Orla Tinsley: Removal of Cystic Fibrosis drug Orkambi is a catapult towards death

I found myself at the Ellis Island museum last month staring at the statue of 17-year-old Annie Moore from Cork the first person to be processed through the famous immigration station to New York City. She stands poised and proud with her gaze upward towards her future. I turned to my mother and told her how funny it was that had I attempted to immigrate back then I would have been sent home to Ireland covered in every chalk mark they could muster.

My mother reminded me that my parents tried to move to Australia when I was born so I could live in a better climate but they were rejected on the basis of my illness. They mentioned this to me so little that I had forgotten. Years ago my illness would have prevented me from accessing the American Dream too but right now in this era I am living in reverse.

I am full of hope and adventure studying in the United States on a drug that is keeping me alive but the news yesterday that the National Centre for Pharmacoeconomics have rejected Orkambi on a cost basis is devastating. I, along with the entire Irish Cystic Fibrosis community, have been waiting for a decision for months. We have been watching how access has been managed internationally and we know the price is astronomically high.

I began Orkambi in Ireland via the worldwide Vertex trial in 2013 which I continued on in America and then the drug was approved here. International students in the US must have health insurance and my health insurance means while it is still expensive to be treated here it is just about manageable. It means because my plan accepts the drug I can access it. I’m making it clear because I’ve been asked about this by lots of people : Why and how did you get this opportunity?

This past week I’ve been particularly attuned to cost effectiveness of drugs in Ireland particularly the cancer drugs that have not been made available to people who really need them. The fear reading about this created followed me around all week. We are all struggling to access a better life and the more inaccessible drugs are the bigger the problem becomes for everyone. Quality of life dwindles, worlds shrink and so many people die while they wait. We have been waiting since before the general election for a decision on Orkambi.

Chronic degenerative illness from day one of life is hard but chronic degenerative illness devoid of hope where a drug is available that can change the outcome is cruelty. The drug was rejected “at submitted cost” which means it is currently too expensive at about €160,000 per person per year or €391 million over five years.

A full out rejection is not just a question of monetary cost when it comes to chronic degenerative illness, it’s a question of hope and what happens when you totally obliterate it for an entire community. Yesterday Sharon Byrne, whose child has CF, posted on Facebook: “It’s a sad day for our family and other CF patients in Ireland . . . Do we have to pack up everything and move our family to another country in order to give our son a chance at a better quality of life? Well let the battle commence . . .”

It is shameful to think such an exit would be the only solution given how far we have come with cystic fibrosis care in our country thanks to the work of so many different communities of people.

My friend in Ireland with CF who is the same age as me and was on the Vertex trial from 2013 has been told by her pharmacist it is “highly unlikely” her Orkambi supply will continue if the drug is rejected. She now waits in limbo and in fear. Removal of Orkambi is a catapult towards death for her. Removal of Orkambi is a catapult towards death for me. People who have come off it for just a week have talked about the rapid decline that occurs.

I am not scared of death but this cannot be the solution. A small percentage of people have access to the drug under compassionate use in Ireland but it’s unclear what will happen with that. The only solution to this problem is for Vertex to reduce the price and the HSE to negotiate it so the drug can become available to Irish people who needed it yesterday.

Orkambi holds me at a place where life is manageable and where positivity is more possible despite the mounting obstacles that come with chronic illness. When I turned 29 in March I needed oxygen to walk around because my lungs could not be without it. Luckily New Yorkers still yelled at me when I lined up at the wrong side of the butcher’s counter, it’s a good city like that.

My life became a routine of not just normal four hours a day CF treatment every single day but weekly o2 delivery men, stocking up o2 tanks and making sure I didn’t lose the little nozzle that cracked open the valves to start the flow of the tanks which I had to change every six hours. I lost it twice and spent hours searching my apartment on hands and knees before taping it to the wall with bright pink tape.

Some weeks later the infections severity lifted and I was myself again. I’m not sure I would have recovered pre-Orkambi but I told myself, as I always do, that whatever happened would be okay.

Since my 28th birthday each time I am sick I remember two things performance artist and musician Laurie Anderson said at a concert I saw in Carnegie Hall and then again in her documentary film "Heart of a Dog". "We tell ourselves stories in order to live" and that "we must learn how to feel sad without being sad".

People with CF are very good at self-motivation despite the odds and practised in the art of turning their devastation at bad government decisions into positive actions. The story that CF offers us is no longer a curvature towards certain death by age 30. It’s changing and being challenged and defied by people and technology fighting against it. The narrative can become one of survival. I have been fighting cf every day since I was born and fighting for facilities and drug access since I was 18. Fighting is part of the lexicon of cystic fibrosis and illness. We are warriors from day one.

‘Fighter’

I used to dislike how the word "fighter" labelled me as a kid but the past 11 years of campaigning has taught me that we have to continuously fight to get to the other side of this. When Patti Smith wrote "Those who have suffered understand suffering and thereby extend their hand" she could have been talking about the CF community, the public response to the community and that collective resilience. Together, through sharing our own narratives, we have proven we can make change happen. We will never stop fighting.

That day at Ellis Island my mother said that thing Irish Mammies say: “Remember when things get tough you can always come home.” We laughed at ourselves and embraced in one of her family famous scrunches. We both know I can’t just come home because my health does not allow for causal plane journeys. I miss important things like my grandmothers funeral and Christmas but that’s the deal.

I feel so proud that my Mom can visit me and I can walk places showing her around my new city. It feels like I am fulfilling my part as a daughter and growing up in a way we thought might not happen. We know now that if the HSE does not negotiate a price drop for Orkambi then coming home would mean a certain accelerated death.

I am so far away from the constant daily struggle I was once confined by that this thought is newly harrowing. Generations need not suffer because a drug company and a government cannot negotiate a real solution. There must be a price reduction. The results of the Orkambi trial were released two years ago this month. That’s two years the 505 Irish people suitable for Orkambi have been waiting to sail on to that new chapter of their lives.