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Prader-Willi Syndrome Weight & Behavior Management
Managing Prader-Willi Syndrome Two of the most outstanding characteristics of Prader-Willi Syndrome (PWS) are insatiable appetite and behavior problems. Prader-Willi Syndrome is a birth defect also characterized by hypotonia, incomplete sexual development, some degree of mental retardation in most cases, short stature (adult), small hands and feet, and developmental delays. The craving for food, which may become compulsive rather than true hunger, is complicated by the fact that people with PWS have poor calorie utilization, gaining weight on approximately one-half the calories required by a normal person. Their lack of the ability to reason creates further difficulty. There are no easy answers to any of the problems surrounding the management of this unique condition. The following "do and don’t" suggestions have come from parents, professionals, and observations of persons with PWS. In addition, use good humor, kindness, affection, determination and respect, seasoned with flexibility and good judgment, and there will be a good amount of success. Behavior management should be intervention/prevention oriented. For best results, preschedule, preplan, avoid arguments and anticipate. Weight management should be individualized and include daily or weekly weigh-ins, complete control of food intake, environmental barriers to food access and diets acknowledging that fewer calories are required.
Prader-Willi: A Multistage Syndrome The initial stage of Prader-Willi Syndrome is characterized by low birth weight and subsequent failure to thrive. The infant is very weak, usually cannot nurse or suck, and must be fed with special nipples or tubes. NOT gaining weight is a problem. Depending on the degree of hypotonia, this stage can continue for the first couple of years. A program of good nutrition, proper food intake, and exercise started as soon as possible - not after weight starts to accumulate - will be extremely beneficial. Behavior problems may be few or nonexistent in the young child, although individuals vary. A little stubbornness usually appears first, often progressing to temper tantrums. The next stage reflects almost thriving too well. The food compulsion generally surfaces with improvement of hypotonia, usually between the ages of two and four, but possibly earlier or later. Behavior may include greater stubbornness, temper tantrums increasing in number, length and intensity, and food seeking, stealing and hoarding. When weight gain starts, complete control of caloric intake, aided by locks on cabinets and refrigerators, is essential. Without such control, 95 percent develop weight problems. A well-balanced diet limiting calories is a lifelong necessity. The Prader-Willi Syndrome Association (USA) was formed in 1975 in order to provide a vehicle of communication for parents, professionals and other interested citizens. It is an organization dedicated to the sharing of experiences in how to cope with the syndrome. Chapters of PWSA(USA) are located in most states and are available for support, education and advocacy. An annual national conference, a bi-monthly newsletter entitled The Gathered View, which incorporates contributions from members as well as professional advice and a wealth of other written materials and publications on Prader-Willi Syndrome, are all part of PWSA's effort to carry out its mission. Supported solely by membership fees and donations, Prader-Willi Syndrome Association (USA) has made a difference in the lives of many affected by this unique syndrome. YOU, TOO, CAN HELP. Donations and membership applications may be sent to the address below. Edited: 02/21/2008 |
