Son’s rare genetic condition makes every day a blessing for family

Patrice Williams smiles at her son, Jonah, as she begins the process of removing his bandages. (Andrew Dye/Journal)

Patrice Williams smiles at her son, Jonah, as she begins the process of removing his bandages. (Andrew Dye/Journal)

WINSTON-SALEM, N.C. — Patrice Williams was going to dress her son Jonah in his “handsome shirt,” the one with the collar, for the Mother’s Day breakfast at his preschool, but the 5-year-old had a different idea. He wanted to wear his T-shirt with the word “mom” in a heart.

“Who can argue with that?” she asked.

Not Patrice.

She doesn’t know how many more Mother’s Days she’ll have with Jonah. When he was born in 2009, she wasn’t sure she’d get this one.

Jonah has epidermolysis bullosa, a genetic condition that separates the top layer of his skin from the bottom. Occurring in one in 20,000 live births in the United States, it makes the skin so fragile that even the slightest touch can cause severe blistering.

So every other day, Patrice and Matt Williams have to change the dressings that cover most of their son’s body.

On Thursday, the process went pretty smoothly.

Jonah had his favorite new toy truck in his hands — bought for him by Granny that afternoon — and his eyes were fixed on the portable DVD player in his basement.

He was watching “Frozen” again, and after who-knows-how-many times still couldn’t be bothered to look away.

His parents welcome the distraction. Matt Williams patches and wraps Jonah’s thin arms. Patrice Williams does the same on her son’s legs. His entire body, thin for his age, is marked with red blisters that range in size from a pencil eraser to the palm of a hand. The worst ones are open, raw and bloody.

The wraps that cover his arms, legs and torso help prevent new wounds, cover current blisters and protect him from infection. Every other day the wraps come off, Jonah gets a thorough but gentle bath, and new bandages, wraps and stockings go on. The entire process takes about 90 minutes, and when it’s done Jonah’s only exposed skin is on his hands, neck and face.

Shared sacrifice

Some days are better than others.

“Frozen” days are better than most.

Jonah only really looks away from the movie once, when Patrice reaches a new blister that needs to be cut, drained and patched. It’s painful, and Jonah winces.

“Stop it mommy,” he says, trying to turn away while tears crop up in big blue eyes.

“I know, I know, I know,” Patrice says, working quickly and efficiently while Matt keeps him still. “I’m sorry.”

Patrice and Matt know their roles, have their tasks and get them done. During the day, Patrice blends foods for Jonah’s meals — which he receives through a feeding tube — and precuts bandages for dressing change. When it’s time for dinner, Matt blends the meals that get pushed into Jonah’s tube one ounce at a time. Patrice gets Jonah’s old bandages off and him into the bath. They do the new bandaging together.

Amid the controlled chaos that is any house with two young children — Matt and Patrice have a 20-month-old foster son, too — the process of caring for Jonah’s special needs seems to run like a well-oiled machine. Patrice, humble and grounded, says it’s with time, practice, a lot of help and even more prayer, though, that they’ve gotten to this point.

“Obviously, it’s hard,” Patrice said. “We have our tasks and by 10, maybe get to sit down and watch a show together.”

‘Nobody knew what it was’

Matt and Patrice went to West Forsyth High School together. She was studious; he was a clown. On their senior trip, those differences fell away — or at least quit mattering enough to be in the way. They graduated from East Carolina University in 2004 and were married the same month. Patrice, a teacher at the time, became pregnant with their first child in 2008. Everything was falling into place.

Until it fell apart.

Their first son, Gabe, was stillborn. When he came out, the skin peeled back on his hands and feet.

“They said it was ‘one of those things,’” Patrice remembers.

They did some general tests, but no one thought to look for EB because most people aren’t even aware of the condition. Patrice explained, you don’t find it if you’re not looking for it. And no one knew to look for it until Jonah was born the next year.

He was born completely raw from the elbows down and knees down.

“Everybody was standing around him,” Patrice said. “Nobody knew what it was.”

Jonah was transferred to Brenner Children’s Hospital within hours. Patrice was still at Forsyth Medical Center, where one of the older doctors said he thought Jonah had something called epidermolysis bullosa.

“I remember Matt calling me and he said, ‘It is EB, there’s no cure, he might die,’” she said.

Jonah spent 32 days in the neonatal intensive care unit. They didn’t get to hold him for the first nine.

“I don’t think (the doctors) thought he’d ever come home,” Patrice says now, looking back at photos from that first month in the hospital, Jonah’s tiny body wrapped like a cocoon. “He was so raw.”

Jonah looks right past all the bandages.

“Look at this cutie patootie,” he says, standing at his mom’s shoulder.

“Failure wasn’t an option,” Matt said. “We weren’t going to lose another one.”

So they learned everything they could about EB. Nurses showed them how to wrap their infant in the bandages that might help him survive, and Jonah came home. The first year was an adrenaline-filled blur, Patrice said. Everyone was just trying to figure out how to survive.

“But we didn’t want to just survive,” Patrice said. “We wanted to thrive.”

Settling into ‘normal’

They moved to changing Jonah’s bandages just four times a week instead of every day. After his feeding tube was put in, he was able to keep more food down and the family settled into a groove. Finally, after about three years, Matt, Patrice and Jonah settled into their “normal.”

And then things turned upside down again, or maybe right side up.

