Observing World Haemophilia Day
Vinod Chandrashekhar Dixit
The World Haemophilia Day was started by the World Federation of Haemophilia in 1989 and 17 April, was chosen to celebrate it in honour of the founder of World Federation Haemophilia Frank Schnabel’s birthday. The day aims to increase awareness about haemophilia and other inherited bleeding disorders. It is a medical condition, mostly inherited, in which the ability of blood to clot is severely reduced so that even a minor injury can cause severe bleeding.
People born with hemophilia are lacking or have very clotting factor, which is the protein needed for blood to clot normally. The clotting factor works together with platelets to clot blood by helping the platelets to stick together and plug cuts on the vessel.
Hemophilia can result in bleeding within joints that can lead to chronic joint disease and pain. Bleeding in the head and sometimes in the brain which can cause long term problems, such as seizures and paralysis. Death can occur if the bleeding cannot be stopped or if it occurs in a vital organ such as the brain. According to the U.S. Centers for Disease Control and Prevention (CDC), hemophilia occurs in about 1 in 5,000 live births. In the U.S. about 20,000 people have hemophilia. Hemophilia A is four times more common than hemophilia B. More than half of people with hemophilia A have the severe form of the disease.
The primary treatment for hemophilia A is a concentrated FVIII product. About 75% of patients with hemophilia A receive an FVIII product developed using recombination DNA technology, with about 25% using FVIII product derived from human donor plasma.
According to the World Federation of Haemophilia’s Annual Global Survey 2017, there were over 1.96 lakh persons living with Haemophilia across the world in 2017.Dr Ruby Reshi, head of Pathology department at Government Medical College Srinagar in Jammu and Kashmir, said India is fast progressing in the field of haemophilia treatment but the core problem is of diagnosis.
In the country-wise data, India emerges with the highest count at nearly 19,000. Experts believe that 80% of cases go unregistered in India due to the absence of proper diagnostic facilities in the remote areas, so the actual count is close to 2 lakh.
Despite improvement in the medical technologies for the treatment of haemophilia, health experts believe that nearly 80 per cent of Indians with the serious blood disorder are not diagnosed due to the absence of proper diagnostic facilities in the remote areas.There is no known cure for this disorder.
If not diagnosed early, the repeated bleeding into joints, bones muscles may lead to synovitis, arthritis and permanent joint deformities. Haemophilia is usually inherited and about one in every 5,000 males is born with the disorder. Today, though every state has been doing well in terms of ensuring treatment for the patients with haemophilia the major challenge remains proper diagnosis.
(The author is a columnist and hails from Jodhpur Tekra Ahmedabad)
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