#haemophiliacs always ask the same question

[l3vi4than]

What if the bear is a panda who is as clumsy as me and mainly herbivore

What if the woman is that nasty school teacher who bullied you for your handwriting

What if the man is an expert in wild hiking

Idk I’d die of bleeding soon after tripping over my own leg anyway, so it’s not even a relevant question to me. Which one of them pilots the search mission helicopter? Or at least has an ampule of coagulants? Anyone? Pls???

Would you rather be stuck in a forest with a man, woman or bear?

i don't think i would mind any kind of human if my other option is a bear

[chasingrobbie]

The Doctor Is In: Chapter 1 - Part 1 (Open Heart Book 1)

Pairing: Dr. Ethan Ramsey x F!MC (Dr. Harper Dorian) 

Word count: 2000

Warning: None 

Author’s Note: I’ve decided to take the plunge and have a go at publishing a bit of a series. As I’ve been replaying Book 1 I’ve just wanted to explore Ethan’s character more, so I started writing entirely from his perspective. 

This ‘series’ (hopefully I don’t get frustrated with myself and stop) takes the events from Book 1 as they are and then I have added my own stuff. I’d love feedback from anyone who give this a read. 

Yes, my MC has the same name and Harper Emery but that’s what it’s always been so yeah. I’ll make a joke out of it somewhere I’m sure.

Aaaand if you want to be tagged in future parts, let me know :)

Ethan’s well-worn running shoes hit the pavement with force as he lengthened his stride. The air was crisp and clean, the kind of air that stung the lungs, the kind of morning that he treasured; he inhaled deeply, desperate to drink in the solitude. This time to himself was never enough to satisfy the drain from the myriad of inane questions that awaited him each day at the hospital. Though today was different. Ethan knew that. The new interns were starting today. Nervous energy would flitter through the wards, trepidation and generally misplaced confidence would permeate the halls. Some of the best young new medical minds in the country had been hand selected by Ethan and the other notable staff of Edenbrook. God, all he could hope was that they didn’t cost him too many patients.

As usual, Ethan arrived at Edenbrook early to seek further sanctuary in the walls of his office. He poured through his current patients charts that had been updated in the short time he had been away from the hospital. It was becoming increasingly commonplace for him to fall asleep on the couch in his office. Jenner was really the only thing that pulled him back to his apartment with much regularity.

Slowly, the morning approached 8:30 and he knew that the interns would be gathering soon for their opening address from Harper. How far she had come still managed to shock Ethan at times. She had always been a formidable woman; it was one of the many reasons he had been attracted to her. But it was different now. She was different now. Ethan drank deeply from his coffee mug, the rich warmth spreading through his chest as he closed his eyes. A languid and purposeful inhalation filled his lungs as the familiar beep of his pager pulled him from his reverie. Naveen wanted to see him. Quickly, Ethan slugged back the last of his coffee, slipped on his white coat and left the privacy of his office.

Making his way through the hospital was always a task, so many people wanted pieces of his time. Though Ethan had not anticipated what was about to quite literally crash into his morning. Into his life. He was discussing one of his patients with a nurse when a commotion could be heard from the waiting area. Without hesitation he moved to help.

“Everybody, step back! I’m a doctor.” A voice cut through the confusion and concern of the onlookers. Ethan had been too far away to see who had spoken but judging by the ridiculousness of the declaration, that they are in fact a doctor in a hospital, he assumed it was an intern. And he wasn’t wrong, he’s never wrong. Quickly, he knelt down by the woman’s side. Adrenaline never took long to flood his veins. There was a time when his breathing would have been erratic because of it but now it fuelled him.

“Pulse is weak. She’s unresponsive.” Ethan stated without incident. It was then that he glanced around him and saw the intern he supposed had called for everyone to clear the area. The juxtaposition smeared across her face was all too familiar – total fear clashing against abject excitement. He remembered the feeling all too well. Their eyes met and Ethan noticed something else etched in the intern’s warm green eyes. It took him a moment to place what it was. It looked like resolve, which in the eyes of an intern was a rarity.

