After spending a month recovering from a bone marrow transplant to cure his sickle-cell anemia, 5-year-old Luke DeBoer went home last weekend with a healthy, fully functioning blood system.
"He's back at home and seems to be in good health," said Dr. John Levine, assistant professor of pediatrics and communicable diseases, who performed the transplant at the University Medical Center. "He passed all of his tests prior to discharge."
Before his transplant, DeBoer received blood transfusions to treat the disease. Now he won't be needing further transfusions, since the sickle cell anemia will never return, Levine said.
The bone marrow transplant was the first ever in Michigan to be performed specifically to treat sickle-cell anemia. University Hospitals is among 15 centers nationwide that have performed the procedure.
"He didn't have any serious complications - it went very smoothly," Levine said.
Bone marrow transplants were first performed in the late 1960s and are used mainly to treat leukemia. But during the past few years, bone marrow transplants have been used more frequently to treat other blood diseases, such as sickle-cell anemia.
"In the last decade there has been an explosion of transplants done, and as transplants have become more common, the occasions for transplants have broadened," Levine said.
Sickle-cell anemia is an inherited blood disease that occurs mainly in blacks. The disease arises when the hemoglobin in red blood cells lack oxygen. This abnormal hemoglobin causes the red blood cells to harden, distorting them into fragile, sickle-like shapes. The sickle cells can then become clogged in blood vessels, causing severe pain in the abdomen, bone and muscles.
Dr. Harry Erba, assistant professor of internal medicine, said that when the blood vessels become clogged by the sickle cells, organs may die because they do not receive enough oxygen.
"Sickle cells do not flow easily through tiny blood vessels," Erba said. "This can cause death in organs that are supplied by that blood."
While sickle-cell anemia can be treated with antibiotics and blood transfusions, a bone marrow transplant is the only way of curing the disease. Erba said he expects the number of bone marrow transplants performed to treat sickle-cell anemia to rise in the future.
"I think we'll be doing more of these," he said. "It is the only potentially curative treatment that I know of."
Erba said that while the gene that makes a person susceptible to sickle-cell anemia can be identified, the potential severity of the disease cannot be determined. This poses the problem of determining whether it is worthwhile to undergo the risks of a bone marrow transplant to prevent future symptoms, which may be mild and treatable.
Erba emphasized the importance of detecting sickle-cell anemia at an early age, and advised tracing young patients for a while to determine if symptoms are severe enough to warrant blood transfusions or a bone marrow transplant.
About one in 10 African Americans carries the genetic trait for sickle-cell anemia. Of those individuals, less than one in 100 will contract the disease.