqUEuqUS1UVglzlcQjtLeztuvKITLzNmQEmaDPKGUSvA6E9PY9IHzjUX1/s640/Capture19.PNG" width="436" /></a></span></div>
<br /></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
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o:title="" croptop="43362f"/>
<w:wrap type="square" anchorx="margin" anchory="margin"/>
</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">So in the end, the
muscle aspect of this pathway is where we see the end results of all the
sensory and integration issues.<span style="mso-spacerun: yes;"> </span>By
understanding the full pathway, we can not only prevent some of the issues that
could occur such as the contractures but we can better understand our children’s
behaviors and provide sensory or integration accommodations to help them cope
or to stop problems before they require surgical intervention.<span style="mso-spacerun: yes;"> </span>For the entire pathway I might have a full
team of medical and educational professionals treating my child with myself being
the bridge between all of them in order to treat my child in full. <o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<o:p><span style="font-family: "calibri";"> </span></o:p></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
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</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">Which brings me to
the final portion of my presentation- how you can use all this knowledge to
become a better advocate.<span style="mso-spacerun: yes;"> </span>Because of my
background in anatomy and teaching, I’ve seen a different kind of perspective
when it comes to advocating for your child.<span style="mso-spacerun: yes;">
</span>I found that these are the three most important aspects of my
conversations with doctors and educators and because of these conversations, I’ve
formed unique relationships with the team and created more open lines of
communication.<span style="mso-spacerun: yes;"> </span>First, I use their language
to discuss my child.<span style="mso-spacerun: yes;"> </span>When you have
conversations with a doctor, use scientific terms if you can comfortably.<span style="mso-spacerun: yes;"> </span>They pick up on this and will offer up more information
than they originally would.<span style="mso-spacerun: yes;"> </span>The same
goes with education.<span style="mso-spacerun: yes;"> </span>If you use
educational terms you can often gain more accommodations or better set the
stage to get those resources, you want.<span style="mso-spacerun: yes;">
</span>I have an infographic that I’ve created with some of the language and
vocabulary used in this presentation that can help you in these
conversations.<span style="mso-spacerun: yes;"> </span></span><br />
<div class="separator" style="clear: both; text-align: center;">
<span style="font-family: "calibri";"><span style="mso-spacerun: yes;"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjovQfKJtxL9p3Jp3zWBPm2BImcYYlNbNyz57O3_5b8g-WZZWINhp4Rm2TocOXrnji9vKQ8NIae3WIcP8BxFwYnCbx1tTO-dxAc9RAldzGJBeczTpCypaulDyNYpUb7JE2k7zoRk-ZZ978O/s1600/Capture20.PNG" imageanchor="1" style="clear: left; float: left; margin-bottom: 1em; margin-right: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjovQfKJtxL9p3Jp3zWBPm2BImcYYlNbNyz57O3_5b8g-WZZWINhp4Rm2TocOXrnji9vKQ8NIae3WIcP8BxFwYnCbx1tTO-dxAc9RAldzGJBeczTpCypaulDyNYpUb7JE2k7zoRk-ZZ978O/s640/Capture20.PNG" width="436" /></a></span></span></div>
<span style="font-family: "calibri";"><o:p></o:p></span><br /></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<span style="font-family: "calibri";">Second you want to become a
resource.<span style="mso-spacerun: yes;"> </span>Offer things that the
professionals are interested in to better prepare them for their job related to
your child.<span style="mso-spacerun: yes;"> </span>Finally, the key to open up
lines of communication where you are a person who can share information will
make you a valuable part of the team.<o:p></o:p></span></div>
<br />
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<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<span style="font-family: "calibri";">So ways you can accomplish this are
to find professional articles which is what I write my blog posts based
on.<span style="mso-spacerun: yes;"> </span>The blog posts are for you to
understand what they say and then at the base of the page I have links to
resources used to find the info.<span style="mso-spacerun: yes;"> </span>The
professional articles listed there are great resources you can use to pass
along the information.<span style="mso-spacerun: yes;"> </span>I often print
them and give them to therapists, teachers and medical doctors.<span style="mso-spacerun: yes;"> </span>I just ask them if they’ve seen it and open
up a conversation about how it sounds like Katie’s behaviors or related to the
issues she has.<span style="mso-spacerun: yes;"> </span>For example, when it was
time for testing during Kindergarten I gave the school psychologist and speech
therapist Dr. Mervis articles on testing and speech development in order to
guide their choices.<span style="mso-spacerun: yes;"> </span><o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<span style="font-family: "calibri";">As parent your therapists and
doctors and educators often will not communicate with each other.<span style="mso-spacerun: yes;"> </span>You are the mediator so openly share the
medical and school info with the teams so that everyone is on the same
page.<span style="mso-spacerun: yes;"> </span></span><br />
<div class="separator" style="clear: both; text-align: center;">
<span style="font-family: "calibri";"><span style="mso-spacerun: yes;"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhMgB3cgjfHB3J06l_LTorPuLyZKGG46ggR-9XBTt3ZPD8tBZzJlCi33LwVZFfv4KT-zgiVZOUaI1K8BI0HRcQum9tBy16ALPuj-4EFxmAtXXr3dv73YPckrWaU3WlNuds6hcLFGgChg-yK/s1600/Capture21.PNG" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhMgB3cgjfHB3J06l_LTorPuLyZKGG46ggR-9XBTt3ZPD8tBZzJlCi33LwVZFfv4KT-zgiVZOUaI1K8BI0HRcQum9tBy16ALPuj-4EFxmAtXXr3dv73YPckrWaU3WlNuds6hcLFGgChg-yK/s640/Capture21.PNG" width="434" /></a></span></span></div>
<span style="font-family: "calibri";"><o:p></o:p></span><br /></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<span style="font-family: "calibri";">Especially for teachers, the
infographics and WSA tear away sheets are wonderful.<span style="mso-spacerun: yes;"> </span>Teachers are busy and have large caseloads so
easy to read, practical information is more likely to be read and studied than
long articles.<o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<o:p><span style="font-family: "calibri";"> </span></o:p></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
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</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">Another aspect of
advocacy that I’ve become hyperaware of is maintaining open communication.<span style="mso-spacerun: yes;"> </span>Sitting on both sides of the IEP table, I can
see where communication breaks down between case managers and parents when
emotion gets in the way.<span style="mso-spacerun: yes;"> </span>It really is a
social art to try and get what you want where tone and approach are so
important.<span style="mso-spacerun: yes;"> </span>You cannot just openly
request or demand things like paras or assistive technology.<span style="mso-spacerun: yes;"> </span>You have to think like a lawyer and show
evidence, set the stage for what you want.<span style="mso-spacerun: yes;">
</span>I’ve been able to get these resources and more for my daughter without
ever requesting them.<span style="mso-spacerun: yes;"> </span>I just make a case
for it and the logical solution is to provide that resource and they have
offered it.<span style="mso-spacerun: yes;"> </span>I’m not guaranteeing success
because every district and state is different, but its’ important to understand
that work with your team should always be your first approach to an IEP.<span style="mso-spacerun: yes;"> </span><o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<span style="font-family: "calibri";">As a teacher I have heard countless
conversations about parents who are unreasonable and I’ve found that the case
managers and teachers, like most people, who feel threatened will disconnect
from the child and become very defensive of their own decision making and
job.<span style="mso-spacerun: yes;"> </span>This is counteractive to your
overall goal of getting what is right for your child so its’ so important to
try as best as you can to remain emotionally neutral throughout the
meeting.<span style="mso-spacerun: yes;"> </span>I’m not saying you should walk
around as a brown noser but you should treat the professionals with respect because
you will in turn gain respect as well.<span style="mso-spacerun: yes;"> </span>I
make a point to thank them for their time, skills and sharing knowledge with me
so that I can better understand them and the decisions they make but in return
I’m also keeping them accountable by asking them to defend their choices in a
respectful manner.<span style="mso-spacerun: yes;"> </span>I also make it a
point to work with them instead of against them.<span style="mso-spacerun: yes;"> </span>When you offer valuable information and show
your knowledge, they will see you in a different way- as a resource that can
help them in their job rather than hinder.<span style="mso-spacerun: yes;">
</span>When emotions get high or difficult one way, as a teacher, that I’ve
learned to reduce the charge in the room is to keep the conversation child
centered.<span style="mso-spacerun: yes;"> </span>The child is why you are there
but all too often people begin to think more about themselves if they feel
threatened which is a natural response.<span style="mso-spacerun: yes;">
</span>If you return to phrases such as can we review how this will help my
child access the curriculum or how will this set my child up for success or how
can we help my child overcome this obstacle, you remove yourself and them from
the conversation and focus on what is important.<o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<span style="font-family: "calibri";">Some reasons why teachers and
professionals are not open to resources you want is because they aren’t
familiar or trained in them.<span style="mso-spacerun: yes;"> </span>When you
request things that are outside a teacher’s comfort zone they will often become
inflexible.<span style="mso-spacerun: yes;"> </span>I found this to be true with
my child’s special education teacher when I mentioned assistive technology.<span style="mso-spacerun: yes;"> </span>With encouragement and persistent requests
and information she became open to the idea and it ended up really transforming
her classroom by opening up the resource to many children including my
own.<span style="mso-spacerun: yes;"> </span>They say that in business you have
to pressure or present your product on average of 5 times before someone will
commit to the platform and I’ve found that in teaching this can be true as
well.<span style="mso-spacerun: yes;"> </span>As an advocate you can remain
respectful but also be offensive in this manner by repeating requests and
keeping open resources that point your teachers in the direction you want them
to go.<span style="mso-spacerun: yes;"> </span>Provide them with evidence and
resources that help make it attainable and practical.<o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<o:p><span style="font-family: "calibri";"> </span></o:p><br />
<div class="separator" style="clear: both; text-align: center;">
<span style="font-family: "calibri";"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEiUbI3-7Uq9ytuSMXfN0KrsbMgH2rbeNW7YJkxHAPm1wwxhWfACZSZpukWK8WqoP__mvw60fIvWVgLLwAMD3MPH6lQbTC3yaey1jFBulQayPufpYLfM4ky0raaIEspyCVmEMck0IEiCmmKr/s1600/Capture22.PNG" imageanchor="1" style="clear: left; float: left; margin-bottom: 1em; margin-right: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEiUbI3-7Uq9ytuSMXfN0KrsbMgH2rbeNW7YJkxHAPm1wwxhWfACZSZpukWK8WqoP__mvw60fIvWVgLLwAMD3MPH6lQbTC3yaey1jFBulQayPufpYLfM4ky0raaIEspyCVmEMck0IEiCmmKr/s640/Capture22.PNG" width="436" /></a></span></div>
<br /></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
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</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">Just like in
education, when you talk to medical professionals, the vocabulary that you use
can open of lines of communication.<span style="mso-spacerun: yes;"> </span>If
you feel comfortable, use scientific vocabulary.<span style="mso-spacerun: yes;"> </span>They will sense that you can handle more of
the details and you will learn much more about your child’s condition and tips
that can lead to better decision making.<span style="mso-spacerun: yes;">
</span>You should also ask for medical reports from your doctors so that you
can better share the information with teachers and therapists.<span style="mso-spacerun: yes;"> </span>This will help them better understand your
child’s condition and make better plans for treatment.<span style="mso-spacerun: yes;"> </span><o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<span style="font-family: "calibri";">The last portion, which can sometimes
be the hardest, is to ask questions.<span style="mso-spacerun: yes;"> </span>Be
open about treatment and procedures with multiple practitioners because they
often give you the best advice.<span style="mso-spacerun: yes;"> </span>When my
daughter was preparing for her surgery on her heel cords, I had a very eye
opening conversation with her cardiologist explaining the studies on anesthesia
and how to choose an anesthesiologist when you often think they are merely
assigned.<span style="mso-spacerun: yes;"> </span>He gave me advice on questions
to ask them in order to gauge their understanding of coronary stenosis and anesthesia
risk before undergoing the procedure and that I had the right to request different
anesthesiologist if I didn’t feel confident in their knowledge of WS.<span style="mso-spacerun: yes;"> </span><o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<span style="font-family: "calibri";">Don’t rely on the internet for
details.<span style="mso-spacerun: yes;"> </span>Go there to learn more but then
ask them about what you learn and its validity.<span style="mso-spacerun: yes;">
</span>Show them articles you find especially if they are related to your child’s
condition and are from reputable journals.<span style="mso-spacerun: yes;">
</span>Ask about treatment options and why they aren’t recommending
others.<span style="mso-spacerun: yes;"> </span>They typically have good reasons
why they chose what they chose and can defend those decisions and if they can’t
find a second opinion!<o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<o:p><span style="font-family: "calibri";"> </span></o:p></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt;">
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</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">As with medical
professionals talk is important.<span style="mso-spacerun: yes;">
</span>Establishing a relationship as soon as possible is ideal.<span style="mso-spacerun: yes;"> </span>As soon as my daughter is placed with a
teacher or therapist, I send them information about WS.<span style="mso-spacerun: yes;"> </span>If you can get it to them before school
begins you can tap into time they have to explore that without the pressures of
the start of school prep work.<span style="mso-spacerun: yes;"> </span>As a
teacher, I always loved when parents reached out to me early and gave me tips
on how to best engage my child.<span style="mso-spacerun: yes;"> </span>It
eliminates so many trial and error days in the classroom that would occur if I
had to figure all that out on my own.<span style="mso-spacerun: yes;">
</span>You’ll find that this open communication will often make the teacher’s
life easier and in turn they will be more likely to adjust schedules, resources
and transitions to accommodate your child because they have more power to do
that before the year begins.<o:p></o:p></span></div>
<br />
<div class="MsoNormal" style="margin: 0in 0in 8pt; text-indent: 0.5in;">
<o:p><span style="font-family: "calibri";"> </span></o:p><br />
<div class="separator" style="clear: both; text-align: center;">
<span style="font-family: "calibri";"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEiM9pHs0fzHcgU0PYU97MNfT497Ir6FjGT2cRs1eiKqUY9ZSaEzP013CoMp3Z-yRKiAwmAYz62kQXSaJ1P4wJXdGZlMk0Z0QJElaggtWuPHkc6EYJJuKP9YpfoMutK76jR6TUfSNKvmXIqh/s1600/Capture23.PNG" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;"><img border="0" height="640" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEiM9pHs0fzHcgU0PYU97MNfT497Ir6FjGT2cRs1eiKqUY9ZSaEzP013CoMp3Z-yRKiAwmAYz62kQXSaJ1P4wJXdGZlMk0Z0QJElaggtWuPHkc6EYJJuKP9YpfoMutK76jR6TUfSNKvmXIqh/s640/Capture23.PNG" width="430" /></a></span></div>
<br /></div>
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</v:shape><![endif]--><!--[if !vml]--><!--[endif]--><span style="font-family: "calibri";">Finally, the tricky
IEP meeting that stresses any parent out.<span style="mso-spacerun: yes;">
</span>It is important to come prepared to this meeting.<span style="mso-spacerun: yes;"> </span>The parent can arguably be the most important
person in the room because no one else knows your child as well as you do.<span style="mso-spacerun: yes;"> </span>I start by creating a skills, needs and
growth sheet.<span style="mso-spacerun: yes;"> </span>All IEPs will include a
section on your child’s strengths, weaknesses and parent statement.<span style="mso-spacerun: yes;"> </span>By taking the time to write al these down
early you will not feel like you have to come up with them on the spot and you’ll
have written documentations of your wishes that will go into the folder.<span style="mso-spacerun: yes;"> </span>I often prepare these along with links or
print outs of the professional articles and infographics and send them to the
case manager about 2 weeks before the meeting.<span style="mso-spacerun: yes;">
</span>They will be able to look this over, do the research and write better
goals because like you, time to reflect and think of these things before the
meeting will make them more throughout and developed.<span style="mso-spacerun: yes;"> </span><o:p></o:p></span></div>
<br />
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<span style="font-family: "calibri";">Another way I prepare for the IEP
is that I become aware of what children are doing at my child’s grade level and
I write those down on paper.<span style="mso-spacerun: yes;"> </span>I reiterate
over and over throughout the meeting that I want to find ways and shape my
daughter’s goals around accessing age appropriate curriculum materials.<span style="mso-spacerun: yes;"> </span>By knowing what that curriculum looks like
you can check off skills as your case manager goes through the goals and then
if there are missing skills you find important you can request to have them
added.<span style="mso-spacerun: yes;"> </span>If the professionals don’t feel
they are developmentally appropriate, open up conversations asking them for
evidence as to why and encourage them to find ways to make it accessible such
as with technology, paraprofessionals etc.<span style="mso-spacerun: yes;">
</span>I never outwardly ask for a para but by setting up the evidence to show
it would be a solution and an area of need, you will more likely be offered
it.<span style="mso-spacerun: yes;"> </span>It should always be centered around
your child’s needs not what the general population of WS children need.<span style="mso-spacerun: yes;"> </span>I have the professional opinion that every
child with WS is uniquely different.<span style="mso-spacerun: yes;"> </span>Because
special education is tailored toward your child you must be mindful of what is
right for your child and aim for that.<span style="mso-spacerun: yes;"> </span>You
should be knowledgeable of the options and flexible to try new things.<span style="mso-spacerun: yes;"> </span>If you show those traits your team often will
as well and you will form a strong working relationship.<span style="mso-spacerun: yes;"> </span>Finally show gratitude when your team is
flexible, uses your resources and shows a desire to go above and beyond.<span style="mso-spacerun: yes;"> </span>It will only make your relationship stronger
for future problem solving and negotiations.<o:p></o:p></span></div>
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<span style="font-family: "calibri";">Resources to share with experts or use at home:<o:p></o:p></span></div>
<ul>
<li><div style="clear: both; text-align: left;">
<a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;" target="_blank" unselectable="on"></a><span style="font-family: "calibri";">Infographics- terms for parent advocacy and visual spatial strategies for teachers: <a href="https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU">https://drive.google.com/drive/folders/0B3vIN5__SQqLVU1xZGtqYThPdVU</a></span></div>
</li>
<li><a href="http://www.schoolmentalhealth.org/Resources/Clin/QuickGuide.pdf"><span style="color: #0563c1; font-family: "calibri";">Practical guide to helping your child overcome anxiety and more</span></a><o:p></o:p></li>
<li><a href="https://research.gold.ac.uk/6724/1/Cowie_WS_Revised6_PlusFigsforGRO.pdf"><span style="color: #0563c1; font-family: "calibri";">Visually guided step decent in children with WS</span></a><o:p></o:p></li>
<li><a href="https://williams-syndrome.org/sites/williams-syndrome.org/files/meyer-lindenbergreview_0.pdf"><span style="color: #0563c1; font-family: "calibri";">Neural mechanisms in WS</span></a></li>
<li><a href="http://lcn.salk.edu/publications/2003/Hirota%20Korenberg%20-%20gtf2i%20geninmed%202003.pdf" target="_blank">Visual spatial deficits related to WS genetics article<o:p></o:p></a></li>
<li><a href="http://www.academia.edu/437927/Spatial_Deficits_In_Children_With_Williams_Syndrome" target="_blank">Spatial deficits in children with WS academic article by Bellugi</a></li>
<li><a href="http://revistas.javeriana.edu.co/index.php/revPsycho/article/viewFile/2610/4853" target="_blank">Assessment of intellectual and visual spatial abilities in WS</a></li>
<li><a href="http://lcn.salk.edu/publications/2006/doron_gothelf-hyperacusis.pdf" target="_blank">Hyperacusis in Williams syndrome</a></li>
<li><a href="http://daniellevitin.com/levitinlab/articles/2005-Levitin-JCP.pdf" target="_blank">Hyperacusis- fears, awareness and attraction professional article</a></li>
<li><a href="http://www.sciencedirect.com/science/article/pii/S0028393214004527" target="_blank">Oromotor praxis in WS</a></li>
<li>Therapy and educational <a href="https://williams-syndrome.org/sites/williams-syndrome.org/files/TherapyFactSheetsFINAL.pdf" target="_blank">sample goals sheet from the WSA</a></li>
<li><a href="file:///C:/Users/mooniersarah/Downloads/Williams+Syndrome+2014.pdf" target="_blank">Parent and educator strategies for students with WS</a></li>
</ul>
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<o:p><span style="font-family: "calibri";"> </span></o:p></div>
Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com0tag:blogger.com,1999:blog-7345537858373518781.post-73693880568426294942016-03-16T16:19:00.000-07:002016-03-17T10:51:11.620-07:00Preparing your child for medical proceduresMy daughter recently underwent surgery and I was anxious about how she would respond to a hospital stay with various tests and procedures. After doing some research, I learned some strategies you can use to help your child understand what will happen at the hospital and tips on reducing your child's and your own anxiety. Here are the resources and strategies I used:<br />
<br />
<span style="color: #3d85c6;"><span style="font-size: large;">Prepare yourself</span></span><br />
<span style="color: #3d85c6;"><span style="font-size: large;"><span style="color: black;"><span style="font-size: small;">Before you communicate with your child about the procedure, you need to prepare yourself. Children can sense anxiety and worry. If you are worried, they'll pick up on that easily and in turn become worried themselves. It's important for you to become educated about your child's condition and the procedure so that you are comfortable talking about it and giving them honest answers to their questions. It's important for you to be knowledgeable and open with your child so that they develop trust. It's best not to have unexpected surprises or to give them false information about what will happen. This can create fearful or negative experiences.</span></span></span></span><br />
<br />
<span style="color: #3d85c6;"><span style="font-size: large;"><span style="color: black;"><span style="font-size: small;"><a href="http://kc.vanderbilt.edu/kennedy_files/ParentGuide.pdf" target="_blank"><span style="color: black;">Resource: </span>Taking the work out of blood work </a></span></span></span></span><br />
<span style="color: #3d85c6;"><span style="font-size: large;"><span style="color: black;"><span style="font-size: small;"> </span></span> </span></span><br />
<span style="color: #3d85c6;"><span style="font-size: large;">Prepare your child with play</span></span><br />
<br />
Role play can be an ideal way to explain what will happen to children in terms they can understand. I used a social story made by the Cleveland Children's hospital to direct my daughter's play. You can print the pdf and let your child color on it if they'd like. Katie has never been interested in coloring so we used it as a story and went through all the procedures with a stuffed animal of her choice. We acted out each step with a toy doctors kit and Katie played doctor. After I read each page, she'd act it out by asking her animal questions and explaining what would happen. It helped her understand what she'd experience when undergoing surgery. We even made a pulse ox monitor, IV and wrapped her kitty with gauze and duct tape to simulate the casts that Katie was going to get. We signed Kitty's casts and took her to the hospital with us. The nurse there gave her Kitty a hospital bracelet to match Kate's. No matter what procedure you are preparing for, there are many social stories on the internet that you can find and use. Role play is a wonderful way to prepare your child for the procedure they'll undergo.<br />
<br />
<div class="separator" style="clear: both; text-align: center;">
<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEiUYN85V5_xvdN4e-cb6oWEwEkoA55tvWKTX2W-BKUOPv1heyoMN8CMhc0XcO13WdZ76yz0nqQlZW0QyMrfJybA9NP21QKsN0dl8UmsiHheGiZXa74IXEj-lkGrYCVb-ZxIuNgcqtcgOY6Z/s1600/20160316_175928.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="360" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEiUYN85V5_xvdN4e-cb6oWEwEkoA55tvWKTX2W-BKUOPv1heyoMN8CMhc0XcO13WdZ76yz0nqQlZW0QyMrfJybA9NP21QKsN0dl8UmsiHheGiZXa74IXEj-lkGrYCVb-ZxIuNgcqtcgOY6Z/s640/20160316_175928.jpg" width="640" /></a></div>
<div style="text-align: center;">
Katie's role play with Kitty</div>
<br />
<a href="http://my.clevelandclinic.org/ccf/media/Files/Social_Work/SurgeryBookChildLife.pdf" target="_blank">Resource: My going to surgery book</a><br />
<br />
<span style="color: #3d85c6;"><span style="font-size: large;">Read books that show a happy ending</span></span><br />
<br />
Since Katie was getting casts on her legs, I found a couple books with related stories. One was a Doc McStuffins book about a toy that hurt her leg and needed a cast. When the cast came off she could dance again. Katie responded very well to this- even asking the day following surgery if she can take the cast off so she can dance :) However you chose to explain the procedure, be sure they understand WHY they are doing this. Knowing the surgery will help them walk or the heart doctor will make sure they have energy to run and play will give your child a tangible reason why they should tolerate the procedure.<br />
<br />
<a href="http://media.chop.edu/data/files/pdfs/surgery-guide-surgery-helpful-books-to-prepare-your-child.pdf" target="_blank">Here is a list of books suitable for preparing your child for hospital visits.</a> There are often resource libraries at your local hospital that you can use to check out books on these topics. The<a href="http://www.stlouischildrens.org/health-resources/family-resource-center" target="_blank"> family resource center at St. Louis Children's Hospital </a>is staffed with nurses that can help you find resources for both parents and children to help you prepare and become more educated on your child's condition.<br />
<br />
<span style="color: #3d85c6;"><span style="font-size: large;">Search for social stories videos and apps</span></span><br />
<br />
Youtube has many videos of procedures that are made for kids to see what they can expect. Preview these videos to make sure the content is appropriate! There are also many apps for apple and android that allow you to build your own stories. You can take pictures of the hospital during a visit of the tests or tools that instill anxiety and help your child view them so they become comfortable with the environment. This is a great way to help them ask questions and slowly become acclimated to the objects that are their source of anxiety. It can be especially helpful if your child has a procedure that they have to do often and don't tolerate. For example, if they don't tolerate the echocardiogram, take pictures when you go of the room, equipment, doctors and nurses and let them look at them often. It'll help make them more comfortable in the long run.<br />
<a href="http://www.friendshipcircle.org/apps/browse/?filter_category=19&query_type_category=or" target="_blank"><br /></a>
<a href="http://www.friendshipcircle.org/apps/browse/?filter_category=19&query_type_category=or" target="_blank">Apps that are helpful to children with special needs</a><br />
<br />
<span style="color: #3d85c6;"><span style="font-size: large;">Prepare your child for painful procedures</span></span><br />
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Don't tell your child that a procedure won't hurt if it will. You can acquaint them with the pain scale so they can better communicate their feelings. If they are getting blood drawn or an IV, you should explain that it'll feel a pinch and then the pain will go away. I counted with Katie during these procedures and told her that she'll feel better when we count to 20. This helps. If your child has anxiety over procedures like blood pressure measurements, use visual cues to help them control their anxiety. Katie went through a phase where the blood pressure cuff would put her in a panic. I told her to be brave and calm and showed her the gauge on the blood pressure cuff so she can see the needle sink. I told her she was getting a tight hug on her arm and then we could watch the needle go down and when it got to the bottom she'd be finished and could cry if she wanted to. It worked! She stayed quiet during the reading and then cried when it was finished but after a few times doing this, she tolerates the screening. <br />
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<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgmDAvWUdf3vFo2zyPxP2NdKPwiZLsJQGLtMxhjza8iwX1HYykvRhEJzrCTKb5MtYrsBsgT-1JnsGdnYkvz2NGmBJCUFJBFDCotNQGZ4lKBj7myhyU7BvJjJll6zsVXHDPqMwQalUPdPSbo/s1600/pain-measurement-scale.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="344" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgmDAvWUdf3vFo2zyPxP2NdKPwiZLsJQGLtMxhjza8iwX1HYykvRhEJzrCTKb5MtYrsBsgT-1JnsGdnYkvz2NGmBJCUFJBFDCotNQGZ4lKBj7myhyU7BvJjJll6zsVXHDPqMwQalUPdPSbo/s640/pain-measurement-scale.jpg" width="640" /></a></div>
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<span style="color: #3d85c6;"><span style="font-size: large;">Take a tour of the hospital</span></span><br />
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If you live close enough, visiting the hospital and doctors that are involved can help alleviate anxiety of being in a new and unfamiliar place. Especially if your child is undergoing anesthesia, a pre-surgical visit will be important to reduce risks and help your child become acquainted with the location. Some hospitals even have pictures and virtual tours you can access on their website.<br />
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<span style="color: #3d85c6;"><span style="font-size: large;">Access the Child Life Services</span></span><br />
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Most hospitals have Child life coaches who are trained to help explain to children what will happen in their own terms. They have games, sample tools and distraction materials you can use before, during and after procedures. They are important resources for parents, too! They are trained to answer questions in easy terms and to relieve anxiety for families.<br />
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<span style="color: #3d85c6;"><span style="font-size: large;">Bring a comfort item</span></span><br />
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Whether is a tablet to watch a favorite show or a stuffed animal to snuggle with, your child will feel better having items that comfort them and are familiar from home. If your child is anything like mine it's a ukulele or a drum :)<br />