“You. Rookie. Get in here.” Ethan’s tone was clear and direct, as always.

“Coming!” The intern shouted without an ounce of hesitation. Ethan eased the patient into his arms and onto a nearby gurney. Before he could register it, the intern was at his side. Ethan could feel the heat rolling from her body. He needed to be careful, there was every chance she could be overtaken by the fire to help and make a costly error. He needed to help her focus, just as he had learnt to do.  

“What was she coming in for? Did she fill out a form yet?” Ethan quickly asked one of the nurses, Danny nearby.

“No, she’d just walked in,” Danny said dejectedly, his shoulders falling, knowing this could likely mean her death.

“If we don’t figure out what’s wrong with her fast, she’s gonna die on this table,” frustration was building in Ethan. Knots formed in his chest, the warmth and relief of his morning coffee replaced with thundering torrents of fear. He took in a languid and purposeful inhalation to steady himself.

“Rookie, check her B.P.” Ethan met the intern’s eyes for a second time, streaks of desperation had replaced her excitement.  

“It’s plummeting. She’s hypotensive. We’ve gotta get fluids in her,” the intern voice was accelerated but the tone was steady. She was doing well. Ethan was impressed. Almost instantly, Danny placed the I.V and the patient weakly clutched at the young intern’s hand.

“Doctor, look at this bruise. It wasn’t there before,” the urgency in the intern’s voice was evident. Ethan’s attention shifted to the deep bruise the was blooming on the patient’s arm.

“That’s the elbow she landed on when she fell. A bruise forming that fast would suggest that this woman is a haemophiliac. Good catch.” Ethan was further impressed. If the patient’s life didn’t hang in the balance, he may have even smiled.

“Also see her fingertips? Low oxygen saturation in her blood. Take a closer listen to her lungs. Hurry.”

“I can’t hear anything on her left side! And her right lung is struggling! Doctor, she’s going to suffocate!” The intern was frantic, but Ethan could still make out the resolve in her eyes. He hadn’t lost her yet.

“Nurse, we’ve got a code blue.” The words left Ethan’s mouth in cool trail. He’d done this enough to breathe through the chaos and the looming vultures, but he could tell that the intern was astonished and impressed by his calm exterior. He took the bag mask from the nurse and gently pumped air into the woman’s lungs. His hands moved with precision and practiced rhythm. The intern’s eyes raked over him and he was acutely aware of her eyes resting on him. Strangely, he liked it.  

“What do we do, Doctor? What’s happening to her?” Fear had continued to strangle the intern’s voice, Ethan knew if he didn’t pull her back from the precipice, she’d be overcome with it. Slowly, deliberately he uttered words of reassurance to tether her to him in this shared moment of crisis.

“Consider all the clues. It’s all there. You know this, Rookie.”

“It’s…it’s, uh…” Ethan watched entranced as she closed her eyes and took the same languid and purposeful inhalation he had taken earlier. Her eyes fell open and Ethan knew she had it.

“It’s a hemothorax!” Relief swum across her features.

“Precisely. A blood vessel ruptured and is filling her pleural cavity…”

“…blocking her lungs from expanding! That’s why she can’t breathe! But we can’t repair the blood vessel here!” The intern finished Ethan’s explanation and quickly reached the conclusion he had come to about a minute earlier. Again, a juxtaposition of emotions was spread across her face; satisfaction, dread and panic.

“We’ll have to do an emergency thoracotomy to drain the cavity instead. Nurse!” The nurse gave the scalpel and chest tube to the intern who had to quite visibly gulp down her clots of fear. Ethan lifted the patient’s shirt to expose the side of her rib cage. The flesh is too hot beneath his fingertips.

“We need a local anesthetic—” The intern’s fear was running away from her. Her voice trailed, lost amongst the temper of the moment. Ethan set his features into a firm and unforgiving line.

“We’re outta time! Do it now, or this woman’s life is on you!” Ethan’s voice was emphatic and authoritative as it rang clear through the throng of anxious onlookers. The intern’s eyes shifted, followed by a sharp intake of air. Ethan could hear her muttering the steps under her breath.

