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Like other parts of the globe, the 'International Thalassemia Day' was observed here on Saturday with a renewed pledge to create awareness among the people so as to check the burden of disease. To mark the day, different health organisations organised functions to raise awareness about the disease, as every year, around 6,000-7,000 children are born with Thalassemia major in Pakistan, while five to six per cent of the total population is Thalassemia minor.
Sundas Foundation in collaboration with Pakistan Medical Society (PMS) organised a function. Senior Minister Raja Riaz Ahmed was the Chief Guest, while Sundas Foundation Chairman Munno Bhai presided. Addressing on the occasion, Munno Bhai said, "Thalassemia is most common genetic disorder in the world and around 250 million people that is 4.5 percent population of the world is affected by Thalassemia. Thalassemia is more common in the people who are originals of Thalassemia belt ie Mediterranean countries like Cyprus, Sardinia Greece, middle-east, Pakistan, India, Burma and Thailand."
Munno Bhai said that Sundas Foundation is a premier organisation working on voluntary basis and extending services to Thalassemia affected children and other haematological disorders. He added that Thalassemia major occurs when a child inherits two Thalassemia genes, one from each parent. When the couple is Thalassemia carrier there is 25 percent chance of having a Thalassemia Major Child, 50 percent Thalassemia carrier/ Minor like parents and 25 percent Normal ie not even a carrier. And if only one or none of the couple is a carrier, none of the child will be a Thalassemia Major.
Pakistan Medical Society Chairman Dr Masood Akhtar Sheikh said that lack of awareness as well as means to treat the disease is the cause of greater incidence of Thalassemia. "Iran, Saudi Arabia, Malaysia, Egypt and India are among the few countries that have controlled the disease through legislation and public awareness. All unmarried persons and those married persons who are planning to have a child (even though their existing children are normal) should get themselves tested for Thalassemia carrier status by a blood test called HbA2, Hb Electrophoresis or Hb HPLC," he said.
Dr Masood Sheikh added that Thalassemia Major patients require life long repeated blood transfusions and medicines for their survival. "Thalassemia is 100 percent preventable", while one cannot always prevent malaria, typhoid, Hepatitis B, C and HIV since there is always risk of exposure to these infections and no vaccine is 100 percent effective. Thalassemia can be easily prevented by pre-marital/pre-conceptual or early pregnancy screening followed by marriage counselling and antenatal diagnosis if required," he said.
He further said that Thalassemia can be cured by Bone Marrow Transplantation but it requires HLA matched sibling. However HLA matched donor is not always available and the procedure is very risky. Thalassemia screening can be a part of Anemia screening and now even HbA2 estimation is also available in all major towns and cities. It costs just around Rs 700, which is nothing when compared with life long trauma of repeated blood transfusions, hospital visits, daily injections and costly medicines.
Iron overload is a cumulative, potentially life-threatening, unavoidable consequence of chronic blood transfusions used to treat Thalassemia if left undiagnosed or untreated, the excess iron in the body is likely to lead to damage of the liver, heart, endocrine glands, and other organs in the body. The body has no inherent mechanism to remove excess iron, so iron chelation is used as an effective treatment for transfusion-related iron overload, he added.

Copyright Business Recorder, 2010

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