Sunday, November 25, 2007
Malaysia, Penang, 17-19 Nov 2007: Living With Thalassaemia
Drugs, Needles & Syringes
Thalassaemia is the commonest gene disorder in the world. In South-East Asia, about 1 in 20 persons carry the genes that can cause the disease. Thalassaemia major occurs when a child inherits affected genes from both parents, who may themselves be healthy carriers. A major thalassaemic suffers severe anaemia and requires  approximately monthly transfusions from infancy. While life-sustaining, cumulative blood transfusions over a prolonged period results in iron deposition in various organs in the body. The iron is toxic, and causes heart, liver and endocrine organ dysfunction. Indeed, heart failure from heart iron overload is the commonest cause of premature death in thalassaemia major.The body is evolutionarily programmed to conserve iron: except for menstrual blood loss, there exists no physiological process to rid the body of excess iron. With regular blood transfusions, many major thalassemics survive childhood only to die from the complications of organ iron overload in their teens and young adulthood. Since the mid 1970s, the iron chelator desferrioxamine has been used to remove excess body iron in thalassaemia major. The drug, which induces negative body iron balance only if given continually throughout life, has to be administered subcutaneously for 8 to 12 hours, at least 5 nights a week. Patients typically start desferrioxamine injections from childhood. Nowadays, there are 2 other iron chelating drugs: deferiprone and deferasirox, both oral drugs. Deferiprone appears to be very good at eluting iron from heart muscle, especially when used in combination with desferrioxamine. A few patients develop dangerously low levels of white blood cells with the drug, which limits its use. Deferasirox is a new oral chelating agent with similar efficacy to parenteral desferrioxamine. It is forbiddingly expensive, and out of the economic reach of most thalassaemia major patients. The Penang Thalassaemia Society is a NGO that is dedicated to promoting the cause of thalassaemia patients in North Malaysia. The committee members comprises patients, parents, medical personnel and other volunteers. Through private charity, it has achieved remarkable success in funding expensive patient treatment: Penang  thalassaemia patients have access to state-of-the-art medical care. Further, the Society organizes camps for patients and families to educate them about the disease and its treatment.I attended the 11th Penang Thalassaemia Camp on 17-18th Nov 2007, my second time in 2 years. The theme focused on compliance to iron chelation treatment. It was a humbling experience to watch the thalassaemia major patients perform their personal nightly ritual of desferrioxamine injection in a communal setting. For one child, it was the first time the parent learnt to prepare the desferrioxamine solution and inject the drug into the abdomen of the child. For another child, it was to be the first time she injected into herself. Alas, she could not do it at the last minute. Mummy did it for her that night, the nth thousandth time. There will always be tomorrow. |
Labels: friends, Malaysia, Penang, people
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