Have you ever heard of Prader-Willi Syndrome (PWS)? I recently met someone who is living with this disorder. Its characteristics include an insatiable appetite, developmentally issues, and behavioral problems. I had quickly looked online to find information, which seemed to focus on the genetics of the birth defect that causes suffers of PWS to eat excessively and face life-threatening obesity. Surprisingly, the husband of a friend, who is working on getting his counseling license, happens to work with a local Prader-Willi Syndrome Association, and actually did some writing about the syndrome. I believe he may have written or contributed to the booklet I stumbled upon that gives an overview of the disease and tips for managing behaviors.

This is a life-threatening illness that does not go away and requires the monitoring of food consumption for individuals suffering from PWS. I am talking about locking cabinets, weight management,  and not snacking in front of these individuals. There are more  helpful bits to read in the Do’s and Don’ts section of the booklet from the association, the following were some that stood out for me: not arguing or nagging, understanding that logic and reasoning do not work for people suffering PWS, providing structure, including the individual in discussions of planning, since they need to feel in control and will be more cooperative, giving praise and recognize a good performance. Do not ignore bad behaviors, and do not argue because there is no winning.

 Prader-Willi Syndrome You make a statement, allow the person to say their piece, warn that the discussion is over, and stick to keeping the discussion closed. Prader-Willi Syndrome Association (USA), 5700 Midnight Pass Rd., Suite #6 Sarasota, FL 34242 Phone: (800) 926-4797, Fax: (914) 312-0142, Email: pwsausa@aol.com

Prader-Willi syndrome is a congenital disease, caused by a gene missing on part of chromosome 15, affecting many parts of the body. Individuals suffering from this condition are obese, have reduced muscle tone and mental ability (typical IQ 70), and have sex glands that produce little or no hormones. The genetic changes occur randomly without a family history of the condition. There are also issues with social and motor skills. Other characteristics include, at birth low birth weight and weak muscles; physical conditions appear to change to one of “thriving too well” typically between the ages of two and five, sometimes later. The insatiable need to consume food lasts throughout the individual’s lifetime.

I found it interesting to read that “children with PWS have sweet and loving personalities”, because that was not the experience we were having in our social education group, which may be due to our staff, of non-clinical people, not understanding the extent or limitations of the abilities for a person suffering from PWS. Through this experience, I have been reminded that even with graduate level training, there is so much more I do not know, and there is always room to learn.  It is disconcerting when a parent, used to coping with a unique illness, expects everyone else to know exactly what to do for her child. This is frustrating especially when one has never heard of the disorder. It would be helped if the information was shared before a child entered into a social learning program, thus making sure it is an appropriate, safe, constructive, and healthy learning environment that will provide the best experience possible for all involved. If it is not the best fit, perhaps, thinking big, a similar social education program could be developed to suit the needs of other children also living with PWS.

Resources: Prader-Willi Syndrome Association www.nature.com