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Sickle cell anemia is a serious condition in which the red blood cells can become sickle-shaped (that is, shaped like a “C”).
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Sickle-shaped cells don’t move easily through the blood. They’re stiff and sticky and tend to form clumps and get stuck in blood vessels. The clumps of sickle cells block blood flow in the blood vessels, causing pain, serious infections, and organ damage.
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Sickle cell anemia is an inherited, lifelong condition. People who have sickle cell anemia are born with it. They inherit two copies of the sickle cell gene, one from each parent.
· People who inherit a sickle cell gene from one parent and a normal gene from the other parent have a condition called sickle cell trait. People with sickle cell trait don’t have the condition, but they can pass the sickle cell gene on to their children.
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Sickle cell anemia affects millions of people worldwide. It’s most common in people whose families come from Africa, South or Central America (especially Panama), Caribbean islands, Mediterranean countries (such as Turkey, Greece, and Italy), India, and Saudi Arabia.
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In the United States, sickle cell anemia affects about 70,000 people. It mainly affects African Americans.
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The signs and symptoms of sickle cell anemia are different in each person. Some people have mild symptoms. Others have very severe symptoms and are often hospitalized for treatment.
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The most common signs and symptoms of sickle cell anemia are linked to anemia and pain (also referred to as a "sickle cell crisis").
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Infection is a major complication of sickle cell anemia. Pneumonia, in particular, can be a life-threatening complication in children with sickle cell anemia. Routine treatment with penicillin and vaccinations are used to prevent infection in children.
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Strokes are a major complication for children with sickle cell anemia. Routine screening and early treatment can prevent strokes.
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Early diagnosis of sickle cell anemia is very important so that children who have the condition can get proper treatment. Screening tests for sickle cell anemia and sickle cell trait are done on newborn infants in most States. One blood test used to diagnose sickle cell anemia looks at how hemoglobin moves in an electric field. This is called electrophoresis, and it’s usually used to diagnose older children and adults.
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Effective treatments are available to help relieve the symptoms and complications of sickle cell anemia, but in most cases there’s no cure. (Some researchers believe that bone marrow transplants may offer a cure in a small number of cases.)
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Sickle cell anemia is treated with medicines, blood transfusions, and other treatments specific to certain complications.
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Researchers are working to develop new treatments for sickle cell anemia, including gene therapy and safer and more effective bone marrow transplants.
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If you have sickle cell anemia, take good care of yourself and see your doctor regularly.
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If your child has sickle cell anemia, you should learn as much about the condition as possible. This will help you recognize early signs of problems, such as fever or chest pain, and seek early treatment.