I have a genetic disorder called cystic fibrosis (CF). The current life expectancy of someone with my condition is 41 years. I’m 38. I’ve been in and out of hospital my entire life, but my admission last year was unlike any other: it almost killed me.
CF sucks. It sabotages a whole host of systems – my own fringe benefits include diabetes, pancreatic insufficiency and osteoporosis – but what ends up getting you is the thick, sticky mucus that clogs up your airways, causing infections, breathing difficulties and progressive lung damage. Normality is several hours of physio and hoovering up more than 20 different pills, potions, inhalers and nebulisers every day.
But I had a mantra – my lungs will not limit me. Nor my bones. And I was fortified with something else: an extra-large dollop of gleeful joy in my heart, so that anything seemed possible. In my early teens, I played rugby like a boy possessed, until I fractured several vertebrae. I went to university, an enormous regime change from parental guidance to full treatment management. And in my early 20s, with a few adjustments, I travelled around Australia and New Zealand, posting out precious parcels of pharmaceuticals to each planned waypoint. As a journalist in my early 30s, I even attempted such bracing assignments as a one-week fitness boot camp, so our readers wouldn’t have to. And every year, a tiny slither of my lung capacity was consigned to history.
It’s hard to envisage a world where you end up with respiratory failure, but many people with CF eventually contend with this. I just never thought I’d be one of them. It all started with a fever one afternoon at work, last year. The flu had been stalking the office, picking colleagues off at random, and I was one of the unlucky ones. Within 24 hours, I had also developed the sort of loud barking cough that a chain smoker would be proud of, and what felt like a baby rhino taking up residence astride my chest. I reluctantly Ubered my way over to A&E. Five days later I was transferred to a specialist lung hospital.
It turns out that, with a condition like mine, when you’ve got the flu it’s like flinging open your immune system’s back door and inviting in every bacterial nasty loitering outside. The unhappy upshot: a three-month hospitalisation peaking in respiratory failure. For 10 days, things were dicey. A steady flow of liquid paracetamol cooled my fevered veins as infections ransacked my airways. My lung capacity was in free fall: I couldn’t leave my bed because it cost too much oxygen to move. It’s bloody scary when you feel like you’re slowly suffocating – lungs desperately clawing in the air with ragged, laboured breaths.
I took some time to figure out what my greatest life-hits were (men love making lists, you see, even at death’s door). 1. Asking my wife, Deborah, to marry me; 2. Deborah saying “Yes”; 3. The backpacking (and I’d thought it was the killer spiders that would get me); 4. The first time I had sex (to Teenage Dirtbag – classy).
They cranked the oxygen up further. I was given a ventilator mask to help inflate my crippled airways. Naturally, I took a selfie. It might seem like an odd thing to do, but when you’re looking for silver linings, you can do worse than realise that through a squinted eye there’s little between yourself in a breathing mask and a Star Wars Tie-fighter pilot. My bed became my universe.
I felt more and more isolated. The late evenings were toughest – when your family leaves, so does much of your hope. You bid them farewell with an aching heart and tell them everything is OK, when you feel far from it. This is what desolation feels like. And then the long night closes in, with only the Darth Vader-like wheeze of your breathing machine and the whirr of the drug pump for company. I’d put off going to sleep for as long as I could, petrified that I might not wake up. The last little dollop of gleeful joy left me.
But daylight grants new hope. And the NHS is amazing. Energetic physios came bouncing into my room, Tigger-like, up to four times a day to help me clear the gunk choking my lungs. Doctors, dietitians, pharmacists and even a psychologist piled in. Nurses bustled in and out constantly checking vital signs. One nurse became a lifeline: one tender glance from that pretty, funny, redheaded super-trouper would banish that end-of-the-world feeling (briefly). “You’ll be OK, sweet,” she’d say, placing a consoling freckled hand on my arm when I was at my lowest. You can see how patients fall for nurses: then you realise. In this state, who would find you desirable?
