Sickle Cell Anemia

Chronic, life-threatening blood disorder affecting mostly Africans and African Americans. Dr. James Herrick first discovered sickle cell anemia (SCA) in 1910. Although the disorder occurs most frequently in persons of African descent, it also occurs in persons from the Middle East, India, and the Mediterranean. In the United States, the National Institutes of Health have estimated that sickle cell affects 72,000 people of African descent and occurs in 1 of every 500 African American births. Studies have also shown that 1 in 12 African Americans carry the sickle cell trait that transmits the disorder.

Sickle cell anemia develops when a person inherits two damaged hemoglobin genes from a mother and father. Normal hemoglobin travels throughout the body by red blood cells, picks up oxygen in the lungs, and releases it throughout the body. Sickle cell anemia prevents cells from getting oxygen because the sickle cells become clogged in the small blood vessels. The lack of oxygen to cells and tissues causes excruciating pain and damage to body organs, and it can lead to death. SCA is believed to have developed from genetically abnormal hemoglobin that evolved as a protection against malaria. Persons with the sickle cell trait were more likely to survive malaria outbreaks in Africa.

Symptoms of sickle cell anemia usually appear during an infant's first year. They include pain in the chest, abdomen, limbs, and joints; an enlarged heart; and nosebleeds. As children age, symptoms of the disorder include fatigue, irritability, delayed puberty, leg sores, and eye and gum disease. There is currently no cure for SCA, but treatment is available. Treatments include nonnarcotic medications, antibiotics to fight infections, and bone marrow transplants. New drugs are being developed to manage the disorder, but no widely used drug is currently available for treatment.

Some preventive steps can also decrease the occurrence of sickle cell crises. Precautionary steps include avoiding environments with low oxygen content; avoiding emotional stress, strenuous physical activity, and excess exposure to the sun; being properly immunized; and wearing a Medic Alert Bracelet. Numerous research organizations and health boards offer information about sickle cell anemia, as well as genetic counseling, extensive research on the disorder, and support groups for persons living with SCA.