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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.

Types and terminology


"Sickle-cell anemia" is the name of a specific form of sickle-cell disease in which there is homozygosity for the mutation that causes Hgb S. Other forms of sickle-cell disease include:
  • sickle-hemoglobin C disease
  • sickle beta-plus-thalassemia
  • sickle beta-zero-thalassemia

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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@monkeybrief I have the sickle cell trait---very different from sickle cell anemia--no such thing as sickness from sickle cell trait
MsRaine (Ms. Raine) Wed, 04 Nov 2009 15:46:40 -0000
@monkeybrief I have the sickle cell trait---very different from sickle cell anemia--no such thing as sickness from sickle cell trait
Someone was telling me about a friend with sickle cell anemia. What a horrible disease- the great thing is I can help her blood flow.
JocelyneWalberg (Jocelyne Walberg) Wed, 04 Nov 2009 14:20:48 -0000
Someone was telling me about a friend with sickle cell anemia. What a horrible disease- the great thing is I can help her blood flow.
Denver: Curing Sickle Cell Anemia http://bit.ly/3yrxLX
DenverCP (Denver CP) Wed, 04 Nov 2009 03:11:54 -0000
Denver: Curing Sickle Cell Anemia http://bit.ly/3yrxLX
Camp Boggy Creek accepts children that have bn diagnosed w/ life threatening illnesses such as cancer, sickle cell anemia, & epilepsy.
fhzwart (Frank Zwart) Wed, 04 Nov 2009 02:28:08 -0000
Camp Boggy Creek accepts children that have bn diagnosed w/ life threatening illnesses such as cancer, sickle cell anemia, & epilepsy.
#microbeoftheday Why has evolution not phased out sickle cell anemia? It confers resistance to P. falciparum http://tinyurl.com/ynmmg4
hithatsmybike (B. ) Wed, 04 Nov 2009 01:47:14 -0000
#microbeoftheday Why has evolution not phased out sickle cell anemia? It confers resistance to P. falciparum http://tinyurl.com/ynmmg4
By all means, lets keep comparing gay people to sufferers of sickle-cell anemia. IT IS PRACTICALLY THE SAME THING.
ClairWM (Clair McCashin) Tue, 03 Nov 2009 22:00:07 -0000
By all means, lets keep comparing gay people to sufferers of sickle-cell anemia. IT IS PRACTICALLY THE SAME THING.

 
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KidsHealth: Do You Know About Sickle Cell Anemia? - Article tells how sickle cell gets its name, causes, symptoms, tests, and treatment.
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? ]

Medline Plus: Sickle Cell Anemia - Illustrated article gives definition, causes, treatment, and risk factors.

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... ]

TeensHealth: Sickle Cell Anemia - Tells what the condition is, how it affects people, how it may be treated, and what young people can do to stay well.
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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