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 8~10 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 5000 children
with Thalassaemia major are born every year in Pakistan. As a rough
estimate, at any given time, more than 100,000 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.6,000/-
per month.
If we start managing all these Thalassaemia major patients on internationally
accepted guide lines, more than Rs.600 million will be required
each month or Rs.7.2 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.