When I became an activist there were no orphan drugs and there were no international standards of care in cystic fibrosis (CF) centres in this country. In order to speak on such things, a great risk was taken and the impact of that risk rippled through in the care I received from some people.
In short, it was difficult. It is never easy to be the first person to speak the truth on something painful out loud. And, over time, as things heal, the biggest tragedy is the assumption that the job is done.
The triumph comes from watching a new generation of activists, unafraid and unapologetic, moving forward. And as progress is made, the world expands. These past two decades, the main focus of CF activism has been on facilities and orphan drugs in Ireland. Now, with the release of the Cystic Fibrosis and Lung Transplantation in Adults: A Model of Care For Ireland, we can finally turn our gaze towards transplant care and, most specifically, post-transplant care on a global and national level.
We cannot have a fully functioning national CF service in Ireland until we can fully support our heart and lung transplant service and all that comes with it. The investment in a concurrent co-creation must happen with an understanding that there cannot be fully realised CF care without fully funded post-transplant CF care. The document released this week draws on international literature, and impressive voices participated in its formation. It is a formidable undertaking from Irish experts across many arenas.
I didn’t understand why people with cystic fibrosis continued to attend anywhere but their transplant centre after the transplant had taken place. In New York; once the transplant happened, all care was centralised at the transplant centre which was also a cystic fibrosis centre. But our only lung transplant centre here - the only one in Ireland- is not. And it does not yet have the resources to be
This is the final frontier in CF care here and we must tackle it immediately. For example, in order to facilitate more inpatient beds in the Mater for transplant patients, the outpatient transplant facilities were gutted. This deficit mindset by successive governments and healthcare leaders – where something in the health service suffers to facilitate something else – cannot be part of a future Ireland.
We can’t have fully resourced national transplant programmes, particularly for those with cystic fibrosis, unless all aspects of care are covered. Supporting our transplant programmes means ultimately supporting every citizen in Ireland. When the miracle of life occurs, we must be able to protect it for as long as possible. There is no reason that Ireland cannot lead in this except for one thing: resources.
This paper is a framework we have needed since Paul Minchin had the first double lung transplant of a person with CF on Irish soil in 2007. That it has taken so long is difficult to understand but thanks to dedicated work, it’s now here. In order to move on, the Mater must be made an accredited CF centre and there must be multidisciplinary care in all CF centres. It must be the framework around which double lung transplant CF care is based, although this still does not satisfactorily address the issue of other transplants.
The section that says this needs no review is a confusing oversight. For example, there is no official transplant co-ordinator at the Mater for kidney transplants but a remarkable nurse doing the job for no additional salary. She deserves better and so do patients. We must create a clear and urgent framework around this for those with CF who are post double lung transplant. And while kidney failure is not a symptom of CF per se, it is more related to post-transplant care, it still matters.
When I went into kidney failure it was not peaceful. After weeks in the high dependency unit (HDU), I was rolled down to a mixed ward without being told where I was going.
Suddenly, as a person with CF, my dialysis was a communal affair and the fact that I was a double-lung transplant recipient – which also meant I must be treated in a single, en-suite room when in the hospital – was no longer anyone’s concern. It was very confusing: the scientific facts regarding both my immunosuppression and my CF were suddenly no longer relevant. I was expected to forget they co-exist and accept the reality of a mixed ward in an Ireland that has constantly failed CF patients because this is just “how it is”. I thought people with CF in this country had left facility issues behind until I returned to Ireland.
When you consider that many original Irish CF activists – if they are lucky enough to have survived this long – may find themselves in a similar situation at some point, one has to ask: why? My valid concern about infection control was disenfranchised and later disappeared.
In the hospital, cross-infection was a massive threat and I was expected to forget the clear, international standard of care tenets that have guided my life as someone with CF
My brilliant nephrologist listened to my concerns about infection control, which she said no one had ever put to her before, but I was in the HDU at that point. When I was well enough, I was transported back and forth to a mixed ward for dialysis because there is no framework around this. The second day of this I refused – not the healthcare but the danger of cross-infection. This minor stand-off meant a manager came up to discuss things and explain how they had been carefully considering the infection-control issue. It is not unusual for someone who has had a double-lung transplant to have kidney failure; in fact, it is common enough. But my dive into this was uncharted.
When I read Cystic Fibrosis and Lung Transplantation in Ireland, it was so promising finally to read a clear and concise path forward for pre- and post-transplant patients in Ireland, but we need urgent and clear information around the topic of kidney transplant in double-lung-transplant recipients with cystic fibrosis. Otherwise, this remarkable policy document, which has been sorely needed these past 15 years since Paul Minchin had the first double-lung transplant of a person with CF in Ireland, misses out.
In the hospital, cross-infection was a massive threat and I was expected to forget the clear, international standard of care tenets that have guided my life as someone with CF and someone who, this December, celebrates five years post successful double lung transplant. I have guarded this second chance at life in honour of my donor and their family and my friends and family. I celebrate the miracle and the CF care for which we have fought. Back in Ireland, I could no longer access the National Referral Centre for CF at St Vincent’s, which I helped to build. It would not accept its post-transplant patients back but Beaumont’s adult CF centre would. This document doesn’t explain this but it does offer ways forward.
Friends who were pre-transplant at Beaumont counted their blessings when attending post-transplant there, because serious issues were found on time due to this access. Thank goodness for the detail in this report which makes real the commitment that we need urgently and for the future of all transplant CF care. Different models of shared and singular care are suggested.
The Mater, with the right resources, funding and staffing ratios, will flourish and become a cystic fibrosis centre to support the work being done by staff already there. The need to ringfence operating theatres for transplant surgery to take place is also important. This means we won’t have a repeat of what was confirmed this time last year when a young woman could not have her double lung transplant despite the organs being available due to lack of space. No one would die because of the lack of ICU beds and operating theatres.
Any forward-thinking CF care must be joined up with the objectives of resourcing our national transplant programmes for all care. The overworked staff giving phenomenal input under impossible circumstances deserve better, as do the older generation of people with CF whose longevity deserves the same attention and advocacy that the orphan drugs of now are given. And let’s get access to them for post-transplant patients when appropriate.
When all of these elements are working together, we will continue to have a national programme that can represent Ireland on an international stage.
Now that would be something to be proud of.