Mommy I’m Hungry (Again): A Look At Prader-Willi Syndrome

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By Mia Bolaris-Forget

Parents have always been typically been concerned with the health and nutrition of their children. And, with all the chemicals contained in “mainstream” foods these days, it would seem that the best option (though they need careful cleansing as well) are organic alternatives. But today’s parents are not only conscientious about what they put into their child’s (children’s) body (bodies), but how much. Obviously you don’t want your child to be malnutritioned, but with obesity becoming and increasingly prevalent and growing problem, portion control is becoming not only important for adults but also for children.

Monitoring food intake is especially important for children who part of a small group afflicted with Prader-Willi Syndrome, a condition that affects, among other things, how much they eat. In fact, this “rare” (yet serious) syndrome often leads to an insatiable appetite that can literally result in gross obesity or the child eating himself (or herself) to death.

Prader-Willi Defined: Prader-Willi syndrome (PWS) is a condition characterized by short stature, below average mental and cognitive abilities, incomplete sexual development, behavioral problems, low muscle tone and a constant urge to eat.

Genetic Predisposition: While this syndrome IS a genetic abnormality linked to an aberration of chromosome 15, it is typically NOT considered hereditary, but rather a spontaneous genetic birth defect that occurs during the time of conception, affecting both sexes and all races.

PWS Prevalence: With this condition affecting an estimated 1 in 14,000 in the United States alone, it is considered one of the 10 most common birth defects identified in genetics clinics and is the most common genetic cause of obesity identified (to date).

Age Of Onset: While this IS considered a birth defect often leading to gross obesity, the eating disorder is not necessarily apparent from birth. In fact, many newborns with this syndrome tend to be “floppy”, and are unable to take to the bottle or nipple well, and are frequently deprived of sufficient nutrients and many often require being fed through a tube for the first few months until muscle tone and control improves. As children grow, approaching preschool age, so does their appetite and their weight gain if calories and food intake are not carefully controlled and monitored.

Why They Can’t Control Their Appetite: Those with PWS have a malfunction in their hypothalamus, a part of the brain that both stimulates and controls hunger. Since the part of their brain that tells them when they are full doesn’t work properly, they never feel full, and thus are always hungry. In addition, these children/individuals generally need significantly fewer calories than normal to maintain an appropriate weight, and many are at risk for serious illness or fatal consequences due to obesity and improper control of their daily diet.

Controlling The Hunger: Experts assert that other than conscience and continual monitoring of their calories and food intake, there is little that can be done to suppress their appetite. In fact, most with PWS need to be on an extremely low-calorie diet all their lives and must have their environment adjusted so they have limited access to food, even if that includes locking cabinets, refrigerators or taking alternate routes to work that don’t allow for stops at delis, groceries, or fast food chains.

Differentiating PWS From Basic Overeating: While it may seem that all those who are overweight may suffer from some form or PWS, experts assert that this is by far NOT true. In fact, they note that there is more to PWS than “simply” overeating. People with PWS also have a characteristic appearance and speech quality, significant learning disabilities or mental disability plus a number of other problems. Many of these features need to be present for a clinical diagnosis and specific tests confirming it are usually available.

Behavioral Problems Associated With The Syndrome: Besides extreme measures taken to sneak or get food, individuals with PWS are noted for temper outbursts, stubbornness, rigidity, argumentativeness, and repetitive thoughts and actions. The best way to deal with a person with PWS is to structure his or her environment using behavioral management techniques and sometimes drug therapy.

Living A Normal Life: According to experts, those with PWS can accomplish just as much and succeed just as well as those without the affliction. However, they note, they need lots of discipline and help. Experts note that youngsters with PWS generally need special education and related services including speech and occupational therapy. In the “real world”, community, work, and residential setting, adolescents and adults with PWS can seek special assistance to learn how to carry out responsibilities and get along with others. And, they add, most need round the clock food supervision and that as adults they tend to fare better in group homes specially designed for people with PWS, where food access is restricted and limited and where people with the condition can be around peers with similar challenges without interfering with those who don’t need such attention. And while in the past, the condition was considered fatal, today greater understanding and prevention of obesity can help many with PWS live a normal life and lifespan.

The Benefits Of Early Diagnosis: The earlier you diagnosis Prader-Willi Syndrome, the sooner you can begin to help your child take control of his or her life and problem, and to avert obesity. Because young children with PWS generally have developmental delays in a variety of area, identification of the condition may result in necessary early intervention and help identify areas of need or risk. Furthermore, early diagnosis allows for families to do amble research and get the necessary information, help, and support from professionals and other families who are dealing with the syndrome.

What To Look For: As with anything, identifying the problem is the first step is “solving” or “controlling” it. The best way to determine whether of not you child has PWS (if you are concerned about some possible symptoms) is by contacting your health care provider for and evaluation and formal diagnostic. Note that many doctors may suggest a medical geneticist specializing in diagnostic tests for such genetic conditions and after a thorough evaluation, examination and family history, will arrive at a conclusion and diagnosis.

For More Information: If you are interested in acquiring more detailed information on PWS you can contact the Prader-Willi Syndrome Association at 1-800-926-4797, which in addition to answering your questions and concerns, provides a newsletter and other publications (on the syndrome), an annual national conference, and chapters throughout the country offering family support and advocacy.