Williams syndrome behavior profile- ADHD

Many 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.

ADHD is tricky to treat...

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.

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. 

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.

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.

Diagnosing ADHD

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. 

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 National Resource center of ADIHD):

"Criteria for the three primary subtypes are: ADHD - Predominantly Inattentive Type

• Fails to give close attention to details or makes careless mistakes.
• Has difficulty sustaining attention.
• Does not appear to listen.
• Struggles to follow through on instructions.
• Has difficulty with organization.
• Avoids or dislikes tasks requiring sustained mental effort.
• Loses things.
• Is easily distracted.
• Is forgetful in daily activities.

ADHD - Predominantly Hyperactive/Impulsive Type

• Fidgets with hands or feet or squirms in chair.
• Has difficulty remaining seated.
• Runs about or climbs excessively.
• Difficulty engaging in activities quietly.
• Acts as if driven by a motor.
• Talks excessively.
• Blurts out answers before questions have been completed.
• Difficulty waiting or taking turns.
• Interrupts or intrudes upon others.

ADHD - Combined Type

• Individual meets both sets of inattention and hyperactive/impulsive criteria."

The science behind ADHD

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)

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.

Brown et al. 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.

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.

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. 

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.

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.

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
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.


Treating ADHD in someone with WS is extra tricky...

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. 

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.

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. 

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.

ADHD treatment for those with WS

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%).

To conclude:

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.

Sources:

• http://www.ncbi.nlm.nih.gov/pubmed/20700845 Abstract of a study that shows the prevalence of ADHD in individuals with WS.
• http://jdc.jefferson.edu/cgi/viewcontent.cgi?article=1003&context=jeffjpsychiatry small psychiatric study that attempted to show that ADHD and anxiety in WS is due to the genetic deletion rather than family inheritance.
• 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 "Understanding williams syndrome behavioral patterns and interventions"
• http://onlinelibrary.wiley.com/doi/10.1002/ajmg.b.31247/full Study about the use of MPH treatments in individuals with WS.
• http://www.ncbi.nlm.nih.gov/pubmed/9203066 Second study about MPH and WS
• http://www.drthomasebrown.com/pdfs/comChapter.pdf A book that summarizes studies on ADHD and explains what trends in research say about the condition.