April 17, 2008
The World Federation of Hemophilia (WFH) started World Hemophilia Day to promote a global effort to secure safe and effective treatment for everyone with a bleeding disorder. In tune with this year's theme 'Count Me In' the WFH is aiming at identifying almost all of those with bleeding disorders in the world.
AdvertisementThe day was chosen in honor of the birth day of WFH founder Frank Schnabel. It is celebrated in more than 100 countries now. Hemophilia organizations and treatment centers all over the world commemorate the day by organizing activities to raise awareness of the issues affecting people with hemophilia or related bleeding disorders.
What is Hemophilia?
Hemophilia is a hereditary bleeding disorder that prevents blood from clotting properly. Being a genetic disorder, it lasts a whole lifetime. People with hemophilia lack a protein in blood that controls bleeding.
Common symptom is prolonged bleeding after an injury, tooth removal, surgery, or an accident. Other serious symptoms involve -
• Internal, uncontrolled bleeds mostly into joints,
• Red blotches on the skin ,
• Sudden bleeding inside the body without a specific reason.
Hemarthrosis, hemorrhage, gastrointestinal bleeding and menorrhagia are other serious problems arising from hemophilia.
Male Inherited Disorder
The most prevalent of bleeding disorders are Hemophilia A, caused by deficiency of coagulation factor VIII, and Hemophilia B, a deficiency of coagulation factor IX. Being a chromosome disorder of the X -linked category, the disease manifests in the males, while females are 'carriers' only and never exhibit any symptoms of the disorder (it must be noted that females have 2 X chromosomes while males have one X and one Y chromosome).
Till the first half of the 20th century, people with Hemophilia had a miserable existence. They were usually disabled before 20 years and the average life expectancy for hemophiliacs was 27 years, mostly due to early deaths from bleeding into vital organs. Now with medical advancement, treatment for Hemophilia and related, rare bleeding disorders like von Willebrand's disease,is available. Although expensive, this allows the patient to live a normal life.
Treatment of Hemophilia
Treatment of severe Hemophilia A or B falls into one of two categories:
• Prophylaxis, or
Prophylaxis treatment involves treating the disorder before the symptoms arise. It is done by infusing the patient with plasma-derived or recombinant clotting factor concentrates on a regular schedule, in order to keep clotting levels sufficiently high to prevent spontaneous bleeding episodes.
In case of 'On-Demand' treatment, bleeding episodes are treated once they arise. In developed countries, where awareness of the disease is high, and factor concentrates are easily available, problems like severely deformed joints, due to repeated bleeding episodes, are a thing of the past.
Hemophilia, Hepatitis and HIV
Though the plasma-derived concentrates have saved lives and prevented disability, their use in the past has been complicated by infection with viral pathogens particularly the Hepatitis virus and Human Immunodeficiency Virus (HIV).
Several hemophiliacs who received blood products manufactured by American companies in the early to mid-1980s were tragically infected with the AIDS and Hepatitis virus. The concentrate of Factor VIII ("antihemophilic factor" or "AHF") and Factor IX, which were allegedly drawn from high- risk groups, were manufactured and sold in the U.S. and exported worldwide, without proper screening.
On May 22, 2003, The New York Times carried a news item that one such company sold millions of dollars of blood-clotting medicine for persons with hemophilia - medicine that carried a high risk of transmitting AIDS - to Asia and Latin America in the mid-1980's while selling a new, safer product in the West.
Managing Hemophilia in children
There have been new developments in the understanding of Hemophilia and its management that has improved the quality of life of hemopliliacs. Earlier, children with hemophilia who did not have access to factor therapy were over-protected and discouraged from participating in active sports and games. Often this resulted in a sense of isolation, irritable behavior and even depression in children and young adults.
Developed countries have psychosocial workers who help parents with strategies in dealing with their hemophiliac child. Concurrently, the child is encouraged to develop self-confidence and cope with regular school activities, while dispelling anxiety and depression in connection with the bleeding disorder.
World Federation of Hemophilia (WFH)
The aim of the WFH is that all patients with hemophilia or related bleeding disorders should have access to proper diagnosis, management, and care by trained specialists, no matter what their circumstances are or where they live. Over the years, the WFH has encouraged communities of hemophiliacs across the world to approach their health ministers, organize walkathons, interact with the media and raise public awareness about bleeding disorders.
'Count Me In'
Massive efforts by healthcare professionals, pharmaceuticals, and psychosocial counselors are on, to create awareness about this genetic disorder and encourage those who have this ailment to join support groups or Hemophilia organizations so they can share their concerns.
The WFH has also made several facilities to help patient organizations, hemophilia treatment centers, and the general public to raise awareness about the need to help identify patients. Among the several tools are posters, news release, fact sheets on outreach and national patient registries, along with educational materials for patients on hemophilia, von Willebrand's disease, and rare bleeding disorders. This year too, the WFH has arranged for Hemophilia workshops that are offered to delegates from all over the world, so talents and skills can be pooled together for the benefit of hemophiliacs spread across the globe.
