Two different treatments are available at present, traditional treatment, and Bone-marrow Transplantation. The traditional treatment consists of three patterns:
1. Blood Transfusion
Blood Transfusion is one of the most regularly practiced treatments for Thalassemia. To be precise, the treatment is not blood transfusion, but transfusion of red blood cells. These transfusions are necessary to provide the patient with a temporary supply of healthy red blood cells with normal hemoglobin capable of carrying the oxygen that the patient’s body needs.
Today, most patients with a major form of Thalassemia receive red blood cell transfusions every two to three weeks. There are three reasons for blood transfusions.
a. To correct anemia and make sure that tissues get a normal amount of oxygen. This allows thalassemics to live and grow normally.
b. To let the bone marrow rest, so that the bones can develop normally and do not get deformed.
c. To slow down or prevent any increase in the size of spleen.
2. Splenectomy (Removing the Spleen)
When the spleen becomes too active and starts to destroy the red blood cell, transfusions become lesser and less effective. Then it may become necessary to take the spleen out through surgery. This operation is called “Splenectomy”.
3. Desferal Treatment
Blood transfusions bring extra iron into the body and if transfusions are regular, iron gradually accumulates in the body. It is stored in certain organs, especially the liver, the heart, and the endocrine glands. The iron behaves like a foreign body, and in the end would damage the organs where it is deposited. Fortunately, there are drugs that help the drainage of iron out of the body. The medication used very regularly is Desferrioxamine, more commonly called ‘Desferal’. Desferal keeps the amount of iron under a safe level in a Thalassemic’s body.
A Thalassemic’s bone marrow is not able to make a normal amount of red blood cells. If the malfunctioning bone marrow can be replaced with a normal bone marrow, this problem is solved.
At present, only young people with a fully compatible donor can have a bone-marrow transplant. A transplant in Pakistan (Zia-ud-Din Hospital, Karachi) or India (Apollo Hospital, Chennai) costs the equivalent of $13,000 to $14,000 (Pak Rs. 9-10 Lakh). A transplant in any other country may vary in cost.
A difficult question perhaps, but Thalassemic’s and their families must be aware of this subject. The illness and its implications are changing almost from day to day, due to advances in treatment. With timely and correct treatment, Thalassemic’s live longer and healthier now. It is reasonable to think that people with Thalassemia, well treated from the beginning, may live as long as people without Thalassemia, mean an excellent life-expectancy.
Quality of life
A chronic illness always causes some limitation of life, especially when it requires frequent and complex treatment, as Thalassemia does.
But still, the treatment should not be allowed to have a profound effect on a Thalassemic’s life. In particular doctors and hospitals should make the effort to arrange out-patients visits for transfusions so that they interfere as little as possible with normal life. Treatment should not interrupt schooling or work. To manage this, some centers arrange transfusions on week-ends, others in late afternoon or at night.
Apart from a few cases, most Thalassemic’s lead a normal life. They go to school, take part in social activities and work, get engaged, and get married like everyone else. We are certain that, as time passes, the quality of life will steadily improve.