[James]

For those not familiar with PWS.


http://www.thearc.org/faqs/pwsynd.html

What is Prader-Willi syndrome (PWS)?

PWS is a complex genetic disorder that includes short stature, mental retardation or learning disabilities, incomplete sexual development, characteristic behavior problems, low muscle tone, and an involuntary urge to eat constantly, which, coupled with a reduced need for calories, leads to obesity.

Does the eating problem associated with PWS begin at birth?

No, newborns with PWS are typically described as "floppy" and are unable to suck well enough to get sufficient nutrients -- due to the low muscle tone (hypotonia). Often they must be fed through a tube for several months after birth, until muscle control improves. Sometime in the following years, usually by preschool age, children with PWS develop an increased interest in food and quickly gain excessive weight if calories are not restricted.

Why do people who have PWS eat so much?

People with PWS have a flaw in the part of their brain (the hypothalamus) that determines hunger and satiety (fullness). These people never feel full enough, so they have a continuous urge to eat. To compound this problem, people with PWS need considerably fewer calories than normal to maintain an appropriate weight. The obesity that results is the major cause of illness and death in this disorder. As in the general population, obesity in PWS can cause high blood pressure, respiratory difficulties, diabetes and other problems.

Can anything be done to control the eating?

Unfortunately, no appetite suppressant has worked consistently for people with PWS. Most must be on an extremely low-calorie diet all their lives and must have their environment designed so that they have very limited access to food. For example, many families have to lock the kitchen or the cabinets and refrigerator.

it possible for people with PWS to lead normal lives?

People with PWS can accomplish many of the things their "normal" peers do -- attend school, enjoy community activities, get jobs, and even move away from home. However, they need a lot of help. School children with PWS are likely to need special education and related services, such as speech and occupational therapy. In community, work and residential settings, adolescents and adults often need special assistance to learn and carry out responsibilities and to get along with others. In all settings, people with PWS need around-the-clock food supervision. As adults, most affected individuals do best in a special group home for people with PWS, where food access can be restricted without interfering with those who do not need such restriction. Although in the past many died in adolescence or young adulthood, it is thought that prevention of obesity will allow a person with PWS to live a normal lifespan.

Does early diagnosis help?

Early diagnosis of Prader-Willi syndrome gives parents an opportunity to manage their child's diet and avoid obesity and its related problems from the start. Since infants and young children with PWS typically have developmental delays in all areas, diagnosis may facilitate a family's access to critical early intervention services and help identify areas of need or risk. Diagnosis also makes it possible for families to get information and support from professionals and other families who are dealing with the syndrome.