April 17, 2021 9:10:55 am
Every year, April 17 is observed as World Hemophilia Day. Hemophilia is a rare disorder of the blood, in which it doesn’t clot normally because it lacks sufficient blood-clotting proteins. According to the World Federation of Hemophilia, “World Hemophilia Day is about bringing the global bleeding disorders community together. And with the “COVID-19 pandemic having a major impact on people with a bleeding disorder, that objective has never been more important”. This year’s theme is ‘Adapting to Change’.
Dr Samir Shah, consultant, Dept of Haematology, Jaslok Hospital and Research Centre, Mumbai says that hemophilia is caused by deficiency of factor VIII called Hemophilia A or factor IX called Hemophilia B; the gene for which is carried on the X chromosome making males susceptible and the females carriers. “These are the commonest factor deficiencies,” he says.
The doctor explains that the manifestations are similar depending on their degree of deficiency. “These are bleeding from sites like gum, or heavy menstrual bleeding. Classically, they present with bleeding into joints and muscles either spontaneously or after minor trauma. They can also present as prolonged bleeding post minor injuries or after dental extractions,” Dr Shah explains, adding that “even today, the facilities to diagnose them in good time and reliably are not widely, and more importantly reliably, available”.
“This results in delayed diagnosis and the patient being left behind with crippling morbidities of joint and muscle bleeds.” Today, however, there is better awareness, which lets people know hemophilia is a high-cost, low-volume disease.
“There are some risks and challenges, which include the transmission of infections like Hepatitis B and C and HIV, by transfusion. Better equipped blood banks with more robust testing for these viruses, making safer components have helped patients. Availability of specific factors by pharmaceutical companies provides reliable and more definitive treatment.
“Since 1983, Hemophilia Federation India (HFI) is the only national umbrella organisation in India working for the welfare of PwH (People with Hemophilia) through a network of 87 chapters spread over four regions. The aim is to reach out to PwH and provide total quality care, education, make treatment available at affordable cost, psycho-social support, and economic rehabilitation, helping them in improving the quality of life without disability and free of pain,” the doctor says.
“Comprehensive Hemophilia Care centers offer genetic diagnosis, help with counselling and prenatal diagnosis. These facilities provide round-the-clock service for emergency care, help plan major surgical interventions, well-trained orthopedic and physiotherapy departments which help in rehabilitation. Also, there is a registry of patients which helps with documentation of the disease, helping with resource allocation for diagnosis and treatment,” he concludes.
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