What is sickle cell anemia?
Sickle cell anemia is a serious genetically inherited blood disorder which affects hemoglobin, the oxygen-transport molecule in red blood cells. Due to miscoding of a single nucleotide and ultimately a single amino acid, the hemoglobin protein is folded abnormally. i The body, in turn, produces crescent-shaped red blood cells rather than the normal doughnut-shaped red blood cells.
Red blood cells contain hemoglobin, which carries oxygen from the lungs to the rest of the body. a. Red blood cells also help to remove carbon dioxide from the body, although play a lesser role than they do in oxygen transport.
Crescent, or “sickle” shaped, cells, on the other hand, do not move easily through the blood vessels.ii Because they are stiff and sticky, these cells often clump together and form blockages in the vessels. These blockages deprive many parts of the body of oxygen which causes severe pain, serious infections, and even organ damage.iii
People who suffer from sickle cell anemia have low red blood cell counts because their abnormally-shaped, stiff, sticky red blood cells survive only 10-20 days compared to the 120-day lifespan of healthy red blood cells.iv The bone marrow cannot manufacture replacement red blood cells fast enough to compensate.