Thalassaemia & Haemoglobinopathy Clinic – Striving to transform the lives of thalassaemic patients

Thalassaemias are a heterogenous group genetically transmitted red blood cell related disorders. It is characterised by varying degrees of anaemia and end organ failure. The basic pathology lies in the defective synthesis of haemoglobin, which is either qualitatively and/or quantitatively deficient. This deficiency leads to destruction of the defective red cells in the bone marrow and peripheral circulation leading to anaemia. The degree of the defect is extremely variable and so is the destruction of red cells. This peculiar variation in presentation makes it as a unique disorder where some affected present to the healthcare set up as early as 3 to 4 months, whereas some do not present to the healthcare set up at all, due to extreme mildness of the symptoms.

India has the largest number of children with thalassaemia major in the world – about 1 to 1.5 lakhs and almost 42 million carriers of Beta-thalassaemia trait. About 10,000 to 15,000 babies with thalassaemia major are born every year. In India, Beta-thalassaemia is prevalent across the country, with an average frequency of carriers being 3 to 4%. A higher frequency has been observed in certain communities, such as Sindhis, Punjabis, Gujaratis, Bengalis, Mahars, Kolis, Saraswats, Lohanas and Gaurs. Haemoglobinopathies is highly prevalent in the tribal populations of Southern, Central and Western states reaching as high as 48% in some communities.

This disease at present has no easy cure. The patients have to survive on regular safe blood transfusion and ancillary therapy. As iron is principal constituent of “haemoglobin”, the red pigment responsible for carrying oxygen, its destruction leads to iron overload also contributed to by the senile destruction of the transfused red cells and that, which is absorbed from the diet. This unbound iron is toxic, and gets ubiquitously deposited in various organs like heart, pancreas, pituitary and other endocrine organs, jeopardising their functional capacity. This leads to fatal complications if left untreated.

The uniqueness of this genetic disorder is that, it is carried by healthy individuals, who look perfectly normal and have a healthy life. If such a healthy carrier has a conjugal relationship with another such carrier, they may produce a baby with the disorder, with a chance factor of 25% in each pregnancy, for that couple.

Armed with this knowledge, it is fair to have 2 modus operandi:

• To maintain the already born thalassaemic children as near optimum health as possible, such that they can have a socially and economically productive life.
• To bring down the birth rate of new thalassaemia patients

To maintain the already born thalassaemic children as near optimum health as possible, such that they can have a socially and economically productive life.

To enable access of such patients to safe, correct and adequate blood components provided through state-of-the-art blood transfusion service.

To keep an eye on different organ systems by way of performing different haematological, biochemical and sero-immunological tests at regular intervals for prediction and management of complications.

Regular monitoring of iron overload by measurement of Serum Ferritin for estimation of the generalized iron overload T2* and R2* by MRI for measurement of organ specific iron overload and subsequent response to chelation therapy.

Prediction and management of Cardiac, Endocrinal, Orthopaedic, Gastroenterological, Growth & Development, Gynaecological and Infectious complications through multidisciplinary approach under one roof.

Why opt for the Thalassaemia and Haemoglobinopathy Clinic in Peerless Hospital?

• Availability of safe and adequate blood components through the Blood Bank and special components of blood for the patients.
• A blood transfusion unit dedicated to serve the needs of the thalassaemia patients
• Provides organ specific iron overload detection facility for Cardiac, Pancreatic, Hepatic and Pituitary gland. This is done with the help of 1.5T MRI machine and dedicated T2* and R2* software. This service is being provided for the first time in the east zone, which would benefit the patients from the neighbouring countries of Nepal, Bangladesh, Bhutan and Maldives in addition to the local ones.
• Provided outdoor and indoor patient care.
• Provides multidisciplinary approach for this multi-system disorder through medical board comprising of experts from different fields, for such patients on regular basis which could have been possible only in a multidisciplinary hospital like Peerless.
• Provides prenatal diagnostic and therapeutic facilities, marriage and pregnancy counselling facilities.