Respiratory - Cystic Fibrosis
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Cystic Fibrosis Care in Ireland compared with other European Countries Written by Philip Watt CEO CF Ireland and President of CF Europe
Cystic Fibrosis care services and treatments in Ireland have progressed steadily over the past decade, but with still much more to do. Cystic Fibrosis is a life-long recessive genetic condition for which there is still no cure. Ireland has among the highest rates of CF in the world with among the most severe manifestations – in large part shaped by the genotype profile of the CF population in Ireland. CF primarily impacts on the respiratory and digestive systems, but also impacts on other organs of the body. Rising care standards in CF care in Ireland have been shaped by access to new and innovative drug therapies; the work of the National Clinical Programme for CF; our expert doctors and their teams; the data and benchmarks provided by the Cystic Fibrosis Registry and of course the sustained advocacy by Cystic Fibrosis Ireland over 60 years.
“We are now in a position in Ireland to aspire to a higher level of care long enjoyed by some of our European Union neighbours, such as Belgium, France and Germany, rather than fighting for basic services and/or exposing the continued significant weaknesses and gaps in promised CF services”
Improved hospital infrastructure in Ireland, additional clinical staff and high quality research, especially over the past decade, has made a major contribution to the progress in CF services. However with this progress, CF care has also become significantly more complex. As predicted survival increases year on year, co-morbidities associated with growing older and CF related liver and kidney disease have emerged as more significant issues for an increasing number of our patient population.
CF, such as Orkambi and most recently Kaftrio. In paediatric care in particular there is a growing sense that while CF remains a life threatening disease, it can now be managed much more effectively. ‘Fire-fighting’ is now being replaced in large part with ‘fire prevention’ for many children and younger people. It is also important to emphasise that CF impacts on individuals differently and not all have being able to access or tolerate Kaftrio for example, especially those with rarer gene mutations.
Predicted survival rates of people with CF have been significantly boosted by the emergence of the new and innovative drug therapies that treat the underlying cause of
However these wonderful new drugs cannot undo the damage to organs that has already occurred. For example, almost 100 people with CF in Ireland have a double
JANUARY 2024 • HPN | HOSPITALPROFESSIONALNEWS.IE
lung transplant. Some now need a kidney or liver transplant. This cohort of patients were also most at risk from the recent COVID-19 pandemic. Further investment in post-transplant CF care in the Mater (MMUH) and other hospitals has commenced and this is to be welcomed but more needs to be done. Transplant services in general in Ireland are still recovering from the COVID-19 pandemic. Rather than fighting for basic services, we are now in a position in Ireland to aspire to the higher level of care long enjoyed by some of our European Union neighbours, such as Belgium, Netherlands, France and Germany. Much of the work of Cystic Fibrosis Ireland over the past decade has been to fight for the full implementation of agreed government policy to enable us to meet accepted European standards. In some aspects we are now among the leading CF care providers in Europe but still with much more to do. CF adult care in Ireland was given a significant boost in recent weeks as a result of green lights being given to the commencement of building of the long promised 20 bed inpatient unit in Beaumont Hospital and the new adult CF outpatient centre in Merlin Park Galway. CFI contributed ¤700,000 to the Galway Unit which will cost a total of ¤3.4m. CFI has also raised more than ¤1m for the Beaumont CF Unit, which will cost around ¤22m. We thank the hospitals and the HSE for providing the main capital funding for both of these important initiatives though delays have made these projects significantly more expensive. Over the past decade Ireland has made strides to catch up with our European neighbours in other areas of CF care. For example in 2011, after 20 years of lobbying, CF was finally added to the list of conditions identified through the Heel Prick Test. This means that treatment for CF mow commences from birth rather than waiting months of inaction because of the absence of an accurate diagnosis.
Other patient groups, such as Sickle Cell and Thalassemia Ireland have been advocating for many years for similar inclusion in new born screening. The new CF drug therapies are still not a cure and up to one quarter of people with CF in Ireland have presently no access to CFTR Modulator drugs such as Kaftrio. In this context it is welcome to see that a dossier has now been forwarded to the European Medicines Agency by Vertex which seeks to broaden the license for Kaftrio in Europe, including Ireland. In June 2023 I was elected as President of Cystic Fibrosis Europe