Prevalence of Hemophilia in India
According to the WHO's prevalence rate, 1 in every 10,000 has this genetic disorder and double this number is prevalent in their mothers, sisters and daughters who carry this gene. Recent statistics show that 13,000 diagnosed Haemophiliacs are present in India.
The actual number of those living with bleeding disorders in India is not known because a lot many cases go unreported due to lack of awareness and education. But the estimate for those with Haemophilia A or B in India is 1,48,000 (as compared with 6000 in the UK and 18,000 in USA).
Most of the hemophiliacs in India do not have, access to the services of a hematologist or to factor therapy and hence seek medical help only after severe joint deformities, pain and near immobility. This entails increased dependence on others, difficulties in completing education or finding employment and severe psychological problems for the patient and his family.
As with everything, prevention is better than cure.
Deformities can be prevented by prompt treatment of acute hemarthrosis with adequate factor replacement analgesics, ice application and application of compression bandages. The patient is also taught a series of exercises to improve the absorption of the hematoma, to strengthen the muscles and joints to prevent fresh bleeding problems.
Fear of the unknown sometimes triggers social stigma. Apart from enduring the suffering caused by the symptoms of hemophilia, many Persons With Hemophilia (PWH) are stigmatized by their communities because there is very little awareness about this disease among the public, especially in villages and suburban areas. The disease has also challenged family relationships and sometimes even disrupted families. There have been reported cases where men have abandoned their families rather than support their hemophiliac sons.
Hemophilia Federation of India
HFI was started in 1983 from the humble premises of Mr.Ashok Bahadur Verma's residence , a PWH who traveled to Italy to have his leg amputated due to complications arising from hemophilia. A very prominent hematologist from Italy, Prof. P. M. Mannucci, who treated him, encouraged Verma to start a hemophilia society for the greater good of the many hemophiliacs in India. Started in an apartment with only 3-4 chapters, HFI now has a four storey building in New Delhi with 65 chapters under its service.
Hemophiliacs can now do "home therapy" which means that they could keep the AHF at home and at the time of bleeding, infuse the same to prevent further bleeding and disability. The Calcutta chapter of HFI first introduced the home therapy, teaching patients the self-infusion skill, but these individuals mostly preferred receiving their treatment with whole blood and sometimes plasma. There were very few treatment centers in India till the beginning of the 21st century.
HFI negotiated with pharmaceutical companies and started importing Factor VIII & IX concentrates,which are high purity products. The AHF was made available in India in small vials of 100, 200 & 500 units @ Rs.2/- per unit, the cheapest in the world. The pricing made the treatment accessible to a fair number of Indian urban hemophilia population.
A major fillip was given to the hemophilia movement in India when HFI got a huge project for 4 years from DANIDA called "Living with Hemophilia" aimed at raising the quality of life of PWHs in India. The project worked through 10 select chapters, but care was taken to see that the activities and benefits spread through the whole of India.
The project undertook to develop lobbying skills and fund raising techniques, training of doctors, physiotherapists, laboratory technicians in different health institutes in India in hemophilia diagnosis and management, psycho-social-support to the PWHs and their family members and economic rehabilitation to the needy PWHs.
Delhi High Court's Landmark Order
All factor concentrates in India are imported and safe treatment is unaffordable to 90% of PWHs in India. In the absence of any social security system for resources, the immediate family of the PWH reels under the burden of dealing with a chronic disorder like hemophilia. It is understandably highly frustrating, physically, socially and economically. Under such circumstances and with severe lack of funds, HFI filed a Public Interest Litigation (PIL) in 2005, requesting the Government to make this life saving drug available and also generate awareness about this disorder.
In 2006, the Delhi High Court directed the Delhi State Government to make the Anti Hemophilic Factor available to people suffering with Hemophilia [Vide Civil writ Petition No. 16326/2006].
2008: Silver Jubilee year for HFI
As HFI gears up for its 25th year celebration, this year will be earmarked for Hemophilia Awareness in India. HFI put up an awareness stall at the Green Schools Award ceremony to sensitize school students, their parents and the teachers about Hemophilia.
HFI is currently providing subsidized treatment to around 6000 hemophilia patients in India. Its goal is to reach a situation where all 1,48,000 Indian hemophiliacs get free access to treatment; HFI is publishing a quarterly Newsletter in 10 languages for the PWHs and their family. It arranges for educational scholarship to children with hemophilia to help them continue their study, net works with some international NGOs for support to the needy PWHs, provides assistance to the PWHs with HIV positive and inhibitors through its 4 regional offices and chapters.
Miles to go...
This year, apart from providing factor and other medical care such as physiotherapy for affected persons, HFI will continue to reach out across the country, educating people about hemophilia and attempting to de-stigmatize the condition.
According to recent statistics more than 75% of hemophilia population cannot afford treatment with AHF. There are many affected persons who are still undiagnosed. There are many hemophiliacs who still get treatment with whole blood transfusion and are unaware of the existence of factor concentrates.
There are miles to go before fulfilling the vision of HFI "Hemophilia without Disability, Children free of Pain."