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Sickle Cell disorders
Background
Sickle Cell disorders: Sickle Cell disease
Sickle Cell disorders are a group of inheritable genetic conditions in which there is an abnormality of the haemoglobin. Haemoglobin carries oxygen to the various organs of the body and is contained in the red blood cells. In the sickle cell disorders some of the red blood cells assume a sickle shape following the release of oxygen. This abnormal shape causes the cells to clump together making their passage through smaller blood vessels difficult, which may lead to blockage of these small blood vessels and an associated inflammatory reaction.
The most common Sickle Cell disorder is Sickle Cell anaemia (Sickle Cell anemia - US), Hb SS, and in the UK the next most common are Hb SC disease (also called Sickle Hb C disease or Hb SC) and sickle beta thalassaemia (Hb S/beta thal).
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