Annexure-II


Thalassemia

Thalassemia is a group of inherited blood disorders characterized by mild to severe anaemia caused by haemoglobin deficiency in the red blood cells. In individuals with thalassemia, the production of the oxygen carrying blood pigment haemoglobin is abnormally low. There are two main types of thalassemia: alpha thalassemia and beta thalassemia. In each variant a different part of the haemoglobin protein is defective. Individuals with mild thalassemia may have symptoms, such as anaemia, enlarged liver and spleen, increased susceptibility to infections, slow growth, thin and brittle bones, and heart failure.

Facts about Thalassemia

Thalassemia is a serious Inherited Blood Disorder.

4.5% of world population (250 million) suffering with thalassemia minor. There are over 35 million Indians are carriers of the abnormal Gene for Thalassemia. It is estimated that about 1,00,000 infants are born with major Haemoglobinopathies every year in the world.

10,000 – 12,000 Thalassemic children are born every year in our country. Survival depends upon repeated blood transfusion and costly medicines. Thalassemia can be prevented by awareness, pre marital or pre conceptual screening followed by antenatal diagnosis is required


Treatment

Thalassemia major should be diagnosed as early as possible in order to prevent growth restriction, fragile bones and infections in the first year of life. The infant’s haemoglobin levels and development should therefore be monitored closely. If Hb is less than 70% or the child shows signs of poor growth and development. Regular transfusion is the treatment of choice. According to the WHO, the aim of this treatment is to retain a median haemoglobin value of 115–120 grams per liter. This can usually be achieved by carrying out transfusions of concentrated red blood cells at intervals of every three to four weeks.

Today thalassemia major can be cured by stem cell transplantation. A prerequisite is usually that the affected individual who has siblings with identical tissue type (HLA type) a transplantation of blood stem cells referred to as a “bone marrow transplant”, can be carried out.




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