Prader Willi Syndrome

by on August 26th, 2010
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Prader Willi is a fairly rare and not well publicized but disturbing genetic illness, one where people can literally eat themselves to death. “PWS, the most common form of inherited obesity associated with mental retardation, is characterized by low intelligence quotient (IQ), behavioral disorders, poor muscle tone, and an insatiable appetite. The disease usually occurs when a particular segment of the 15th chromosome is missing” (1- “Better Genetic testing”, para. 4). According to this article, a simple blood test can provide the answers to whether children are merely obese and/or overeaters or whether they are afflicted with PWS. “Without supervision, children with PWS can balloon to two or three times their normal weight, putting them at risk for respiratory and heart problems. Along with a voracious appetite, they may have varying degrees of mental retardation and learning disabilities. They tend to be short, with small hands and feet and large almond-shaped eyes’ (1- “Better genetic testing, para. 8).

What is extremely difficult for parents to deal with is the fact that while parents may not be affected more than one of their children could “inherit” this genetic mutation. Recent statistics provide some insight: “It is estimated that one in 12,000 to 15,000 people has PWS. Although considered 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” (2- “Questions and Answers”, para. 3).

There seems to be no cure for PWS, but there are efforts that can be made, even by family and other non-medical people- friends and neighbors, for example, to help keep the children with PWS from overeating. “Obesity is the greatest threat to health. Limiting calories will control the obesity but the family, neighbors, and school must work together closely, because the child will try to get food wherever possible. Exercise can increase lean body mass in children with Prader-Willi syndrome” (3-Kanesheiro and Zieve, para.9).

There are experiments underway to see if various types of growth hormone treatments might help, but not really cure, this syndrome. In fact, growth hormonal therapy might cause other problems, such as in the lungs or heart disease. There are many health professionals who advise parents that their children with this illness ought to seek some psychiatric help for behavioral problems. There are really no complete statistics about life-span for children with this syndrome. But obesity and an eagerness to overeat no matter what can cause diabetes and heart disease that could shorten one’s life. Rare as this syndrome may be, for the parents of children with Prader Willi syndrome, raising those children is a daunting and difficult life-long task.

References:

1.”Better genetic testing for Rare Syndrome”

Nutrition Health Review: The Consumer’s Medical

Journal, Issue 63, 1992 accessed Nov, 17, 2011 from

Alt Health Watch

2.Questions and Answers about PWS” Prader Willi Syndrome

Association, accessed Nov. 17, 2001 on

www.pwsausa.org/faq.htm

3.Kaneshiro, N. K., and Zieve, D.: “Prader Willi Syndrome”,

Revised May 11, 2011, A.D.A.M Medical Encyclopedia

accessed Nov, 17,, 2011 on www.ncbi.nlm.nih.gov


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