WHAT IS SICKLE CELL ANEMIA?

Sickle cell anemia is a disease in which your body produces abnormally shaped red blood cells. The cells are shaped like a crescent or sickle and break down prematurely, which can lead to anemia. Anemia can cause shortness of breath, fatigue, and delayed growth and development in children. The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice. Painful episodes can occur when sickle-shaped red blood cells, which are stiff and inflexible, get stuck in small blood vessels. 

These episodes deprive tissues and organs of oxygen-rich blood and can lead to organ damage, especially in the lungs, kidneys, spleen and brain. A particularly serious complication of sickle cell anemia is high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension). Pulmonary hypertension occurs in about one-third of adults with sickle cell anemia and can lead to heart failure.

A genetic problem causes sickle cell anemia. People with the disease are born with two sickle cell genes, one from each parent. If you only have one sickle cell gene, it's called sickle cell trait. About 1 in 12 African Americans and 1 in 100 Hispanic Americans have sickle cell trait. People with sickle cell trait don't have sickle cell disease or exhibit any signs of the disorder, but they can pass the gene for the disease to their children. With each pregnancy, two people with sickle cell traits have:

•  A 25 percent chance of having an unaffected child with normal hemoglobin

•  A 50 percent chance of having a child who also is a carrier

•  A 25 percent chance of having a child with sickle cell anemia

A blood test can show if you have the trait or anemia. Most states test newborn babies as part of their newborn screening programs.

Sickle cell anemia affects millions of people worldwide. It is most common among people whose ancestors come from Africa; Mediterranean countries such as Greece, Turkey, and Italy; the Arabian Peninsula; India; and Spanish-speaking regions in South America, Central America, and parts of the Caribbean. Sickle cell anemia is the most commonly inherited blood disorder in the United States, affecting 70,000 to 80,000 Americans. The disease is estimated to occur in 1 in 500 African Americans and 1 in 1,000 to 1,400 Hispanic Americans. 

People who inherit a sickle cell gene from one parent and a gene for another type of abnormal hemoglobin (the protein that allows red blood cells to carry oxygen to all parts of the body) from the other parent have a different type of sickle cell disease. Some of these less common forms of sickle cell disease are very severe and some are milder. The disease affects each person differently.