May 17, 2005
Bridgette Tangen must monitor every aspect of her 12-year-old daughter’s diet. The child’s life depends on it.
The Gilbert resident installed locks on cabinets, the refrigerator and even garbage cans — anything to prevent her daughter from gorging to death.
Yet no matter how much Tangen’s daughter eats, she’s always starving.
Those born with the genetic condition Prader-Willi syndrome never feel full after eating due to a defect in the brain’s hypothalamus. Metabolism slows to a crawl.
Severe hunger fuels an obsession with food or money to get food. The biological response to extreme appetite has led some children to sneak out at night or eat unthinkable things.
If given unlimited access to food, they will eat until they pass out. Some have died.
Both children and adults with Prader-Willi require constant supervision and disciplined nutrition, as well as regular medical checks to address obesity-related ailments, including diabetes. Learning impairment ranges from mild to severe.
Ironically, parents often receive undue criticism for being the cause of their child’s weight issues despite every effort to manage them.
"I was blamed for Sean’s overeating," said Sheila McMahon, whose 10-year-old son made significant strides with growth hormones. The treatment has improved the characteristic poor muscle tone, and kick-started his metabolism.
Even after reaching a healthy weight through strict diet and exercise during the past five years, the true challenge still comes in getting Sean’s mind off of food.
"We try to keep him busy," said McMahon, an Ahwatukee Foothills resident. "Before we were diagnosed, he was very heavy.’’
Tangen’s daughter, whose name is being withheld to protect her privacy, is limited to a diet of just 1,100 Calories a day because of her nearly nonexistent metabolism. The child has a full-time aide at school who watches over her food intake.
With all the food restrictions, those with Prader-Willi become manipulative to get food. Likewise, sneaking food is common for the rest of the Tangen family.
"She’s very clever on getting food from you or getting people to give her food. The hand is quicker than the eye,’’ Tangen said. "If you have it, she’s going to want it.’’
Adults with Prader-Willi cannot live independently. Group homes that care for these individuals must check with the state’s Division of Developmental Disabilities and justify a medical reason to withhold food from Prader-Willi residents.
"This state is a bit more enlightened than other states, but it’s still a struggle sometimes," said John Cunningham, program director with the Tucson Residence Foundation, which serves about 10 Prader-Willi adults.
Cunningham said increased awareness has improved earlier diagnosing for the condition that affects an estimated 1 in 15,000 births.
"We didn’t find out until my son was 5," said McMahon, adding that she’s proud of Sean’s progress.
"You would not believe the relief just to know what’s going on with your child."