Spectrum - Spring 2023

SPECTRUM Spring 2023

A sneak peak at 65 Roses Day - 14th April

www.cfireland.ie

i

CONTENTS Latest News | 2-4 * Reflections to 1963 * The future of Spectrum

Member Services | 5 *PPI: Your Voice Matters

Spotlight | 6-17 * Introducing CFI's new Chairperson: Bernie Priestley *Brendan Lonergan - A Life Less Limits

Team Feature| 18-21 * What Generation are you?

CFRI | 22-24 *Using Data for Good

Research Update| 25-26 * Update on Jen Balfe's research study * Steps Ahead: Optimising physical activity in adults with cystic fibrosis

CFI Conference Report| 27-34 Fundraising| 35-45

A massive congratulations to all the families who fought to secure Kaftrio for their 35 children previously denised access. The children and familes are celebrating further as they begin receiving their presription and a new phase begins.

Pictured above is Hazel Buckley who collected her prescription recently.

Many thanks to her parents for allowing us to share this wonderful moment with you.

* Challenges and Events * Thank You

Back Cover: CFI Branch Information

EDITORS :

Nicola Delaney Foxe FRONT COVER: Ben and Sarah Healy (6) from Portmarnock

DISCLAIMER: The views of contributors, when expressed in this publication, do not necessarily reflect the position or policy of Cystic Fibrosis Ireland.

ii

Spectrum / Spring 2023

Someday, CF will stand for ‘Cure Found’ These are the words of hope from our newly elected CFI Chairperson, Bernie Priestley, in a specially commissioned 11 minute documentary to mark the 60th year of our association that is now available to view on our website and social media links.

There was a special atmosphere at the CF annual conference this year in Dunboyne Castle Hotel in Meath, held at the beginning of April. It was our first CFI Conference held ‘in person’ in four years because of COVID19. We acknowledged those who founded what is now Cystic Fibrosis Ireland, which was was formed in 1963 in a lecture room in Crumlin Children’s Hospital.

Philip Watt, CEO, CFI

It was an honour to thank our Board, our branches, our staff, our clinicians and their teams, and all those who continue to support our wonderful association. Thanks also to Minister for Equality and Children, Roderic O’Gorman TD who opened the conference.

The CFI conference this year was also special because of the decision by the HSE on March 21st to provide access for the 35 children excluded from Kaftrio since May 2022. There were many tears of relief (and hope) among the families of those impacted when they heard the news, though we all still question why we had to wait for 10 long months for this decision.

The dispute between the HSE and VERTEX highlighted continuing gaps and weaknesses in our drug reimbursement system, some of which was acknowledged by Minister Donnelly, that indirectly discriminates against all those with rare diseases in Ireland when a drug therapy becomes available. We have only 30% of the available EMA approved rare disease drugs in Ireland compared with 95% in Germany and 61% in England. Is this denial of human rights fair or equitable? We hope the Government will legislate for the rare diseases drug legislation before the Oireachtas in a private members bill.

Philip Watt CEO CFI

www.cfireland.ie

1

LATEST NEWS 1963 - The dawn of hope for those with CF and why we have often have to fight for adequate CF services in Ireland It is truly remarkable to look back to the very early days of what was the Cystic Fibrosis Association of Ireland (CFAI) and which is now Cystic fibrosis Ireland (CFI).

In a lecture theatre in Crumlin Children’s Hospital in November 1963 a small group of parents, with support from a couple of clinicians, established CFAI. Anne O’Dwyer and Bridie Maguire will never be forgotten as the founders with many others of CFAI, one of the first patient groups in Ireland.

The early aims of the association were to provide 'oxygen tents' and 'aids to breathing’ and CF research inspired by what was to become the CF Foundation in the US. It was estimated there were 175 cases around the country by the mid 1960’s and ‘failure to thrive’ through malabsorption of food and a 'wheezing cough' were identified as key symptoms. Misdiagnosis was often a problem facing parents at this time.

The first effective medications for CF only really commenced with the introduction of Creon in 1989 and the emergence of effective antibiotics such as inhaled Tobi in the 1990’s.

