The sickle cell disease causes the genetic disorders due to the sickle hemoglobin.
A change in the shape of the red blood cells is the primary feature of this disease.
The change in shape results due to the deoxygenation caused by the polymerization of the abnormal hemoglobin.
Instead of the normal shape and size the cells become bigger and take a sickle shape.
Causes of Sickle Cell Disease
The causes of this sickle cell disease are related to the inheritance of the hemoglobin gene responsible for developing this disease. As mentioned above the red blood cells transform into an abnormal shape.
The membrane gets damaged and as a result these cells get stuck up in the blood vessels blocking the way of blood flow. This leads to immense pain and also the organs are affected as oxygen supply is not sufficient.
This sickle cell disease leads to anemia as the cells have low life span. Normally the red blood cells life span is about 120 days but these sickle cells just live for 10-20 days.
Sickle Cell Disease Symptoms
The sickle cell disease patients experience anemia and the symptoms vary depending on the severity of the disease. The level of the Patients typical hemoglobin ranges from 6-9 g/dl. Anemia is a result of reduced life span of the red blood cells.
During this condition the reticulocyte counts are also elevated, reflecting new red blood cells replacing the rapidly destroyed older cells. The vaso-occlusion occurs due to the increased count of white blood cell and platelet.
Sickle Cell Disease Treatment
The bone marrow transplant is the only method of sickle cell disease treatment however this method also does not cure the disease. These bone marrow transplants are rather complicated procedures which are not opted for all the diseased patients. Before undergoing the transplant the appropriate donor is must.
The donor should match the bone marrow of the patient with a very low risk of rejecting. But unfortunately many cases are reported not successful due to mismatch of bone marrow and rejection.
Good news about this disease is that even without a cure many patients be it children or adults are able to lead a normal life through proper medications and a proper diet and supplements.
These steps are able to control the pain to large extent and avoid the other complications of the disease along with decreasing the chances of anemia. Folic acid, in particular, can help the sickle cell disease patient produce new red blood cells decreasing the chances of developing anemia.
The infection can be very well prevented by immunizations and daily doses of penicillin. Some patients may receive regular transfusions of red blood cells to prevent or treat these complications.
Hydroxyurea is a drug that has been used in patients over 18 years old, however, it has still not been approved for use in children. This drug is said to give the patients a better life suffering from sickle cell disease. It has helped in decreasing the pain interfering with the sickling process of sickle cell disease.
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