The Life of Brian
Brian Ilg wakes up every morning with deep red stains smudged into his white t-shirt — blood from his back, shoulders and underarms. “Most of the time, it doesn’t hurt,” says Brian. “They have to fix up my bandages so it doesn’t wrinkle.”
Brian’s wounds aren’t from a rough football practice. The abstract maroon splotches on his clothing come from the friction of sleep. “They get it the worst,” says Brian’s mother, Arlene Ilg, about her son’s sleepwear. “He’s sleeping and rubbing on everything.”
The slightest bump or scratch can tear Brian’s skin open as the result of a genetic disorder that has plagued Brian with fragile, blister-prone skin since birth.
Half of the shelves in Brian’s bedroom are lined with Skylanders, Minecraft toys and games. The other halves have cardboard boxes full of medical supplies. A tall white cabinet next to Brian’s bed is also full of supplies – bandages, gauze, ointments, needles and different types of dressings. Brian’s bed is equipped for movement like a hospital bed so his nurses can adjust it as they treat him.
“Brian likes to tell people, ‘My skin is very delicate,’” says Mary Emerson, a Stony Brook University Hospital nurse who has cared for Brian since he was two years old.
There is no school bus waiting for Brian – his nurse is also his driver, and on Monday’s, that nurse is Mary Emerson. She strategically buckles him into the backseat of her car so the seatbelt doesn’t irritate his skin. When the pair arrives at River Elementary, Mary walks Brian to his class but stays with him throughout the day like a private bodyguard. Different nurses accompany Brian at school on other days of the week.
Emerson is in the process of building a tiny house on wheels for Brian. The house is nicknamed the Honey House, because Emerson calls Brian her "little honey." The Honey House will function as a mobile medical unit to help with Brian’s condition. Emerson says the it will make Brian’s treatment easier for him and his family, and will free up space in the house occupied with medical materials.
“It’s also tough on a kid to have your medical care done right in your bedroom,” says Emerson.
There was only one thing that concerned Arlene and her husband Greg during Arlene’s pregnancy with Brian. The baby’s sonogram showed Brian might be born with clubfoot, a condition where the foot turns inward at the ankle and points down. Everything else seemed fine. Brian's father, Greg, had a local for retail job in Long Island, and before Brian, Arlene worked as an attorney in Manhattan. Annalise, the Ilg's first child, was born a year and a half before Brian without any issues.
But complications arose during Brian's delivery, and Arlene’s 24-hour labor into an emergency cesarean section. The doctor took Brian out, cut the umbilical cord and passed the baby to the nurse on his left.
Greg was standing behind the doctor during the delivery, and he remembers seeing the nurse glance at baby Brian with an uncomfortable expression. Brian was covered in blood as all babies are at birth, but the difference between Brian and other babies was that his skin was hanging off. The nurse looked to the doctor for guidance. “The doctor just kind of looked at her like, keep going, ‘cause the doctor was having problems with Arlene at the time,” says Greg.
The nurse took Brian to a cleaning station, but when she tried to wipe the blood from his tiny body, his skin came off. “Brian was hysterical,” says Greg. “He was hemorrhaging from the mouth, the nose and everything else.”
When the delivering doctor finally saw Brian, the medical staff moved Greg to a private waiting room and called in a specialist. After hours of waiting, a dermatologist told Greg the doctors weren’t 100 percent sure, but Brian might something called epidermolysis bullosa. “I was like, ‘What the heck is that?’” says Greg.
Epidermolysis bullosa is a rare genetic skin disorder that causes skin to blister and bleed with any kind of friction or agitation. “My husband did some research online because we had never heard about it before,” says Arlene.
A few hours after the delivery, Brian and Arlene were transferred from their local hospital to the Stony Brook University Hospital where, where doctors performed tests on Brian and confirmed EB as his diagnosis.
“They took me aside and told me what it was. There was no cure, there was no treatment,” says Greg. "I wish he would have had the darn club foot."
EB varies in severity. Some people only experience a mild blistering on their hands and feet. Others with a more extreme diagnosis are susceptible to both external and internal blistering all over their bodies. “We knew that the prognosis wasn’t great if it was a more severe kind,” says Arlene.
People with a severe form of EB don’t generally live past their late teens, early twenties. The disease stops children from being children – bumps, bruises and scratches can lead to bloody and painful blisters or even a trip to the hospital. Brian’s hands are cupped and curved inward like tiny mittens. His fingers webbed together after years of blisters and skin regeneration.
“It’s like an eraser,” says Emerson about how EB affects the skin on Brian’s hands. “It disappears after a while.”
What is Epidermolysis Bullosa?
Epidermolysis bullosa is a rare genetic skin disorder that causes skin to blister and bleed with any kind of friction or agitation.
