St

St. Petersburg Times

August 5, 2001

Weight loss, life gain

People with a rare syndrome that causes insatiable hunger find help.

By CHRISTINA JEWETT

GAINESVILLE -- It's 1 p.m., and Ervin Currie has just 430 calories left

for the day.

Before dinner he can earn 100 more calories with a walk on the treadmill

and another 100 for a day of good behavior.

That means no tantrums, no sneaking food, and remembering to ask

permission before entering the dining room or kitchen.

The psychological barbed wire strung around the kitchen and dining room at

the group home in Gainesville replaces the bike chain wound around the

refrigerator doors at Currie's home in St. Petersburg.

Boundaries enforced by rigid rules render the flour and sugar in canisters

on the kitchen counter off limits. Without vigilant supervision in the

house, they would hold the allure of cocaine to an addict.

Currie, 22, and the five other residents of the group home have

Prader-Willi syndrome, an illness characterized by insatiable appetite,

low muscle mass and obsessive-compulsive behavior. Left unchecked, the

disease can lead from morbid obesity to death.

The disorder results from a random loss of a gene during conception,

causing an incomplete development of the brain. The error shows up in the

hypothalamus region, which tells the heart to beat and the body to cool.

It also registers hunger and fullness.

For people with Prader-Willi, the sense of satisfaction after a meal never

comes. The "fullness" sensor in the brain is blocked, or simply absent.

Faculty and staff at the Association for Retarded Citizens, or ARC, in

Gainesville see the same patterns year after year among the Prader-Willi

patients. So many of the residents are loving and sweet-natured. They tend

to come in with backpacks full of crossword puzzles.

"They come from different places in the country and completely different

families," said Dr. Kevin Jackson, a behavior analyst who works at the ARC

in Gainesville. "One difference in the 15th chromosome results in all of

these behavioral similarities."

The staff is successful in limiting the residents to 1,200 to 1,500

calories per day, but in doing so they beat back a predatory drive for

food.

Staff members hear new stories that echo the last: Patients cajole

delivery drivers into giving them pizza and persuade neighbors they are

starving. There was the kid who drank a bottle of cooking oil and the ones

caught eating the cat's food. Even the story about the parakeet that came

up missing, its feet left in the bottom of the cage.

From cute to depressing

Currie was typical of children with Prader-Willi syndrome. He was slow to

develop muscle mass and coordination. He was an exceptionally small infant

and a petite child.

At first it was cute, the way that skinny little boy could put away a

burger. By the time he reached 4 feet 8, the average height for a

10-year-old, he stopped growing taller but continued to gain weight.

"You could set down a soda and if you didn't watch Ervin, the soda is gone

but the can is still right there," said his mother, Linda Currie.

If he could get his hands on $5, he would turn it into a big bag of Fritos

and the biggest root beer he could buy. If he didn't have money, he'd

steal food, or forage in trash bins. That was just the beginning. As

people with Prader-Willi reach their late teens and early 20s, rebellion

and tantrums peak.

Such behavior does not always make sense to people untouched by the

syndrome. At semiannual support group meetings, Deborah Peaton of Tampa

can air ideas and express frustration, such as the defeat Mrs. Peaton and

her husband felt when they had to start locking their son out of the

kitchen.

"We were depressed. It felt as if he was giving in to the syndrome," she

said. "Instead, my son thanked us because he didn't feel the pressure of

having to sneak to get to the food."

Mark Lister, residential services director for ARC homes in Gainesville,

has seen the same thing.

"They get a sense of security when they are in an environment where they

are watched, when the loopholes are closed," he said. At the next meeting,

Mrs. Peaton can tell other parents about her son's decision to take 60

anti-depressants as a snack. His stomach was pumped before the pills took

effect.

"You learn to find humor in a lot of things kids do," she said, "while

other people would stare at you in shock."

Adhering to a routine

Ervin Currie is a charmer. His long eyelashes curl upward and he has a

wide, ready smile. He won't hesitate to tell a woman how lovely she is or

rattle off sports statistics with a man.

He wailed and crooned during services at Friendship Baptist Church in St.

Petersburg, oblivious of the words or the tune.

Still 4 feet 8, Currie weighs 207 pounds. His hands are small and his face

is narrow, traits characteristic of Prader-Willi patients.

He is a sharp-dressed man -- one who is married to his habits.

Growing up in St. Petersburg, his routine was rigid. For his mother, that

meant rising at 5 a.m. to wake him for his bath. He couldn't bathe the

night before; he was "fresher" if he bathed in the morning.

"If I got him out (of the bath) too soon, he had a tantrum later," said

Linda Currie.

The two-hour ritual involved lotions and brushes, each with a specific

function. When that process was through, he'd iron his clothes for the

day. Twice.

Currie's routine is an example of the obsessive-compulsive traits that

distinguish most people with Prader-Willi. Recurring drives compel them to

repeat the same behaviors.

