Pamela Marriott is a teacher, mother and daughter and she launches our latest Sunday Sessions series on love and relationships in the RARE world. Pamela reflects on her relationship with her dad, her hero and protector, and how the rare blood disorder haempohilia impacted his life and stole him from her.
That pervasive shadow pushed him to experience everything fully as it hovered over his life. So, he took risks as he knew the clock was ticking.
My dad had a rare blood clotting disorder called haemophilia. In layman’s terms it meant if he cut himself or even bruised himself his body wouldn’t respond the way it should, he wouldn’t stop bleeding, his blood wouldn't clot, and the reality was he could bleed to death.
He went skiing when he shouldn’t have, he built things and mended things using innocuous tools that we all use at home for DIY. A saw, hammer or nails could be lethal. So many everyday activities that we take for granted, had the potential to stop him in his tracks, putting him at very real risk.
He once required dental surgery after eating a bar of chocolate containing a piece of metal. Thankfully he recovered. As my dad was a solicitor he had no qualms about suing the company and as a poke at Cadburys he used the compensation he received to build my mum a summer house, putting a sign over the door with the inscription “Cadbury’s Folly” his sense of humour helped him through some dark times!
He wanted to fight in the Second World War but because of his haempohilia he wasn't allowed to. He was determined to fight for his country and so instead he became a member of the Royal Observer Corps spotting enemy aircraft. He was an air raid warden during the blitz, while the city of London was burning all around and the night skies were glowing with an eerie orange hue my dad would fearlessly go into buildings, charging up the stairs he'd climb through smoking hot attics out onto roofs and throw burning incendiary bombs into the gardens below to stop the houses catching fire.
The real danger for my dad was not the war but his rare disease as the possibility of him dying young was always there.
In a study by Birch in 1937 it was found that out of 98 patients and affected relatives only six survived until their 40th birthday. The other 82 patients dying in childhood or adolescence from trivial accidents or minor surgery.
My dad was often in hospital because of his disease, from my limited knowledge of the history of haemophilia it seems he was probably receiving ground-breaking treatment involving giving fresh frozen plasma intravenously, but it only contained tiny amounts of the necessary clotting factors VIII and IX which meant huge amounts were needed.
It was during one of my dad’s stays in hospital that he met my mum, she was a pretty flame haired Irish nurse who like many Irish girls of her generation had left her home country for better prospects and a career in nursing.
There always seemed to me to be something deeply romantic about their meeting. Two people bonding through healing, she gave him his health and he gave her a good life. They fell in love and got married and the silver lining was she had the experience and understanding to nurse him at home when it was possible.
In 1965 Dr Judith Graham Pool discovered that the precipitate left from thawing plasma was rich in factor VIII.
Because cryoprecipitate contained a substantial amount of factor in a smaller volume, it could be used to control serious bleeding, blood banks could produce and store the component making emergency surgery and elective procedures for haemophilia patients more manageable.
By the 1970s freeze dried powdered concentrates were available factor concentrates revolutionised haemophilia care because it could be stored at home allowing patients to self-infuse without the need to go to hospital for treatment.
In 1966 we were at the end of a lovely family holiday in Ireland, we’d been staying at my mum’s family home, a small dairy farm down a little twisty lane at the foot of a mountain, it was idyllic.
We were packing the car, a little blue mini traveller, the back doors were open and Dad and I were carrying cases, precariously hobbling over an ancient, rough and uneven cobbled farm yard, Dad lost his footing, stumbled and fell hard on his side, he got up, he was ok, smiling he held out his bloody hand, just a superficial cut to his palm.
We finished packing the car throwing suitcases haphazardly into the back and hopped in, my older brother and I in the back, Mum and Dad in the front.
I sat right behind my dad, no seat belts back then! I gripped the back of his seat tightly feeling his shirt, soft against my hands. We set off on our journey home, ten minutes into the journey my dad collapsed over the steering wheel.
My mum managed to pull the car over and screamed at a passer-by for help, she shouted in anguish, ''Call an ambulance!''
But it was too late, my dad was dead, his spleen had ruptured in the fall and he had haemorrhaged internally, there was nothing anyone could have done.
My lovely Dad died when I was four years old because of haemophilia. Which is why it is so vital that we research for all rare diseases to prevent such tragedies. I still have flashbacks to that day. It was extremely traumatic and although I've intellectualised it, emotionally I’ll never come to terms with what happened to him. He was stolen from us, from me, far too young, at the age of 48.
World Haemophilia Day on Saturday 17 April
Haemophilia is a rare condition that affects the blood's ability to clot. It is usually inherited, and males are most affected. Normally, when you cut yourself, substances in your blood known as clotting factors mix with blood cells called platelets to make your blood sticky and form a clot. (NHS)
You can find more information about rare blood disorders in this edition of RARE Revolution Magazine:
Rare Revolution Editor