Feb 12, 2012 2:27 PM by Sharlee Barriere
A New Iberia, La., teenager says he's missed a lot of life to hospital stays for sickle cell disease. In spite of eight-hour blood transfusions every three weeks, 16-year-old Ollie Green can be stricken at any time with instant, debilitating pain.
On Feb. 24, he's scheduled for a bone marrow transplant - the first time Children's Hospital of New Orleans has given marrow from an unrelated donor to a sickle cell patient.
Ollie's hoping for a cure - an end to the episodes of pain caused when stress or something else makes his red blood cells collapse from disks to crescents that clog small blood vessels. Such crises strike up to four times a month; sometimes he winds up hospitalized for two weeks, according to the hospital.
He often needs morphine to cope with the pain, his mother, Tanya Green, told a hospital spokesman.
Ollie is among patients around the country in tests of a new technique: killing only part of a patient's own bone marrow rather than all of it before a transplant. Doctors from the University of Mississippi and Tulane University medical schools also are or will be taking part in the nationwide study in which Ollie's taking part - one of several trying different combinations of low-dose radiation and immune-suppressing drugs.
The idea is that the lower doses do less harm to the patient, and the donor's and patient's marrow can work together.
Marrow transplants from unrelated donors aren't common.
Although more than 50,000 people have had bone marrow transplants from unrelated donors to treat leukemia, lymphoma, and other diseases, the National Marrow Donor Program says it has matched only about 100 donors for sickle cell patients.
"In general, the donor comes from within the family, but most patients only have a 25 percent chance of finding an appropriate match from a relative," said Dr. Lolie Yu, director of Children's hematology and oncology department and its bone marrow transplant program, and a pediatrics professor at LSU Health Sciences Center New Orleans.
Unrelated donor transplants are still relatively new for sickle cell because there are so few appropriate donors, said Dr. Julie Kanter, a Tulane University medical professor and director of the Sickle Cell Center of Southern Louisiana.
That's a matter of race. Different races have different distributions of antigens, the blood proteins that are used to match donors and recipients. The better the match, the lower the chance that the new immune cells will attack and possibly kill the patient.
Most of the nation's 100,000 sickle cell patients are African-American - one in 500 blacks is born with the condition, which is caused by getting a sickle cell gene from each parent. Among whites, the figure is about one in 58,000, according to the Centers for Disease Control and Prevention.
But African-Americans make up only about 7 percent of the 9.5 million registered U.S. donors.
And, though blood cancers have less stringent requirements for transplants from unrelated donors, those for sickle cell are taken only from "perfect matches" - eight of eight or 10 of 10 antigens.
Unlike sickle cell patients, leukemia and lymphoma patients may have little other chance for long-term survival, said Dr. Willis Navarro, the donor program's medical director for transplant services.
"In sickle cell anemia, though the patient's life is likely to be shortened by the disease, the patient will likely survive for many years without the transplant, though with all the complications and quality of life impairment that sickle cell disease brings," he wrote in an email.
Those complications can include organ damage, infections and stroke. People with sickle cell disease also often are weak and tired from anemia - a shortage of red blood cells, which carry oxygen throughout the body.
But one non-matching antigen will cut about 10 percent from a patient's chances of living for a year after the transplant, Navarro said.
Ollie is to be admitted to Children's Hospital on Wednesday, and will stay until he recovers from the transplant, the hospital said in a news release.
Because the radiation and chemotherapy will suppress his immune system, he'll have to be in isolation.
His parents brought him a mobile phone with face-to-face messaging so he can stay in touch with family and friends.
"It's going to be tough to be in New Orleans and not be part of Carnival," the hospital quoted him as saying, "but I'm looking forward to being finished with sickle cell and being able to play."