Thalassaemia

Q: My niece is suffering from disease called thalassaemia. We are keep on hearing that there is possible treatment of the affected red cells in these patients. Please tell the status of this research, as how long will this research take more time? What are the success possiblities percentage for these cases. Please do reply. I am giving personal information about my niece here under.

A:Thalassaemia is a common inherited disorder, for which no cure has yet been found. It is estimated that in India there are over 100,000 cases of major thalassaemia and nearly 30 million carriers of the thalassaemia gene. The disease is more prevalent in certain communities like Sindhis, Punjabis, Gujaratis, Bengalis, Gaurs and Saraswats. The only treatment for Thalassaemia major is regular blood transfusions, usually every three or four weeks. Most children who have these transfusions, usually every three or four weeks grow normally and live quite happily into their early twenties. But to live longer, they need other treatment as well. After each blood transfusion the red cells in the new blood are broken down slowly over the next four months. The iron from the red blood cells stays in the body. If it is not removed, it builds up and can damage the liver, the heart and other parts of the body. At present the only way to remove the extra iron from the body is to give injections of a drug called Desferal (correct name is Desferrioxamine) This medication starts between the ages of 4 to 8 years old. Desferal is injected under the skin. This treatment is very successful and most children treated with blood transfusions and Desferal can now lead fairly normal healthy lives. Treatment today is more advanced then what it was. A Thalassaemia person can enjoy a good quality life, lively in normal activities such as sport, study, work, marriage and family.

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