Cathy and Steve (not their real names) dream the same dreams other young couples do. They talk about marrying someday, having a home, and raising a family.
"Not big dreams," Cathy says, leaning forward to touch my shoulders. "Normal dreams."
She pauses a moment, stands up straight, and then tugs her pink-and- white sweater down over her stomach.
"Dreams like you have," she concludes, apple-green eyes reflecting a maturity that seems beyond her 22 years. "Right, Steve?" she half yells.
"Right, Steve?" she bellows.
Steve, 24, standing just an arm's length away, toddles over, puts his tiny pale hand over Cathy's mouth, and declares, "Women can be such a pain in the neck."
Both throw their heads back and roar with laughter. For an instant they are happy, their minds seemingly far away from the syndrome that consumes their every thought.
Then Cathy whispers, "We try not to think about food and eating. We really do." She leans forward to touch my shoulders again. "It's just so maddening sometimes."
Steve's thick brown-rimmed glasses slide down his nose, revealing narrow, bleached-blue eyes. "We understand what we have," he says, eyes staring through me. "We have Prader-Willi. The doctors even explained that we have Prader-Willi. We just don't know why us."
Again Cathy stands straight, pulls her sweater back down over her stomach, and echoes his question. "Yeah. Why us?"
Prader-Willi syndrome has been estimated to occur in as many as one out of every 10,000 births. This rate is less frequent than that of Down's syndrome, which is estimated to occur once in every 700 births, but the Prader-Willi numbers are still surprisingly high, especially considering that the birth defect remains obscure, almost unheard-of.
The syndrome was first described in medical literature in 1956 by three Swiss doctors named Prader, Willi, and Labhart. The syndrome was defined by a team of researchers in the journal Current Problems in Pediatrics as "a multi-system birth disorder characterized by . . . gross obesity, slight mental retardation, short body stature and underdeveloped sex organs." Yet the most bizarre characteristic of individuals born with the syndrome is "the development of an insatiable hunger in toddlerhood . . . which results in an uncontrollable urge to eat." The journal also states that if not continually monitored, those with Prader-Willi "would literally eat themselves to death."
A number of leading medical magazines, including Science News, have reported over the past few years that the syndrome, which has no cure, is believed to be caused by an abnormality in chromosome 15. This is the chromosome that is thought to affect the hypothalamus, the area of the brain that controls the appetite-regulating center. Researchers have discovered that a small portion of chromosome 15 is missing in more than half of all known individuals with Prader-Willi. Yet it is difficult for doctors to test whether a baby will be born with the syndrome, since about half of those who have it show no prenatal abnormality.
Dr. Andree Walczak, a specialist in children's dysfunctional syndromes at Michael Reese Hospital, was the first to diagnose Steve and Cathy as having Prader-Willi syndrome. She is largely responsible for bringing the syndrome to light in Illinois. "I had studied Prader-Willi in Switzerland, where it was fairly well-known," she says. In fact, she attended a number of Prader's guest lectures during her medical training at the University of Geneva. "When I came to Michael Reese Hospital in the 60s, I was shocked at the lack of medical attention the syndrome received--only two or three pages in the best medical journals.
"Then, in the fall of 1975, I had an alarming number of families come in with Prader-Willi children who had no idea of what was wrong. That's when I started the Prader-Willi clinic here."
Michael Reese now houses the only Prader-Willi diagnostic and treatment clinic in Illinois. Since its inception, Walczak says she has diagnosed 60 to 70 patients as having the syndrome.
"As babies, Prader-Willis are easy to recognize if you know what to look for--but that was the catch, of course," says Walczak. "Unlike normal babies, the Prader-Willis have no muscle tone to move, cry, or suck.
"I compare them to newborn kittens--they just lie there and mew. But when they become toddlers, they develop such voracious appetites, food must literally be locked away--even the garbage."
Walczak states that older people with Prader-Willi are most often characterized by their extreme obesity, short stature, and slumped shoulders. They also have abnormally tiny hands and feet, the result of a disorder in growth-hormone production. Typical facial features include almond eyes and a small mouth.
"I continue to see grown adults in the streets or in stores who have Prader-Willi that I am certain have not been diagnosed," Walczak says. "It's been over 30 years since its discovery, and people still do not recognize it."
It is lunchtime at Glenkirk's Vocational Training Center, a workshop in Northbrook where the developmentally disabled can learn basic work skills doing simple jobs contracted out to them by area businesses. While the rest of the group is led into the lunch area by several counselors, Cathy, Steve, and the seven other individuals at Glenkirk with Prader-Willi stay behind. They are asked to eat at a separate table.
"People who have Prader-Willi syndrome often act out of desperation to get food," says former Glenkirk program coordinator Mark Robinson. "This is a main characteristic of the syndrome."
Indeed, the refrigerators at Glenkirk are always locked, and the workers' sack lunches are kept stored in cabinets with combination locks.
Robinson and Walczak agree that the lengths people with Prader-Willi will go to in acquiring food is often astonishing. "Most Prader-Willis are not severely retarded," Walczak says. "They can actually be very sociable, polite, and talkative most of the time. But they are unusually bright and cunning when it comes to stealing food--and it's all due to the defect controlling their appetite. They simply never, ever feel full, and so they have this continual urge to eat everything."
