It’s World Sickle Cell Day. How much of the difference in access to care and research dollars for sickle cell disease is due to racism and economic differences, I wondered, especially framed now by the racial tensions fueled by the recent deaths of young African-Americans in Ferguson, Baltimore, Cleveland and the pool incident in McKinney. The answer is not entirely straightforward.
What is Sickle Cell Disease?
In making hemoglobin, which carries oxygen in our red blood cells, a defect can occur when the amino acid glutamate is replaced by valine. This simple substitution can cause the red blood cell to sometimes collapse, assuming the characteristic sickle shape. The red blood cells, normally very elastic and able to conform to the shape of tiny capillary blood vessels, become rigid and can block the blood vessels, depriving tissue of oxygen and resulting in severe pain.
More than 2 million people in the U.S. carry this genetic mutation; about 100,000 have frank sickle cell disease (SCD). Most carriers are African-American (1 in 12), with lesser numbers of Hispanic (1 in 100), Middle Eastern, or Indian descent. The disease itself affects 1 in 400 in black and 1 in 19,000 in Hispanic populations.