by Kathleen Keating

Prader-Willi syndrome (PWS) is a complex genetic disorder that typically causes low muscle tone, short stature, incomplete sexual development, cognitive disabilities, emotional sensitivity, and a chronic feeling of hunger that can lead to excessive eating and life-threatening obesity.

Most cases of PWS are attributed to a spontaneous genetic error that occurs at or near the time of conception for unknown reasons. A PWS-like disorder can also be acquired after birth if the hypothalamus portion of the brain is damaged through injury or surgery.

A rare disorder, Prader-Willi syndrome is one of the most common conditions seen in genetics clinics and is the most common genetic cause of obesity that has been identified. PWS is found in people of both sexes and all races.

People with PWS have a flaw in the hypothalamus part of their brain, which normally registers feelings of hunger and satiety. While the problem is not yet fully understood, it is apparent that people with this flaw never feel full; they have a continuous urge to eat that they cannot learn to control. To compound this problem, people with PWS need less food because their bodies have less muscle and tend to burn fewer calories.

Overeating does not begin at birth. In fact, newborns with PWS often cannot get enough nourishment because low muscle tone impairs their sucking ability. Many require special feeding techniques or tube feeding for several months after birth, until muscle control improves. Sometime in the following years, usually before school age, children with PWS develop an intense interest in food and can quickly gain excess weight if calories are not restricted.

Unfortunately, no appetite suppressant has worked consistently for people with PWS. Most require 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.

In addition to their involuntary focus on food, people with PWS tend to have obsessive/compulsive behaviors that are not related to food, such as repetitive thoughts and verbalizations, collecting and hoarding of possessions, picking at skin irritations, and a strong need for routine and predictability. While psychotropic medications can help some individuals, careful structuring of the person's environment and consistent use of positive behavior management works well for everyone.

With help, people with PWS can expect to accomplish many of the things their "normal" peers do—complete school, achieve in their outside areas of interest, be successfully employed, even move away from their family home. They do, however, need support from their families and from school, work, and residential service providers to both achieve these goals and avoid obesity and the serious health consequences that accompany it. Even those with IQs in the normal range need lifelong diet supervision and protection from food availability.

Although in the past many people with PWS died in adolescence or young adulthood, prevention of obesity can enable those with the syndrome to live a normal lifespan. New medications, including psychotropic drugs and synthetic growth hormone, are already improving the quality of life for some people with PWS. Ongoing research offers the hope of new discoveries that will enable people affected by this unusual condition to live more independent lives.

For more information about PWS contact the Prader-Willi Syndrome Association (USA) at 1-800-926-4797 or email pwsausa@aol.com

Vegan diets adequately meet the nutritional needs of children according to the American Dietetic Association’s position paper on vegetarian diets.

Pediatric developmental tests in vegetarian children indicated mental age advanced over a year beyond chronological age, and mean IQ was well above average (with an average of 116 points), providing reassurance that brain development is normal.

In 1998, in the seventh edition of Dr. Spock’s Baby and Child Care, Dr. Spock recommends a vegan diet for children. A document was prepared by a panel of nutritionists to address three main areas: the advantages of vegetarian and vegan diets, the safety of vegan diets, and planning meals for children. (Click on Physicians Committee For Responsible Medicine above for full article and helpful information on nutrition for children.)