A Most Fragile Boy
"See?" says his mom, Whitney. "From getting all crazy, your finger is bleeding."
"Oh well," says Jonathon. Look closer, and a visitor can see that his small hands are covered with blisters, that his fingernails are gone and that, beneath his gray T-shirt Jonathon's skinny arms are swathed in gauze. Jonathon calls them his "boo-boos"—the constellations of small lesions that cover parts of his body at any given time. To his doctors they are the ever-present symptoms of epidermolysis bullosa (EB), an incurable genetic disorder that affects about 25,000 people in the U.S. and causes skin to become as fragile and easily torn as tissue paper. To his parents they are reminders that their oldest child faces a life of pain and potential loneliness.
Kids like Jonathon are known as "butterfly children" since, like the wings of a butterfly, their skin can be damaged by even the lightest contact. In Jonathon's case, simply holding a pencil, standing in ocean surf—even the flow of his own tears—can cause cuts, splits, blisters or a rash that looks like a bad sunburn. One of the boy's first blisters came after he tumbled from his car seat at the age of 2 months. The angry red lesion is still there today, on the tip of his nose. And then there's what his parents call "the scream"—that terrifying shriek, audible anywhere in the house, when Jonathon has hurt himself. "It's horrific," says Whitney, 25. "He can be playing next door, and I'll be in the kitchen and hear it. I don't think we ever run as quick as we do when we hear that cry."
How often is that? "Almost every day," she says. That's no exaggeration. When he was 4, Jonathon's first serious infection erupted from a blister on the back of his leg. "We went to the doctor that morning," says Whitney, "and by the time we got to the hospital four hours later, his calf was red, hot and the size of his thigh." When he was 5, perhaps because of something as simple as a bump, Jonathon—who is speech-delayed and either would not or could not say how it happened—got a cut on his right middle finger "almost down to the bone," says Whitney. "It was black, just wide-open"—and almost led to an amputation. And in 2003, in what Whitney remembers as one of his worst falls, Jonathon slipped on a sidewalk and slid down a hill outside their house. "He screamed, and all the skin on his knee just came sliding to the top of his knee," she says. "It scraped off on the bottom of his palm, turned into a huge blood blister and took three weeks to heal." After a bad accident, Jonathon becomes withdrawn, sitting in front of the TV rather than risk bumping into something outside. "He'll scream and his face has pain written all over it," says Whitney, "but I can only think of three times where he's admitted something really hurt."
Jonathon's days always end with the same painstaking ritual. After switching on his police scanner, a birthday gift from the boy's grandfather, or tuning the TV to Cops, his favorite show, Whitney or Jonathon's father, Jonathon Rose, 25, a mason, spends 90 minutes dressing wounds and wrapping their son's body with gauze. (Before insurance began picking up the tab in 2003, they were spending half their income each month on supplies alone.) At Pentucket Lake Elementary School, where he's in first grade, the classroom, nurse's office and school bus are all stocked with wrapping supplies—although Jonathon will often take off the bandages if they become uncomfortable. "He's very sly about it," says his first-grade teacher Jan Scanlan. "One day I was cleaning out his desk and there were these little gauze pads inside."
Like most parents of kids with EB, Whitney, who's studying criminal justice at a community college, and Jonathon at first had no inkling that anything was wrong with their child. "When he was born, he was perfectly fine—the cutest, healthiest little thing," says Whitney, who met Jonathon Rose through friends on Cape Cod seven years ago. "Then, when he was 5 days old, my sister noticed a blister on his left heel." Doctors said it would disappear. But a few days later it was the size of a quarter.
It took three months for Jonathon's parents to get a diagnosis from doctors, who at first believed he had only a mild form of EB. At 8 months, Jonathon began getting blisters on his hands and feet, and after further tests physicians at Massachusetts General Hospital in Boston found that he was suffering from a serious form of the disease. "I was shocked. I couldn't believe it," says Jonathon Rose. Pulling information from the library and Internet, the couple learned through trial and error. They kept bumpers on their son's crib until he switched to a bed and learned to dress him only in soft clothes and extra-roomy shoes. Otherwise, says Whitney, "he'd say, "It hurts.'"
