Sickle Cell Anemia

Research

Sickle cell anemia was first identified by American physician James B. Herrick (1861–1954) in 1904. In the course of treating the illness of a black student from Grenada who became ill while attending the Chicago College of Dental Surgery, Herrick discovered [Page 1542]that some of the student's red blood cells were sickle-like in shape. Subsequent research revealed that most or all hemoglobin cells are sickle shaped in SCA patients. Sickled cells tend to be both rigid and fragile. The rigid shape prevents blood from flowing normally and inhibits the production of healthy hemoglobin. A normal blood cell lives for approximately 120 days. In a sickle cell patient, however, that life span is reduced to between six and 30 days. Consequently, the patient's bone marrow is unable to replace deteriorating cells, resulting in persistent anemia.

There is strong evidence that SCA had been present in some cultures for centuries where it was known by a variety of names that tended to mirror the effects of the disease. Among the Ga of Ghana, for instance, the disease was known as chwechweechwe, which represented the recurrent gnawing pain of SCA. Herrick's identification of SCA launched the first stage of modern sickle cell research, which focused on understanding the effects of the disease. By mid-20th century, biochemists and geneticists had identified the difference between sickle cell trait and sickle cell disease. In 1949, Noble laureate Linus Pauling (1901–1994) determined that SCS was molecular in origin, noting that hemoglobin molecules of individuals with sickle cell conditions were chemically different from others in the general population. Researchers also documented the genetic benefits of sickle cell trait and thalassemia in countries in where malaria was endemic because of the inherent resistance it provided. However, no effort was made to test at-risk individuals before they gave birth to children with sickle cell disease.

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