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<span style="color: #3d85c6;"><span style="font-size: large;">Listen and Watch your child</span></span><br />
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Many of our kids have trouble expressing their thoughts and feelings so be their advocate. Watch for signs of pain or fear and using a calming voice and touch to help relieve their anxiety and fears. Katie was abnormally withdrawn and quiet during her hospital visit. She was quiet but based on her behaviors I knew when she was frightened or hurting. I'd give her an opportunity to speak for herself but eventually would translate her nonverbal cues for the nurses and doctors. You know your child better than anyone so it's important to be with them the entire time and speak for them when they can't speak for themselves. If your child is older, you can coach them to speak for themselves. Many of the special needs high school students I work with have trouble with this. It makes a great IEP goal that can benefit them for a lifetime.<br />
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<span style="color: #3d85c6;"><span style="font-size: large;">Seek support</span></span><br />
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No matter what you are facing, building a community of support will always make it easier. If you keep the procedures a secret you are putting a lot of pressure on yourself to carry the weight. I have found that throughout my experiences with Katie, the more I share with others, the more my community surprises me with encouragement and help. If sharing makes you uncomfortable, start small with your partner, trusted friends and family. I have found that talking to my child's teachers and therapists have been very beneficial. They are often experts in these situations and a source of great advice, encouragement and resources. They also spend a lot of time with your child so they can watch for signs of anxiety and help them in times of need. Another wonderful resource are the facebook support groups. Finding a closed group gives you opportunity to voice concerns, ask for prayers or encouragement and talk to people who are wearing your shoes. As the years have gone by I'm more and more open with our experiences and find that sharing provides me with people resources, professional resources and a phone filled with encouraging messages. Whatever level of support you seek, don't enter a stressful situation alone. It's very liberating to ask for help and gratefully take on the support.<br />
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<a href="https://www.facebook.com/groups/WS.Support/permalink/1153708691306246/?comment_id=1153840541293061&notif_t=group_comment_reply" target="_blank">WS support page</a>- affiliated with the Williams Syndrome Association<br />
<a href="https://www.facebook.com/groups/177212419068234/" target="_blank">Williams syndrome Hope support group</a><br />
<a href="https://www.facebook.com/groups/948459265222733/" target="_blank">WS Moms in prayer</a><br />
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Other sources that helped me prepare for medical procedures:<br />
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<a href="http://www.friendshipcircle.org/blog/2013/08/06/10-ways-to-help-prepare-your-special-needs-child-for-a-hospital-emergency-visit/" target="_blank">Friendship circle blog for special needs parents</a><br />
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<a href="https://www.childlife.org/The%20Child%20Life%20Profession/" target="_blank">Child life specialists </a><br />
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<a href="http://www.stlouischildrens.org/our-services/family-services/child-life-services/child-life-specialists" target="_blank">Child life specialist services at St. Louis Children's Hospital</a><br />
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<a href="https://williams-syndrome.org/TreatingFearsandPhobias" target="_blank">Treating fears and phobias by Dr. Pober </a>the Replay approach to alleviating anxiety during medical exams.<br />
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If you have any other approaches to help your child understand their medical procedure, feel free to share below in the comments! <br />
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<br />Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com1tag:blogger.com,1999:blog-7345537858373518781.post-11516627444347731442016-02-24T15:29:00.000-08:002016-02-24T15:29:11.296-08:00Navigating the School System- KindergartenAs my daughter enters her fourth semester of Kindergarten, I've been reflecting on many of the experiences. In the beginning I wished there existed a guide for new parents on what to expect. This post is my guide for you now that I've nearly completed the special needs Kindergarten experience.<br />
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Before I begin, I just want to point out that school systems vary from state to state (in different countries, of course) and even from city to city and district to district, so the meetings I've had may be different than yours. My family lives in Missouri.<br />
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<h2>
Getting started...</h2>
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Believe it or not, Kindergarten begins in preschool. That may sound ridiculous but the planning it takes to be ready for your child's entry will start around January of the previous school year. School districts vary but you'll need to register your child around that time of year. I began registering Katie just like she was planning on attending regular education classes. This will get the process rolling. At this point, you may have many questions and doubts about what services your child will need but those conversations will come later. One question you might ask is about Kindergarten testing. Most kindergartners undergo readiness testing to give teachers baseline data about their pre-academic skills. If your child already has an IEP (individualized educational plan), they may not be necessary. I went ahead and signed Katie up but then talked to the Kindergarten teacher at her school about opting out. Your child's Kindergarten teacher will learn much more about your child's readiness through an IEP than in that testing.<br />
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Later in Kindergarten, your child will go through various intelligent testing, developmental assessments, and tests to determine their need for additional therapy. These are separate from the school-wide kindergarten testing that all Kindergartners take when entering the elementary school.<br />
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<h2>
Creating the IEP:</h2>
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After registration, you'll expect to have an IEP meeting at some point in the spring to determine your child's placement. Some districts might do testing depending on your child's cycle. This will be a big meeting. You should expect to have the typical team there- the principal, preschool team (preschool teacher and all their preschool therapists) but you'll also have representatives from your child's school age team. We had a Kindergarten regular ed teacher, School age special education teacher, occupational therapist, physical therapist, speech therapist, resource teacher and a music therapist. You will also have a case manager from the department of special education who will lead the meeting and if your child was tested, you may have a diagnostician as well.<br />
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Because there are so many new faces who have never met you or your child, it's smart to come prepared with something that the team can use to acquaint them with your child. I created a "Meet Katie" flyer. Here are some things you might want to consider including on your meet sheet:<br />
<ul>
<li> Several pictures of your child- pick ones that show your child's personality. This was the most popular part of my child's IEP meeting (news flash- our kids are cute!) Seeing her sweet face makes the people in the room want to meet her and work with her. It keeps your child's best interests in mind and makes the meeting more personal. It is important to keep it child centered and the pictures will help accomplish that. </li>
<li>Your child's likes and dislikes- this will save your educators time figuring your child out but will also give them ideas of ways to motivate them and write appropriate goals. This is an important part of planning.</li>
<li>Academic and personality strengths- your IEP team will want to monopolize on your child's strengths. By knowing things like if they love to listen to music or that they learn well by singing, they can use those things to give your child access to the curriculum.</li>
<li>Academic weaknesses or personality challenges- Many times these will help shape the goals your team writes. You want them to work on the challenges to help your child overcome them.</li>
<li>A condensed medical bio with information that is specific to her school setting- if your child is like mine, the medical information could be a textbook so for this purpose, stick to the important things that will affect them academically. You'll have opportunities to discuss the medical issues later and many of that information may already be recorded in their records. Examples of things I shared are allergies and visual needs and muscle tone.</li>
<li>List of concerns- Write down all your worries for the new school year. Most of mine were centered around safety and personal care such as potty training, roaming on the playground, and physical education and playground safety. If you want your child to have a one-on-one aide or paraprofessional, this is the information that will be important in determining that. I never came out and requested that. I just set the stage for the obvious importance of it and let them suggest it. </li>
<li>Parent wishes we have for her kindergarten setting and future- This was the part I wasn't really ready for but it's really important. Think about what you want for your child. It's really easy to get lost in the details of what they need today and overlook the big picture. Think about your child's interests and how that might influence important skills if they turn those interests into career choices. Also think about the level of support your child will need to access regular education classes. Inclusion is very important and something you might want to ask for in regards to shaping her goals and support.</li>
</ul>
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This "meet" sheet can be an important tool that you can use in order to gain the support you want for your child. For example, some teachers may not be familiar with the growing resources available in assistive technology. If this is something you want for your child, tell them about how you might use a tablet at home to motivate your child for potty training or picky eating or that the tablet is used at home to help them practice their writing skills. Spelling out small things that you know are successful for your child in this sheet will open up dialogue about how the school can extend those "tricks" to the classroom and improve their chance for success. I also used the sheet to get a paraprofessional aide for my daughter. I didn't spell out that it was my goal to have a 1:1, but I told them about how my daughter has wandered away from our house when unsupervised before or that she has trouble navigating on the playground to the point that it's dangerous for her to climb and slide without support or that she walks very slowly and tires easily. These are all setting the stage to show the importance of an aide. The team actually ended up suggesting it first before I even had to ask.</div>
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In addition to my child's meet sheet, I also came with tear aways from the WSA website, professional articles that might interest the team and the infographic I created in a previous post to share great strategies for helping kids with WS. I had very good reviews from the team over these materials. Not only does it provide them with a good idea of who your child is and what they can do but it also gives them research driven practices, something teachers value.<br />
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<a href="https://williams-syndrome.org/teacher/information-for-teachers" target="_blank">Williams syndrome association website- Information for Educators</a><br />
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<a href="https://williams-syndrome.org/resource-library" target="_blank">Williams syndrome association website- Resources for Education and IEPs</a><br />
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<a href="http://understandingwilliamssyndrome.blogspot.com/2014/08/back-to-school-tips-for-teachers.html" target="_blank">Understanding Williams syndrome infographics for teachers</a><br />
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The final bring along is not a material thing, but an advocate. Often these meetings can feel surreal and stressful and it helps to have someone in the room who is not committed to either side who listens objectively and is familiar with the IEP process. They can point out overlooked needs and concerns and act as a mediator. I brought our case manager from the Department of Mental Health.<br />
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<h2>
The meeting...</h2>
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In the meeting, you'll discuss the child's success and progress in the preschool setting. You'll probably move around the room from teachers to therapists to share their goals and recent data they collected. Then if you are due for an IEP update in the preschool setting you might set or revise those existing goals. From there, you'll move on to the school age team. They'll build on the preschool goals but add in some others using Kindergarten curriculum in mind. Many parents entering Kindergarten have trouble coming up with goals they want for their child. For this portion, I would come prepared with a copy of the regular education curriculum. I made a list of the curricular goals and starred the ones that were appropriate topics for my daughter developmentally or ones that had skills she needed work on to bridge her access to those goals. Then as the team went through their prepared goal ideas, I'd check them off. When you reach the end, you can bring up any that they didn't discuss. This helped me keep my priorities in mind so when I got home I didn't feel like something important was overlooked. Also, don't forget to think of self care needs that will help your child make their way through the school day more independently. Examples include zipping their jacket, navigating stairs, playing on equipment, participating in PE, carrying a lunch tray and using the restroom. <br />
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To find the General education curriculum for Kindergarten, I used my school's website. They make the curricular goals public on the website for parents to see. If you can't find that, you can contact the school before your IEP and ask them for a curriculum handout and/or copy of the Kindergarten report card. Using the grade level expectations to make your goals is smart. Curriculum is carefully written by a team of teacher experts and is aligned with developmental growth and preparation for future grades. Even if my child may not fully meet all those goals by the end of the Kindergarten school year, those are standards I want her to reach in her own developmental time. <br />
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Here are common Kindergarten goal progressions in my child's school district that you can use as a guideline. We started with the standards listed early in the list and then as she learned those, progressed into the more advance skills later on the list. In my daughter's second year of Kindergarten, she focused on the skills at the later parts of the list.<br />
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<ul>
<li>Math: counting to 100, Writing to 31 (may be part of an OT goal also), counting objects, comparing numbers 0-31 such as greater than less than (a spatial task that may need special education support), adding and subtraction, patterns, sort graphing, position words (ST goal as well)</li>
<li>Reading: Phonics, letter recognition, tracking print, writing full name (also an OT goal), parts of a book, rhyming word families, counting syllables, blending sounds to read, vocabulary- opposites (also a ST goal), recall and comprehension, making inferences in text, writing alphabet (focus on lowercase first), sight words (we have been very unsuccessful with this)</li>
<li>Physical education: walking, hopping, skip, gallop, jump, run, slide, throw, catch, striking, kicking, dribbling, hand/foot coordination and motor planning. We focused goals on many spatial activities to build her balance and confidence with motor planning so she could participate in PE such as visual tracking items and balance and coordination. We also made goals on following multi-step directions and following rules of a game.</li>
</ul>
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In addition to these academic goals we had self care and social pragmatic goals. These include using buttons, scissors, snaps, zippers and answering wh- questions appropriately, and social conversation skills.</div>
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<h2>
Placement:</h2>
After determining the goals, the team will discuss the appropriate placement for your child. You will want to use terms such as least restrictive environment, inclusion and meeting your child's needs. It's important for you to create a balance between your child's need for independent or small groups settings and inclusion in a regular education setting. It's important to understand, your child can have their own individualized mixture of these throughout their day. This placement will be a very individualized decision but here are some potential options. <br />
<ul>
<li>Full inclusion in a regular classroom</li>
<li>Full inclusion with a paraprofessional aide and/or push-ins of special education teachers and therapists.</li>
<li>Participation in a regular education classroom with pull outs with a resource teacher who works one-on-one over IEP goals. </li>
<li>Participation in a regular ed classroom with pull outs for therapy or time for individualized instruction in a special education classroom. </li>
<li>Full day special education classroom</li>
</ul>
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While full inclusion can be the ultimate goal, you may find that your child really responds well to individualized instruction. Ultimately we chose partial inclusion with pull outs for therapy and individualized instruction in reading, writing and math since Katie's skills are below grade level and she shows more growth in individualized instruction in these areas. It's important to remember that what ever you chose, there is no wrong choice as long as you have your child's best interest in mind. You can always change those minutes based on their needs. For example, my daughter responded well to the social activities that supported her vocabulary and social pragmatics goals so they moved her from individualized speech and occupational therapy to a group session and her growth has been substantial. These choices can be fluid and can be changed anytime in the year with the request for an IEP revision from the parent or teacher.</div>
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<h2>
Before school begins...</h2>
Sometime around August you'll learn who the teachers and therapists your child is placed with. I used this time to send them an email and give them copies of all the meet sheets, infographics and WSA information. This will give them places they can go to learn more about WS and plan their instruction. This is a great time of year to contact teachers because they are refreshed and excited for their new students. There is plenty of time for them to read about research since their schedules haven't been filled with the endless school year tasks. It's also really nice as a teacher to understand their students before they even meet them. It'll reduce the transition time where they are trying to figure your child out and get to know them. It is not uncommon, especially if you belong to a large school district that the people at the transition IEP are different than the professionals your child ends up working with. It's important to establish a good, professional relationship with them early. It will open up easy lines of communication and provide them with resources in case this is the first time they have worked with a child with Williams syndrome.<br />
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<h2>
Managing school wide events:</h2>
One of the hardest parts of school transition for me was school events. Nights like open house, fall festivals, field trips, etc. are a whole other source of stress and trepidation because your child has those special needs and may not be able to fully or independently participate in those situations. Never fear, you can become an advocate for them so they are fully integrated and you can become an important part of your school community. It takes a little work and a lot of talking, however. <br />
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To start, within the first month of school you'll have open house. For me, this was a really difficult night. If your child is in a regular education setting for at least part of the day, you'll hear about things they will do that you don't feel ready for yet. It's also hard to hear about things your child might miss because they are in therapy or in the special ed setting. I found that it's much easier to talk to your child's regular education teacher separately so you can get a better sense of their day versus everyone else's schedule. This is also a good time to really express what expectations you have for your child to participate in. For example, my daughter is highly motivated by social opportunities so when she would have missed the "carpet time" where the whole class participates in calendar, story telling, weather lessons, etc, she was missing out on a very motivating activity for her. Her teacher happily switched her daily schedule around to include Katie in this activity. That kind of minor change probably wouldn't happen if you as a parent don't express to the teachers what your child needs. It's important to say, I didn't outright ask for this, it was just implied through open dialogue based on Katie's needs.<br />
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Field trips and school community events can also be a source of stress. I do everything I can to participate in these and help modify the setting in simple ways to make it fully inclusive. When there is a carnival game at the school festival and Katie couldn't possibly throw the ball that far, I would simply talk to the game supervisor and explain that she has special needs and would be more successful if she could step closer to the game. If she's at a field trip at the crowded zoo, be sure she has a school staff member there in her group to specifically keep an eye on her. I've even volunteered to come and managed to have two adults to my one child. All it takes is thinking about what issues you might encounter and being proactive to your child's needs by bringing those issue up in advance.<br />
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I will note that although your child may have difficulties in these situations, it is not worth missing them! Your involvement with the school community allows your family and your child to become closer to the people she sees on a daily basis and by making those strong connections, the more opportunities and support you will find as people grow to know and love your kid and your family. I've made a big effort to get my daughter involved in typical community activities such as scouts, after school clubs and school events in additional to special ed activities. I've found that people are very accommodating and it quickly spreads awareness and understanding for others. It's also important to note that parent involvement is important. Although we all need the respite, scouts may not be the best place to drop your kid off so you can take a break. I always volunteer to help my child during those meetings if she needs the one on one attention. Most after school activities are not going to have the same adult support as expected in school. I always make a point to attend until I can establish that my child will be independent enough not to put extra stress on volunteers such as scout parents.<br />
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<h2>
Reporting </h2>
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Depending on how your IEP goals are set up, reports from school can come in various ways. For a while, my daughter was sent home a daily behavioral report that on every day said "she was happy! She had happy hands! (didn't touch others or hit) She worked well! She had a great day!" This didn't help me understand her academic growth or truly understand what she should work on at home. By telling my daughter's teachers this, we changed up reporting by getting some simple tasks we could do at home to help support her school day. This doesn't mean homework, necessarily, but would include things like stretching, letters she's working on that we can point out in books when we read or topics aligned to games she can play on the tablet.<br />
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Report cards are another way you'll learn about your child's progress. Katie has a modified curriculum. She's basically graded by her goals in her IEP, not the standards on a report card. The only thing she is graded on is behavioral expectations by her general education teacher. The special ed teacher and her therapists report on her academic growth via IEP benchmark reports. She receives quarterly reports about her progress towards those goals. Your child's reporting may be different depending on their goals and the level of inclusion they have in the school setting.<br />
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<h2>
Getting a School Age Diagnosis</h2>
Some time within the school year your child will most likely undergo school age diagnostics. Your child will undergo a series of developmental measures in order to get a school age diagnosis. A school psychologist will contact you to set up testing and send home surveys for you to fill out. They'll most likely determine your child's IQ based on one of the psychology measures. I provided her with information from the Williams Syndrome Association website on academic testing so she picked the appropriate measurement tool for assessing children with WS. I also provided the speech therapist the academic journals on testing for speech and language. Both people were very interested in this literature and used it to make the best choices for their testing and analysis methods.<br />
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<a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3383614/" target="_blank">Dr. Mervis' published paper on Williams syndrome speech development</a><br />
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<a href="https://williams-syndrome.org/teacher/testing-evaluation-strategies" target="_blank">Williams syndrome association- Testing and evaluation strategies</a><br />
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<a href="https://www.princeton.edu/~adele/LIN_106:_UCB_files/Bellugi_JCNS_2000.pdf" target="_blank">Neurocognitive profile of Williams Syndrome</a><br />
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<h2>
School Age IEP</h2>
After your school age diagnosis has been determined, the full team will meet to go over the results and reevaluate eligibility of services. Often your services will remain the same and other times you might find there will be adjustments made in the minutes offered in various therapy and special education settings. Depending on the type of measure they use to determine your child's IQ, the data will vary. It's important to focus on the subtest numbers, however rather than the overall IQ and classification. Because WS is a disability that has strengths as well as deficiencies, your child may have an overall lower score IQ than expected because the spatial tasks will likely be very low making the overall IQ a poor predictor of their success in the general education setting. The subset scores will be a better measure of your child's strengths and needs to help your team determine the best settings and goals to help your child grow academically.<br />
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During this meeting, it is possible that the IEP is revisited and adjustments can be made to goals you made during the transitional IEP. The diagnostic tests can contribute to this discussion but benchmark goal assessments by the educators and therapists should also be taken into account to see if goals should be adjusted based on your child's growth.<br />
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<h2>
Requesting additional services</h2>
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There are other services that your child can benefit from that are not a part of the preliminary testing and service determination. After you have established some goal assessment during the school year and your first IEP report is delivered, you can request your child be tested for eligibility for assistive technology, adaptive PE and music therapy. All three of these programs are designed to use measurements that are established, offer the program and retest under those program's conditions and compare to see if the child shows increased success using an alternative form of instruction.<br />
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For example, say your child is learning to count 1-100. On their benchmark, they can count to 39 but then get the numbers in the wrong sequence or skip the 40's. The music therapist can then do an assessment to see if they are more successful when counting if it is taught with music. If the child shows a significant difference in the numbers they can count to with music than without, she will qualify for music therapy. Music therapy goals are supportive of the main IEP goals so a few will be chosen from the already established IEP that they will work on in support of the general or special education teacher. They'll report on the child's success on those goals in the music therapy sessions.<br />
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In adaptive PE, a special education teacher will accompany your child during the regular PE class and determine if their is a need for special instruction on skills used during that gym unit such as staying focused on the activity such as knowing where to stay put during stretching or warm-ups or understanding the rules of the game. <br />
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Assistive Technology is using tools and technology to bridge the gap between your child's ability and the general education setting. It can be low tech items such as a slanted desktop, adaptive pencils to improve grip or schedules printed out on their desk. It can also be adaptive technologies such as one button mouses for computers, touch screen computers or tablets. During the assistive technology meeting, the specialist will want to know skill gaps, motivational strategies and needs of the student to determine their technology needs. Basically you can't come out and say you want your child to receive a school issued tablet. You have to establish a need for one through well crafted descriptions of your child's needs. Some examples of ways my daughter benefits from assistive technology:<br />
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<ul>
<li>She uses a touch screen computer to navigate school programs used in the classroom such as reading programs with computer voice to text programs and games such as starfall. She can navigate these programs independently during center time with a touch screen versus using a mouse.</li>
<li>She uses a tablet with a program called "clicker connect" for reading comprehension and writing activities. Since she is still working on writing but has good comprehension skills and story telling skills, she can use this program by clicking on word boxes that they read the word to her and then drag and drop them into sentences. With this program she can answer comprehension questions, do writing activities and practice Kindergarten activities such as building sentences using proper nouns and punctuation. This program is very successful in activities where physically printing the sentence is less important than crafting the words to form sentences or answer content related questions related to the story the children are studying. If the child could not originally participate in these activities because they couldn't write their answers before, then this gives them access to that portion of the curriculum.</li>
