WORLD THALASSEMIA DAY Are you living with thalassemia?

Today is World Thalassemia Day

World Thalassemia Day is celebrated on May 8 every year to commemorate thalassemia victims and encourage the ones who struggle to live with the disease. An inherited blood disorder, thalassemia causes the body to have less haemoglobin than normal. Haemoglobin enables the red blood cells to carry oxygen. Thalassemia can also cause anaemia and leave you fatigued though mild thalassemia may not need any treatment. The more severe forms may require regular blood transfusions and you can take steps to cope with fatigue, such as choosing a healthy diet and exercising regularly.​

Dr Rahul Bhargava

Thalassemia is an inherited blood disorder in which patients requires blood transfusion at regular interval for whole life until they can be either cured by a bone marrow transplant or gene therapy. With each unit of blood goes 200 mg of iron which tends to get deposited into various parts of the body, leading to short stature, blackly pigmentation, dysmorphic, increased in size, liver enlargement and cardiac failure leading to death.

Iron deposition can be prevented by taking iron chelation agents, either orally or parenterally lifelong. Bone marrow transplant is a curative procedure where source for stem cell could be either brother or sister or from various registries Indian or internationally.

Bone marrow transplant can cure 80 per cent thalassemic babies, if done early. Over the past many years, the Government of India has taken innumerable constructive steps like free blood transfusion and regular supply of free iron chelating medicines. This has led to improvement in quality of life and better survival.

There have been two new developments that have the potential to change the way we treat thalassemia. One is gene therapy which is now on more firm footing, showing persistence of haemoglobin production over two years without causing any malignancy or other side effects. The other is a new drug called luspatercept which is given under the skin. It has shown a reduction in blood transfusion requirement in 70 per cent cases. For a resource limited country like ours, prevention will always remain the best the cure.

If both husband and wife are thalassemia-carriers then there are 25 per cent chance of baby having thalassemia major. In India, 10,000 thalassemia babies are born each year which leads to a burden on the families and parents.

The need of the hour is universal screening of all pregnant females between their nine and 12^th week of pregnancy. This way, we will be to achieve a target of thalassemia-free India.

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Dr Bhargava is Director and Head, Haematology, Haemato-Oncology
and Bone Marrow Transplant, Fortis Memorial Research Institute, Gurugram