“Incision at the fifth intercostal space…anterior…to the mid axillary line…” Her hand was quivering. Almost instinctively Ethan reached out and took her trembling fingers in his own to steady them. Her skin was ablaze, tension radiated from it but he felt her relax into him. He found her eyes, his gaze softening as he took them in. The green edges of her irises were mottled with a golden hue. He’d never seen anything like them before.

“Hey…you can do this.” Ethan breathed the words out and the room had melted away. The onlookers, the nursing staff, no one else was there but them. She nodded and focused on her unsteady hand. Ethan still had her hand enshrouded in his own but now he could feel the trembling subside. Tension was replaced with resolve.

“There you go. Nice and easy.” Ethan voice cuts the air once more. Time had been suspended but the incision of the scalpel brought the rest of the world back into clear focus.

“Now the tube.” Hands still connected; they push the tube into the woman’s chest. Warm, crimson blood spurts from her side but quickly the blood begins to drain. The woman takes in a sharp breath. The intern is visibly elated. Fear and resolve are replaced with genuine delight and a little disbelief.

“We…we did it!” Ethan still has her hand in his, he squeezes it reassuringly. For a moment, Ethan thought she was going to attempt to hug him. Maybe she was.

“She’s stable. Get her into surgery…She’s gonna make it.” Applause erupts around them. For the first time in minutes, Ethan is reminded of the crowd gathered in the waiting room. The patient is wheeled away, the familiar wave of relief washes over Ethan. These moments. These victories. They are what give him the strength to continue through the tedium of his days, the times when he doesn’t succeed, when he can’t help.

“Doctor…that was…absolutely amazing!” The young intern turned to Ethan; she was still obviously riding the high of this first real-world victory. Ethan knew what he had to do. She had shown so much promise. He needed to push her.

“You’re right. It’s pretty amazing you didn’t get her killed.” The words fell from Ethan’s mouth in a cold tumble, heavy and loaded. He watched the joy crumble and confusion stretch its away across her face before she even spoken.

“Wait, what?” Her voice was smaller now. Ethan had to be pedantic, he needed her to walk away from this questioning what she could have done any better. The endless pursuit of perfection is what every doctor needed.

Ethan continued, matter of fact and critical. “Your examination was slow and superficial. Your scalpel technique, amateur at best…”

“Amateur?” She cut him off before he could continue. The edge to her voice was hostile not apologetic. She wasn’t upset, she was frustrated. He liked that. A glimmer sparked in her eyes.

“Maybe you could give me private lessons.” She smirked at him coyly.

“Ha! I just might, Doctor…” Ethan couldn’t help but smile. Her confidence took him aback. He grabbed the ID card hanging from her lanyard and inspected it closely. A realisation dawned on him; he knew who she is. Ethan recovered his composure in the split second.

“…Dorian. But I sincerely doubt you could afford my salary.”  

Ethan turned to leave, smiling to himself, and continued making his way to Naveen’s office. He could hear the murmurs of the onlookers as he departed. Yet, Ethan could only think about the intern. She was the one he had selected. She truly lived up to her application and test scores. Few interns could do what she just did on their first day. But there was something else. Ethan ruminated on it for a moment before her eyes seeped into his consciousness again. And that was where he left them as he continued down the hall.

The eyes of the intern remained fixed on Ethan as he walked away.

------------------------------------------------------------------------------------------

Soooooo yeah let me know what you think about if you’d be interested in reading Part 2 of Chapter 1. 

Thanks for reading!

Adding a tag for @choicesficwriterscreations 😘

[chamrosh]

Okaaay

So. I promise this’ll end up somewhere a heck ton different to where it’ll initially look but yes I just need a long vent okay and honestly this whole first section is probably useful to loads of people so it’s not going to be abridged by me (though if anyone wants to add a tl;dr if this somehow get’s reblogged, go ahead).  There’s probably gonna be more of these covering... different things, some of which will be related.

Anywho.

