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 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 disease, but have no symptoms of the disease). If both
parents carry the trait, the chance of having a child with sickle cell disease is 1 out of
4. About 1 in every 400 African Americans and about 1 in every 1,000 Hispanic Americans
are 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 between the ages of 40 and 50.
Signs and Symptoms
Bone and joint pain, ranging from
mild to severe. The pain can also be in the chest, back, or abdomen.
Shortness of breath and difficulty
breathing
Swollen hands and feet
Jaundice
Repeated infections, especially
Kidney problems, including kidney failure
Gallstones (at an early age)
Strokes (at an early age)
Treatment, Care, and Prevention
Medical treatment and monitoring are needed. Painful episodes are treated
with painkillers, fluids, and oxygen. The diet is supplemented with folic acid, and
B-vitamin. Persons over the age of 2 should get a pneumonia vaccine. Annual flu shots and
H. influenzae type b vaccines should be given to all persons with sickle cell disease.
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.
{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 © 2002 by Don R. Powell. American Institute for Preventive Medicine.
Date updated 06/10/02