An Honest Retrospective

It seems crazy that, relative to where things were only a month ago, I'd be able to participate in an event like GearUp4CF in any capacity.  I'm not really great at sharing the ups and even worse at sharing the downs of my life.  As a result, while most knew I'd been facing some challenges with my care over the last 7 years, I'd been downplaying the actual depth and severity.  In truth, there has never been a more challenging period of time in my life. 

Really things began fairly innocuously during a heatwave in July of 2014 when I was feeling a bit run down.  Heat always bothers me so I just attributed a lot of my symptoms to that.  I really just noticed above everything else, I was feeling short of breath.  I went to the CF Clinic just to be safe, X-Ray, get myself poked and prodded, the usual.  I did not expect my Dr to come in and say "Well, you've got a pneumothorax, a collapsed lung.".  Things all sort of unspooled from there.

Now most that know me, know my outlook on my life with CF.  Contrary to what most expect, I've often considered myself a pretty lucky person.  That's right me, that same guy whose lung can't seem to stay inflated and some days struggles to get up the stairs without having to stop at the top for a breather.  My good fortune is no better shown in the reason why I don't have any good friends my age with CF.  The hard cruel fact is that every CF patient I met during my hospital stays as a child is dead.   I'm a statistical outlier for my generation.  I've had the luxury to lead, on the surface at least, a normal life.  Kids, wife, a home and a university degree followed by a stable career.  I'd love to take credit for all this, but this is not a one man show.  From day one and the 42 years that followed, we've waged a war of attrition with my lung function.  Since the day I was diagnosed at 6 months, my parents set out to fight this challenge head on.  They continued on as I became old enough to join the fight.  We've added more and more fighters, my sister, obviously, but also the true friends that I've been lucky enough to find over the years.  Most importantly my wife, who knew what she was buying in for day-in and day-out and still said 'I do'.  Even our two daughters, only ten years old, are already starting to help pull that rope.  Id also be seriously remiss if I didn't also mention the tireless efforts of my medical team over the many years.  They have dedicated themselves with such a personal investment that it can feel like they are almost akin to family as well, fighting this thing with me.  As a child my lung function relative to my size was consistently in the 100th percentile and sometimes even more.  Where most kids with CF spend weeks and months in hospital multiple times a year, I made it through high school having only 3 brief routine two week stints in hospital for a total of 6 weeks in the first 17 years of my life.  It all adds up though, Cystic Fibrosis is unrelenting and the cracks were starting to show.  Not so much that it prevented me from making my way through university, but it did begin to affect my daily life more and more.  More time on treatments, more coughing and hacking my way out of the classroom so I wouldn't be interrupting and most importantly the slow decline of that 100th percentile lung function.  By the time I graduated university it was down to the low 80's.  As life went on, so did the decline.  Despite increasing treatments and better reactionary medications they were clearly just not enough.  After another ten years as a 35 year old, my lung function was now down to the 60's as we headed into that summer of 2014 and all that followed.

Back to that meeting with my Doc, if I'm being honest, I don't really remember a whole lot more after that.  Hard as it may seem to believe, that was one of the rare times with regards to my health that I'd been thrown a real curve ball.  I knew IVs and physio and cramming calories, but collapsed lung, that's something new.  It knocked things off balance and in a lot of ways we've been struggling to regain that balance for any length of time ever since.  Zooming out from that moment and looking forward, it turned out to be the first of a total of five collapsed lungs, some smaller, others much more significant.  The first 4 had all affected the right lung occuring over a period of 8 months.  To prevent this from continuing to happen we performed surgery, removing a small section of dead tissue that was causing these.  The recovery from this surgery had it's own early complications, but that didn't prevent it from being an overall win.  Less than a year later we had a repeat of that situation, but this time it was the left lung that collapsed.  Based on what happened with the right lung, we opted to go straight to the same surgery we had done the year before, this time on the left lung. 

