Genetic counseling is recommended for all known carriers of the sickle cell trait. Prenatal diagnosis of sickle cell anemia is also available. Prompt treatment of infections, adequate oxygena-tion, avoidance of extreme temperatures, and prevention of dehydration may prevent sickling of the RBCs in these patients.

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An abnormal type of hemoglobin— hemoglobin S—causes sickle cell anemia. In tropical regions of the world where malaria is prevalent, individuals with a single copy of this particular genetic mutation have a survival advantage. In areas where malaria is prevalent, inheriting one copy of the mutation is beneficial because it aids in combating the disease. Inheriting two copies of the mutation, however, portends tragedy (Figure 1). People from these regions migrated over time, married one another, and had children, and some of these children inherited two copies of the mutation.

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Approximately 90,000 Americans have sickle cell disease, making it the most common genetic disease in the U.S. Its occurrence is most common in people who are descendants of residents in regions of the world where the parasite-borne disease malaria is prevalent. The ethnic groups most affected are African-Americans, Arabs, Turks, Greeks, Italians, Iranians, and Asiatic Indians.

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