Anemia

Sickle Cell Anemia

Sickle cell anemia is most commonly diagnosed in children of African descent. It is an inherited condition caused by an abnormal form of hemoglobin. The defective hemoglobin (hemoglobin S) often causes cells to assume a crescent or sickled shape. These sickle cells die prematurely and can also block blood flow by getting caught in narrow parts of the blood system. This can cause pain in muscles, joints, bones or the trunk that specialists define as a sickle crisis. Other serious symptoms can occur, such as a stroke or severe lung damage.

Patients who have only one abnormal sickle gene (carriers, "sickle trait") are generally healthy and only need to be aware that they can have a child with sickle cell anemia. Individuals with two abnormal sickle genes or one sickle gene combined with another abnormal hemoglobin gene have the disease and usually develop symptoms.

Treatment

Medications used to control sickle cell anemia include:

• Antibiotics help patients with sickle cell anemia fight or avoid certain infections that could be life threatening based on their condition. Antibiotics are necessary because most patients do not have a functioning spleen, and the spleen helps filter germs from the blood.
• Hydroxyurea, a prescription drug typically used for leukemia, can decrease deaths from sickle cell anemia. The drug encourages the growth of fetal hemoglobin, a type of hemoglobin in newborns. Fetal hemoglobin helps prevent the formation of sickle cells.
• Pain relievers help patients with sickle cell anemia get through times of severe pain caused by the buildup of the abnormally shaped red blood cells in joints and other areas of the body. These episodes of pain can last hours or weeks and are called sickle crises.

Blood Transfusions

Blood transfusions rapidly increase red blood cells circulating in the body. This treatment helps relieve anemic symptoms. For children at risk of a stroke, regular blood transfusions may reduce the risk by 90 percent. Unfortunately, with repeated transfusions, the level of iron can build up to dangerous levels in the body. Other medications may become necessary to help reduce iron levels.

Oxygen Supplements

During a sickle crisis, oxygen may be given to help the patient breath easier.

Bone Marrow Transplant

In severe cases of sickle cell anemia, a bone marrow transplant may be considered. The risks for this procedure are very high. Experienced physicians at Mayo Clinic work with the patient to carefully weigh the options, risks and benefits. Physicians use chemotherapy or radiation to destroy the patient's bone marrow before matched bone marrow from a donor is transplanted. Bone marrow transplant may require a lengthy stay at a Mayo Clinic hospital. Read more about bone marrow transplant.

Read more about sickle cell anemia at
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