Health Watch -- Sickle Cell Survival

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The future looks brighter for children with sickle cell disease, thanks to improved medical treatments.

Thirty years ago, only about half of all children born with sickle cell disease were expected to live to adulthood. This inherited disease affects red blood cells. The cells are rigid and sickle-shaped, which sometimes causes them to block blood vessels, starving tissues of oxygen. That can lead to potentially fatal complications, like infections and stroke.

Researchers at UT Southwestern Medical Center at Dallas recently reported that children today with this disease are living longer, less likely to die of the disease and are developing fewer infections than ever before. Researchers tracked more than 700 children over nearly two decades in one of the largest published studies of its kind.

Researchers found that more than 85 percent of children with sickle cell anemia, the most serious form of sickle cell disease, lived to the age of 18. More than 97 percent of children with milder forms lived to be 18. While stroke rates remained even, children today are less likely to die of a stroke.

Dr. George Buchanan, the UT Southwestern pediatrician who heads the Southwestern Comprehensive Sickle Cell Center, says the improvement is largely due to research developments over the past several decades. Better infant screening for early intervention, improved antibiotics and other treatments like blood transfusions and bone marrow transplants have all helped improve survival rates and quality of life for children with sickle cell disease.

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April 2004

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