It’s a long story, sorry.
I had my Niemann Pick B diagnosis at age 3 – a pot-bellied, skinny, sickly and sleepy goblinous screamer misdiagnosed with Leukaemia. I remember the hospital as a prison cell-like concrete box, though it definitely wasn’t. My mum would recount how she couldn’t take a shower because any door between us felt like abandonment to me, and at the same time our family cat was dying. That time an evident imprint upon her, I’ve often thought about the weight of my existence on others.
I don’t remember much from that age. I’m told children occasionally tried to make fun of me at school because I was ET-shaped; skinny arms and big belly, the effects of a storage disorder on a child’s shape, but I don’t remember it. Around age 8 I finally lost my fear of blood tests, sat next to another child at St Helier’s, who screamed and cried in a way so distracting that the phlebotomist took my blood without me noticing.
I was 15 or so when I was first told an experimental treatment is being developed. Decline had become this familiar prospect, close enough that I couldn’t see the full scale of it. I often wished it made me more focused, determined, grateful – but instead it created a sense of drift that I can’t shake. At 17 the treatment is delayed as they run their first phase and unexpectedly kill all of their test mice. I don’t remember what happened exactly, but I get very sick. For a few weeks I’m kept in isolation, a light white box, with a mystery infection. Antibiotics aren’t getting a grip on it, and I remember people visiting in full PPE lamenting my situation, smiling and joking to reassure them it’s all ok when it’s probably not. My mum doesn’t get that side of me, especially in reaction to painful attempts to brush my sweat-matted hair. A student doctor tries five times to take my blood, I grit my teeth, but eventually both he and I are crying. In all these situations I want to shrink away, coated with guilt and hyper aware that what is happening to me is all around me. Some friends try to sneak a drum kit and guitar into my room to play me a hospital gig. It is still one of the most romantic things anyone has ever done for me.
After I’m discharged, I go straight to a house party. I begin thinking a lot about my own death and so everything hovers between feeling like it matters too much and not at all. Around this time there was a rumour being spread that I was sleeping with half the year at a local boys’ school. My boyfriend at the time was one of them, an ultra-confident nouveau riche full of labrador energy, an antithesis to everything about me through which I live vicariously. As a teenager there’s no way to be without being interpreted as something else, and trying to be Good feels exhausting in every single circle, as it still often does. So I ignore it all, banned from contact sports at school but drunk on a trampoline at 8pm, everything’s a drama and a laugh because it’s all going to end someday soon.
At 19 the treatment was delayed again; a contamination reportedly resulting in the collapse and absorption of the drug company into a larger one. It’s time to Grow Up. As an exemplary product of the school system, I could probably have done whatever I wanted at (or instead of) university, but having never considered what I wanted, I followed a boy. Not even a year in I was really seriously depressed, in a situation where I’d become very isolated. There’s a particular loneliness in feeling your life become less liveable slowly enough that barely anyone notices, unless you don’t do your washing up, so you hide it in your room until you can. I’m sleeping half the days away and I feel like I only half exist. I lost count of the times I ended up in hospital, struggling to look after myself and dealing with infection after infection. One time my parents happen to be visiting. I’m so delirious with a fever I think there are worms under my skin. I have a vivid memory – or maybe it’s another like the concrete box – of resisting being undressed for an ECG in emergency care in front of my mum, someone being resuscitated, feeling the weight of comparison, futility, my mum’s pain, all in one go, watching myself from the ceiling. Shame and shame about my shame, shame upon shame upon shame.
I beg those I love not to abandon me, and they promise me they’re not, but at this point I can tell people who’d known me a long time had understandably got bored of talking to me when I had this bleak outlook and nothing ever changed. You wait; you delay your life in waiting; you don’t dare to want or plan or dream, and in the meantime everyone else moves on with their lives. I’d learned early that my distress made people uncomfortable and I thought that if I wanted to be loved, I had to soften undesirable thoughts and feelings, quell that part of myself, joking from hospital beds, full of apologies and gratitude, downplaying physical and emotional pain, easy patient, undemanding friend and partner. But I was exhausted. My frustration increasingly played out in the worst of myself, bubbling up into unrehearsed anger which corroded away much I loved. I wanted to manage how my discomfort landed on other people to not lose them, but at the same time, I didn’t want to lie. My hope for things to get better was built around wanting to relate to and be with people, but also for what was rotten in me to stop affecting them so much.
