Earlier this year, I was lucky enough to find myself in Italy after my first hospital admission of 2016. A little part of me even feels sad that I say first in full confidence that I knew there would be more than one because every year since I was 9 has followed the same general pattern. The opportunity arose and like a bee to honey, I went straight for it.
Cystic fibrosis or ‘CF’ for short, is many medical terms. But more than anything, it’s getting on that plane and going on that trip if you think you can, with or against medical advice. It’s focusing on NOW, putting all your eggs in one basket “just in case” and hoping either way that you have enough days to chase them one by one. CF is growing up before your time or never growing up at all. You know this from early childhood and right through adolescence but it doesn’t resonate until that first gruelling hospital stay. I don’t mean the one your parents remember when you were four, I mean the one when you were 13 and wondered how you would respond to your friends curious questions after you returned from 2 weeks of having not been in school. I recall one of my granddad’s great sayings (he had many) “If you don’t have what you like, get to like what you have!”. I really did want that motto to work for me and so I tried that for a while. I tried to like missing winter tests and awkward biology experiments where the rest of the class would partner up and I would look around wishing the day would be over and the ground would swallow me. I let on that I was fine with my exemption from P.E because I was fragile, but of course I was not. I didn’t like sitting on the balcony upstairs doing homework whilst everyone my age was fit and having fun. I was a teenager with all the paranoid and self conscious thoughts of a regular teenager except I was living a very irregular teenage life. Orkambi now could mean kids don’t have to go through this anymore. It could allow them their teenage years, their days of mischief! The ones I was robbed of and can’t get back.
I was sick all through my school years and attained a Leaving Cert I wasn’t proud of, twice. Not because I wasn’t academically capable, but because cystic fibrosis held be back. My liver was is in it’s final stages of failure and I didn’t even know it. I wore my brother’s shoes to school to do my exams the second time because there was so much fluid in my ankles I couldn’t fit into my own and consequently ended up on a trolly in A&E that night. Thankfully it was the last exam and I didn’t have to worry about further missing any but I wasn’t delighted with my results. I contemplated going back a third time out of disappointment to myself and desperation to show my peers that I was every bit as clever as them, and prove it. But I couldn’t face the same teachers, same halls, same old classrooms and I only had a weak idea of what I might be interested studying at third level. I waited 4 years feeling useless and humiliated by comments about my work & education life, and I still get those comments! I know now that I couldn’t have decided what I wanted back then. It wasn’t Biology or Home-Economics that influenced my interests in nutrition or the field of medicine and pharmaceuticals, it took me my whole life experience up ’til now and if I hadn’t been through the wars to some degree, I would probably have a different way of looking at things and a lesser thirst to know more of what goes on behind the scenes of one of (if not the biggest) money making industies in the world. Finally, I am going to college in September and can use my brain for exciting and fulfilling purpose. But whilst I am not the girl I was all those years ago, I still have CF and it still has a rule on my life whether I like it or not. I still have the harrowing lung implications and over time some part of me fears that this will worsen out of the blue and restrain me like it did before, but I’ll dwell on them possibilities rather than probablilities. I want to complete something for the first time in my life, and I will. If I have to struggle and wriggle my way through it, I will. I will get there.
I never cared about any of this before Gg’s death. I wasn’t bothered about fighting for it because I hadn’t reached boiling point yet. It wasn’t until recently after a conversation I had with her sister one sleepless night that I realised my own worth and what little value I had put on myself. I am deserving of this drug too but whilst I have the compatible gene for Orkambi, having had a liver transplant years ago relating to my CF could mean that the medication I take to lower my immune system would deem me an unlikely candidate as it may have a negative interaction. If I didn’t comply with the suppression, my body would recognise the transplanted organ as a foreign intrusive and produce high levels of antibodies to attack it. Ultimately if this happened, it would mean chronic rejection and my early death.
Fifteen of my Facebook friends are dead to cystic fibrosis since 2014. That’s 15 families still grieving and every day that list is growing here in Ireland the UK right across Europe. I have been to their funerals. I have comforted their families. None of it is right. But how many deaths will it take? When will enough be enough? How many more of us will lose ourselves in the coming of this battle? We are people and we are dropping off like flies in our early twenties, thirties and below. 21 is not a life lived, it’s a life stolen. And every minute delayed then and now is more time we don’t have.
I never expected my liver disease to decline as rapidly as it did and I don’t know if the same will happen with my lungs. To be honest, I’d rather not know. But I do not live in blissful ignoracnce that it will all be okay and “sure we’ll all be grand”. Sometimes that is the case but life has also taught me that it is as often not.
My brother said I have a stronger heart than him, but maybe this isn’t strength. Maybe this is what a heavy heart feels in all its compassion for what is has lost. Maybe this is what it is to have a soul and a spirit that’s alive. And if it is, I want that beat to keep on ticking as long as I have the air in my lungs. And I want to keep the air in my lungs, and never lose it. But my reality is a glimpse of that if my disease progresses beyond my control. Now I am far from the pessimist, but you just never know. My heart is not strong or resilient, it is broken. It has bared loss and still fights because it cannot risk anymore damage to it. I am not into hugs or pity parties. I cringe at the very hint of a remorseful thought put in my way, but I am honest and sincere enough to be real with myself. I accepted CF a long time ago for what it is and what I am sure it will eventually do to my mind and body. I can’t defy it, I can only live with it and do my utmost to try to overcome it. I will be blunt and say that it is an insult to injury when no1 really hears what our community is saying, when nobody understands and friends don’t share your heartfelt pleas. It is hard to be open and honest and not be afraid of what others view of you. To put yourself on the line again and again in hope of change is something I can only compare to what I’d imagine stage fright to feel like. To be honest, I am not at all comfortable with bearing my most raw and inner thoughts and feelings to the eyes and ears of just anyone, but I am one of the only ones who can for this particular cause. I’m 24 now. 24 but I want to be 25. And I want to be 25 until I reach 26, and so on. Life at any age is as important as when you begun it and everything should be done to preserve it as long as we can.
Whether or not Orkambi is suitable for me is not important, and not the pressing issue. I already know it may not be a possibility for me, but I would sleep better at night if life was made a little bit easier for the rest of us. It would make me happy if Vertex let go of their egotistical notions and offered a reasonable price. It would make a lot of people happy. That was my wish at the Trevi Fountain. It was just that, a wish among millions. To be happy and healthy and never fear what others don’t have to. But millions across the world are hundreds and thousands relying on an act of moral decency. We are not just fighting for ourselves. We are fighting for a future generation we haven’t even met yet. We’ve come so far in medicine, it can’t just stop here.
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