An Incurable Disease
Omar G Rabbany
Thalassaemia is a seemingly harmless word, but it has devastating consequences for a family with a child born with the disease.
What is thalassaemia? Simply put, it is a genetic blood disorder inherited from both parents by a child. However, to pass on the disease, both parents must be genetic carriers of thalassaemia. If only one of the parents is a carrier, the child may become a carrier but will never develop the disease. The disorder is present right from the moment of conception. Only a sophisticated test on the foetus can reveal if the child to be born would be normal, a carrier or have the disease. In most cases, however, we come to know that a child is thalassaemic when (s)he shows clear signs of acute anaemia within one to two years of age.
A disease inherited from both parents
Consequences of Thalassaemia
Our blood is composed of many components like plasma, red and white blood cells, platelet etc. Red cells contain haemoglobin that makes it red. It is this haemoglobin that carries oxygen to all parts of our body and keeps us alive and well.
Red cells have a life span of about 120 days. Our body continuously produces red cells to replace the dying red cells.
Red cells of thalassaemia patients may not live for 120 days, or the haemoglobin in it may not be able to carry oxygen properly. These defects make the patients anaemic. Until now, there is no simple cure for such patients. The patients usually die within 5 to 6 years of age, if not treated.
Treatment of Thalassaemia
With early and proper diagnosis and regular safe blood transfusion along with some specific medication, a patient may live a near-normal life. World Health Organisation (WHO) worked out a “Protocol of Treatment for Thalassaemia Patients” with the help of top experts of the world. There are numerous cases worldwide where thalassaemia patients live a near normal life, get education, work gainfully, get married and have children. That is possible only through getting treatment as suggested in the WHO protocol. Unfortunately, the recommended protocol of treatment is very expensive and beyond the reach of most Bangladeshi patients. In Bangladesh, the cost varies between Tk 5,000 and Tk 15,000 per month. One has to understand that the treatment, and hence this expense, will be needed as long as the patient lives.
Adverse Effects of Blood Transfusion
Blood transfusion is the primary treatment for thalassaemia patients. But that is the least expensive part of the total cost of treatment. The major part is the cost of medication. Regular blood transfusion causes extra iron deposit in the body. This deposit keeps on increasing with each transfusion. The extra iron deposits in vital organs like the liver, pancreas, heart etc. This results in their malfunction causing jaundice, diabetes and finally heart failure. Iron deposit also affects hormone production hampering normal growth. Medication is needed to remove the excess iron.
How to prevent Thalassaemia
It has been mentioned above that for a child to be born with Thalassaemia, both parents must be genetic carriers of the disease. Therein lies an opportunity for us to prevent the disease. With a simple blood test costing about Tk 700/= (carried out by Bangladesh Thalassaemia Samity) one can know if s/he is a carrier or not. Thus, if a carrier avoids marrying another carrier, birth of a diseased child can be prevented. This simple awareness can help avoid the agony, financial strain etc. of having a child with thalassaemia.
Thalassaemia in Bangladesh
In Bangladesh about 10-12 percent of the population are carriers. Two carriers getting married is not a rare event given the high percentage of carriers. That is why an estimated 12-15000 babies are born each year in Bangladesh with this disease. Excepting a handful that are diagnosed with this disease and subsequently have some treatment, the vast majority die without any diagnosis of the cause.
Blood transfusion is the primary treatment for Thalassaemia. Photo: Internet
Bangladesh Thalassaemia Samity and its activities
Bangladesh Thalassaemia Samity was formed in 1989 with a few parents of thalassaemia patients. The Samity started for the first time in Bangladesh to provide treatment for patients as prescribed in the WHO protocol. Ever since, the Samity has expanded its activities in different spheres to mitigate sufferings of the thalassaemia patients.
Currently, the Samity runs a transfusion centre (Thalassaemia Hospital) equipped with blood bank and all necessary wherewithal. Its laboratory performs tests to detect if a person is a carrier. It performs diagnosis of the disease in suspected cases. It also imports iron removal medicines and sells to patients without any profit.
The Samity also strives to create awareness among the population through various means to encourage people, especially of marriageable age, to go for carrier detection test.
As mentioned earlier, treatment of thalassaemia is very expensive and is beyond the means of most patients. The Samity encourages the affluent people of the society to sponsor a child for his/her treatment cost. Some people have come forward to sponsor this noble cause but the response is negligible. The Samity hopes more people will respond with compassion. This might also be taken as a part of Zakat.
The writer is the President, Bangladesh Thalassaemia Samity and Hospital.
(R) thedailystar.net 2012