At this time, things stabilized a bit, but the next battle really began in the fall of 2018 and really didn't let up until just a few weeks ago.  That fall the cultures from my lungs showed I had a serious bacterial infection, a Non-Tuberculous Mycobacterium named Mycobacterium Abscessus.  This meant stopping everything and diving head first into a treatment regiment that was more intense than any I'd done before.  Historically my typical tune-up treatment routine consisted of two IV Medications over the course of two to three weeks.  The M. Abscessus treatment routine necessitated coordinating three different IV Medications at the same time as 4 different oral antibiotics for six months.  The good news was that my body responded really well to treatment and it looked from early on that we'd be able to be done within the expected time frame.  However, like I said earlier, as soon as one thing started to clear up another thing would crop up and not everything I had to face was CF related.  Later that year, just after finishing the M Abscessus treatment, a momentary lapse of attention and a slip on the stairs led to broken ribs.  Just as that was mending, a few months later, I managed to break three ribs on the other side just by coughing. The impact of these events coming one after another without time to recover became more and more evident.

Jump to now and despite all the efforts of everyone supporting me, the fact is less than a month ago I was admitted to hospital in perhaps the worst condition I've ever been in.  That war of attrition was slowly getting grimmer and grimmer.  My lung function, even despite the previous years leading up to now, had still rarely dipped below 40% efficiency.  Last month though it had dropped to the point where I was at an all time low.  My lungs on the 11th of May were now working at 26%.  I could barely carry in a bag of groceries from the car without needing to stop to catch my breath.  Have a conversation while walking, forget about it. I've always been a very pragmatic person when it comes to my health, allowing myself to dwell in sadness and frustration all seemed like a waste of energy that could be put to better use in a time of challenge. Being 100% honest, this admission was different, for the first and only time I felt something I hadn't really felt before going into hospital.  I felt scared.  Scared that despite everyone's best efforts we were slowly losing this battle and running out of options.  Luckily this was short lived when everything changed on the 18th of May.  For months my medical team had been pleading with a company for a medication that had been released in the US in October of 2019, but had yet to be approved for use in Canada, except for on passionate grounds.  On the 18th of May we got the answer we'd been trying so hard to get.  Compassionate Access Approved.  This was the reprieve we'd been hoping for, we knew how life changing Trikafta had been to the CF patient community all around the world.

Within two days of starting Trikafta, I was already out of the hospital.  Fast forward four weeks and it's nothing short of miraculous.  It's a gift that I'm already at a point where I can debate what sport I want to play with my kids this weekend, as opposed to debating if I have the energy to climb back up the stairs if I go downstairs to watch a movie with them.  I hiked 6 kilometers today, up and down terrain without needing to stop to catch my breath.  While appreciating the impact this has had on my life, it needs to be clear, a gift like this is directly tied to both the exhaustive fundraising efforts and the generous donations from people, friends and family alike, to the CF Foundation.  

It's donations like these that funded the science of the astonishing medication that, like flipping a switch, started to reverse my state almost instantaneously.  A change giving me a second lease on life to an extent that I wouldn't have believed myself if I were not living through it.  The continued support allows for more and more radical scientific breakthroughs until one day we will be able to honestly say 'Cure Found'.  When I was born, CF was still considered a death sentence.  I told you before, I was one of the lucky few.   The few that managed to battle through, propped up in no small part by the scientific advances brought about from the tireless fundraising of donors and volunteers.  There is every reason to believe that the money earned through events like Gear Up 4 CF, will helpbfund the next major breakthrough in Cystic Fibrosis.

Again, thanks to my physicians, my family and thanks to the latest cutting edge advances in CF pharmaceutical treatments, there's true hope now that we are through the worst for a while.  The feeling is I've emerged and that I remain defiantly unbroken, perhaps a little bent, but not in any way that is going to prevent me from getting the opportunity to enjoy playing around with my crazy, nutbar children.  Most importantly that imminent fear has disappeared.