Thalassaemia is an inherited disease of the blood. It reduces the amount of hemoglobin the body can make therefore it causes anemia. In order to treat this anemia, the traditional treatment consists of:
- Blood Transfusion
- Removing the spleen (splenectomy)
- Desferal Treatment
Precisely, this treatment does not involve blood transfusion, but the transfusion of red blood cells. Thalassaemics are only short of red blood cells but they make the other parts of the blood quite normally. Blood transfusion should be arranged to keep the child’s hemoglobin in the normal range (between 11 – 14 g/dl). So thalassaemics should be transfused when the hemoglobin is around 70% and it should be raised to around 100%.
Due to repeated transfusions, iron gradually accumulates in the body. It is stored in certain organs, particularly the liver, heart and endocrine glands and can cause damage. Therefore, the drug ‘Desferal’ is used regularly to keep the amount of iron in the body of a thalassaemic down to a safe level.
If the treatment of Thalassaemia is not proper, the spleen whose normal job is to destroy red blood cells in the circulation, begins to destroy young red blood cells as well. Therefore, transfusion becomes less and les effective. Then it becomes necessary to take the spleen out which is called ‘Splenectomy”.