Rare syndrome has 3 victims, medical experts say
In cases of Prader-Willi syndrome, experts say there are
really three victims: the child who must suffer through a
lifetime of insatiable hunger, and the mother and father who
must constantly deny food to their child so he doesn't die.
by Heath Foster, April 30, 2003, Seattle
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In cases of Prader-Willi syndrome, experts say there are really
three victims: the child who must suffer through a lifetime of
insatiable hunger, and the mother and father who must constantly
deny food to their child so he doesn't die.
Prader-Willi is a genetic birth defect that damages the
functioning of the hypothalamus, the region of the brain that
controls feelings of hunger and fullness. People with the
syndrome never feel full and also have slower metabolisms than
A life of constant dieting and locked refrigerators is only part
of the syndrome. People born with it also typically experience
mental retardation or learning disabilities, incomplete sexual
development, disruptive behavioral problems and
obsessive-compulsive tendencies. And they tend to be short, have
higher percentages of body fat and low muscle tone.
An estimated one in 12,000 to 15,000 people in the United States
suffers from the syndrome. That translates into about 21,360
nationally and 450 in Washington state.
Dr. Vanja Holm, a neuro-developmental pediatrician at the
University of Washington, said that when the syndrome first
began to be diagnosed in the 1970s, it was common for people
with Prader-Willi to die young.
"It was sort of assumed they would die in their teens from
obesity," she said. "But as we have learned it can be
controlled, a lot of children have lived into adulthood, and
even to their 40s and 50s."
Today, children diagnosed with syndrome early are given
growth-hormone injections to increase their height and lower
their body fat. Their leaner bodies in turn can lead to speedier
metabolisms that allow them to eat more without gaining weight.
Depending on how controllable their behaviors are, some adults
with Prader-Willi are able to graduate into independent living
situations where caregivers help them with their meal
preparation. But experts say most people with the disease do
best living in group settings with others on similarly
Although there is no cure for the syndrome, researchers believe
it may hold a key to dealing with the nation's obesity epidemic.
The UW's Dr. David Cummings discovered in 2001 that people with
Prader-Willi have much greater levels than normal of a hormone
called ghrelin. When injected into humans, ghrelin intensely
stimulates hunger. Now, major pharmaceutical companies are
researching drugs that may be successful in treating obesity by
blocking the hormone.
TO LEARN MORE
For more information about Prader-Willi syndrome, go to
The only agency in the state with specialized community-based
programs for people with Prader-Willi syndrome is the non-profit
Camelot Society in Shoreline, which can be reached at
206-364-6680. Such programs did not exist when Chad Phillips was
first diagnosed with the disease in 1975.
P-I reporter Heath Foster can be reached at 206-448-8337 or