People with Prader-Willi Syndrome

Among the persons supported by Community Systems, Inc. (currently by CSI/Northern Virginia and CSI/Massachusetts) are persons with Prader Willi Syndrome (PWS).

PWS is a rare genetic disorder (believed to result from a spontaneous genetic birth defect, not an inherited condition) that is experienced by approximately 1 in 14,000 persons living in the United States. A person with PWS has a characteristic physical appearance which includes short stature, poor muscle tone, and a tendency to obesity. He or she presents with learning disabilities or mild intellectual disabilities and oftentimes aberrant behaviors (including tantrums, stealing, lying, and obsessive-compulsive behaviors).

Because persons with PWS experience constant hunger which cannot be satiated, they will resort to extreme attempts to obtain food, which can include seeking garbage, stealing food, and other extreme measures. Without restrictions on their lifestyle, persons with PWS will acquire high blood pressure, respiratory distress, diabetes, and/or other weight associated disorders. The latter is compounded by the fact that persons with PWS may only eat 800 to 1,000 calories/day without gaining weight. Without external controls, weight gain can occur very rapidly and become life threatening.

As a result of the inherent risks of living with PWS, persons will require life-long special supports. Without such supports, persons with PWS may die in their young adult years. Persons supported by CSI may live singly or with others having PWS, because restrictions on food access are warranted which would be viewed as inappropriate for other persons. Positive behavioral supports are an important program component. CSI staff plan carefully for people with PWS to live fully with staff guidance and discrete behavioral controls. CSI is noted for the normalcy it has helped young people with PWS achieve in their lifestyles.

Families and persons with PWS have the benefit of a very active national association, the Prader-Willi Syndrome Association, which provides newsletters, family support groups, and a national conference, among other services. (1-800-926-4797).