Transplant was a word I never wanted to hear. I was fearful, having been born in the early 80s, when being born with Cystic Fibrosis meant an unpredictable, often rocky future. Defense mechanisms felt necessary. CF used to be labeled a "fatal" disease, a "childhood" illness, and lung transplants, which only became a risky reality in the late 80s, were connected to words like "end-stage" and "last resort." Just the word transplant could make me burst into tears. The tone doctors used when talking about it was also a tip off -- they were always very measured and careful, a subconscious signal in hospitals for "this is scary and sad, even for us.” 

It never occurred to me that transplant could be anything other than scary and sad.  

I believed in positivity in the purest sense, in that I rejected any insinuation that I could ever be sicker than I was.  I worked hard at staying healthy, and educated myself constantly about ways to improve.  My mother instilled the importance of great nutrition in me, and I believed, and still do believe, that sometimes you can summon unexpected strength from within, and get better. I always smiled brightly and said I felt great at doctor's appointments, and took a sort of pitiful pride in answering emphatic "NO's" regarding the working order of the rest of my body, the parts that were healthy.  “Any vision problems? Ringing in the ears? Fatigue?" “Nope nope nope!  Everything's great." I would march out of the hospital, if all was well, with a fire lit under me, furiously prideful in that way that happens when deep down, you know that so much is out of your control. 

I am 32 now, but it’s been nearly ten years since my doctor first brought up transplant evaluation.  At the time, I was managing. I enjoyed my life and could not connect at all to the idea of removing my lungs and replacing them with somebody else’s.  I ended up putting the process on hold – a decision that my doctor and I agreed upon, as I really was too healthy to be listed.  He told me though, that when I did reach the point where my quality of life was no longer satisfactory to me and me only, that that would be the time to think about transplant. It was important to remember, he said, that this had nothing to do with how my health related to anyone else’s.  I filed this away, and revisited it throughout my twenties.

For nearly 8 years I operated on a lung function of about 45%.  But during that time, I would think about what he said and think, "I am still OK with my quality of life."  I was terrified to lose that life, terrified to think of how losing it would play out.

To give a sense of what 45% of lung function looked like, for me, I began using oxygen at night at 22, and by the time I was 25, could not exercise effectively without it.  I couldn't walk up hills or flights of stairs without getting short of breath, and I planned all of my outings around avoiding long walks.  I drove everywhere, and when out with friends, would lobby to take a cab for just a couple of blocks.  I worked part-time.  None of my limitations were obvious, and unless you did something like climb stairs or walk a few blocks with me, which I would probably avoid, you would never know I was sick.  I was hospitalized, but I would bounce back to my baseline, or slightly below. I adapted to my limitations, and kept living. When I felt worse, or worried, I would think quickly about what my doctor said and think "I am still OK with my quality of life."  I could manage, I was living, and if I forced myself, I could do almost anything anyone else could.

I had a very very slow decline. The last time that I think I felt close to what ‘normal’ people feel was my freshman year in college.  I could go running with others, and could climb the nine flights to my dorm room if the elevator was out.  This is unfathomable to me now. 

I was finally evaluated for transplant in 2010, but the agreed-upon goal was to continue maintaining, to keep transplant at bay for as long as possible. I didn’t need constant oxygen; I was too well to receive organs. But for the next few years, I felt like I was in limbo because I was in limbo. I was waiting to get sicker, yet I already felt terrible. With transplant, you cannot get listed if you are too healthy, or too sick.  Even if you could get listed, your score would most likely never be high enough to get you an organ.  Since you also can't be too sick – this is a very real and important point-- it is vital to stay healthy. If you operate under the assumption that you'll just wait until you decline, or something really bad happens, and then let transplant swoop in, you are taking a big risk by being so sick, potentially not getting an organ in time, or not recovering well.

Around summer of 2013, less than a year before I would be listed, I was particularly weary.  I was tired of straining to maintain.  I had a conversation with my doctor about it, just to confirm that what I knew to be true was true – I would have to wait, potentially years more, in this gray area, because I was too healthy for transplant, yet too sick to feel like I was fully living. I remember the thought creeping up on me, that I was no longer OK with my quality of life anymore, but I knew this was the situation for many people with CF.  There comes a point when you don’t feel well, all the time, and your thoughts are the only place your power lies. 

