October 6, 2010
It takes about five hours.
Then Charlie, swaddled in gauze, sits in the elevated tub.
“Once he’s in there, we start taking things off,” Trisha Knuth said.
Caring for her 4-year-old son is a full-time job for Knuth. Charlie has the genetic skin disease epidermolysis bullosa, better known as EB. The state Medicaid office recently refused to pay for a stem cell transplant that could save Charlie’s life, sparking community outrage and drawing the ire of some Fox Valley lawmakers.
Charlie was born missing the gene that binds skin together. Charlie’s skin is as fragile as a ripe peach, gingerly falling away. Internal and external blistering could one day claim his life.
To protect Charlie, his mother layers him in bandages. It is, quite literally, his second skin.
Every other day, she removes his dressings, baths him and applies a fresh coat of gauze.
“It’s extremely painful and it takes a long time,” she said. “When you put those two things together with a 4-year-old, it is a nightmare. As bandages come off and he’s no longer protected, he’ll start ripping his skin off as we’re trying to get him clean.”
There is no cure for EB, but doctors hope a bone marrow stem cell transplant will make Charlie’s skin stronger, potentially saving his life.
The state Department of Health Services initially denied Trisha and Kevin Knuths’ request for Medicaid coverage of the treatment, which could cost $1 million.
But after Trisha Knuth turned to Fox Valley officials for help, health services decided to review the denial. The review could take weeks.
Trisha Knuth called the treatment “medically necessary.”
“If Charlie doesn’t have something done, he will die,” she said.
Fewer than 1,000 people in the U.S. have EB. It is difficult to determine an exact number, because people with mild forms of EB sometimes mistake it for other skin disorders, which delays proper diagnosis for years.
“If it’s very mild, you’ll have flaking of the skin or blisters, but not as severe as some patients whose skin completely sheers off,” said Maxine Silent, development manager for the Dystrophic Epidermolysis Bullosa Research Association of America.
Silent said that while many people with EB don’t live to see adulthood, the oldest known EB patient is in her 70s.
The Knuths hope the stem cell treatment will help Charlie overcome difficult odds as well.
“Everyone says he’s going to beat this because he’s such a fighter,” Trisha Knuth said. “Charlie’s skin may be fragile, but he’s tough as nails.”
Abandoned at birth, Charlie came home with the Knuths from a Milwaukee hospital when he was 2 weeks old. His adoption was finalized a year later.
Charlie’s twin 15-year-old brothers, Alex and Hunter, and his 7-year-old sister, Chloe, help look after him.
“Sometimes Chloe can handle him better than I can,” Trisha Knuth said.
Charlie recently started 4-year-old kindergarten at Sunrise Elementary School, which Chloe also attends. A personal aide makes sure Charlie doesn’t hurt himself.
“If he were to fall down and catch himself with his hands, he would take all the skin off his palms,” Trisha Knuth said.
Charlie’s classmates have embraced him. Often, Knuth said, it is adults who don’t know how to handle him.
EB research association representative Silent said attitudes and curiosities will change once awareness grows.
“A lot of people don’t know how to treat people who have EB or don’t know what EB is,” Silent said. She hopes EB Awareness Week, which occurs the last week in October, will bring EB out of the shadows.
“We need people to learn about the disease and spread awareness,” she said.
Michael Louis Vinson: 920-993-1000, ext. 368, or firstname.lastname@example.org; on Twitter @MichaelVinson