Sickle Cell Anemia

Alternate Names : Sickle Cell Trait

Sickle cell anemia is an inherited blood disease. It causes serious health problems among African Americans or individuals of African descent.

What is going on in the body?

Hemoglobin is the key molecule in the blood that carries oxygen to all parts of the body. A change in a certain gene causes a change in the structure of hemoglobin. Persons with normal hemoglobin have a genetic coding of AA. Persons with sickle cell trait have a genetic coding of AS. (The "S" refers to the sickle cell gene.)

Eight percent of African Americans are carriers of the sickle cell gene, and are said to have sickle cell trait. Most people with sickle cell trait are completely healthy and lead normal lives. Persons with the genetic coding SS have a double dose of the abnormal hemoglobin gene. They develop sickle cell anemia. It affects approximately 15 out of every 1000 African Americans.

In sickle cell anemia the red blood cells change shape from a rounded disk to a crescent or "sickle" shape. These abnormal red blood cells are more rigid and are more likely to clump together. They are also more likely to stick to the walls of blood vessels. They can even block the blood vessels themselves. These changes also mean that the hemoglobin molecule isn't able to carry oxygen as well as it should.

What are the causes and risks of the disease?

Sickle cell anemia is caused by a change in a gene that changes the structure of the hemoglobin molecule.

Sickle cell anemia is a long-term disease that causes a number of health risks. Individuals have a severe anemia that puts extra strain on the heart, which can lead to heart enlargement. These individuals have a reduced life expectancy. They are at increased risk of the following:

Pregnant women with sickle cell anemia are likely to have kidney infections, bleeding, premature infants, and fetal death.