Patients come to UF&Shands for a variety of reasons, and many face the same problem as Hunter Dandurand — a rare disease few have ever heard of, let alone understand how to treat. In this issue of The POST, we take you inside Hunter’s life with the uncommon and painful condition epidermolysis bullosa.
By Mina Radman
His dad, John Dandurand, thought Hunter would lose his legs. Doctors at Tampa General Hospital thought the amniotic sac had ruptured, causing bands to wrap around his legs and stop development, a condition known as amniotic band syndrome. They wanted Hunter to see a plastic surgeon.
A plastic surgeon with a specialty in infant care spent several days diagnosing Hunter. He told John that Hunter’s legs would look different, but the skin would grow back and his legs could stay.
The surgeon said there was only one other possible diagnosis to Hunter’s underdevelopment: epidermolysis bullosa, or EB, an inherited dermatologic disease that causes constant mild to severe blisters on the skin.
“He goes, ‘Thank God it’s not that,’” John says, “‘because that’s the worst disease you’ve never heard of.’”
Several days after birth, Hunter was transferred to All Children’s Hospital in St. Petersburg. His parents stayed at the nearby Ronald McDonald House while Hunter stayed in the hospital. Once they were settled, John began to research EB.
“I’m looking at this picture of EB in the book and looking at Hunter, and it sure looks like that’s what he has,” John says. “But he had no blisters.”
Two days later, the first blister appeared on Hunter’s right hand. Doctors said the insertion of an IV in his hand caused the blister, but John disagreed. The next day, he noticed that every time Hunter scratched his face, another blister appeared. Doctors agreed to send a biopsy for confirmation. The biopsy came back positive: Hunter had the disease.
“Hunter’s mom was miserable,” John says. “She asked how this could happen to us, and I said ‘Well, there are six babies in the ICU with Hunter that are all crack babies and who don’t have parents around because their parents are in jail or worse. Hunter has parents. He has family that can take care of him.’”
Doctors said Hunter would live for about two months. When Hunter passed that, they said he would live to be 2 years old. When Hunter turned 2, doctors said he’d live to be 20 years old.
Today, Hunter is 15.
“He’s going to live to be as old as me,” John says.
A doctor who listens
Hunter lives in Crystal River, Fla., and now comes to UF&Shands, the UF Academic Health Center, for his care. Kimberly Merkel, M.D., an assistant professor of dermatology in the UF College of Medicine, monitors the condition of Hunter’s skin and provides Hunter with necessary resources.
Hunter received a referral to see Merkel in the fall of 2011. Merkel says Hunter and his parents were skeptical to see her because many physicians aren’t aware of EB, but they quickly bonded.
Merkel works with several children who have EB. She became interested in the rare disease in September 2011 when an infant with EB was transferred to Shands at UF. As the on-call dermatologist, she was called in to work with the baby.
“The parents and family really needed someone who could come in and provide them with treatments and resources, because it’s a devastating thing to have your child born with EB and feel helpless,” she says.
Merkel began to research EB to learn as much about the disease as she could. At that point, UF experts were not well-versed on the disease, so Merkel began acting as a liaison between her patients and other doctors or resources that she knew could help them.
“Patients with EB have usually gone through this disease alone,” she says.
Hunter gets blisters as often as three or four times a week to three or four times a month. The blisters occur all over his body: legs, arms, hands, throat and even his eyelids. People who are born with EB lack the proteins needed to anchor the layers of skin together, and the epidermis, or outer layer of skin, easily breaks and blisters. They’re often called “butterfly children” because of their skin’s fragility.
Many people with EB use wheelchairs and cannot use their joints. Unlike some kids, Hunter can walk — although it’s painful — and he can use his hands.
Hunter says if he could remove any one part of his disease, he’d get rid of the eye blisters.
“It’s the equivalent of having a burn on your eyeball itself,” he says.
There are no treatments to ease the condition, just steps patients can take to combat the constant pain from the blisters and prevent complications and infections.
For as much as Hunter and his family gain from Merkel, she also learns from him. She says that working with Hunter reminds her why she went into medicine.
“He reminds me to listen,” she says. “We are taught so much and know so much, but we still need to be reminded to listen to our patients. Hunter teaches me that he’s strong and resilient and that even just listening and providing a resource can be incredibly rewarding and helpful.”