When I was in year 5, I had my first period. I lost 1/8 of my bodyweight in a week and the blood kept coming for another week after that. I was maybe 10, but probably 9. I had already grown intensely uncomfortable with the idea of being expected to be a woman when I grew up. I think I was born with a clock inside me, because exactly a month later, I had my second period. I lost 1/10 of my bodyweight, not having regained the weight from the previous month enough to support that. I tipped into being dangerously underweight at that point and didn’t have another period for a couple of months. 

I complained to my mother about how it was too painful and how much blood I had lost and how much blood there was everywhere and OH MY GOODNESS why did it have to hurt so much, what were those great big clumps - And... and her fucking response was to tell me “it’ll hurt less once you get pregnant”. Ah yes. The solution to a 10 year old being in pain. Encouraging them to give your grandchildren already. A++ parenting. And whenever I asked, I’d always be told some variant of “it’ll hurt less the more children you have.”

That summer, my brother had been spending a month in Germany with my mother’s penpal from school, to help pick up enough to be able to do well in GCSEs. All fair and good there. Except that the youngest child of the family he was staying with, let’s call him Mike, had been to Ecuador to help with anti-poverty work for a month before that. Mike had been sick while there, but he had recovered after a day, and it was a week before he came back to Germany. My brother woke up a few days after arriving and started violently vomiting. My mother’s penpal is a pharmacist, so she rushed to her practice and grabbed as many things that would help and not cause complications together as possible, from her own pocket, and started giving him the doses of each. My brother started having violent diarrhoea too, and this had blood in it. 

He was taken to hospital, and spent the rest of the month abroad there instead. Every day he lost 6L of fluid in excess just from the mixture of blood and diarrhoea, before the additional sweating he was going through. The hospital diagnosed him with a bleeding disorder, which isn’t haemophilia, but I shall call “haemophilia” for reasons of what it actually is being pretty rare and haemophilia being really similar and far more common, and honestly haemophiliacs need more recognition than peeps with my condition do based purely on numbers and i’m happy for any recognition of bleeding disorders because of me to go there (especially as most of the time my bleeding disorder is covered under the same hospital departments...). He was sent home after this and we had to keep him essentially quarantined for another 2 months. The hospital told my parents to get me and my other brother checked up for “haemophilia” as soon as possible. We did not receive that check up then, but instead nearly two years later. We were advised to get hepatitis injections too, after it was seen what hepatitis C could do to us, and to get those as soon after we were diagnosed as possible... and I’m pretty sure I still haven’t had my Hep shots. 

Note that my monster periods starting happened after I was recommended to be checked out for bleeding issues and yet I was still just told “it’ll be fine if you have a ton of kids” by my mother. And may I point out that the idea of anyone putting anything up there in me makes me physically feel ill, and my imagination kind of glitches and physically won’t let me imagine any version of myself being pregnant or giving birth or anything like that and oh goodness did I try to force myself to manage it when I didn’t realise that even just not having kids was a valid option for people...

The hospital (which does have a proper name, but, again, rare disorder, I’m not naming it) eventually had to nag my mother to take me and my other brother up there for checks.

I remember when I started secondary school, and there was an assembly where “all” the boys and all the “girls” had to be split off for basically crap sex ed classes, and the teacher who did the “girls” one basically said that “oh your first one doesn’t hurt” and “on your first one this teeny tiny pad will do” and just, trust me, on my first period I’d have bled through the starter pads that were given out within about 5 minutes if I were lucky. Both a comment on menorrhagia and on the tininess of these pads. In a moment of disgust I took the first opportunity to get them out of my sight (by burying them at the bottom of my PE kit) and utterly forgot about them being given out for about 5 years. 

I hated being in that room so much on so many levels. First, because it was a girls’ assembly, second, because the teacher kept handing out things for girls, which I just flat out refused to believe would be useful to me (because I’m a stubborn lil git when I want to be, but also because most of them would genuinely have been), and third, because she flat out lied. At least, from my view. I thought that losing 1/8 of your body weight on your first period was normal. Bear in mind as well that the puberty related info I had from my periods was entirely contained by giving me a book on puberty and walking out the room. I flicked through it once, realised I’d grow breasts, started crying, and threw it in a corner. I had no further interaction with that book (beyond actually closing it) for about 3 years.

