Family

WHITE PLAINS — Payton Thornton tries to ignore the pain, closing his eyes as if to wish it away.

Still it lingers — the blisters, scars and scabs all serving as fresh reminders of what was and what will be again.

But for all the pain and tears, the fury and frustration that a lifetime of hurt has brought, there's little that Joy Thornton can do to help her son. She can't hug it away or kiss it away — that would only make it worse.

"As a mother, I felt so helpless," Joy says, her voice soft and fragile as cracked glass. "Here was my child, my baby, and there was nothing that I could do to make him better."

Even when there is no pain, the fear looms heavy like the sound of distant thunder … always threatening. The slightest touch, a fall, an accidental bump and it returns with a vengeance.

That's a tough way for a 3-year-old boy to live. But that is exactly what Payton is … tough.

Diagnosed with a rare genetic skin disorder, Payton has struggled since the day he was born. Rather than being wrapped in soft blankets, he was swaddled in bandages and the medicinal lotions that served as armor against a barrage of lethal infections.

As his mother, Joy Thornton can take nothing for granted, given that something as simple as a Cheerio could rip her son's esophagus and lead to yet another trip to the Birmingham hospital where he was born.

For Payton, reality is 18-gauge needles and four-hour dressing changes. It's feeding tubes and wounds that won't heal. It's itching scabs and stares from strangers.

But pain isn't all that Payton Thornton knows. He also knows hope.

There are whispers of a cure, of a bone marrow transplant and a doctor in Minnesota who could help Payton, and others like him, grow normal skin.

"New blood," Payton says, his eyes brightening at the thought of a life he's never known. "New blood and Jesus are gonna make my boo-boos go away."

Promises to keep

Joy Thornton was 37 weeks pregnant when she went into labor at St. Vincent's Hospital in Birmingham. Payton was born at 8:03 p.m., but even before she saw her baby, Joy knew something was wrong.

"When he came out, there was this cry but nothing like the cry of a baby coming into the world," she says. "It was a scream, a painful, hurt scream."

On Payton's left foot, the skin stopped just above the ankle revealing muscle, blood vessels and bone.

"The skin was just gone," Joy says.

She was only allowed to hold her baby for a moment before Payton was taken by doctors and nurses all scrambling to find out what was wrong. The next time Joy saw her son he was bandaged from head to toe and laying in an incubator in the Intensive Care Unit. The only thing exposed was his tiny red torso.

"It was horrific," Joy remembers. "It's like he'd been burned … dipped in scalding hot water."

It wasn't until days later, after meeting with a team of doctors, that Joy first heard the term Epidermolysis bullosa — "EB" for short — a rare genetic skin disorder affecting about 1 in 50,000 births.

Children with EB are missing collagen VII, which serves as the glue affixing the top layer of skin to the next.

Because they lack this protein, the slightest friction can cause blisters, large areas of the body are often devoid of skin, fingers and toes can become fused and immobile, scarring occurs on exposed skin surfaces and mucous membranes. Those with EB often have difficulty chewing and swallowing.

There is no prenatal test to detect carriers for EB. Parents learn they are carriers only after their child is born. Despite being a registered nurse working at Regional Medical Center in Anniston, Joy had never heard of EB.

"Is there a cure?" was her first question.

"No," was all anyone could say.

For the first two weeks of his life, Joy wasn't allowed to hold her baby. To pick Payton up, to cup her hands around the base of his neck or to lift his legs and change a diaper could rip his skin. When she carried him, it was on a pillow.

Payton remained in ICU where Joy sat beside his incubator in a rocking chair and read him Bible stories.

"I promised Payton that I'd do whatever it took to make him better," she says. "And I'm going to keep that promise … no mater what."

Joy was released and allowed to go home without knowing the severity of Payton's illness. She made the trip back and fourth to St. Vincent's every day, waiting to learn what course Payton's life would take.

Finally, the call came. At St. Vincent's, Joy and her husband, Reid, were taken into a small room filled with doctors and specialists, where they were told that Payton had recessive dystrophic epidermolysis bullosa — the most severe and aggressive form of EB, which affects between 2,000 and 3,000 Americans.

He wasn't expected to survive past his early 20s, and it would be a life marred with intense, debilitating pain, isolation, disfigurement and deformity.

The doctors had little advice other than to take Payton home, love him and make him as comfortable as possible.

"I went blank," Joy says, glancing down at Payton sitting in a child-sized chair at her feet. "They were telling me that my child's going to die and that his whole life is going to be spent in pain."

Staring up at the TV, halfway watching an episode of "Dora the Explorer," Payton cuts his eyes up as his mom, listening and grinning whenever his name is mentioned as if silently reveling in having already proven so many people wrong.

Among the greatest risks for children with EB are staph infections and skin cancer. Localized skin cell tumors can grow faster and spread to other areas of the body more rapidly than they would on a less-compromised individual. Swallowing is also a huge concern. Because of the damage to his mucus membranes, anything rougher than baby food can easily tear Payton's esophagus.

Payton needed morphine to endure the four hours it took to give him a bath and change his multiple layers of bandages.

"He would scream so much, he'd be absolutely exhausted," Joy says. "When the morphine took effect, he'd sleep for six to eight hours."

That was three years ago and little has changed — save for the fact that everything has changed.

Payton still lives in constant pain. The slightest touch can cause blood-filled blisters the size of water balloons, which Joy has to pop with an 18-gauge needle. He eats through a feeding tube, can't stand to be outside in the heat and takes upward of 12 medications a day.

"He can't do a lot of the things that other kids can, and he knows it," Joy says. "He'd rather sit back and watch the other kids play, long as he can be there."

