HYDERABAD : K Aravind Nikhil’s life is an embodiment of the phrase ‘living on a knife-edge’. Aravind is a regular 25-year-old cheerful, IT professional with dreams of a good life. Until he says one would never know that he suffers from haemophilia, an incurable congenital genetic bleeding disorder.
Aravind’s life is dotted with many ‘near misses’. Since the age of six months, he has suffered from spontaneous internal bleeds, i.e., bleeds without any injury to joints, muscles, hands or other parts.
This World Haemophilia Day observed every April 17 experts highlight the need for awareness, early diagnosis, the right treatment with recombinant therapy and the importance of prophylaxis for effective management of the disease. With the Covid-19 pandemic having a major impact on people with a bleeding disorder, the subject has never been more important.
Haemophilia is a rare inherited bleeding disorder where the blood fails to clot normally due to a lack of blood clotting proteins known as clotting factors. The condition leads to spontaneous bleeding mostly in the joints, leading to disability and sometimes even untimely death. Prem Roop Alva, regional council chairman (South), Haemophilia Federation of India (HFI), suffers from Haemophilia B. He was diagnosed with the condition when he was nine months old and the 45-year-old has been raising awareness about this disease for the past 29 years.
According to Research Gate, in India one per 10,000 births is inherited with haemophilia. The reported number of patients with Haemophilia A is 11,586, while the estimated prevalence could be around 50,000 patients. The latest Indian data published in the World Federation Haemophilia Annual Global Survey shows a significant increase (79.3 per cent) in the number of people known to have the condition. However, estimates based on population data in comparison with haemophilia prevalence in countries with higher rates of diagnosis and better-developed registries suggest that the actual number of people with haemophilia in India is likely to be in excess of 70,000.
Life expectancy was just 20 years in the 1980s, but now with medicines available, one can live up to 10 years lesser than other normal human beings. Injections are expensive though and can range from Rs 15,000-Rs 4,35,000 per bleed per day (depending on the factor).
Managing haemophilia in the second wave
According to Dr Nita Radhakrishnan, associate professor and HOD, Department of Paediatric Haematology-Oncology, Super Specialty Paediatric Hospital and PG Teaching Institute, Noida, PWH can effectively manage haemophilia during the second wave of Covid-19 by:
- Practising hand hygiene along with observation of strict cough etiquette, wearing masks and social distancing
- If a bleed occurs, do not ignore it and delay clotting factor support for the fear of contracting Covid from hospitals
- Children with haemophilia should do regular non-impact exercises and physiotherapy sessions at their homes
- Tele-consults may be approached for physiotherapy services
Can PWH take Covid-19 vaccination?
It is advised that all persons with haemophilia should get the Covid-19 vaccine on priority. “We request the government to include blood disorders as comorbidities,” appeals Prem Roop. However, PWH should take certain precautions before vaccination:
- People with bleeding disorders are not at a greater risk of contracting Covid or developing a severe form of the disease
- The vaccine should be administered intramuscularly with the smallest gauge needle
- For patients with severe/moderate haemophilia, the injection should be given after a factor VIII or factor IX injection, according to experts.
70,000 people in India are believed to have haemophilia
What is Haemophilia?
A haemophilic has one or more of the 10 clotting factors in the blood either missing or deficient. The disease is almost exclusively seen in men, while women are asymptomatic carriers. Rarely does it affect women. One in every 10,000 males is afflicted by it. The main deficient factors are VIII and IX and 80 per cent of patients belong to Haemophilia-A (VIII).
Haemophilia is also known as a royal disease as it figured prominently in European royalty in the 19th and 20th centuries. Britain’s Queen Victoria, through her daughters Princess Alice and Princess Beatrice, is said to have passed the mutation to various royal houses across the continent, including the royal families of Spain, Germany and Russia.
— Tamanna S Mehdi tamanna@ newindianexpress.com @tamannamehdi