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Sickle cell anemia is one of today’s most common inherited blood disorders. It affects several ethnic groups but predominantly Africans and African Americans, with approximately 90,000 to 100,000 Americans having the condition. Overall, it is estimated that sickle cell disease affects one in 500 U.S. African American births.

Why is it called “sickle cell” anemia?

When red blood cells are healthy, they are flexible and round, which allows them to easily flow through even the smallest blood vessels. In sickle cell disease, the red blood cells are originally round like a donut but can and do change shape, while flowing through blood vessels, into a sickle-like form or the letter “C”.  When this change occurs, the cells become more rigid.  As a result, sickle cells are more likely to bunch up with one another, leading to blockages in blood flow and slowing the delivery of oxygen, resulting in pain, infections, and other complications. 

Symptoms of sickle cell anemia

Virtually all major symptoms of sickle cell disease are the direct result of the abnormally shaped blood cells blocking the flow of blood to body tissue. The tissue with impaired circulation becomes damaged from lack of oxygen. Patients can suffer from episodes of pain, varying in frequency and severity, as well as damage to important organs such as the spleen and the lungs.

Signs and symptoms of sickle cell disease can be fairly mild or severe enough to require hospitalization. They may include:

• Anemia (fatigue, dizziness, shortness of breath)
• Pain (most often occurs in the chest, and long bones such as arms and legs)
• Dactylitis (swelling of hands and feet)
• Dark urine
• Yellow eyes
• Bacterial infection
• Eye damage
• Lung injury
• Enlarged hearts
• Spleen enlargement and poor function

Sickle cell anemia can become apparent in the first year of life. Infants and young children can suffer from fever, abdominal pain, bacterial infections, and painful swelling in the hands and feet.  Adolescents and young adults often develop leg ulcers, aseptic necrosis (bone weakening/bone death), and eye damage. Adults often suffer intermittent or even daily chronic pain episodes due to repeated injury of bones or internal organs.

How do you get sickle cell disease?

The disease can only be inherited. In order to inherit sickle cell disease, both parents must have either sickle cell disease (two sickle cell genes) or sickle cell trait (one sickle cell gene).  If you have sickle cell disease, you will pass one sickle cell gene on to your children.

Risk factors

Sickle cell disease is more common among certain ethnic groups:

• People of African descent, including African Americans
• Hispanic-Americans from Central and South America
• People of Middle Eastern, Asian, Indian, and Mediterranean descent

Sickle cell disease symptoms can begin as early as four months of age so early diagnosis is critical. All newborns in the United States are now tested for the disease. 

Treatment

There is no single standard treatment today to cure sickle cell anemia. However, there are therapies that can help people manage the disease. These include:

• Pain medications
• Drinking plenty of water daily to prevent and treat pain crises—in some situations intravenous fluids may be required
• Blood transfusions to dilute the sickled red blood cells with normal red blood cells
• Vaccinations and antibiotics to prevent infections
• Folic acid to help prevent severe anemia
• Hydroxyurea—this medication helps reduce the frequency of pain crises and helps decrease the need for blood transfusions
• Bone marrow transplant—this procedure can cure some people with sickle cell disease (the decision to have this procedure is based on the severity of the disease and the ability to find a suitable bone marrow donor)

Living with sickle cell disease

Following a healthy lifestyle and staying clear of certain medications and situations can reduce some of the complications.

• Eat a diet with lots of fruits, vegetables, whole grains, and protein, and as mentioned above, drink lots of fluids.
• Do not take decongestants—these cause constriction of blood vessels and could trigger a crisis.
• Other factors that may trigger a crisis include high altitudes, cold weather, swimming in cold water, and arduous physical labor.
• Avoid infections by getting an annual flu shot, washing your hands frequently, avoiding those who are sick, and getting regular dental exams.

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