— Mary Emerson
Photo by Kerlern Rae Tuitt
It took a week after Brian was discharged from the hospital for his biopsy results to return. The dermatologist confirmed Brian’s condition as recessive dystrophic epidermoslysis bullosa – the second-worst type.
The only available treatment for Brian was a daily dressing change similar to what hospitals do for burn victims. Brian would have to be covered from the neck down in a mixture of brown and white bandages, wrappings and gauze to help protect his skin. Arlene and Greg were distraught and clueless as how they would wrap a baby with such delicate skin, especially when they had little control over his flailing arms and legs.
“At Stony Brook, we got lucky in the sense that there was another child born with it before Brian,” says Arlene. “There were still some people on staff there that knew how to do dressing changes and manage it. There was also a dermatologist who treated that little girl, who has since passed away.”
The dressing change is a long, and often painful, process that has been a critical part of Brian’s life since he was born. It can take anywhere from two and a half to three hours to get Brian unwrapped, bathed and bandaged. When Brian was a baby, Greg and Arlene spent close to six hours a day doing dressing changes for Brian. “Both of us almost lost our jobs because we were doing dressing changes and we didn’t have nurses at the time,” says Greg.
Medicaid provides aid for children with severe medical conditions through a care at home waiver. In addition to certain requirements, Brian had to have been hospitalized for 30 out of his first 90 days since birth to qualify. Medicaid granted the Ilg family help in the form of at-home nurses who now help with the dressing change process.
Brian’s nurses were originally sent through an agency, but the nurses were ill-equipped to take care of a boy whose skin wouldn’t stay on. “They didn’t know anything about EB,” said Greg about the first six months with the nurses. “And with the agency, you get a different nurse whenever they send one.”
The Ilgs eventually changed from agency nurses to resident nurses who stayed with the family and could be trained in EB baby care. Brian now has a rotation of five in-home nurses who care for him morning, afternoon and night. “I kind of ignore them so it doesn’t feel like they’re there, and I’m by myself,” says Brian. “So it really doesn’t both me that much.”
Nurse care is essential to Brian’s wellbeing, but having a nurse around all the time also has an impact on the rest of the family. “There’s no… no get up in the morning and walk around in your underwear,” says Greg. “There’s no silly stuff that you take for granted. If we go out to dinner, guess what? The nurses are going out to dinner.”
Brian’s nurses also take him to school in the morning and attend classes with him. “They assess him medically during the day, they help him with his food, they help with toileting,” Arlene says, “and they keep him safe from regular child behavior."
Despite Brian’s positive attitude, his physical limitations still restrict how the Ilg family functions. Before Brian was born, Arlene and her husband were very active – horseback riding, skiing, snowboarding, going to the beach and boating were just some of the activities they did but now refrain from to accommodate Brian. “I’m not going to do those things and leave him home,” says Arlene.
Children with EB are referred to as “butterfly children” because their skin is so fragile. So Brian can’t play and roughhouse as boys his age do. But he did have the chance to play baseball with the Miracle League, a league for young athletes who are physically or mentally disabled. “I used to, but it was way too easy,” says Brian, “because the people – sorry people that were playing, but – weren’t that very good.”
When the students at his school played a game of crab soccer, Brian got to be a goalie, but his nurse did most of the blocking. “He’s a frustrated athlete,” says Arlene, “But he knows his limits. He entertains himself.”
Brian plays video games, takes drum lessons and is also in an acting class. (“I’m pretty sure I’m going to be moving on to stunt-double,” Brian said in a News 12 interview last Fall.)
If Brian stayed in bed and never moved, he’d be fine – there wouldn’t be any friction to pull at his skin. But with EB, the top layer of skin just floats over the second layer, so blisters for Brian are a big threat.
“If you push a blister on us, it will anchor,” says Jennifer Levasseur, a veteran nurse training to work with Brian. “But with him [Brian], if you push it, it will keep growing and traveling.”
The time of year also affects what Brian and his family can and can’t do. Summer keeps the Ilgs housebound in 24-hour air conditioning because Brian’s bandages cause him to sweat and overheat in the hot weather. “He’s bandaged from the neck down so it’s like wearing a sweat suit,” says Arlene.
The hotter he gets, the itchier he gets. If he gets itchy, he scratches, and if he scratches, he breaks his skin open and causes more wounds. “In another house, you might be able to do things to try to save money like not use the conditioner on a night where it’s not that hot but you just can’t do that with him,” says Arlene. “It has to go and you got to find a way to pay for it and everything else.”
The Worst Disease You've Never Heard Of.— The Dystrophic Epidermolysis Bullosa Research Association of America (Debra)