A Paper Cut Can Kill! Why Haemophilia Needs Urgent Attention

1 lakh Indians are haemophiliacs. For them, a minor cut can be life threatening. That’s why you have to read this.

1 in 10,000 Indians suffer from a rare genetic disease – haemophilia which gets negligible attention from both the government and the press. Here’s why you need to listen up and take notice (Photo: iStockphoto)

(17 April is observed as World Haemophilia Day. India has the highest number of haemophilia patients in the world)

Paper cuts.

Experienced them?

The perfect amalgamation of pure horror, agony and disbelief. A bl***y paper gives you a jagged wound, injuring the most sensitive nerve cells.

But this incredible misery for you (pardon the exaggeration) can result in endless hours of oozing blood, injections, and even RIP for about one lakh Indians living with a rare blood disorder, haemophilia.

(Photo Courtesy: Tumblr/DAINFAGERHOLM)

Normally when healthy people bleed, substances called clotting ‘factors’ in the blood combine with platelets to make the blood sticky and eventually the bleeding stops.

An inherited mistake in the genes stops blood from clotting in haemophiliacs – so even minor cuts, bruises and fall can result in internal bleeding of the joints, muscles and brain accompanied with external bleeding.

Did you know that even though haemophilia is a rare disease, India has the highest burden of patients in the world? Approximately one in 10,000 Indians suffer from haemophilia, and yet, there is no comprehensive public health intervention to prevent or control the genetic disorder, and there is absolutely no national policy on this disease.

Scroll below to find out more on this complicated disease and why India needs to give it special attention urgently.

1. There Is No Cure, Treatment Is Expensive, Subsidised In Only Major Cities

(Photo Courtesy: Tumblr/InGordWeTrust)

When a haemophiliac is injured, shots of factor 8 or 9 need to be given immediately which will help their bodies synthesise blood.

An Anti-Haemophilia Factor (AHF) injection costs Rs 20 per unit and a person with haemophilia requires around 4,000 every week, sometimes even more, depending on the severity of the disease. Do the math and the cost of caring for a single person could cost a family around Rs 10 lakhs annually.

The only relief is that in some states, the Haemophilia Society’s run primarily on donations and government grants provide subsidised, sometimes free of cost factor shots in two-tier cities, but they run out of funds for long periods and are completely absent in smaller towns and villages in most parts of India.

2. Joint Damage & Disability

In the absence of a factor injection, the patient’s joints weaken, which over time, leads to lifelong disability or death. A prosperous state like Maharashtra also provides subsidised Haemophilia treatment only in four cities – Mumbai, Pune, Nagpur, and Nashik.

A study conducted in India found that only 9 out of 148 haemophilia patients did not have a disability. Most of these patients were young boys from socio-economically weaker sections of society.

3. Possible Drug Price Rise Will Hit Haemophiliacs the Hardest

Dear Health Minister, in all of the developed world, haemophiliacs receive regular injections of the clotting factor even in the absence of an injury. India, which has the highest living burden of the disease, the basic treatment is inaccessible and unaffordable to a majority of the patients. (Photo Courtesy: Tumblr/ InGordWeTrust)

The Union Health Ministry’s recent decision to withdraw the customs duty exemption on the import of 74 critical life-saving drugs to treat cancer, HIV and haemophilia will see a sharp spike in prices. The move is meant to push the Prime Minster’s flagship project Make In India.

This will hit haemophiliacs the hardest because all the anti-haemophilia factor shots are imported.

The expense is likely to go up by a minimum of 20% after the Factor VIII and IX being removed from the list. And it will make treatment unaffordable for India’s 3 lakh patients, who are living with this condition.

Though recently India has started manufacturing blood factors, the scale is extremely insufficient.

<b> Leena Menghaney, Regional Head MSF Access Campaign, South Asia</b>

I spoke to a family in Gurugram where an 18-year-old son is a haemophiliac, and they said, their monthly medical costs will shoot up by at least Rs 35,000.

4. Haemophilia Affects Mostly Men – Traditionally the Bread Winners of the Family

(Photo: iStockphoto)

There are two types of haemophilia caused by a problem in one of the genes on the X chromosome (factor 8 or factor 9) that tell the body to make the clotting factor proteins needed to form a blood clot.

Males have one X and one Y chromosome (XY), and females have two X chromosomes (XX).

Remember school biology classes?

Girls get the X chromosome from their dads while boys get it from their mothers. Now even if girls get the haemophilia-stricken-chromosome from their dads, since they have two XX, the healthy one dominates. But they can still be carriers of the disease –experience heavy periods and bleeding, but the severity of the disorder is not so pronounced.

Boys have only one X chromosome, if it is missing the factor 8 or 9, they pretty much have to bear the emotional and physical trauma of getting injected with factors for the rest of their life.

(This article is republished from The Quint’s archives on the occasion of World Haemophilia Day, originally published on 17 April 2016)