Since 1963 it as often been a struggle to convince the Government of the need for bespoke services, drugs and supports for CF. Early CFAI struggles focused on: •

Advocacy and funding for the first CF paediatric centre in Crumlin in 1969

•

The ending of having to pay for a doctor and CF medications which finally came about with the long-term illness scheme in 1971

•

The building and staffing of a network of CF centres that followed the CFAI Pollock Report in 2005

•

The introduction of newborn screening for CF in 2011, which was first mooted in the 1970’s

•

The campaign for the building of the Nutley Wing in SVUH that lasted a decade. This set the template for isolation and air-filtrated rooms to follow

•

Down to the present day when significant advocacy was needed to support the 35 children excluded from Kaftrio for 10 months.

Why has it often been a battle to convince and secure adequate CF services in Ireland? Key factors over the past decade from my experience include: •

2

Delays in enacting agreed government policy - just because its named in the Programme for Government or an HSE planning document does not necessarily mean it will be prioritised - often further advocacy is

Spectrum / Spring 2023

needed.

•

Reluctance at times to accept clear evidence without the addition of advocacy

•

A lack of full understanding of rare/orphan illnesses in Ireland.

While there has been much progress in CF services over the past decade, it has often been a battle, even now. I remember talking to one former Professor of health (unconnected with CF) who is a strong believer in ‘utilitarianism', including in health policy. Put crudely, this concept contends that at times you have to leave some cohorts of people behind ('tough decisions’) if you want to adequately treat the health of the majority of the population.

Those who hold such beliefs highlight that treating rare, long term diseases (including drug reimbursement) is often more expensive for those with ‘common’ diseases and thus, people with CF could be effectively depriving others from care. While this concept is unlikely to be shared by many decision makers and is unlikely to be the view of those with a child living with a rare disease, utilitarianism attitudes certainly do persist.

It is a concept that internationally is increasingly viewed as flawed, outdated and contrary to the human rights of those with less common health conditions. While of course, difficult decisions have to be made in allocating resources to all public health services, the default should not be at the expense of those with rare diseases.

It should never be a question of either/or - but to allocate adequate resources for both common and rare diseases. Sometimes that means assigning additional resourcing for certain conditions (targeting) as well as mainstreaming the needs of all into the public health policy.

It is about time we consign such attitudes to the dustbin of history once and for all. In respect of medications, gig Pharma has of course its role to play in making medications affordable but public debate often solely focuses on this part of the equation and rarely on the underlying principles and ethos of the system that assesses such medications.

Philip Watt CEO CFI

www.cfireland.ie

3

LATEST NEWS Spectrum: The need to reduce increasing printing and posting costs Alas, like many things, It has become increasingly expensive to print and post our magazine Spectrum. For example, the costs for the printing and postage of 4 editions of Spectrum in 2022 were as follows: Edition of Spectrum cost of print and Postage •

April 2022 - €4,939

•

July 2022 - €5,142

•

Oct 2022 - €6,237

•

Dec 2022- €5,492

Total P&P for Spectrum for 2022 was €21,810. This is an unsustainable cost and rate of increase. If we distribute Spectrum via e-mail/link we would make a similar saving of €21,000 per annum (and rising) or, for example, if we print and post one issue the total cost at present would be around €6000 per annum.

We will be writing to you by e-mail and letter (for those who receive a posted version of Spectrum) with a short survey and proposal and we would welcome your opinion. In doing so, we are conscious that some of our members: •

Do not have access to a computer/have insufficient IT skills, even if there is a computer in the home

•

Receive a printed version Spectrum by post as a preference for various reasons - even with adequate IT skills and a computer/tablet

An added issue to consider is that the print cost of 100 copies of Spectrum is often not much different than 500 copies, so even if we reduce the print run, there will remain substantial print costs. A further added issue is that it often takes 2 weeks to print and post Spectrum, and by that stage some of the information can feel dated, especially the news section.

Proposal to make savings Any final decision will be made by the CFI Board taking into account costs and access and all other issues: One option would be that two issues of Spectrum per annum are printed and posted for those who wish to avail of this choice.

We would ask also all who currently could read Spectrum online but currently receive a printed version by choice, would consider receiving a digital version only. We would ask family members who are computer literate to assist in making other issues of Spectrum available with IT support to other family members. As an experiment we are sending out this issue in digital PDF form.

PS Following discussion at the AGM the annual report will be posted to those who wish ro receive a ‘hard copy’. We will be shortly sending out a short survey, so please let us know what you think.

4

Spectrum / Spring 2023

MEMBER SERVICES Patient and Public Involvement in Research (PPI): Your voice matters!

