As part of this year’s CF week, I’ve been asked to write a little something about my life with Cystic Fibrosis. I’ve been alive for 38 years (which is a pretty long time when you consider that the average life expectancy of someone with CF is around 40), so I’ll try to summarise as best I can!
I was diagnosed at 6 weeks old, primarily because my brother, who was 9 years older than me, was also Cystic, so my mum knew the signs. Luckily for me I was an extremely healthy kid – in fact there were times when I was young when I wondered if I’d been misdiagnosed! Unfortunately my big brother was not so lucky and lost his fight a few weeks short of his 21st birthday, when I was just 12 years old.
There’s a lot of emphasis on the physical effects of CF (and rightly so) but just as devastating are the psychological ramifications. My brother’s death, along with my own prognosis triggered a lifelong battle with depression, which only became manageable when I trained as a Solution Focused Hypnotherapist last year and learned to take charge of my own life in order to help others do the same.
Physically, as I said, I was pretty lucky. I suffered a lot with the digestive complications of CF as a teenager. It was painful, embarrassing and put a serious crimp in my social life, but it wasn’t life threatening.
I didn’t actually have too much trouble with my chest until I was 28, when I had my first bout of a very nasty bug called Pseudomonas. Luckily by then I had met and married an incredible man who, along with my wonderful mum, has been my greatest support and my greatest cheerleader.
The Pseudomonas saw my lung function drop to under 35% which, as a previously pretty healthy person, was extremely impactful. At 28 years old I had my first ever IV (is that a CF record?) and considered the possibility that this really was the end. Luckily for me I was enrolled on a trial for a form of gene therapy which, although not successful enough to be continued long term, helped me get my lung function back to 58% – not much by ‘civvy’ standards, but pretty life changing for me.
Fast Forward
Anyway, these days I’m doing… well… better than I was at my worst! My mental health is in check, thanks to my career change, and my physical health is the best it’s been in years, thanks to two dogs and a newly found love of pole fitness (if you’d told me when I was 20 that I’d be hanging upside down by one leg at nearly 40 I would have laughed and then slapped you in your obviously lying face). My lung function is in the high 50s, low 60s and I now have a portable catheter in my chest to make the IVs I need two or three times a year easier. I’m not eligible for any of the current CF ‘wonder’ drugs, but have got everything crossed that Trikafta will be approved later this year.
So why am I telling you this? Well, CF sort of falls under the category of ‘invisible illness’. To look at me you wouldn’t know there was anything wrong (especially if my port scar is covered), but living with CF is never easy. When my husband takes the dogs for their early morning walk I set to doing my physio. I have a nebuliser and various physio techniques to help clear my airways, plus a host of pills (ask around and you’ll find that I’m on way less medication than most CFers, but people still get a look of horror on their faces when you say you take 40 plus pills a day!). Physio can take anywhere from 45 minutes to an hour and a half, and is done from one to three times a day depending on how well I am. I’m not going to lie – physio hurts. Remember a time when you had a chest infection and you couldn’t stop coughing. Now imagine forcing yourself to cough over and over again, every day, until you simply can’t do it anymore. Sucks right?
Anyway, as well as the physio and the pills (and inhalers, I forgot the inhalers) I have to make sure I eat well (healthy but lots of it – daren’t lose too much weight) and exercise as much as possible. I try to walk the dogs with hubby in the afternoons but there’s a hill at the back of our house and going even half way up it makes me feels like my lungs are going to explode. In fact, sometimes I feel like that walking up my garden, but that’s the biggest problem with CF…
It’s unpredictable
Sometimes I push through the ‘explodey-lung’ feeling and can walk for an hour or more. Sometimes I can pole for an hour straight. Sometimes I can go out with friends. Sometimes I can dig the garden.
Sometimes I can’t get out of bed.
Obviously the worst thing is having an ‘active’ chest infection (like other cystics I’m ‘colonised’ with several bugs and fungi so technically I always have a chest infection) – physios are at maximum, IVs (either at home or in the hospital) add another 5 or 6 hours onto daily treatment time, and I’m generally feeling very sorry for myself.
However, even when I’m not ‘ill’ I still have days when, for no good reason, I just flop. Maybe I’ve overdone it the day before, maybe the weather is too hot or too cold or too damp, maybe my CF is just throwing a tantrum… it’s hard to tell.
For me the worst thing about these days is that they make me extremely unreliable. I have a close circle of very understanding friends who know not to take it personally when I cancel on them, and I can work from home as I do online sessions with my clients (I tried 9-5 working once: disastrous), but it still feels horrible. I feel like I’m letting people down. I feel like my body (which I take pretty damn good care of) is betraying me.
So here’s my takeaway for anybody reading this article: please don’t assume that because we look ok we are ok. Please don’t assume that because we cancel on you we’re flaky. Please treat us with compassion but please, whatever you do, don’t treat us like ‘invalids’ – we’re tougher than the average human and we never give up. We’re waging a war on CF every single day but that doesn’t make us victims – it makes us warriors.