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Submitted by brettkc on March 12, 2005
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Sickle cell anemia is a hereditary 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 - and one in 12 African Americans - have sickle cell trait (this means they carry one gene for the disease, but do not have the disease itself).
Sickle cell anemia occurs when a person inherits two abnormal genes (one from each parent) that cause their red blood cells to change shape. Instead of being flexible and round, these cells are more rigid and curved in the shape of the farm tool known as a sickle - that's where the disease gets its name. The shape is similar to a crescent moon.
What Is Sickle Cell Anemia?
Sickle cell anemia is a blood disorder that affects hemoglobin (pronounced: hee-muh-glow-bin), a protein found in red blood cells that helps carry oxygen throughout the body.
Red blood cells with normal hemoglobin (HbA) move easily through the bloodstream, delivering oxygen to all of the cells of the body. Normal red blood cells are shaped like doughnuts with the centers partially scooped out and are soft and flexible.
Sickle cell anemia occurs when an abnormal form of hemoglobin (HbS) is produced. HbS molecules tend to clump together, making red blood cells sticky, stiff, and more fragile, and causing them to form into a curved, sickle shape. Red blood cells containing HbS can go back and forth between being shaped normally and being sickle shaped until they eventually become sickle shaped permanently. Instead of moving through the bloodstream easily, these sickle cells can clog blood vessels and deprive the body's tissues and organs of the oxygen they need to stay healthy.
Unlike normal red blood cells that last about 4 months in the bloodstream, fragile sickle cells break down after only about 10 to 20 days, which usually...
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