<li>She uses various other programs on her tablet during school activities such as tracing letters and shapes, matching rhyming words, phonics programs and vocabulary building programs.</li>
</ul>
<h2>
Determining if you should repeat Kindergarten:</h2>
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At the end of the school year, many WS families are faced with the choice on whether you should repeat Kindergarten or move on to 1st grade. This is a personal decision that should be considered based on the child's developmental level. These are a few things we considered when weighing this decision:</div>
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<ul>
<li>Would your child benefit most by repeating this grade level's curriculum? Kindergarten has elevated in rigor and complexity with common core expectations. No longer do Kindergartners only learn their letters but reading and arithmetic is expected. It's important to look at the skills that a 1st grader and a kindergartner are doing in the general ed classroom and compare them to the developmental level of your child. Are they still trying to master Kindergarten skills or are they ready to move on? Even if your child has a modified curriculum and get special education services if they spend time in the general ed setting you'll want them to access appropriate curriculum in the general ed settings.</li>
<li>How would retention affect your child socially? Although we decided to keep my daughter in Kindergarten another year, I do have some social regrets with holding her back. She created some strong friendships in her first year of Kindergarten and the transition away from those friends was hard on her at first. At times we see her 1st grade friends at school community events and scouting events and part of me regrets breaking those ties.</li>
<li>How will retention now affect her services during transition to adulthood programs? This is a hard one to answer because services offered between the ages of 18-21 may look very different by the time my daughter reaches that age level. But, these are important questions to ask. I did some investigating in the special school district in our city to consider what my daughter's choices might be. If you hold back your child now, they'll graduate a year later than usual and may have less time to participate in transition programs that prepare your child for post-high school programs and job placement opportunities. </li>
</ul>
<div>
Ultimately, we decided that since our daughter was delayed approximately one year in age developmentally, it would benefit her socially and academically to have another year to developing the basic skills she needs to access general education standards. It would give her a greater opportunity to successfully participate in the general ed setting. It's important to know, also that if your child has a modified curriculum, they can move on with 1st grade level skills while still in Kindergarten if they are ready before the end of the year. For example, in the 2nd year IEP we determined that some first grade math skills such as time telling would be an appropriate next step since she has responded well to counting and adding and subtracting with manipulatives.</div>
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<h2>
The takeaway...</h2>
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Kindergarten can be a stressful time because any transition is! The fear of the unknown, a new team, a new school and new classmates... it is a lot of people to educate and hope they have your child's best interest in mind! The best advice I can give you is 1) establish yourself as an expert on your child's needs and educate your child's team on WS 2) Participate in as much as you can so that you become part of your school community and build strong, positive relationships with the teachers, parents and therapists 3) Educate yourself on the school curriculum, resources and services your child can benefit from and ask for them 4) Take a deep breath! Your kid will do great and you'll be amazed as you watch them grow before your eyes.</div>
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<br />Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com0tag:blogger.com,1999:blog-7345537858373518781.post-12782800736899702302015-01-01T08:53:00.000-08:002015-01-01T08:53:04.162-08:00New resources for you!I just wanted to let you all know of a new resource that is available through St. Louis Children's Hospital! Childrens has a Williams syndrome clinic that is fabulous (I highly encourage making a trip to see them). I had the opportunity to work with the team there in May to write a series of new resources for WS families over various topics. I personally wrote the parent perspective brochure and then co-authored the school brochure. I also collaborated on the topics on the informational brochure. Anyhow, I think they all have a fabulous overview for parents on various topics of concern. I especially like the hypercalcemia and diet ones. I wish I had those as a new parent because the colic was the hardest part of Katie's early years.<br />
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Here is a screen shot of the topics and you can find the link below. <br />
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<a href="http://www.stlouischildrens.org/our-services/williams-syndrome-center/resources" target="_blank">St. Louis Children's Hospital Williams Syndrome Center Resources Page </a></div>
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On a personal note, I'm very thankful for all the support you have given my blog and education efforts! 2014 has been nothing but amazing and I hope to provide you with the most current information possible in the future!<br />
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Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com1tag:blogger.com,1999:blog-7345537858373518781.post-3435241644373919612014-08-09T18:50:00.000-07:002016-03-16T12:29:30.336-07:00Back to School Tips for TeachersJust this week I walked my daughter into her Kindergarten classroom to meet her new teacher. This time of year brings me joy mixed with fear... definitely bittersweet. I know Katie will thrive in her new school with her new team of teachers, therapists and aides, but part of me always worries about her. I'm sure you can relate. So, I challenge you to ask yourself- what can you do about that? I hope your answer is the same as mine...I advocate for her.<br />
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In my own classroom I typically have 20+ students with IEPs and among them I've had very few parents reach out to me before the beginning of the school year. But when they do, I immediately have an elevated level of respect and ambition to go out of my way to help them achieve their goals and needs! Normally at the start of a new school year, it takes me time to get to know my students and identify their needs. It takes me even longer to find the best solution to meet their needs. Why not, as a parent who knows what our children need, help accelerate that by reaching out to teachers and giving them a toolkit of research based accommodations specific to your child? By contacting your child's teacher you can share those resources so they can start the year with strategies and plans to help your child succeed from day one. So, in this post, I'm sharing my WS toolkit with you.<br />
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In addition to the toolkit, I gave my daughter's teacher the bio I made called "All about Katie". I used the bio last spring at her annual IEP. It includes her picture, nickname, strengths, weaknesses, concerns and family goals. I think that we often share these at IEP meetings but forget regular ed. teachers often don't get to see those. They usually only receive a copy of the legal IEP at the beginning of the school year. <br />
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A simple email establishing positive communication with your child's teachers will create a smoother transition to their new classroom. Approach your new teacher with an open mind and be cognizant that they are extremely busy and stressed this time of year. When I sent these materials I was mindful of how I worded my email to show that I respected their time and dedication to their students. I kept it short explaining that I wanted to help them by providing resources that could act as an easy guide to help them solve problems that may come up as they get to know Katie and her needs. They can also use them as a way to be proactive in her learning. For example, it makes a teacher's job easier if they know if a child needs preferential seating or an audio book in advance so it can be available on day one. Plus, opening up that communication breaks the ice and shows them that you have high expectations but that you are also part of the team and want to work with them in a collaborative fashion.<br />
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Below you will find my newest infographics that go along with topics I presented at
the convention on visual-spatial issues followed by a collection of educational
strategies that I learned about this summer. Also I included a quick chart showing common behavioral challenges and social strategies that are common in children with WS. Following the infographic,
you can find links to my other infographics and blog posts that are
education centered and WSA resources that would benefit both a regular
education and special education teacher.<br />
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I wish you and your children the best of luck in the new school year! </div>
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Links to other infographics and resources I shared with my child's new classroom teachers:</div>
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<a href="http://understandingwilliamssyndrome.blogspot.com/p/nervous-system.html" target="_blank">Understanding speech development in children with WS </a></div>
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<a href="http://understandingwilliamssyndrome.blogspot.com/2014/03/teaching-strategies-guide-for-educators.html" target="_blank">Teaching Strategies specific to WS</a></div>
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<a href="http://understandingwilliamssyndrome.blogspot.com/2013/02/teaching-math-to-children-with-ws.html" target="_blank">Teaching math to a child with WS</a></div>
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<a href="https://williams-syndrome.org/sites/williams-syndrome.org/files/Quick%20Facts%20for%20Teachers.pdf" target="_blank"> WSA's Quick facts for teachers</a></div>
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If you find any of these infographics difficult to print, feel free to email me at mooniersarah@yahoo.com and I'll send you a pdf version.</div>
Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com1tag:blogger.com,1999:blog-7345537858373518781.post-42460421723031813182014-07-20T11:06:00.001-07:002014-07-20T11:06:54.513-07:002014 WSA Convention Questions Answered Part 2- Radial-ulnar synostosis<br />
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<span style="color: blue;"><span style="background-color: white;"><span style="color: black;">This is the second of my series of blog posts in response to questions presented to me at the 2014 Williams Syndrome Association National Convention. See Q1 <a href="http://understandingwilliamssyndrome.blogspot.com/2014/07/2014-wsa-national-convention-questions.html" target="_blank">here</a>.</span><i> </i></span></span><br />
<h2>
<span style="color: blue;"><span style="background-color: white;"><i>Q2: Is <span class="st">Radio-ulnar synostosis</span> related to WS?</i></span></span></h2>
One of the clinical symptoms related to WS is radio-ulnar synostosis or an
issue where the radius and ulna are fused in a person's lower arm(s). A typical
forearm is designed in a way to allow the hand to maneuver in a variety of ways
in order to manipulate objects in the hand. When you extend your arm out with the palm facing downward (pronation) and then twist your palm to face upwards (supination), the bones in the lower arm direct that motion. <br />
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<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhCIZWgdQLgK-CWPZksMRh3Hy4rtsvDJ5KKRLa66wRG-xdV9Lngf-lBFm-mBgI8r9X1CPbqfuXqoGo-2KkMUxg3BjRKaJRwGeJZ7Clvd4OqpVpdb5xt9ELBEn0azwzw2g466Ft1wRALirge/s1600/ulna.jpg" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;"><img border="0" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhCIZWgdQLgK-CWPZksMRh3Hy4rtsvDJ5KKRLa66wRG-xdV9Lngf-lBFm-mBgI8r9X1CPbqfuXqoGo-2KkMUxg3BjRKaJRwGeJZ7Clvd4OqpVpdb5xt9ELBEn0azwzw2g466Ft1wRALirge/s1600/ulna.jpg" /></a><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEivggypbaANler7izPPkmLNh2GnzgeV9rb0j8Pk70U6LiMgVug9xXABIWZ4isZKGrBvQ_U4_7fWsj-imRpsLCLns6k-NoGjZGs6SD8A5YhRsx8dUjQKAhhdypfz3OV_qdvyaTI7elqDOksm/s1600/radius.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEivggypbaANler7izPPkmLNh2GnzgeV9rb0j8Pk70U6LiMgVug9xXABIWZ4isZKGrBvQ_U4_7fWsj-imRpsLCLns6k-NoGjZGs6SD8A5YhRsx8dUjQKAhhdypfz3OV_qdvyaTI7elqDOksm/s1600/radius.jpg" /></a></div>
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<i><span style="font-size: x-small;">In the pictures above, the radius is highlighted on the left drawing and the ulna is highlighted in the right drawing.</span></i></div>
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The lower arm is made up of two bones, the ulna and the radius. The ulna is the bone that makes up the elbow. Touch your elbow, you are touching a large protrusion on the superior portion of the ulna, called the trochlea. The ulna also articulates or creates a joint at the outer most edge of the wrist but in a much less pronounced way. The second bone of the lower arm is the radius. The radius articulates or connects to the majority of the bones in the wrist and extends to the elbow where it ends with a small concave circular notch that allows it to turn. The radius is named for this motion. Place your hand on your ulna (elbow) and follow that bone down the outside of your arm. Now twist your palm up and down and notice that bone moves very little. The other bone, the radius, moves quite a lot! The radius is on the inside of the arm and it rotates around the ulna. Watch this short youtube video to see an "insider's" view of how these bones twist the hand.<br />
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<iframe allowfullscreen='allowfullscreen' webkitallowfullscreen='webkitallowfullscreen' mozallowfullscreen='mozallowfullscreen' width='320' height='266' src='https://www.youtube.com/embed/DsjFkk6Nuzo?feature=player_embedded' frameborder='0'></iframe></div>
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Now, imagine the radius and ulna bones are connected. Would you still be able to twist your palm up and down? No! This is what happens during radial-ulnar synostosis or the fusion of the radius and ulna. It affects 26% of individuals with WS. My daughter has this in her right arm and it restricts the amount of motion and actions she can do with her right hand. <br />
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<span style="font-family: inherit;"><span style="font-size: x-small;"><i> In the photos above, the left is an x-ray view of a typical bone arrangement in the elbow. This is in supination position (palm rotated upwards). The photo on the right is one of an individual with radial-ulnar synostosis. Their radius and ulna are fused at the elbow making it impossible for them to rotate their lower arm.</i></span></span></div>
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<span style="font-size: small;"><span style="font-family: inherit;">If your child has radial-ulnar synostosis, there isn't much you can do, medically, to treat it. Even if the bones were surgically separated, they will not have the same bone structure, vascular supply and ligament/muscle attachment that would allow the bones to rotate properly. You would be hard pressed to find a surgeon to do this type of treatment unless the fusion very severely limits any use of the arm.</span></span><br />
<span style="font-size: small;"><span style="font-family: inherit;"><br /></span></span>
<span style="font-size: small;"><span style="font-family: inherit;">The best way to handle this condition is to modify surroundings and teach the affected individual adaptations they can use to manipulate objects. You may often find that they have a harder time manipulating small objects and holding writing utensils, such as pencils and crayons. My daughter shows preference in her left hand for writing but that wasn't always the case. She often manipulates items with her right and then will switch to the left. This may be her way of adapting to her environment since she has more movement in the left than her fused right arm. Many find that being ambidextrous out of necessity is the case for those with radial-ulnar synostosis. Other actions that may be difficult if they have a fused arm includes:</span></span><br />
<ul>
<li><span style="font-size: small;"><span style="font-family: inherit;">holding spoons/forks during feeding</span></span></li>
<li><span style="font-size: small;"><span style="font-family: inherit;">manipulating buttons, belts and zippers</span></span></li>
<li><span style="font-size: small;"><span style="font-family: inherit;">sports that require wrist rotation such as catching a ball</span></span></li>
<li><span style="font-size: small;"><span style="font-family: inherit;">turning a doorknob</span></span></li>
<li><span style="font-size: small;"><span style="font-family: inherit;">handwriting</span></span></li>
<li><span style="font-size: small;"><span style="font-family: inherit;">manipulating coins and other small objects</span></span></li>
<li><span style="font-size: small;"><span style="font-family: inherit;">pouring liquids</span></span></li>
<li><span style="font-size: small;"><span style="font-family: inherit;">playing instruments such as guitar that requires wrist manipulation </span></span></li>
</ul>
<span style="font-size: small;"><span style="font-family: inherit;">This website has some very interesting stories about the challenges and accommodations that come with this condition in the comments section. The commenters are individuals who have lived with radioulnar synostosis. *Note, this condition exists outside the world of WS so please don't assume the commentators have Williams syndrome. <a href="http://boneandspine.com/congenital-radioulnar-synostosis/" target="_blank">Check it out here</a>.</span></span></div>
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<span style="font-size: small;"><span style="font-family: inherit;">Coupled with the visuospatial issues and motor control that individuals with WS have, the tasks listed above can be even more difficult. You will find that if your child has radioulnar synostosis, they will adapt on their own but there are many ways you can help. For example, you can chose clothing that doesn't require buttons or replace knobs on doors with handles. There are also many adaptive technology apps that are available to help your child with academics and self help skills. This was a major topic of interest at the convention. To access the WSA's list of useful apps visit the website <a href="http://bit.ly/1qGlmS7" target="_blank">here</a>. </span></span></div>
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<span style="font-size: small;"><span style="font-family: inherit;"><br /></span></span></div>
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<span style="font-size: small;"><span style="font-family: inherit;">If you have specific ways you have helped your loved one or students adapt to radioulnar synostosis, I'd love for you to share. Please feel free to leave your experiences with this in the comments section of the blog or on the WS support group thread!</span></span></div>
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<span style="font-size: small;"><span style="font-family: inherit;"><br /></span></span></div>
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Source:<br />
<a href="http://link.springer.com/article/10.1007%2FBF02011725#page-1" target="_blank">Radio- ulnar synostosis and its occurance in WS</a>; 1991 *Note
this was published prior to the use of FISH testing for WS diagnosis so
information is clinical.<br />
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During the convention, I spoke about sensory motor pathways and the genetics of WS. I plan to have a few new posts that share the information I presented at the convention in the near future. Look for those and others that have been requested (such as urinary and kidney issues in WS and sleep).<br />
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I was busy keeping notes of questions that ventured my way throughout the week. Since many questions we have are on other's minds, I thought I'd answer them in a series of blog posts. I'll post info as I learn them :) <br />
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<i><span style="color: blue;">Q1: Is the likelihood of a WS parent having a child without WS really 50%?</span></i><br />
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This question came up during the genetics session. The reason behind this is that some families are skeptical that there really is a 50% chance of their child with WS eventually having a typical offspring. We rarely hear of WS adults with children and those we do hear of often have children with WS. While I don't know of nor have actual records of parents who have Williams syndrome and have typical children, all the literature says that the likelihood is 50%.<br />
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This is because of the process of meiosis. When a person creates eggs or sperm their cells go through a process called meiosis where they duplicate their chromosomes, shuffle the genes during crossing over and then distribute them into two groups and divide. In the second half of this process, they divide again to create two unique cells. The result is four unique gametes each containing half of the parent's chromosomes.<br />
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So, if a person with WS divides their chromosomes in half, one half of them will contain the deleted portion and one half would contain the full genetic sequence. From this point, we call the 50% the probability of inheritance but in reality, it's all chance. If you flip a coin a couple of times is it likely you'll get heads both times? Yes! So it's fair to say that a person with WS could potentially have children who all have WS. Now consider the full genetic potential of one person. In her lifetime, one female creates upwards of 6 million eggs before she is born and a male can produce nearly 525 billion sperm! So if you flip a coin 6 million or 525 billion times guess what the likelihood of getting heads? 50%. That explains why you might see families with parents who have WS and their children all have WS. It's the game of statistics. <br />
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I'm unaware of studies that are following data on this topic but with the improvement of the WS registry and parents asking for this information I could see this information being available in the future.<br />
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Learn more about inheritance and the basics of genetics and WS <a href="http://understandingwilliamssyndrome.blogspot.com/p/genetics.html" target="_blank">here</a>.<br />
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If you have never experienced a National convention for WS, I highly recommend you try for the Ohio convention in 2016. I found many outlets for financial support and made the trip affordable for my family. The wealth of information you gain and the networking you can participate in is worth every cent. In the meantime, check out www.williams-syndrome.org to find audio recordings of the convention sessions for sale.<br />
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<u>Future blog topic requests that came my way during the convention:</u><br />
Urinary and kidney issues in WS<br />
Improve the gene list and functions<br />
Anxiety, obsessions and meltdowns <br />
Causes of sleep issues in WS<br />
Teaching social skills and friendly conversation<br />
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Have any other requests or more info on this blog post? Leave a comment. I'd love to have powerful dialog occur on these posts from parents like you. Stay tuned for more Convention Questions Answered within the next few days!<br />
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<a href="http://understandingwilliamssyndrome.blogspot.com/2014/07/2014-wsa-convention-questions-answered.html" target="_blank">See the answer to question 2: radioulnar synostosis here</a>.<br />
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<br />Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com0tag:blogger.com,1999:blog-7345537858373518781.post-15557810502736013332014-03-19T19:25:00.002-07:002016-03-16T12:27:13.454-07:00Teaching Strategies Guide for EducatorsSpring marks the beginning of many annual IEP meetings including my own Katie's transition to Kindergarten! This time of year can be stressful for many families, so to prepare, I've created an infographic you can print out and share with your IEP team to help identify research-based best practices and appropriate accommodations for kids with WS. Feel free to download or save this and print as many as you'd like. I hope it can help facilitate positive conversations about your child's needs... and remember, every child is an individual so some of these strategies will be effective and others will not. You, as the parent, know your child the best so be an advocate and vocalize what you know works! All the information on the infographic is compiled from the educator guides at <a href="https://williams-syndrome.org/teacher/educational-strategies" target="_blank">www.williams-syndrome.org</a>. Best of luck!<br />
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<br />Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com4tag:blogger.com,1999:blog-7345537858373518781.post-36784018841587909702014-02-18T16:33:00.000-08:002016-03-16T13:02:54.898-07:00Understanding ElastinElastin. It's a term many families affected by WS recognize immediately, yet in regards to what it really is, many may not fully understand. Elastin is a famous term used in the WS world because it is used to obtain a diagnosis using a FISH test. So what is elastin and how does it cause some of the more famous symptoms of WS? In this blog post- and on my <a href="http://www.williams-syndrome.org/parent/webcasts" target="_blank">webcast</a> through the Williams Syndrome Association- we'll explore the ELN gene, how it is used by the body and its role in many WS symptoms. This blog post will give you an overall idea of its role and links to posts on symptoms affected by the absence of ELN. The webcast has more details and a Q&A at the end that you may be interested in.<br />
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<span style="color: #3d85c6;">So what is ELN?</span></h2>
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ELN is one of the genes affected by the microdeletion on chromosome 7 that causes WS. I often hear many refer to it as a chromosomal deletion which is not the case at all. In fact, it's a gene deletion called a microdeletion because very few genes are missing- only about 25 on average. Your body has 46 different chromosomes. 23 came from your mother and 23 came from your father. Together, they make you a whole person. Inside those chromosomes sit a series of genes that code for various proteins. On chromosome 7, the one affected in WS, there sits between 1,000-1,300 genes. If you had a chromosomal deletion, you'd be missing all 1,000+ because one entire chromosome would be missing. WS, in contrast is missing roughly 25 genes that sit on one of the lower arms of the chromosome. This is called a microdeletion. It's just a small section of the gene sequence was left behind during a phase of meiosis when the body jumbles the genes to create diverse offspring. (See the <a href="http://understandingwilliamssyndrome.blogspot.com/p/genetics.html" target="_blank">genetics page</a> of this blog for more info on crossing over).<br />
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Out of those 25 genes, one of them is called ELN. ELN is deleted in such a high majority of WS individuals, it creates a very reliable gene to "look for" in genetic testing. Before we knew so much about ELN, we focused most of our research about WS on symptoms such as narrow arteries. The presence of narrow arteries is the number one reason individuals with WS have life-threatening issues. Because of this, it is considered a high priority area to study in the WS research world. At the time, they took a backwards approach to genetics. Researchers would study the disorder, identify how the tissues were arranged or functioned differently and then tried to pinpoint the protein that caused that change. From there they would look for the gene that coded for that protein. In SVAS, they determined that gene was ELN. Now that we know the region where the genes are missing, we can use a much more efficient molecular genetics to identify proteins and explore WS. The discovery of ELN not only helped better diagnose the disorder, it opened many doors in genetic research to better understand that portion of the genome.<br />
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Genetic testing used to diagnose WS is a relatively "new" method. Prior to this diagnostic test WS facial characteristics and common symptoms had to be recognized by a medical doctor. In the 1990's the FISH test (Fluorescence in situ hybridization) for ELN was created. The use of this diagnostic tool increased the means to diagnose and better understand WS. (learn more about <a href="http://understandingwilliamssyndrome.blogspot.com/p/genetics.html" target="_blank">FISH testing here</a>). As our knowledge of the genetic world increases, we learn more and more about ELN, increase information available to doctors and families by new diagnostic testing, such as in <a href="http://understandingwilliamssyndrome.blogspot.com/2011/07/interpreting-microarray-results.html" target="_blank">microarrays</a> and increase the potential to lessen the effects of the missing genes using gene therapy.<br />
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<span style="color: #3d85c6;">How does the absence of ELN become a problem such as a heart defect?</span></h3>
Your chromosomes are made up of so many genes, each like a book in a library or a chapter in an instruction manual. Each gene codes for a specific protein. Proteins are the workers of your body. Their functions span many areas such as building materials, enzymes that make important reactions happen, tunnels that transport materials across membranes, even tubes that transport materials around the cell. Some of your genes are only active during specific events in your life such as embryonic development or puberty and others are active all the time- maintaining cell structures or aiding in reactions that help you digest food. <br />
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ELN is the type of gene that is expressed or "read" during fetal development and during the first few years of life and then continually through adolescence until your body completes its growth. After puberty, the ELN gene essentially sits dormant for the remainder of your life. Because of its relatively long lifespan- lasting up to 70 years, the body's need for making new elastin decreases greatly as we age. <br />
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When geneticists talk about a gene being expressed, they are referring to the process that occurs in the cell where the DNA is transcribed to RNA and RNA is used to create a protein. During fetal development, the baby's body is building many new structures. The organs in your body are made up of many different combinations of materials and tissues. The gene sequences such as ELN are very active during this stage of life in order to build functional organs and structures. It all starts inside the nucleus during transcription. The section of chromosome 7 that contains ELN unwinds. An enzyme named RNA polymerase unzips the section of DNA and matches the base pairs with RNA bases, essentially copying it. When it reaches the end of the segment, the new RNA strand (called messenger RNA or mRNA) leaves the nucleus to deliver the sequence to the protein maker- the ribosome. <br />
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When it reaches the ribosome, the mRNA feeds through this structure and is translated. During translation, the ribosome matches codons- or groups of 3 base pairs to an anticodon on a transfer RNA. The transfer RNA are aptly called this because they transfer the amino acid or protein building block to the ribosome. This match allows the cell to build or connect each amino acid into a strand in the proper order needed to make the desired protein. <br />
