Hemophilia is an inherited bleeding disorder that prevents blood from clotting effectively. For a person with hemophilia, any injury to a blood vessel can result in an uncontrolled discharge of blood either internally or, when the skin is broken, externally. A bump or a knock, which in a normal person would produce a bruise, may result in a major bleeding episode in a person with hemophilia.People with hemophilia are prone to spontaneous bleeds, particularly in joints such as ankles, elbows and knees. Because these bleeds are accompanied by symptoms of pain and swelling, they are generally obvious and get prompt attention. Nevertheless, over a period of time, the enzymes from these hemorrhages corrode the cartilage, bones and nerves causing chronic pain and arthritis in the affected joints. Another insidious aspect of the disorder is that spontaneous, life-threatening bleeds may occur internally without any specific symptoms, requiring emergency hospitalization.
Except in rare cases, hemophilia occurs only in men; women, however, are the carriers of the hemophilia gene. This condition is incurable but temporarily manageable. The only possible treatment is infusion of blood, plasma, cryoprecipitate or the anti-hemophilic factor concentrate at every instance of bleeding. The treatment is prohibitively expensive.
In line with the World Federation of Hemophilia’s estimated prevalence rate of hemophilia in general population (one in 10,000 live births), there should be around 3,000 persons with hemophilia (PWH) in Nepal. But only around 600 of them have been identified. Nepal Hemophilia Society is a social non-profit, non-governmental, non-political organization that works for these PWH, their families and care-givers. Established in 1992, it is run by a group of voluntary workers who either have hemophilia or are parents of such children.
NHS is working in all aspects of hemophilia care that includes diagnosis, treatment, rehabilitation, psychosocial counseling and social support. Other important works of NHS include advocacy of hemophilia rights and awareness generation.
NHS is a sole caregiver, the only advocacy group, and a social network for the PWHs and their families in Nepal. After a long strategic planning, advocacy and continuous effort, an important landmark has been achieved i.e. for the first time hemophilia has been categorized as a disability in Nepal under the Persons with Disabilities Rights Act, 2074. Issues of hemophilia have, however, remained largely 'invisible', often side-lined in the rights debate, making those with it unable to enjoy their human rights like everyone else.
As we celebrated the World Hemophilia Day on April 17, the hemophilia fraternity of Nepal was still struggling to get that one vial of anti-hemophiliac factor concentrate for the next bleeding episode.
By SURAKSHA THAPA
The author is a program officer at Nepal Hemophilia Society
