Monday, May 12, 2014

PWS Awareness Day 12: Behavior (A Start)

Most parents of kids with Prader-Willi syndrome will tell you that the most challenging aspect of the disorder is the behavior, not the food. I would agree. We parents eventually get used to managing the food.  We start to eat more healthily ourselves.  We put in place a food security plan and stick to it.  We lock when the time comes.  We cry about that, and then we get over it.  We educate our schools, friends and families. We are clear and brave about asking others for what our kid requires to function.  And we avoid the situations that are unsafe or cause stress because of food uncertainty or access.  It sounds hard, and it definitely is.  But it's not the hardest part for many families.

People with PWS struggle with a wide assortment of behavior issues -- tenuous emotional control, rigid thinking, skin picking, perseveration, impulse control, and OCD-like behaviors to start.  Many behavior problems can be traced to or are exacerbated by stress....including a breach in food security, unexpected changes in the schedule or the plan, uncertainty or inconsistency of any kind.  My favorite graphic from two of my favorite PWS experts (Dr. Linda Gourash and Dr. Janice Forster) is one that shows a smiley face representing a typical person.  Lighting bolts of stress are striking a nice wide "environmental buffer" surrounding the smiley face.  A typical person has lots of inherent coping mechanisms to manage the stressors of every day life.  A person with PWS does not.  The graphic for a person with PWS is a sad face -- the lighting bolts are easily permeating their thin environmental buffer causing stress and leading to a whole array of behavioral issues. 

We've seen most PWS-related behaviors in Oscar: tantrums, skin-picking, anxiety, non-compliance, sneaking, hoarding, inflexibility, arguing.  Whenever behavior issues spike (as they have in the last couple of days) we consider any shifts in the environment.  Is Oscar getting enough sleep?  Is there a big event he's anticipating (an A's game, a grandparent visit, a trip to the zoo?)  Is he worried about something?  Is there uncertainty about the plan?

Oscar has demonstrated over and over that he needs a structured, predictable, consistent, low-stress, (and food-safe!) environment.  And so, the goal for him is not independence.  The goal is, in the words of Drs. Gourash and Forster, maximum function with support. 

He will, in short, never live on his own.   And that's due to both the food and the behavior.   But this does not mean that we have given up -- we work on that "maximum function" every day, helping Oscar develop coping mechanisms, helping him deal with uncertainty or unexpected changes.  Helping him navigate this complex and crazy world a little more comfortably.

Here he is, giving me a thumbs up after a five day camping trip in Yosemite with his school.  A trip that was successful in large part due to the safe environment and consistent approach his teachers were able to carry from school to mountains. 


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