What Is Sickle Cell?

The National Institutes of Health estimates that a sickle cell trait exists in one in 12 African-Americans. However, not all of these people have sickle cell anemia. The disease only affects African-Americans and is a deformity of the red blood cells. Instead of the natural circular shape, affected red blood cells are shaped like a half moon or sickle. The complications caused by these cells are the reason that the disease is more complex than a genetic cell deformity.

Causes

• Sickle cell anemia begins with a gene that causes two types of hemoglobin (the protein that makes up red blood cells) to become abnormal. The normal hemoglobin A becomes hemoglobin S. People who carry this gene are said to have the sickle cell trait. You must inherit two sets of the gene that carries the trait in order to have sickle cell anemia. This means both of your parents must have the sickle cell trait. Even then, the Sickle Cell Disease Association of America states that two parent carriers have a 25 percent chance of creating one child with sickle cell anemia.

Anemia Causes

• The sickle-shaped cells do not live as long as the regular red blood cells, because of the sickle shape. Instead of the normal 120 days, the sickle cells last between 10 and 20 days. The result is anemia or deficit in red blood cells as the bone marrow struggles to create new cells.

Complications

• The anemia causes extreme fatigue. Pain comes from the anemia. Pain is also caused when the crescent-shaped sickle cell tries to enter the blood vessels that are configured only for the circular cells. This is also called pain crisis. The cells can form a blockage, leading to stroke. Other complications include kidney and liver damage, gallstones and excessive bacterial infections.

Life Span

• Sickle cell anemia is a lifelong or chronic disease. Years ago, children with the disease were not expected to reach adulthood. Today, however, there are 40- and 50-year-olds living and thriving with sickle cell anemia.

Treatment

• The illness is treated through prevention. Vitamin and mineral supplements (especially folic acid) along with vaccinations for illnesses like pneumonia help keep the patient healthy. Pain management is used to deal with the chronic pain, while blood transfusions and bone marrow transplants are used to remove an abundance of sickled cells from the body. Because of these transfusions, iron elimination is needed to get rid of the excess iron in the blood. Drugs to help prevent sickle cell creation are also available.