48% of PWS Global Registry participants report anxiety. Anxiety may manifest itself as repetitive questioning, rigidity, obsessively engagement in an activity, talking louder or faster, moving in an agitated way, skin picking, chewing finger, picking lips, tics, etc. and can limit ones ability to participate in life.

People with PWS often have tenuous emotional control. They have problems managing their feelings and appropriately exhibiting emotions. Some of the common challenging behaviors that are often manifested in people with Prader-Willi syndrome include anxiety, frustration and anger. The most common precipitating factors seen in behavior outbursts are food and change. They are often irrational when it comes to anything having to do with food. Change can also cause extreme anxiety. Positive change as well as negative change may result negatively is these people.

Frustration often becomes a manifestation of anxiety. For example, when change occurs and the person feels unprepared for this change, he/she may exhibit frustration. Frustration can quickly escalate to anger. Threats feed the anger. Behavior can quickly go from words of anger – yelling and swearing –to action of words – pushing, property destruction and even personal attack. People with PWS often describe this anger as an uncontrollable “feeling that must come out.

Anxiety, Frustration and Anger Behavior Manifestations of Prader-Willi Syndrome By Barb Dorn, Crisis Intervention Counselor PWSA (USA)

Anxiety is our single biggest struggle. Clementine lives with a constant sense of anxiety. It is always simmering below the surface. Anxiety makes regular appearances throughout the day and in many different situations.

Clem first started showing signs of anxiety when she was about 4 years old. From what seemed like out of the blue, she developed a habit of picking little raw spots on her forearms. The picking grew in frequency until she was covered in wounds. It was very disturbing both for her and for me , and of course it presented with health concerns. Because Clem is a T1D, her ability to heal is impaired and we were very worried that she could develop a skin infection that could lead into something even more serious. Thank goodness for Dr. Miller! She was able to suggest a few different supplements and medications and with her assistance, we were able to lessen the frequency of this behavior. Eventually the skin picking stopped, but not before causing scarring on her arms that is visible even today.

PWS is like Whack- A-Mole. Just when you’ve managed to get one symptom or problem under control, another one pops up. As the skin picking faded, another manifestation of her anxiety arrived. This time it was hair pulling. It started slowly but ramped up in intensity very quickly. At it’s height, Clem had pulled all of the hair out of one side of her head. She looked like Cyndi Lauper, the famous singer from the 1980s. Clem was very upset by the loss of her hair but simply couldn’t control her actions. We addressed the issue by changing some of her medications and by having her work with a psychologist. The psychologist tried to help Clem recognize her anxiety, be able to vocalize it and find other ways of dealing with it. Over time, this behavior lessened and Clem’s hair regrew. It’s now quite long and pink and it is her favorite thing about herself!

At the present time, Clem is not consistently picking or pulling. But it does flare up occasionally and we know that it is not something that is gone for good. Just a few weeks ago she plucked out a large part of her eyelashes on both of her eyes. When asked why she did it, she said that she didn’t know but that the urge was overwhelming. Most likely there was a precipitating event but these events are often very hard to identify.

As I mentioned in a previous post, Clem’s anxiety is generalized and constant. Even when she is relaxed, in her favorite spot and doing something she enjoys, her anxiety is present. You can see it in her body posture and sometime on her facial expressions. It breaks my heart that she always feels a bit ” on edge”.

Clem’s anxiety is exacerbated by changes to her schedule, although less so with changes to meal times or menu choices. It’s usually changes to the order of tasks that really throws her. For that reason, we try to maintain a consistent schedule but as we all know, life doesn’t always cooperate. We are a large family so changes happen with some frequency. I don’t know if it’s this exposure or if it’s just her personality, but Clem never has the violent temper tantrums that are so common in PWS and that typically accompany change. While she doesn’t have outbursts, she does suffer from change anxiety. She just does it quietly.

Clem’s anxiety also manifests itself in repetitive question asking. This might be the single thing that is the most difficult for me. I’m not someone who has a large supply of patience so this drives me nuts! It also irritates everyone else in the family. Her siblings have to display a level of patience they often don’t have but they do their very best. My personal strategy is to answer a question calmly and clearly 2 times. Upon the third asking, I will tell Clem that I’ve given her the answer already and she may not ask me again. Usually that does the trick, albeit temporarily, until a new subject pops into her head.

Overall, anxiety is the biggest influencing factor of Clem’s behavior. It drives many of her actions and causes her to be distracted both at school (which hampers her ability to learn) and at home. Anxiety wears on her and saps her potential to be truly happy.

More research must be done in this area. We need to develop more treatment strategies, both medicinally and therapeutically. Any advances in this area would be very meaningful to the majority of the PWS community!

This picture is one I posted a while back. While I think it’s a really cute photo, if you look closely, you can see that Clem has no hair on the left side of her head. It looks like her hair is pulled back but in reality, it’s not there at all! This is anxiety at work.