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Sickle cell disease
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Inside every red blood cell there is one molecule that does all the work: hemoglobin, the one that picks up oxygen and delivers it to the cells of the body. Sickle cell disease is a group of disorders of that molecule.
People who have it make a different hemoglobin, hemoglobin S, and that version can deform the red blood cell. Instead of the flexible disc that bends and slips through blood vessels without trouble, the cell ends up stiff and shaped like a sickle.
Everything else starts there. Those stiff cells get stuck in the smallest vessels, like stones jamming a narrow pipe, and that is when the episodes of pain show up and tissues and organs are left without their oxygen-rich blood. On top of that, the deformed cell breaks apart early, and that can lead to anemia, which is having too few red blood cells. One single changed molecule, and out of it come the pain, the anemia and the organs left without oxygen.
In brief
Sickle cell disease is a group of disorders of hemoglobin, the molecule inside the red blood cell that delivers oxygen to the body.
People who have it make an atypical hemoglobin, hemoglobin S, which can give the red blood cell a sickle shape instead of the flexible disc that slips easily through blood vessels.
Those stiff cells get stuck in small vessels, causing episodes of pain and depriving tissues and organs of oxygen-rich blood, and they break down early, which can lead to anemia.
Sources (2)
- MedlinePlus, U.S. National Library of MedicineSickle cell disease. MedlinePlus Genetics
- National Heart, Lung, and Blood InstituteSickle Cell Disease
This page explains the topic in general terms; it does not tell anyone what treatment to follow.
Updated