Sickle cell anemia is an inherited condition in which red blood cells, which are normally round and flexible, become c-shaped, hard and sticky. The name comes from the farm tool called a sickle that has a crescent shape. Because of their abnormal shape, these cells do not move smoothly through the blood vessels and may get stuck, creating a blockage that impedes blood flow.

In addition, while normal blood cells live for approximately 120 days before being replaced, sickle cells die within 10 to 20 days. Consequently, people with sickle cell anemia have fewer blood cells in circulation and therefore decreased oxygen delivery to the tissues.

What is Sickle Cell Anemia and How Does it Affect the Heart?

The specific set and severity of sickle cell anemia symptoms may differ from one person to another, but in general, they include:

• Fatigue. Because their body isn’t getting enough oxygen, people with sickle cell anemia tend to feel tired and lacking in energy.
• Swelling of the hands and feet. Insufficient blood flow to the hands and feet as a result of sickle cell-related blockage can cause swelling and discomfort.
• Pain. Sickle cells can decrease blood flow in the joints, chest and abdomen, resulting in pain. These episodes called crises can be anywhere from mild to intense, lasting from hours to weeks. They may occur rarely or many times each year. In addition, damage caused by the sickle cells can lead to chronic pain in the bones, joints and other areas.
• Increased risk of infection. Sickle cell anemia can damage the spleen, which plays an important role in fighting infection. Consequently, people with the condition may have more infections.
• Poor vision. Plugging of the blood vessels that supply the retina can cause vision problems.
• Delayed growth. Because sickle cell anemia deprives the body of adequate oxygen and nutrients, children may not grow at a normal pace and may experience delayed puberty.

Impact on the Heart

While sickle cell anemia affects many areas of the body, its impact on the heart can be especially problematic. Issues including pulmonary hypertension (high blood pressure in the arteries leading from the heart to the lungs), diastolic dysfunction (a condition in which the heart’s ventricles do not fully relax and cannot properly fill with blood) and sudden death may occur.

The link between sickle cell anemia and heart disease is complicated. Not only does the sickle shape of the cells make them more likely to cause blockages, but they can also damage the lining of blood vessels, resulting in inflammation. Plus, the short lifespan of sickle cells contributes to tissue hypoxia, or oxygen deprivation, which can harm tissue in the heart and elsewhere.

Treating Sickle Cell Anemia

Sickle cell anemia can only be cured through a bone marrow or stem cell transplant. These procedures, which take healthy cells involved in the production of blood from a donor and put them into the recipient, require a closely-matched donor, like a sibling, and can have serious side effects, including death. Consequently, they’re generally only used in cases where a child under the age of 16 has a severe form of the condition and only minimal organ damage.

In most cases of sickle cell anemia, doctors focus on preventing complications from the disease and helping patients avoid crises and other symptoms. Medication, blood transfusions and other interventions