Sickle-cell disease is a general term for a group of genetic disorders caused by sickle hemoglobin (Hgb S or Hb S). In many forms of the disease, the red blood cells change shape upon deoxygenation because of polymerization of the abnormal sickle hemoglobin. This process damages the red blood cell membrane, and can cause the cells to become stuck in blood vessels. This deprives the downstream tissues of oxygen and causes ischemia and infarction. The disease is chronic and lifelong. Individuals are most often well, but their lives are punctuated by periodic painful attacks. In addition to periodic pain, there may be damage of internal organs, such as stroke. Lifespan is often shortened with sufferers living to an average of 40 years. It is common in people from parts of the world where malaria is or was common, especially in sub-saharan Africa or in descendents of those peoples. Sickle-cell disease can occur in any individual of any color or ethnicity, however.
Unlike sickle-cell anemia, these other forms of sickle-cell disease are compound heterozygous states in which the person has only one copy of the mutation that causes Hgb S and one copy of another abnormal hemoglobin gene. It is important to know that "sickle-cell anemia" is the proper name of a specific type of "sickle-cell disease", and that "sickle-cell disease" is a non-specific term. Because the different forms of sickle-cell disease are quite different, one must be sure to specify the exact form of the disease in question.
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Meta Description: [ Sickle cell anemia gets its name because a person's red blood cells turn from a doughnut shape into a curved sickle shape. What's a sickle look like? ]
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Sickle Cell Society - A UK-based charity which provides care and information. Site includes research findings, pain management techniques, and teaching resources.
Meta Description: [ Sickle Cell Society - UK based charity which provides care and information on sickle cell anaemia (anemia) and other sickle cell disorders to sufferers, health professionals, students and everyone interested in understanding this genetic disease. Includes up to date research findings and pain man... ]
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Meta Description: [ Sickle cell anemia occurs when a person inherits two abnormal genes that cause their red blood cells to change shape. Find out more about what sickle cell anemia is in this article for teens. ]
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