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.

A Normal Kind of Life

So it's obviously been an age and a half since I've posted anything in here, but given that lately I seem to have some time on my hands I felt the need to write something.  I'm involved in various CF community message boards and lately I've heard a lot about how CF Clinics will advise people to try to live a "normal" life when dealing with CF.  I just felt the need to say how this may be the single-most important piece of advice these families ever hear.


Following that last statement many may be looking at me with a bit of a “what the” facial expression, while others may have already closed the viewing pane disregarding anything I have to say.  Hopefully, not too many of you have left and for the few that have remained, allow me to explain.  CF can be a brutal and time consuming disease, this is no secret, but you only have to let it be life consuming for you if YOU allow it to.  I was diagnosed in 1980 in a time where CF treatment and prognosis was much bleaker, where treatments weren't nearly as effective and were a little sniffle stood to present an even bigger danger.  That being said, when I was diagnosed this same advice of living a normal life was proffered on my parents and they took it to heart. As I grew up, I went to school with all the other kids my age.  I played sports like hockey, baseball, soccer and cross country. I even went away on weekend trips on my own by the age of ten.  My parents didn't have me do these things because it was easy or because they didn’t recognize the risks.  Anyone who knows my parents know this is the furthest thing from the truth, heck my mom was a CF dietician for nearly a decade who saw daily the ugly side of this disease in the kids she treated.  Matter of fact, as a parent myself, I can only imagine how hard it must have been to just let go and accept me doing all these things as opposed to protecting me from every little bug.  They instead made the choice to simply deal with the result of allowing me to do these things the best they could.  If I got sick, so be it, they would be strong and help me fight it as best they could.  It is because of this parenting choice that I am a father, a husband, a college graduate, a person with a career and lastly and least importantly, a person with CF.

The other day, a young woman was minding her business walking along the sidewalk when a car lost control and killed her.  This is without question a tragedy, but perfectly exemplifies my point.  You can spend every waking moment fighting every bogeyman you know of letting it dominate your life ... and even succeed at knocking them all back, only to have something unexpected come out of left field and tap you on the shoulder telling you you're done.

Right now I'm in hospital after having surgery on my right lung to have a Cyst removed.  This after having my lung collapse four times in the past 7 months.  While this situation may have been exacerbated by my CF, it is something that could have happened to anyone and is completely unrelated to my CF.  In fact a good friend of mine went through the same thing in her twenties.  There is no amount of treatments or further precautions I could have taken to prevent this.  My point is, still do all your treatments, don't ignore warning signs and don't do stupid things intentionally like drink the tap water in Mexico, but at the same time, live.

Perhaps the best way the CF Clinic could pose it is instead of living a normal life, live as normal a life as possible.  I know it’s not easy advice, and you don’t have to take it, but please, just try.  You or your child with CF will never regret it.

Self-image: The importance to take action where we can and to accept when we can't

