Thalassemia

Thalassemia( Cooley’s anemia ) : This is a hereditary blood disease that causes anemia. There are two forms of the thalassemia.

Thalassemia trait: People with thalassemia trait, also called thalassemia minor or carriers, carry thalassemia but they are not ill. They are absolutely healthy and normal but some of them have slight anemia. You only discover it if you a have a special blood test, or if you have a child with thalassemia major.

The red blood cells of people with thalassemia trait are smaller than usual. They also contain slightly more of a kind of hemoglobin called hemoglobin A in their blood. Thalassemia trait is present at birth. It remains the same for life and is passed on from parents to children.

It is important to know if you carry thalassemia trait because sometimes people with thalassemia trait have children with thalassemia major , a serious blood disease.

If one parent has thalassemia trait and one is not a carrier there is 1:2 (50%) chance that each of their children will have a thalassemia trait. Name of their children can have thalassemia major. If both parents carry thalassemia trait, there is a 1:2 (50%) chance that children may have thalassemia trait and 1:4 (25%) chance that they may have completely normal blood or thalassemia major.

Thalassemia major : Thalassemia major is a serious inherited anemia which begins in early childhood. Children with thalassemia major cannot makes enough hemoglobin. Because of this their bone marrow cannot produce enough red blood cells. The red blood cells that are produced are nearly empty.

The main treatment for thalassemia major is regular blood transfusions, Usually every  4 weeks. Most children who have these transfusions grow normally and live quite happily. After each blood transfusion the red blood cells in the new blood are broken down slowly releasing iron that 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 under the skin from a small pump 5-7 nights of every week.

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