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. © Copyright 2001 St. Petersburg Times.