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A to Z: Sickle Cell Disease

May also be called: SCD; Sickle Cell Anemia

Sickle cell disease is an inherited disorder in which red blood cells (RBCs) are abnormally shaped. This abnormality can result in painful episodes, serious infections, chronic anemia, and damage to body organs.

More to Know

Normally, RBCs have a round, doughnut shape and move easily through small blood vessels. Sickle cell disease causes RBCs to form in the elongated shape of a sickle, or the shape of the letter "C." These abnormal RBCs have a tendency to get stuck in narrow blood vessels and block the flow of blood. This can cause episodes of pain and lead to organ damage because the tissues aren't getting enough oxygen.

Sickle cells also have a shorter-than-normal life span, which leads to anemia (low RBC count). People with sickle cell disease are also at increased risk for certain bacterial infections and stroke.

Sickle cell disease is caused by a defect in the genes that affect hemoglobin, a protein found in RBCs that transports oxygen throughout the body. If a child inherits a sickle cell gene from each parent, he or she will have sickle cell disease. If the child inherits one sickle cell gene and one normal gene, he or she will have sickle cell trait. People with sickle cell trait usually have no symptoms, but they can pass the sickle cell gene on to their children.

In most cases, treatment for sickle cell disease involves medicines to help manage the pain, and immunizations and daily doses of penicillin (an antibiotic) to help prevent infection. Severe anemia may be treated with blood transfusions.

Keep in Mind

Thanks to advancements in early diagnosis and treatment, most kids born with sickle cell disease grow up to live relatively healthy and productive lives. The disease is a constant presence, though, so seek immediate medical attention for any signs of infection, severe anemia, chest pain, or seizures.

All A to Z dictionary entries are regularly reviewed by KidsHealth medical experts.

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