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Sickle Cell Anemia

Sickle cell anemia is a disease that causes your body to produce abnormal red blood cells.

Red blood cells carry oxygen throughout your body. Normally, they are round and flexible. In people with sickle cell anemia, they are shaped like sickles and are not flexible. These “sickle cells” die off too early, causing a shortage of red blood cells.

Sickle cells are prone to getting stuck in small blood vessels. This can lead to a dangerous lack of blood flow to the organs. This can cause a stroke.

Diagnosis

These tests can help detect and diagnose sickle cell anemia:

• Blood tests checking for hemoglobin S, the defective genetic form of hemoglobin causing sickle cell anemia
• Sampling amniotic fluid surrounding an unborn baby in the mother’s womb

Symptoms

Sickle cell anemia may cause some of these symptoms, but other conditions may cause the same symptoms. See a doctor if you have any of these problems:

• Swollen hands and feet (in babies)
• Fatigue
• Episodes of pain in the chest, abdomen, joints and bones
• Jaundice (yellowish skin and eyes)
• Frequent infections
• Slow growth in infants and children
• Vision problems
• Rapid heart rate
• Skin ulcers

Treatment

A bone marrow/stem cell transplant is the only treatment option that offers a possible cure for sickle cell anemia. The goal of treatment is to manage symptoms and limit the number of painful episodes (crises).

Other treatment options include:

• Pain-relieving medicines
• Antibiotics to prevent infections such as pneumonia
• Hydroxyurea: This reduces the need for blood transfusions and triggers production of fetal hemoglobin
• Immunizations to prevent infections
• Blood transfusions
• Oxygen therapy

Risk Factors

Sickle cell anemia is an inherited disease. African-Americans and Hispanic people are at increased risk. In most states, newborn babies are tested for sickle cell anemia.

Related Links

Vanderbilt Hematology

Monroe Carell, Jr. Children's Hospital at Vanderbilt

American Sickle Cell Anemia Association

March of Dimes