Sickle cell disease, a group of inherited blood disorders, affects almost 100,000 individuals in the U.S.
Routine blood transfusions can greatly reduce the recurrence of silent strokes and strokes among children with sickle cell anemia and who have pre-existing silent strokes, according to a new study. The results were released in the August 20 edition of New England Journal of Medicine.
The study’s principal investigator was Michael R. DeBaun, MD, MPH, director of the Vanderbilt-Meharry Sickle Cell Disease Center of Excellence and professor of Pediatrics. The study, the largest of its kind involving children with sickle cell, was an over $20 million federally-funded trial.
Sickle cell disease, a group of inherited blood disorders, affects almost 100,000 individuals in the U.S. The disease most commonly affects African-Americans, and is present in one out of every 396 births within this ethnic group.
Silent strokes are common in sickle cell anemia and affect approximately three percent of school-aged children with the disease, which can lead to poor performance in school and may inhibit performance of complex tasks.
DeBaun said in a statement, “The success of the trial is a tribute to over 1,000 families of children who were screened with an MRI of the brain and the almost 200 families of children who committed to monthly visits for three years. The successful recruitment, adherence and retention of black families, many of whom were working poor, to an arduous, complex trial, should lay to rest the misperception that blacks are not willing participants in research.”
He continued, “The results of the trail indicate members of the community are interested in research, when they are well-informed, empowered to make their own decision, and have a trusting relationship with the health care provider who is independent of their participation in the study.”
According to the National Heart, Lung, and Blood Institute, sickle cell anemia is the most common form of sickle cell disease, a serious disorder in which the body makes crescent-shaped red blood cells. These cells are often stiff and sticky and inhibit blood flow in the blood vessels within the limbs and organs.
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