At 21 the gaps in my social circle left space for people I could crumble away into. In them I experienced a love that was quiet and practical, never accompanied with hints of how much they were doing and what I couldn’t. They would and did rescue me many times, bring me home and wrap me up, figuratively and literally, in this weird unquestioning unconditional way I often couldn’t understand and often struggled to return. Maybe it was easier to sit with my reality as an evident part of me that was all that they’d known, rather than a slide away from something I’d been. I seemed to be in hospital less, but could still never be in hospital without crying myself to sleep the first night. I was used to being poked and prodded, but I felt the exact same deep fog of vulnerability each time; the same feeling I had as a three year old child in a strange blank space, when I left home, when my relationship ended; fully at the mercy of others, at the mercy of yourself. The hospital tested me for neurological impacts of the disease. Staring out the window at the park they conclude I’m just easily distracted.
At 26, finally the trial was here!
Life has impeccable timing, and I’m in Lincolnshire with Oli for his mum’s last weeks. Simultaneously we’d been undertaking a blur of assessments in London to determine my eligibility, all of which had to be done in a month-long window. I’m worried I’m not eligible, that surely they’ll assess me and find I’ve been making it all up. When I’m asked to repeat my MRI for the third time, we have to refuse. What happens after we both draw a blank. My partner through space and time sits in the windowsill watching the rain, everything is a mist of waiting. In the days before Shelley dies, we lie to her and say I’ve been accepted and it’s all going ahead. We don’t remember when I actually get accepted, disguised under layers of memories we’ve both repressed. My spleen is 15x normal size (fifteenspleen™) and my lung function is at 40% of normal. Shelley dies late September, and the trial starts 31st October, my mum’s birthday.
[Oli says “It’s nice to be able to pinpoint the worst year of my life so far”]
Trial eligibility begins a life structured around hospital stays and appointments. Hundreds of fortnightly infusions, MRIs, CAT scans, ultrasounds, dexa scans, treadmill tests, lung function tests, biopsies, thousands of vials of bloods. The trial is placebo controlled for a year. Some of us are doing everything for saltwater. Being on a placebo-controlled trial is like a test of what you’ve learned to live with and whether you’ve been making shit up your whole life. I still try to gauge if what I’m feeling is ‘reasonable’ or not.
Hospital life long term is isolated, by both necessity and by indifference. Everyone is alarmed about the possibility of you soon dying, but far less excited about the possibility you not soon being dead. Over three years, visits aren’t forthcoming from any but the dearest of friends. Some people never come, and at times I slip into the pattern of that begging creature of my early twenties, a smaller and smaller voice trying to communicate the weight of the situation whilst trying not to push them away. It’s 2018 and it’s the era of self-care and eliminating negative energy. Disablity is prime fodder for Inspirational Content, but I can’t figure out how to feel positive about someone sticking a giant needle in my liver. Work is a whole other source of frustration, in the masochistic art world where everything is again about constantly saying and being The Right Thing whilst salaried council workers and PhD intellectuals remind you of everything you’re doing Wrong. Part of inspiration culture is the idea that is suffering should put everything into perspective. I find it doesn’t make the petty bullshit not matter, it just makes it more annoying.
