“I have seen broken marriages, deserted children and family feuds just because of this one disease,” says Dr Haroon Memon. He has been treating thalassemia patients for years in interior Sindh and is thoroughly aware of the devastation the disease has and can cause.
Working at the Thalassemia Care Centre in Badin, Dr Memon heads the facility where patients flock from eight districts of the province for a cure. The centre was built under the US Agency for International Development’s (USAID) District Grants Programme in 2005 and now functions with the help of Badin’s district government.
Victims of the inherited blood disorder — that results in severe blood shortage — suffer from an increase of iron in their bodies, diabetes and other major side effects. In the rural parts of the country where poverty, lack of resources and illiteracy result in the late or no diagnosis of the disorder, the social implications become more prominent than the physical ones. Pakistan is counted among those less-developed countries where thalassemia patients are multiplying at a very fast rate. Although no proper research has been done in the country, experts say almost seven per 100 people are carriers, known as thalassemia minors. The carriers are normal themselves but have the potential to transmit it to the next generation causing thalassemia major.
When two carriers reproduce, there are 25 per cent chances that the child will be normal, 25 per cent chances that he or she will have thalassemia major and 50 per cent chances that the child will also be a carrier, says Dr Memon. Therefore a large proportion of thalassemia patients are children.
There is growing concern that thalassemia may become a very serious problem in the next 50 years – one that will burden the world’s blood bank supplies and the health system in general.
With the requirement of a bottle of blood almost every few days, the life of a thalassemia patient is like that of a car, one whose engine runs on fuel, explains Dr Memon. In such a scenario, prevention not cure is what needs to be done to fight the genetically transmitted disease.
In Sindh, most people suffer from beta thalassemia, which is one of the most familiar types. It involves decreased production of normal adult haemoglobin (Hb A), which is the predominant type of haemoglobin.
(All haemoglobin consists of two parts: heme and globin). The globin part of Hb A has four protein sections called polypeptide chains.
Two of these chains are identical and are designated as the alpha chains. The other two chains are also identical to one another but differ from the alpha chains and are termed the beta chains. In people with beta thalassemia, there is a reduced or absent production of beta globin chains.
Dr Memon says that there are patients at his centre who, because of continuous blood transfusions, have developed a concentration of iron in different parts of the body and have to be treated for that. The iron concentrates in the heart, pancreas, liver, spleen and the endocrine glands, he adds.
Such children show signs of a ballooned stomach, broader forehead and jaw bones and prominently bad teeth.
Our job is to maintain the haemoglobin level of the thalassemia patients at 10.5 grams, to control the iron concentration in different parts of the body and to counsel the parents, says Dr Memon.
Law in the making
Concerned doctors are lobbying for a law to be passed for the identification of carriers in the province, informs Dr Saqib Ansari, a haematologist at the National Institute of Blood Diseases (NIBD). “In the year 2010, close to the World Thalassemia Day (May 8), the Sindh Assembly adopted a resolution to make the thalassemia test a pre-condition for nikah, but it could never be formed into a bill,” he says. Dr Ansari claims that this will successfully reduce the danger of thalassemia transmission without stigmatising women.
Meanwhile, Dr Ansari also says that a fatwa has been taken that allows a child with thalassemia major can be aborted before the 16th week.
The government needs to realise that apart from human suffering, supporting thalassemia patients is a huge financial cost, he says. “The disease needs to be controlled before the numbers shoot up.”