WALLINGFORD - For years, Danielle O'Keefe's family tried to live a normal life. But that just didn't work out, so now they're trying something different.

"We've decided to be a little abnormal for a while," said John O'Keefe, Danielle's father.

Twelve-year-old Danielle suffers from an extremely rare skin disease called epidermolysis bullosa, which affects about 100,000 Americans, mostly children. The genetic disease, which has no cure, renders the skin so fragile that even slight contact can lead to painful irritation, bleeding and blistering.

At any given time, estimates Danielle's mother, Jennifer O'Keefe, Danielle has from 50 to 100 separate sores on her body. She also has to be careful about what she eats, because the affliction affects her on the inside as well.

Danielle "has the heart of an athlete," says her mother. She also desperately wants to be a regular kid and desperately wants friends. But if she tries to keep up with her peers at play, she pays heavily for it later, spending the night in tears because of the sores and blisters that result.

Danielle has been hospitalized five times in the last year, for stays of three to five days, because of recurrent infections, and the consequence, generally, of a body constantly facing the task of repairing itself.

There is psychological trauma as well. Danielle has bipolar depression and anxiety, particularly from the fear of death. The fabric of a chair in the family living room is worn from incessant rocking, which Jennifer O'Keefe says is a coping mechanism.

In grammar school, Danielle found it very difficult to make friends, and found other children far from compassionate when it came to her condition. The efforts of school staff just made it worse, Danielle said.

As an example, she recalled a band concert she'd performed poorly in, as a drummer, because she'd missed so much time due to her infections. One of the band members "yelled at me in front of the whole band and they laughed," she recalled. "He said, 'you sucked, and messed the whole band up.' "

Because she missed so much school, Danielle lacked the consistent contact that can lead to friendships, said Jennifer O'Keefe.

"The thing with Danielle is that she wants so much to be accepted," she said. "And as a parent your heart just aches for her."

Now a seventh-grader, Danielle receives home-bound schooling from Moran Middle School, and her parents are pleased with the school's efforts on her behalf. Though Danielle would like to return to the regular classroom at some point, her parents doubt it will happen. The family is turning inward, though there is support from their church congregation, the Wallingford Kingdom Hall of Jehovah's Witnesses.

Rethinking everything

Both John and Jennifer O'Keefe grew up in Wallingford. He went to Sheehan High School, she went to Lyman Hall. Neither has been able to trace any evidence of epidermolysis bullosa in family history. Son Joshua is free of the disease.

An excellent student, Joshua left Sheehan High and is now pursuing his high school diploma through the American School of Correspondence. Jennifer has retrained, in medical billing, so she can work at home. John left his career in mechanical design.

"We just discovered we would have to rethink what we could do as a family," explained Jennifer O'Keefe.

Their experience is not unusual among families affected by epidermolysis bullosa, or EB, as it's often called, said Mary Sprague, executive director of the nonprofit organization DebRa, which stands for the Dystrophic Epidermolysis Bullosa Association. The organization is devoted to supporting families and research.

"This disease manages to devastate families physically, emotionally and financially," said Sprague. "It does affect how you live your life. Everything is complicated."

There has been little encouragement when it comes to searching for a cure. EB actually describes several inherited disorders affecting the skin. Some cases are horrifyingly severe. There are EB babies who do not survive childbirth because the process strips away the skin so severely.

Sprague said that recent research into gene therapy is beginning to show promise.

"But it's still a horrific disease," she said.

In leaving his career so he could stay at home and help take care of Danielle, John O'Keefe returned to a childhood passion, painting. One of his pieces was on display at the Lyme Art Association Summer Show, a remarkable achievement for a relative newcomer.

He'd gone back to painting as a way of dealing with stress, which mirrored the activities of his daughter. Danielle typically stays up and draws, paints or sculpts well into the night. One of her drawings earned first prize at the Durham Fair when she was a fifth-grader. The entire family has reorganized to fit her schedule, staying up until 3 a.m. and sleeping to noon.

The art of living

On a recent afternoon, John O'Keefe pointed to his oil-on-canvas painting titled "The Field's Edge," which he painted in April while Danielle was in the Connecticut Children's Medical Center in Hartford.

"Here's the strange thing," he said, "the sicker she is, the better my paintings come out."

Danielle also took out some of her own art work, including a rendition of a butterfly. The butterfly is significant to those suffering from EB, because the delicacy of its wings is symbolic of their fragility.

Danielle's work is on display until the end of the month at Landmark Gallery & Framing, on North Main Street. Some of the artwork is for sale, with proceeds to benefit EB research.

Galley owner Gillian Zimmerman said she'd never heard of EB until meeting the O'Keefes, and was moved by a family "putting their lives on hold and doing whatever they could to support her."

"So this just seemed like a little thing in comparison," she said.

Danielle's work is also "really good," said Zimmerman. "So the apple doesn't fall far."

Developing a life indoors

The family has not given up on reaching out. A year ago, Danielle went to New York City for the start of the "Walk a Mile in My Shoes" relay, and in the summer was part of a photo shoot for the campaign. She was also featured in a slide show presentation shown to DebRA chapters during a recent meeting in Belgium.

She would also like the opportunity to talk to teachers and students at her school about the condition, and try raise money for research.

"I've seen Danielle desperate for people to understand," said John O'Keefe. "She thought that making them understand would make them like her, but that hasn't happened. She feels so much on the outside, I think."

Some of Danielle's tastes are typical of a young girl: "Hannah Montana" and "High School Musical." Others are more on the idiosyncratic side. She likes "CSI: Miami" and baseball, for example. She also likes to sing. "Anything but classical," she said.

The basement of the family's Summerhill Drive home is devoted to the youngsters. Danielle's room was recently outfitted with a jungle-themed mural. The room is not far from a closet stocked with bandages and other medical supplies needed for her care.

"Because we're inside so much, we've just kind of developed a life in here," said John O'Keefe.

DebRA is online at www.debra.org. The Epidermolysis Medical Research Foundation is at www.ebkids.org.

jkurz@record-journal.com

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