I know this post is long winded but I hope those reading will manage to stick around to the end.
It's funny how people can be when it comes to looking at themselves and what they see.  It seems that people have the ability when looking at themselves in the mirror to focus in and fixate on the one thing or things about themselves that drives them crazy.  For some people its a specific feature, maybe their nose, or how their eyes are set or the way their ears stick out too much.  For others it can be the color tone of their skin, be it too fair or too dark, or if they have a lot of freckles.  One that seems to be the most common is how people view themselves with regards to their weight, it seems almost everyone wishes they either weighed a little more or a little less.  When it comes to self-image we seem to be able to stick the knife in, but also twist it better and more cruelly than any bully or mean-spirited person could ever accomplish.
So where is this coming from?  Well the other day I was finally getting around to putting together some photo albums of the girls first year.  As I was doing this I came across a picture of my wife and her sister up in Penticton pushing their strollers along the lake shore.  Now, not being in this picture didn't seem strange to me because there have been lots of times my wife has gone up to visit her family and I've stayed behind to do some extra meds and tune myself up.  What did seem strange was that I didn't recognize the third person walking with them.  It took some time until I suddenly realized that this stranger walking with my wife and my sister-in-law was in fact me.  I was dumbstruck, I couldn't believe how terrible I looked.  Now I know what lots of people are thinking, similar to a mirror, there are a lot of pictures that are taken of us that we look and go "No, I can't look like that" or "There's no way that shirt looks that bad" etc, etc, etc.  But that wasn't the case here, I honestly did not recognize my own self in this picture.  Now in fairness this picture was taken in month two of new born twins, I was exhausted and if I recall correctly began IV meds not too long after.  However, the cold hard truth is that the person in this picture did not look like a well person.  In fact, not trying to be too harsh, he looked like a skeleton draped in skin and little else.
In general, I'm someone who is probably more comfortable with my self image than most.  This probably stems from the same pragmatism that has been so invaluable when dealing with my CF. My appearance is what it is, better to accept it then to beat the hell out of myself.  I've got enough beating me up as it is :).  I can only imagine how hard it must be for the parents of children with CF as they watch their children deal with this condition, because the honest truth is that it is rarely a condition that is flattering to ones appearance.  I remember having a conversation a number of years ago with my friend Eva.  We were discussing who it's harder on when it comes to appearance and the impacts of CF, men or women.  I felt it had to be women as, in my opinion as unfair as it may be, it seems women are more often judged for their appearance then men.  Eva disagreed as she felt at least women have the ability to cover up their appearance with make-up and accessories.  It was just the typical sort of conversation we'd have as we always seemed to feel that there was some other person who had it tougher regardless of how bad our own situation was.  I only wish more kids with CF would be blessed with this type of outlook on life.
I guess the point of today's blog is a call to everyone to realize that you are not the only one dealing with self-image issues.  As the saying goes, to a man, or woman, every person on this planet has something they don't necessarily like about their appearance.  It's on us as individuals and realizing what of these are things we can actually do something about.  After that it's equally important to do one of two things, if there is nothing you can do about it, do your best to accept and not beat yourself up about it.  Or two, get off your ass and take action.  It's especially important for us as parents to do this so that we can try our best to instill this outlook on our children.  In March, I looked at the mirror and was embarrassed at the gaunt face looking back at me, I tipped the scales (or perhaps more accurately, maybe they tipped me) at 128 lbs.  Now 3 months later, I've put on over 25lbs and have been working out consistently with both cardio and weights.  I know that I'm not done, there's still work to be done, but I'm proud not only of what I've accomplished, but how I look too.  Thanks for taking the time to read this.

NIght guy fun and Morning Guys problems

I guess it's time for a little update since I haven't posted in like 3 weeks.  Damn time flies.  Now for those who have been reading this blog before they know that I've said in the past when I don't post it's usually that I'm avoiding it because I don't want to have to own up to the fact that maybe I haven't been giving it my all.  Well, not this time.  The truth is, for the last three weeks, and more specifically I've been busting my hump trying to all all the marks, parenting - exercising - eating - sleep - work, and so far so good.  We hit up the Zoo and promptly bought ourselves seasons passes as the girls absolutely loved it.  We also got to have some good family fun with easter egg hunts and trips to Costco with Grandma and Grandpa.

I've also been back at work now for two weeks trying to work hard to clean up the mess I left for everybody when I had to disappear for four weeks.  I've been hitting the gym three days a week and also cranking out some solid cardio four times a week.  The diet has kept up, I'm sitting at around 146lbs now and am only four pounds from my minimum weight threshold I like to be at and 14lbs from my max goal.  The truth is the last time I hit 160 is almost a decade ago and while it may not be realistic, it's a target to keep looking towards.  

To be honest, the part that has been the hardest in all this is the sleep part, not that I struggle with falling asleep, I just really really suck at getting my butt into bed.  There's a Seinfeld bit where he describes it perfectly, I'm night guy, night guy never wants to go to bed.  Now getting up after five hours sleep, Oh, that's morning guys problem, not my problem, I'm night guy.  So I've been trying to make sure I start my night shake no later than quarter to ten and my light is out no later than eleven.  It really shouldn't be so hard, but it's something I really struggle with and know that if I'm gonna have the energy to do the other things above, I'm going to need the proper rest.

