Running For Research - PWSPeople with PWS may have an increased risk of developing dangerous blood clots. Deep vein thrombosis (DVT) happens when blood clots form in the deep veins of the legs. Those blood clots can ultimately break loose, travel through the bloodstream, and become lodged in the lungs, causing pulmonary embolism.
DVT’s are the 4th leading cause of death in persons with PWS. According to the most recently gathered information, the top risk factors for developing a DVT are as follows: obesity, edema( swelling), skin picking and vasculitis.
Vasculitis and skin picking are often associated and together may be the most frequently occurring predictive behaviors associated with DVTs. Vasculitis is an inflammation of the blood vessels. It happens when the body’s immune system attacks the blood vessel by mistake. It can happen because of an infection, a medicine, or another disease. The cause is often unknown. Vasculitis can affect arteries, veins and capillaries. Skin picking can cause infection. Infection is the assumed link between the 2 conditions in PWS.
Clementine has a history of skin picking. It is under control right now but has been a significant problem in the past. I believe the behavior is driven by stress and anxiety. She is definitely in the high risk group for this deadly complication because of her skin picking history, which could lead to vasculitis , but also because she has diabetes. Diabetes is also listed as a leading contributor to DVTs in persons with PWS. People with diabetes are less likely to heal quickly from skin wounds and are more likely to suffer from infections for this reason.
For Clem, having PWS coupled with T1D and skin picking puts her in the highest risk category. The only real preventative action we can take is to manage her blood sugars as effectively as possible, keep her slim and make sure any wounds are treated to prevent infection.
A portion of the funds raised from last year’s Running for Research Prader-Willi fundraising campaign is earmarked for an investigational medication being trialed in the typical population to treat skin picking and hair pulling. We are very interested in how it will work in our population. Dr. Miller has been in close contact with the drug manufacturer. She is waiting for the drug to be granted phase 2 approval in the typical population. Once this occurs, it will potentially be available for trial in persons with PWS.
