Running For Research - PWSTemper outbursts and disruptive behaviors are among the most challenging aspects of PWS, both for the individual with PWS and their family. These behaviors can severely limit the entire family’s ability to do many of the things most of us take for granted, like going to restaurants, or attending family events.
Many people with Prader-Willi syndrome experience temper outbursts. Several common triggers to temper outbursts have been identified and include a change to routine or expectation, receiving conflicting information and losing something. The frequency and duration of temper outbursts varies from person to person.
Research has shown similarities in the sequence of behaviours and emotions during temper outbursts. Often emotional behaviours, such as crying and emotional vocalisations occur early in an outburst sequence, and are followed by quickly rising anger and more overt behaviours such as aggression. Outbursts are frequently followed by expressions of remorse and the emotional behaviours that indicate distress can also occur towards the end of an outburst sequence.
A number of studies have been done that focused specifically on the temper outbursts that individuals can show following changes to their routines or their expectations (plans). It was found that such temper outbursts are linked to experiencing a difficulty with change, which is a broader characteristic of behaviour that is commonly shown by individuals with Prader-Willi syndrome. Many people with Prader-Willi syndrome find it very difficult to deal with changes to routines or plans and – even if they do not show temper outbursts following such changes – often get upset following such changes.
It has been found that people with Prader-Willi syndrome seem to have a specific deficit in the brain process called “task switching” or “attention switching”. Task switching is the brain process that allows us to switch from thinking about a set of stimuli or events in one way, to thinking about the same set of stimuli or events in a different way.
Not only do people with Prader-Willi syndrome find switching particularly difficult, but when they engage in switching, the brain functions in a different way compared to people who don’t have Prader-Willi syndrome. A study found that people with Prader-Willi syndrome who have greater difficulties with switching, also show more difficulty with change. This research showed that two important characteristics, which can come together to cause temper outbursts in people with Prader-Willi syndrome, are a cognitive deficit in switching and a difficulty with change.
It has been observed that some individuals with Prader-Willi syndrome showed very little or no upset following changes, unless those changes happened in routines that they had been accustomed to for a very long time. It is known that routines are important for children growing up with Prader-Willi syndrome, particularly around food, to reduce anxiety that might be experienced about not knowing what is going to happen when. If allowing a routine to be established for longer results in people showing greater difficulty when this routine is subsequently changed, this would suggest that we should try to develop ways to help people with Prader-Willi syndrome grow up with the routines that they need with flexibility built in.
FIND ( Further Inform Neurogenetic Disorders)
Clementine doesn’t yet show the temper outbursts that are typical to people with PWS. She is very mild mannered most of the time. But we do see that change is difficult for her and it seems to be getting harder as she gets older. When there is a change to her normal routine Clem will exhibit signs of anxiety and distress. For her, changes that involve the sequence of daily tasks or activities are much more upsetting than changes involving meal times or food choices. Even so, these changes don’t usually cause a show of temper, crying or any sort of aggression but rather nervousness and repetitive question asking.
I have found that if there will be a change to Clem’s schedule, it is best to wait until the change is getting ready to take place before informing her of its occurrence. If I give her advance notice, she will worry and perseverate, which I find to be worse than the real time reaction to the change.
Because we are a family of 6, including 4 children, our schedule has always been the subject of frequent last minute revision. I do believe that this frequent schedule changing is something Clem has grown somewhat accustomed to, and as a result, has less impact on her behavior and has resulted in fewer temper outbursts than might be typical of people with PWS. If I were just starting out on this PWS journey, I would definitely recommend early exposure to change as a possible way to mitigate future behavioral issues. I think of it as similar to exposure therapy that is often used to help people suffering from irrational fears or phobias.
While I don’t know how Clem’s temper or anxiety will change as she ages, I do know that we as a family will do everything we can to make sure she is able to adapt and thrive. The ability to process change is crucial for her ongoing happiness and her ability to manage stress. Additional research is needed in this and many other areas of PWS related behaviors.
