Sickle cell disease is an inherited blood disorder. Normally, red blood cells are disc-shaped and flexible. In sickle cell disease, hemoglobin, which is the chemical within red blood cells that carries oxygen around the body, is abnormal. This causes red blood cells to collapse into a crescent or sickle shape. It also causes the red blood cells to be abnormally stiff and fragile. These cells clump together and clog up small blood vessels throughout the body. When blood vessels are blocked by sickle-shaped red blood cells, parts of the body are deprived of oxygen. This can cause severe pain and damage to organs and tissues. Abnormal red blood cells are also destroyed at a high rate, causing a shortage of red blood cells called anemia.
Red Blood Cells: Normal and Sickled
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Sickle cell disease is a genetic disorder. If you receive one defective gene from each of your parents, then you will have sickle cell disease. If you only have one defective gene, you are said to have sickle cell trait, but not sickle cell disease. Although you won’t usually have any symptoms, you can pass this gene on to your children.
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http://www.ebscohost.com/dynamed. Updated June 6, 2013. Accessed July 1, 2013.
Sickle cell disease. Nemours' KidsHealth.org website. Available at:
http://kidshealth.org/parent/medical/heart/sickle_cell_anemia.html. Updated September 2012. Accessed July 1, 2013.
Sickle cell disease (SCD). Centers for Disease Control and Prevention website. Available at:
http://www.cdc.gov/ncbddd/sicklecell/index.html. Updated September 27, 2012. Accessed July 1, 2013.
What is sickle cell anemia?
National Heart, Lung, and Blood Institute
website. Available at:
http://www.nhlbi.nih.gov/health/health-topics/topics/sca/. Updated September 28, 2012. Accessed July 1, 2013.
Last reviewed July 2013 by Michael Woods, MD
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