Finally, a light at the end of the tunnel. The latest combination of intravenous antibiotics stopped the rot. The fever abated and my lungs stabilised. I didn’t die. I started shuffling to the loo on my own, pulling an oxygen trolley behind me, lungs heaving with the effort. An exercise bike got wheeled in and an exercise rehabilitation lady joined the fray. I began taking short, halting excursions around the ward, oxygen trolley in tow.
Two months and several setbacks later (if I ever catch the pig that gave me that dose of swine flu …), I was finally ready for discharge. But how do you cope coming home to your old life, tethered to a bulky oxygen machine and realising your mantra is dead?
When the doctors first broke the news, near the end of my hospital stay, that I’d be needing oxygen at home 24/7, for ever, I was still holding out for a miraculous, final-straight recovery. When that didn’t happen, and a new reality began to sink in, random questions flooded my thoughts. Why did we ever pick a second-floor flat accessible only by stairs? With just 35% lung capacity left and oxygen tubing flailing everywhere, would sex be a brutal joke? And could I at least now park in the disabled car spaces at supermarkets? Part of me preferred to stay in the safe cocoon of my hospital room.
On discharge, you get a lot of hospital kit to take home but no Life-On-O2 orientation guide. No instructions on how to cope in the dead of night as waves of fear engulf you: you wonder if the flexible plastic prongs stuffed in each nostril will slip out and, deprived of oxygen, you’ll asphyxiate in your sleep. That first night home was the hardest. I didn’t sleep a wink.
Imagine that this chafing plastic “nasal cannula” is itself connected to 30ft of oxygen tubing which snakes along the floor and drapes itself over and around every object in its path.
I needed that how-to manual. And after several close calls, I realised what chapter one might be titled: Navigating Around Your Home On Oxygen Without Breaking Your Bloody Neck.
Step one: ignore potential tubing hazards at your peril. Your house is now an assault course designed by an evil genius. At first, as you pace anxiously around your home, trailing tubing wherever you go, you’ll be oblivious to the dangers of both rogue furniture, and your own body. The first ETA (Errant Tubing Attack), happened when my foot inserted itself into a loop of oxygen tubing lying in wait. Unfortunately, I’m known neither for my poise, nor my quick reactions. And as the rest of my body surged onwards obliviously, my foot did not, causing me to wrench my knee, fall flat on my face and sprain my back. I lay there for a while, and swore a lot. Chapter one of this guide should also have a subsection entitled, Why You’ll Be Swearing A Lot More On Oxygen.
Beware, too, the under-choreographed dash out of the lounge to answer the front door. You see, tubing loves to wrap itself around sofa arms and even door handles; they’re made for each other. The first time I attempted a swift exit from the living-room, I resembled a youthful chocolate labrador violently brought to heel.
So learn to move like a pro. Hitch up that first metre of tubing, and re-orientate it – spare length in hand – before advancing forwards like an intrepid mountaineer, twirling it around obstacles. The number of times I’ve accidentally tripped up my wife with a delinquent length of tubing? Five times. She thinks this will be the death of her.
Prepare for another new fact of life: there will never be enough length to reach all those nooks and crannies. Whole corners of your home will lie abandoned and unloved. Accept it. Embrace the fact that freshly laundered clothes no longer make it all the way to your wardrobe in the far corner alcove of the bedroom. Instead, your boxer shorts and T-shirts will travel only as far as the bed, and will now take up residence there. Follow this and, save the odd repeat garrotting incident and tubing stumble, you may slowly learn to adjust to life on oxygen on the home front. Increase learning curve by double if you’re an uncoordinated idiot like me.
But how will I get to grips with navigating the outside world? The answer is now clear: I’ll need to create an entire manual as I go along. And once I’ve grasped How to Venture Out into the World Without Running Out of Oxygen, I’ll be ready for something special: How to Bomb it Down a Center Parcs Water Slide While Lifting an Oxygen Tank in the Air. Because that’s when I realise, maybe, there’s a way to regrow those dollops of gleeful joy in my heart, after all.
For more information about Cystic Fibrosis visit The CF Trust
Adam tweets at @adamjacques88
