Woman battles rare disease, tries to help others

By SAMANTHA FRANK

Palm Beach Post Staff Writer

Thursday, August 20, 2009

Lymphangiomatosis. If the word looks foreign to you, you're not alone. Many doctors don't even know what it is, nor have they agreed upon a simple definition.

But for suburban Boca Raton resident Jack Kelly and his 35-year-old daughter, Jana Sheets, this rare disease has consumed their lives.

Sheets broke her leg in the same place twice as a young child, which led to some raised eyebrows and extensive testing. But it took doctors five years to settle on a diagnosis of lymphangiomatosis - a rare disease in which many tumors or cysts grow in the lymphatic system of the body. It often affects infants and children, most of whom don't reach the age of 20, Kelly said.

The family immediately began researching the disease, only to find that there wasn't much information out there.

Before one of Sheets' many operations, Kelly remembers a doctor saying to his daughter, "You know more about your disease than I do."

In the mid-'90s, Sheets moved to Delray Beach while she was recovering from an arm surgery related to the disease.

"I just absolutely fell in love with the area," she said.

She got a job in retail and spent her weekends at the beach. She was managing with her disease until fluid began to accumulate between the lining of her lungs and chest wall. From there, her lungs began to fail.

In August 2003, she married her husband, Eric, and by December, she found out she was accepted as a double lung transplant patient at Duke University Medical Center.

They moved to North Carolina to be near the hospital, and in May 2004, doctors performed the surgery. Sheets was the first documented double lung transplant patient with lymphangiomatosis.

During her lengthy recovery, she met other transplant patients, mostly who suffered from cystic fibrosis, and she saw the amount of support they got from outside organizations. It was then that she began reaching out to others with her disease to try to create a similar support network.

In July 2007, she founded the not-for-profit Lymphangiomatosis and Gorham's Disease Alliance. Although the transplant has left her weak and tired and she must be on oxygen 24/7, her work for the organization has kept her going, along with the support of her family.

Her father is retired and spends his days doing what he can as president of the organization. He attends medical conferences, takes continuing education classes and is constantly doing some type of research.

"I'm sometimes mistaken for a doctor," he said.

With hit shows like House and Grey's Anatomy, many rare medical conditions are getting some time in the spotlight. That day has come for lymphangiomatosis. The disease is being featured on Monday night's episode of Mystery Diagnosis on the Discovery Health Channel.

"It's a major accomplishment," Kelly said. "It's hard to get interest in the disease, but this might help."

Sheets said it's a step in the right direction, although they have a long way to go.

"We're trying to make sure that no other patients have to go through living life the way I had to, with no information," she said.