When in class, one of my friends said that their teacher in a different subject had said that during periods you only lose about 3 teaspoons of blood, I refused to believe that a period that light was even possible. 

And... all the girls seemed to be able to keep doing everything through the whole month. They didn’t seem to have to curl up into balls and spend their break times curled up down the back end of the school just praying the pain would pass. 

When I finally got to the hospital, a year and a half after I was meant to, they did the blood test, I was super proud of how strong and manly I’d been that I didn’t faint at losing a ton of blood to the needle and my brother did, and this is yet another mini-installment in signs of gender dysphoria that small me didn’t register right here. And they said they’d call up about stuff after too - but before we left, the doctor said I should go on the contraceptive pill. It should probably horrify you to know that I knew what rape was when I was five, but I didn’t know what contraceptives were until I was twelve. But either way, I heard my mother refuse, and I wondered what it was, so I asked, and she explained that it gave you female hormones to make you not have children and that it was very bad because then you might not ever have children. I disagreed. It was very bad because it was female hormones. But even so, I was glad at the time that she’d said no.

Every single appointment - that is, twice a year - I got a call. Every single time I was asked if I wanted to go on the pill. I said no. I came up with lame excuses every time but I knew deep down it was always because I didn’t want to have any female hormones. My periods awfulness would vary. Initially it was always losing huge chunks of my weight, but more and more it’s manifested as me not being able to swallow anything at all bitter, and throwing up anything i’ve eaten if I try, and in having to pass enormous clumps through down there. 

They started out smaller, like the size of the top joint of my thumb. It’s a sign of significant medical issues once you have a lump larger than a nickle / about a pound coin. I jumped from teeny tiny lumps to lumps about twice a diagnosable size. I had not been taught that lumps that size were not normal, and so I didn’t think it was anything significant when I was asked about it... plus, I knew they’d only suggest putting me on the Pill again... 

My periods have always been pretty regular, as long as they’re not disrupted by intense stress (although I learned I could sleep less and make the periods less frequent, and that has to have been one of the worst decisions in terms of my grades I’ve ever made...), such that through the whole of biology in year 11 the worst stage of clumping would always be within the same half hour span on a Tuesday morning, during double biology. I used to deliberately hyperventilate, because when I was on the edge of fainting, I couldn’t feel it anymore. I couldn’t feel that disgusting lump making me acutely aware of an organ I do not want and did not ask for. I love biology. I hated having to miss periods of it for - hah - periods, every single month, but it was better than the alternative. 

When I was 15, I started getting intense shooting pains through both sides, about the length of my hand below my ribs. When I went to the GP, I was questioned  for what felt like hours, - with my mother STILL IN THE ROOM - if I had had sex with any boys, and whether I was pregnant. It made me feel genuinely ill to have the suggestion that I could ever be pregnant. And! Me! Having sex with someone putting their penis in me? No!

Turns out, once that questioning had stopped, I had ovarian cysts. On both sides. I’m almost guaranteed to be infertile - and I was told such at the time - because both of my ovaries had had it, and I’d had it on and off, and it had worsened over ovulation... and they were causing me enough pain that when they flaired up, I’d tense up, my back would curl defensively whether I wanted it or not, and I couldn’t get myself to move or talk. Those are not healthy ovaries. Honestly, it came as a relief to hear. I love the idea of having children, I really do, but to hear I wouldn’t be giving birth! Fucking great feeling, my dudes.

I hated going in the bathroom so much... I’d refuse to go. There were concert days, at least one each term where I’d have to leave the house at 8am and only get home at 10pm and I wouldn’t have gone to the loo in all that time because I hated the loo that much. It was relatively common to have to leave the house at 8am and get back at 6pm, or anything up to 8pm, and to have not gone to the loo in all that time. Anything more than about 4 hours gap regularly is bad for your health. The only time I would go to the loo in school was to get changed for PE on my own if I couldn’t deal with being with the girls (which happened a lot) or to deal with period matter. 