There is a reason for this push to see if I can handle this work load.  After my last clinic I was somewhat underwhelmed with my latest PFT results and looking at my trend over the last 5 and ten years there is a clear and overall decline.  Not too serious yet, but something that needs to be watched.  THe subject of reducing my hours at work was lightly raised by my CF doc.  Now, at some point the reality has always been out there that I will have to scale back my workload, maybe work a few less hours per week, but in my mind right now, I'm not at that point yet. So, hence the push to see if I hit all the right marks when it comes to my health AND still get to have that crucial quality time with my fam, can I also handle a full time work load. In my mind I have little doubt that I can.  Until next time.

Flipping the Switch

So the other night I had my parents over for dinner and we were discussing how things were going in my latest attempt at getting back into good shape.  It led to me using the analogy of a light switch.  The truth is, so far in my life I've been really fortunate.  Up until now anytime I've let things slide, perhaps lapsed a bit on treatments, or the amount of exercising and eating I should be doing, I've always been able to right the ship simply by reestablishing good habits and a tune-up run of IV antibiotics.  Now equating it to flipping a switch might seem like I'm trivializing the effort it takes to get back in shape, or make it sound easy.  Doing everything we should for our health is never easy, that holds true for anybody, but especially for those of us with CF. The good news, is that this time, my switch still worked.  I've been managing Cardio 4-5 times a week going for 2-3 miles at a time at a really good clip.  I've also put on about a dozen pounds in the last three weeks which means I'm almost halfway to my goal weight.  I'm not writing this to brag or for some pat on the back where's my cookie type of moment.  I simply want to illustrate the two pretty important lessons I need to take from this, one positive and one a bit more ominous.  The positive, is that my switch still works, the more ominous is how many more times do I have before my switch looks like this ...

These are lessons that anyone with CF can do well to remember because the fact is, just because we bounced back last time, doesn't necessarily mean it'll happen next time too.  If I'm on the IV antibiotics too frequently the bacteria I'm culturing could eventually develop a resistance.  This means that anything I can do to extend the time between IV tune-ups becomes more and more important the older I get.  For now though, I think I'll bask in the happies, tomorrow my three ladies come home after a two week visit with Grandma and Grandpa.  I should be able to return to work within the next week or so and now that the train is back on the rails, I'm gonna do my best to keep it there for as long as I can.

Don't Call it a Comeback ... (And my thoughts on Change)

Has it been that long since I last did this ... whats that you see more than half a year?  Pfft there's not really much difference between a few weeks and over 7 months is there?  First things first, thanks to everyone who encouraged me to get back to writing these, it meant a lot to know there were people out there that wanted to see how things were going.

Anywho, I don't have any really epic stories of supreme highs or devastating lows.  Mostly things have been even keel for the most part, though that keel has been slightly listing to port and taking on water bit-by-bit since my last IV run in July.  As a result both the lung function and the weight have plunged back into the not so good and that means its time to get back onto the magic juice that is Miropenum and Tobramycin.  On the good side it means in less then a few weeks my energy level will be back up, I won't be out of breath just getting up to go the bathroom in the middle of the night and I won't look quite so much like this guy...

I exagerate and use hyperbole for effect but, when the truth of the situation hits a little too close to the exageration that's when it's time to get off my butt, hunker down and get back to work.  That means forcing in the calories (I know, I know, woe is me I have to eat fatty foods most people would die for), back to the gym (3 times this week and counting) and time to ramp up the cardio again.

What got me back to writing was reading a friends blog about some of the recent changes coming in the states for CFF events.  Now the truth is, these changes have been in place at most hospitals and CF events here in Canada for a while.  The basic gist seemed to be the following (forgive me if there's anything wrong here), at events only one CFer present at an indoor event at any one time, nobody with a history of Cepacia, minimum 6 feet distance should be maintained between any two CFer's at out door events.

