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My cystic fibrosis is worsening every day that I don’t have Orkambi drug

The word ‘Orkambi’ means nothing to most people. To anyone who has cystic fibrosis, it’s a pot of gold at the end of therainbow.

Two years ago, I was one of eight patients over the age of 14 in Ireland to take part in phase three of Orkambi trials. The day I got the phone call to participate, I cried. I then smiled so hard my jaws hurt. I had won the lotto. I might not have had millions in my bank account, but I was being given a chance of life; I had won the health lotto.

In the few years previous, my cystic fibrosis had worsened, and my lung functions had a massive 20% drop in the year before Orkambi. And while I was as active as I could be — running, cycling — it took its toll on my body. A two-kilometre run took me treble the time it took a healthy person. I needed naps to get through the day. Life was a

struggle. I could never plan anything more than a week or two in advance because CF would get in the way. Hospital admissions were every 4-5 weeks, for a minimum of six-weeks. My average stay was 7-8 weeks though and I had even been in for 15 weeks.

Pre-Orkambi, I managed, I pushed through. My stubborn nature made sure of it. If you’re a healthy person reading this, I bet you never give a second thought to washing your hair or having a shower? Something so simple became such a painful battle within my body when I had a CF exacerbation. Taking a shower became so difficult it hurt. I needed a chair to sit on. My whole body ached as I gasped, trying to take in as much breath as I could to function.

Then came Orkambi. Within a few months, my body started changing. During my runs, I could breathe deeper. It’s not something I can explain, because I don’t think I’ve ever known what it’s like to breathe properly.

But I was no longer coughing and spluttering and, gradually, my need for naps became less and less, until, one day, it hit me that I hadn’t napped that week at all. Oh, my god, Orkambi was working.

I was only admitted to hospital every three months. Fast forward to December, 2015. I had been out of hospital for 11 months and, for the first time, could plan ahead in my life. I no longer needed daily naps to function. I was a normal person.

But then I got an unequivocal reminder of how things can change. Last month, I finished phase three of the trial but there was a delay in the Orkambi arriving under the open-access programme that entitles me to it until it becomes available in Ireland.

I was without Orkambi for three weeks and struggled, really struggled. My life returned to how it was two years ago. I was extremely breathless; even walking a few steps exhausted me and brought on coughing attacks. My lungs just felt heavy again. Chest physiotherapy got harder by the day; mucous wasn’t moving the way it had been on Orkambi.

I began having these symptoms within 24 hours of being off Orkambi, Once more, I was fearful that I would end up in hospital. And I was right to be fearful as I did end up back in a hospital bed.

It was heartbreaking, as I was almost a year out of hospital, the longest period of time I’d ever had hospital-free since my hospital admissions began.

But I knew I would have it again, so I would be okay.

But for those who haven’t even had the opportunity to give this drug a try, the wait for them is

agonising, while the battle for recommendation/reimbursement by our health service continues.

So, can someone please tell me why my life, and the lives of other CF patients in Ireland, have prices tag on them, and why we have to endure a

harrowing wait while the National Pharmacoeconomic Centre (NPCE) assesses the drug before making a recommendation on whether the State should fund it?

The news, last month, that the HSE ruled out funding for Orkambi was utterly devastating.

There are people that desperately need to start this drug now. They are desperately clinging to the hope that one pill may help them live a near-normal life without CF getting in the way.

Orkambi represents a chance for CF sufferers to, perhaps, stop progression in its tracks and get to spend longer with their families and loved-ones. We are generation that just wants to have a quality of life we deserve.

CF is a cruel, unforgiving illness and when there is an opportunity to acquire a drug like Orkambi, it should be fast-tracked and made available to patients within a few weeks of European approval.

CF will not wait for a decision to be made over months and months. CF will progress while people are made wait. CF will steal more lives while the decision to put a price tag on us is being discussed. I’ve seen, first-hand, the difference one pill can make to my life. I was shown quality of life and quality of life is priceless.

Jillian McNulty

Glack

Longford town

Co Longford


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