Helping Children With Prader-Willi Syndrome

Prader-Willi Syndrome affects thousands of children in the United States, yet is relatively unknown to the general population.

This genetic condition, resulting from an abnormality on the 15th chromosome, affects both genders and all races equally, with estimates of 1 in 15,000 children currently living with this condition in the United States.

Newborns present with low muscle tone and difficulty feeding. Other physical characteristics include almond-shaped eyes, a narrow head shape near the temples, a thin upper lip, and a turned-down mouth. In addition, cognitive delays and behavioral problems, including tantrums and compulsive-type behaviors, are common.

Between the ages of one to six years, a child with Prader-Willi Syndrome will suddenly develop a voracious appetite, consuming large quantities of food and never appearing full. Rapid weight gain is common and often results in morbid obesity - a life-threatening condition, especially for children. The effects of obesity can not be underestimated, as many children with Prader-Willi Syndrome who consume large quantities of food are hospitalized each year from the side effects of binge eating.

This disorder is especially trying for families, educators, and caregivers who must monitor the child at all times to prevent over eating, as well as support their emotional and cognitive needs. Often times, refrigerators and food pantries are locked to minimize the child's access to food, as children with this disorder find ways to manipulate limits that are set and gain access to desired items. In some states, limiting access to nourishment could cross the line and be considered child abuse, so parents are often mindful of how they monitor their child's food intake, even if it is a life-saving technique.

Jason John of Jacksonville, Fla., is raising 15-year-old Austin, who was diagnosed with Prader-Willi Syndrome at age five. John says he is only comfortable leaving his son, who has exhibited violent outbursts recently, with close family members.

"The irony of Prader-Willi is that what these children love will kill them," John said. He says while the food issues are still prevalent in his son's day-to-day life, the behavioral issues have taken center-stage as of late. There was an incident at school where he attacked two students and a teaching assistant. The teaching assistant called the police. About a year ago, Austin accidentally burned the house down because he found a lighter and played with it. John put Austin's name on a waiting list for a well-known medical and behavioral rehabilitation program at Pittsburgh Children's Hospital.

"I've come to the realization that I can't help him any more than I am. They can help him and really make a difference."

John credits his family, his partner Jason, and the organization Prader-Willi Syndrome Association (PWSA) with supporting him as he faces the challenges of raising a child with this disorder.

In regards to limiting food intake, he says "I go to bed every night being the bad guy. I got over that a long time ago. I'm just doing what's best for my child."

Thousands of parents raising children with Prade-Willi Syndrome across the country can echo that sentiment.

For more information about Prader Willi Syndrome go to www.pwsusa.org.