About Sickle Cell Disease

Name of the medical condition and other names that it’s been known by

• Sickle cell disease (a broad term that includes sickle cell anemia)

• HbS disease

• Hemoglobin S disease

• Hemoglobin SS disease

• Sickle cell disorders

• Sickling disorder due to hemoglobin S.

What is Sickle cell disease?

It’s a genetic blood disorder that affects the red blood cells in your body. Normally, red blood cells are round and flexible, which allows them to move easily through blood vessels to deliver oxygen to different parts of the body. However, in people with Sickle Cell Disease, the red blood cells become rigid and shaped like a crescent moon or a sickle. A mutation in the hemoglobin gene causes this abnormal shape.

Hemoglobin is the protein in red blood cells that carries oxygen. In Sickle Cell Disease, the hemoglobin is abnormal (called hemoglobin S), which makes the cells stick together and block blood flow. This can lead to a variety of health problems including pain, infections, and damage to organs. The disease is inherited, meaning it is passed down from parents to their children. For someone to have Sickle Cell Disease, they must inherit two sickle cell genes—one from each parent. If they inherit only one sickle cell gene, they are said to have "sickle cell trait," which usually does not cause symptoms but can be passed on to their children.

Trend

• Approximately 100,000 Americans have Sickle Cell Disease.

• The number of people affected by Sickle Cell Disease in the United States has remained relatively stable over the last five years, with about 100,000 people living with the condition each year.

• The number of people with Sickle Cell Disease in the United States is projected to remain around 100,000 over the next five years. This projection is based on the stable incidence rate and current population trends.

History

Sickle Cell Disease was first described in Western medical literature in 1910 by Dr. James B. Herrick, who observed a patient with anemia and peculiar-shaped red blood cells. However, the condition has likely existed for thousands of years, particularly in regions where malaria is common. The sickle cell gene provides some protection against malaria, which is why it is more prevalent in people of African, Mediterranean, Middle Eastern, and Indian ancestry. The genetic mutation causing the disease likely arose independently in different geographic areas, including Africa, the Middle East, and India. This mutation has been identified as a survival advantage in malaria-endemic regions, which explains its higher frequency in these populations.

Source of Information

• American Society of Hematology

• Cleveland Clinic

• Hematology Oncology Associates of Central New York

For informational purposes only. Consult a medical professional for advice.