Those aren't the only ways the family has learned to adapt to his illness. When his dad lightly tosses him a ball, "most of the time he won't catch it. He'll let it fall, then pick it up and throw it back to me," says Jonathon Rose. Whitney confesses that, for no reason, she feels guilt about her son's condition. "What could I have done to prevent it? Was it something I ate or drank?" she asks. Jonathon Rose becomes so upset seeing his son handled by doctors that he rarely goes with him to the hospital. "It's not that he doesn't want to be there, but he breaks down," says Whitney. "He gets very emotional. He'll kill me for saying it, but he will cry."
Yet the couple says that, if anything, their son's illness has brought them closer. "I don't have anybody else to talk about it with," says Whitney, "so we come home and vent with each other." As for getting married one day, Whitney says, "there's so much going on in our lives that that honestly doesn't come up very much." Jonathon's little sister has no signs of EB, but it was five days after Ariana's birth before Whitney could relax and pick up her daughter without worrying about causing her harm. Now it's a struggle to spend equal time with their children, given the amount of attention that Jonathon requires: "If he falls, it's a big deal. If she falls, it's not. So she'll kind of go overboard [feigning pain], and we've started to feed into that a little, like, 'Okay, we'll wrap you with gauze.'" When Jonathon goes to the hospital, Ariana cries—and that makes her parents cry.
Four months ago, a classmate accidentally hit Jonathon in the head with a paddle at school, causing a cut that required two metal staples. Jonathon's reaction? "I didn't cry," he says. "I don't care." But for all his apparent bravery, he sometimes reveals his true feelings. His classmates "love him to bits," says Scanlan. But it was in kindergarten that he first began asking, "'So-and-so doesn't have boo-boos. How come I do?'" recalls Whitney. "I tell him, "It's how you were born, and it just makes you special.'" Angela Sousa, 21, a biology student interested in EB who met Jonathon after posting on a Web site, has become a friend. Not long ago, as she showed Whitney a new technique for lancing his blisters, Jonathon suddenly tensed up. "He was afraid it was going to hurt," says Sousa. "Then he asked, 'Are you going to fix my hands for me?' I told him, 'I'm going to try to make it feel better.'"
That, of course, is what everyone wants for Jonathon. With no cure in sight, his future is uncertain (see box page 64). His parents know that some adult EB patients live lonely lives, afraid, quite literally, of ever being touched. "He'd love to be a cop or a firefighter, the stuff normal boys dream of," says Whitney. "But unless they come up with a miracle cure, I don't think he could do it. Maybe an office job in a police station, something not too physical." For now she's grateful that Jonathon "has a good set of friends who look past the bandages. I just hope they'll always be his friends." And that someday he'll be able to live on his own and find "a nice girl, who will accept him for who he is and who'll look beyond the "boo-boos.'"
In the meantime she knows there are limits to what she—or anyone—can do for her son. With one notable exception. "I can give him a hug," she says. "I just won't squeeze him too hard."
WHAT'S IN STORE FOR 'BUTTERFLY CHILDREN'?
EB is caused by genetically mutated proteins in the so-called basement membrane separating the first and second layers of skin. Those proteins "act like glue that keeps the two layers together," says Dr. Jo-David Fine, an EB expert at Vanderbilt University. In patients such as Jonathon, that glue does not work. As EB patients age, they often lose their teeth and develop lesions in the throat (making eating difficult) and on internal organs. They also face a higher incidence of skin cancer. According to nurse Madeline Weiner, the psychological impact can be serious: "The most difficult part is isolation and loneliness."
On Newsstands Now
- Brad's Devotion: The Inside Story
- Oklahoma Tornado: Heroic Rescues
- Michael Douglas on Catherine's Health
Pick up your copy on newsstands
Click here for instant access to the Digital Magazine