If you’re like me, hyper-vigilant about staying on top of your health, you try as hard as you possibly can to stay healthy, so what you end up experiencing is a tedious, incremental decline that can last years, all the while knowing, anticipating, that transplant lies at the end.  Transplant – that doomed scenario at the end of an unknown series of events. 

My health plummeted in late 2013 due to two viruses, caught despite my vigilance, and by the holidays, I was very ill and using oxygen 24/7. In early 2014, I was actively listed for transplant.

Once I was listed it was like a relief. A few years ago, I remember thinking that if I was ever on oxygen full-time, I would be too mortified to leave the house. This makes me laugh now. Although part of me is grateful for my ability to blend in all those years, (we with CF are graced with the ability to look great even if we feel miserable), it wasn't until recently that I realized how exhausted I'd been.  That weariness I felt in the summer of 2013 all came flooding in once I was on oxygen 24/7, and listed. I felt in a way like finally things matched up, everyone knew that I was as sick as I felt.  After years of my mother suggesting it, I finally got a handicap placard. I understand why I never wanted one, though, and I am grateful for that fiery side of me.  Even though I may laugh now, that kind of attitude keeps a person going, I still rely on it.  I still believe in forcing a smile and making the effort, maybe not all the time, but when you can, and sometimes when you don’t want to.

I've been listed for 15 months now, and its been difficult in a foggy "what happened to my life" kind of way. Yet remarkably, there has also been a jarring realization, one that came out of getting to know a few wonderful people who also have CF, and have been through transplant.  They are all in different stages of their lives “post-transplant;” one man received his just last December, and one woman will soon celebrate 8 years.  “Meeting” them has been a comfort, a joy, and unexpectedly eye-opening.  They are all very different people, but the common denominator among them is they are incredibly happy, active, and grateful, and they are all living truly wonderful lives: taking up new sports, traveling across the world, going back to school, having children!  I consider the question about quality of life that led me here, and I realize, with a bit of amazement that I had never considered this—that their quality of life is tenfold what mine has ever been during any time in my adult life.  There is potential for me to live a life that was better than before, and for feeling better than I’ve ever felt. 

It’s a risky surgery, it’s a hard recovery, and it’s a complicated concept.  Someone has to lose a life for you to get a chance.  As the old adage goes, there are no guarantees for anyone in life, and there is no guarantee that it will happen, that I will get to live an incredible life like I see my friends living.  But without transplant, that life is unquestionably never going to happen.  With that logic, I can’t help but feel that talking about transplant should be easier, and happier, because you are really talking about a chance for the most incredible miracle of science that we have access to.  It’s not a failure, it’s an opportunity, and a gift, unlike any one I’ve been offered before.

I never quite saw far enough beyond the fear, in all those years of anticipation, to feel this way.  And I wish I had.  Maybe I would’ve been less worried, less uptight, and as a result, happier and healthier.  I thought being happy and living with CF meant always fighting, and not embracing the darkness and fear.  But if we don’t embrace them, then we become them.  I failed to see that what I was afraid of wasn’t really a darkness at all.  Living with CF is a constant learning experience, and right now that is in overdrive; it serves me well to embrace that opportunity to learn.  I see how afraid people just being listed feel, how stricken they are to have found themselves here.  I feel grateful for my friends who have been through it, but I know not everyone has friends like that. To the credit of my wonderful doctors, they introduced me to two of my transplant friends, and I am thankful for that.

As a whole though, I can’t help but feel that the CF community avoids talking about transplant because its hard to do it without feeling like somehow, someone failed the patient—the research failed, all the fundraising efforts failed. Or the patient failed. But nothing failed. CF is a complicated disease.  Antibiotics, inhaled treatments, chest PT, good nutrition – all the products of research, money, and care – contributed to me putting off transplant for this long.  We have come so far in so little time.  Again, CF is a complicated disease; there is nothing but success here.  It is just time for this next part, and I feel so grateful that it even exists.  Many diseases have no threads of hope.  Maybe others out there will benefit from hearing this; maybe it will affect, in some small way, how we talk about transplant. 

 

       INSPIRED BY THIS ARTICLE?      

 

Caitlin O'Hara is a 32 year old living with Cystic Fibrosis.  She is from Boston, but has temporarily relocated to Pittsburgh while she waits for a lung transplant at University of Pittsburgh Medical Center (UPMC).  She loves (and sometimes misses right now):  Travel, great conversation with family and friends, time in Maine with her boyfriend, New England seafood, and the ocean.  After transplant, she is thinking about pursuing a master's degree.