World observes April 17 as Haemophilia Day

World observes April 17 as Haemophilia Day

Treatment for haemophilia costly, limited
Annie Samson in New Delhi |
As the World observes April 17 as Haemophilia Day, there is little awareness in the country about this inherited genetic disorder for which treatment is costly and difficult to get, experts say. Over one lakh people are estimated to be affected by haemophilia or the silent killer that is inherited through a defect in the genes and causes severe bleeding even from very small cuts and injuries, says Dr Uppal Roy, one of the founding members of Haemophilia Federation of India (HFI).

In a country with one billion that works out to be one in every ten thousand people, he says. "Out of the one lakh estimated population of haemophiliacs in the country the HFI has been able to register 12,844 patients so far," says Roy who informs that the foundation has 60 centres countrywide.
Haemophilia A commonly known as classical haemophilia is the most common form and is caused due to the inability of the blood to clot properly. When a normal person is injured his blood quickly clots to prevent further loss of blood. However, this does not happen in the case of people with haemophilia. The Anti Haemophilic Factor (AHF) that produces clotting in blood is not manufactured in the country but imported from abroad. "In India the HFI is the nodal agency that negotiates the prices of drugs with manufacturer linked with the Canada-based World Federation of Haemophilia," says Roy. "Each unit of the AHF costs Rs seven. The blood is dried and powdered and contained in a small vial and given to the bleeding person according to his body weight. So, a child weighing 20 kg needs at least 500 units to stop severe bleeding. A patient has to bear at least a cost of Rs 50,000 to Rs one lakh to lead a near normal life," he says. In order to form a blood clot the body uses several proteins and blood cells. Patients with Haemophilia A have a deficiency of clotting factor 8 and those with Haemophilia B are deficient in clotting factor 9. "Replacements of factor 8 or factor 9 made out of human blood are given to patients depending on their requirements. Apart from that if patients can provide a donor we give blood transfusions," says Dr Sumita Bhasin, Professor of Medicine, Safdarjung Hospital. The disorder usually transfers from father to daughter who then becomes a carrier of the gene with the chance of passing it on to her children. Bhasin points out there are at least three hospitals in Delhi other than Safdarjung that gives free medicines for treating the condition. "We provide free treatment for the poor people who come to our hospital. Obviously we are able to do so for only those patients who come to us in a critically ill condition and those who are involved in accidents and the like. For other patients we usually charge," she says. Anil Lalwani a severe haemophilia 8 patient and part of the HFI says, "There are very limited diagnostic facilities available for haemophiliacs. There is also not proper treatment available in the country." "In HFI chapters across the country, some preliminary treatment is available," says 48-year-old Lalwani who was diagnosed with the condition when he was one-and-a-half-years old. What compounds the treatment is the fact that most haemophiliacs are not unaware that they are suffering from this disorder. There is no specific cause for haemophilia as it is an inherited disorder. "We have the facility to check for the disorder and detect the carriers. Apart from HFIs chapters five other hospitals- AIIMS at Delhi, CMC at Vellore, KEM at Mumbai and CCMB at Hyderabad-have provisions to test and give free medicines," says Roy. "These hospitals give the factor constituent free of charge. Others like the HFI and the Lion's hospitals provide medicines at subsidised rates," says Bhasin. Other cures apart from replacement of the AHF are under progress worldwide. "Everything is in a trial stage and nothing can be said so far," says Bhasin. In the US and UK doctors have experimented with gene therapy to find a cure for haemophilia. In India, however, there is no such therapy. "Even in the west the therapy has not been very successful because it was found that it was always done for severe haemophiliacs to reduce their condition from severe to moderate or mild," says Roy. "If children affected with haemophilia are not given treatment then it can lead to disability and even death in some cases. Also since patients need frequent blood transfusions they are at the risk of contracting infections like HIV and hepatitis C virus," he says.

Dr Marwah