Luckily I’m smooth to frustration and disappointment like a well-worn rock. So, over time, I begin to feel simultaneously incredibly content. I stop defining my experience by measure of other peoples’. My body’s not my body, but it’s started to feel like the most ‘mine’ its ever been, under the influence of my own desires and decisions I’ve made for myself. I live lording it up as queen of the holiday inn every fortnight so I can be in early enough for PK bloods, the hotel receptionist’s nominated honoured guest. I’m on a private ward being brought mac and cheese under a silver cloche, feeling full of bravado like I always imagined the private school labradors of my youth felt. I still grit my teeth every time they stick a needle in my arm, force of habit. But if it hurts, I learn to say so. I have time nobody dare touch. In the daytime I gaze at the pigeons and Simpsons clouds in a rectangle of sky I can see through the ward room window. At night in-between 3am ECGs, I sleep like a baby in the safety cage of a hospital bed.
I try not to think about it, but I don’t think I’m on saltwater. Learning what I’d learned to live with, it turns out it’s not normal for your lungs to hurt when you breathe. My lung function improves to 70% and I spend more of my time outdoors, up with the mountains and trees and moss. Days recovering from any exertion are a thing of the past, so I’m not pushing myself beyond my limits just to feel like part of something. My body changes shape, fifteenspleen™ to five. It’d be called miraculous but it’s fucking not, it’s actually science! The treatment halts and even partially reverses the progression of the disease to a point where I can function and thrive on a daily basis, help others, build friendships, experience things I never thought I’d be able to, think about the future and think about what I want. Some damage might be irreparable, but life is irreparable damage.
At aged 31 Covid puts a abrupt stop to the side room idyll of beautiful research nurses, dinner ladies, night staff, with no goodbyes. We have to stay inside for the best part of a year of shielding. It’s a whole other story of isolation, with everyone’s cruelty seemingly amplified at the end of it. A few times I’m ferried up to the National in a black cab through completely empty, ghostly streets to hospital, but ultimately the situation accelerates the process of switching to the home infusions my consultant had been chasing for the past year. I’m not keen; a load of medical equipment in my house breaks the ordered compartmentalisation of my life and the nest is a shambles of hospital, work, and cohabitation. Trying to keep ghosts at bay whilst trapped with them inside, they take on a cosier, familiar form. As much as my physical shape can change, the inner life takes a little longer, and so she retreats back to the pattern and squashes it down; 𝑖𝑡’𝑠 𝑎𝑙𝑙 𝑓𝑖𝑛𝑒; 𝑎 𝑛𝑜𝑟𝑚𝑎𝑙 𝑟𝑒𝑎𝑐𝑡𝑖𝑜𝑛 𝑡𝑜 𝑎𝑛 𝑎𝑏𝑛𝑜𝑟𝑚𝑎𝑙 𝑠𝑖𝑡𝑢𝑎𝑡𝑖𝑜𝑛. That’s my life really. It’s obviously not fine, but again that’s another long story.
As quickly and unceremoniously as it began, the five year trial period is finished. The tests stop, the bloods stop, and I continue fortnightly infusions at home on something called ‘compassionate use’. Compassion here takes the form of the big American drug company not withdrawing my treatment. As long as they remain compassionate, I’ll do this for the rest of my life, hooked up for 6 hours, tethered to place and time.
I have an embarrassing habit of thinking change is going to be a grand bookmark in my life; this is the day! I wanted to write a logical timeline of events but ended up with an emotional outburst, because that’s the truth of it, and concluding when there isn’t a conclusion feels false and trite. Maybe, a few things I learned. Little me thought things mattered too much or not at all, but it’s always been both. Part of change was acknowledging that coexistence. No matter where you go, you bring yourself with you; it’s not necessarily being attached to a drip that means you’re stuck in the same place. Change was all little things; going to sleep in a hospital bed without crying, not apologising for needing help, not softening ‘negative’ feelings to stay loved or bothering to beg, not saying you’re fine, having the strength to stand your ground, refusing. One day you find yourself changed and find pain noticeable by its usual absence, find you’re no longer sleeping your life away, find yourself inexplicably at the top of the mountain, a day when you go to bed and realise you’ve not thought about death once.
It’s a long story, I’m not sorry and it’s going to be even longer than expected.