A day with EB
Hunter follows a strict cleaning regimen to keep the blisters under control. He wakes up each morning at 5 a.m. and begins the day by taking a bath with warm water and an eighth of a cup of bleach, which helps wash out the blistered skin. He uses a special medicated soap for his knees, and slathers his joints in coconut oil because it helps the skin to stretch and move freely.
After his bath, Hunter wakes up his dad, who applies ointment and wraps bandages around Hunter’s legs. The living room is filled with boxes of bandages, gauze and Aquaphor healing ointment. John uses a box of gauze a day to wrap Hunter. If Hunter doesn’t have many blisters, he’ll use only gauze. If he has a lot of blisters, he’ll use medicated wipes, then Aquaphor and gauze. Hunter is wrapped twice daily.
The insurance company began covering the supplies several years ago, but before then, the Dandurands would purchase a box of medical supplies for $1,000 per month. They wouldn’t know what types of medical supplies were in the 4-by-4 feet boxes. One time, the box contained supplies for newborns, so the Dandurands donated it to a nearby women’s clinic.
“The insurance company didn’t see that bandages are necessary,” John says. “They think that if you’ll need something or you’ll die, you should take him to a hospital. So, what you do is threaten to take him to the hospital every day, which costs a lot more than supplying the bandages.”
Without the bandages, Hunter would be confined to a sterile environment.
“That’s the scariest part,” John says. “If you don’t have the bandages, then he can’t leave.”
After wrapping his legs, Hunter and John go to AJ’s café for breakfast. Then they spend the day working at their pawn shop, called Hunter’s Hideout, which John started to help pay medical bills. Hunter’s teachers come in around 3 p.m. each day to administer homework and tests. After work, Hunter and John head across the street to Lollygaggers Sports Pub & Grill for dinner and go home, where they watch a movie on Netflix before bed.
A typical kid
At 4’10”, Hunter doesn’t look like a high school sophomore. He hasn’t been through puberty yet, and doctors are unsure if EB has played a role.
Hunter can’t eat chips, and he chews ice cream because EB constricts the esophageal opening. His favorite food is clam chowder soup because it’s the easiest to eat.
Despite the difficulties, Hunter tries to be a normal teenager. He plays Halo 4 on his Xbox 360, takes photographs and watches “That ’70s Show” and “Doctor Who” on Netflix. He spends weekdays doing schoolwork and working at the pawn shop, and weekends with his mom and stepdad.
Hunter says his height and skin resulted in a lot of bullying in middle school and the beginning of high school. His cousin walked with him to and from classes and acted as a protector to scare off freshman students who tried to bully Hunter. But once people got to know him, the bullying stopped, he says.
“It’s not much of a problem,” he says. “I’ve got a lot of friends in school now.”
But Hunter does suffer from severe anxiety because of EB. When he feels anxious, he scratches at his skin and reopens old wounds. His biggest fear is that he’ll be confined to a wheelchair later in life, and he wishes he could do more physical activities right now.
“I want to be able to walk around with my friends from school to the mall,” he says. “But I can’t because it will constrict my legs and hurt.”
Despite the pain, blisters and scarring, Hunter finds joy within his disease. He claims to be the best person to attend Disney World with because he receives “the ultimate fast pass” that allows him to skip to the front of lines whenever he wants.
“At Disney, it’s you’re off, I’m on,” he says.
When people say rude things about Hunter’s skin, he responds with snarky comments of his own. If anyone should have fun with his disease, it’s him, he says.
“People sit there and ask, ‘Is it contagious?’ and I say, ‘Is your ugly contagious?’” he says.
A bright future
Hunter currently does not attend regular classes at his high school because he has several upcoming surgeries to remove his wisdom teeth and fix the skin on his hand. However, the teachers who come to the pawn shop will help Hunter graduate on time and walk across the stage with his classmates. Despite the EB, he’ll be able to live in a dormitory when he attends college, too.
“Hunter is timid and fragile, like tissue paper — maybe even wet tissue paper at times — but that’s only what he’s made of,” John says. “If you put the world champion wrestler in the room, and they got to work stuff out, I am betting on Hunter. He might not win the wrestling contest, but he’ll figure out how to get the guy tied up.”
Hunter wants to graduate high school, attend UF and double major in genetics and journalism so that he can find a cure for EB and write about it. He wants to start UF’s first Recessive Dystrophic Epidermolysis Bullosa program because that’s the specific type of EB he has.
“I want to make it possible for others to not have to go through what I went through,” he says. “I think I’ll be able to find a cure.”