… to Haemophilia Management
Haemophilia is the oldest known genetic/ hereditary bleeding disorder that hampers the blood clotting process in the body.
- It remains the second most common hereditary disorder responsible for bleeding.
- The disorder has been widely recognized through the ages, mainly because of its association with the royal families of Europe.
- Haemophilia is also unique in as much as its prevalence is same across the globe- be it the developed vs. developing or the rich vs. poor.
Haemophilia results from the deficiency of the clotting proteins- Factor VIII or Factor IX- which lead to excessive bleeding. The patients with haemophilia bleed more than normal and for longer times, at times spontaneously with no trauma. Often the bleeding is into the muscles and joints like the knee, ankles, elbows with lot of pain and disability. If the bleeding is internal into the brain, chest, or abdomen, it can be life-threatening too. The survivors suffer from a continuing damage and long-term morbidity from the left over blood products inside. Repeated bleeds make the situation worse by the day.
This once-dreaded disease has long since ceased to be synonymous with misfortune and gloom. The immense resourcefulness and perseverance displayed by medical researchers worldwide has meant continuously evolving treatment modalities and near normal life-span for the persons with haemophiliacs. Even when a good complete cure still eludes us, modern medicine has very effective treatment for haemophilia in the form of injections- called the anti-haemophilia factors (AHF) – for replacing the deficient clotting proteins viz. Factor VIII and Factor IX. These are magical remedies and stop the bleeding instantly, thereby limiting the future damage to the muscles, joints and other body organs. Though these came into use half a century earlier, the scenario is still not at all good for the haemophilics in developing countries who have poor access to treatment.
Haemophilia incidences in India
The incidence of haemophilia worldwide is 1 in 10,000 births. It is estimated that in India, with a population of over 1.2 billion, there should be over 100,000 haemophilia-A patients and 30,000 haemophilia-B patients in the country. Half of them are likely to have severe disease. The big challenge is that most of them are still undiagnosed.
The countrywide estimated number of people diagnosed with haemophilia ia around 16,000 only. It is for this lack of a definitive diagnosis that majority of haemophilics cannot avail of the immense benefits of the highly effective treatment now available across India. And to diagnose this disease in emergency when they are bleeding is not really feasible in the current scenario. Lack of reliable laboratory facilities are of major concern. Another issue is the lack of awareness even amongst the urban educated class in India is. These need to be addressed.
The percentage of haemophiliacs diagnosed, the number of haemophilia treatment centres countrywide and the per capita use of AHF replacement therapy can be construed as a reliable criteria for judging the level of care available. Compared to the USA, which has around 80% of its haemophiliacs diagnosed, with a network of 136 treatment centres and a per capita consumption of 3.4 units, India has only 10% who are diagnosed with a per capita AHF consumption estimated at 0.01-0.0 units. However the scenario is changing rapidly.
Awareness and Support from Government
The awareness levels on haemophilia are remarkably low. Hospital data indicate that patients in India suffer from preventable morbidity because doctors do not get adequate exposure in the context of suboptimal availability of infrastructure for diagnosis or treatment of haemophilia. This is also one of the major reasons for the majority of remaining undiagnosed. Now, over a dozen States in India including Delhi are supporting the treatment of Haemophilia patients.
Delhi government has in this regard has created one of the best facilities for completeand comprehensive care of haemophilia.Now Haemophilia Day Care Centre (HDCC) at MAMC and LN Hospital is not only reagrded as the best Haemophilia Centre in the country but widely acclaimed in the world haemophilai community as a pioneering approach in providing holistic care as par with the devloped countries but at a fraction of cost. This centre has over 1,250 patients with haemophilia who have access to 24-hour treatment mechanism. It takes pride in being the only Centre carrying out specific laboratory assays on a regular basis free-of-charge to one and all. Patients have access to dedicated and specially trained doctors, nurses, physiotherapist, social worker, and laboratory technical staff over here. No wonder, haemophilics from across the counytry get referred to this Hemophilia Centre especially when complications arise. These patients get a multi-specilaity care, possible at only a couple of centres in the country. Patients from all across the country have access to our center for free treatment. Also, this is India’s only comprehensive Haemophilia treatment centre where we look beyond just medicines and try to provide quality care to the patients for making them feel absolutely confident of themselves.
The Centre provides a comprehensive acre including preventive vaccines, nutrition, and other supportive and rehabillitative care to persons with haemophilia. And also undertakes clinical research in this domain Amongst the dozen research projects completed at the Centre, the one pioneering the use the ‘skin route’ for administering Hepatitis-B vaccine provided a big relief to haemophilics. The Centre also presented 5 research papers in the last World Haemophilia Congress in July 2012 (in Paris).
Earlier, the Centre had won the global award for the innovative activity for haemophilia community-for bringing in the techniques yoga to benefit haemophilics with a scientific basis. The award was instituted by a purely philanthropic organization- NNHF- Novo Nordisk Haemophilia Foundation and was shared & celebrated with the haemophilia community in MAMC on the Baisakhi-day in March 2012.
Along with these strengths, we also realize our weaknesses and are striving to make our two other Centres- in East and West of Delhi- to share the same pride. There is also a need for more treatment centres to make the lives of haemophilia patients better. Establishing diagnostic infrastructure will also bring the benfits of the specialized laboratory assays to a large number of other departments like blood banks, intensive care (ICU), cardiology, gastroenerology, neurology, infections etc.
Women are stronger in not manifesting this disease. They however need to be identified for any silent carrear staus. In the 12th Plan, we have also envisaged the setting up of prenaatl diagnostic facilties for informed decisions and possible prevention of haemophilia in society.
To be fair, it must be stated that the treatment of haemophilia especially with newer anti-haemophilia factors (AHF) is expensive even for the developed countries. Affordable medical care in health is becoming an important agenda across the globe. Use of generic drugs is one means of making available drugs at affordable price- the Government initiative of Jan Aushdhalaya is one such successful venture. However in the case of anti-haemophilia injections, there are only a few quality manufacturers. The manufacturing of the blood products is regulated very stringently under Schedule F of our Drug and Cosmetics Act 1940. Producing cheaper AHF of good quality and safety is either difficult (as in the case of blood plasma-derived) or more expensive (as in the recombinant AHF) or both as in the case of bypassing agents for haemophilia. There is therefore a dire need for rational & optimal use of AHF.
A step-by-step approach focusing mainly on spreading awareness, establishing basic laboratories, training technical personnel and most importantly making the AHF concentrates available has been passionately advocated by needy patients and advocacy groups.
Comprehensive care is very important
Thus, when we look at a disease like Haemophilia which somewhere or the other affects your normal being, we need treatment beyond just providing medicines. The concept of comprehensive care is to treat the patient and the family, through continuous supervision of all the medical and psychosocial aspects of bleeding disorders. Comprehensive care is total care because every facet of the person is addressed, including their physical, emotional, psychological, educational, financial and vocational factors. The development of comprehensive care has greatly improved the quality of life for people with bleeding disorders, helping them to be more independent and productive.
In this fast evolving and expanding scenario of haemophilia care, the role of NGOs in Delhi is also getting re-defined.