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When ELN is translated, it creates the protein called tropoelastin. When translation finishes the assembly of the amino acid strand the endoplasmic reticulum or ER takes the protein and coaxes it into a properly folded formation. Protein amino acids vary in their chemical composition. Many of them have polar or charged portions that attract to oppositely charge areas on other amino acids. This allows the protein to fold twist and connect to areas creating a unique shape. This shape is very functional. It gives the protein functional active sites that are designed to attract or repel molecules and "make things happen" within the cell. <br />
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The shape of tropoelastin is that of three parts or regions. The head of the molecule (labeled NC in the figure) is the portion that gives elastin its spring. It can stretch up to 8 times its relaxed state and then spring right back to its original structure unharmed. This becomes very important to its function in the tissues, which we'll get to in a bit. The second region just under the head is called the bridge. The bridge is an area that acts like a shock absorber. It absorbs energy from the coiled portion in order to prevent its base from becoming dislodged. The base functions to connect tropoelastin to an area of the tissue called the extracellular matrix. It is essentially an anchor to hold the tropoelastin in place. <br />
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<h2>
<span style="color: #3d85c6;">So, is tropoelastin the same as elastin?</span> </h2>
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<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEinJOPbxa1Codu-EXPszRCiSKHsCac_GplYJl_qdKIh7L2Qj7oDAyQXcXYWV7tSIhwzfkLeUpip1r4aG4JqoN2gMDI6CwHE_XLaX9dVvVk6eSgiGjLjs2B7cYRGaJvmCZzfEcoEz7Uni3f4/s1600/elastin.gif" imageanchor="1" style="clear: right; float: right; margin-bottom: 1em; margin-left: 1em;"><img border="0" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEinJOPbxa1Codu-EXPszRCiSKHsCac_GplYJl_qdKIh7L2Qj7oDAyQXcXYWV7tSIhwzfkLeUpip1r4aG4JqoN2gMDI6CwHE_XLaX9dVvVk6eSgiGjLjs2B7cYRGaJvmCZzfEcoEz7Uni3f4/s1600/elastin.gif" /></a>No! Tropoelastin is the main building block to a fiber called elastin. Once tropoelastin is created and packaged into its unique shape by the ER, it then is used as a building material to make elastin. Elastin is a fiber made of tropoelastin, microfibrils and is assembled by a group of five enzymes- called lysyl oxidases. As tropoelastin is created, it is shipped an area outside of the cell membrane where they accumulate. As they accumulate, one of the enzymes facilitates a chemical reaction on the tropoelastin to create cross-links or areas where they can soon connect. Essentially, it's like nailing brackets onto the structural material so you can connect them into a sheet. The cross-linked tropoelastin are then attached and woven to a series of microfibrils or tiny protein fibers that make up the extracellular matrix of connective tissue. This is basically a net that creates the foundation of a tissue and contains fibers, cells and is surrounded by nutrient rich fluids. The result is the fiber elastin.<br />
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So, in an individual with WS, this assembly line of elastin production has a decrease output because one set of the ELN is absent. ELN is still transcribed and tropoelastin is still assembled but only in half the output as a typical person. Think about a factory that assembles a product. If you cut your workforce and materials by half, you'll only get half the product. That is what occurs in WS. They still make the tissues and build the organs but because less tropoelastin accumulates outside the cell, the resulting elastin fibers are smaller and less springy.<br />
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<span style="color: #3d85c6;">How does this cause symptoms of WS?</span></h2>
Elastin is a major component of many connective tissues. There are several different types of connective tissue that have many different functions- the most important being support. Most connective tissue acts to do just that- connect organs in the body. They, for example, provide a net of support for epithelial (skin) layers in the body, they connect muscle to various organs to provide that organ movement. They might connect vessels and fat to the organ to provide important nutrients. They can store water, fat and salts needed for the organ's function. They also provide support to maintain the organ's shape- a key function of elastin. <br />
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Within all connective tissue are many different structures- there are the cells, often called fibroblasts which make the fibers, like tropoelastin. There are fibers such as elastin and collagen that provide elastic properties or collagen which is strong and structural. There are several proteins such as microfibrils that provide a framework or net and the extracellular matrix is often filled with fluids. So, as you can see, the structure of an organ often requires elastin as a major structural component needed for the connective tissue to function properly. <br />
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Elastin is essentially needed in any organ that requires some sort of stretch in order to work properly. These organs include the heart and vessels, the skin, the lungs, and the joints. As those organs stretch or widen, elastin stretches, (much like a rubber band but so much better!) and then springs back to an unaffected relaxed state. This molecule is so good at this stretching job that most people's elastin can function properly for 70 plus years... pretty amazing material! <br />
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<i>Much of the symptoms related to elastin have been discussed elsewhere in this blog. Below is some additional information about the disorders related to elastin and then you'll find a link to the blog page that gives more information.</i> <br />
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<span style="color: #3d85c6;">Elastin and Arteries</span></h2>
Until the early 1990's, little was known about the link between elastin and one of the most common vessel issues in WS- Supra-valvular aortic stenosis (SVAS) refers to the narrowing of the major vessel that leaves the heart- the aorta. The narrowing occurs just above a valve or doorway that prevents the blood from falling backwards into the heart. Typically in WS there can be overall narrowing in all the major arteries of the body- four of primary concern are the aorta, pulmonary arteries (going to the lungs), the coronaries (delivering blood directly to the heart tissue) and renal arteries (those that deliver blood to the kidneys). <br />
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When the body builds an artery, it assembles the structure using four main tissues- inside, the endothelial layer is built of epithelial tissue. This is like a skin-like lining that comes into contact with the blood. Outside the inner layer is the media tunic. This is composed of connective tissue and smooth muscle. In a typical artery, the media layer is made up of very organized parallel bundles of smooth tissue and elastin. This layer functions to control the size of the artery and regulate blood pressure. In WS, the elastin, like discussed early, is much smaller in size due to the lack of tropoelastin present in the tissue. Studies of the media tissue layer suggest that the pattern of elastin and smooth muscle becomes very disorganized and due to the lack of elastin, excess smooth muscle is layed down in an effort to compensate causing the vessel to loose it's stretchy quality and a much narrower formation is created. <br />
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<i>Diagram shows WS elastin on the left (notice the lack of tropoelastin) and a typical elastin on the right.</i></div>
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Considering that SVAS is the most life-threatening condition for those with WS, there is a large amount of research being conducted to better understand the mechanism or ways the vessel becomes narrow. Unlike pulmonary stenosis, SVAS can worsen as a person ages. As scientists isolate exactly how this occurs, there is hope that they can develop medications that might decrease the inflammation and decrease the degradation of elastin to control the worsening of the disorder.<br />
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Learn more about SVAS and it's affect on the body on the <a href="http://understandingwilliamssyndrome.blogspot.com/p/cardiovascular.html" target="_blank">cardiovascular page</a> of this blog.<br />
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<span style="color: #3d85c6;">ELN and its task force</span></h2>
While scientists have identified that the lack of one ELN gene is the cause of SVAS, they are suspect that ELN in combination with other genes that regulate its expression are involved in many other symptoms of WS including soft skin, premature aging, and facial features such as puffiness above the eyes. Studies of ELN began with SVAS because it was so prevalent in individuals with WS. As many parents are aware, WS has a spectrum of symptoms. Even though 99% of individuals are missing one ELN then why doesn't everyone have the same symptoms at the same level of severity? The answer is in the enzymes. The expression of a gene takes an entire task force to copy the gene, create the protein, organize the protein, and build it into its final structure. Even then when the fiber is damaged, there is a task force to either repair or replace it. This is all orchestrated by proteins and that is probably where the spectrum effect lies.<br />
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Scientists have been busy at work trying to identify the genes and enzymes that have a hand in causing the more severe cases of WS. As the amount of research improves and these genes and enzymes are identified, we may find better ways of predicting issues and treating them. <br />
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For a great example of this, visit the section on <a href="http://understandingwilliamssyndrome.blogspot.com/2012/12/posture-issues-associated-with-ws.html" target="_blank">scoliosis</a> in this blog.<br />
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<span style="color: #3d85c6;">ELN and the skin</span></h2>
Elastin is an important component of the skin. It's found in a layer called the dermis which sits under a thin protective layer called the epidermis. The dermis has many different functions and is the working portion of the skin. In the layer closest to the epidermis is called areolar tissue. It's loosely woven with collagen (for strength), elastin (for stretch), cells called fibroblasts (for building more fibers), and a salty water environment. You use this portion to store water and salts and create sweat. It has many blood vessels, nerves and hair follicles that live here, too. Under the areolar tissue is a layer called dense irregular. This is densely packed with collagen and elastin fibers in bundles that twist and turn in many different directions. <br />
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This is the portion that creates structure to your skin. Imagine a pregnant belly. As it grows and grows the skin must stretch and adapt. Then after pregnancy it (ideally!) returns back to normal. Now I can't speak from experience with this (ha ha) but if you can maintain the integrity of the elastin and collagen fibers, the tissue can remain in tact. If you can't, there are enzymes that gobble up the damaged skin and quickly lay down a repaired section- leaving you with stretch marks (which is essentially scar tissue). <br />
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Now, your probably thinking "how does this all have to do with WS?" I use the pregnancy example because its easy to visualize the damage that can occur. Damage also occurs with everyday life. Aging is definitely something that everyone has to deal with. Overtime, the lifespan of elastin can break down and lose its integrity. As we become exposed to sun, smoke and other carcinogens the damage can accelerate. Individuals with WS tend to have early onset of aging and it all has to do with damaged elastin. As damaged elastin is discovered, the body disassembles it with an enzyme called elastase. You also have another enzyme called alpha 1 antitrypsin (AAT) that slows down or inhibits elastase. It's basically a control so the enzyme doesn't go crazy and gobble up all the elastin in site. Scientists have been studying AAT trying to identify its role in WS. There is some evidence that some variations of AAT may contribute to more severe issues related to elastin. There are still many questions unanswered but many clues to the complicated role to how genes and proteins influence one another.<br />
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So, in conclusion, everyone has a degree of elastin damage as we age. In WS, where they are beginning with less elastin present in the dermis, the aging process will become more transparent over time. <br />
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<span style="color: #3d85c6;">ELN and the vocal cords</span></h2>
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Another area of the body that is affected by missing elastin is the vocal cords. Almost universally those with WS have a hoarse voice. The root of this lies in the flexibility of the vocal cords. Vocal cords sit in the larynx or voice box of the wind pipe. Men with prominent Adams apples make it easy to identify the location. The Adams apple or larynx is composed of tough cartilage that creates a somewhat stiff box. The cartilage is supported by many muscles and ligaments that attach to a bone called the hyoid. <br />
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As we speak, we manipulate the pressure within the larynx which moves and vibrates a portion called the vocal folds. The histology or layers within the vocal folds are mainly made of elastin. There is one jelly-like layer that is primarily elastic and another layer called the lamina propria that is thicker with elastin. This provides the flexibility of the folds to move with the pressure difference of the larynx during speech. Another, leaf shaped flap called the epiglottis sits over the vocal folds. This flap is responsible for closing off the windpipe when you swallow food. It can also vibrate as well, contributing to the sound of your voice. The vocal folds are primarily composed of elastin layers so in WS they do not vibrate and move as easily causing a hoarse tone of sound. <br />
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<span style="color: #0b5394;">ELN and the digestive system</span></h2>
The last place in the body that is most affected by the absence of elastin is the digestive system. The abdomen is a relatively open area, not containing any bones to shelter the organs. Because of this it relies on a combination of muscle and connective tissue for support. There is a layer of integument or skin that creates the internal lining of the abdomen, called the peritoneum. The peritoneum, like the skin, has a layer composed of elastin netting that allows it to stretch. This lining is important in pulling in the abdomen and supporting the core. <br />
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When elastin is weak here there, the internal organs, mainly the intestines, can bulge through the netting and get caught up in the abdominal wall. This is called a hernia and can be pretty common in WS. The problem with hernias is that they can be uncomfortable but they can also get infected if feces or bacteria get stuck in them. This can cause inflammation. Hernias are typically noticeable on the outside of the skin because a pocket or bulge will form under the skin. Hernias need to be repaired surgically.<br />
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The most common type of hernia is the inguinal hernia. This occurs during infancy and is most common in males but can still occur in females. In males, as the reproductive system develops, there is a canal, called the inguinal canal, that the testes descend or move down through. This canal then closes up, typically. In inguinal hernias the intestines slip down through the canal as well and a hernia develops in the groin. This can be attributed to missing elastin because the wall of the abdomen and the canal itself is looser than typical.<br />
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Another issue that can occur as people age is diverticulitis. This is similar to hernias but instead of the intestine getting caught in weak spots of the abdominal wall, weak spots on the intestine, itself create loose pockets. This too can get infected. This disorder is usually found in the elderly population but because of the nature of the elastin in WS, it can happen much sooner. There are records of people as young as 17 who have developed diverticulitis in the WS population. <br />
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<span style="color: #3d85c6;">ELN in the joints</span></h2>
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The final area of the body that is affected by elastin is the joints. Most notably the intervertebral discs of the spine. I have a lengthy post on posture that discusses this topic. Go <a href="http://understandingwilliamssyndrome.blogspot.com/2012/12/posture-issues-associated-with-ws.html" target="_blank">here</a> for more info.<br />
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<span style="color: #3d85c6;">In sum...</span></h2>
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As you can see, not all symptoms or complications of WS are attributed to ELN but its discovery was infinitely important in today's understanding of WS. It opened doors in genetics to help diagnose and better understand the region where WS occurs. It opened doors in cardiology to help understand and treat the #1 cause of fatal complications. It's discovery has completely changed the care and open avenues for research in the WS world.<br />
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<h2>
<span style="color: #3d85c6;">Sources:</span> </h2>
<ul>
<li><a href="http://www.ncbi.nlm.nih.gov/pubmed/16007633" target="_blank">Sigmoid divertculitis in patients with Williams Van-Beurun syndrome</a></li>
<li><a href="http://www.scielo.br/pdf/gmb/v29n1/28166.pdf" target="_blank">Elastin gene point mutation in patients with inguinal hernia</a></li>
<li><a href="http://www.ncbi.nlm.nih.gov/pubmed/18307085/" target="_blank">An investigation of voice quality in individuals with inherited elastin deficiencies</a></li>
<li><a href="http://hmg.oxfordjournals.org/content/8/10/1947.full" target="_blank">Williams-Van Beuren Syndrome Genes and Mechanisms</a></li>
<li><a href="http://www.williams-syndrome.org/sites/williams-syndrome.org/files/NEJM-WBS-MedicalProgress.pdf" target="_blank">Medical Process: Williams syndrome New England Journal of Medicine by Pober</a></li>
<li><a href="http://www.pnas.org/content/90/8/3226.full.pdf+html" target="_blank">A Human Vascular disorder, supravalvular disorder, maps to chromosome 7 by Ewart et al.</a></li>
<li><a href="http://circ.ahajournals.org/content/92/1/142.figures-only" target="_blank">Genetic approaches to cardiovascular disease: supra-valvular aortic stenosis, Williams syndrome and Long-QT syndrome by Keating</a></li>
<li><a href="http://homepage.smc.edu/wissmann_paul/anatomy2textbook/extracellmatrix.html" target="_blank">The extracellular matrix</a></li>
<li><a href="http://www.elastagen.com/media/The_Science_of_Elastin.pdf" target="_blank">The science of elastin</a></li>
<li><a href="http://ghr.nlm.nih.gov/gene/ELN" target="_blank">Genetics Home reference: US National reference of genetics</a></li>
<li><a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3286909/" target="_blank">Tropoelastin bridge region positions the cell-interactive C terminus and contributes to elastic fiber assembly by Yeo et. all</a></li>
<li><a href="http://www.intechopen.com/books/regenerative-medicine-and-tissue-engineering-cells-and-biomaterials/elastin-based-constructs" target="_blank">Elastin Based Constructs; </a><a href="http://www.intechopen.com/books/regenerative-medicine-and-tissue-engineering-cells-and-biomaterials/elastin-based-constructs" target="_blank">Lisa Nivison-Smith and Anthony Weiss</a></li>
</ul>
Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com0tag:blogger.com,1999:blog-7345537858373518781.post-81640221650470998542014-02-15T11:49:00.001-08:002014-02-15T11:49:24.799-08:00Webcast- Understanding ElastinI'm excited to announce that I'll be hosting a webcast for the Williams syndrome Association on Tuesday February 18th and 7 p.m. central time. Join me by <a href="http://www.williams-syndrome.org/parent/webcasts" target="_blank">clicking here</a> and registering! The webcast will be archived on the same webpage after it airs so if you miss it, you can hear it at anytime. <br />
Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com0tag:blogger.com,1999:blog-7345537858373518781.post-34397835069383250562013-11-27T12:06:00.000-08:002013-11-27T12:06:07.096-08:00Music, brain studies and its link to emotional learningAsk any parent whose child has Williams syndrome and they will tell you they are musical. My daughter has been drawn to music since infancy and shows high emotional ties to it. Sad songs get tears. Happy songs get squeals of joy. She's like a musical sponge. When Katy Perry's "Roar" hit the airwaves, Katie knew the lyrics after listening to it about 3 times. She's four. Since before she even started school, music has been a way for us to teach her things. I've invented more songs than Paul McCartney. In all seriousness, though, there is not an overabounding amount of research that explains the underlying connection to WS and music. We know there is something there but science hasn't had the chance to explore it in depth, yet.<br />
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There are several brain studies that show that individuals with WS have more active brain patterns when music is used in learning than the typical person. A recent study published in summer of 2013, looked into the link between this musical affiliation to the social and empathetic personalities of a person with WS.<br />
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Although the study used a relatively small sample size (a total of 55 individuals including a control group of typically developing adults) it resulted in significant data that suggests music therapy can positively affect individuals in learning environments and can help them cope with difficult social behaviors such as anxiety and heightened emotional response. The study began with a goal to find a connection between verbal comprehension and use of music. They also wanted to discover if those with WS had a greater emotional response to music than someone who is typically developing.<br />
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Previous studies suggest that the brain of a WS individual functions differently than most people. Particularly, the amygdala's function is overly active during music. There is also an activated visual cortex during music which is unusual. This study suggests that there is a connection between music emotion and learning activities.<br />
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The amygdala is found inside of the brain towards the base and it is understood to correlate with emotion, emotional behavior and has strong links to learning. It receives information from all major sensory organs especially those of sight, smell, and internal stimuli. It is strongly linked with pathways to the hypothalamus which is the area of the brain that controls internal regulators. The hypothalamus controls portions of the pituitary, the master endocrine gland that controls various hormones. It also controls important vital functions such as heart rate, digestion, breathing rate and temperature regulation. The pathway that connects the amygdala and hypothalamus are primarily involved in motivation and drive concerning emotional interest and response to rewards and consequences. (Wright, Neuroscience online). This is a major motivator in this study. If this area of the brain is more active during music there is a strong suggestion that there is a correlation between emotional response to music and learning. The activity of this region is associated with empathy. The ability to read other's faces and recognize their emotion is primarily controlled by this area, explaining a strong state of empathy in those with WS.<br />
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This study focused on three aspects of music:<br />
<ul>
<li>Interest in music- Specifically the amount of time spent in music related activities </li>
<li>Creation of music- The ability to remember songs. This portion of the study also considers the level of complex music they participate in relation to instruments, rhythm and lyrics. </li>
<li>Emotional response to music- How frequently they express emotion during musical activities. It particularly measures empathic and sensitivity responses to others emotions. This portion also considered verbal comprehension when music is present. </li>
</ul>
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Findings of the study show that individuals with WS don't necessarily have a talent or ability to create music compared to the typical person but they have a stronger emotional response and chose music over other activities at a higher frequency.They develop an interest in music at an earlier age despite any additional influence of their parents or caregivers and spend more time throughout the day playing instruments or participating in music related activities. They found a strong connection between music and emotional response. They also found that those with WS will learn their language more successfully if emotional and social use of music is incorporated in the introduction of new vocabulary. Important to note, there are also related studies that show music can increase mathematical comprehension as well.<br />
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This study and others related to social dysfunctions such as anxiety find that music can act as a learning strategy as well as a therapeutic activity. It is shown to reduce anxiety and depression in individuals with WS.<br />
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On a personal note, my daughter is an active participant in music therapy and she shows remarkable gains in her IEP goals when music is involved in the activity. Although she is only one child, her data suggests and increase in up to 30% comprehension of vocabulary than without music! We are very thankful that our school district offers this therapy as a part of her education.<br />
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This blog post is a summary of the findings from the scholarly article: </h3>
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<div class="cit">
<a href="http://daniellevitin.com/levitinlab/articles/2013_Ng_JMHRID.pdf" target="_blank">Musicality Correlates With Sociability and Emotionality in <span class="highlight">Williams Syndrome</span></a>.</div>
<div class="auths">
Ng R, Lai P, Levitin DJ, Bellugi U.; Laboratory for Cognitive Neuroscience, Salk Institute for Biological Studies, La Jolla, California.<span role="menubar">J Ment Health Res Intellect Disabil.</span> 2013;6(4):268-279.</div>
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<h3 class="auths">
Additional sources used to enhance understanding of this article from:</h3>
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<div class="auths">
<span class="chapterTitle"><a href="http://neuroscience.uth.tmc.edu/s4/chapter06.html" target="_blank">Chapter 6: Limbic System: Amygdala</a>; <a href="http://www.blogger.com/null" id="content" name="content"></a>Anthony Wright, Ph.D., Department of Neurobiology and Anatomy, The UT Medical School at Houston</span></div>
Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com0tag:blogger.com,1999:blog-7345537858373518781.post-89321859911085195242013-02-21T18:10:00.002-08:002016-03-16T12:41:25.973-07:00Teaching math to children with WSOne of the most fascinating aspects of studying the WS brain is that they exhibit both strengths and weaknesses when it comes to brain function. Very few disabilities have this combination of traits which makes WS a potential goldmine for learning how parts of the brain work together to complete tasks. Scientists find the WS mind an amazing tool to unlocking some mysterious aspects of psychology. The WS brain has amazing verbal and musical abilities paired with many educational difficulties in spatial learning and ability to focus. These deficits can affect many aspects of the educational development of a child, including those in mathematics. <br />
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The difficulties associated with math are complex in nature. You will meet some students with WS who perform very poorly, others perform at grade level and still others only struggle with some areas such as time and money. This unpredictability of math skills is something that psychologists find interesting. It may lead to clues as to what areas of the brain are used for certain types of problem solving. It may also open doors to understanding new teaching methods that could help many students who struggle with math- whether they have WS or not. Due to the unique nature of the WS brain, much can be learned by our little ones. Their unique abilities give psychologists clues to how humans learn. <br />
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When compared to other intellectually challenged groups of students - such as those with Downs Syndrome, Turners syndrome and children diagnosed with math learning disabilities, those with WS have unique deficits. The other groups tend to score low in all aspects of mathematics versus WS who have a mixture of high and low scores based on the skills required for each type of math skill assessed.<br />
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<span style="color: #3d85c6;">Brain science- why is math hard for my child?</span></h3>
There are two theories as to why those with WS struggle in math. One theory is that the grey matter in the parietal lobe of those with WS is known to exist in low amounts. Grey matter is a collection of neurons (or nerve cells) that specialize in thinking and problem solving. In general, the more grey matter in an area of the brain, the greater talent that person has to do those tasks. The brain is covered in ridges called gyri. Each gyri, built from grey matter, has its own job or function in one aspect of thinking. For example, the precentral gyrus (also known as the motor cortex) is used to control when muscles contract in all parts of your body. The post central gyrus (also known as the primary somatosensory cortex) is designed to identify touch and other sensory ques. In between the gyri are narrow dips or grooves called sulci (sulcus). These also contain grey matter and divide the functional regions of the cerebrum. WS brain studies suggest that the intraparietal sulcus of the parietal lobe contains low amounts of grey matter. This affects the intellectual ability of a person with WS to understand some parts of math, especially those that are related to spatial cues. There is a well known link between the amount of grey matter and a person's IQ. Lower IQ and intellectual disability which could explain a lower overall understanding of mathematics.<br />
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Another theory that explains math disability in those with WS is that their brain just works differently than most. In addition to low amounts of grey matter, there are impairments to the pathways that lead to the parietal lobe. Some psychologists feel that difficulties related to math in kids with WS are more due to the flow of information within the brain rather than the amount of grey matter in the parietal lobe. These pathways are made of white matter, which are made of neurons coated in fat. The fat makes the messages move quickly from one area to another. These pathways are used to link different gyri and sulci together to coordinate a more complex task. There are some pathways that are used more often- like highways of the brain. The dorsal stream pathway runs from the visual area of the brain up to the parietal area. This stream is known to be impaired in WS and may be one reason why mathematics is difficult for kids with WS. You can see in the picture, below, that the dorsal stream is used to figure out where things are in space. <br />
