An overwhelming hunger that never eases Syndrome: Prader-Willi, a chemical imbalance that makes the appetite go wild, gets a closer look after 680-pound teen's death.

December 23, 1997|By Elizabeth Fernandez | Elizabeth Fernandez,SAN FRANCISCO EXAMINER

SAN FRANCISCO -- They live to eat, driven by a desperate compulsion they can never control.

Locks don't stop them -- they'll pry the hinges off refrigerator doors or jimmy padlocks to reach inside kitchen cupboards.

They'll steal to buy food. They'll forage for chicken fat in trash cans. They'll gorge at restaurants, then skip out on the bill, not understanding the consequences.

If left alone, they'd quite literally eat themselves to death.

This is Prader-Willi syndrome, a genetic accident in which the body's internal register never measures full, an ailment that revolves around a particularly cruel combination: an overwhelming desire to eat tied to a metabolism that processes only half the calories of a typical person.

The little known, incurable chemical imbalance will take center stage this week in the defense of Marlene Corrigan of El Cerrito, Calif., charged with child endangerment over the death last year of her daughter, Christina, 13, who weighed 680 pounds.

Corrigan, a federal employee who works in San Francisco, faces a maximum of six years in prison on charges of felony neglect.

Prosecutors, citing bedsores, fungal infections and insect bites on Christina's limbs, say Corrigan should have sought medical treatment for her daughter.

A pathologist for Contra Costa County concluded that Christina had died from heart failure related to morbid obesity.

But San Francisco defense attorney Michael Cardoza, contending that Corrigan coped as best she could under extremely trying circumstances, plans to introduce evidence that Christina may have suffered from the uncontrollable eating syndrome.

Cardoza says Christina was "Prader-Willi-like, if not Prader-Willi."

The disorder, he says, would have made her impervious to the sores discovered at death.

The prosecutor, Deputy District Attorney Brian Haynes, declined to comment on the possibility that the girl suffered Prader-Willi disorder.

The county's pathologist performed only a visual examination on Christina -- not an autopsy, which could have established with a blood test whether the girl was affected by the disorder.

"We'll never know if she had Prader-Willi, [but] she had most of the major symptoms -- appetite, early weight gain, behavior problems," said Janalee Heinemann, executive director of the national Prader-Willi Syndrome Foundation in Florida.

"Our kids are typically mild and sweet, but when they have a temper tantrum, they can go on for hours," Heinemann said. "Christina was very much like that; she would get temper tantrums and be very stubborn. She also had some of the minor symptoms, [such as] food foraging and skin picking. They pick at sores. It doesn't hurt them. They can keep a sore open for months. They have a high pain threshold."

The illness affects an estimated 18,000 people in the United States. It typically causes lifelong behavior problems and mental retardation ranging from slight to severe. It also causes sterility, diabetes, and often, premature death.

For parents of Prader-Willies, as they are called, life becomes an exhausting cycle of kitchen lockup and calorie counting, of driving youngsters to physical and occupational therapy, of fighting insurance companies.

"When you have a child with unique disabilities, you either become the passive parent who gives up and tries to cope with a nightmare situation, which, I think, is the kind of parent [Corrigan] was," said Heinemann, whose stepson, Matt, 24, has the syndrome.

"That's not a bad mother, that's a tragic mother. Or you become an aggressive parent who learns to scale brick walls."

David and Wendy Young, a San Francisco couple, are learning to scale those walls.

Their 11-month-old son, Oliver, has the syndrome, diagnosed when he was 3 weeks old.

"It is devastating," said David Young, a surgeon. "Any time you have a child that is less than perfect, it is a severe blow to your expectations. There is no one or no thing to blame.

"He'll always have to live in a group home because he won't be able to control his eating. He'll have below-normal intelligence; he won't be able to have children. But he may have a satisfying life, we hope."

Young vaguely remembers hearing of the illness when he was in medical school. During the past year, he has become expert. Wendy Young, a vice president of the Bank of America, has cut her schedule to four days a week to care for her son.

Ironically, Oliver, like other babies with the syndrome, had difficulty suckling and little appetite.

"We had to cajole him to eat, but now if we stick a bottle in his mouth, he will suck it down," David Young said.

"For the first year or two they don't eat, and then they don't want to stop eating."

Prader-Willies can eat a full meal but still think they are starving. They can gorge but not vomit; their muscle system is too weak. Their growth is stunted at puberty; they typically range in height from 4-foot-10 to 5-foot-5.

The primary treatment is weight regulation, with caloric intake about half normal.

"They have to be on a diet for life," said Prader-Willie specialist Bryan Hainline, a biochemical geneticist at Riley Hospital for Children in Indianapolis.

Pub Date: 12/23/97

Baltimore Sun Articles
|
|
|
Please note the green-lined linked article text has been applied commercially without any involvement from our newsroom editors, reporters or any other editorial staff.