Nov 28, 2007 7:47 pm US/Central
Syndrome Makes People Always Feel Hungry
DALLAS (CBS 11 / TXA 21) ―
Imagine having a starving feeling 24 hours a day. No matter how much you eat, you never feel full. That uncontrollable hunger is just one of several symptoms of a very rare genetic disorder that affects one in 12,000 children.
By looking at her, you couldn't tell six-year-old Hannah McGhee has a debilitating condition. She was born with Prader Willi Syndrome, a rare, genetic, non-inherited defect that causes immense hunger and other symptoms.
"Kids with Prader-Willi, at some point, they're never satiated," said Kathryn McGhee, Hannah's mother. "They're always hungry. They're always thinking about food."
Prader-Willi is a result of a defect on the 15th chromosome, which controls the hypothalamus in the brain. That's responsible for controlling hunger, behavior, even balance.
Because of the defect, sufferers are constantly hungry. We sat down with Hannah and her family for a meal.
"She doesn't chew properly, and she still puts food in when she's still got food in," Kathryn said. "That's across the board with kids with Prader-Willi."
After Hannah had finished every last crumb on her plate and the table, she turned around and asked for a snack. Her mother said no, but while no one is looking, Hannah snatched food off her sibling's plate.
A few minutes later, Hannah got upset when no one would give her a pretzel. But just one minute later, while her mother was distracted, Hannah grabbed another piece of food off the stove.
"She's like a heat-seeking missile on food," said John McGhee, Hannah's dad. "It's a constant battle."
Hannah's parents take drastic steps to ensure her safety in the home. Everything, including the refrigerator and pantry door, must be locked.
"You close one door, and then she goes and finds something else," Kathryn said. "I locked the refrigerator and then she started going after the toothpaste."
The insatiable hunger obviously fills every aspect of their lives. Even something as simple as chili powder could do harm to Hannah.
But there is hope in the form of research and awareness. The Foundation for Prader-Willi Research has raised nearly $1 million and funded more than 20 research projects. But at this point, the prognosis is still grim.
"Most Prader-Willi kids will never be self-sufficient because of the hunger," said John.
Many sufferers eventually move into group homes specifically designed to deal with the disorder. The McGhees say that's where Hannah will most likely go as she gets older. But they hope with more funding and research someone will find a cure.
"My greatest hope would be to find more treatment and cures for Prader Willi, and I guess mainly the hunger... because that's what we deal with the most," Kathryn said.
Constant hunger isn't the only symptom. Children with Prader Willi are also born with low muscle tone and most have developmental problems. Hannah is given a human growth hormone shot every night to help her body grow and become stronger.
Doctors say there is a prenatal test that can be done to detect Prader Willi, but it's not part of the usual regiment of tests done on an expectant mother.
(© MMX, CBS Broadcasting Inc. All Rights Reserved.)
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