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The other stream of information is called the ventral stream. This flow of information is used to identify what things are in your environment. This stream of information is actually used more in the WS brain and has links to short term memories and language. Therefore the cognitive profile of someone with WS involves rich short term memories and language gifts yet they have a high amount of difficulty navigating spatially. Based on these brain studies, there are theories that the spatial tasks involved in mathematics is weak yet verbal aspects of math are strong, giving them a lopsided ability to fully understand mathematics. Due to the quizzical nature of the WS mind there are several research studies that test these two theories. While no definitive answers exist, there are definite findings that the WS mind can learn math, though it must be addressed differently than a typical child would learn.<br />
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(Visit <a href="http://understandingwilliamssyndrome.blogspot.com/2012/04/visuo-spatial-difficulties-and-how-they.html" target="_blank">this post</a> on my blog to learn more about the brain science associated with WS.)<br />
<h3>
<span style="color: #3d85c6;">Verbal vs. Spatial</span></h3>
When learning mathematics, there are two categories of problems- verbal math and spatial math. Considering the dorsal stream being spatial and ventral stream being language based, anyone with some knowledge of WS could probably predict that kids with WS will score strong in verbal math and perform poorly on the spatial math.<br />
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Examples of verbal math are language centered- such as naming and identifying numbers and counting including counting by 5 and 10s. These skills use the verbal stream of information and tend to be a relative strength for most kids with WS. <br />
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Examples of spatial math would be recognizing relationships between numbers such as using number lines, greater-than versus less-than and estimating amounts. For example, on one test students were given a triangle with two numbers (such as 5 and 9) at the base and one number (such as 6) at the point. The student was asked to choose the base number that is closest in size to the 6. Children with WS performed poorly on this task, most likely due to the spatial disability that is so strong in WS. In addition, if children or adults are given numbers and were asked to estimate which of them are closest together on a mental number line, those with WS had a much harder time identifying the correct answer. They also had increased difficulty the closer those base numbers become. If the outlying numbers are very different, they can usually figure out the answer.. the smaller the numerical gap, the more difficult the task becomes. This ability does not tend to improve with age. Many children with WS struggle with these same spatial tasks as they age and will need to learn how to compensate them with verbal memorization. <br />
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<h3>
<span style="color: #3d85c6;">Developing math skills</span></h3>
Studies that focus on WS mathematical disabilities vary greatly in their results. Overall testing tends to show that young children with WS tend to score comparably to those who are typically developing. As they mature and mathematics becomes more conceptual rather than concrete, some students begin to fall behind. Also, as a young child, math tends to focus more on verbal ability- counting, naming numbers and memorizing basic math facts. Children tend to excel in these task that require the ventral nerve stream. As they age, the spatial concepts are introduced such as greater than/less than, money and telling time. This is when parents often find their child struggling to understand mathematics.<br />
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In some of the mathematics studies, adults with WS performed math facts as expected for a typical adult. Psychologists who studied the active areas of the brain in those participants have found that those with WS use different parts of their brains to solve those math tasks in contrast to the average person, thus suggesting that they are "wired differently". This suggests that mathematics can be learned by those with WS but different methods of learning should be explored. The path to success is different for those with WS because they think differently than the other children in their classroom.<br />
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Another theory of why mathematics becomes more difficult for a person with WS is due to developmental aspects of mathematics. As a young learner, children use their frontal lobe to learn and recall mathematics facts and processes. The frontal lobe is used when you have to think about difficult and more complex thoughts. As students practice these mathematics principles they become rote and easy. The task then in stored in areas of the parietal lobe, particularly in the back portion of the brain along that dorsal stream. Therefore, adults can complete simple math that once challenged them mentally but now are more like reflexes. Because the area where those reflexes are stored is atypical in WS, some psychologists think that maybe adults with WS do not store those principles in their parietal lobe but always use their frontal lobes to figure out those types of problems.<br />
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<h3>
<span style="color: #3d85c6;">Strategies to help teach math</span></h3>
Because memorization is a strong skill for those with WS, the approach to learning mathematics should be language centered. Also, if a child with WS never really understands spatial math such as cardinality, number lines, etc. they should just move on to other skills that are more attainable. The mathematics instruction should be modified because some kids will never grow to understand those concepts despite the practice. They need to learn how to solve mathematics in other ways that focus on their verbal strengths rather than using spatial skills.<br />
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The Williams Syndrome association also provides a list of strategies, compiled by Dr. Karen Levine, that can be used to modify a child's curriculum. These suggestions are based on the spatial difficulties that most individuals with WS may never really develop even into adulthood- such as telling time with a analog clock and counting money.<br />
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Karen Levine, Ph.D. suggests the following modifications:<br />
<ul>
<li> "Digital clocks and watches</li>
<li> Calculator use</li>
<li> Teach time concepts by personalizing</li>
<li> Use wall calendars for daily, weekly and monthly schedules with events sketched or written in</li>
<li> Encourage the elementary school aged child to have a date book</li>
<li> Be flexible in curriculum, avoiding a rigid 'prerequisite' curriculum design</li>
<li> Some children may never learn coin values but should move on to the next curriculum phase which they may be able to more readily understand" (WSA)</li>
</ul>
There is also extensive evidence that the use of music therapy can help improve a child's ability to learn difficult, spatial concepts such as money, time telling, measurements and fractions. There is so much out there in regards to using music to help kids with WS, I decided it was too much for just one post... Look for future posts on the use of music therapy to help improve math success!<br />
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Sources:<br />
<ul>
<li><a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2104493/" target="_blank">Mathematical skill in individuals with Williams Syndrome: Evidence from a standardized mathematics battery</a></li>
<li><a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2104493/" target="_blank">Mathematical skills in Williams syndrome: insight into the importance of underlying representations; O'Hearn K</a></li>
<li><a href="http://www.williams-syndrome.org/teacher/information-for-teachers" target="_blank">Williams Syndrome Information for Teachers by: Karen Levine, Ph.D. </a></li>
<li><span style="font-size: small;"><a href="http://www.coastmusictherapy.com/how-music-helps/williams-syndrome-research/" target="_blank">Music therapy resources related to academics</a></span></li>
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Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com2tag:blogger.com,1999:blog-7345537858373518781.post-52553493556688557652012-12-31T11:09:00.003-08:002013-01-25T11:13:24.892-08:00Posture issues associated with WSAnyone familiar with someone with williams syndrome knows that the majority of individuals have an unusual posture. Their backs may sway and their bellies stick out in front. They may also walk with a stagger or in an uncoordinated fashion. Part of this has to do with spatial awareness and muscle coordination (see other blog posts on these topics). As an individual ages, these muscle abnormalities can take a toll on their posture. This is the main cause to some of the spinal issues that can develop in someone with WS.<br />
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Williams syndrome can be associated with various issues related to the spine- lordosis and kyphosis being the most common; scoliosis being the least. Lordosis and kyphosis typically develop during adolescence and/or adulthood and are preventable. Scoliosis may develop late in childhood. Research on WS individuals with scoliosis indicates that it may be coupled with other inheritable mutations outside the WS area and are linked to familial inheritance.<br />
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<h2>
<span style="color: #6fa8dc;">Bends in the spine due to muscle tone</span></h2>
Lordosis is commonly called swayback. It is when the spine has a pronounced curve in the lower back, called the lumbar region which can cause a belly that sticks out further than normal. It is the most common postural issue in WS, found in 38% of individuals.<br />
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<img height="320" id="il_fi" src="http://www.umm.edu/graphics/images/en/9583.jpg" style="padding-bottom: 8px; padding-right: 8px; padding-top: 8px;" width="400" /><br />
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Kyphosis is also known as humpback. This is when the spine typically curves outwards towards the back of a person. This condition is most common in the upper back just below the shoulder blades and behind the chest. It is found in 20% of individuals with WS.<br />
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<img height="320" id="il_fi" src="http://www.umm.edu/graphics/images/en/9499.jpg" style="padding-bottom: 8px; padding-right: 8px; padding-top: 8px;" width="400" /><br />
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The causes of kyphosis and lordosis in WS are rooted in two issues- the missing elastin (ELN) gene and the muscle tone of the lower extremities. Elastin deletion is the underlying genetic mutation used to diagnose WS. Elastin is a protein found in many connective tissues of the body that give the tissue both strength and flexibility. It can work much like a spring, allowing the tissue to lengthen but bounce back and retain its original structure. It is found in virtually every organ in the body and arranged in a way to improve the function of the structure. For example, it is found in rings around the arteries, in strips down a ligament or in net like sheets within the skin. <br />
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<img height="242" id="il_fi" src="http://mayoresearch.mayo.edu/mayo/research/biomechanics/images/figure05.jpg" style="padding-bottom: 8px; padding-right: 8px; padding-top: 8px;" width="350" /><br />
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Most notably in kyphosis and lordosis, the lack of elastin can cause ligaments that support the vertebrae to loose their strength. There are several sets of ligaments that cradle the vertebrae. The anterior longitudinal ligaments run long ways down the body of the vertebrae. This is the section of the vertebrae most central to the body, or towards the front. The posterior longitudinal ligaments run beneath the spinal process or points of the spine on the back side of the vertebrae. This is the area that you would touch if you ran your hand down your back. Both of these sets of ligaments are arranged with a connective tissue called dense regular tissue. This tissue has long sections of collagen and elastin that are packed tightly together and all run in the same direction. This allows some give to the ligament but due to the direction of the fibers, gives strength in the direction of the primary force (supporting the weight of the body on the pelvis). When there is less elastin, the ligaments become looser and strength is reduced. This can cause the vertebrae to slip out of alignment and compress the intervertebral discs in an abnormal fashion.<br />
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<img height="310" id="il_fi" src="http://antranik.org/wp-content/uploads/2011/10/ligaments-of-the-spine.jpg" style="padding-bottom: 8px; padding-right: 8px; padding-top: 8px;" width="419" /><br />
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The second issue with kyphosis and lordosis is muscle contractures or high tone in the lower extremities. Contractures are tightening of muscles due to increased tone. The contraction of the muscle pulls with extra force on tendons. This in turn can pull on bones and create an uneven posture, affecting bone alignment and the development of kyphosis or lordosis. It is important for individuals with WS to monitor their muscle tightness, especially in the legs and hips and to stretch them so that contractures do not get severe and cause further debilitating symptoms. (See my blog post on muscles for more information on this.) The prevalence of lordosis and kyphosis in WS is attributed to the muscle contractures that often occur in the lower body, particularly the hips, and lax joints due to the missing elastin in the ligaments of the spine. <br />
<br />
The tightening of the muscles are much more common in adults with WS than in children and postural issues tend to be very mild. These conditions are preventable through physical therapy. Typically children with williams syndrome receive physical therapy at an early age but as they reach adolescence that service often ceases. It is suggested that adults with WS get physical therapy evaluations occasionally to determine if kyphosis or lordosis is occurring due to tight muscles in the lower body. Stretching the tight muscles is the easiest and best preventive treatment you can take to avoid these issues. If muscles become very tight, it is important to get further treatment from a physical therapist and to see orthopedics. Some individuals will be fitted with orthodics that are designed to sustain a long stretch, sometimes overnight to treat the muscle. Nurturing muscle health is the primary way to treat kyphosis and lordosis related to WS.<br />
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<h2>
<span style="color: #6fa8dc;">Scoliosis</span></h2>
Unlike kyphosis and lordosis being the prevalent postural issues in WS adults, some individuals are born with or develop scolisis at a young age. Scoliosis is a curvature to the side. These can often be in a C shape or S shape. It is found in 12% of individuals with WS, which is considered a low incidence and typically presents itself by the 8th birthday. <br />
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<img height="320" id="il_fi" src="http://www.nortonhealthcare.com/images/spine_scoliosis.jpg" style="padding-bottom: 8px; padding-right: 8px; padding-top: 8px;" width="428" /><br />
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Scoliosis is known in the general population to be caused by a mutation on the fibrillin 1 (FBN1) gene, which is NOT in the WS region. It is found on chromosome 15. In all individuals with scoliosis in the general population, research indicates that this mutation accounts for 60% of cases. FBN1 is known to affect the ability for the body to properly create strong elastin. This is similar to the WS deletion which affects elastin's ability to bounce back under stress. The difference between the two causes of scoliosis is that those with the FBN1 mutation are more likely to develop scoliosis than those with WS, showing that the FBN1 gene more negatively affects elastin's strength in terms of spinal development. . <br />
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In Williams syndrome, scoliosis can be linked to a specific gene mutation in conjuction with the classic deletion in the WS region. The mutation is on a gene called SERPINA1. SERPINA1 is a gene that is responsible for creating a protein called α-1-antitrypsin (AAT for short). An AAT mutation is relatively common in the general population. If a person inherits the mutation on both of their genes it can cause serious health issues such as emphysema and liver disorder, although, a double mutation is rare. Carriers of one mutated gene can also have adverse health issues, such as scoliosis. Since many people carry this mutation on the SERPINA1 gene, it is possible that some individuals with WS would also be carriers. Researchers believe that this is the connection between a low incidence of scoliosis with WS. <br />
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AAT is a protein that is an inhibitor of an enzyme. Enzymes are proteins that help speed up reactions within the body. Without them our body would not be able to survive. The functioning of the body is basically a collection of complicated chemical reactions. Most of these chemical reactions wouldn't take place fast enough for us to survive so we have proteins called enzymes that reduce the amount of energy needed for those reactions to take place. We have thousands of enzymes in the body, each made to help speed up one particular reaction that we rely on for survival. <br />
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<img height="295" id="il_fi" src="http://waynesword.palomar.edu/images/enzyme5.gif" style="padding-bottom: 8px; padding-right: 8px; padding-top: 8px;" width="600" /><br />
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The AAT protein affects an enyme called elastase. Elastase is used by the body to reorganize elastin in the connective tissues. When the body is laying down elastin in large amounts, usually during rapid growth at the end of pregnancy and throughout the first year of life, the body undergoes large amounts of physical stress. This stress can create some inflammation within tissues, such as connective tissue. The body's response to this is to repair the tissue by breaking down the damaged elastin fibers and replacing them with new. The enzyme used to break down the elastin is elastase. <br />
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<span style="color: black;">With every enzyme it is important for the body to have a counteractive protein to control its reactions.</span> Enzymes are renewable meaning after they perform their desired reaction, they can disconnect from the substrate (or molecule they are breaking) and work on another. In the case with elastase, it would break down a portion of elastin and then disconnect and work on another. Therefore, the body makes a second protein, called an inhibitor, that slows down or stops the action of an enzyme within the body. This is the body's way of preventing too much degradation of the elastin proteins. When an individual has a mutation on the SERPINA1 gene, they fail to make enough of the AAT protein which is elastase's inhibitor. This means that their body can not slow down elastase from destroying elastin, causing an absence of elastin in areas of the body such as the joints.<br />
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<img height="280" id="il_fi" src="http://gleesonbiology.pbworks.com/f/1228731132/Competitive_inhibition.png" style="padding-bottom: 8px; padding-right: 8px; padding-top: 8px;" width="400" /><br />
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The destruction of elastin by AAT coupled with the inability to make elastin by the WS deletion is thought to be the main reason why some individuals (about 12%) with WS have scoliosis. The lack of the elastin compromises the structure of the discs that are in between the vertebral bones that function to cushion the vertebrae. An intervetebral disc is structured with a ball-shaped section of cartilage that is in the center, called the nucleus pulposis. This area of the intervertebral disc is very dense with elastic fibers that are designed to compress and absorb stress from the weight of your body on the vertebrae. In fact, over the course of a day the discs are said to actually be thinner than when you first wake up. This is the nature of the elastin, absorbing the force and then springing back to it's original shape. Circling around the nucleus pulposis are rings of connective tissue called the anulus. The anulus has connective tissues thick with collagen (fibers designed for strength) that alternate with rings of elastic fibers for flexibility. As you get closer to the center of the rings, the elastic fibers increase in number and are organized in a way where they sit against each other in different angles than its neighboring rings. The structure of these layers reflect the function of the disc (giving it strength from pressure in various directions). <br />
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<img height="320" id="il_fi" src="http://www.umm.edu/graphics/images/en/19469.jpg" style="padding-bottom: 8px; padding-right: 8px; padding-top: 8px;" width="400" /><br />
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Knowing the structure of intervertebral discs, it's not surprising to find that the lack of elastin can devastate the integrity of the structure. When a child goes through stages of rapid growth, the intervertebral disc, the bone in the spine and the ligaments that support those structures undergo a drastic change in force placed on them by the body. This can cause stress on the tissues and minor injuries. The distressed tissue will release chemicals indicating that it is injured, activating elastase, the enzyme designed to destroy elastin. If a individual has the AAT mutation, it is difficult for their body to reduce or stop this enzyme action resulting in lower levels of elastin in the body. Coupled with the inability for the body to make adequate amounts of elastin (due to the WS ELN deletion), the intervetebral discs and ligaments can lose their strength and fall out of alignment, resulting in curvature of the spine- scoliosis.<br />
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The spine or vertebrae are normally positioned in a way that they sit directly over the pelvis. Their primary function is to protect the spinal cord but they also contain a large base that absorbs much of the body's weight and allows us to stand erect and walk on two legs. When the spine is displaced such as in these issues, it can cause back pain, putting additional stress on the muscles of the back, and awkward gait when walking since the body is not positioned properly in line with the pelvis.<br />
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<h4>
<span style="color: #6fa8dc;">Other rarer conditions related to the spine</span></h4>
Some WS individuals may develop kyphoscoliosis which is a combination of kyphosis in the upper back and scoliosis. This condition is often due to having hypertonic (high tone in the legs and hips) coupled with hypotonia in the core of the body (low tone of the muscles). In addition to the unbalanced tone in the muscles, the muscles have a lax nature due to elastin deficiencey in the tendons that attach them to the spine and in the ligaments that connect each vertebrae to one another. Rarely if this occurs there can be a large curvature inwards that puts pressure on the heart and lungs and could create a need for surgery. Incidences of this occurring are all recorded in children.<br />
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<h4>
<span style="color: #6fa8dc;">Treatments of scoliosis</span></h4>
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Doctors and schools typically check for scoliosis between the ages of 8 to 10. They will often look at the curvature of the spine by having the child bend and touch their toes. If scoliosis is suspected, x rays will follow to determine if there is a degree of curvature. Anything less than 25 degrees is typically not treated, just followed to be sure it doesn't progress. If the curvature is 25-30 degrees, a back brace may be fitted. Curves greater than 30 degrees will often lead to surgical repair where they fuse the vertebrae or insert metal rods to support it. Surgery and treatment is always determined based on the patient, their needs and how much growth they still have in their future.<br />
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Sources used:<br />
<ul dir="ltr">
<li><div class="gb-volume-title">
<a href="http://books.google.com/books?id=Uv31LvYO4PMC&pg=PA151&dq=williams+syndrome+spinal+deformities&hl=en&sa=X&ei=rGvWULL5B4PsqAGz4IHQDg&ved=0CFsQ6AEwBQ#v=onepage&q=williams%20syndrome%20spinal%20deformities&f=false" target="_blank">Understanding Williams Syndrome: Behavioral Patterns and Interventions <span class="addmd">By Eleanor Messing Semel, Sue R. Rosner</span></a></div>
</li>
<li><div class="gb-volume-title">
<a href="http://books.google.com/books?id=jlYUi-f5bnIC&pg=PA204&lpg=PA204&dq=williams+syndrome+kyphosis&source=bl&ots=JVe79PUgoK&sig=V11Lae6cqKbETkUslKS5d291kH4&hl=en&sa=X&ei=44XWUKicLZHPqwG--4GQBQ&ved=0CDcQ6AEwATgK#v=onepage&q=williams%20syndrome%20kyphosis&f=false" target="_blank">Neurogenetic Developmental Disorders: Variation Of Manifestation in Childhood</a> <a href="http://books.google.com/books?id=jlYUi-f5bnIC&pg=PA204&lpg=PA204&dq=williams+syndrome+kyphosis&source=bl&ots=JVe79PUgoK&sig=V11Lae6cqKbETkUslKS5d291kH4&hl=en&sa=X&ei=44XWUKicLZHPqwG--4GQBQ&ved=0CDcQ6AEwATgK#v=onepage&q=williams%20syndrome%20kyphosis&f=false" target="_blank"><span class="addmd">By Michele M. M. Mazzocco, Ph.D., Judith L. Ross</span></a></div>
</li>
<li><div class="content-title">
<a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2911626/" target="_blank">Alpha 1 antitrypsin deficiency alleles are associated with joint dislocation and scoliosis in Williams syndrome; Colleen A. Morris, Ariel M. Pani, Carolyn B. Mervis, Cecilia M. Rios, Doris J. Kistler, and Ronald G. Gregg</a>.</div>
</li>
<li><a href="http://www.emedicinehealth.com/scoliosis/article_em.htm">http://www.emedicinehealth.com/scoliosis/article_em.htm</a><div class="content-title">
</div>
</li>
</ul>
Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com4tag:blogger.com,1999:blog-7345537858373518781.post-85979356655116166142012-09-16T19:48:00.001-07:002016-03-16T12:46:11.399-07:00Williams syndrome behavior profile- ADHDMany people are familiar with the symptoms of attention deficit/hyperactivity disorder. You envision a child who can't sit still nor concentrate on anything for very long. In the Williams syndrome community, it is not unlikely to find many of the individuals diagnosed with ADHD. Many individuals with WS have a very hard time paying attention to a task for a prolonged period of time. In my own experience we noticed inattentive issues from a very early age of one. Katie has trouble paying attention to a task during therapy for longer than only a few minutes, especially if it's a task that she doesn't enjoy. We have since used strategies to help her remain focused, such as centering activities around highly motivating topics, using music, sometimes eliminating objects that are too interesting to the point she won't do anything else and reducing environmental distractions. Considering she is only three, I foresee us needing to explore the ADHD topic in her future, but for now we use these strategies. Many families find that help from a psychologist is necessary for their child to be successful at home and school. This blog post is dedicated to them.<br />
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<span style="color: #3d85c6; font-size: large;">ADHD is tricky to treat...</span><br />
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In today's age, ADHD is so mainstream the acronym has become a term used in every day language. Its diagnosis in children has increased so much over the past 10-20 years that many believe students today are over diagnosed. With over diagnosis, many feel that kids are also over medicated. In the clinical world, however, psychologists see patients improving with the treatments and argue that ADHD is diagnosed more today than in the past because we are becoming more educated about the symptoms. Parents and educators can identify kids who need help better now than in the past. Psychologists don't see patients as unmotivated, lazy or free spirits. They see them as a person with a brain that functions differently and one that can be treated when they work closely with a physician. The increase in awareness has lead to more people receiving diagnoses and getting the treatment that they need to become more focused and successful.<br />
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ADHD is becoming more and more understood over time and as new research has answered baffling questions, the medical and psychological treatments offered have improved. Despite the improvements, ADHD is a very frustrating condition for parents, doctors and educators to address because the basis of the condition is centered around brain chemistry which can be very complicated. The variety of medications and the differences between how people's brains react to them can make treatment a long endeavor. You often have to start with what works for most and modify it with different combinations of medications and/or change medication schedules. <br />
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Another reason ADHD is so hard to treat is because it is often found paired with another disorder. Those with ADHD also tend to display other psychiatric disorders such as anxiety, learning or behavioral disorders or mood disorders. This rings true in WS, especially with anxiety, making the combinations of treatments very tricky to find the right balance. When treating ADHD, the treatment has to mesh with all the psychiatric disorders and often symptoms of one will mask symptoms of another, complicating treatment. This is why a child will often be put on one type of medication and will have to be carefully monitored to insure there aren't any adverse symptoms.<br />
<img src="http://www.hms.harvard.edu/hmni/On_The_Brain/Volume05/Number1/Picts/ADHD1.gif" height="243" id="il_fi" style="padding-bottom: 8px; padding-right: 8px; padding-top: 8px;" width="282" /><br />
Research has also furthered our understanding of the variety of symptoms of ADHD. It was once thought that ADHD was more prevalent in boys than in girls and the symptoms of hyperactivity. Today we know that there are various forms of ADHD that affect both genders. Research also indicates that 50% of children with ADHD don't actually grow out of it, the symptoms just change and the person often adapts. The condition, though, will still affect them throughout life. They have found that kids who are hyperactive and impulsive will shift as they age from the hyperactive classification towards a more inattentive classification. Their outward behavior may change indicating that they have "grown out of ADHD" but in reality the inattentive state is easier to mask or is often misunderstood. It is seen as a chosen behavior rather than a psychological disability.<br />
<span style="color: #3d85c6; font-size: large;"></span><br />
<span style="color: #3d85c6; font-size: large;">Diagnosing ADHD</span><br />
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The classification system for ADHD has frequently changed in the past. Today psychiatrists diagnose patients as Attention deficit-hyperactivity disorder followed by three types- inattentive type, hyperactive-impulsive type or a combination type. <br />
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All of us have experienced periods of inattentiveness or hyperactivity throughout times in our lives. The difference between an energetic kid and one with ADHD is that they have to have 6 out of the 9 behaviors outlined as ADHD and it must interfere with their normal functioning at TWO aspects of life: at school, work, social settings and/or at home for a period of 6 months or more. Here is a list of the behaviors associated with this condition (from the <a href="http://www.chadd.org/" target="_blank">National Resource center of ADIHD</a>):<br />
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</div>
<div>
"Criteria for the three primary subtypes are: <b>ADHD - Predominantly Inattentive Type</b><br />
<ul>
<li>Fails to give close attention to details or makes careless mistakes.
