Wrapped in Love

from Children's Magazine, Fall 2004

By Kristin Armstrong
Photography by Steve Kast

When Chrishana White was pregnant with her son Bryce, she had a recurring dream. “I dreamed my child was born without skin,” she said. “It was awful. I cried constantly.” A few months later, Bryce was born “healthy as can be,” Chrishana said. But years later, when Chrishana had a baby girl, the dream came true.

‘Please Find Out What’s Wrong’

Abriana Renee White-Hulce was born April 29, 1998. As soon as she was born, doctors knew something was wrong. 

“She had little chicken legs,” Chrishana said. “My dad said it looked like she was missing some toes or something.”

Nurses wrapped little Abbi in a blanket and handed her to mom.

“She started to cry, and blood came trickling out of her mouth,” Chrishana said. “I started to cry right away. I handed her to the nurse and said ‘please find out what’s wrong.’”

Abbi was whisked to the Neonatal Intensive Care Unit. After a few hours, the pediatrician came in and explained that Abbi had thin skin on her hands and was missing a significant amount of skin on her left foot up to her knee. Her left foot also was smaller than her right. Abbi had kicked her way out of the amniotic sac in the womb, and the sac had fused around her foot. Her left foot hadn’t grown at the same rate as the right, the doctor said.

Doctors were “stumped” as to the cause of Abbi’s skin condition, so the pediatrician called in dermatologists from The Children’s Hospital and the University of Colorado Health Sciences Center (UCHSC).

William Weston, MD, and Gary Bellus, MD, took one look at Abbi and recognized the problem right away. Abbi had dystrophic Epidermolysis Bullosa (EB), a rare skin disease characterized by recurring painful blisters and open sores as a result of the unusually fragile nature of the skin.

Blood came out of her mouth when she cried, the doctors said, because nurses had suctioned out her mouth after birth – as hospitals do with all newborns – and had removed all the skin inside her mouth.

The doctors told Chrishana that Abbi would face many problems with this disease, which has no cure.

“Being a new mom, there are certain things you absorb and things you don’t,” Chrishana said. “I didn’t absorb a lot at first.”

The next day, a nurse showed Chrishana how to dress Abbi’s skin and how to lance blisters – time-consuming tasks Chrishana would have to do every day of Abbi’s life until Abbi was old enough to do them herself.

“I cried every second of every day,” Chrishana said. “I was devastated. I thought it was something that could be taken care of.”

Reality Sinks In

But whatever hopes Chrishana had that Abbi would outgrow or overcome the disease were dashed when she started reading more about EB.

Dystrophic EB affects not just the skin outside the body; it attacks tissues inside as well – the gums, the esophagus, even the eyes. Children don’t outgrow EB, and it doesn’t get better as a child gets older. Most children with the worst form of recessive dystrophic EB die from skin cancer by their 20s or 30s.

“I would read things and break down,” she said. “It was a very big blow in the beginning.”

Chrishana and Abbi stayed in the hospital for seven days, until Chrishana felt comfortable changing Abbi’s dressings.

For the first 18 months of Abbi’s life, it took three people – Chrishana and grandmas “Nacy” and “Mimi” – three hours every day to wrap Abbi’s limbs and torso with Vaseline-soaked gauze.

Although she had been taught how to change Abbi’s dressings, Chrishana soon realized not everything in Abbi’s life came with instructions.

“It took a good month to figure out how to give her a bath,” Chrishana said.

Abbi couldn’t sit in a bath chair – sitting on the hard plastic blistered her skin. Chrishana had to pad the chair with towels. But once mom and daughter figured out a system, Abbi did well. She started crawling at 9 months, Chrishana said – late for some children, but not uncommon for children with EB.

Abbi was born with fingernails and toenails, but blisters would form under the nails, and they eventually fell off – also common with dystrophic EB.

“I knew this would be an uphill battle,” Chrishana said. “You don’t realize it until it’s happening to you. They told me she could get blisters in her eyes, and I’m like, ‘no, that won’t happen.’ Then she got her first one. You don’t really accept things until they happen.”

Little did Chrishana realize, but things would only get worse.

A Welcome Discovery

Like most children with dystrophic EB, Abbi was plagued with dental problems. She had blisters in her mouth, sometimes covering her whole tongue. Her teeth started to grow in but would rot at the gumline because her gum tissue wasn’t strong enough to anchor the teeth. At about 16 months old, she had 12 teeth removed at Children’s dental clinic.

Two months later, her smaller left foot “dropped:” Abbi walked flat-footed on her right foot, but on her tiptoes on the left. After a visit to pediatrician Jay Rabinowitz, MD, Chrishana decided it was time to take Abbi back to Children’s, this time for surgery on her foot. Doctors lengthened Abbi’s Achilles cord and pinned her foot to keep it at a 90-degree angle. Normally doctors would cast the foot, but children with EB cannot wear casts because the skin will break down under the plaster. So doctors put Abbi’s foot in a splint, and as soon as they removed the pins, Abbi was “walking like a champ,” Chrishana said.

Soon after Abbi’s foot surgery, Chrishana was informed of Children’s multidisciplinary EB clinic – one of just three in the United States , said Alan Arbuckle, MD, co-director of the EB clinic at UCHSC, affiliated with Children’s EB clinic, and member of UCHSC’s department of dermatology.

Children with dystrophic EB have complex medical issues; most see dermatologists, dentists, occupational and physical therapists, gastrointestinal doctors, pain management specialists, nutritionists, psychologists, optometrists and hand surgeons.

Two years ago, before Children’s launched the multidisciplinary clinic, parents of children with EB would have to schedule appointments with individual specialists. A UCHSC doctor had the idea to launch a once-a-month clinic where the specialists came to the children. But it was Steve Berman, MD, professor of pediatrics at UCHSC and head of academic general pediatrics at Children’s, who implemented the idea.

“This is a complex disease that involves a lot of different organ systems,” Dr. Berman said. “Families needed better coordination of care among all the specialties involved. The clinic started out of the desire to do better for our children and their families.”

Registered Dietitian Stephanie Effrein works with EB patients to make sure they get enough calories and protein to rebuild their ever-healing skin.

“Having EB is equivalent to having third-degree burns,” she said. “There’s so much fluid, protein and mineral loss.”

“They almost can’t consume enough calories to grow,” said Procedure Nurse Deb Bracken, who helps EB kids and their families learn how to change dressings and started a support group at Children’s for parents of children with EB.

Specialists at Children’s see about 20 children with dystrophic EB a year, including Abbi. Each specialist meets with each child, then all the specialists meet at the end of the day to develop a care plan for each child.

“The multidisciplinary clinic is really very helpful,” Chrishana said. “Children’s has a lot of doctors who really want to help these kids. It’s so nice to have professionals who know what they are doing.”

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