Fighting to improve care for people living with cystic fibrosis in Ireland Mr Daniel Costigan, Senior Research and Policy Co-Ordinator, Cystic Fibrosis Ireland (CFI), reports on the annual patient conference of CFI, which was held recently in Carlow
n spite of the incredible evolution of cystic fibrosis (CF) care over the last 10 years, CF remains a very challenging illness for the patients and families affected by it. That was the take-home message from this year’s 2019 Cystic Fibrosis Ireland (CFI) Annual Conference and AGM, which took place in the Mount Wolseley Hotel in Tullow, Co Carlow, on 29-31 March.
‘Incredible’ evolution in CF care
Mr Philip Watt CFI CEO Mr Philip Watt opened the threeday event by drawing conference delegates’ attention to the incredible evolution in CF care that has occurred since the 2009 publication of the HSE’s CF services report. This report largely endorsed the findings of the earlier CFI-commissioned Pollock Report (2005), which called for a dramatic overhaul of CF services in Ireland in order to reach acceptable clinical standards. Since then, a network of specialised CF hospital centres has been developed, transplant rates have improved, newborn screening for CF was introduced in 2011 and a range of innovative and ground-breaking drug therapies which address the root cause of CF, including ivacaftor (Kalydeco), lumacaftor/ivacaftor (Orkambi) and tezacaftor/ ivacaftor (Symkevi) have been approved. In hindsight, there was also a further crucial, and largely unsung development, which was to have a very profound impact on CF care in Ireland. Because of the shocking findings of the Pollock Report, significant additional resources were made available to increase the number of CF consultants in Ireland. These new, dynamic CF consultants were in large part recruited from experienced CF and general respiratory doctors who were Irish but working in hospitals in the US, such as Seattle in Washington State, Philadelphia, Baltimore in Maryland and New York. Their return gave CF care in Ireland a huge boost. Dr Laura Kirwan (PhD), Research Statistician at the CF patient registry, outlined the dramatic impact that these improvements have had on the quality-of life for people living with CF in Ireland. Mean lung function, BMI and median age of survival have all seen marked improvement over the last 10 years. The median age at death has also increased, from 25 years of age in 2009, to 32 years of age in 2017.
Prof Barry Plant
Dr Laura Kirwan
Dr Michelle Murray, Consultant Transplant Respiratory Physician at the Mater Misericordiae University Hospital, Dublin, highlighted that, as a result of these improvements, many patients are now not requiring transplant until well into adulthood. She went on to say that transplants are not for everybody. According to Dr Murray, transplant patients are a very unique group of CF patients and they face their own particular challenges and have their own specialised needs. Lung transplant is a relatively new intervention and has become a more attractive option as the field of transplantation has evolved. The first lung transplants were done rarely and as an experimental therapy in the early 1960s to 1980s, with very poor one-year survival of <50 per cent. In the early days of transplant, CF was considered a “contraindication” until Joel Cooper and colleagues in Toronto performed the first such transplant for CF in 1984. Ireland carried out its first single lung transplant in 2005, its first double lung transplant in 2006 and opened its first adult lung transplant facility in the Mater Hospital in 2011. For individuals with CF, post-transplant survival is increasing, so Dr Murray recommended periodic discussion of lung transplantation to destigmatise the procedure. This discussion would include the risk of various complications that can develop post-transplant, such as chronic rejection and various malignancies. It would also include discussion of the transplant and post-transplant journey, which can be very psychologically challenging. Patients and their families may be disheartened as the steep rate of recovery during the early post-operative pe-
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