Every yr, April 17 is noticed as World Hemophilia Day. Hemophilia is a uncommon dysfunction of the blood, during which it doesn’t clot usually as a result of it lacks adequate blood-clotting proteins. According to the World Federation of Hemophilia, “World Hemophilia Day is about bringing the worldwide bleeding issues neighborhood collectively. And with the “COVID-19 pandemic having a serious influence on individuals with a bleeding dysfunction, that goal has by no means been extra necessary”. This yr’s theme is ‘Adapting to Change’.
Dr Samir Shah, guide, Dept of Haematology, Jaslok Hospital and Research Centre, Mumbai says that hemophilia is attributable to deficiency of issue VIII known as Hemophilia A or issue IX known as Hemophilia B; the gene for which is carried on the X chromosome making males inclined and the females carriers. “These are the commonest factor deficiencies,” he says.
The physician explains that the manifestations are comparable relying on their diploma of deficiency. “These are bleeding from websites like gum, or heavy menstrual bleeding. Classically, they current with bleeding into joints and muscular tissues both spontaneously or after minor trauma. They can even current as extended bleeding put up minor accidents or after dental extractions,” Dr Shah explains, including 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, nonetheless, there’s higher consciousness, which lets individuals know hemophilia is a high-cost, low-volume illness.
“There are some dangers and challenges, which embody the transmission of infections like Hepatitis B and C and HIV, by transfusion. Better geared up blood banks with extra strong testing for these viruses, making safer elements have helped sufferers. Availability of particular components by pharmaceutical firms offers dependable and extra definitive therapy.
“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 physician 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.