Red blood cells are normally round. In sickle cell disease, the red blood cells take on a sickle shape. This makes the blood thicker and affects the red blood cells' ability to carry oxygen to the body's tissues. The result is a chronic disease that affects many systems in the body.
Causes
Sickle cell disease is inherited. It mostly affects blacks. 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 disease, but have no symptoms of the
disease). If both parents carry the trait, the chance of having a child with sickle cell
anemia is 1 out of 4, or 25%. About 1 in every 400 African Americans is born with sickle
cell disease.
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 now between the ages of 40 and 50.
Signs and Symptoms
A blood test can detect sickle cell disease. Signs and symptoms include the
following:
Pain, ranging from mild to severe,
in the chest, joints, back, or abdomen
Swollen hands and feet
Jaundice
Repeated infections, particularly pneumonia
or meningitis
Kidney failure
Gallstones (at an early age)
Strokes (at an early age)
Treatment, Care, and Prevention
For now, no medicines exist to effectively treat sickle cell disease. At
best, treatment aims to prevent complications. Painful episodes are treated with
painkillers, fluids, and oxygen. The diet is supplemented with folic acid, a B-vitamin.
Because people with sickle cell disease are prone to developing pneumonia, they should be
vaccinated against pneumonia.
To prevent sickle cell disease 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 disease can be diagnosed by amniocentesis in the second trimester of pregnancy. If the fetus has sickle cell disease, the parents may elect to terminate the pregnancy.
{Note: Recently, some persons have been cured of sickle cell disease with chemotherapy drugs and a bone marrow transplant. The transplant used bone marrow from a sibling with similar genetic makeup. This new form of treatment is risky and may not be suited for all persons with sickle cell disease.}
(See "Places to Get Information & Help" under Sickle Cell Disease.)
HEALTH AT HOME - Your Complete Guide to Symptoms, Solutions, and Self-Care © 1999 by Don R. Powell. American Institute for Preventive Medicine.
Date updated 04/21/99