As Currie got older, completing his morning ritual no longer set the tone

for a good day. Unexpected changes sparked tantrums. As his rages

escalated, police around his Lake Maggiore Shores neighborhood got used to

answering calls -- their presence cooled his temper.

Authority figures that barred him from bingeing became subject to

rebellion. To spite his mother, he would pocket the pills meant to

regulate his hypertension and diabetes. He would clog the plumbing by

stuffing the toilet with tissue. He threatened to kill himself, or kill

his mother.

Linda Currie learned to sleep with one eye open and knew that she couldn't

handle her son anymore. The disease was taking over. She was too weary to

fight.

When Ms. Currie learned of an opening at a Gainesville house run by ARC,

she and her son made the trip in May.

The house was nice, not just a sterile institution but bright and

cheerful. It is ruled first by love and then by laws, by a woman capable

of pinning a disruptive resident on the ground.

Ms. Currie knew she would miss her son, who didn't want to leave home.

Currie knew that he'd have to work, control his temper and overcome his

hunger.

A decision had to be made. He turned to his mother.

"Mom," he said, "I want to be a man."

Turning things around

The door slams in the cafeteria. At 2 p.m. Currie is in the workshop on

the other side of the door, assembling grooming kits supplied to inmates

at the Gainesville jail. This is his job.

Inside the cafeteria, a worker with Prader-Willi slips away from his post,

beyond the watch of his group leader. He is rooting through empty foam

containers in the cafeteria garbage bin.

Bingo.

Just as the half-eaten sandwich goes from his hand to his mouth, the group

leader charges through the door. She leads him back to his work station.

Michelle Kennedy, a woman with Prader-Willi syndrome, enters through

another door to the cafeteria wearing a green winter jacket. At 113

pounds, it's easy to catch a chill in the air-conditioned workshop where

she also assembles kits for inmates.

She is a leader among the workers. Like many residents in the Gainesville

homes, she has fought back the syndrome and has reached her goal weight.

Today she's wondering when Gov. Jeb Bush's people will call her back.

Kennedy, 27, has been given a Governor's Points of Light award for her

participation in the March of Dimes, and a lunch meeting with Bush is

being set up. When she came to Gainesville in 1993, she weighed nearly 300

pounds. One of her early fits brought down a glass display cabinet; others

caused the resignation of two ARC employees.

But she latched onto her new lifestyle quickly.

Once she got started, there was hardly a force that could bar her from a

45-minute daily walk on the treadmill. The energy and intelligence devoted

to bingeing was redirected. She filled out her daily calorie allotment

like a child divvying up the first dollar from the tooth fairy.

The weight came off slowly as she replaced destructive patterns with good

habits. She even resists bingeing when she visits her Seminole home. But

the disease always will be present.

She finishes talking about the March of Dimes, and her gaze rests on the

open garbage bin in the cafeteria.

"Jackie, you might want to put a lid on that," Kennedy says to Jackie

Hargrove, director of development at the ARC.

"Why? Is there food in there?" Hargrove asks.

"There might be," Kennedy says.

The best place

It has been a good day for Currie. He has lost 7 pounds in the month he's

been in Gainesville and has fallen into a comfortable routine.

He even skipped the lotion today. And there's nothing like love to quiet a

rebellious heart. Currie asks permission several times each day to visit

his girlfriend, Angela. She is a petite blond who also has Prader-Willi.

She beats him when they play Bingo, but he outscores her when they bowl.

"I try to keep her happy," Currie said. "If I can treat this girl good, I

can have a good girlfriend."

For Linda Currie in St. Petersburg, the 5 a.m. wake up calls are over. She

has moved the couch against another wall, away from the sentinel position

where she would guard her son from slipping through the front door.

She missed him at first but accepts that he's in the best place now.

"He's so sweet. He's the type of person you fall in love with regardless,"

she said.

The program in Gainesville is meant to appeal to residents'

obsessive-compulsive orientation by letting them choose every ounce of

food. Amid a setting of rigid rules and consistent schedules, positive

habits are rewarded and tantrums are suppressed.

The residents can earn extra calories for good behavior and exercise.

There are no loopholes and no sneaking.

"When they can fit into fashionable clothes, it changes their effect

almost immediately," said Steve Drago, ARC associate director. "They are

calmer and feel better about themselves."

Greater public awareness has led to earlier diagnosis of the disorder.

When doctors give growth hormones earlier, muscle tone and height

increase.

If the disorder is managed and target weight maintained, people with

Prader-Willi can live a normal life in a group home or with full-time

care.

It's 5:30 p.m., and Currie has earned another 200 calories, bringing his

total to 630 calories left for the day. His head bobbed from side to side

when he took a walk on the treadmill. He remembered to seek permission

before walking into the dining room.

He bows his head to thank God for his mother and his girlfriend. He

blesses the meal, each portion measured by the half-cup.