Orkambi – A Love Story (Part Three)

Orkambi – A Love Story (Part Three)




The study conditions were strict and my physicians had rightly put the fear of god in me if I was to even entertain the idea of breaking them or do so accidentally. It was with due reason, sharing my experience could jeopardise the trial integrity, something that could have serious consequences for not only myself but potentially anyone waiting to benefit from the trial.


It meant telling very few people that I was on the trial – only our very closest family on the condition they could ask no questions. In fear of saying or doing the wrong thing I initially withdraw from posting on social media and my blog. It was the most beautiful of treasures kept secret between two people who couldn’t have wanted it more.


On the 30th of June 2013 I walked away from the hospital that had witnessed my most terrifying days, with an awe inspiring pile of precious pink pills. Pink has always been my colour obsession.


The morning after taking my first doses I awoke with nervous anticipation. There was a 1 in 3 chance that I could have been on placebo for the first six months of the trial so I was hesitantly optimistic.


The first thing I noticed was that I woke without being engulfed by a coughing fit. At the time, most mornings started with coughing so violently I vomited, my tiny bodying shaking and cramping with the force. On this morning, there was none of that. Cautious and protecting my heart from disappointment, I told myself that it was just a coincidence. Not as accepting, Cameron got to work to test it out scientifically with the tickle test.


I have always been stupidly ticklish, the very thought of it would sending me into a coughing fit because laughing was no longer possible. It took a good few minutes until Cam finally got a single cough out of me. I could laugh! We silently observed and set about enjoying the great start to the day – the day I now remember as the first day of my new life.


Part 3.2


In the coming weeks there were little signs here and there but nothing that would indicate huge shifts in my lungs specifically. One night about a week after starting the trial I experienced terrible abdominal pain – similar to that which had caused me so much grief when my lungs began to decline. The fear it sent me into was probably worse than the pain itself – what if it was something that could jeopardise me being on the trial? After a few anxious hours I reported it to my clinic and the pain subsided.


The first significant sign that I was on active drug was the amount of energy I had. I could be on my feet and on the go all day without a nap or accidently getting so exhausted I started to the sickly plummet to a hospital admission. Showering no longer exhausted me and I was able to carry more and more each time I went to the grocery story. I started to be able to do things like go on a coffee date or go out for dinner without planning – experience the joy of spontaneity. These activities no longer required ‘recovery days’. The constant achy pain that I lived with became only sporadic before eventually becoming a rarity.


As I started to breathe easier being alone became less daunting. I could walk the dog on my own, I could go into the city on my own, I could even go to the gym on my own. I began to be able to do things that had become a luxury and freedom that just wasn’t safe and rediscover the freedom of independence.


The better I felt, the more able I became to feel better – I was able to push that bit harder in my gym sessions, I had more energy to breathe deeper and focus more intently on my physio sessions. I found a love for spending hours in the kitchen cooking beautiful, sugar-free meals for myself and Cameron. We became quite the healthy foodies and loved being able to go to cafes and try new things that we could go home and try to recreate. It felt good to be able to feel the rewards of every little thing I was doing for my health – it felt like I was calling the shots again, not CF.


Unlike many people who start this therapy, I experienced no massive purge of gunk from my lungs. I can theorise only two reasons for this – that my extensive airway clearance routine (which included 20 mls of hypertonic saline twice a day for those in the know) was effective at minimising the goop or, because I was so used to being unwell, I didn’t notice as much. There were a few episodes of lung pain, but again, that had become so ‘normal’ to me that it didn’t indicate drastic change.


Life became so exhilarating and comparatively ‘fast paced’ that when I contracted a virus a couple of months after starting the trial, it was only upon being admitted to hospital I realised I had remained out for over two months without a decline or counting down the days until I was admitted. Life was starting to look very different.


In being admitted to hospital it began to hit me how lucky I had been. Silly perhaps, but life had been so much about just keeping going that there had been little time to get involved with the thoughts of ‘what if?’. On the hospital ward I saw the familiar faces of regulars, lifelessly walking the corridors. For the first time I could no longer see myself in them.



It was during this time that in a period of 10 weeks, 8 young women passed away in the cystic fibrosis community. There were actually more but these women were the ones who I had had personal interaction with – the ones I was friends with on Facebook, had encountered online or had passed in hospital hallways. Some of them had been healthier than me and all of them were around my age. Unlike me, their pink pills had not come in time.


I remember seeing one girl as she was rushed into the hospital. Critically unwell she was on a stretcher followed by a game faced team of medical professionals and machines. I will never forget the fear in her eyes as they hit mine underneath her oxygen mask. They pleaded for help, for someone to something. I instantly knew she had CF. Cam was with me and squeezed my hand as he felt my fear for her. She was a stranger yet I wanted to grab her and tell her it would be ok.


It wasn’t ok though. She spent a day or so in the room next to mine in hospital. Alarms would go all through the night, people would hurry in and out and I would lie awake wondering. Eventually she was transferred to ICU. I was discharged from hospital and my life went on.  A few weeks later, I learnt she had died. There were 7 others stories just like hers in short progression.


My heart ached for each of the girls who had been given their wings – for their families, friends and especially for their husbands and partners. I could feel their battles. At a time, my health was flourishing, others health was failing and there was nothing I could do to help them despite that neat stack of beautiful boxes in my medication cupboard. The thing that gave me comfort was that by participating in the trial and adhering as perfectly as I could to the study conditions it might prevent others going through the same pain in the future.


As the reality of what was happening to my health became clearer each day, I realised how far I was from where I had been and how low and unstable the rock bottom had been. It terrified me. My life had become so bright and shiny and new… So much so, I no longer felt I belonged there. Fear and guilt started to consume my joy. Those ‘what ifs’ and ‘whys’ became overwhelming. I was paralysed in the good unable to move into the great.


In September that year amidst the whirlwind of changes, Cam and I heard the band that we had been fortunate enough to have play at our wedding was playing at a venue an hour from home. We were excited to be able to book a night away and see them.


That night stays with me in my mind clear as it had been our actual wedding day (though funnily enough or perhaps perfectly, my phone went flat). It was the point we came face to face with what my new life meant to Cameron and I. As the music that played at our wedding filled the room, I had powerful flashbacks of how I felt at our wedding – what I wanted… what I dreamed of. And in those moments I realised that I now had it. The greatest wedding gift we could have received.


I had the opportunity to do the things that had once been impossible. I just had to be a little braver and let go of the fear, the guilt and all the things that did not serve me. Those feelings couldn’t change the past but they could change my future. It was that night as the man I loved and I danced and sang with freedom and ease – something that was impossible on our wedding night, I decided it was time to truly LIVE my new life. Our new life… (To be continued).


Part 2

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