During one lesson in year 9, double history, I felt the pad stick to the chair, and I didn’t dare budge an inch from where I was for the entire hour and a half. I procrastinated until I was the very last person sitting down from class still, and when I stood up, the pad ripped, and within a few seconds, the whole of the insides of both my legs were covered in blood. I knew I had to go to the loo to clear it up and replace the pad, but I still didn’t want to.

I started having clumps comparable to the size of the whole of the palm of my hand.

When I finally spoke about this to the doctor (and came up with yet more dumb excuses for why I didn’t want to be on the pill), they finally got me booked for an ultrasound. The forms stating what the procedure is say, by default, that you have to have instruments stuffed up your there so that they can see what’s going on internally, and I started presumably visibly panicking, judging by the fact that they immediately started discussing alternatives. You can have an external one through the front if you’ve not used a tampon or had vaginal sex, so if you’ve not done either of those, and you have period issues (especially to the same extent as me!), and the thought of anything up there also makes you panic, it’s probably best to continue to avoid them.

When I went up to the hospital, first i was super uncomfy because you have to drink a litre of water an hour before the ultrasound is done, and I knew that I’d have to go to the toilet there... but second, because the nurse doing it needs to have a lot of skin exposed. I get why. I also get why they picked a small, non-threatening looking woman to do it, but that also didn’t really help the discomfort. Nor did having to go into gynecology...

Anyway, normal period lining thickness is around 14mm thick at peak (obviously there’s a variation around that that’s perfectly healthy that’s a few mm wide). Mine was 34mm thick halfway through to ovulation. Which would explain how I basically manage to have a baby bump every month... And again, the nurse said I wouldn’t be getting pregnant. Embryos are not going to fare great in terms of getting enough nutrients there.

I liked the idea that my body was trying to provide for some stupidly manly baby. Only stupidly manly babies who could obliterate a uterus from the inside were welcome. Yep. It’s best not to question how I think sometimes but honestly I think I’ve made it sound as close to rational as I can there. 

I had a panic attack over the phone call a year ago. I so wanted to say why I really didn’t want to go on the pill! And I was so scared that it was the only way to end the size of the clumping.

In July I managed to produce a whole collection of huge clumps, one the size of my whole thumb, one that was the length from my middle finger tip to the butt of my palm, and several others, all of which were very safely in menorrhagia territory... In September I managed to produce a clump the size of my fist...

I knew I didn’t want to have to deal with that any longer. But I’d also finally accepted I wanted nothing to do with me being feminine, and I knew what I had to say. And I started out the phonecall, literally last week, saying what I’d need to say as a numbered list and everything, setting it out. It still took me about 5 minutes from saying I had a third point and being prompted to say it that I finally got out my reasoning. I was asked what the issue was. I said again that it was female hormones and I didn’t want them. And again. And again. And again. And again. And then finally they got it. 

I finally had an alternative suggested (which I still need to go and get sorted out because oh boy am I disorganised). And they said that the appointment was already longer than it technically should be, and that they really needed a good section of time to talk about how my gender interacted with my “haemophilia” and so they said they’d book my an appointment, not say what the appointment was about on the letter, but that that appointment would be about gender, and would be in my Easter holidays. 

I think I practically died of excitement at having something gender-affirming to do officially that’d maybe be a first step in transitioning.

And then I checked my email this morning.

The letter has arrived at my parents’ house. My mother opened it. And she scanned it and sent it to me.

Her thoughts weren’t to scribble out her address and put mine and mail it along like a sensible human being with a basic comprehension of what boundaries are. NOPE, not my mother, not the woman who’d recommend that a 10 year old become pregnant. Of course not. No, she had to go and open confidential medical letters. And she didn’t even have any shame about that! Just straight up emailing me about having done so, and showing me proof that she had done so!