Antimicrobial Resistance in CF Research CFI are inviting people living with CF, and their families, to join our panel of PPI contributors to support CF research development around Ireland. This is an important opportunity for people with CF to contribute to shaping the future CF research and directing investment to the questions that matter most.

What research could I get involved with?

CFI is providing support to an Irish research team from Maynooth University who are looking at Antimicrobial Resistance (AMR) in CF. The lead researcher is Dr Fiona Walsh, Professor of Microbiology (BSc, PhD). Dr Walsh’s team are planning a number of different research projects that focus on AMR, including how certain bacteria go from colonisers/bacteria surviving outside the patient to those causing infections.

This may involve investigating the bacteria; how they differ between those not causing infection (including surviving in the hospital) and those causing infection, the human host; do the people’s immune systems differ; how people’s behaviour effects these bacteria and using chemicals to understand how the bacteria stick to surfaces and people. It is a wide ranging subject and the exact direction of the research question/s are still to be clarified – with the help of our PPI Panel.

What would I be doing?

As a PPI contributor, you are not a participant in a research study but a valuable member of the research team. You will be actively involved in the decisions made about the research as it is being designed and developed. You bring a wealth of expertise from your lived-experience of CF and, in this case, AMR infections.

This will include providing valuable insights into the actual practices within the hospitals and your treatment experience.

For this specific project, PPI contributors will attend one-hour meetings approximate four times per year (via Zoom) to share insight, answer questions and give personal perspective. This project is expected to last for 4 years and so you will become familiar with other panel members and the research group. PPI contributors will be reimbursed for their time. The purpose of being a PPI contributor is to ensure that the research that occurs in the world of CF is targeting the issues that really matter to the CF community.

I’d love to join! What do I do next?

Contact Liz Jacques at CF Ireland to register your interest and get more information: Ljacques@cfireland.ie or phone 01 496 2433.

If you have any questions or concerns around any of the topics in the Member Services' Section, please get in touch with our Member Services Team.

Sam Byrne sbyrne@cfireland.ie

www.cfireland.ie

Rory Tallon

rtallon@cfireland.ie

Caroline Heffernan

cheffernan@cfireland.ie

5

SPOTLIGHT

A N

Chairperson is crucial to the effective running of any organisation. The role of the chairperson is to provide leadership but they must also be an effective strategist and a good networker.

At the beginning of 2023, we bid farewell and many thanks to Keith McCabe, who held this important position for 2 years. ow, in our 60th year, we are delighted to welcome on board Bernie Priestley as our new Chairperson who will take CFI forward into it's next chapter. While most of you will know or have met Bernie, not all may have had the pleasure, so Bernie has taken time to tell her story in this edition of Spectrum by way of introduction to herself both with, and without, her Chairperson hat on.

Bernie Priestley from Dublin, is married to Sean and mother to five children, Ciaran, Colm, Eoin, Aidan and Triona.

Colm and Triona were diagnosed with cystic fibrosis and while Colm is in his thirties now with two children of his own, Triona sadly passed away in 2014 aged 15.

Colm was born smaller than most but having arrived five weeks early, no one thought anything was amiss. It wasn’t until he was three months old and began getting recurring chest infections and couldn’t hold his food in, that Bernie brought Colm to his GP for further investigation. This was 1986 and CF wasn’t very well known at the time. However Bernie’s GP was familiar with the condition and referred Colm to Temple Street for further tests.

Once in Temple Street, Colm was looked after by the consultant, Dr. Carson, who organised for Colm to be given three sweat tests.

Bernie, who knew two children with CF, knew what this could mean for Colm. On the 10th September 1986, Doctor Carson’s said ‘I’m disappointed, but I’m not surprised, Colm has cystic fibrosis.” Cystic fibrosis had entered the Priestley family and Bridie Maguire, then CEO of CFI, visited the family. She offered crucial advice which Bernie remembered for years to come.

“At that time CF was a very life limiting condition. The people I knew were very sick with it and it was a very scary place to be. I remember we were brought to social workers and we went home with all this stuff that we had to do. We hadn’t got a clue,” Bernie explains. “Bridie visited and told us that Colm was our son and not to compare him to anybody else. We are all individuals, we are all different and work only with our child.”

