Wednesday, April 17, 2002
PUTTING FOOD UNDER LOCK AND KEY
Brought to our attention for the first time is the existence of Prader-Willi Syndrome, a horrible disease that renders its victims unable to control their appetites. Without the help of others to maintain strict diets -- assistance that requires round-the-clock supervision and locks on refrigerators and kitchen cabinets -- people with Prader-Willi Syndrome might literally eat themselves to death.
"Prader-Willi is a genetic disorder that causes low IQ and a physical quadruple whammy: overwhelming desire to eat, slow metabolism, low muscle mass, and little impulse to vomit. Few adults with Prader-Willi reach more than five feet tall. In the past, such people rarely made it out of their 20s, dying of obesity-related problems when their weights reached 400 to 500 pounds. Some ate till their stomachs ruptured," according to a report in today's Philadelphia Inquirer.
The condition, which is the result of a genetic defect, affects one in every 12,000 to 15,000 babies, most of them males. Only a third have IQs above 70, though even at this level or below, they often show a special aptitude for jigsaw puzzles and some are good readers.
"Prader-Willi parents . . . tell of children breaking into neighbors' homes to get to their refrigerators, or eating food from the school bus floor, or eating frozen meat. People with the syndrome are so notorious for eating late at night that their caregivers often say: 'Being a Prader-Willi parent means hearing a noise at 2 a.m. and hoping it's a burglar.' "
"We feel strongly that Prader-Willi is something the world needs to know about," says Bob Capitani, whose sister died of complications resulting from the condition. "It's a nasty, nasty disease."
That's putting it mildly. One can't help but share in the frustration of those dealing with chronic and mysterious conditions such as this. Apparently there is no effective medical treatment, leaving parents of Prader-Willi children to fend for themselves or to place their children in special homes where they often remain until adulthood -- and death. We hope that will change some day soon.
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JAMES MARTIN CAPOZZOLA
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James Martin (Jim) Capozzola launched The Rittenhouse Review in April 2002, TRR: The Lighter Side of Rittenhouse, HorowitzWatch, and Smarter Andrew Sullivan in July 2002, and Bulldogs for Kerry-Edwards in October 2004. He is also a contributing member of President Boxer.
He received the 2002 Koufax Award for Best Post> for "Al Gore and the Alpha Girls" (published November 25, 2002). Capozzola's record in the Koufax Awards includes two additional nominations for 2002 (Best Blog and Best Writing), three nominations for 2003 (Best Blog, Best Series, and Best Writing), and two finalist nominations in 2004 (Best Blog and Best Writing).
Capozzola’s experience beyond the blogosphere includes a lengthy career in financial journalism, securities analysis, and investment research, and in freelance writing, editing, ghost-writing, and writing instruction.
He earned his bachelor's degree in political science from the University at Albany and a master's in foreign affairs from the University of Virginia.
Capozzola lives in Philadelphia with his bulldog, Mildred.
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