I’m so bloody relieved that the hospital were truthful about that, and that it wasn’t specific at all and just listed the hospital department I have to go to for it (which actually is the haemophilia department). That would have been a fricking awful way to be outed. Can you imagine that? Parents who told a 12 year old that if he turned out to be a lesbian, they’d kick him out the house. As a 12 year old. Who said to not even talk to trans people, let alone make friends with them. Who nearly broke off contact with their kids’ godparents’ son because he came out as pansexual. Who rant about how “society’s gone too far” and that “you can’t just choose” and that TERFs are completely and utterly right about everything for a full week after a single comment is made. Honestly I don’t think I’ve ever more concisely said why it’s taken me so long to actually admit that yep, I’m trans, and also to try coming out to any family members. Can you imagine? Finding out from having precisely 0% of a concept of privacy? My mother was horrified enough when my brother mentioned he was getting his tubes cut and that he and his wife are planning to adopt kids instead. Can you imagine her reaction? 

I really really need to be able to safely permanently move out, if only so that my mother doesn’t think it’s okay to look through my medical letters. 

Also yes that whole first bit was there because I never feel like I’ve vented enough about it ever and it’s fucking awful and it needs a lot of venting. ... but also to give a scope of the medical neglect from my parents and the level of reproductive control in their house, and to give some context to the stupid lengths they’ll go to to avoid having to deal that some people would really rather not have anything to do with what would make them fertile. 

Hopefully now all that is vented I’ll actually be able to focus on what I’m meant to be doing. Which is working out where I’m gonna go for my year abroad. Which, incidentally, I’m going to be Out for, whether my parents approve or not. Also hopefully me actually posting this gives some people a reassurance that yes it’s fine to hate your periods, they suck, and honestly I feel bad for everyone on their periods no matter how much lighter they are than mine, and even if they aren’t a dysphoria inducing nightmare. All periods suck.

[storyswept]

Troubled Blood: Genetics Strike Again

After Lethal White and its mention of lethal white syndrome, a genetic disorder which affects horses, followed Troubled Blood which, guess what, also features a genetic disorder, this one related to bleeding.

‘Have you met Roy? Margot’s husband?’ (...) He’s a haemophiliac, isn’t he?’ asked Gupta. ‘Not that I’ve heard,’ said Strike, surprised. ‘Yes, yes,’ said Gupta ‘I think so, I think he is. He was a haematologist by profession, and his mother told me that he had chosen the specialty because of his own condition.

- Troubled Blood, Chapter 10

We learn in the same conversation that Roy had been ruled out early on as a suspect in Margot's disappeareance, but not the reason why:

‘Now, that was a curious thing. Inspector Talbot told me at the start of my own police interview that Roy had been completely ruled out of their enquiries – which I’ve always thought was an odd thing to tell me. Don’t you find it so? This was barely a week after Margot’s disappearance. (...)'

- Troubled Blood, Chapter 10

While Dr. Gupta believes him to be a haemophiliac, we later learn that Roy Phipps is actually afflicted with a severe form of von Willebrand disease and that he couldn't have left the house at the time of Margot's diseapperance, being bedridden after a serious bleed.

her husband, Dr Roy Phipps, who suffers from von Willebrand Disease, was ill at home and confined to bed at the marital home in Ham on the 11th October. ‘Following several inaccurate and irresponsible press reports, we would like to state clearly that we are satisfied that Dr Roy Phipps had nothing to do with his wife’s disappearance,’ DI Bill Talbot, the detective in charge of the investigation, told newsmen. ‘His own doctors have confirmed that walking and driving would both have been beyond Dr Phipps on the day in question and both Dr Phipps’ nanny and his cleaner have given sworn statements confirming that Dr Phipps did not leave the house on the day of his wife’s disappearance.’

‘What’s von Willebrand Disease?’ asked Robin. ‘A bleeding disorder. I looked it up. You don’t clot properly. Gupta remembered that wrong; he thought Roy was a haemophiliac. ‘There are three kinds of von Willebrand Disease,’ said Strike. ‘Type One just means you’d take a bit longer than normal to clot, but it shouldn’t leave you bedbound, or unable to drive. I’m assuming Roy Phipps is Type Three, which can be as serious as haemophilia, and could lay him up for a while. But we’ll need to check that.

