75. Sickle Cell Anemia: Information Is the Best Defense
Sickle cell anemia is inherited. It mostly affects African Americans, but can occur in other ethnic groups, such as Hispanic Americans. About 1 in 12 African Americans carries the gene for the sickle cell trait (that is, they have the ability to produce children with sickle cell anemia but have no symptoms of the disease). If both parents carry the trait, the chance of having a child with sickle cell anemia is one out of four. About 1 in every 1,000 Hispanic Americans are born with sickle cell anemia.
Sickle cell anemia affects the red blood cells’ ability to carry oxygen to the body’s tissues. The disease usually doesn’t become apparent until the end of the child’s first year. The average life expectancy with proper medical care is between the ages of 40 and 50.
Signs and symptoms include:
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•Bone and joint pain, ranging from mild to severe. The pain can also be in the chest, back, or abdomen.
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•Shortness of breath and difficulty breathing.
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•Swollen hands and feet.
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•Jaundice.
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•Repeated infections, especially pneumonia or meningitis.
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•Kidney problems, including kidney failure.
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•Gallstones (at an early age).
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•Strokes (at an early age).
A blood test can detect sickle cell anemia. Medical treatment and monitoring are needed. Painful episodes are treated with painkillers, fluids, and oxygen. The diet is supplemented with folic acid, a B-vitamin. Because people with sickle cell anemia are prone to developing pneumonia, they should be vaccinated against pneumonia. Annual flu and H.influenzae type 6 vaccines should be given to all persons with sickle cell anemia.
To prevent sickle cell anemia in offspring, couples, especially African American couples, should have a blood test to determine if they are carriers for the sickle cell trait. Genetic counseling can help them decide what to do.
After conception, sickle cell anemia can be diagnosed by amniocentesis in the second trimester of pregnancy.
