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What is sickle cell disease?

Sickle cell disease (SCD) is an inherited blood disorder that affects the red blood cells. It is inherited from both parents and can affect both males and females. Among African Americans in the United States, 1 in 10 persons are carriers of the sickle gene and 1 in 400 has sickle cell disease. Because the symptoms of SCD vary from person to person, no two children with SCD will necessarily have the same problems or the same intensity of a problem.

The science behind the disease: Red blood cells carry oxygen from the lungs to all parts of the body. Red blood cells travel in arteries and veins (blood vessels). Our bodies need oxygen for every function: eating, walking, even sleeping requires oxygen. Normal red blood cells are soft and round and can squeeze through blood vessels. Normal red blood cells live for about 120 days before new ones are made by the body to replace them.

People with sickle cell disease have red blood cells that are crescent shaped (sickle-shaped) and are stickier. These sickle red blood cells get stuck in the small blood vessels in the body. In addition, these sickle red blood cells do not live as long as normal red blood cells (only about 16 days). As a result, people with sickle cell disease are anemic meaning they have less blood in their bodies than people without SCD.

 

 

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