</li>
<li>Has difficulty sustaining attention.
</li>
<li>Does not appear to listen.
</li>
<li>Struggles to follow through on instructions.
</li>
<li>Has difficulty with organization.
</li>
<li>Avoids or dislikes tasks requiring sustained mental effort.
</li>
<li>Loses things.
</li>
<li>Is easily distracted.
</li>
<li>Is forgetful in daily activities.</li>
</ul>
<b>ADHD - Predominantly Hyperactive/Impulsive Type</b><br />
<ul>
<li>Fidgets with hands or feet or squirms in chair.
</li>
<li>Has difficulty remaining seated.
</li>
<li>Runs about or climbs excessively.
</li>
<li>Difficulty engaging in activities quietly.
</li>
<li>Acts as if driven by a motor.
</li>
<li>Talks excessively.
</li>
<li>Blurts out answers before questions have been completed.
</li>
<li>Difficulty waiting or taking turns.
</li>
<li>Interrupts or intrudes upon others.</li>
</ul>
<b>ADHD - Combined Type</b><br />
<ul>
<li>Individual meets both sets of inattention and hyperactive/impulsive criteria."</li>
</ul>
</div>
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<span style="color: #3d85c6; font-size: large;"><span style="color: #3d85c6; font-size: large;">The science behind ADHD</span><br />
<span style="color: #3d85c6; font-size: large;"></span> <br />
<span style="color: black; font-size: small;">There is still a lot to learn about the cause of ADHD. Scientists know that it has to do with the brain chemistry and neural connections. There is some evidence that certain environmental factors contribute to this condition but the current belief is that it is genetic and a child's environment can contribute to the severity of the symptoms (such as aggravating the condition due to food allergies or environmental toxins)</span><br />
<span style="color: black; font-size: small;"></span><br />
<span style="color: black; font-size: small;">ADHD has been identified as a disorder of the brain's ability to coordinate its executive functions. In an average person, the brain has neural pathways that coordinate your working memory, your ability to organize a task and your use of internal language where you think through and "talk to yourself" in order to make sense and coordinate a task. All of these tasks are difficult for someone with ADHD because those neural pathways do not work as efficiently as they should. </span><br />
<span style="color: black; font-size: small;"></span><br />
<span style="color: black; font-size: small;"><a href="http://www.drthomasebrown.com/pdfs/comChapter.pdf" target="_blank">Brown et al.</a> explains this impairment of executive function as being synonymous to a type of leadership role. Think of a conductor of an orchestra who has to coordinate several types of musicians to play at the right time and tempo in order to produce beautiful harmony. Your brain works in much of the same way. You are receiving information from your environment and you need to choose what to act on, pull from memory on how to act on it, coordinate muscles and glands to produce the right combinations of actions, etc. It takes quite a bit of organization for your brain to maintain this task. This executive function of coordinating all the thoughts, memories, actions and interpreting the information your getting is coordinated by the executive function of the brain. The very place where ADHD has deficits.</span><br />
<span style="color: black; font-size: small;"><img src="http://www.nwresd.k12.or.us/autism/files/brain_000.jpg" height="220" id="il_fi" style="padding-bottom: 8px; padding-right: 8px; padding-top: 8px;" width="320" /></span><br />
<span style="color: black; font-size: small;">Furthermore, as a person ages, they are called upon to use this executive function more and more. As a youngster, kids are hyper, they don't have big responsibilities and as they age they will be challenged more and more to use that executive function. This is why the inattentiveness becomes more apparent as a child ages. Often if a child doesn't have the hyperactive part of ADHD, they aren't even diagnosed as having inattentiveness until they reach middle-high school where they are called upon to take on more responsibility.</span><br />
<span style="color: black; font-size: small;"></span><br />
<span style="color: black; font-size: small;">In addition to executive functions, those with ADHD have deficits in their working memory. Your working memory is a portion of your brain that takes information from the long term storage of memory and puts it into action. Basically its like opening a file cabinet of things you know and reading a folder you need to use at the moment. It is linked to acting on what you know, making connections between what you are learning to what you have learned and it is essential for understanding and initiating tasks. Deficits in this area will lead to students who don't finish tasks or have trouble starting them. </span><br />
<span style="color: black; font-size: small;"></span><br />
<span style="color: black; font-size: small;">There are emotional ties to those with ADHD as well. They often become hyper focused on something interesting and although they know they should be engaged in another activity and that if they don't it will cause them "trouble" down the road, they physically cannot find attention for the less interesting stimulus. This type of ADHD is often coupled with other psychological disorders such as mood disorders.</span><br />
<span style="color: black; font-size: small;"></span><br />
<span style="color: black; font-size: small;">Research is unclear on the actual brain science that causes ADHD. Much of the early research has pointed to the neural pathways in the pre-frontal cortex (the portion of your brain behind your forehead). This area of the brain is what creates your personality, your ability to problem solve and think through academics. It essentially is the part of the brain that makes you, well, you. Later research indicates that, yes, this area of the brain is affected, but so are neural pathways or highways between memory in the thalamus, deep in the center of the brain and the parietal lobe where sensory information is processed in the top back of your brain. All these areas must coordinate efforts to produce a behavior and this is the essential workings of that executive function of the brain.</span><br />
<img src="http://functionalcranialrelease.com/wp-content/uploads/2011/11/prefrontal_cortex.jpg" height="261" id="il_fi" style="padding-bottom: 8px; padding-right: 8px; padding-top: 8px;" width="400" /><br />
<span style="font-size: small;"><span style="color: black;">There is also evidence that brain chemistry has a lot to do with ADHD, particularly, dopamine. Dopamine is a neurotransmitter, a tiny chemical that is made by cells in the brain that allow one neuron to communicate with another. It is thought that ADHD has a deficit of dopamine and <span style="font-family: "timesnewromanpsmt";"></span></span></span><br />
<span style="font-size: small;"><span style="color: black;"><span style="font-family: "timesnewromanpsmt";">catecholamines. There is a lot left to understand about this chemistry but it is widely known that medication that focuses on the increase of dopamine is effective in preventing inattentiveness in those with ADHD.</span></span></span><br />
<span style="color: black; font-size: small;"><img src="http://www.cs.stedwards.edu/chem/Chemistry/CHEM43/CHEM43/NeuroT/dopamine%20diagram.jpg" height="376" id="il_fi" style="padding-bottom: 8px; padding-right: 8px; padding-top: 8px;" width="289" /></span><br />
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Treating ADHD in someone with WS is extra tricky...</span></div>
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More than 50% of individuals with Williams syndrome are diagnosed with ADD or ADHD. In studies, children with WS were compared to those with ADHD with comparable verbal abilities versus a control group of typical children. The children with WS were most like those diagnosed with ADHD and scored abnormally on the Conners ADHD rating scale. One Williams syndrome study showed that 43% of their study participants had ADHD and most of them were due to inattentiveness, not hyperactivity nor impulsiveness. <br />
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There are only a handful of researchers who have studied ADHD and WS together. This means that your WS child will most likely baffle a psychologist. As mentioned before, ADHD as a whole is difficult to treat in anyone because most with ADHD have another condition in conjunction with it. Most individuals with WS will have learning difficulties, anxiety, ADHD and their unique hyper-social personalities that will make identifying a treatment very difficult for most.<br />
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Most kids with WS don't meet every criteria of inattentiveness. For example, a child with WS that is highly interested in something, such as a tv show, learning about their favorite item or are participating in highly motivating activities, such as music, will stay on task whereas the classic ADHD child will not be able to sit still regardless of the activity. Also, kids with WS tend to become distracted by specific environmental triggers, such as noise, music, peer conversations, shiny objects and unexpected or novel items introduced to their environment. Kids with WS tend to have selective attentiveness. They have trouble maintaining their focus with external distractions that are interesting to them and as a result retain partial information. Typically kids with ADHD will be inattentive for longer periods of time. Other differences stem from the WS profile. While kids with ADHD are often found to have trouble reading people socially, are less able to become empathetic with others. WS is the exact opposite of this. <br />
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In addition to a slightly different inattentive profile, individuals with WS display different behaviors than other children with ADHD inattentive type. There are some researchers that argue against labeling WS with ADHD because kids with WS lack aggression that is oppositional to adults. Kids with WS that act out are often due to anxiety or frustration due to their verbal ability rather than due to hyperactivity. This is just another example of how ADHD is not black and white as far as treatment goes. The combination of inattentiveness, anxiety and the WS behavior profile make diagnosing and treating ADHD very difficult for psychologists.<br />
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<span style="color: #3d85c6; font-size: large;">ADHD treatment for those with WS</span><br />
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There are only 3 studies as of date that studied the effectiveness of medications for ADHD in children with WS, making the treatment difficult for doctors and the families. The studies also have small sample sizes so further studies are really needed before anyone should suggest one treatment over another. Treating ADHD has always been very difficult and often require various trials from psychologists. One study focused on the effectiveness to treat individuals with WS using methylphenidate (MPH), the medication found in Ritalin. In the study, of 30 children treated, 60% of them improved (3 highly improved and another showed moderate improvement). This improvement rate is the same as the effectiveness in the general population of ADHD children. The main side effect was sadness, quiet, and withdrawn behaviors which was shown in 61% of the children taking MPH, so any children showing signs of depression should not be given this medication. This side effect is much higher in those with WS than in individuals with ADHD alone (8%-22%).</div>
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<span style="color: #3d85c6; font-size: large;">To conclude</span>:</div>
<div>
So, in conclusion, there is a lot to learn about WS and the treatment of inattentiveness. It is important for doctors and educators to work with the children. Treatment will take time and will require parents to communicate effectively with psychologists so that the proper medications and timing is discovered for your child. It is also important that schools include modifications and strategies for classroom teachers to use that will help your child maintain better focus in school. Patience and open communication are essential for treating children with WS and ADHD.</div>
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<span style="color: #3d85c6; font-size: large;">Sources</span>:<br />
<ul>
<li><a href="http://www.ncbi.nlm.nih.gov/pubmed/20700845" target="_blank">http://www.ncbi.nlm.nih.gov/pubmed/20700845</a> Abstract of a study that shows the prevalence of ADHD in individuals with WS.</li>
<li><a href="http://jdc.jefferson.edu/cgi/viewcontent.cgi?article=1003&context=jeffjpsychiatry" target="_blank">http://jdc.jefferson.edu/cgi/viewcontent.cgi?article=1003&context=jeffjpsychiatry</a> small psychiatric study that attempted to show that ADHD and anxiety in WS is due to the genetic deletion rather than family inheritance.</li>
<li><a href="http://books.google.com/books?id=9E9q5112WBgC&pg=PA286&lpg=PA286&dq=adhd+williams+syndrome&source=bl&ots=LdcJeiR7v0&sig=1iiN2afkh91X4siWCceAmkkXO-4&hl=en&sa=X&ei=0M_AT8-mDrKBsgLU9PTzCQ&ved=0CH0Q6AEwBA#v=onepage&q=adhd%20williams%20syndrome&f=false" target="_blank">http://books.google.com/books?id=9E9q5112WBgC&pg=PA286&lpg=PA286&dq=adhd+williams+syndrome&source=bl&ots=LdcJeiR7v0&sig=1iiN2afkh91X4siWCceAmkkXO-4&hl=en&sa=X&ei=0M_AT8-mDrKBsgLU9PTzCQ&ved=0CH0Q6AEwBA#v=onepage&q=adhd%20williams%20syndrome&f=false</a> "Understanding williams syndrome behavioral patterns and interventions"</li>
<li><a href="http://onlinelibrary.wiley.com/doi/10.1002/ajmg.b.31247/full" target="_blank">http://onlinelibrary.wiley.com/doi/10.1002/ajmg.b.31247/full</a> Study about the use of MPH treatments in individuals with WS.</li>
<li><a href="http://www.ncbi.nlm.nih.gov/pubmed/9203066" target="_blank">http://www.ncbi.nlm.nih.gov/pubmed/9203066</a> Second study about MPH and WS</li>
<li><a href="http://www.drthomasebrown.com/pdfs/comChapter.pdf">http://www.drthomasebrown.com/pdfs/comChapter.pdf</a> A book that summarizes studies on ADHD and explains what trends in research say about the condition.</li>
</ul>
Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com5tag:blogger.com,1999:blog-7345537858373518781.post-418594274999930882012-04-23T10:15:00.001-07:002016-03-16T12:52:02.211-07:00Visuo-spatial difficulties and how they cause motor delayYou're standing in a field, crouched in position for a fly ball. Crack! You hear bat against ball. Your attention sharpens, your eyes focus on the movement in the air, you run to position your body in its path, hold out your glove, anchor your body to absorb the force, make adjustments in your stance and position as it approaches and you catch it. All of these actions, although simple to most, are nearly impossible for someone with Williams syndrome. As mentioned in other sections of this blog, those with Williams syndrome have low tone so their muscle strength and response is slow, but that is only part of the equation of motor delay. Many of the brain studies that were discussed in the speech section of this blog focus on the spatial difficulties that are prominent in Williams syndrome (WS). This section of the blog focuses on how this spatial disability inhibits movements in ways separate from low tone. <br />
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<span style="color: #3d85c6; font-size: large;">So, what does visuo-spatial mean?</span><br />
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Many individuals with WS have a hard time interpreting where they are in space. They also struggle with directional orientation, such as understanding right from left and mirror images. Visuo-spatial difficulties mean a person would have a hard time judging their surroundings, primarily with visual information, in order to understand where they are in their environment. For example, imagine yourself navigating down a busy staircase. It is crowded with people and you must walk in a cramped space. Now make that stairway spiral and you must move with a swift motion to keep in pace with the crowd. What do you do? You may run your finger tips along the stair railing as you move. You keep your eyes down to the ground to evaluate where you will step. You tense up the trunk of your body for stability. All of these actions are your adaptations to that environment. Your fingertips are gathering information about your position and balance. Your eyes relay info to your brain about where it is safe to step and your core is in guard to stabilize your body. These are all visuo-spatial skills. <br />
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People constantly interpret a large amount of sensory information about their environment. You use peripheral vision, cues from receptors in your muscles about your physical orientation (proprioreceptors), balance information from the inner ear (the vestibular apparatus) and visual cues about what is around you in space. Those with WS seem to have difficulty coordinating this information. They struggle when presented with situations where they need to make shifts in their space, such as changing their posture on a crowded bus to let someone walk by. This body awareness issue along with their difficulties in motor planning, spatial cues and directional cues make it hard for them to do planning activities such as when it is appropriate to cross a busy street or the ability to judge the speed of oncoming traffic. This is one reason many of them do not drive as an adult (along with anxiety issues- see a future blog post on this topic) <br />
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The directional disability also contributes to reasons why many of the individuals have difficulty understanding left from right, even as an adult and they have some difficulties understanding mirror images. This directional disability also contributes to handedness. Most children establish whether they are right handed or left handed by the age of 4-6. Individuals with WS often don't achieve this until the age range of 5-8. Many studies suggest this is due to the brain disorganization. Most with WS will alternate between a preferred hand, use one hand for household tasks, such as eating, and another for writing. They may alternate the use of their hand when activities require them to cross over the body to complete a task, such as building a large block tower. Most with WS become left handed. <br />
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There are several theories on why those with WS have this visuo-spatial disability:<br />
<ul>
<li><u>deletion of the LIM-kinase I gene</u>. There is research out there, although in its infancy, that the deletion of this gene is correlated with the visuo-spatial disability. However, there are case studies of children missing this gene who do not display spatial delays, so evidence is inconclusive.</li>
<li><u>A disconnect in the dorsal stream nervous pathway</u></li>
<li><u>An atypical pattern of brain activity</u></li>
</ul>
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<span style="color: #3d85c6; font-size: large;">It all has to do with the cellular pathways in the brain</span><br />
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Many of the researchers in brain studies are psychologists who study the brain function of children and adults with WS. Their goal is to attempt to identify the areas of the brain that are medically classified as "dysfunctional" or have slower motor pathways. Before getting into the brain studies, lets take a look at some basic brain anatomy that will help you picture why this "dysfunctional" classification is assigned.<br />
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<span style="color: #3d85c6; font-size: large;">Background on Neural pathways</span><br />
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The human brain is made up of many neurons, or nerve cells. These cells have cell bodies that are unique in shape and extending from the main portion of the cell are processes or "arms", so to speak. There are processes, called dendrites, that receive messages. Sensory neurons sit outside the central nervous system and collect information from the environment using their dendrites. These sensory neurons have endings called receptors that monitor the environment. This message containing information about the environment is sent down a long process (or arm) called the axon to a second neuron in the central nervous system. Neurons in the central nervous system, called association neurons, are located in the brain and spinal cord. They function to process this sensory information- by interpreting what is happening around you and how the body should react to it. Then, the association neuron will communicate a new message, send it down its axon to a motor neuron. The axon releases a chemical (called a neurotransmitter) which travels across a gap and talks to the motor neuron. This motor neuron then takes that message and tells the muscles how to move.<br />
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Inside the brain there are many of these "thinking" neurons. Depending on where they are in the brain, they have different jobs. Some areas of the brain receive visual information whereas a separate part receives auditory info, for example. There are also areas that are for figuring out the sensory info and then a separate area for linking that info to a memory so you can label it or attach it to an emotion. All of this takes quite a bit of coordination within the brain in order to take in information from multiple senses and combine it to create a scene of what is happening in your environment.<br />
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<span style="color: #3d85c6; font-size: large;">Neurons have jobs </span><br />
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There are special nerve tracts within the center portion of the brain that connect the all the sensory pathways so the brain can share the info. These pathways are called white matter. White matter is buried deep inside the brain and is the color white because of tiny cells that wrap themselves around the neurons, called myelin. The myelin is a fatty layer that allows the message to move quickly down the axon. It makes for very fast messages and is essentially a "highway" system of neurons that move info from one side of the brain to another. <br />
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The outer surface of the brain, which sits around the outside of the white matter is called grey matter. The grey matter creates what we called the "cerebral cortex". This is where the "magic" happens. The cortex is made up of unmyelinated neurons, or neurons that are "naked" without that fatty layer. The messages are sent more slowly here. In these regions, your brain decides what to do, problem solves and determines how you will behave. It is well known that the higher IQ or the better "thinker" you are, the thicker this portion of the brain is. The grey matter builds up in folds called gyri. These ridges of the brain are the same on everyone, but they are thicker/thinner based on your genetics and how much you challenge yourself as a learner. In between the gyri are shallow grooves called sulci. <br />
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In the speech section of this blog, I mentioned that in brain studies, researchers have found that individuals with WS tend to have very thick gyri in areas that are strengths for them- particularly in the auditory region and language centers of the temporal lobe. There are regions of the brain that have much thinner gyri. These thinner areas of the occipital (visual) and parietal (sensory) lobes result in a visuo-spatial disability in those with WS.<br />
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<span style="font-size: x-small;">Figure shows comparisons of gyri in controls (samples from the general public) versus gyri of individuals with WS. Red areas indicate increases in gyri thickness and blue indicates smaller gyri. Green shows areas that are comparable between the two groups. </span><br />
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<span style="color: #3d85c6; font-size: large;">Streams- flow of information within the highway of the brain </span><br />
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The flow of sensory information that moves through the white matter in the brain can take a variety of different routes. Two of the more important visual routes are the dorsal stream and the ventral stream. <br />
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There is a section of grey matter in the back of the brain that makes up one gyrus in the parietal lobe. This gyrus is smaller in the brain of someone with WS than in a typical person. This section of the brain is involved in the dorsal visual stream. In the dorsal visual stream, the brain uses visual information to interpret its surroundings, such as an obstacle, and determines how you will move around it. This stream of information is very slow in an individual with WS due to the small amount of grey matter, making it more difficult for them to navigate. Research has also shown that in individuals with WS, the brain often doesn't even use this stream when you'd expect it should. In MRI's this area of the brain shows low activity during movement tasks.</div>
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<span style="font-size: x-small;">The highlighted area on this picture shows the gyri that is abnormal in WS. This disrupts the dorsal stream of visual information that is used to produce motor activities.</span><br />
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The ventral stream, in contrast is a strength for those with WS. It involves information moving from the parietal lobe to the temporal lobe where the gyri are much thicker. This stream of neural activity is used to recognize people using visual information and labeling. In case studies, these streams can be tested fairly easily. If you ask someone with WS to identify the a pathway through an obstacle course they could look at it and tell you where the midpoint of the path is (using the ventral stream) but if you ask them to walk it (which uses their dorsal stream) they would move very slowly and clumsily through the pathway.<br />
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Particular motor difficulties that are directly related to deficits in the dorsal stream and are seen in the majority (97%) of individuals with WS include:<br />
<ul>
<li>poor dexterity</li>
<li>slow speed in movements with the arms and legs</li>
<li>inability to move in response to visual information</li>
<li>difficulty manipulating an object in the proper orientation to place it in a slot that is shape specific (such as a card in a slot or a block in a shape sorter) </li>
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Problems with nervous pathways are a increasing area of study in WS research. The nerve interactions between the frontal lobe and parietal lobe point toward behavioral difficulties that are very common in individuals with WS- including high distractability, inability to maintain prolonged attention to a task, acting impulsively and having difficulty understanding global concepts (topics that are not concrete in thinking). (Look for a future blog post on ADHD and behavioral profiles of individuals with WS.) <br />
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<span style="color: #3d85c6; font-size: large;">Making plans...</span><br />
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Another skill that is inhibited by dorsal stream dysfunction has to do with motor planning. Motor planning means that the child would see what is in their environment (such as a ball flying at them through the air) have to think of how they want to respond (such as catch it), plan on what muscles need to be used to do so and where that ball will land in space and then relay the message to those muscles to complete the task. Typically developing children will accomplish this task but many of those with WS often watch the ball as it hits them. This disability in motor planning- often called apraxia, seems to be a difficulty in about 92% of individuals with WS. This skill is even more difficult in certain situations such as bouncing the ball because they have to predict what direction it will land. These tasks that require a person to use a familiar task and modify them to match the spatial information is very difficult for them.<br />
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The motor planning dysfunction will often delay their ability to throw and catch a ball. Although most kids with WS will throw and catch a ball by the age of 6.5, they will likely have a lifelong inability to throw (51%) and catch (67%) in a coordinated fashion. When throwing a ball, one must rotate their body, move their arm and often step forward with their leg. Those with WS display an inability to do this at all ages. They often will throw their arm but lack the body positioning and rotation in the upper body to make a decent throw. The catching action is mainly due to the visuo-spatial tracking and motor planning required to predict where the ball will land and those skills needed to right the body and extend the arms quickly enough to catch the ball in time. They simply process this information too slowly and inaccurately in order to accomplish the task.<br />
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Studies have shown that although visuo-spatial difficulties are an issue for nearly everyone with WS, there are tools that children can learn to help minimize this disability. Case studies frequently note that those individuals who learned or utilized verbal cues were better able to navigate obstacle courses. For example, if the person who is walking through the course studies it first and vocalizes a plan, then as they walk they talk about how to move their body, they move less awkwardly and accomplish the task with better timing. It is also important to note that individuals that participated in these studies had varying degrees of difficulty. Some were only slightly impaired in the task and others had higher difficulties with most having a moderate level of challenge. Therefore, while spatial navigation is a disability for all individuals with WS, the magnitude of that disability lies on a spectrum and can be different for each individual.</div>
