Maple Syrup Urine Disease hits family

By DEE MORRISON
GRAND RAPIDS - Imagine you had a rare disorder that wouldn't allow your body to process proteins and you can eat only specially pre-packaged foods. Imagine that if you did eat too much protein, you could suffer brain damage and die.
There are only 10 people in Michigan with this disease known as Maple Syrup Urine Disease, and two of them are in the same family.
Jenna and Jesse Kiel, are teenagers who've learned to deal with this. But their mom, Sandy, is working to change the law to help them.
MSUD doesn't allow their bodies to process protein. They miss the staples like peanut butter. "I'd eat it every day, if I could," said Jenna, 15.
To give you an idea what these kids are dealing with, a typical Thanksgiving dinner -- dressing, turkey, roll, cranberry sauce, green beans, potatoes and pumpkin pie, is about 40 grams of protein. That's 10 days worth of protein for Jenna.
Foods have to be chemically treated to remove the proteins. Those pre-packaged items are very expensive, and not covered by any insurance. Sandy Kiel is part of a parents' group trying to change the law.
Two bills have been proposed in the Michigan House of Representatives that would require insurance companies to cover some cost of what's called "Medical Food" for people with metabolic disorders.
"It's ironic to me," Sandy said, "that if my kids had any other disorder that required medicine, of course it would be covered."
But Jesse, 13, has found a way to add a little flavor. As everyone laughs, he called himself the King of Ketchup.
Even when everyone else is loading their plates, he just deals with it. "I've had this for so long that I'm used to it now."
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