Thalassaemia

What Is Thalassaemia?

 

Thalassaemia is a genetic blood disorder, transmitted to the offspring from their parents. Due to this disease, haemoglobin which is contained in every RBC, is abnormally produced so the RBCs are misshapen and are broken down soon after their formation. This leads to a drop in Hb% and the resulting anaemia needs to be treated with blood transfusion.



Facts About Thalassaemia

 

5-7% of our population (approx 9~13 million) are carrying genes of this disorder and are known as Thalassaemia minor. They do not need blood transfusions themselves but due to a by chance marriage of two such persons, they may give birth to a child with Thalassaemia major, who will need regular blood transfusion for the rest of their life. Due to a high carrier rate in our society more than 6,000 - 7,000 children with Thalassaemia major are born every year in Pakistan. As a rough estimate, at any given time, more than 120,000 Thalassaemia major children may be found in our country, getting half hearted treatment with infrequent blood transfusions and inadequate iron chelation therapy. For optimum treatment every Thalassaemic child will need frequent transfusions of screened packed red cells and regular iron chelation therapy. This will cost more than Rs.10,000 - 16,000 per month.

 

If we start managing all these Thalassaemia major patients on internationally accepted guide lines,Rs.14.0 billion will be required each year, which is out of reach of our health budget and an impossible target at the present moment. More over all the items used for treating these children are imported. Empty blood collection bags, Blood screening kits for Hep.B, Hep.C, HIV, VDRL, Malaria Parasites and Iron chelating agents are all imported and need foreign exchange for purchasing these items

 

This situation leaves only one alternative - stop birth of Thalassaemic children.

 

This is possible. Countries like Cyprus and Turkey where carrier rates were very high and 18% of population carried different genes of haemoglobinopathies, have completely stopped birth of new Thalassaemic children. In Iran, compulsory pre-marriage blood tests and genetic counselling have resulted in very high success rate. In our country due to illiteracy and poverty this problem needs to be tackled in a little different way.