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<span style="color: #6fa8dc; font-size: large;">Walk this way</span><br />
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Poor motor abilities in an individual with WS extend to many other issues that are rooted in the nervous system. Individuals with WS, especially in the early years, have a very distinguishable gait, or walk, that is described as clumsy and uncoordinated. There are a variety of reasons for this.<br />
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First, young children who are new to walking have ingrained protective reflexes that they use to maintain balance. If they find themselves fighting gravity or on an uneven surface, they will right their head over their body, tighten their core and throw their arms outwards to steady their bodies and protect them from a fall. Kids with WS seem to lack this reflex (I can personally attest that my daughter has fallen many times without ever extending her arms out to catch herself, leading to minor head injuries). <br />
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The majority (between 60-80%) of children with WS have gross motor delays or unorganized motor skills and delays associated with climbing stairs, walking down stairs, running, jumping (especially off an elevated surface), transitioning from one variegated surface to another, walking on uneven terrain (such as grass or mulch/sand), skipping and running. These delays or motor planning deficiencies are associated with balance issues. Balance is related to the processing of sensory information by the nervous system. Approximately 60% of those with WS have balance processing disorders and another 80% have trouble interpreting gravitational signals. <br />
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Those with WS have trouble navigating quickly through obstacles that require them to take longer than average strides. They improve this skill when sensory cues are present such as lights to step in to determine stride length. But even with sensory cues their walk is much slower than typical. This indicates there may be some dysfunction within the cerebellum, which is the part of the brain that controls balance and coordination. Other cerebellar studies have found that in WS, the neocerebellar lobules are enlarged.<br />
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These are regions on the sides of the cerebellum that have major nervous pathways that communicate with the thalamus and the cerebral cortex. The thalamus is the main area in the center of the brain that associates sensory information with memory. Major nerve tracts link problem solving to memory to the cerebellum through this nerve tract. <br />
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Scientists have linked this stream directly to motor coordination when learning a new motor skill. It's used for following a series of steps used to follow a motor procedure, such as riding a bike. It coordinates limb movements in order to achieve the desired action. This area of the cerebellum is also heavily linked to an area of the brainstem called the superior colliculi. This is a visual reflex area that helps coordinate the motor movements in the eyes. Dysfunction in this can lead to poor muscle control in the eyes and can be another cause of strabismus (see the eyes section of this blog). The neocerebellar area also helps to coordinate motor movements used to coordinate speech.<br />
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Besides brian studies, there are other reasons individuals with WS may have a harder time with motor activities. <br />
<ul>
<li>Tone- The ability for the nervous system to control muscle contractions in appropriate times; previously discussed in this blog (See the muscles section).</li>
<li>Sleep- sleep is a well known difficulty for up to 97% of individuals with WS which can further affect cognitive development and motor planning.</li>
<li>Vision- Although vision is not an issue for all children with WS, if a child has strabismis or crossing of the eyes sends conflicting information to the brain about the person's surroundings. This can lead to increased delay in motor skills- particularly spatial understanding. This is even more evident if the individual has lost vision in the weaker eye. This causes the body to lose their depth perception. Everything will appear flat and in 2 dimensions. This will cause additional issues with motor development. (see the eye section of this blog)</li>
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<span style="color: #3d85c6; font-size: large;">Fine motor delays due to visuo-spatial disabilities</span><br />
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Spatial difficulties offer up difficulties in a variety of motor tasks. Early in a child's life occupational skills will seem less serious than gross motor skills but as the child ages, their abilities will change and fine motor skills will become increasingly important as they gain independent living skills. <br />
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<span style="color: #3d85c6; font-size: large;">Self help</span><br />
Most children (80%) have delays in fine motor skills required for self help. These can be related to directional disability (used to set a table, for example) but most are due to the visuo-spatial disability. Through therapy, most of these skills can be mastered, but approximately 30% of adults still find difficulty in some skills such as tying shoes, buttoning clothing, etc. For example, many will have high difficulty using knife skills, such as those used to make a peanut butter sandwich. They may have trouble grasping the knife, creating the motion to spread the butter, applying the proper amount of force and stabilizing the bread. This takes motor planning and the ability to judge the environment of the bread and make small motor adjustments to have the proper movement. Other self help skills such as writing, cooking, buttoning clothing, using a zipper and tying shoes are difficult for a person with WS due to the need to plan motor movements during these activities and have spatial awareness. One study found that, on average there is a 2 year delay in children with WS, aged 4-12, in both fine motor skills and gross motor skills that require visuo-spatial ability.<br />
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One major fine motor activity a child with WS will find difficulty in is the ability to manipulate objects in space, such as placing mail into a narrow slot. This skill is processed by the dorsal stream in the parietal lobe of the cerebrum. Other examples of difficult motor task include movement planning time. In one case study, researchers had adults draw a line between two circles using a stylus. When the shapes changed sizes, those with WS had significantly slower times completing the task. This study linked difficulties with this task to the inability for those with WS to anticipate the movement of an object and plan their motor response to it.</div>
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<span style="color: #3d85c6; font-size: large;">Drawing</span><br />
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Most typical children will draw recognizable pictures of objects by the age of 5 or 6 whereas those with WS draw them closer to the age of 9 or 10. This is due to the visuo-spatial delay. They draw comparably, though, to peers with mental disabilities. IQ and drawing ability do not match in WS indicating it is an area of disability. It is also notable to say that they do eventually achieve the ability to do this task by adulthood.<br />
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<span style="font-size: x-small;">This photo is from the study completed by Dr. Mervis et al. and it shows a drawing of a bicycle, completed by a 12 year old with WS. All the components of the bike are present, but those with WS have a difficult time picturing how they are connected- a spatial skill.</span><br />
Therapists have identified a strategy that help individuals with WS improve their ability to draw. This should be used in OT sessions. The increase in gains when using the face as a drawing tool stems to brain studies that show individuals with WS use their brain differently to interpret faces. Typical adults will process facial recognition with the right side of their temporal lobe. Those with WS use a much larger area of the brain and primarily use the left side of the brain to do this. The study also had interesting evidence that those with WS use the same amount of processing to interpret the face of a picture of a person that is upright versus on that is flipped up side down. In typical adults, there is a delay in processing the flipped images as the brain has to try and associate the image with what they'd look like right side up. Those with WS use more brain activity looking at a face in any position than a typical person would and they use the same brain activity despite the picture orientation.<br />
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Ways to help improve their drawing skill is to allow them to draw motivating pictures- focus on drawing people, facial expressions, etc rather than shapes. Kids showed greater gains when they had developmental interpretation therapy session to help them process how the picture should fit together. They also improved with frequent practice. Case studies show that in children, ages 4-6, who participate in the developmental interpretation sessions and practiced drawing people and houses showed significant gains in elaboration of the picture, increases in inclusion of objects, improved their ability to draw an object in its proper context (like a person in a house) and increased in the ability to combine features (all the parts of the picture connected in the proper ways such as heads were on necks and legs attached to bodies). Improvement has also been shown to have the subject verbally express what they are drawing and how it should connect the lines. When they talk through the process, the picture ends up more organized.<br />
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In the same study the kids were assessed again between the ages of 12-15. After 6 years of growth, the ability to draw more organized pictures improved in all subjects of the study. So, although the skill is delayed, it does improve with time. In all age groups, the subjects were able to draw more organized pictures of people and flowers versus objects such as houses, bikes and animals. <br />
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<span style="color: #3d85c6; font-size: large;">In conclusion</span><br />
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Visuo-spatial difficulties are an issue for all individuals with WS but with purposeful and educated therapists, there are skills and techniques that they can learn to help them overcome this obstacle and improve their self help and motor skills as they age.<br />
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<span style="color: #3d85c6; font-size: large;">Sources:</span><br />
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<ul>
<li><a href="http://www.nature.com/nrn/journal/v7/n5/full/nrn1906.html" target="_blank">http://www.nature.com/nrn/journal/v7/n5/full/nrn1906.html</a> Primary source on brain studies linked to WS</li>
<li><a href="http://www.psych-it.com.au/Psychlopedia/article.asp?id=412" target="_blank">http://www.psych-it.com.au/Psychlopedia/article.asp?id=412</a> A summary of psychatric studies related to brain and behaviors in WS.</li>
<li><a href="http://www.dartmouth.edu/~rswenson/NeuroSci/chapter_8B.html" target="_blank">http://www.dartmouth.edu/~rswenson/NeuroSci/chapter_8B.html</a> Information on cerebellum function</li>
<li><a href="http://www.n-acetylaspartate.com/" target="_blank">http://www.n-acetylaspartate.com/</a> Information on the neurotransmitter, N-acetylaspartate</li>
<li><a href="http://www.tandfonline.com/doi/abs/10.1080/87565641.2003.9651890" target="_blank">Neurobiological models of visiospatial cognition in children with williams syndrome</a></li>
<li><a href="http://www.springerlink.com/content/p57121g83734300g/" target="_blank">http://www.springerlink.com/content/p57121g83734300g/</a></li>
<li><a href="http://www.physio-pedia.com/index.php/William's_Syndrome#cite_note-Seventeen-16" target="_blank">http://www.physio-pedia.com/index.php/William's_Syndrome#cite_note-Seventeen-16</a> An overview of Williams syndrome manifestations related to physio-motor intervention</li>
<li><a href="http://books.google.com/books?id=9E9q5112WBgC&pg=PA136&lpg=PA136&dq=motor+delay+williams+syndrome&source=bl&ots=LdcBfjN6p1&sig=4uSFQrsE1J7LibATNjKYZnFb6I8&hl=en&sa=X&ei=QVtIT62ACJD_sQLj_anNCg&ved=0CCkQ6AEwATgK#v=onepage&q=motor%20delay%20williams%20syndrome&f=false">http://books.google.com/books?id=9E9q5112WBgC&pg=PA136&lpg=PA136&dq=motor+delay+williams+syndrome&source=bl&ots=LdcBfjN6p1&sig=4uSFQrsE1J7LibATNjKYZnFb6I8&hl=en&sa=X&ei=QVtIT62ACJD_sQLj_anNCg&ved=0CCkQ6AEwATgK#v=onepage&q=motor%20delay%20williams%20syndrome&f=false</a> A google sampling from the book "Understanding Williams Syndrome"- used the sections of motor delay</li>
</ul>
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Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com2tag:blogger.com,1999:blog-7345537858373518781.post-89829557504015974322011-12-30T09:43:00.000-08:002011-12-30T10:01:00.519-08:00Chiari MalformationChiari malformation (spoken as kee-AHR-ee) is a disorder of the skull where the cranium that holds the brain is small at its base. The brain is cradled and protected in a casing of bone called the cranium. The cranium is made up of 6 fused bones- the frontal bone, sphenoid bone, ethmoid bone, parietal bone, temporal bone and occipital bone. The occipital bone is the most inferior or lowest bone in the cranium and it protects the back of the brain.<br />
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The occipital bone in humans is curved at the base which allows it to cradle the round brain tissue inside. This curved portion of the occipital bone is called the posterior fossa and it sits just under the ridge in the back of the head that you can feel (called the occipital protrubance). <br />
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The posterior fossa cradles the most posterior (back) and inferior (lowest) portion of the brain called the cerebellum. The cerebellum is a smaller domain of the brain that coordinates motor function- such as balance and coordination. It has many nerve tracts that communicate with the cerebrum where your brain "decides" on how to move and react to its environment. Also in the cerebellum are tiny channels and chambers that act as canals to move nutrient-rich fluid (called cerebral spinal fluid or CSF) around the brain feeding it and cleansing it of waste. CSF acts much like the blood supply but unlike blood, it does not carry cells other than those of your immune system; essentially protecting it from viral and bacterial infection. The CSF travels through the cerebellum in a canal, called the cerebral aquaduct, that moves from the cerebral area down into the brainstem. The CSF accumulates in a chamber that sits just under the cerebellum, called the 4th ventricle. From here it drains down another canal into the spinal cord through the central canal. At the base of the occipital bone is a large opening called the foramen magnum where the brain stem exits the cranium and leads to the spinal cord. <br />
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In WS, mis-shapen cranial bones are prevalent making Chiari an unfortunate complication of the disorder for some. When the cranium is formed during fetal development, some people's posterior fossa is mis-shapen. This can happen in two ways. First the base of the skull that fuses with the facial bones, called the clivus, can be shorter than normal consequently crowding the cerebellum. The second malformation that can occur is in the tentorium cerebelli. This is a fiberous covering that separates the cerebrum from the cerebellum. This membrane when situated in a steep fashion pushes down on the cerebellum and crowds the space.<br />
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Due to the crowding, a portion of the cerebellum called the cerebellar tonsils can be compressed into the base of the skull and/or extend down into the foramen magnum (the hole where the brainstem exits the skull and becomes the spinal cord). If the cerebellum is crowded in this space, the tonsils extend down into the hole and essentially block the passage way of the CSF flowing out of the 4th ventricle into the central canal of the spinal cord. </div>
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The arrows in the picture on the left show the normal flow of CSF through the brain. The picture on the right shows that when the cerebellum sits too low in the posterior fossa it cuts off the flow of the CSF around the cerebellum. <br />
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The severity of crowding can cause pressure to be exerted on the cerebellum creating neurological or motor issues. It can also close off the channels where CSF moves causing it to build up pressure and stop its flow which essentially will cut of supply of CSF to parts of the brain stem and spinal cord. </div>
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<span style="font-size: x-small;">picture from </span><a href="http://www.chiariinstitute.com/"><span style="font-size: x-small;">www.chiariinstitute.com</span></a> </div>
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<span style="color: #6fa8dc; font-size: large;">Types of Chiari</span><br />
Chiari Malformation can occur in various degrees of severity. It is considered a congenital disorder (where it is formed at birth) but symptoms are often not witnessed until adolescence or early adulthood because the pressure can cause neurological damage over time. In Williams Syndrome (WS), type 1 Chiari Malformation occurs in 10% of cases. Type 1 is less severe and often is not accompanied by any symptoms. It's found more commonly than the other types and is often diagnosed in conjunction with other neurological disorders rather than on its own. Type I is often considered an adult form because it is often not usually discovered until later in life. <br />
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<span style="font-size: x-small;">A brain scan showing Chiari type 1.</span></div>
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There are also two other types of Chiari Malformation. Type II, also called Arnold-Chiari Malformation is typically found in conjunction with a disorder of the spinal cord called spina bifida where the spine doesn't close properly during growth in-utero and the spinal cord protrudes from the back. This type is usually discovered during pregnancy in an ultrasound because it is accompanied with other spinal abnormalities that are more obvious. The third type, which is rare and most severe is Type III which leads to long term debilitating neurological issues and requires surgery and long term treatment. <br />
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There is not much known if Chiari is a hereditary disorder. There are a few documented instances where it seems to run in a few families but not much is known about genetic links to this disorder.<br />
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<span style="color: #6fa8dc; font-size: large;">Symptoms</span><br />
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Most people with Chiari will experience headaches typically at the base of the head or neck and are often treated for pain. Many end up with severe headaches after coughing and sneezing. Other symptoms include changes in the voice and difficulty swallowing often with gagging or choking. Because the cerebellum controls coordination, body movements can become difficult. This can include spatial difficulties, dizziness, blurred vision, poor fine motor control (such as holding a pencil and writing), numbness and tingling in the hands and slurred speech. Other symptoms that are considered more rare are sleep apnea, ringing in the ears, poor bladder control, scoliosis and chest pain.<br />
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Unfortunately for those with WS, the symptoms of Chiari malformation are common issues in WS such as coordination issues, gagging and swallowing issues and fine motor delays so it may be difficult to spot them if your child cannot communicate that they have a headache.<br />
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If Chari malformation is suspected a neurologist will often complete an MRI to assess the bone formation. They will conduct a special test called a cine-MRI which tests the flow of the CSF through the brain to see if there is blockage. <br />
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In some people with Chiari, syringomyelia may develop due to nerve tract damage. In this complication, a canal or cyst will develop in the spinal cord and fills with fluid. This can cause additional pressure in the canals that carry CSF and can cause further nerve damage.<br />
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<span style="font-size: x-small;">Arrows indicate regions of syringomyelia</span></div>
<span style="color: #6fa8dc; font-size: large;">Treatment</span><br />
Surgery is the only treatment that can stop neurological damage to the central nervous system and depending on the severity several surgeries may be needed to effectively repair the cranial crowding. If there is any doubt about need for surgery it will usually be delayed. There are three reasons a neurologist would suggest surgery for this disorder: 1. There is obvious neurological damage especially if it worsens over time; 2. If other spinal cord issues are present as well (such as tethered cord, scoliosis, spina bifida, etc); and 3. If the symptoms of Chiari greatly affect the person's ability to cope day to day (such as if the headaches are too great to manage). <br />
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The surgery itself is called decompression surgery. The goal of the surgery is to relieve pressure exerted on the channels that carry CSF so that proper flow is restored and to relieve any pressure exerted on the cerebellum by the skull. This is accomplished by performing an occipital craniectomy which means they remove some of the skull bone at the base of the skull to increase the space in the occipital posterior fossa. This may not require going into the brain itself; it focuses on changing the shape of the bone. The surgery can also include a C1 laminectomy which is surgical change to the first bone in the vertebrae (cervical vertebrae #1). In this procedure, they remove an archway that surrounds the spinal cord and possibly the ridge that forms the portion of the spine that you feel when you touch the back. The goal of this is to relieve pressure where the cranium and vertebrae meet (where the headaches often exist). In these sections where the bone is removed, the tough brain coverings that sit underneath the bone remain intact. <br />
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At this point of the surgery some surgeons (5-10%) stop; about 45% also remove dura mater (called a duraplasty) which is the most common type of surgery for this disorder. Dura mater (meaning "tough mother") is the very tough covering that surrounds the brain and spinal cord. It has the consistency of thin plastic, like that of a water bottle.<br />
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The dura mater would be cut and then a synthetic patch of it would be sewn in to increase the area and free up more space. This procedure, called a graft, can use tissue from the patient itself, bovine pericardium (a sack lining that surrounds the heart of the cow) or synthetic material. The material used is really the preference of the surgeon. There is no research that shows one is better than another. <br />
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From here another 45% of doctors will also remove the second layer surrounding the brain called the arachnoid space. This layer is part of the channel system that skirts CSF around the brain and it often has lesions in it that when broken, can free up room for the cerebellum. In some cases, the surgeon may also shrink the cerebellar tonsils themselves by either cauderizing them (which makes them shrink) or resecting them (cutting a portion out) to relieve the crowding and open up the channels for CSF flow. This manipulation of the cerebellum itself is more risky because by interrupting the arachnoid layer, the patient can be exposed to risks of bacterial or viral infections in the brain such as meningitis. <br />
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During surgery, ultrasounds are often use to constantly assess the flow of the CSF and the position of the cerebellar tonsils as room is freed up. The success of the surgery really depends on the patient's severity and how the brain is compressed. Some patients only need one surgery but approximately 30%, typically those with spinal or cranial deformities and other spinal complications, will need follow-up surgeries because the condition will relapse later in life.<br />
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<span style="color: #6fa8dc; font-size: large;">What to do if you suspect Chiari</span><br />
If you suspect Chiari malformation in your child with WS, it is important to see a neurologist. The <a href="http://www.mayoclinic.com/health/chiari-malformation/DS00839/DSECTION=preparing-for-your-appointment" target="_blank">Mayo Clinic</a> has a great web page with information you should bring to your first meeting and a list of questions to ask so you are well informed about the condition.<br />
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Sources used in this blog post:<br />
<a href="http://www.ninds.nih.gov/disorders/chiari/chiari.htm" target="_blank">National Institute of Neurological Disorders and Stroke</a><br />
<a href="http://www.csfinfo.org/physinfo" target="_blank">Chari and Syringomyelia Foundation</a><br />
<a href="http://www.mayoclinic.com/health/chiari-malformation/DS00839" target="_blank">The Mayo Clinic</a><br />
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<a href="http://www.upright-health.com/posterior-fossa-chiari.html" target="_blank">Upright Health: Posterior fossa and Chiari Malformation</a></div>Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com1tag:blogger.com,1999:blog-7345537858373518781.post-91240260063323947612011-10-30T08:58:00.000-07:002016-03-16T11:52:43.901-07:00Curved fingers and toes- Clinodactyly<div class="MsoNormal" style="margin: 0in 0in 10pt;">
<span style="font-family: "calibri";">Although it is a harmless condition and usually requires no treatment or even sought after advice from a specialist, many parents with children who have Williams syndrome remark about their curly fingers and toes.<span style="mso-spacerun: yes;"> </span>Most often present in the 5<sup>th</sup> finger (pinkie) or the 3, 4<sup>th</sup> or 5<sup>th</sup> toes, the digit is often seen curving inwards or overlapping with a neighboring finger or toe and the finger itself may look “stubby” or have a triangular or trapezoidal shape to its tip.<span style="mso-spacerun: yes;"> </span></span></div>
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<span style="font-family: "calibri";"><span style="color: #3d85c6; font-size: large;">Curly Toes</span> (<a href="http://www.sciencephoto.com/media/89125/enlarge">see a great picture of curly toes here</a> )</span></div>
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<span style="font-family: "calibri";">Curly and overlapping toes are considered highly common in general population and don’t suggest any DNA issues.<span style="mso-spacerun: yes;"> </span>They are usually found passed down through family lines. <span style="mso-spacerun: yes;"> </span>The most common place to have a curly toe is when the 4<sup>th</sup> and 5<sup>th</sup> toes overlap.<span style="mso-spacerun: yes;"> </span>Although there hasn’t been a great deal of research as to why this happens, there are some accepted theories as to why this occurs.<span style="mso-spacerun: yes;"> </span>Many believe it is either due to the baby’s position in the womb or there is a slight deformity in the joint of the toe.<span style="mso-spacerun: yes;"> </span>When the misaligned joint is coupled with either hypotonic or hypertonic muscles of the foot (see the muscles section of this blog), tension is placed on the tendon of the small toes pulling them out of alignment.<span style="mso-spacerun: yes;"> </span></span></div>
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<span style="font-family: "calibri";">A tendon is a strong cord of connective tissue that attaches muscles to bone.<span style="mso-spacerun: yes;"> </span>Tendons often act like an ace bandage, creating tension around a joint to stabilize it and allow the muscle to move the bone to create motion in the body.<span style="mso-spacerun: yes;"> </span>If the muscle is too weak or too tight it can cause the tension of the tendon to either be too low or too high.<span style="mso-spacerun: yes;"> </span>This unbalanced tension then will cause the bone to become misaligned. <span style="mso-spacerun: yes;"> </span>This can cause a more extreme overlapping of the toe than what you’d see in your family members.<span style="mso-spacerun: yes;"> </span></span></div>
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<span style="font-family: "calibri";"><span style="mso-spacerun: yes;"></span>Usually clinodactyly doesn’t require any medical attention unless it progressively becomes worse turning into a joint contracture or if it interferes with walking.</span></div>
