Sickle Cell Anemia

What is it?
Sickle cell anemia is an inherited blood disorder that causes red blood cells to be abnormally shaped. The condition can cause anemia, painful events and various complications.
Who gets it?
Sickle cell anemia primarily affects people of African, Mediterranean, Middle Eastern, and Asian Indian ancestry. In the United States, the disease occurs in one out of every 600 African-Americans.
What causes it?
Sickle cell anemia is cause by a genetic change in hemoglobin, the oxygen-carrying protein inside the red blood cells. This causes the cells to take on a sickle-shape and have a shorter life span, which can cause anemia. Sickle cells are also less flexible and stickier than normal red blood cells, and can become trapped in small blood vessels preventing blood flow and starving tissues and organs. This compromises the delivery of oxygen, which may result in damage to associated tissues and organs. The disease is often inherited.
What are the symptoms?
Symptoms of sickle cell anemia include paleness of skin as the anemia worsens, especially on the insides of eyelids, under fingernails and in the creases of the palm of the hand. The skin may also turn yellow (jaundiced). If the anemia is severe, the shortage of normal red blood cells may cause weakness, shortness of breath, or even heart failure. The symptoms of shock caused by heart failure are low blood pressure, rapid pulse, and decreasing consciousness.
How is it diagnosed?
Sickle cell anemia may be diagnosed through a test called hemoglobin electrophoresis. This blood test detects the sickle cell hemoglobin S. It can also be used to find carriers of the sickle cell trait. In addition, the doctor may order a complete blood count to confirm the effects or complications of sickle cell anemia. During this test, red blood cells are counted under a microscope and checked for abnormal shapes.
What is the treatment?
There is no cure for sickle cell anemia, so treatment focuses on lessening a patient’s symptoms and prolonging their lives. Recent studies have shown that the drug hydroxyurea may reduce pain attacks in people with sickle cell anemia. It also has been shown to prolong the lives of patients diagnosed with sickle cell anemia. Patients with sickle cell anemia are more likely to have serious infections such as the flu or pneumonia. Prevention of serious infections is an important part of treatment. The doctor may recommend immunizations are kept up-to-date, get a flu shot each year, and take antibiotics to prevent infections.
Blood transfusions are not usually given on a regular basis but may be used to treat patients who experience frequent and severe painful events, severe anemia, and other emergencies.
Self care tips
Couples who have sickle cell anemia or who are carriers of the disease may consider not having children to prevent the spread of the disease. Screening tests can be used to find carriers. Couples who find that they are carriers should seek genetic counseling.