With the support of family and friends, Bernie, Ciaran, Colm and Sean settled into daily life with CF. Colm’s regime was strict and included three physio ‘thumper’ sessions a day. With careful planning and help from their neighbour, Anne, the young parents were able to share the load, taking one session a day each, and combined with medication, Colm began to thrive.

6

Spectrum / Spring 2023

Introducing CFI's New Chairperson: Bernie Priestley Bernie and Sean’s family grew and Eoin was welcomed into the family in 1988. Having CF in the family, Eoin had to be tested as Ciaran was when Colm was diagnosed. Bernie and Sean’s family life was typical but for one child with CF and one without. So when the call came to let Bernie know that ‘Eoin’s test result was normal’, Bernie had to ask did that mean ‘normal CF’ or ‘normal no CF?’ “When Ciaran got the all clear, the relief was immense but when Eoin’s results came back clear, I had mixed emotion. CF was ‘normal’ to us. I didn’t know what way to feel about it actually.”

Bernie explains.

In 1991, Aidan arrived and went through the same process as Ciaran, Colm and Eoin before him and his results were also clear. When both parents carry the CF gene, there is 1 in 4 chance that their child will be born with cystic fibrosis, and in many ways, Bernie and Sean, had the typical one in four family.

After the family’s first foreign family holiday in 1997, Bernie discovered she was pregnant for a fifth time. She remembers that although Colm worked very hard to keep himself well and was generally well, Bernie and Sean decided to have an amniocentesis test in the Rotunda to determine the growing baby’s genetics. They couple also discussed the possibility of a termination if the test came back positive.

“They brought us in and were told ‘there’s your baby’s heartbeat’ or ‘there’s your baby’s hand’ and I remember walking out and saying to Sean, I can’t even consider a termination now,” Bernie explained.

The results of the amniocentesis came back when Bernie was 16 weeks pregnant.

“For the second time in our lives we heard the words ‘I’m sorry your baby has cystic fibrosis’ but we also heard ‘it’s a little girl.’ Bearing in mind we had four boys and we were dumb founded and in shock. The best news for us turned into the worst news. We were having a little girl, but we were having a little girl with cystic fibrosis.”

There was twelve years between Colm and Triona, and interventions and treatments had greatly improved in

www.cfireland.ie

7

that time, but from the moment Triona was born, she fought struggle after struggle, challenge after challenge. Despite this, Triona was an absolute delight and lit up the family home and Bernie remembered the truth in Bridie’s advice given when Colm was diagnosed.

“For us as a family, we are the same genetics, we are the same DNA. We had two children with the condition and it progressed differently in them. And there are so many families that could say that,” Bernie explains.

The boys adored Triona and she adored them, and she had a different relationship with each member of the family. Her Dad was her best friend and her Mam was her carer. Triona never hid her CF in primary school and her friends accepted her for who she was. It was when she started secondary school that she began talking to others about CF, starting with her class and friends, and her advocacy journey began.

Triona’s goal became to educate people about CF. It frustrated her that although CF was one of the most common genetic conditions in Ireland, not enough people knew about it. Social media was just emerging and Triona connected with a global group called ‘CF Sisters’. She began making videos of her life and showing the world how every day she fought, but every day she won.

It was the tight knit circle of friends that stood strong for Triona as the illness progressed. When she went into hospital, they visited. When she couldn’t walk, they carried her. And as their lives moved on, they kept her in their circle, never leaving her behind.

As Triona became progressively sicker, she began to prepare her friends and her family for the day that she would no longer be there. But her dream to spread awareness of CF never diminished. When news broke that Triona’s outlook was not good and she would not be coming home from the latest hospital stay, all her friends gathered together and began to tweet the hashtag #songforTri to capture the attention of the famous singer, Ed Sheeran, online.

Back in ICU, the family kept Triona updated with her friends efforts and at two o’clock in the morning, Ed Sheeran’s tweet came in and the family were connected with the singer. On the 1st April 2014 at 12.15 he rang Colm and began to sing Triona’s favourite song Little Bird and when he finished, Triona was gone. When Bernie took Triona’s mask off, she saw Triona was smiling.

“I have no doubt, Triona heard everything. We didn’t tell Ed at that time as we didn’t feel it was fair on him but afterward, Colm let him know,” Bernie explains.

