Prader-Willi Syndrome

Prader-Willi Syndrome is a rare genetic disorder that some children are born with. This is a very rare disorder that affects the hypothalamus. It is caused by a spontaneous gene defect on chromosome 15 which can sometimes occur at the moment of conception. The hypothalamus is the part of the brain that signals feelings of hunger or fullness. Someone who has Prader-Willi Syndrome (PW) never feels full. They feel like they are hungry all the time. The result is that they overeat to the point of obesity.

There are two stages of Prader-Willi syndrome. The first stage involves the infant not being able to nurse properly because of poor muscle tone in the mouth and tongue. Special feeding techniques must be used to help them eat. As the infant grows older the muscle tone improves and then they can eat on their own. Prader-Willi Syndrome enters the second stage between the ages of 1 and 6. At this point the child is constantly hungry and wants to eat continually. This condition is complicated by poor muscle mass which means the child actually needs fewer calories but is demanding more and more calories due to excessive hunger. Consuming more food than is really needed leads to life-threatening obesity. The parent must control the amount of food the child is eating. Sometime the kitchen has to be closed off or food has to be stored in locked storage containers for the health and well being of the child. All the while, the child is crying and wanting more food because the hypothalamus is not signaling a sense of fullness.

There are some other major symptoms that are associated with PW. These include such things as behavioral problems, temper tantrums and stubbornness because the child wants to eat more food. The child usually has delayed motor skills and low muscle tone. Skills like talking and walking develop later than normal. Children with Prader-Willi Syndrome can also have learning disabilities and may have less than average intelligence. The child may repeat words over and over and have repetitive thoughts and pick at their skin. Children with this syndrome also hoard their possessions. They also have low sex hormone levels. Only some of these symptoms can be experienced or all, depending on the severity of the syndrome in each child. Children born with PW have some common physical characteristics such as almond-shaped eyes, downward turned mouth and a thin upper lip. Their elbows and joints can remain extended rather than fixed during times of agitation as well.

There are some treatments available for Prader-Willi syndrome. However, there is no cure for it. When the child is diagnosed with PW early the parents can learn better coping skills and can learn early about the challenges they will be facing as they raise the child. Human growth hormone is given to increase the height of the child and to reduce body fat. Human growth hormone can also increase muscle mass. A daily exercise routine should be adhered to help control body weight. A high protein low calorie diet should be given. Fiber is essential and other essential nutrients are needed to help maintain optimum health. To treat problems with delayed speech the doctor will prescribe speech therapy. Unfortunately there are now medications that can be prescribed to lessen the feeling of hunger or to control the appetite since they will not work for people with this syndrome. Psychological counseling is also given to help the child cope with his or her disorder. At puberty the child may be given sex hormones to trigger normal growth.

Last updated on Feb 23rd, 2011 and filed under Other Conditions & Diseases. Both comments and pings are currently closed.

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