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<span style="font-family: "calibri";"><span style="color: #3d85c6;"><span style="font-size: large;">A hooked finger</span></span></span></div>
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<span style="font-family: "calibri";">While curly toes are not used as a clinical diagnosis of any genetic anomalies, hooked fingers are.<span style="mso-spacerun: yes;"> </span>A curved finger is considered typical in 10% of the general population but more likely than not, it indicates a DNA anomaly.<span style="mso-spacerun: yes;"> </span>Found most common in individuals with Down Syndrome, a hooked pinkie is a structural abnormality that is present in approximately 60 different syndromes. <span style="mso-spacerun: yes;"> </span>A hooked or curled pinkie finger indicates some sort of bone deformity usually attributed to delayed in utero growth (growth in the womb).<span style="mso-spacerun: yes;"> </span>It often shows up on ultrasound and can be used to suggest further prenatal testing or guidance of a perinatalist although it is rarely a means of diagnosing any syndrome since it is so common in many genetic issues.<span style="mso-spacerun: yes;"> </span>Basically, it can be used as a red flag. </span></div>
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<span style="font-family: "calibri";">The cause of clinodactyly in the pinky finger is due to a structural issue in the growth plate on the bone, called the epiphyseal plate.<span style="mso-spacerun: yes;"> </span>The growth plate is a layer of cartilage at the end of a long bone (like those in the fingers and limbs).<span style="mso-spacerun: yes;"> </span>As the child’s body grows, the cartilage is continually replaced with a bone matrix causing the bone to grow in length.<span style="mso-spacerun: yes;"> </span>In clinodactyly, the bone’s growth plate is misaligned.<span style="mso-spacerun: yes;"> </span>This causes the toe to grow in a curved fashion rather than straight.<span style="mso-spacerun: yes;"> </span>In addition to growth in an abnormal direction, the tip of the finger will often end up looking triangular or trapezoid in shape.<span style="mso-spacerun: yes;"> </span></span></div>
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<span style="font-family: "calibri";"><span style="mso-spacerun: yes;"></span>Again, unless there is extreme overlap on the finger to the point that it inhibits the child from gripping properly or having proper hand function, no treatment or worry is warranted. </span></div>
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<span style="font-family: "calibri";">Sources:</span></div>
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<a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1255945/" target="_blank"><span style="font-family: "calibri";">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1255945/</span></a><span style="font-family: "calibri";"> </span></div>
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<a href="http://www.podiatrynetwork.com/document_disorders.cfm?id=194" target="_blank"><span style="font-family: "calibri";">http://www.podiatrynetwork.com/document_disorders.cfm?id=194</span></a><span style="font-family: "calibri";"> </span></div>
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<a href="http://childrenshospital.org/az/Site1160/mainpageS1160P0.html" target="_blank"><span style="font-family: "calibri";">http://childrenshospital.org/az/Site1160/mainpageS1160P0.html</span></a><span style="font-family: "calibri";"> </span></div>
Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com7tag:blogger.com,1999:blog-7345537858373518781.post-56664953024915255562011-09-18T14:14:00.000-07:002011-09-18T14:14:42.228-07:00Updated muscles sectionGreetings followers! I have added more to the muscle page. If you scroll past part 1 I've added a section on high tone in the muscles and joint contractures. I spent most of the time talking about toe walking, which is the most common type. I hope you find it useful!<br />
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If you have any topics that you'd like to learn more about, leave a comment. I'd be happy to make a post for you.<br />
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Take care and happy fall!<br />
SarahSarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com1tag:blogger.com,1999:blog-7345537858373518781.post-89611960952062127852011-08-04T18:44:00.000-07:002011-10-08T09:30:00.422-07:00Absorbing calciumAlthough the cause of <span class="blsp-spelling-error" id="SPELLING_ERROR_0">hypercalcemia</span> is a mystery 1in Williams syndrome, we do know a little about how the calcium is absorbed in the gut. Many people think that the stomach is the main area of digestion in your body. Although it does digest proteins, the majority of the food is broken down and absorbed by the small intestine. The lining inside of your small intestine is a network of finger-like bumps that are filled with blood vessels and covered by a very thin layer of skin that sits between the vessels and the food/enzyme mixture in your gut.<br />
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<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhXforZgycjJ4UQNwlR2fuwHIWcXkHdOFP4b7uDktac5MI5Asq26r72BqTfIJDIH4CBvJ5xMcBOgynsOwvIdRNRKfEAH4FvuL_aKVtB8mCb72aHSY8qskY_6ywJuf-cbevwntmuiGQ-RDLJ/s1600/Small-Intestine-300x227.jpg"><img alt="" border="0" id="BLOGGER_PHOTO_ID_5637378817493431410" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhXforZgycjJ4UQNwlR2fuwHIWcXkHdOFP4b7uDktac5MI5Asq26r72BqTfIJDIH4CBvJ5xMcBOgynsOwvIdRNRKfEAH4FvuL_aKVtB8mCb72aHSY8qskY_6ywJuf-cbevwntmuiGQ-RDLJ/s320/Small-Intestine-300x227.jpg" style="cursor: hand; float: left; height: 227px; margin: 0px 10px 10px 0px; width: 300px;" /></a>Most calcium in your small intestine is absorbed in the lower regions called the <span class="blsp-spelling-corrected" id="SPELLING_ERROR_1">ileum</span> and jejunum. These are the main portions of your intestine that absorb nutrients and minerals. Calcium here will move passively into the blood stream. This means that it can easily slip through little spaces in the skin layer (called the epithelium) and into the blood. If your diet provides your body with enough calcium, this is the main type of transport you'll use.<br />
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If calcium levels are low, your body has to work a little harder to get the amount it needs. This is where vitamin D comes in. Vitamin D operates channels that collect calcium in the upper third of the small intestine, called the duodenum. These channels are activated when vitamin D binds to proteins in the epithelium (skin) layer. These proteins work with active transport, where the body uses energy to pump the calcium into the blood stream, increasing its levels in the blood. This is why, if you increase the vitamin D in your diet, you end up increasing the calcium in your blood stream.<br />
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This vitamin D metabolism is one of three theories I could find that try to explain infantile <span class="blsp-spelling-error" id="SPELLING_ERROR_2">hypercalcemia</span> in <span class="blsp-spelling-error" id="SPELLING_ERROR_3">WS</span>. Researchers have found that when children with <span class="blsp-spelling-error" id="SPELLING_ERROR_4">hypercalcemia</span> are managing their calcium levels and only slightly rise the vitamin D in their diet, their calcium levels increase dramatically. They found that by only making small increases in vitamin D, children with <span class="blsp-spelling-error" id="SPELLING_ERROR_5">WS</span> absorb 2-3 times more calcium than what would be expected in a typical child.<br />
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Another factor that influences calcium absorption is the type of food you eat. If you are a milk drinker, you're going to have more passive calcium absorption- the easy kind in the lower intestine. Milk contains <a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjDp-7tkFw3tCHUYA7VhRSXMXXTWhod178OLg7XYl-gwADxTxRrQ7nHw6f-_XBbks3Vju-vln6qiBSs2Zz3yYATO8acrOrGAjAOQs905moTYiBS23XB0ybgQgHfn71rFVteihYCQZ5UaJqN/s1600/High-Oxalate-Foods.jpg"><img alt="" border="0" id="BLOGGER_PHOTO_ID_5637378813428663698" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjDp-7tkFw3tCHUYA7VhRSXMXXTWhod178OLg7XYl-gwADxTxRrQ7nHw6f-_XBbks3Vju-vln6qiBSs2Zz3yYATO8acrOrGAjAOQs905moTYiBS23XB0ybgQgHfn71rFVteihYCQZ5UaJqN/s320/High-Oxalate-Foods.jpg" style="cursor: hand; float: left; height: 225px; margin: 0px 10px 10px 0px; width: 225px;" /></a>sugars called lactose and an enzyme called <span class="blsp-spelling-error" id="SPELLING_ERROR_6">lactase</span> both of which help the body collect the calcium and absorb it into the blood stream. Other foods also contain calcium, such as spinach. Foods that are high in fiber and contain calcium tend to be harder for your body to digest. The fiber, called oxalate, binds to the calcium and holds on to it as it passes through the gut. Therefore, if your primary calcium sources are in high fiber foods, you will essentially absorb less of them and excrete more due to the food's chemical nature.<br />
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Oxalate has other affects on your body, too. If the<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEi9rERqFmbkyaFq8SbN1S3iKcYWlb7FUzyjymZlC_KMtl_tg_XlTjdhSS2IWD7mCoh-mrFO1KAuuL2tfbu0geqaiqMQpVf1GBhFCZz8K9WZNcCT_CuQJ6kAP0lJxMOBqk-NXHoZmZFji1o5/s1600/kidney+stone.jpg"><img alt="" border="0" id="BLOGGER_PHOTO_ID_5637394704932095746" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEi9rERqFmbkyaFq8SbN1S3iKcYWlb7FUzyjymZlC_KMtl_tg_XlTjdhSS2IWD7mCoh-mrFO1KAuuL2tfbu0geqaiqMQpVf1GBhFCZz8K9WZNcCT_CuQJ6kAP0lJxMOBqk-NXHoZmZFji1o5/s320/kidney+stone.jpg" style="cursor: hand; float: right; height: 251px; margin: 0px 0px 10px 10px; width: 230px;" /></a> level of calcium is low, your body will start to absorb more oxalate instead of excreting it. Essentially, if you are in a pinch for calcium, you'll take what you can get even if it's in a form that you don't really prefer. The increase in oxalate signals the kidneys to work harder to get rid of it. Calcium oxalate then builds up in the kidneys and can cause stones, or <span class="blsp-spelling-error" id="SPELLING_ERROR_7">nephrocalcinosis</span> (see the growth and diet page on this blog). This is why some doctors will place a child with <span class="blsp-spelling-error" id="SPELLING_ERROR_8">hypercalcemia</span> on a low oxalate diet- to prevent the uptake of calcium and reduce dangers of developing kidney stones.<br />
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It's important to note that although many <span class="blsp-spelling-error" id="SPELLING_ERROR_9">WS</span> infants with <span class="blsp-spelling-error" id="SPELLING_ERROR_10">hypercalcemia</span> have higher than normal levels of vitamin D, there are exceptions to the rule. There is a significant population of individuals who have high levels of calcium and LOW levels of vitamin D. Since most foods contain both, this can create quite the <span class="blsp-spelling-corrected" id="SPELLING_ERROR_11">dilemma</span> to try and maintain proper levels of each. Stay tuned for future blog posts discussing this topic and other theories of why our little ones have <span class="blsp-spelling-error" id="SPELLING_ERROR_12">hypercalcemia</span>!<br />
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Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com4tag:blogger.com,1999:blog-7345537858373518781.post-34232916947910245572011-08-02T20:08:00.000-07:002011-08-05T08:59:13.655-07:00WS- a major player in what we "know" about vitamin DOver the past couple of days I've had a couple of parents send me questions about calcium and vitamin D and how they affect <span id="SPELLING_ERROR_0" class="blsp-spelling-error">WS</span>. Those questions will be answered in future blog posts but until then, I found some interesting information about how <span id="SPELLING_ERROR_1" class="blsp-spelling-error">WS</span> has changed the way many researchers have viewed vitamin D. I've enjoyed getting questions from other parents because I've learned so much in the quest to answer them! Keep them coming :)<br /><br /><br /><br />Although this is a science-based blog, here is your history lesson for the day:<br /><br /><br /><br /><ul><br /><br /><li><br /><br /><p>In the 1960's it was thought that <span id="SPELLING_ERROR_2" class="blsp-spelling-error">supravalvular</span> aortic <span id="SPELLING_ERROR_3" class="blsp-spelling-error">stenosis</span> (the characteristic heart defect of <span id="SPELLING_ERROR_4" class="blsp-spelling-error">WS</span>) was caused by the mother ingesting too much vitamin D during pregnancy. At the time, researchers didn't know about Williams syndrome and its genetic component. A study performed in the late 1950's drew a connection between <span id="SPELLING_ERROR_5" class="blsp-spelling-error">SVAS</span> and <span id="SPELLING_ERROR_6" class="blsp-spelling-error">hypercalcemia</span> (or high calcium in the blood). <span id="SPELLING_ERROR_7" class="blsp-spelling-error">WS</span> is the only identified disorder that has unexplained <span id="SPELLING_ERROR_8" class="blsp-spelling-error">hypercalcemia</span> before the age of 1. So, the researchers put two and two together and concluded that high levels of vitamin D lead to heart defects and all the symptoms of what we today know as Williams syndrome- <span id="SPELLING_ERROR_9" class="blsp-spelling-error">SVAS</span>, low IQ, and <span id="SPELLING_ERROR_10" class="blsp-spelling-error">hypercalcemia</span>. The study started a Vitamin D scare that changed the Food and Nutrition Board recommended values for Vitamin D to lower levels in Britain and the US. Until the 1980's when genetics identified that <span id="SPELLING_ERROR_11" class="blsp-spelling-error">SVAS</span> is due to a genetic defect did the vitamin D theory change.</p></li></ul><br /><br /><p>Recently, a new vitamin D theory has surfaced and again, it's basic idea is based on what else, Williams syndrome:</p><br /><br /><ul><br /><br /><li>A group of researchers began in 2007, studying a link between vitamin D deficiencies with the increase of autistic children. The researchers studying this connection are using Williams syndrome as the basis of their hypothesis. Their reasoning is that the highly sociable personalities of <span id="SPELLING_ERROR_12" class="blsp-spelling-error">WS</span> are opposite those of autistic children. They think that social behavior is directly related to vitamin D levels- <span id="SPELLING_ERROR_13" class="blsp-spelling-error">WS</span>, having high levels of calcium and vitamin D in the first year of life leads to high social nature versus autism that could potentially have low levels of vitamin D and exhibiting anti-social behaviors. Their hypothesis is that vitamin D levels in the body determine the social nature of each disorder. </li></ul><br /><br /><p>They don't comment on the fact that some kids with <span id="SPELLING_ERROR_14" class="blsp-spelling-error">WS</span> are also autistic, making me skeptical, but still an interesting study! It just goes to show you, what you "know" today may not be considered right in the future. And that my friends, is what makes science so interesting to me. There is always more to learn.</p>Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com0tag:blogger.com,1999:blog-7345537858373518781.post-2827531011208933672011-07-23T13:04:00.001-07:002011-07-23T13:13:51.830-07:00updated eye sectionA fellow <span id="SPELLING_ERROR_0" class="blsp-spelling-error">WS</span> mom pointed out that <span id="SPELLING_ERROR_1" class="blsp-spelling-error">elastin</span> may not cause the star burst pattern in the eye, so I dug a little deeper on the subject. Although I couldn't find information on genetically why our little ones have the star burst pattern in their eyes, I did find great information about how the iris is structured differently to cause the pattern... if you've already read the eyes section, check it out one more time. <br /><br />I also fixed the links to blogs and websites on the right. It seems I had some broken links... enjoy!Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com0tag:blogger.com,1999:blog-7345537858373518781.post-80422767857371499342011-07-20T19:35:00.001-07:002011-07-21T07:16:59.793-07:00Interpreting microarray results<span style="font-size:100%;">So, if you are one of the chosen few to get a microarray genetics test to diagnose Williams syndrome, you will receive a result that will look something like this:</span><br /><br /><br /><div><br /><div><br /><div><br /><div><span style="font-size:100%;"></span></div><br /><div><span style="font-size:100%;"></span></div><br /><div><span style="font-size:100%;">"A 1.55 Mb deletion was observed at 7q11.23 from linear location 72,337,897 - 73,837,643"</span></div><br /><br /><div></div><br /><div><span style="font-size:180%;color:#66cccc;"></span></div><br /><div><span style="font-size:180%;color:#66cccc;">Size of the deletion</span></div><br /><br /><div></div><br /><div></div><br /><div></div><br /><div>So what does this mean? Let's start with the 1.55 Mb. Mb stands for million basepairs. DNA is made up of a string of base pairs (adenine or A, thymine or T, guanine or G and cytosine or C). A stretch of base pairs or gene is basically the blueprint for one functional protein. So, if you get results that there is a 1.55 Mb deletion then your child is missing 1, 550,000 total base pairs from their DNA. The classic or average deletion in a person with WS is 1.5-1.8 Mb.<br /></div><br /><br /><br /><div><span style="font-size:180%;color:#66cccc;">Chromosome maps</span></div><br /><br /><div><span style="font-size:180%;color:#66cccc;"></span></div><br /><div></div><br /><div>Chromosome labels give specific information about the location of the gene so that scientists can communicate information easily. The WS deletion is at 7q11.23. The first number on the location marker represents the chromosome the gene is found on. There are 23 total chromosomes in the human genome and Williams syndrome is a deletion on the 7th chromosome.<br /></div><br /><br /><br /><div>The next letter tells the scientists which half of the chromosome to find the gene. Chromosomes look like two threads tied together near the center by something called the centromere. Think of the centromere like a belt. The belt usually fits off-center so one side of the chromosome will have longer sections (called arms) than the opposite side of the centromere. This is where the q comes in. The q (short for queue) means that the deletion sits on the long arm of the chromosome. If the deletion was on the short arm it would say p (short for petite).</div><br /><br /><div><br /></div><img style="TEXT-ALIGN: center; MARGIN: 0px auto 10px; WIDTH: 249px; DISPLAY: block; HEIGHT: 251px; CURSOR: hand" id="BLOGGER_PHOTO_ID_5631647888269996050" border="0" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjbs56oOkV5CceMp9yTv5PneFIy3-0MFeZ7pgT3VDcYYXjiqxTDvr3eyPdDnWa2LvAQYHCk9cWteyjBNA5M2ih-fXIM6jI51Q8moYDmLn2EAC3d_mZxYK1A8Mj0namLONFMXKq4LgtBNxhj/s400/arm_chromosome.jpg" /> </div><br /><br /><div>When scientists study a chromosome, they stain it and take a photograph called a karyotype. The stain will create a banding pattern on the chromosome. This band is how genes are grouped and labeled. The numbers at the end of the chromosome map indicate how far that band sits from the centromere. The 11 indicates that the WS region is the 11th band away from the centromere and sits at a sub-band 23.</div><br /><br /><div><img style="TEXT-ALIGN: center; MARGIN: 0px auto 10px; WIDTH: 400px; DISPLAY: block; HEIGHT: 171px; CURSOR: hand" id="BLOGGER_PHOTO_ID_5631648954477139634" border="0" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEh5YozSXhRyWSmlsA49ewSH8MuU74KFeLV5z_z6ZgyjsClDSMfWCjn02EUWHSqA2A8pYY3tpOqIpvzX126AlLpOrWfw1yv9XNKkCNYfUjKJws-KCnWE1N7hZBf7VaZGa0ykO7gcnVzshexv/s400/ws+chromosome.jpg" /><br /><br /><div><span style="font-size:180%;color:#66cccc;">So what genes are missing?</span></div><br /><div><span style="font-size:180%;color:#66cccc;"></span></div><br /><div></div><br /><div>The final section of the microarray results will tell you what gene locations are missing. The base pairs are numbered throughout the chromosome. So, this hypothetical person is missing base pairs #72,337,897-73,837,643. At the end of this blog post, I've listed the genes in the WS region with their base pair ranges. You can see what region of the chromosome your child is missing and then look up the functions of the genes, some of which I've described on the genetics page of this blog. You'll find that there are some numbers missing from the list. These areas are considered "genetic junk" Most genes have long strands of base pairs that do not code for any useful protein and are largely ignored by the body.</div><br /><br /><br /><div></div><img style="TEXT-ALIGN: center; MARGIN: 0px auto 10px; WIDTH: 360px; DISPLAY: block; HEIGHT: 579px; CURSOR: hand" id="BLOGGER_PHOTO_ID_5631654388541236082" border="0" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEiaB9Kj6WdT9gBlvH07NK2IghZjpqksSc3Kat-ltsPbDVAzR_eSnuYuu87H1_2ZkGYPlfIqvR-2wYcq5fVQbW4PJfB_BbL6U7t1SvOoBN0i71DdicugncAN57l2XfB996v3q08HGuqkUyaX/s400/base+pairs.png" /><br /><br /><br /><br /><br /><br /><div></div><br /><br /><br /><br /><br /><br /><div></div></div></div></div>Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com6tag:blogger.com,1999:blog-7345537858373518781.post-7338457987334369822011-07-19T17:01:00.000-07:002016-03-16T12:07:25.268-07:00Mitral valve prolapse<div>
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Valve prolapse is not as common among individuals with <span class="blsp-spelling-error" id="SPELLING_ERROR_0">WS</span> but it does occur in some. In the world of congenital heart defects (<span class="blsp-spelling-error" id="SPELLING_ERROR_1">CHD</span>) which are heart defects present at birth, it actually affects about 2-3% of the <span class="blsp-spelling-error" id="SPELLING_ERROR_2">CHD</span> population.</div>
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<span style="color: #66cccc; font-size: 180%;">What is a valve?</span></div>
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In order to understand how prolapse occurs, you should first be familiar with the function and anatomy of a valve. Valves are used throughout the cardiovascular system to direct blood flow. They act like one-way doorways that swing open, allowing blood to move through, and close behind, preventing it from flowing backwards. This essentially seals off each chamber in the heart so that when the muscles squeeze and push blood into a vessel, blood isn't pushed backwards. This is important for many reasons. Without valves, blood pressure would be more difficult to <span class="blsp-spelling-corrected" id="SPELLING_ERROR_3">achieve</span>. You wouldn't have the pressure of the blood pushing on the vessels if it had another outlet to flow into. Second, if there were multiple outlets, some blood could get caught in limbo between the two chambers, just swishing back and forth and not really moving anywhere. This is dangerous because it could cause it to clot. Clots can lead to several issues including heart attack. So, you can see, valves are important structures to keep everything flowing properly.</div>
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Valves are structured much like a balloon or parachute, attached to cords called <span class="blsp-spelling-error" id="SPELLING_ERROR_4">chordae</span> <span class="blsp-spelling-error" id="SPELLING_ERROR_5">tendonae</span> and anchored to the muscle wall of the ventricle (lower heart chamber). The valve itself is made up of cusps or flaps that fit together tightly to create a seal. On the left side of the heart you find the <span class="blsp-spelling-error" id="SPELLING_ERROR_6">mitral</span> valve between the two chambers (left atrium and left ventricle). The <span class="blsp-spelling-error" id="SPELLING_ERROR_7">mitral</span> valve also has two other names- the bicuspid valve, because it has 2 cusps or flaps, and the left a-v valve, because it separates the left atrium (a) from the ventricle (v). There is an <span class="blsp-spelling-corrected" id="SPELLING_ERROR_8">analogous</span> valve on the right side of the heart called the <span class="blsp-spelling-error" id="SPELLING_ERROR_9">tricuspid</span> or right a-v valve that functions in the same way and can also undergo prolapse for all the same reasons.</div>
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<img alt="" border="0" id="BLOGGER_PHOTO_ID_5631810634669065554" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjZ_LFmg_O5cnxXI7kDU4n98QB6Y-jbykQ-xLvPznGc_UeXZ1XcehPMLuhbmE7iSaTZK2ggqQ57jhR6mFD-jiePJNuDu35OJcf62fU4IHZT5DM_DpJY1m9GlwjibfCdB91cfXPxpu2KAY47/s400/mitral_v.jpg" style="cursor: hand; display: block; height: 272px; margin: 0px auto 10px; text-align: center; width: 306px;" /></div>
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<span style="color: #66cccc; font-size: 180%;">What does it mean to have valve prolapse?</span></div>
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When a person is born with valve prolapse, the layers of tissue that form the flaps or cusps of the valve are thicker than normal. The valve cusps are made up of three layers of tissue. One of those layers is connective tissue. When prolapse is present, the connective tissue builds up into a thicker layer than normal. This causes the cusp to fit improperly with the others and makes a leaky seal.</div>
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<img alt="" border="0" id="BLOGGER_PHOTO_ID_5631810992374111650" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgd1lgQNpUFatPAme6xdXYHoly1MUt0WlTDnuPgI524X-1K8sFD0nrx0f19W9gxMAuNkSMuM1mqdhapyjI0cxmRJU7Yn8_fMW3A6C1jeParsJxOfTvQB-RnGGrSEWWoRVUA08kmMHLxoJeR/s400/mitral+valve+closing.jpg" style="cursor: hand; display: block; height: 312px; margin: 0px auto 10px; text-align: center; width: 400px;" /></div>
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Most people with <span class="blsp-spelling-error" id="SPELLING_ERROR_10">mitral</span> valve prolapse show no signs of heart distress. If the seal does not allow too much blood to flow backwards, the overall function of the heart is maintained. Doctors will hear a murmur or click when they listen to the person's heart. A murmur is a swishing sound created when the blood squeezes through that opening. The smaller the opening, the louder the murmur will actually be. </div>
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<img alt="" border="0" id="BLOGGER_PHOTO_ID_5631812349913149634" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjMzb6OjkS0zNbOpYOzSUUKikQcdgsZ7PKBdWrTFWdZZ6c0Y2Dqh7zVXRLcIJGV_wuuwVIkxXK0LZXQ9QWz8pW1WT7u7mu5uxB0UAvmDj3dAPt-V6EHNryEV1Xck9baxulY2sfFwQWvarBT/s400/valve+function.jpg" style="cursor: hand; display: block; height: 400px; margin: 0px auto 10px; text-align: center; width: 385px;" />Some people have a larger opening in the valve or it can progressively become worse over time. If the opening becomes large and a significant amount of blood flows backwards you can have issues with clotting and blood pressure. When blood flows backwards into the atrium it is called <span class="blsp-spelling-error" id="SPELLING_ERROR_11">mitral</span> regurgitation. If this occurs, a cardiologist will most likely prescribe medication called beta blockers or blood thinners to prevent clotting.</div>
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As the opening becomes larger, a lot of pulling can take place on the cords that anchor the valve. They can become stretched out, causing the opening to become larger still and eventually can rupture or break. You can imagine what would happen to a parachute if you cut one of the cords that attaches it. The parachute would become loose and wouldn't catch the air properly. The same thing can happen to the valve. If this happens, the heart simply wouldn't function properly and a valve repair or replacement surgery may be necessary.</div>
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<img alt="" border="0" id="BLOGGER_PHOTO_ID_5631810794655406914" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEimish_O-L1mijo443nJUqvi3Cqt3ElswXzGM6vLY5Uaz6v0ePNqYlGFHeurPvSboIweshw3LtC9_U-Milz4LPhAQ8Ctb9yNI8EviwcNLaI_4hv5V-Vw3Iadx-8gzZbAqpYBqql1pjLvdhH/s400/mitral+valve+prolapse.jpg" style="cursor: hand; display: block; height: 320px; margin: 0px auto 10px; text-align: center; width: 400px;" /></div>
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<span style="color: #66cccc; font-size: 180%;">If my child has valve prolapse, what can I do to prevent this from progressing?</span></div>
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Although the progressive nature of this condition can be scary, most people go their entire life without any issues. Whenever a child has a <span class="blsp-spelling-error" id="SPELLING_ERROR_12">CHD</span>, it's important to maintain optimal cardiovascular health by monitoring their diet, getting proper cardiovascular exercise and seeing a cardiologist regularly to monitor the defect. Diet, especially watching sodium intake and avoiding caffeine and energy drinks, will keep their heart functioning properly.<br />
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Sources:<br />
<a href="http://www.ncbi.nlm.nih.gov/pubmed/21545515" target="_blank">Mitral valve diseases in Williams syndrome by Collins </a><br />
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<a href="https://www.nlm.nih.gov/medlineplus/ency/article/000180.htm" target="_blank">Mitral valve prolapse by Medline</a></div>
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Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com0tag:blogger.com,1999:blog-7345537858373518781.post-41687446395343803232011-07-19T15:26:00.000-07:002011-07-19T15:27:45.981-07:00New genetics pageCheck out the genetics page, which is now complete!Sarahhttp://www.blogger.com/profile/09705279977152428555noreply@blogger.com0