“The media picked up on it and Triona’s wish came true. Thanks to Ed Sheeran, thanks to her friends and thanks

8

Spectrum / Spring 2023

to #songforTri, the whole world heard about CF. For us as a family it was devastating but it was what we felt she deserved and it was an amazing thing for fifteen year olds to have achieved.”

When Bernie was a young mum, she didn’t have the time or headspace to become more involved in CFI. Her involvement was mainly volunteer based at events or fundraising but a volunteer at heart, Bernie was always involved in several organisations helping out where and when she could.

Shortly after Triona began her advocacy, Bernie was connected with a young Mum whose child had just been diagnosed and together with a third parent, they set up the Dublin West branch. That marked the beginning of Bernie’s deeper involvement in the CFI. In memory of Triona, Bernie was then involved in setting up Malin2Mizen, a long standing fundraiser for CFI and sometime later, she was approached to sit on the Staff and Finance committee and eventually the Board of CFI. Now in the 60th year of CFI, Bernie has just taken up the position of Chairperson of CFI. With the advancements and progress of CF Care in recent years, Bernie has a clear vision for the future.

“My hopes for the present and the future of CFI involves the young members. I would love to see a Youth Advisory Panel being set up. I think the voice of the young people is hugely important and I’d love to see young parents getting involved,” Bernie says.

“The future is very different than the future that I thought I was facing all those years ago. Colm is still here. He is married with two children.

So none of us know what is coming. The only guarantees in life we have is an entry and exit.

“In between we should do the best we can because it isn’t a dress rehearsal. So if you can give a bit of time to supporting your local branch or bringing your skillset to the table, then please do. Because the union is only as strong as its members. And the members are the CF Community.”

www.cfireland.ie

9

SPOTLIGHT

10

Spectrum / Spring 2023

A life less limits:

by Brendan Lonergan

www.cfireland.ie

11

SPOTLIGHT

12

Spectrum / Spring 2023

A life less limits:

by Brendan Lonergan

www.cfireland.ie

13

SPOTLIGHT

14

Spectrum / Spring 2023

A life less limits:

by Brendan Lonergan

www.cfireland.ie

15

SPOTLIGHT

16

Spectrum / Spring 2023

A life less limits:

by Brendan Lonergan

www.cfireland.ie

17

TEAM FEATURE

T

What generation are you?

his year we celebrate 60 years of our CF Association now CF Ireland - what an achievement. As I reflected on this, I realised that our association is actually a 'Baby Boomer' - and it got me thinking. With the advent of CFTR modulator therapies for those of us with CF lucky enough to have drugs that work for our mutations, it means we are now transitioning to a new era; from CF from classical CF to mitigated CF, and with it new generations may be emerging.

CFTR modulators can’t reverse lung changes from years of infections and inflammation - but they can hopefully stabilise our CF. With progress in CFTR modulator treatments, children born with CF now (where these treatments exist to treat their mutation specific CF) may experience a childhood like others without CF. Should we call them CF Generation Progress???

We do not forget those with CF with no existing CFTR modulator treatments and I do not make light of their CF. They are not a forgotten generation and research continues to find treatments for all with CF. So if CFI is a Baby Boomer, and children with CF born now are a different generation altogether, as a 50 something year old PWCF, what generation am I? This is not something I have ever really paid any attention to. I assumed I was maybe a baby boomer too, although I identify more with the “Jilted Generation” for any Prodigy fans among us! Going by generation definitions though, which each span 15 years, it turns out I’m actually Gen X (1965 - 1981). But this got me thinking what are the generations exactly and where does CF fit within them?

With children born 2023 and 2024 being among the last of the Gen Alpha (2010 - 2024), and children born from 2025 to 2039 making up Gen Beta, we are progressing through the Greek alphabet of generations.

18

Spectrum / Spring 2023

What Generation are you?

by Rory Tallon, CF Advocate

Generations by Definition So there are seven living generations apparently following the Lost Generation (the generation in early adulthood during World War I). I’ll save you the Google effort - as I know you are just bursting with curiosity.

There’s maybe some debate over the naming, the exact years and some of these cohorts refer specifically to American population - but let’s apply these generations to us here in Ireland too for simplicity - this is what I found: who grew up during WWI and many fought in G1: The Greatest Generation Americans World War II. This generation followed the Lost generation 1901-1927 1883-1900

G2: The Silent Generation 1928 -1945 G3: The Babyboomers 1946 - 1964 G4: Generation X 1965-1980 G5: Generation Y 1981-1996 G6: Generation Z 1997 - 2012 G7: Generation Alpha 2012- 2024

www.cfireland.ie

Aka, the Traditionalist gneeration in Canada, they knuckled down, got things done. 'Silent' comes possibly from keeping quiet and knowing not to speak out during the McCarthy era in the US, and growing up knowing to be seen but not heard in UK society.

