My Journey with IPF, so far
I want to give you some context to show where I started with this disease and roughly how I arrived at where I am today. This is an insight into the milestones I’ve faced to date.
It was late 2011 when I noticed I was coughing more than normal. I was cycling to work and back, reasonably fit on an excellent diet with the perfect body mass index; what could possibly go wrong?
I started going to my GP with suspected chest infections and was given a variety of antibiotics over the next few months. Nothing changed and an equally inconclusive chest x-ray was followed by a CT scan, then in May 2012 I was introduced to the three letters that would come to occupy my every waking thought; IPF.
Once I got used to the idea, and it took a while, I realised I was potentially quite fortunate in that my diagnosis was made before I had experienced breathlessness. For the majority it seems that isn’t the case. I was pretty much symptom free for the next year or so, although a shortness of breath was creeping in quietly.
Shortly after diagnosis I was sent for my first ‘Pulmonary Function Test’. I passed! I was like Popeye on spinach, belting that fairground thingy with a sledgehammer, you know; the one where the bell rings if you hit it hard enough?
“Why are you here?” asked the slightly puzzled looking technician. I told him and I remember his face changing very slightly on hearing those letters IPF, but he said nothing. I now know he faced one of those ‘Who dare tell the lambs in Spring what fate the later seasons bring?’ moments. He passed his test too.
And so it began; the periodic trips to lay bare my slowly fading ability to perform what was once so effortless; puffing and panting long and hard into a series of translucent tubes. Initially these tests were something of a novelty but I began to realise they would prove to be the barometer that signalled the impending and irreversible path of my own internal climate change.
I remember the excitement of being told I qualified for Perfenidone, a recent front runner in the fight to restrict the progress of the disease. This was mid 2013 and my condition had taken a dip, resulting in a noticeable loss of stamina. I proudly returned home with my introductory pack, only to find I was intolerant to them. After nearly a year of trying I made the rather easy decision to dump them and choose quality of life over length.
There had been talk of a lung transplant, but it wasn’t until April 2015 when I was sent for an assessment. Until something is a realistic possibility, it commands little of my brain time and I knew that statistically this was an extreme long-shot. Those four days at the Freeman in Newcastle were simply an astounding insight into a world of professional expertise at a level I would have struggled to even imagine. To be told at the end of it that I was a contender was a hugely emotional moment for my wife Jackie and I.
Being a contender however and being on the ‘active’ list transpire to be two quite different things and it wasn’t until September 2015 that I got the promotion. After four months of being ‘active’ it then dawns on you that there is a ‘priority’ active list that I still needed to aspire to. I can proudly announce that I now qualify, and as such sit here with fingers crossed, feeling nervous and grateful in equal measure.
Any excitement generated by the transplant news was more than reclaimed by a telephone call out of the blue one day, from our local hospice; enquiring if they could come and meet me to see how they might be able to assist. The eventual meeting was a well intentioned and skilfully conducted if cold affair, made all the more unwelcome as I was feeling and looking extremely well at the time. In fairness I later remembered that I had ticked a box requesting the intervention; I had presumed it would have been more than a little later.
I guess it was around August 2014 when I realised things were starting to slip, I’d been battling with Perfenidone and by October I was glad of my ambulatory oxygen. At the time 2 litres per minute was pulsed at me every time I took a breath. That was easily enough to allow me to ramp up my walking pace to a respectable stride. Currently I have to blast 10 litres a minute on a constant supply into my mouth to attempt the stairs at home, The equipment available only has one more notch and I’m on maximum, sadly there’s nothing else available.
It was October 2015 when I first ventured out in a wheelchair; the decision was made due to the uncertainty of how far away a car park might be from a concert venue. It wasn’t really necessary as it transpired but it was an enjoyable first outing as I was among fit friends and there was beer involved. Wishbone Ash were excellent and I have only needed the chair three times since but it stays in the car just in case.
Exercise is important and a recent policy change means that anyone with IPF is entitled access to a program of Pulmonary Rehabilitation. If you are unfamiliar with this, it’s a gentle exercise class for those with chronic lung conditions, it’s deserving of a blog entry on it’s own because it’s a great thing medically as well being hugely, if unintentionally entertaining. Being on the active transplant list entitles me to be enlisted permanently so I go twice a week every week, which is a very good thing, but there’s a sitcom in there believe me and I’ll attempt to convince you at a later date.
So, as it stands I’ve been on Nintedanib for approaching a year and apart the fighting weight loss and the occasional bowel related firework display (a common side effect!) I’m fine, just extremely short of breath and occasionally massively fatigued. My concern now more than ever is avoiding chest infections and colds. I’ve done well up to now and see my persistent cough as an ally in helping clear my lungs but I have to be honest and say that I’m approaching the sharp end of things. I’m pretty good at being able to manage my state of mind and no longer fear dying or depression, In Jackie I have the best wife on the planet and a loving family. I have a mountain of things to be grateful for.
Before long the cylinders will be unable to make any impact as the fabric of my lungs continue it’s inexorable transformation from ‘life giving’, to ‘life taking’. My inability to exert myself will multiply the risks I face as my internal organs compete for the ever decreasing supply of oxygen, ironically one of the most plentiful gases in the world.
The fact that something is plentiful is of little comfort if it can’t get to where it is needed most. I was reminded of this through tearful eyes watching footage of a baby dying of malnutrition on Sport Relief the other day; it’s a tragedy that never fails to get to me. The helplessness as loved ones look on, the sense of inevitability in the eyes of the clinicians, the knowledge that whispers to us that this will just repeat, time after time until the will and the funding are there to prevent it.
I guess this is the first year that I can say with some sincerity that I’m beginning to know how it feels.
More from Tony
Read Tony’s first blog post “I’d rather have cancer”.