With an estimated prevalence rate of 1:100,00 approximately 1lac people are affected with hemophilia in India, out of which 60000 are likely to be severe. There are about 13,000 registered patients out of which many are receiving inadequate treatment. Haemophilia treatment in India is more or less in its infancy and that the infrastructure in place is, if anything, meager, would be stating the obvious. (Ref: HFI website)
Particularly alarming is the fact that awareness levels are remarkably low. Hospital data indicate that patients in India suffer from preventable morbidity either because doctors do not know enough about the disease and its management or because there is not enough therapeutic material available. Laboratory diagnostic facilities are inadequate and as a result only 12% of the haemophiliacs get diagnosed. This, for a majority of the haemophiliacs, translates to a poor quality of life and a very slim chance of survival into adulthood. Most of who survive into adulthood lives either with some disability or with debilitating infections such as hepatitis B/C and HIV (AIDS).
Statistics indicate that India together with China, Bangladesh and Indonesia represent only 10% of the people with haemophilia who have been diagnosed worldwide. These countries account for less than 2% of the factor concentrates used worldwide for treatment of haemophilia. This affords proof, if any was needed, that there is a yawning gap in haemophilia care between the developed and the developing countries.
The percentage of haemophiliacs diagnosed, the number of treatment centres countrywide and the per capita use of factor replacement therapy can be construed as a reliable criteria for judging the level of care available. Compared to the USA, which has 76% of its haemophiliacs diagnosed, a network of 136 treatment centres and a per capita consumption of 3.4 units, India has only 12%diagnosed haemophiliacs, 55 centres and a per capita consumption of 0.01 units. (Please ensure reference source is there)
What is obvious is that there is a gross disproportion between the levels of care available. This also means that in India factor VIII is not readily available even to the small number of diagnosed haemophiliacs. This has implications in terms of the quality of life and chances of survival. Most of these patients are often given old and highly unsafe options such as plasma, cryoprecipitate and FFP, which have been, discarded worldwide. A relative paucity of the factor VIII concentrates necessarily means that even after diagnosis the quality of life or for that matter the chances of survival for these people does not improve.
In India there is little support from the government as only a few state governments have started limited dispensing of free factors to the needy patients. The World Federation for Haemophilia (WFH) recognizes that the number of Haemophiliacs to whom treatment is available in India is negligible. Even where treatment is available, haemophilia is managed with bare minimal replacement therapy .It was to address problems like these that WHO and WFH collaborated to work on guidelines to improve delivery of hemophilia care
A step – by – step approach focusing mainly on spreading awareness, establishing basic laboratories, training technical personnel and most importantly making the Factor VIII concentrates available has been passionately advocated by needy patients and advocacy groups.
Treaters and patients have finally started accepting that products like plasma, cryoprecipitate and FFP are no substitutes for highly purified factor VIII concentrates for the following reasons
Early administration of factor concentrates reduces the extent of joint bleeding and its complications. This is possible only if the patient has lyophilized concentrates stored in the home refrigerator, which is not possible with blood bank products.
Risk of disease transmission such as AIDS and hepatitis has become negligible with highly purified factors
Large volumes of plasma, cryoprecipitate and FFP cannot be administered safely because of the risk of volume overload and cardiac failure
Major surgery is difficult with blood bank products alone
Recombinant factor VIII is emerging as an excellent treatment option amply available and accepted worldwide for hemophilia. It is manufactured through recombinant DNA technology and not derived from plasma, which is in short supply worldwide, this recombinant product addresses supply gap and availability issues. It is easily available in many parts of the world as compared to plasma derived factors and more than 70% diagnosed hemophiliacs have shifted to recombinant products worldwide.