Cystic Fibrosis: New Era, New Challenges, New Services
New Era: In the last decade, improvements in Cystic Fibrosis (CF) care have moved at a very welcome, yet rapid rate. The development of CF transmembrane conductance regulator (CFTR) modulator therapies and standardised multidisciplinary patient care have brought numerous benefits to people living with CF. Roll-out of the triple combination CFTR modulator compound, Kaftrio (Vertex pharmaceuticals), began in Ireland in September 2020.
Dubbed ‘wonder drugs’, many people with CF (pwCF) have benefited from these medications feeling less burden of disease and experiencing a new lease of life. Among the improvements are less frequent exacerbations, less hospital admissions, as well as new opportunities to travel, to work and to contemplate a future they hadn’t dared before.
New Challenges
While the landscape for CF care is changing, CF remains an extraordinarily challenging condition. It must be acknowledged that there are still pwCF who are ineligible for modulator therapy, as they have a rare mutation causing their CF which has not been included in clinical trials. Others may not be able to tolerate the therapies.
For these groups, the challenges faced are as almost as historical as the condition itself – access to medication, extended hospital stays, financial constraints and, tragically, death due to CF related complications – some still as young as infants. For those with access and benefiting positively from these drugs, there are also still significant challenges which have never before been anticipated – age related conditions with CF such as menopause, the impact of co-morbidities on a person with CF, liver disease, increased cancer risks and managing a family-work- CF-life balance to name but a few.
For others, the challenges of living with CF have changed, not lessened, with the advent of the modulators. As this group moves through the natural stages of life, they are navigating fertility options, access to mortgage related products, insurance, pensions and the issues that arise when planning for a future never before considered while living with CF.
New CEO
Always standing side by side with our members, Cystic Fibrosis Ireland are facing into this new era and these changing challenges with an open mind and flexibility to remain agile and respond to the needs of our membership.
In November 2024, Sarah Tecklenborg was announced as permanent CEO, having served as Interim CEO for most of 2024. Sarah joined CFI initially as Senior Research and Policy Coordinator bringing extensive research and management experience with her and since then, her vision has been to have the voice of the CF Community at the centre of activities.
Prior to 2024, Sarah initiated the Public and Patient Involvement (PPI) programme, support groups such as the Pregnancy Support and Advocacy Group, and since taking up the CEO position has been instrumental in campaigning for better CF care and access to medications.
New Services
Emma Wallace, CFI’s new Senior Member Services Co-ordinator came on board last June and has sought to enhance current supports and offer new services to support people to live well with CF. New services, campaigns and initiatives have since been introduced in direct response to feedback received from CFI’s members.
Among these are:
- Access to Modulators for those with rare mutations campaign A working group has been established to support the campaign for access to the modulators for the 6.7% who do not have access due to having rare mutations. CFI are calling for access to individual trials of the modulator therapies for all those who may stand to benefit from them.
- Access to mortgages and mortgage protection products campaign The core aim of this campaign is to address mortgage protection and its barriers for those who seek access for home ownership. The working group have also joined a subgroup with members of the Disability Federation of Ireland in efforts to collectively share knowledge and drive change in this area.
- Inpatient care packs Another new initiative is the role out of inpatient hospital care packs to many Hospital Network Teams. Within these packs are items that may benefit patients during their stay – in addition to information detailing the services which will support them in living with CF. To support the emotional wellbeing of a patient during a hospitalisation, CFI now also offer a hospital call back service which members can register for and ensure that those who do register receive a weekly check in.
- Parent Connect CFI, in partnership with our parents working group have formed the peer-led Parent Connect Team which aims to provide initial support and guidance to parents and create a sense of community and connection among those facing similar challenges. As part of the service offering, the group have developed a newborn care pack for distribution to new parents at the time of diagnosis by their hospital team and run a monthly online support group for new parents to connect into.
Much done, more to do
The phrase ‘much done, more to do’ has been frequently used to describe just how far CF care has come since the foundation of CFI in 1963, but how much more work is needed. In 2025, this still rings true. Research remains a key CFI priority to further the treatment of CF and ultimately f ind a cure. Among the projects being funded by CFI is research to develop a peer connect service for young people living with CF.
Communication and Collaboration
However, fundamental to all the work of CFI is communication and feedback. From members, from health care professionals (HCPs), from you.
“In spite of progress challenges remain, medication shortages, access to treatments for those with rare mutations, barriers to home ownership, mortgages and pensions – all which are a focus for us in the years ahead,” Sarah shares.
“The CF Community has always been and remains to be an incredibly strong group which has overcome many, many obstacles in the past. Our CF medical community have always been a crucial part of the journey and CFI would like to extend our thanks and gratitude to you for all the work that you do for and with our members.”
To ensure continued communication and collaboration, CFI invite HCPs to sign up for the new CFI monthly hospital newsletter, to receive updates of our new and existing services, grants, webinars and conferences.
Teams are also invited to share activities and news with our members through our quarterly magazine Spectrum – a key channel for our members to receive updates from hospitals and clinics for over 10 years.
Sarah continues, “I am excited about the future for people with CF. I look forward to building on the progress that has been achieved through decades of hard work and advocacy and to continuing the great work of those before us. As a team, we can continue to drive change and deliver on our goal of lives free from the challenges of CF – whatever form they may take.”
To join the hospital newsletter, please email memberservices@cfireland.ie. To contribute to Spectrum, please email ndelaneyfoxe@cfireland.ie.
To get in touch with the Parent Connect team, please email parentconnect@cfireland.ie
Written by Nicola Delaney Foxe, Cystic Fibrosis Ireland
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Read the HPN Jan/Feb 2025