By James N. Martin, Jr, MD
President, The American Congress of Obstetricians and Gynecologists
An estimated 100,000 people in the US have sickle cell anemia, an inherited, lifelong disorder that affects the red blood cells. While the disease is most common among African Americans, it also occurs in people of Hispanic, Indian, Caribbean, Mediterranean, Middle Eastern, and South Asian descent.
Red blood cells move oxygen from the lungs throughout the body. Normal red blood cells are round, doughnut-shaped, and able to move easily through the blood vessels. They live for about 120 days. In contrast, sickle cells are abnormal, crescent-shaped red blood cells. Stiff and sticky, they have a tendency to block blood flow in the blood vessels of the limbs and organs which can lead to pain, serious infections, and organ damage. Sickle cells are also fragile, dying after just 10 to 20 days and leaving the blood with a lower than normal amount of red blood cells (anemia).
An estimated two million people in the US are sickle cell carriers, meaning that though they have no symptoms, they have inherited one sickle cell gene from a parent and could pass it along to their children. When two sickle cell carriers have a child together, there is a 25% chance that the child will be born with the disease. Most states routinely test newborns for sickle cell disease before they leave the hospital.
Symptoms of sickle cell disease include swelling in the hands and feet (often the first sign of sickle cell anemia in babies), episodes of pain, frequent infections, delayed growth in babies and children and late onset of puberty in teens, vision problems, and an increased risk of stroke. Classic symptoms of anemia such as weakness, tiredness, a pale or washed out complexion, and yellowing of the skin and whites of the eyes (jaundice) may also be present.
Sickle cell disease can worsen during pregnancy and increase the risk of preterm birth and low-birth-weight babies. Special prenatal care is needed, so women with sickle cell disease who are pregnant or considering pregnancy should consult with their doctors.
Daily antibiotics for infants and children from two months to five years of age, routine childhood immunizations, blood transfusions, and medications are commonly used treatments. Eating right, staying hydrated, exercising, getting enough sleep, and developing a pain management plan are important strategies for improving quality of life. Blood and marrow stem cell transplants may cure sickle cell disease in some individuals, but the procedure is risky and success is not guaranteed.
People with sickle cell anemia may have a reduced life expectancy, though with the medical advances and treatments available today, many live into their 40s, 50s, and beyond.
To learn more, visit www.nhlbi.nih.gov/health/health-topics/topics/sca/. ?
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