New University Study Offers Hope for Children Suffering From Sickle Cell Disease

sickle-cellsA new study originated by researchers at the Washington University School of Medicine in St. Louis has found that monthly blood transfusions can reduce the incidence of strokes in children with sickle cell disease. An estimated one third of children with sickle cell disease experiences silent strokes — loss of blood flow to parts of the brain. Such strokes do not cause immediate symptoms and typically go undiagnosed. But damage from these incidents, which often recur, can lower a child’s IQ. The study data showed that monthly transfusions can reduce the recurrence of strokes by 58 percent in children who have had these silent strokes in the past.

Transfusions are  “the only evidence-based option to prevent stroke recurrence and further brain injury in this vulnerable population,” said the study’s co-author Michael Noetzel, a professor of neurology and of pediatrics at Washington University. “Now that we have identified a viable treatment option, early detection of silent cerebral strokes should become a major focus for clinicians and families of children with sickle cell disease.”

Sickle cell disease affects about 100,000 people in the United States and occurs most commonly in African-Americans. The disease, inherited from both parents, causes some of the patient’s red blood cells, normally shaped like a saucer, to take on a crescent or sickle shape. These malformed cells are less effective at their primary job, conveying oxygen from the lungs to the rest of the body. The cells also clump together, blocking circulation and leading to organ damage, strokes and episodes of intense pain.

The research, “Controlled Trial of Transfusions for Silent Cerebral Infarcts in Sickle Cell Anemia,” was published in the August 21 issue of the New England Journal of Medicine. It may be accessed here.


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