Whey heeey (!!) their parents went mad after World War II! Named as Baby Boomer due to population expansion and post war prosperity. Many of these were in their prime during the hippy era.

Born to become early technologically savvy as the first generation to grow up with personal computers. Hard to define this generation, hence the X as an unknown entity - this generation witnessed great political and societal liberalisation and an explosion of music genres, film and gaming.

Aka the Transition Generation living through the electronics, digital information, communications and technology revolution. Known also as the Millennials as they were growing up approaching the Millennium. Anyone remember Y2K?!

Aka the Zoomers, the population grew/are growing up surrounded by technology, exposure to screen time and their education incorporates multimedia technology. This generation is growing up to inherit the earth as threatened by global warming.

The current generation, the first generation to be born entirely in the 21st Century and born in era of falling global fertility rates and threat of global warming. They lived through Covid19 as young children and are swamped by technology with excessive exposure to screen time.

19

After that?

AFter that comes Generation Beta, those born from 2025 to 2039, then Generation Gamma, born 2040 to 2054, and so it continues.

So where does CF fit in all these generations?

Babies born with CF pre-Baby Boomers went undiagnosed and most likely sadly died in very early childhood.

Meanwhile The Greatest Generation and

The Silent Generation would have been the parents

and the grandparents of children diagnosed with CF from 1950’s onwards when CF was first discovered. These would be the pioneers who established our CF association and were our first doctors and allied health professionals to treat CF. It’s so important to remember these pioneers and to fully appreciate their efforts to pave the way to the modern CF centres, CF research, CF medications and CF healthcare professionals that we have now. We owe them our existence and it is fitting to reflect on these generations in this our 60th Anniversary of CF Ireland as our CF association.

CF Baby Boomers may have been diagnosed with CF but many were never diagnosed and even

with diagnosis most died in their first or second decade of life. Baby Boomer parents of children with CF have been very active and proactive in establishing services and facilities for their CF children and remain so still today many now grandparents or even great-grandparents of children with CF. These parents would have taken over the mantle from previous generations in running our CF Association.

CF Generation X went through gradual improvements

to CF treatments from low fat diets to high fat high calorie diets with pancreatic enzyme supplementation, development of specialist CF treatment centres and inclusion of CF medications on Long Term Illness Scheme – the “green card” in Ireland along with developments of antibiotics, improved diagnosis and awareness of CF in specialist CF centres and maternity hospitals.

CF Generation Y were born during a rapid biological revolution of knowledge advancement in

genetics of CF with the CF gene discovered in 1989 and further advances in CF treatments like Pulmozyme

20

Spectrum / Spring 2023

and nebulised antibiotics such as Tobi. There was also great progress in establishing CF units around Ireland.

This generation also embraced the electronic and technological advances seen throughout society with mobile phones, internet and computing in addition to seeing a transition to technology solutions in medicine with improvements in medical diagnostics and imaging.

CF Generation Z were born during the promise of new

hope on the horizon while first CFTR modulator active compounds were discovered and initiated through early laboratory and subsequent clinical trials.

This generation of CF contributed as clinical trial subjects for the CFTR drugs now on the market. Babies born at the end of CF Gen Z were the first screened for CF at birth in Ireland as part of the neonatal blood spot screening programme since July 2011.

CF Generation Alpha The first generation to be entirely screened from birth for CF in Ireland.

The current generation of CF babies are born since 2012 and have been born into the dawn of CFTR modulator treatments with exciting promise and hope of additional or improved corrective CF treatments such as mRNA therapies and possible gene editing or gene therapies. Survival estimates for CF Gen Alpha extend to 6th decade of life and possibly beyond.

www.cfireland.ie

21

The CFRI Using Data for Good

Key messages from the CFRI patient survey 2021 Launch of the 2021 annual report We are delighted to have recently launched our 2021 annual report. The report summarises data collected by the registry in 2021. You can find the report and a summary infographic at https://cfri.ie/annual-reports/ and we will be posting some key insights from the report on our social media pages over the next few weeks – follow us at @CFRegistryIE to see our updates.