Now all this combined with me being back on the juice got me thinking about something.  I have a confession to make and it is going to come as a shock to those who know me fairly well, I ... wait for it ... am not a huge an of change.  I know, minds blown, right?  I wouldn't be too surprised to find out most Fibro's and Cyster's as well as any parents that have a big hand in their day-to-day care are not overly keen on change, especially when it has anything to do with their care.  Our initial reaction is one of instant push back, no way, not doing it I want my old way back.  But in truth, rarely are these changes earth shattering, nor do the affect us that much.  My own recent change came when I started this recent run on the IV.  I learned that my days of carrying around the man purse are no more.  The reasons were legitimate, but even still my initial reaction was not one of instant and gracious acceptance.  What that means is that every one of my doses has to be administered via the IV pole, meaning that 4 times a day I'm not going anywhere for the next hour.  It also means that I can't just sleep and let the machine take care of things.  I have to set an alarm, get up go downstairs do my dose and then head back up to bed.  Luckily, I don't usually experience much difficulty with interrupted sleep.  Is there more hassle, a bit, but there are good things about it too.  It means helps me to keep to a regular eating schedule.  Since I rarely am hit by that whole feeling of hunger as a sign of it being time to eat, whenever I am dosing, I make sure I take that as a cue that it's time to have a meal.

Now as an honorable send off to the great man purse that was my CAD Prism pump, my top three stories that it provided me with.

1) During the Vancouver Olympics it allowed me to enter GM Place ahead of all the lines and people waiting outside in the cold.  It also allowed Carrie and I chance to be the only ones witnessing Jaromir Jagr's pre-game ritual of running stairs about five feet away from us.

2) Going into a movie on opening night and having the usher stop me because he thought I might be smuggling in a camcorder or something.  Almost felt bad when I saw his face blanch as he turned white as sheet looking in at the tubes and wires and what not.

3) The classic first time I did home IV and saw a small amount of air in my tubing.  I looked in the manual and all it said was call 911.  I made the call and proceeded to sit on the front stoop.  Fairly classic moment when within a few minutes I was looking at 2 fire trucks, 2 ambulances and whole bunch of confused EMS workers when I said I was the one they were here for.

Well, there we go.  Back to it.  If you read this far thanks for sticking with it through my ramblings and until next time, which I promise will be less than 7 months from now.

Back from silence

Well it's been longer than I meant to let it get between posts, but I figure I've been putting it off long enough.  A couple of posts ago I talked about how when I disappear from posting its usually a sign I'm slacking off and afraid to face my own music ... but that's really not the case.  At least mostly not.  The fact is the last three weeks have been a fairly action-packed time.  They included mom going away from the girls from the first time for four days and leaving dad in charge.  They also included dad successfully having two happy healthy baby girls there to see her when she got back (along with a little help from the vital cogs of Grandma and Grandpa).  It also included me coming down with a pretty nasty head cold and lovely run of my favorite Cipro to knock it back down before it settled too badly into my lungs.  I've been keeping up with most of my exercise at least trying to do a pretty good clip walking the dog (though I know I should be trying to do more).  Appetite has gone a bit off a cliff but that's usually a fairly common side effect for me when I'm on Cipro, hopefully know that that's done the appetite will start to pick back up.  I've also learned that I'm a bit of a marshmallow when it comes to heat.  After giving up on summer to finally arrive at home we traveled to visit my wife's parents.  Now for me, growing up in a Sea side climate, it always takes a bit of getting used to desert climate.  Its even more difficult when the temperatures hit the mid 30's and this delicate jam tart is forced to retreat to the basement.  I love the sun, I love the ability to take the dog for a dip in the lake.  The part I care for less is the feeling I've got a band strapped around my chest.  On top of that, the girls are little unused to the warm nights as well which is forcing mommy into some rough night's sleeps.  But all in all its been really nice to get on the road here and its great watching the girls enjoy themselves in a big back yard as they continually bring Fiona piles and piles of sticks and balls.  Hopefully I won;t be so long between posts here and while I may not give my self an A over this past three weeks for doing everything I need to, I do give myself at least a low to mid B.