On Razor’s Edge: Living with Haemophilia in rural Kashmir

SRINAGAR: She stirs a pot of soup as she holds on to her youngest son, the tin roof and the cardboard walls turn the attic which houses her kitchen, almost scorching.

When her first child slightly injured himself, 22-year-old Mubeena of Kupwara’s Halmatpora was baffled by his uncontrollable bleeding. That day, she didn’t understand why it happened, but life was soon to make her fully acquainted with the reason.

“We rushed him to the local dispensary, they tried everything but couldn’t stop the bleeding,” she recalls.

The doctors at the dispensary wrapped the wound with everything they could find-cotton, bandages, cloth, and more bandages- then asked them to shift the child to tertiary care in Srinagar, she says, nine years later.

At SMHS, the doctors diagnosed the kid with Haemophilia - a blood disorder wherein the body’s clotting mechanism is dysfunctional, internal bleeding is fatal, and the joints are fragile.

The doctors at the hospital then administered Factor-VIII to the child - a stopgap measure which activates the clotting mechanism for around a month, health experts note.

“There is no permanent cure for this disorder,” says Dr Sheikh Bilal, Head of Pathology at SMHS.

The only clinically proven modern antidote for haemophilia patients is Factor-VIII, Dr Bilal and other health experts said.

The people suffering from haemophilia need to take care of their physical activities, because if they hurt themselves, “they can be in real trouble-from internal bleeding to injuring their joints permanently, even a small injury can have serious consequences,” said Dr Bilal.

To prevent uncontrollable bleeding, the missing clotting factor in the blood is compensated by infusion of clotting factor concentrate. “It increases the level of Factor-VIII temporarily, therefore the infusions need to be repeated every month,” Dr Bilal told the Kashmir Times.

A Rare Case of Three Siblings

The diagnosis of her son wasn’t the end of Mubeena’s tribulations. She delivered two more sons, one after the other, in subsequent years. Both were diagnosed with the same disorder.

A rare case, according to the doctors, because no one in Mubeena or her husband Mushtaq's family suffered from the disorder.

“This is a genetic disease, and it is a mystery how all her children without any genetic history were affected by it,” said Dr Sheikh Bilal.

It might be that someone among their ancestors had the disorder, and because the disease is often undetected in this part of the world, they might not have been aware of it, adds Bilal.

Since the diagnosis of her three children, once every month Mubeena hires a cab from her remote village of Halmatpora in north Kashmir to the main town of Kupwara, carrying her three sons. From there, they board a bus to Srinagar, and then, they either walk to SMHS, or if their wallet allows, they hire an auto rickshaw, she says. 

This monthly pilgrimage is important to keep her sons surviving. But the journey is a tightrope walk.

“I have to remain extra cautious,” says Mubeena.

Her fear stems from an event that happened three years ago when her youngest son, in his burst of childish glee, gashed himself in the bus while the family was on their way to SMHS in Srinagar.

“So much blood oozed out from his small wound that we all thought he would die,” recalls Mubeena, now 31, as her voice becomes heavy and her throat seems to be choked.

Passengers, horrified as they were, offered their scarves and handkerchiefs, she says, “I then tied them all on my son’s wound and prayed he lives till we reach Srinagar.”

“Every month, I take my sons on a journey to inject Factor-VIII into them, ensuring they live without fear, for next 30-days,” she explains.

During the journey Mubeena prays that no one among her three sons hurt themselves, she says.

While her elder and the middle child go to school, Mubeena keeps her youngest son at home, as she fears if he hurts himself again, the family would be racing against death to reach the hospital.

After they are back from school, they home tutor their little brother, and then play in the courtyard of their home.

The siblings play with each other, and avoid going out of the house to play with other children, a direction given by Mubeena to ensure their safety, she says.

After they got to know about their children’s disorder and were educated about it by doctors and NGO workers, Mubeena and her husband Mushtaq briefed the school authorities about it.

The teachers at the school ensure that Mubeena’s sons are not involved in physical activities like other children even as they are given a separate bench to sit comfortably in the classroom, says Mubeena.

Most Cases are Undiagnosed, Discovered at 50s

Across Kashmir, Mubeena’s story finds many echoes.

While official count says there are around 450 haemophilia patients across Kashmir, health experts, NGO workers, and research scholars maintain that the numbers could be as huge as 2000, given the fact that the disease is undetectable until one gets injured, or isn’t properly diagnosed, especially in the rural areas.  

Most of the cases are undiagnosed until they don’t suffer from any trauma or a surgery, says Syed Majid founder of Haemophilia Society of Kashmir (HSK).

Majid’s NGO, he says, is receiving cases who are in the 50-60 age group.

