TLC Required: Rare skin disorder causes baby's skin to be extra fragile

But you may already know that.

Jonah's mother, Patrice Williams, is a dedicated blogger, and it's how much of the world has heard about her son.

Jonah was born with a rare genetic skin disorder -- epidermolysis bullosa, or EB -- on Feb. 27 at Forsyth Medical Center. People with EB lack proteins in their skin. The missing proteins cause their skin to blister easily, repeatedly and often severely under pressure or friction.

There are four major forms of EB and depending on the type, the outlook for people with the disorder ranges from a normal lifespan to dying in infancy.

The Williamses didn't know what form of EB Jonah had until a few weeks after he was born. He likely has a type of EB called junctional non-Herlitz, a rare form of an already uncommon disorder.

Patients with non-Herlitz EB can live a normal lifespan. Non-Herlitz is considered a moderate type of EB, but people with it face complications such as weak teeth enamel, thinning hair, loss of fingernails and respiratory problems.

Jonah will likely continue to have problems with his skin, too, though he may not have to wear as many bandages.

"It's medically complex care and it's not predictable," said Geri Kelly-Mancuso, the nurse educator for the Dystrophic Epidermolysis Bullosa Research Association of America, or DebRA.

"We can't say, on day 5 you'll look like this, and at age 12, this is what you'll look like. It's hard for the parents to hear."

DebRA estimates that between 25,000 and 50,000 people in the United States have EB. Only a small fraction of them -- less than 1 percent -- have non-Herlitz.

Milder forms may go undiagnosed, Kelly-Mancuso said.

Jonah spent the first month of his life in the neonatal intensive care unit at Brenner Children's Hospital. There were a lot of unknowns then. Because of concern about infection, the Williamses didn't get to hold Jonah until he was 9 days old. And they didn't know how much touch his fragile skin could take.

Dr. Heather Furlong, a neonatologist who cared for Jonah, has watched Jonah's skin improve.

"He has healed his skin on his hands. He has very beautiful new pink skin on his hands and his feet," she said. "When he was first admitted to the hospital, he looked very similar to a burn patient."

Patrice Williams started to pressure the medical staff to discharge him after she became concerned about the chance of him getting an infection in the hospital. But to go home, Patrice and Matt Williams had to learn how to change Jonah's bandages, a process that takes about two hours every evening. They did, and they brought Jonah home to their house in southeastern Winston-Salem on March 30.

But the work has just started. As Matt Williams likes to say, "it will take an army to raise Jonah."

Last week, Jonah was back in the hospital briefly after his parents became concerned about a potential problem with his trachea. Two symptoms of respiratory problems are hoarse cries and a stridor, a high-pitched sound that indicates airway obstruction.

Furlong is optimistic about Jonah's outlook, but the Williamses know they still have a lot of uncertainty ahead -- including how the outside world will look at Jonah as he grows up and starts school.

They know that kids can be cruel, and they know their job as parents will be to help him be confident and make his own decisions. But they must also weigh the consequences of rites of passage that wouldn't be a big deal for most kids, such as riding a bike and dealing with blisters that may come with it.

"I think it's our plan not to hold him back," Matt said. "If he wants to try to ride a bike, he can try to ride a bike."

"He's not playing football," Patrice Williams joked. "Non-negotiable."

Patrice Williams uses her blog to chronicle the ups and downs and the roller-coaster living that is part of her life as a new parent. It's how she became connected to other people dealing with EB, too, particularly one mother in Illinois who has a young daughter with non-Herlitz EB. She's been the most helpful with advice, the Williamses said.

And with a sharp sense of humor and strong faith, it's how Patrice Williams deals with things they don't know, even if the blog is just a place to unload her worries. With each post, she's regularly attracting from 50 to 90 comments from readers. She takes solace in many of them.

"Some days I can find humor and look at the bright side, and some days you feel like crap, and I want to show that, too," she said. "Day-to-day you feel different. Sometimes you just need people to say, ‘It's OK to be pissed off.'"

Caring for Jonah right now means a lot of work and not a lot of rest -- above and beyond the usual newborn life of new parents. In between feedings and diaper and dressing changes, the Williamses count on their family -- many who live near -- and their church for help. Their church, Pinedale Christian, is finishing the family's basement so that they have a place set aside just for Jonah's dressing changes. Somewhere among all her EB reading, Patrice read that it's best not to change dressings in a child's bedroom. That room needs to remain a place that is safe, peaceful and not associated with pain.

For now, Jonah gets cleaned, re-wrapped and re-swaddled on the dining-room table. A corner of the room has the clutter of a medical clinic, with drawers and boxes of scissors, gauze, ointments and Aquaphor, a slick petroleum-jelly-like cream that helps protect Jonah's skin. It gets slathered on everything, including his pacifiers. The bandages are around his arms, legs and torso, and if they're not there to protect his skin from blistering, they're there to promote healing of existing wounds.

Life with Jonah prompted the Williamses to invent a new word -- "to frict," the verb form of friction, as in "You can't frict Jonah."

Life with Jonah means seemingly small notions, such as what fabrics are soft, have changed. Typical baby clothing, with its cuddly appliqués and prints, aren't safe for him to wear. They can damage his skin.

"I have a whole nursery full of stuff upstairs … the cutest outfits that Jonah will never wear because they have seams, they have tags, they have collars, they have elastic," Patrice Williams said, sitting in the family's living room on a recent evening.

Matt Williams sat on a couch across the room, feeding Jonah, his lap full of baby, blankets and a bottle.

"Are you getting this down?" he asked Jonah. "You're pretty much the cutest thing I've seen ever."

■ Laura Giovanelli can be reached at 727-7302 or at lgiovanelli@wsjournal.com.

■ You can read Patrice Williams' blog at www.patriceandmattwilliams.blogspot.com.