source: The New England Journal Of Medicine

year: 2020

authors: Alexandra Power-Hays, Patrick T. McGann

SCD is a life-threatening, inherited blood disorder, affecting more than 100,000 Americans. Painful vaso-occlusive crises, the hallmark of SCD, result in substantial suffering and lead to associated stigma. Without adequate treatment, SCD affects all organs and is associated with decreased quality of life and a shortened life span. Among the dozens of conditions that are screened for in state newborn-screening programs, SCD is the most commonly detected condition, regardless of ethnicity. It is thus important to recognize SCD as a common and important medical condition among Americans, and not “just Black Americans.”

The causative mutation in SCD primarily arose on the African continent because of the protective effect of the carrier state against malaria, so most patients with SCD have a shared African ancestry. Although SCD is a global disorder affecting people of all races, in the United States, as a direct result of the transatlantic slave trade, nearly all patients with SCD are Black. This fact would be mere medical trivia if we did not live in such a highly racialized society. Unfortunately, the social construct of race in America requires the majority of patients with SCD not only to face the consequences of a serious health condition, but also to navigate a society in which the color of their skin is often an unfair disadvantage.

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