Like last year, when a 60-year-old man’s tooth was removed following which his bleeding didn’t stop, the man was diagnosed with Haemophilia Mild, said Majid.

Another man, who was in his forties was also diagnosed with Haemophilia Mild after he broke his leg, Majid said.

Majid cites lack of proper testing at the time of birth.

“Despite our repeated requests to health authorities to make Prothrombin Time (PT) and Activated Partial Thromboplastin Time (aPTT) tests mandatory for the new born, the government hasn’t acted on it,” Majid says.

While the registered patients of Haemophilia in Kashmir is not high which doesn’t overburden the tertiary care, Majid says, the doctors at district hospitals are unable to diagnose due to unawareness.

“If the doctors at district hospitals are trained to identify and diagnose the Haemophilia, then many lives could be saved even as the patients will receive proper health care from the very beginning,” says Majid.

In April this year, the Health and Medical Education Department in Kashmir ordered that doctors working at GMC Srinagar and Jammu associated hospitals be recruited for Directorates of health Jammu and Kashmir. The order was issued without appointing a replacement for the positions, leaving the Haemophilia Centre, at SMHS hospital, the only one in Kashmir, without a qualified doctor.

Three experienced medical officers who had received training in Haemophilia treatment were “relieved”. GMC Srinagar has only one Haematologist, who is responsible for Blood Bank, teaching and now expected to run the daily affairs of Haemophilia Centre that caters to nearly 450 Haemophilia patients.

Two kids of a man died after they both lost heavy amounts of blood through vomiting.

The man’s neighbour knew a Clinical Haematologist in Srinagar. He told him about this incident, the doctor asked him if his neighbour has anymore children. The man said, yes, he has.

The doctor asked him to bring the kid immediately to the hospital.

After the child was brought to the hospital, the doctor conducted his baseline tests for Haemophilia. The results came positive-Haemophilia Severe.

The treatment was started, and two years have passed, the child is alive, Majid told Kashmir Times.

The man had lost his first two children to intracerebral haemorrhage (ICH) or as it is commonly known IC Bleed, the doctor concluded.

“This is the reality; many are unaware of their disorder, and lose their lives.”

While some people are unaware of their disorder, others tend to hide it fearing societal stereotypes, Majid says.

One such case is where a man hid his disorder from the family, and when his sister was married and conceived a child, the child was circumcised. He lost a lot of blood and died, said Majid.

“This is a genetic disease, and females are often the carriers,” he says.

To counter these challenges, Majid says it is necessary to conduct awareness at a mass level, so health centres in rural areas and those in urban as well, conduct baseline tests when a child is born.

While Majid’s NGO often holds awareness programs, he says, if the government does on its own level, “that would be much helpful with a mighty overreach.”

But the testing facility is only available at GMC Srinagar and SKIMS, Majid says.

The challenge is made stronger by the unavailability of reagents that are used to conduct the tests.

Drain on the Wallet & Spirit 

In south Kashmir’s Shopian, 60 kilometers away from the capital city of Srinagar, 15-year-old Faizan-ul-Haq wages his own battle. 

His haemophilia revealed itself when nine years ago a playful tussle split his eyebrow, unleashing a stream of blood.

“We rushed to the district hospital, and they referred us to the SMHS,” says Faizan as he stammers while reliving the terror. 

“Tests confirmed my disorder,” he says. 

A sports lover, Faizan hurt his ankle during a football game in the village, the internal bleeding crippled his joint forever.

“The real danger lurks between the joints,” explains Dr. Sheikh Bilal, “It can paralyse, or worse, claim a life, he explains”

Despite a crippled ankle joint, Faizan rises at dawn, helps his father, Riyaz Ahmad, with breakfast before limping through meadows, shingled roads, and a wilderness to reach his school. Faizan doesn’t play football anymore. In fact, he hardly plays anything.

He is now interested in science experiments, he says as he shows a picture of him receiving an award for a science project at his school.

“I leave early,” he says, pausing along the roadside with a grin, “because it takes me over an hour to reach the school.”

Riyaz, a labourer with calloused hands and meagre wages, ensures his sons eat well and study. Their mother fled shortly after Faizan’s diagnosis, unwilling to bear the weight of a fragile child, says the single father. 

On treatment days, like today, Riyaz skips work, borrows 1,500 rupees from a friend, and takes Faizan from Sadpora to Shopian, then Srinagar—cab, bus, autorickshaw—a journey that drains both the wallet and the spirit, he says. 

“If the antidote was available at our district hospital, life wouldn’t be such an uphill trek,” sighs Riyaz, his sentiment is shared by Kupwara’s Mubeena.