- Troubled Blood, Chapter 14

As Dr Phipps was bedbound due to his recent accident—” ’ ‘Anna told you about Roy’s von Willebrand Disease?’ said Cynthia anxiously. ‘Er – I don’t think she told us, but it’s mentioned in the police report.’ ‘Oh, didn’t she say?’ said Cynthia, who seemed unhappy to hear it. ‘Well, he’s a Type Three. That’s serious, as bad as haemophilia. His knee swelled up and he was in a lot of pain, could hardly move,’ said Cynthia. ‘Yes,’ said Strike, ‘it’s all in the police—’ ‘No, because he’d had an accident on the seventh,’ said Cynthia, who seemed determined to say this. ‘It was a wet day, pouring with rain, you can check that. He was walking around a corner of the hospital, heading for the car park, and an out-patient rode right into him on a pushbike. Roy got tangled up in the front wheel, slipped, hit his knee and had a major bleed. These days he has prophylactic injections so it doesn’t happen the way it used to, but back then, if he injured himself, it could lay him up for weeks.’

- Troubled Blood, Chapter 35

Dr Gupta's mistake might be a historical nod to this disease's original name, “hereditary pseudohemophilia”. First described by Dr Erik von Willebrand in 1926, the clinical presentation of von Willebrand disease differs from haemophilia's in two major ways:

different genetic transmission: whereas von Willebrand disease is a autosomal genetic disorder, haemophilia is a X-linked genetic disorder. This means that the likelihood of having von Willebrand disease isn't influenced by one's sex, unlike in haemophilia which manifests mostly in males (as they only inherit one X chromosome).

different bleeding pattern: while haemophilia is characterised by deep subcutaneous or joint bleeding, von Willebrand disease mostly presents in the form of mucocutaneous bleeding (e.g. easy bruising, nosebleeds, gum bleeding, heavy menstrual bleeding, excessive bleeding after operations or trauma...) However, some forms of von Willebrand disease (such as type 3, the particular form of von Willebrand disease that Roy Phipps has) can cause haemophilia-like bleeding, in this case resulting in a swollen knee.

Von Willebrand's disease is the most frequent genetic bleeding disorder, estimated to occur in 0.1% to 1% of the population. Nowadays, we know that this disease is caused by a deficency or defect of a particular plasma protein, known as von Willebrand factor. As mentioned in Troubled Blood, von Willebrand disease is currently classified in 3 types:

Type 1, a partial deficiency of von Willebrand Factor, is generally the mildest form of the disease.

Type 2 results from qualitative defects of the von Willebrand Factor.

Type 3, a complete deficiency of von Willebrand Factor, presents as severe bleeding. Because the deficiency is complete, it doesn't respond to treatment with desmopressin. Desmopressin, which increases the release of von Willebrand factor in the bloodstream, was first used to prevent bleeding during interventions on von Willebrand disease patients in 1977. This discovery would have had little impact on Dr. Phipps' life who would have had to continue to rely on plasma derivates. Lately, recombinant von Willebrand factor has become a therapeutic option.

Interestingly, though it's kind of logical when you think about it, consanguinity increases the odds of having type 3. Are we to understand that Roy wasn't the only one to marry a relative in the family? I do wonder what caused Galbraith/Rowling to afflict Margot's husband (who would normally be the number one suspect) with this particular disease. Was it merely to create a parallel between "Lethal White" and "Troubled Blood" or did she wish to raise awareness for the disease? I don't think I have read a novel so far where this disease is mentioned by name, have you? Further reading / Sources:

Swami A., Kaur V. von Willebrand Disease: A Concise Review and Update for the Practicing Physician. Clinical and Applied Thrombosis/Hemostasis. 2016;23(8):900-910. doi:10.1177/1076029616675969

Favaloro E. Rare forms of von Willebrand disease. Annals of Translational Medicine. 2018;6(17):345-345.  doi: 10.21037/atm.2018.09.10. PMID: 30306084; PMCID: PMC6174185.

Mannucci P. Desmopressin: an historical introduction. Haemophilia. 2007;14:1-4. doi:10.1111/j.1365-2516.2007.01609.x

Keeney S. Recombinant VWF: is this the answer for treatment of von Willebrand disease?. Clinical Investigation. 2012;2(8):755-757. doi:10.4155/cli.12.79