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Sickle cell anemia (SCA) is an autosomal recessive disorder that affects the structure of hemaglobin (Hb). In low oxygen levels, the mutant hemaglobin molecules clump and the red blood cells (RBC) are deformed into a sickle-like shape. This differs from their normal doughnut shape. Sickle cell RBCs become trapped in capillary walls, causing pain and possibly damaging organs.
Although this disease can affect practically any ethnicity, sickle cell anemia predominantly occurs in the African-American population. One in ten African-Americans is a carrier of the defective gene.
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