LAHORE, 1 February (IRIN) - Minaal, 8, seems at home in the chaotic environment of the Fatimid Centre in Lahore, capital of Pakistan's populous Punjab province. She smiles confidently, looking up to see how fast the blood bag above her is emptying.

"I am quite used to the needles now and this place, but at first I used to cry," Minaal says. She has been coming in about once a month for transfusions since she was an infant.

Minaal is one of thousands of children across Pakistan with thalassaemia, an inherited blood disorder. Sufferers do not produce adequate amounts of haemoglobin, which carries oxygen through the blood, and need regular blood transfusions to keep levels near normal.

There is a higher prevalence of the disease in Mediterranean countries, the Middle East, parts of India, Pakistan, Southeast Asia and China than elsewhere in the world.

In Pakistan alone, it is estimated that 5-7 percent of the population carries the disease, translating into nearly 12 million carriers out of a population of 170 million.

Although there is no process for registering thalassaemic children, it is estimated about 8,000 to 9,000 are born each year.

A 2004 study in the Pakistan Medical Journal (PMJ) showed there were at least 90,000 to 100,000 thalassaemia sufferers throughout the South Asian nation.

Most experts believe there has been no decrease in the rate since then, and point out that many cases among impoverished families with almost no access to healthcare may never be diagnosed. The actual figure for sufferers could therefore be higher.

Pakistan, unlike high prevalence countries, such as Cyprus and Palestine, where numbers of new cases are declining each year, has so far failed to combat the disease, mainly because of a lack of awareness or genetic testing when people get married.

High illiteracy rates of 50 percent and the absence of a health infrastructure in remote areas compound the problem. This means the disease, which when untreated usually proves fatal within the first decade of life, lingers on from one generation to the next.

"There is only one way to stop this disease: by preventing more children suffering from it from being born," says Shahida Shah, who specialises in haematology and heads the charitable Fatimid Foundation medical staff.

"Other countries, including Islamic countries, have introduced mandatory testing for all adult men, so they know if they are carriers. It is unfortunate Pakistan has taken no steps towards this," she told IRIN.

The Fatimid Foundation, set up in 1980 and represented in five Pakistani cities, handles 70 percent of the country's cases of thalassaemia, according to a survey carried out by the Aga Khan University in the port city of Karachi in 2002.

Fatimid collects blood from donors, screens it and offers transfusions, without any cost, to the thousands of children who knock on its doors.

"We have 2,000 children registered here," says Muhammad Shahid, a doctor at the Lahore centre. He says the foundation is forced to turn down hundreds of applications, since it lacks sufficient capacity.

"The issue is that there is still very little knowledge about this illness. What is needed is large-scale testing, at schools, in rural areas and elsewhere to identify carriers, and let them know about the disease and the fact that even if they are not ill themselves, their children could be afflicted," says Shah.

If two carriers have a child, there is a one in four chance it will be thalassaemic. Working on averages, of four children produced by such a couple, one would be sick, one normal and two would be carriers.

Dr Shah also stresses the need for family planning. "Even when a family has a child with thalassaemia, they often continue to have more children," she says, explaining that women usually have little choice in the matter, while communities are also influenced by clerics who oppose birth control. The result is an increased number of carriers in the population, even if these children do not show symptoms of thalassaemia.

The evidence of this ignorance is there at the Fatimid Centre. Parveen Bibi, 45, sits by the couch where her daughter, Aneela Rani, 12, is receiving her fortnightly transfusion.

"Two of my children died in their infancy. We had no idea what was wrong. We had never heard of thalassaemia. Then when my next child, a son, was born, he was diagnosed after weeks of sickness. He died last year, aged 13," she says, adding softly, "This is a really terrible disease."

Parveen now clings on to the hope that her only surviving child, Aneela Rani, will live. But the odds are against her. Whereas in developed countries, thalassaemics are living into their 60s or beyond and often lead normal lives, limited facilities in Pakistan mean only a small number make it into their mid-30s.

The lack of safe blood is an added problem. Whereas the Fatimid aims to collect 800 bags a month from donors, it generally manages only 8,000 per year.

"People don't know that for any healthy adult, it is absolutely safe to donate. They are scared of giving blood," explains Shah.

There are also other problems. Because they require frequent transfusions, thalassaemics are at high risk of contracting infections from unsafe blood. Whereas the Fatimid screens each pint donated, not all blood is handled so carefully.

According to the 2004 study, 35 percent of thalassaemia sufferers undergoing treatment at the Institute of Haematology and Blood Transfusion Service in Lahore tested positive for Hepatitis C. Another 1.7 percent were infected with Hepatitis B. There were no HIV positive patients, but the risk is there.

While legislation to make testing compulsory on marriage has been discussed, no such measures have been taken.

"Testing has been discussed, but the main prong of the government of Pakistan's strategy is to support organisations that are working to treat thalassaemia and carry out advocacy work on the issue," explained Athar Saeed Dil, chief health officer at Pakistan's Ministry of Health in the capital, Islamabad.

He said the government gave grants to organisations such as the Fatimid Foundation and to other organisations that raise awareness about thalassaemia.

"We also support detection, diagnosis and treatment at hospitals and other centers," Dil told IRIN.

However, until there is greater awareness of the disease, which has continued for decades to rampage through the country's population, deaths and blighted lives will continue.

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