Bone marrow transplant performed for sickle cell disease

(Republished with permission from the St. Louis Post-Dispatch. This article originally ran in the Health & Fitness section on Monday, September 24, 2007)

By Kay Quinn St. Louis Post-Dispatch

Bone marrow transplants are best known for their potential to cure people with certain types of cancer. But they also hold promise for people with severe cases of sickle cell anemia, also known as sickle cell disease. Now, in a first-of-its-kind procedure, St. Louis Children’s Hospital has performed a bone marrow transplant in a teenager with sickle cell disease, using cells from a donor who is unrelated to him.

“I was nervous,” said Kenize Cathey, 15, who goes by the nickname Ke Ke. “A lot of people came in and prayed, and it was fun.”

Ke Ke underwent the transplant Aug. 22. He is now in isolation in the hospital to ward off infection as his body attempts to graft the donor cells. Facing weeks in a small hospital room has been frustrating at times.

“It’s tough for him,” said Tyneka Brandon, KeKe’s mom. “He gets cranky and upset, but he thinks of the long-term goal.”

The long-term goal is for the donor bone marrow to take over the production of red blood cells in the recipient’s body. The cells are given through an intravenous line.

“If donor cells settle in permanently, they’re cured of their disease,” said Dr. Shalini Shenoy, a bone marrow transplant physician at St. Louis Children’s Hospital, associate professor of pediatrics at Washington University School of Medicine and a faculty member at the Siteman Cancer Center. “Their lifestyle is normalized, and they don’t have to put up with the complications of sickle cell disease anymore.”

Brandon says her son has had some scary episodes due to his illness.

“He had a lot of pain crises,” Brandon said. “He’s had several strokes, but thank God he came through that.”

Sickle cell anemia is an inherited blood disorder that mainly affects African-Americans. It affects hemoglobin, which is a protein found in red blood cells. In people with sickle cell disease, red blood cells are shaped like a crescent moon, so they don’t move easily through the bloodstream. They also die much earlier than normal blood cells.

The disease’s severity varies from patient to patient. Symptoms include a higher risk of stroke, trouble in fighting off infections and complications from poor blood circulation. About 70,000 people in this country have sickle cell disease.

Only about 5 to 10 percent of those would be candidates for a bone marrow transplant.

“Transplants have been most successful in sickle cell disease when they are done in children, so we like our patients to be younger than sort of their teenage years,” Shenoy said.

Patients older than 18 are rarely considered for transplant because of the chances that they’ve already suffered devastating organ damage from their disease. Organ damage can increase the risk of post-transplant complications.

Ke Ke’s family members were tested as potential donors, but none was a good match. An unrelated donor was found to be an excellent match.

Now, Ke Ke’s looking forward to the day he can help others with sickle cell anemia.

“I want to go out and tell the world about my story,” Ke Ke said.

His mother just hopes he will enjoy a better quality of life. “He’ll be pain-free,” Brandon said.

Kay Quinn is a reporter and anchorwoman at KSDK (Channel 5).

Copyright 2007 St. Louis Post-Dispatch, Inc.