India has the unfortunate tag of being the thalassemia capital of the world with 40 million carriers already, and 10,000 more being added annually. While the annual blood transfusion burden is stated to be 2 million units of packed red blood cells, spends on thalassemia treatment each year is pegged at about Rs. 15,000 crores, as per a study supported by Indian Council of Medical Research and the article titled Burden of thalassemia in India: The road map for control published in the Pediatric Hematology Oncology Journal, authored by three research scholars at National Institute of Immunohaematology.
On the occasion of World Thalassemia Day, Apollo Gleneagles Hospitals Kolkata organized an awareness program for the disease in presence of doctors, patients and survivors, who shared their success stories. Case of Tapan Parua – a 16 yr old boy with Thalassemia Major, who has successfully undergone Bone Marrow Transplant from stem cells of his younger brother in Apollo Gleneagles Hospitals Kolkata, was also discussed. There were recreation programs like chess competition, magic show etc for juvenile survivors and patients. Thalassemia is a blood disorder passed down through families (inherited) in which the body makes an abnormal form of hemoglobin. Hemoglobin is the protein in red blood cells that carries oxygen. The disorder results in large numbers of red blood cells being destroyed, which leads to anemia. Hemoglobin is made of 2 proteins: Alpha globin and Beta globin. Thalassemia occurs when there is a defect in a gene that helps control production of one of these proteins.
There are 2 main types of thalassemia: Alpha thalassemia occurs when a gene or genes related to the alpha globin protein are missing or changed (mutated). Beta thalassemia occurs when similar gene defects affect production of the beta globin protein. Both alpha and beta thalassemia include the following 2 forms: Thalassemia major and Thalassemia minor.Dr Soumya Bhattacharya, Senior Haemato-Oncologist, Apollo Gleneagles Cancer Hospital, Kolkata “Preventive health checkups is the need of the hour – it’s crucial that two beta thalassemia carriers don’t marry each other to prevent birth of a beta thalassemia major. If they do, then there is a 25% chance of the baby being thalassemia major. Hence the only way to prevent increase in number of such patients is to make screening mandatory before marriage. Managing the disease is extremely expensive apart from being painful for the patients so it is imperative to have pre-natal diagnosis as well”
In case no screening has been done before birth and a child is diagnosed with thalassemia, still a proper management can help the child to live a near normal life. This would include regular blood transfusions the quality of which is very important. Apollo Gleneagles Cancer Hospital provides NAT tested antigen matched blood. This greatly reduces the risk of viral infection and development of antibodies. In addition to transfusion, it is also crucial to monitor bone health, regular growth and pubertal development. Iron chelation (removal of excess iron from the body) is extremely important else it can cause liver and heart failure. Hence thalassemia requires comprehensive management to ensure a steady progress in life. With proper management of the disease and with multidisciplinary approach including cardiac, endocrinology, gastro and gynae care, women can even plan for motherhood.
Dr Shilpa Bhartia, Haemato-Oncologist, Apollo Gleneagles Cancer Hospital, Kolkata, “The main cure available today for Thalassemia is Bone Marrow Transplantation (BMT). Human leukocyte antigen (HLA) typing is used to match patients and donors for BMT. The replacement marrow for the stem cell transplant can be procured from an HLA matched related or unrelated donor. Thankfully, newer drugs used during transplants have reduced the risk of BMT related complications significantly with cure in more than 80% of the patients, so patients can choose BMT without any worry”
Dr Anupam Chakrapani, Haemato-Oncologist & Program Coordinator – Bone Marrow Transplant, Apollo Gleneagles Cancer Hospital, Kolkata“Thalassemia is one of those diseases, which can be easily prevented, but often due to socio-cultural barriers and stigma associated with it, the progress on preventing births of thalassemia major children is slow. Parents generally do not wish to reveal the carrier status of their children because they fear difficulty in getting them married but it is important that we change this mindset.”