Sickle cell anemia is a blood disease that affects red blood cells. Normal red blood cells are round. In people with sickle cell anemia, hemoglobin – a substance in red blood cells – becomes defective and causes the red blood cells to change shape. The faulty hemoglobin is called hemoglobin S, (HgbS) and it replaces normal hemoglobin called: hemoglobin A (HgbA). Over time, the red blood cells become rigid and shaped like crescent moons or sickles.
The sickle-shaped red blood cells:
Clog blood vessels, causing episodes of pain and cutting off oxygen to tissues and organs.
Get trapped in the spleen (an organ that gets rid of old cells) where they are destroyed. The body cannot replace the lost cells fast enough. As a result, the body has too few red blood cells, a condition known as anemia.
Sickle cell anemia is a serious disease that can require frequent hospital stays. Children and young adults can die from the disease.
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