World Thalassemia day !

May 8 marks Thalassemia Day and it is estimated that nearly one lakh Indians suffer from this dreaded disease with more than 8,000 thalassemic births recorded every year in India.

Thalassemia is a hereditary blood disorder wherein the patient has to undergo repeated blood transfusions as the body cannot produce enough haemoglobin.

Problems of a thalassemic's family are compounded by the fact that repeated blood transfusions leads to accumulation of excessive iron in the heart, liver and other vital organs causing an 'iron overload'.

Apart from the psychological and emotional trauma, thalassemia also poses a huge financial burden for the family. The costs of chelation for removing excessive iron and for blood transfusions is close to Rs 10-15,000 per month which can be prohibitive for families with limited means.

Chelation injections cost Rs 2 lakh per year. Seema Arya, mother of an eight-yr-old thalassemic said that as a parent a lot of counselling is required for supporting the child as he has to endure regular injections that are slowly injected to remove the iron deposits sometimes lasting upto 12 hours or more.

Essentially the red blood cells are very fragile and are subject to early destruction, whereas the normal life span is three weeks. This means frequent blood transfusions and the necessity of accessing safe blood everytime a transfusion takes place.

Otherwise the patient is susceptible to the added risk of contracting Hepatitis and HIV/AIDS. Hence proper screening of blood and blood banks is very important.

Another problem is that thalassemia is often confused with anaemia and timely medical intervention is sometimes not available. Without treatment a child with this chromosomal defect cannot survive for more than 20-30 days.

Lack of awareness regarding this genetic disorder is exacerbating the spread of thalassemia. Cyprus is one of the countries where the disease was widely prevalent, but with timely intervention of the government, including sustained mass awareness campaigns, thalassemia has been contained.

In this context it is very important that thalassemia minor carriers be identified through a simple blood screening.

Nearly 6 per cent of the Indian population are T-minor carriers of the thalassemia gene defect. Film stars like Amitabh Bachchan and Amisha Patel are thalassemia minors, but are leading perfectly active and normal lives.

The only precaution that T minors with this genetic defect need to take is not marrying another T minor so that their progeny is not born a T major and is protected from this disease.
Bone marrow treatment and stem cell replacement are two alternative therapies that are also being explored but the costs are prohibitive.