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About Sickle Cell Disease

What is Sickle Cell Disease?

Sickle cell disease is a group of inherited blood disorders that affect the hemoglobin found in red blood cells. Hemoglobin is the part of the blood that carries oxygen to all parts of the body. Red blood cells that contain normal hemoglobin are soft and round and easily move through blood vessels to deliver oxygen throughout the body. With sickle cell disease, hemoglobin becomes stiff and crescent-shaped - hence the "sickle" name. Sickle hemoglobin can get stuck in blood vessels and block the flow of blood and oxygen to tissues in the body. These blockages can cause severe pain, organ damage, stroke, and even death.

What Causes Sickle Cell Disease?

Sickle cell disease is inherited, passed onto a person by their parents' genes. If, for example, one parent has normal hemoglobin (type AA) and the second parent has abnormal hemoglobin (type AS, the sickle cell trait), there is a 50% chance that each child will have the sickle cell trait, but they will not have sickle cell disease (type SS).

Hemoglobin SS, or sickle cell anemia, is the most common and most severe kind of sickle cell disease. Other common forms are hemoglobin SC disease and hemoglobin sickle beta thalassemia, a form of Cooley's anemia. Each of these can cause painful "crisis" episodes and in severe cases lead to stroke or death.

Signs and Symptoms of Sickle Cell Disease

Symptoms of sickle cell disease are different in each person. Some people have mild symptoms, while others have severe symptoms that require hospitalization for treatment. Signs and symptoms of sickle cell disease include:

  • Fatigue
  • Shortness of breath
  • Dizziness
  • Headache
  • Coldness in hands and feet
  • Pale skin
  • Chest pain

Who is Affected by Sickle Cell Disease?

The Centers for Disease Control and Prevention estimates that sickle cell disease affects up to 100,000 people in the United States. The disease occurs in nearly one out of every 500 African-American births and about one in every 36,000 Hispanic births. Sickle is particularly common among people of African, Mediterranean, Middle East, Latin American, and Asian descent.