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Blood drive to spotlight little-known disease
When my old friend Eddie Conley called Monday morning, I was glad to hear his voice. We go back more than three decades, to our student days at Western Carolina University, and hearing him on the phone brought back echoes of the Tilley Creek Ramblers and Cullowhee jam sessions.
But then Eddie told me why he was calling. He needed to get something in the paper, he said, about a Red Cross blood drive his wife Debbie is spearheading in Cashiers on Sunday, March 20. The reason for the event is their 16-year-old daughter, Shandy, was diagnosed with paroxysmal nocturnal hemoglobinuria last summer.
“Shandy’s needed a lot of blood,” Eddie said. “So far she’s received 23 units of red blood and 14 of platelets. We just want to do our part to give back to the Red Cross.”
Blood donated at the Cashiers Community Center will not be specifically for Shandy, her dad said.
“That’s just not feasible,” Eddie said. “We never know when she’ll need it, and blood has a short life span.”
Instead of collecting blood for Shandy, the March 20 event is being held in Shandy’s honor and is part of an ongoing effort by Eddie and Debbie to focus attention on their daughter’s rare disease.
“PNH needs some attention,” Eddie said Tuesday from Asheville, where Shandy was to have a shunt put in that will make blood transfusions easier. “There’s almost no research being done even though it’s becoming more common.”
According to Eddie, PNH was discovered and observed in the late 19th century, but a cause was never isolated and a cure never found.
And that remains the case today.
“It’s an acquired genetic malfunction,” Eddie said. “They know exactly which gene causes it, but they don’t know how to fix it.”
Shandy was an active 15-year-old academic achiever and volleyball player when the disease struck in June. She was attending a youth leadership conference at N.C. State when she collapsed with what doctors first thought was a heat stroke. At the hospital, her anxious parents gave the go-ahead for a blood test and were told an hour later that it was likely leukemia or lymphoma. After a bone marrow biopsy the next morning, they were first relieved to learn Shandy didn’t have cancer and then devastated by the news that what she did have was bone marrow failure due to PNH.
Eddie, an electrician, explained Shandy’s condition in layman’s terms.
It seems that in a healthy body, blood cells created in bone marrow have a genetic code that causes them to secrete an enzyme that works like glue to attract and fasten a special protein to the cell. Once fastened, the protein warns off the body’s immune system, in effect saying: “Attention: this cell is ours. It’s a good guy – do not attack!”
But in Shandy’s case, this system broke down. The glue needed to attach the protein is not present and her body’s own immune system destroys her blood cells, believing it has done its job by finding and eliminating a foreign object, just as it would attack a virus or germ.
“Simply put, the PNH individual is literally killing its own red blood cells and inhibiting white blood cell and blood platelet production,” Eddie said. “Her system’s eating its own blood, and doctors don’t know what causes it or how to make it go away.”
Shandy’s been able to keep up with her classes, but she had to give up volleyball, her dad said.
“Team sports are a no-no,” Eddie said. “She’s at risk both from free bleeding and from blood clots – there’s no way to regulate it.”
Shandy’s attitude is good, and all three Conleys remain hopeful, Eddie said.
“In PNH patients who live long enough, 30 to 40 percent recover,” he said. “The body starts working again and the disease goes away.”
Eddie says he and Debbie will continue to be involved in events like the March 20 blood drive to raise awareness of PNH, a disease with victims as young as 2 and as old as 80.
A longtime musician, Eddie plans to have a number of his fellow pickers drop by to liven up the afternoon for those who turn out to donate blood. He doesn’t have a formal program, but said he expects local favorites like Bill Fisher, Jim Yoder, C.W. Stewart and John Warren to be among those sharing their talents.
The Red Cross target for the Cashiers drive is 50 pints, Eddie said, and they’ll be set up to accommodate four donors every 15 minutes between 1 and 5 p.m.
Eddie and Debbie are requesting that potential donors make an appointment to assist Red Cross personnel with scheduling. To reserve a slot, call the Conleys at 743-2766.
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