Prader-Willi Syndrome

 
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Prader-Willi Syndrome by Mary Lynn Larson

Imagine that your desire to get food is so strong that you would do ANYTHING to get it, even crawl out of your bedroom window in the middle of the night to walk to the store several miles away.
Imagine that you are hungry all the time, and that you are on a diet, all the time, and that you can only eat about half as much as everybody else, not to lose weight, but just so you don’t gain weight. Imagine that if you do gain weight, you will have to go on an even stricter diet, getting about as many calories in one day as there are in just one cheeseburger and fries. Boy, would you like to have a cheeseburger and fries! But that’s not in your diet. You are told that your diet is very important, because if you gain weight you could get really sick and die, because your heart can’t handle the burden of obesity.
This is an excerpt from Imagine…What it would be like if you were born with Prader-Willi Syndrome by Teresa Kellerman as published in the Gathered View, the bimonthly newsletter of Prader-Willi Syndrome Association (USA).

“What is Prader-Willi syndrome? I have never heard of it.” That is the response I hear when I tell people about my children’s disability. Prader-Willi syndrome (PWS) is a unique, very complex developmental disability. Because of an abnormality on the 15th chromosome, persons with this disability face many life long challenges. The area of the brain called the hypothalamus doesn’t work properly. Three of the most outstanding characteristics include learning problems, (approximately 60% of individuals have some degree of cognitive limitation/mental retardation), behavior problems and an uncontrollable appetite. The message of fullness never reaches the brain – they are always hungry. Because of a hormone deficiency, they can gain weight on half the calories of another person their age.

As infants, most persons with PWS are termed ‘failure to thrive’ – meaning they require special feeding techniques or sometimes even tube feedings to ensure they get adequate nutrition. As they hit school age years, they start ‘thriving too well’ and begin to gain large amounts of weight on a diet ‘appropriate’ for most their own age. Some children start obsessing on, stealing and accessing food from anywhere in their environment. It is frequently at this age that families have to implement ‘environmental controls’ to limit their access to food. This might include locks on refrigerators, alarms on areas that allow access to food, constant supervision in all environments with access to food and keeping money out of their hands – as money buys food.

In addition to always feeling hungry, persons with PWS often have learning problems and behavioral issues. Some of the complex behavioral issues may include: rigid thought process, tenuous emotional control, perseverative and obsessive thinking, sequential processing deficit and difficulty understanding abstract concepts, risk of elopement, lying and stealing, difficulty with social interactions with peers, poor stamina and skin picking. The implementation of a behavioral management program, maintaining structure, routine and consistency in the individual’s schedule and staff education of the unique challenges specific to the person with PWS have been most effective in allowing persons with PWS to achieve the greatest level of independence with supports in their community.

For more information on this disability, contact the Prader Willi Syndrome Association of Wisconsin, Inc. Toll free at 1-866-797-2947 or via email at wisconsin@pwsaofwi.org. Another resource for information is the National association – Prader-Willi Syndrome Association (USA). Their contact information is www.pwsausa.org or call toll free at
1-800-926-4797

Mary Lynn Larson