ECFS Winter Meeting in Brussels Some members of the CFRI team attended the ECFS Winter Meeting in Brussels at the end of January 2023. We attended a number of meetings about the ECFS patient registry and connected with international colleagues. CFRI attended the steering group of the ECFS patient registry, at which Dr Laura Kirwan presented the CFRI experience of undergoing an ECFS patient registry data quality audit.

The Future of Patient Registries in Ireland event On 2nd February, CFRI, alongside Health Research Charities Ireland (HRCI), hosted an event in the Ashling Hotel in Dublin. We co-hosted this event to celebrate over 20 years of collecting and reporting on CF data in Ireland. The event focussed more generally on discussing the future of patient registries in Ireland and its aim was to bring together people interested in patient registries to highlight the challenges and opportunities for registries now and in the future.

CFRI co-ordinated the event and were co-hosts alongside HRCI. It was a case of all hands on deck and the whole CFRI team were there on the day doing everything to make the event run smoothly. It was also a chance for us to showcase our work as a registry.

Who was there & what was discussed?

The event had a wide range of speakers and attracted a very diverse audience representing many stakeholders interested in patient registries. Overall, around 100 people attended the event. The speakers/panel members were: Godfrey Fletcher (CFRI), Caroline Heffernan (CF Ireland), Killian Hurley (Interstitial Lung Disease registry), Lara Cutlar (National & International Skin Registry Solutions),

22

Spectrum / Spring 2023

Laura Kirwan (CFRI), Maria Ryan (HIQA), Sarah Gibney (Department of Health), Avril Kennan (HRCI), Barbara Foley (HIQA), Philip Watt (CF Ireland), Ed McKone (SVUH), and Derrick Mitchel (IPPOSI).

Presentations focussed on giving the audience an insight into how registries work through 3 different examples of registries (Interstitial Lung Disease, dermatology registries, and CFRI) as well as information on the policy and standards context in which registries operate. There was also a panel discussion which addressed what the future of registries in Ireland looked like. The panel discussed the questions of what supports registries need in the future, what actions need to be taken to support registries, and ultimately realising the value of patient registries for patients in Ireland and internationally.

CF Ireland played a huge role in the event. Alongside Godfrey Fletcher, Caroline Heffernan opened the event speaking about the importance of patient registries to patients and importantly highlighting how patient registries need to be able to adapt to changing disease landscapes in order to collect the most relevant information for patients. Philip Watt sat on the panel and commented on the future of patient registries in Ireland emphasising the valuable role of registries from the policy and service delivery perspectives, in particular noting the role played by registry data in the publication of the 2005 Pollock Report.

Philip also spoke to the importance of working collaboratively across the system including with patients and clinicians to ensure patient registries are fit-for-purpose.

Why was this event important & what’s going to happen now?

Ultimately, this event was intended to be a starting point to ensure that patient registries are given the support they need for the future. It also provided an opportunity to share learning across the registry and health information landscape to those working in or with registries, those wanting to set-up registries, and those who use registry data.

Not only was this event a timely gathering of people interested in patient registries due to the new health information bill in Ireland as well as the ongoing policy discussions around e-health, but it was a reminder that registries need to have their voice heard at this level of decision-making to ensure that we can sustain registry operations in Ireland in the longterm.

www.cfireland.ie

23

In short, we will be continuing the conversations started at the event by engaging with stakeholders going forward to ensure that registry needs can be met and their importance realised.

Want to get involved?

Consent Participating in the registry has indirect benefits in terms of having better information that can help improve planning and delivery of care and services that a person with CF receives. The more people that participate, the better the quality of the information that can come out of the registry. If you would like to participate in the registry, please contact your CF Centre of the CF Registry at info@cfri.ie. Participation is entirely voluntary. You are free to revoke your consent and withdraw from the registry at any time.

Social media We would love if you could follow us on social media. We post updates on our work and on the exciting projects we are working on. Use the QR code to access all our social media channels.

Get in touch Do you have any questions about participating in the registry or about the data we collect? Get in touch with us at info@cfri.ie.

