Orkambi – A Love Story (Part Two)

Orkambi – A Love Story (Part Two)




As the number of days to our wedding declined, so did my health. I became incredibly fragile – physically and emotionally. Most of the final planning for our big day was done from an uninspiring hospital room and my tank started to run on empty – I was loosing the ground we had fought so hard to gain in the previous years.


I would escape for dress fittings sporting my PICC line and hospital hair. It was hardly the dreamy experience you read about in wedding magazines.


In the nine months leading up to the wedding I spent half my time in hospital – including the final weeks before the big day. I became fixated on creating the perfect day because I wanted my husband to be able to have those memories forever. Even if he didn’t have me.


The wedding day came and went in a blur. The weather was perfect, we were surrounded by friends, family and fond familiarity and the wedding decorations (made from my hospital bed or by my fiancé who had obligingly learnt to craft) looked deceptively fabulous.  Most importantly for me, we had secured our dream photographer to capture the day and ensure it would forever be locked in time in the most beautiful way.


On the outside everything would have appeared perfect. Both Cam and I are pretty experienced at putting on our game faces to protect the ones we love – and sometimes ourselves.


Shortly after the wedding Cameron and I returned to Brisbane where, instead of a honeymoon, I was admitted to hospital for what would be a terrifying yet perfectly timed admission. What started out as a very ‘routine admission’ – something that had become a very normal part of our lives, quickly spiralled downward into a hot mess.


I was having troubles with medications I needed to get me well and my doctors could see I was fading fast. Not that they ever admitted this to me! But I could tell. I could tell in the muffled conversations outside my door, I could tell in questions like “Do you know what blood type you are?”.  I could tell in the new tests I was instructed to have done.


As a patient in the age of social media and technology there is not a lot you are given the fortune of being shielded from. Those tests meant serious business. Business that I wasn’t sure I wanted. I mean nobody would ever want a transplant but to me it was more than fear – even if faced with the ultimate decision I still wasn’t sure transplant was the right choice for me.


Thank goodness I was blessed with amazing doctors who were determined not to give up and do all they could before sending me down that road. Even without a word from me, I think they knew what my decision would have been. A couple in particular, knew how desperately I wanted to make it onto the trial. I would tell myself a thousand times a day – the next trial phase HAD to recruit soon, just hang on a little longer.


You know when you do something that at the time makes no sense but later the reason becomes perfectly clear? I honestly have no idea why (gosh I could barely look a day into the future with any clarity of what would happen) but it was during this gruelling admission I heard about an event called ‘Soulprenurs’ that was being held in Brisbane and I felt the need to go. For a couple scraping together enough to cover the bills each week the $60 ticket seemed like a luxury and there was no way I could justify buying one for Cam to accompany me. Still, something called to me and gave me the confidence to buy a ticket.


I got a hospital leave pass for the evening and for the first time in longer than I could remember went to a social gathering on my own.  It was here I first heard of the term ‘life coach’ and found some of the ladies that would later influence my current path the most. I went away inspired wishing more than ever that things were different so I too could inspire and support others just as I had witnessed from the speakers that night. Taking the time to dream – just for a moment, was the boost I needed to keep going.


Days turned into weeks and weeks turned into months. I remained in hospital determined to get well. After a month my health stabilised and after two my doctors thought with a lung function of 41%, I was ready to go home. Stay as well as you possibly can they urged me more than ever.


I have never taken anything so seriously in all my life. In terms of patient compliancy, in the years prior I had been incredibly strict with myself – a model patient, but the scary place that I had so recently been and the prospect of gaining access to the new therapy had me tipping into OCD territory. Allowing my husband to plan my funeral in the same year we had been married wasn’t an option I was prepared to consider, I couldn’t do that to him.


I dared not to miss a single dose of medication, skimp on any of my daily physiotherapy sessions or miss a bi-daily appointment with the gym. On the days I didn’t have the energy, Cameron found it for me and did whatever I needed to keep me going.


It was during this time that I started considering the importance of nourishing my body – trying to get any gain I possible. After our wedding. Cameron and I had spent some time speaking to our photographer’s husband – an orthopaedic surgeon, cancer thriver and passionate advocate for eliminating sugar and particularly fructose from the diet in light of his own comprehensive research.


At this stage we were in late April and Cam had been following his suggestions since a week after our wedding. By default, I was eating this way too and I started to realise how empowered it made me feel to make the choice to nourish my body with real foods instead of just filling it up. This became an important part of our daily routine, something else that helped us feel like we were fighting our best fight.


Two weeks after my discharge from hospital I fronted up to CF clinic praying that my lung function wouldn’t have fallen too far, as had become the pattern between my hospital admissions. For once it seemed like my hard work was paying off and my numbers remained stable. The study was again mentioned. IF (I-F!!!) I could hold my lung function steady for 8 weeks they expected recruitment for the trial would be open and I might have the opportunity to attend a screening visit. In order to be accepted my lung function needed to be above 40%, no negotiations, no exceptions, no second shots. Mine was still sitting just 1% above. From where I was it seemed a long shot, but that 1% was all I needed. Finally, I could see a light shining.


Part 1



Our lives became laser focused on one goal. We refused to go out in public due to the risk posed by ‘cold and flu season’. We did our shopping at midnight when the isles were empty or had it delivered. We ate, therapied, exercised and slept. In an attempt to get out of the house and avoid crowds we spent a lot of time at the beach.


May is a beautiful time in Queensland. The skies are blue; the sun is out but it isn’t too hot to enjoy it. We would walk very very slowly along the beach for hours. It was the first time in years that I allowed myself to get involved with dreams of a future. Cam and our border collie would run along the beach with ease and I would close my eyes and picture myself running freely with them. Hope felt like the most amazing and luxurious feeling in the world.


Each day I would wake and sniff and swallow. No blocked nose, no sore throat. I was still in the game. Time dragged but as long as I had that hope, it didn’t matter.


The day I went in to the hospital for a pre-screening visit discussion stays clearly in my mind – I have no doubt it always will. I remember where we sat and what I wore. I remember the blue sunny day looked even more beautiful than usual and the feeling of elation and joy as I walked away. I was indeed eligible for a screening visit but that 40% was still the magic number, the biggest obstacle standing in my way.


I find it funny now that I never actually felt fear about participating in the trial. Not once. Despite in-depth discussions with my doctor, warnings from my husband and the pages upon pages of documents from the pharmaceutical company outlining the potential risks, I was undeterred. Usually I am one to question anything yet I felt incredibly at peace with the idea. Even though I was physically doing everything I could to make it on to the trial, in my mind I became uncharacteristically peaceful about the outcome. The girl who liked to control everything found a new level of acceptance or maybe an intuitive knowing that I could do this.


Two weeks later I screened for the trial. As I held the mouth piece for the lung function machine my future was literally in my hands. I had one chance, one attempt, at securing not only my own but the future Cameron and I had as husband and wife. In that single blow my life – our lives, changed forever… (to be continued)





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