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It is one form of sickle cell disease, a category of blood disorders caused by defective hemoglobin. Hemoglobin is a substance in all red blood cells that enables them to carry oxygen from the lungs, through the bloodstream, to all parts of the body (systemic circulation). With Sickle Cell Anemia, the hemoglobin in the red blood cells is defective, instead of being smooth and donut or disc-shaped, the red blood cells become stiff and sickle-shaped. Therefore, organs and tissues are deprived of oxygen-rich blood, which produces periodic extreme episodes of pain and permanent damage to vital organs (NIH, 1987)...
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