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 (anemia).
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.
Facts about sickle cell disease. Centers for Disease Control and Prevention website. Available at: http://www.cdc.gov/ncbddd/sicklecell/facts.html. Updated November 17, 2016. Accessed December 13, 2016.
Sickle cell disease. Kids Health—Nemours Foundation website. Available at: http://kidshealth.org/en/parents/sickle-cell-anemia.html. Updated September 2012. Accessed July 1, 2013.
Sickle cell disease in adults and adolescents. EBSCO DynaMed website. Available at: https://www.dynamed.com/topics/dmp~AN~T902929/Sickle-cell-disease-in-adults-and-adolescents. Updated October 29, 2016. Accessed December 13, 2016.
Sickle cell disease in infants and children. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T902928/Sickle-cell-disease-in-infants-and-children. Updated September 20, 2016. Accessed December 13, 2016.
Sickle cell trait. Centers for Disease Control and Prevention website. Available at: http://www.cdc.gov/ncbddd/sicklecell/traits.html. Updated September 14, 2015. Accessed December 13, 2016.
What is sickle cell disease? National Heart, Lung, and Blood Institute website. Available at: http://www.nhlbi.nih.gov/health/health-topics/topics/sca. Updated August 2, 2016. Accessed December 13, 2016.
Last reviewed December 2016 by Marcin Chwistek, MD
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