“Our hearts were called to adoption,” Patrice said.

They wanted to add to their family, but with a 25 percent chance of another child being born with EB, it was a risk they weren’t willing to take. Patrice said they had initially planned on adopting older children.

And then one night they got a call that a baby had just been born and needed a foster home. They’ve had him since day two.

Patrice said she was afraid to rock the boat after they finally felt like they had reached a stable place with Jonah.

“I was nervous about adding to our family,” Patrice said. “But it’s been such a wonderful improvement on all of our lives in some ways.”

And Jonah loves being a big brother.

“This is the big brother room,” Jonah says, showing off his bunk beds and dark blue walls decorated with train memorabilia.

Their foster son’s case has gone to adoption but hasn’t been finalized. At 20 months old, he’s a bouncing ball of energy — giggling, smiling and shouting as he bobs around Deac, the family dog.

Independent, adventurous

Jonah will always need special attention and care, but otherwise the family leads a pretty typical life.

Their walls are lined with family photos; the dining table is half race track, half dinner spot.

Matt works as an accountant, where the salary is a “bonus.”

“He works for the insurance,” Patrice said. She’s half-joking, but Jonah’s bandages alone would cost $7,000 a month without it.

Patrice stays home with the boys and takes Jonah to preschool four days a week. They knew he was ready for preschool and found one that was half a day, so they wouldn’t have to worry about feeding him through his tube at school.

“I cried my eyes out,” she said. “I think all moms feel connected to their kids, but I had been his everything for four years.

“Now, he does things I never even thought he would try.”

When he started at Fountain of Life last fall he was cautious, his teachers remember. He would hang back, stay out of the middle of the pack when the boys are running wild.

Just a couple weeks from graduation now, Jonah’s racing around the mulch playground. He won’t go down the tall slide, but he likes the short one. Arms in the air, he will slide down over and over.

But this time of year it’s starting to get too hot. Spring is “Jonah months.” Wrapped in bandages, he overheats quickly in the summer and has to leave the playground earlier than the other kids. It’s equally hard for Jonah and the teachers who have to coax him into the air conditioning. But having Jonah has been a blessing, says Debbie Travis, the preschool director.

“He’s such a sweet little boy,” Travis said. “The other children never look at him any different.”

Preschool has made him more independent, more adventurous. Where he used to gag at the sight of food, he shares snacks with his classmates each day and learned he loves M&Ms. Patrice calls it “positive peer pressure.”

Next year, Jonah will start kindergarten at Redeemer School. He will have just half days through second grade, which makes Patrice happy.

But Jonah is nervous.

“They’ll ask about my boo-boos,” he says about the other kids.

“And you’ll tell them,” Patrice says, comforting her son.

“I don’t want to tell them,” he counters. “I’m shy.”

“Then I’ll tell them,” she says.

‘Live more in hope’

Patrice and Matt know he’ll do fine.

They want Jonah to have as normal a childhood as he can.

And for the most part, he does. He plays with toy cars, blows bubbles and has lightsaber battles with his dad.

He has multiple girlfriends at school.

“It’s complicated,” he explains, matter-of-factly.

He fights with his little brother and is learning to love Wake Forest basketball. Matt took him to seven games last season — Jonah’s first since he was a toddler. It took several years to get past the memories (or nightmare) of that attempt.

“He hurled everywhere during the starting lineup,” Matt said, laughing about it now.

But the ticket is still in Jonah’s scrapbook.

And like every little boy, he dreams.

He wants to be a train conductor.

“At Disney World,” Jonah says.

At the same turn, Matt and Patrice are honest with Jonah. They have to be — at 5, he’s been through more than most. And right now, there is no cure for EB.

There’s research, but a rare disease like EB doesn’t get a lot of attention or financing. Saturday was the third annual “Jogging for Jonah,” a 5k and fun run that the family hosts to raise awareness for EB and money for DebRA — a nonprofit dedicated to EB research and support services. The event raised $15,000 each of the last two years. Patrice’s goal for this year’s was $20,000.

Jonah hasn’t been prone to infection, as many other EB children are. It’s a constant danger, but they’re careful. He hasn’t been in the hospital since he had the surgery to insert his feeding tube at 14 months old. Nutrition is important for Jonah, too. Because his skin in constantly regenerating, his body is always in a state of healing and fighting infection and he requires more energy. He tires easily.

Right now, Jonah still requires a lot of help. He can’t get dressed or go to the bathroom by himself. But Matt says he thinks Jonah will be able do to those things on his own one day. Skin cancer is common in EB patients, which reduces life expectancy to somewhere between 15 and 35.

The severity of EB and the prognosis depends on the type. Matt and Patrice like to say that Jonah is “one in 2 million” because he has Junctional EB, which accounts for just about one percent of EB cases. Jonah has two different gene mutations, though, that have never been documented together. So really, he’s unique.

“We live more in hope than in fear,” Patrice said. “We hope for a cure, we pray for a cure, but we can’t promise him a cure. He knows he won’t have boo-boos in heaven.”

By Arika Herron/Winston-Salem Journal

1 Comment

  • pattym

    God Bless you all and keep you through all of this..I LOVE the attitude you have had as parents in letting him be a boy as much as possible. I know that he and little brother LOVE each other too, that is so precious.

Comments are closed.

Follow

Get every new post delivered to your Inbox.

Join 30,149 other followers