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What is sickle cell anemia?

Sickle cell anemia is an inherited blood disorder that mostly affects people of African ancestry, but also occurs in other ethnic groups, including people who are of Mediterranean and Middle Eastern descent.

More than 70,000 Americans have sickle cell anemia. And about 2 million Americans — including 1 in 12 African Americans — have sickle cell trait, which means they carry a single gene for the disease and can pass this gene along to their children, but do not have the disease itself.

Sickle cell anemia is a blood disorder that affects hemoglobin the protein found in red blood cells (RBCs) that help to carry oxygen throughout the body.
Sickle cell anemia occurs when a person inherits two abnormal genes (one from each parent) that cause their RBCs to change shape. Instead of being flexible and disc-shaped, these cells are more stiff and curved in the shape of the old farm tool known as a sickle… that’s where the disease gets its name. The shape is similar to a crescent moon. Red blood cells with normal hemoglobin (hemoglobin A, or HbA) move easily through the bloodstream, delivering oxygen to all of the cells of the body.

Normal Red Blood Cells are shaped like discs or doughnuts with the centers partially “scooped out” and are soft and flexible. They can easily “squeeze” through even very small blood vessels.

 

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