Monroe Carell Jr. Children's Hospital at Vanderbilt
Monroe Carell Jr. Children's Hospital at Vanderbilt
Monroe Carell Jr. Children's Hospital at Vanderbilt
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Monroe Carell Jr.
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About Sickle Cell Disease

 

Sickled Cells
Image Credit: National Heart Lung and Blood Institute

Sickle cell disease is a group of inherited red blood cell disorders. Healthy red blood cells are doughnut-shaped, soft, flexible, and contain normal hemoglobin A. They flow easily through small blood vessels and carry oxygen to tissues throughout the body. In sickle cell disease, red blood cells contain abnormal hemoglobin molecules, called hemoglobin S, for sickle hemoglobin. Red blood cells in sickle cell disease become hard, sticky, and take on the shape of a “C” shaped farmers’ tool called the sickle.

Sickle cells live a short time, causing a shortage of red blood cells, or anemia. When sickle cells travel through small blood vessels, they can get stuck and clog the blood flow. This causes pain episodes, called a sickle cell pain crisis, often in the arms, legs, chest, back, or abdomen. Constant sickling also causes blood vessel damage and injuries to the organs in the body.

Sickle cell anemia is an inherited disease. It occurs most frequently in the African-American population, and less frequently in Hispanic and Middle Eastern populations. There are about 70,000 to 100,000 people with sickle cell disease in the United States. 

One out of 12, or about 10 percent of African-Americans have the sickle cell trait. When both parents carry sickle cell trait, their child has a one in four chance of being born with sickle cell disease. People with sickle cell trait do not have symptoms and lead a normal life.

There are several forms of sickle cell disease. The most common type is Hemoglobin SS disease, in which the patient carries two copies of the abnormal hemoglobin S gene. The second-most common form is Hemoglobin SC disease, in which the patient has one copy of hemoglobin S and one copy of hemoglobin C. Another type of sickle cell disease, sickle/beta-thalassemia is made up of one copy of hemoglobin S and one copy of hemoglobin A is not working normally. A genetic counselor or a hematologist can help you understand how the different forms of sickle cell disease are inherited and the risks of having a child with sickle cell disease.

More sickle cell disease resources

Strategy to improve physician attitudes about sickle cell disease


Last Edited: June 28, 2017
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