Glenkirk has been in existence 35 years. In addition to the vocational center, it runs the only residential treatment program for individuals with Prader-Willi syndrome in Illinois--one of only ten programs nationwide. It has three small group homes, located a few miles from the vocational center. One of the homes is also Glenkirk's office.
"Only after funding from the state came through, and the residential home was acquired, did we initiate the whole program," Robinson says. "We wanted those with Prader-Willi syndrome to have a continuity and total support in their everyday lives."
Robinson says it was difficult deciding which individuals would live at Glenkirk's group homes and work at the vocational-training center. "We had 17 applicants for just eight spots in the house," says Robinson. "We accepted four males and four females. It was not easy to turn down the other parents and their children. But for five of the individuals we chose, their parents were in their 70s. And, honestly speaking, they weren't going to be around forever to take care of their children."
At the group homes each participant has a roommate, and supervisors are always on hand. A dietitian provides strict, balanced diets, ranging from 800 to 1,800 calories. Daily exercising, which includes aerobics and stationary bicycling, is used to help build muscle tone.
Cathy and Steve, who are both residents at one of the group homes, have done quite well. "I've lost 32 pounds so far," says Cathy, who is four feet 11 and now weighs 158 pounds. "I exercise every day, and my parents say I've changed.
"It's nice to go home, because Mom wants to do everything for me. But here we have to do everything for ourselves."
Steve, who is five feet four, exceeds the average height of those with the syndrome by three inches. He has lost 39 pounds. "I weigh 159 or so, I think," he says. "I know what I'm supposed to do, but it gets so damn hard."
"Steve and Cathy are lucky to be in a program like Glenkirk," Walczak says. "They are being treated, exercised, monitored, and helped there."
She says that people with Prader-Willi can have normal life expectancies if they are under constant supervision, mainly so they can keep their weight down. "But due to the limited availability of such programs, most individuals with the syndrome are often on their own."
Cathy and Steve have recently been promoted from their training positions as paper collators to new jobs. They are now bulk mailers.
In the work area, they stand in front of large tables covered with business advertisements and fliers, and separate them according to zip code. They then group together bundles of ten with rubber bands. They are compulsively meticulous about their work--a trait common to those with Prader-Willi.
Cathy bundles ten fliers, puts the bundle in a box, and then stops. Grabbing the bundle once more, she removes the rubber band and counts the fliers again, making sure there are ten--no more, no less. She repeats this process two more times before moving on.
Steve starts counting his out loud, trying to disrupt Cathy. "One, two, three, four!" he screams. "Five, six--"
"Shut up, Steve!" Cathy shouts.
"Three, four, five!" he screams.
Confused himself, Steve starts again too.
Suddenly, both stop and look up. A counselor who had been drinking a soda puts it down on a nearby table and walks a couple of steps away. Cathy's eyes widen, Steve's eyes narrow. They both stare.
Within a few seconds, the counselor returns. She picks up the soda, finishes it off, and takes the can with her.
Cathy and Steve continue to stare.
The two have IQ levels higher than those of most people with Prader-Willi, who usually average in the 70s. Their higher intelligence allows them to talk about their syndrome in a way most others with Prader-Willi cannot--with candor, hope, and understanding.
"My mom explained Prader-Willi to me when I got to be a little older," Cathy says, as she counts her fliers. "I understood it, I really did. Sometimes I think I'm getting better, you know? And then I know I probably won't get better, because I can't."
She sits down and runs a hand through her curly brown hair. "How much you learning about us?" she asks quickly. "They tell you about any new cures?"
She waits for an answer. When none comes, she sits placidly and sulks.
Counselors say Cathy's emotions are hard to control or predict, which makes her just like others with Prader-Willi. "When they get upset, as they often do, you just let them go," says Robinson.
In spite of their syndrome, Steve and Cathy are graduates of regular high schools. Steve knows that he is bright; he calls the other workers "the kids."
"But high school was not fun," Steve says suddenly, angrily. "I hated it, I hated it, I hated it."
For a moment, he looks as though he's going to cry. Then he turns quickly, plops down in a metal chair beside Cathy, and whispers, "Kiss me."
It's 2:30, time to go home. Steve and Cathy are told to put up their work.
They pack their bundles into the boxes as carefully as if they were made of glass, making sure everything is just right. Then they both sit down to wait for the van that will take them back to their group home. Steve places his arm around Cathy with all the poise of a first date. The counselors call him the Don Juan of Glenkirk.
"I first met Cathy in high school, when I was 12," Steve says nonchalantly. "We fell in love instantly." He places his hand over Cathy's mouth.
"Did not," she mumbles through his fingers.
"Did so!" he screams.
"Did not!" Cathy yells, her face turning red and blotchy, the way it often does when Steve is near.
Finally the van arrives. Cathy and Steve toss on their coats, tie their scarves, and put on their gloves.
On his way out, Steve pauses a second and looks around thoughtfully at the others. "Sometimes I think it would be better to just be one of the kids," he says.
Art accompanying story in printed newspaper (not available in this archive): illustration/Peter Hannan.