About 90,000 to 100,000 people in the United States have sickle cell disease, an inherited red blood cell disorder with lifelong effects. National Sickle Cell Awareness Month in September is an opportunity to learn more about this disease, who is at risk and how it is treated.

Sickle Cell Disease

Sickle cell disease, also often called sickle cell anemia, occurs when a person inherits two sickle cell genes, one from each parent. The disease affects red blood cells (RBCs), which are normally disc-shaped and contain a protein called hemoglobin that carries oxygen through the body.

With sickle cell disease, RBCs are shaped more like the letter C or a curved tool called a sickle. These cells deliver less oxygen to the body’s cells. They’re also stickier and more likely to clump together.

Sickle cell disease is most common in people of African descent, including African Americans. It can also show up in other ethnic groups, including people of Mediterranean descent as well as people from South and Central America, the Caribbean and the Middle East.

Effects of Sickle Cell Disease

Most people with sickle cell disease go through episodes called pain crises. These occur when red blood cells stick together and cause blocked blood vessels, making it hard to deliver oxygen to the body’s cells. These crises can cause pain in the chest, lower back, abdomen, arms and legs. They can last from hours to days and sometimes require hospitalization.

Sickle cell disease can affect people’s health in other ways as they grow older:

• Decreased spleen function
• Kidney disease
• Gallstone disease
• Eye problems
• Leg ulcers
• Increased risk of infections throughout the body

Treatment

The goal of treatment is to make the patient comfortable by alleviating symptoms such as pain or infection. Treatment is ongoing and can include the use of supplements, blood transfusions, pain medications and fluids. Patients may also develop complications from sickle cell disease, and those treatments can include antibiotics, surgery and counseling.

For severe sickle cell disease, a medicine called hydroxyurea might be recommended. In 2023, the U.S. Food and Drug Administration approved two cell-based gene therapies for the treatment of sickle cell disease in patients aged 12 years and older.

Bone marrow or stem cell transplants can cure sickle cell disease, but this option is not available to most people as it requires a well-matched donor.

Outlook for Patients

Sickle cell disease does shorten people’s lives due to the complications that arise from it. In the past, patients with this diagnosis lived to age 20 to 40 years old. More modern care has extended patients’ lives past the age of 50.

For more information on sickle cell disease and its treatments, visit Sickle Cell Disease from the Centers for Disease Control and Prevention.

Sources:
Centers for Disease Control and Prevention
MedlinePlus.gov
National Heart, Lung, and Blood Institute
The Staywell Company, LLC