“If the drug is available in the Halmatpora dispensary or even at the hospital in Kupwara, I could let my youngest son join the school, and it will also save us from the stressful monthly journey,” Mubeena remarks.

A Long Journey Difficult to Survive

The journey is not only long and winding. Some don’t survive it.

In Kulgam in south Kashmir, 65-year-old Gulshan Ayoub believes her son would have been alive if the facility was available in her village.

Ayoub lost her son to internal bleeding over a decade ago. 

On that fateful day, her son, who was 22-years-old at that time, complained of massive headache, it was so agonising, that his screams made all the neighbours in the hamlet rushing out of their homes, says Ayoub.

During those days, the antidotes were available at the Sher-i-Kashmir Institute of Medical Sciences (SKIMS) in Srinagar’s Soura, she says.

And as his father and uncle rushed him to SKIMS, he pleaded for her mother’s company. 

“I had my two-year-old son and two daughters aged seven and nine to take care of,” she sobs, “so I gave him two walnuts and promised to follow.”

That evening, his body returned, the walnuts still nestled in his pocket, recalls Ayoub regretfully as she goes down the toughest memory lane of her life.

Her second son, Muzamil, also suffers from haemophilia, and Ayoub makes sure she accompanies him on his monthly journey of getting the elixir of life.

Navigating 2019 & Covid Lockdowns

Amid this despair, a beacon of hope shines from someone who knows what it means to live with the disorder.

Syed Majid, a haemophiliac, in his late thirties, founded the Haemophilia Society of Kashmir in 2017. Diagnosed after biting his tongue as a child, he endured pain that was “unbearable and unexplainable,” he says.

“I wanted to spare others that agony. The dearth of proper healthcare for haemophilia patients compelled me to start the initiative,” he explains as he goes through the list of patients behind his desk inside his office in Srinagar. 

Through curfews, strikes, military curbs of 2019, and the 2020 COVID-19 induced lockdown, Majid’s team, armed with just one ambulance, delivered Factor-VIII to doorsteps, from Srinagar to Ladakh’s rocky mountains, Majid and his team ensured the patients remained alive. 

Following the reading down of Article-370 in 2019, Kashmir was put under massive communications blackout – blocking all internet, mobile phone and even landline phones - and a military lockdown. Some areas, including Soura where SKIMS is located, also witnessed violent protests for a few days.

Several villages were guarded by young men, to keep out the security forces, who often raided the places where violence happened.

The entry to people not belonging to the area was restricted, and after every kilometre or two, patients were left to navigate both the protestors and the armed forces. 

Those were the days, “when we really put our lives on the line,” say Majid and his team. 

With no way to communicate to the patients or for them to contact the NGO, Majid and his team took it upon themselves to reach every patient they knew and deliver the medicine.

The NGO members had a close shave with their lives when during that period, they went to south Kashmir to deliver Factor-VIII in their ambulance and were met with protestors who asked them to take their injured to the hospital.

“They said they would destroy our vehicle if we didn’t take their injured to the hospital,” says Parvez, a senior member at the NGO.

It was even harder was to reach the patients during the COVID-19 lockdown, says Parvez. 

“We were stopped at least at 20 checkpoints before reaching the remote, hilly area of Ladakh to deliver the antidote to a single patient there,” the NGO members recall. 

Since its inception, the NGO has been a major contributor to the healthcare of haemophilia patients in Kashmir. 

State is a powerful entity, and if it wants, it can improve the healthcare for these patients to a large extent, the NGO members say. 

“And that is a need of the hour,” they collectively assert.

It Can Cost Your Limbs

While Mubeena’s sons and Faizan still have their limbs intact, Srinagar’s Shafia wasn’t that fortunate.

Shafia*, 31, from Srinagar’s Bemina, lost her right leg a decade ago after a chore-induced injury festered without proper care.

After losing her leg, Shafia decided to help her father by stitching clothes at home; however, it didn’t last long.

“While using a needle, I slightly injured my fingers which led to more amputations,” she says as she raises her right hand, which has only a thumb and three fingers.

“Now, confined to home and monthly hospital visits, I’m a burden,” she laments as she moves on her wheelchair in the corridor of her house. 

For Kashmir’s haemophiliacs, each day is a fragile thread, stretched taut between hope and haemorrhage. The antidote remains their hinge to life—yet its inaccessibility turns survival into an odyssey of blood and tears.

“What to do, to whom shall we take our matters to, we are a community forgotten by the government and unknown to the society,” says Faizan as he sits on the façade of his home, watching sky full of stars.

(*Shaifa* Name changed on family’s request to protect her privacy.)