'Careful affection'

Bored with "Dora the Explorer," Payton's eyes follow his older brother, Parker, who's headed toward his room. Quicker than anyone might expect, Payton's up and lumbering after Parker, who's already shut the door.

Payton doesn't hesitate. Stretching out as far as his bandages will allow Payton wraps his tiny fingers around the brass doorknob and pushes it open.

He moves slowly, methodically — like the world's oldest 3-year-old — practiced in the art of not falling down. Because his toes are fused together, balance is a problem. A year ago, walking from one room to the next would have been almost impossible.

It's a credit to Ginger Roberts, the only physical therapist who could reach him.

"For everyone else … it was a bloodbath," Joy says, watching as Payton walks into Parker's room. And yet she doesn't appear the least bit concerned when the door closes behind him. "Mrs. Ginger is the only one who wouldn't give up."

Ginger Roberts is a physical therapist and facility director at Champion Sports Medicine & Rehabilitation Center in Oxford. Before she met Payton, Roberts had never heard of EB.

At their first session, Payton couldn't stand or walk on his own. All he would do was sit in Joy's lap. Steadily, Mrs. Ginger coaxed him out.

"We'd play games. I'd bribe him with candy. Whatever it took to get him moving," Roberts says. "And now … it's a miracle."

It was six months before Payton stood without holding someone's hand. Then he took a step, then another and then another.

Today, he's chasing Parker around the living room, squaring off against his older brother like they're pro wrestlers inside an invisible ring.

Payton goes to therapy three times a week where most of his sessions involve stretching — every finger, every toe, every joint and muscle. Because of the blisters and scarring, Payton's joints contract and without proper stretching exercises, both at home and with Mrs. Ginger, he wouldn't be able to move.

But it hasn't been easy.

"Some days, he comes in ready to work," Roberts says. "But some days, he comes in mad — mad at me, mad at his mom, mad at the world — and nothing I do or say can break through."

For all the fits and accomplishments, Payton holds a special place in Mrs. Ginger's heart. She's made a lifetime out of working with the patients no one else could reach, but to watch and work with a child having to overcome so much is hard even for a seasoned professional.

"To me, he's like a sad little boy living in a bubble, wanting to get out but knowing he can't," Roberts says after a long, contemplative silence. "There's so much joy and love inside him, but because of all that pain … it's trapped just like he is."

But don't feel too sorry for Payton.

For all the promises made by his mother and the father that practically lets him get away with murder, Payton leads as normal a life as possible. He loves being outside — as long as the sun's not too bright and hot. He plays, has friends, jumps on the bed, rides around in the golf cart with the wind in his face, goes to the beach every October and wanders out in the woods alongside his Dad.

"We hold and hug him a lot," Joy says. "He's not lacking attention or affection. It's just careful affection."

Out in public when the stares from strangers drift his way and questions about burns creep into conversations, Payton never has to defend himself. He's got Parker for that.

If somebody dares ask, what's wrong with his little brother, Parker's likely to look them in the eye and answer, "Don't you know? He's got EB."

Hope for a cure

It was well before sunrise one morning in November when Joy came home, having worked second shift at RMC, and started reading some newspaper postings on an EB Web site.

One story was so incredible she had to read it twice.

It was about Dr. John Wagner, a hematologist at the University of Minnesota Medical School, who performed a bone marrow transplant on a toddler from New Jersey, named Nate Liao.

The boy suffered from the same skin disorder as Payton. And seven months after the experimental procedure, the stem cells from transplanted bone marrow helped Nate's body produce collagen VII.

"Every now and then," Dr. Wagner said in an interview with USA Today, which broke the initial story, "you really feel like you've done something great."

It was just the kind of news that Joy had been praying for — a cure.

The 2-year-old in the USA Today article, who also had an older brother with the same rare disorder, had, in a matter of months, gone from eating baby food to Oreos and replaced bandages with regular Hanes T-shirts.

Soon after the story ran, hundreds of families from around the world contacted Wagner's team in hopes of their children receiving the same treatment. Joy was quickly among them.

"I was just so aggressive and persistent," she says, almost apologetically. "I wanted something done for my son, not even a cure but something to help ease his suffering.

"I want Payton to have the chance to live a normal life. … to go to college, get married and grow old like everybody else."

But getting on the transplant list is no guarantee. Dr. Wagner has been honest about the risks. "There will be deaths," he said.

The procedure is dangerous because, like all who receive bone marrow transplants, these patients will have to get intense doses of chemotherapy to wipe out their immune systems. Children with EB are particularly vulnerable because their entire body is essentially an open wound, making them acutely susceptible to infection.

To be placed on the transplant list, Payton first had to meet with the team in Minnesota just to see if he'd be accepted. After months of writing letters, e-mails and making phone calls, Joy got word that the team wanted to meet Payton.

They leave for Minnesota on Nov. 5.

Still, there are a number of obstacles in the way. Unlike Nate Liao, no one in Payton's family was a 100 percent bone marrow match. So before he can even get the transplant, Payton will need to find the right donor.

Then there's the procedure, which will $500,000. And because it's experimental, there's a strong chance it won't be covered by insurance.

But those are worries for another day. When he boards the plane, Payton will be one step closer to getting the "new blood," that could take all his pain away.

"I can finally catch my breath," Joy says, watching as Payton carefully sits back down in front of the TV. "There is hope. Who knows what happens next, but at least Payton's got a chance for a normal life.

"And that's all anyone can ask for."

For more information:

• To learn more about EB, visit the Dystrophic Epidermolysis Bullosa Research Association of America at www.debra.org

• To learn more about Payton Thornton and his hopes for a transplant, e-mail Joy Thornton at joyrose@cableone.net