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What are the causes and treatment for sickle cell anaemia?

Q: What are the causes and treatment of sickle cell anaemia? I want the information regarding an adult female who is suffering badly. Please advise.

A:Sickle cell disease is a group of inherited disorders with abnormalities caused by the presence of an abnormal haemoglobin called haemoglobin S (Hb S). Sickle cell anaemia, in which only haemoglobin S is produced (Hb SS), is the severest form while individuals who produce some normal adult haemoglobin have much less severe disease (Hb AS). Affected individuals suffer from a wide range of clinical problems that result from vascular obstruction and lack of blood supply to affected areas (ischaemia). Although the disease can be diagnosed at birth, clinical abnormalities usually do not occur before the age of 6 months, when the function of the spleen is affected, leading to susceptibility to overwhelming infection with certain respiratory bacteria. Subsequently, damage to other organs occurs. Typical manifestations include recurrent pain and progressive incremental infarction. The abnormal haemoglobin carries oxygen normally but begins to form semisolid aggregate structures once oxygen is unloaded to the tissues. These haemoglobin S aggregates distort the red blood cells and cause them to lose their normal elasticity. At first, haemoglobin S retains its ability to return to its soluble form, and the red cells can regain their elasticity upon reoxygenation. However, this process damages the red cell, and with repeated deoxygenation cycles, permanent red-cell damage ensues. The resultant rigid cells are responsible for, or participate in, the blocking small capillaries (vascular occlusion) and lack of blood supply to tissues that is the basis for abnormalities typical of this disease. Treatment is aimed towards relieving anaemia, preventing/treating infections, relief of pain, preventing complications like stroke, gall bladder infection, bone disease, etc. You need to discuss with your treating physician and haematologist to plan for future.


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