KARACHI, May 7 With the world set to mark International Thalassaemia Day on Friday, May 8, there continues to be a lack of government action aimed at controlling the spread of the inherited blood disorder in children, and some non-governmental organisations who are working in the field are now reluctant to register new patients for want of resources, Dawn has learnt.
Though there is no adequate government survey on the matter, it is disturbing to note that Karachi, a city of roughly 18 million, is witnessing the addition of about 600 thalassaemic children every year. Meanwhile, thalassaemia centres in city, which help patients of the genetic blood disorder by providing blood transfusions and iron-chelating facilities on a regular basis, say they are facing serious resource constraints.
Sources in the thalassaemia treatment community say there are currently about six NGOs or foundations which have been providing services free of cost to thalassaemic children and adolescents for the many years, but now prefer not to register new patients.
The reasons for this include inadequate blood donations and a lack of the financial resources required to maintain existing day-care systems for patients. There was also an absence of measures on the part of the government, a source said, adding that it was the government's fault that a registry of thalassaemia patients did not exist in Pakistan. Further, awareness campaigns and the launching of support and counselling campaigns for thalassaemia patients have also not occurred.
Legislation urgently required
Like its predecessors, the current provincial government also declared that it would pass legislation making it mandatory for citizens to state their thalassaemia status at the time they registered their marriages. Nothing, however, has been seen on the ground.
Those in the thalassaemia treatment community say that private sector organisations, many of whom do not charge for services such as blood transfusions and iron-chelating, attend to between 2,000 and 2,500 patients every fortnight. Other patients, they say, are either irregular in getting treatment or cannot afford it. Some, they add, are dying in ignorance, without even knowing that they have the disease.
India, Pakistan and Iran have the largest population of transfusion-dependent thalassaemia patients.
A consultant haematologist and transplant physician, Dr Tahir S. Shamsi, told Dawn that about 6,000 new cases were added each year to the current tally of 80,000 thalassaemics in Pakistan.
Pakistan would have a staggering over 200,000 thalassaemic patients in the next 10 years, he added, saying that the economic burden on society to provide treatment would be approximately Rs4 billion in 2019, while another Rs10 billion would be lost due to the non-productivity of those who brought patients to treatment centres.
According to Dr Shamsi, however, an investment of only 10 per cent of this expenditure in any consolidated thalassaemia prevention programme can eradicate the disease from the country in 25 years.
“Cyprus, Greece and Sicily set a good example in the 1970s of how this deadly disease can be eradicated. They made it mandatory for everyone to get a thalassaemia test done before marriage and to submit it for marriage registration,” he said, adding that more recently, Iran, the Maldives and Saudi Arabia passed similar laws.
Admitting that the thalassaemia situation was more critical in rural areas, where 70 per cent of the country's population largely lacks literacy and health initiatives, the physician remarked that Pakistan needed an infrastructure to provide test facilities at the district/taluka level. The general public also needs to be educated about this deadly disease, and a mass awareness campaign should be launched. Further, legislation should also be passed to discourage the marriage of people infected with the disease to each other.
Conception by infected parents carries high risks
The relevant quarters claim that every year a number of babies born with thalassaemia-major remain at risk of not living to be teenagers due to non-availability of screened and matched blood. These mostly include those in the lower income areas. Thalassaemia-major is managed by having blood transfusions every 15 to 20 days.
According to Dr Saquib H. Ansari, a haematologist and oncologist, the preventable thalassaemia is hereditary genetic blood disorder. There are two forms of thalassaemia beta thalassaemia major and beta thalassaemia minor.
A person with thalassaemia minor may appear to be perfectly healthy, displaying no symptoms at all. If a man and a woman having thalassaemia minor get married, there is a 1 in 4 chance at each pregnancy that the offspring will be a beta thalassaemia major patient, for which the signs generally appear in the child at the age of four months.
The only treatment of thalassaemia is by bone marrow transplant, which costs about Rs1.8 to 2 million. Most of the patients depend upon regular blood transfusions throughout the rest of their life, and iron-chelating medicines which on average cost about Rs6,000-8,000 every month, he added. Pakistan is seeing a large increase in thalassaemia patients due to the lack of screening and genetic counselling, he said.
Test can be carried out during pregnancy
He said that if both partners are thalassaemia minor patients, a test named CVS (chorionic villous sampling) can be carried out during the tenth and 16th week of pregnancy to determine if the baby has thalassaemia major. If the test reveals that the pregnancy is affected, the couple should then be offered counselling and support to help them to make an informed choice about the termination of the pregnancy before the 16th week, he noted.
Zubeda Baher, official in charge of the thalassaemia prevention programme at Omair Sana Foundation, which, through philanthropic contributions, is bearing the full or partial treatment expenses of about 245 thalassemic children, said that there was an urgent need to address the prevention issue, as she had information that parents already having a thalassaemia-major affected child sometimes continue to have more children.
More blood donations needed
Dawn further learnt that different NGOs in the city are providing free of cost treatment to about 2,000 patients on a regular basis, including children from the interior of Sindh, and for the purpose they need a continuous supply of safe blood (screened for hepatitis B, C, HIV/Aids, syphilis and malaria) adding up to 4,000 bags every month.
Currently the blood supply to these NGOs is shrinking to a decreased number of donations, and an increased demand for blood for emergency patients, including victims of road accidents, violence and bomb blasts.
Medical Director of the Fatimid Foundation Dr Zia-ur-Rehman said that their blood collection teams were occasionally sent to colleges and universities, in addition to the daily collection of blood from donors in various residential areas, which helped manage the growing shortage of blood.
“It is due to the crisis of blood and manpower that we are not only adhering to a tougher policy for new registration, but have also asked the parents of patients to make arrangements for some of the blood supply for their wards,” he said, adding that the longevity of thalassaemia patients due to quality transfusion and medication had also caused an increase in the demand for blood.
He observed that the number of thalassaemia patients ranged from 5,000 to 6,000 in the city, and as such there was a need for the government to take up the problem and to facilitate not only the private sector, but to also set up its own day-care centres. Moreover, the government should have a data registry for thalassaemia and should set up blood screening centres all over the province, he said.
Experts said that increased public awareness and the prevention of the transfer of the thalassemic gene from parents to children are the only steps that can ensure a thalassaemia-free Pakistan. Policy makers, legislators, healthcare providers, thalassaemia centres and philanthropists should get their acts together at the earliest in order to control and eradicate the disease, they added.