Update on Jen Balfe’s research study Recruitment will be commencing in the coming weeks for a study exploring the experiences of people with CF and their healthcare teams as they navigate fertility and pregnancy journeys in Cystic Fibrosis. This study is funded by the Irish Research Council and co-funded by Cystic Fibrosis Ireland. Jen Balfe is a PhD scholar in the RCSI, a mam of two and a person with CF. If you would like to find out more about the study and perhaps take part in an interview on your experiences as a person with CF who has experienced a fertility/pregnancy journey or as a healthcare professional with experience of providing care to pwCF during the pre-conception to post-partum period, please contact jenbalfe21@rcsi.ie.

This study has a strong Public and Patient Involvement (PPI) focus, with a panel of pwCF supporting the research. If you would be interested in joining the PPI panel as an advisor for this project please contact CF Ireland at stecklenborg@cfireland.ie or Jen Balfe at jenbalfe21@rcsi.ie to register your interest.

24

Spectrum / Spring 2023

Research Update Steps Ahead: Optimising physical activity in adults with cystic fibrosis The amount of CF research that is achieved in Ireland is increasing year by year. Ireland is lucky to have such an enthusiastic cohort of clinicians working across the country who share CFI’s ambition to improve the lives of people with CF through research. A great example of this is the ‘Steps Ahead’ project, which was recently published in the Journal of Cystic Fibrosis in November 2022.

“Steps Ahead: Optimising physical activity in adults with cystic fibrosis: A pilot randomised trial using wearable technology, goal setting and text message feedback” is a research project that was run out of University Hospital Limerick (UHL) over the last four years with the support of CFI and TLC4CF (Tipperary, Limerick & Clare for Cystic Fibrosis). The project was led by UHL CF physiotherapist Máire Curran, under the supervision of Dr Roisin Cahalan and Dr Audrey Tierney from the University of Limerick (UL). The project looked at the use of “wearable fitness technology” (aka Fitbit watches) plus targeted text messaging feedback between the physiotherapists and CF patients to see if this had an impact on physical activity levels.

UHL CF Physiotherapist Máire Curran

CF Ireland were delighted to sit down with Máire to hear about her research project and what she learnt from ‘Steps Ahead’.

Why did you want to do a research project in the first place?

I started working in the area of adult Cystic Fibrosis (CF) in UHL back in 2017. As a physiotherapist, I am naturally interested in physical activity & exercise, and I have always been personally interested in research.

As I started to look at some of the evidence and research articles around increasing physical activity levels in CF, I quickly realised that there was very limited research conducted in this area.

Fitness wearables had become quite popular in the years previous and I thought it would be interesting and novel to investigate if using these devices could increase physical activity and health outcomes for people with CF. My project originally started as a small research study but as I read more into the area, as well as my wonderful experience of working with adults with CF in UHL, I was inspired to take it further and do a larger project, with the supervision and assistance from the research experts in UL and do my PhD in the process.

Why is your research important?

The aim of this research was to determine if wearing a Fitbit watch along with text message feedback and goal setting (the ‘intervention’) could increase physical activity levels more than using a Fitbit watch on its own. We also wanted to see out if this intervention improved other health outcomes such as fitness levels

www.cfireland.ie

25

and lung function.

We all know that regular exercise improves lung function, fitness levels and quality of life for people with CF. Despite these benefits, the effectiveness of fitness wearable watches (such as Fitbits) to increase physical activity in people with CF is still largely unknown. Fitness wearables have become very popular in recent years as a means of encouraging and tracking activity levels. Prior to this research in UHL, the use of Fitbits had not been investigated in CF.

What did you do?

We ran this study in UHL and recruited patients from the adult CF clinics there. All participants were tested at baseline (exercise test, lung function and quality of life questionnaires) and they were randomly allocated to one of two groups. Group 1 received a Fitbit Charge 2 and participants set step count goals with their physiotherapist. Step count goals were individualised based on each person with CF. The participants were sent a text message each week for 12 weeks to encourage increased physical activity levels.

Group 2 received the Fitbit only. Both groups were re-tested at 12 weeks and at 24 weeks.

What did you find?

Our results were really exciting. We found that step count increased significantly for Group 1. Step count increased by 28% over 12 weeks and this was maintained at the 24 week follow up.

Furthermore, Group 1 also demonstrated improved fitness levels at 12 weeks.

Group 2 on the other hand actually reduced their step count by 1% during the first 12 weeks. Importantly there was no significant effect on lung function, sleep, well-being, or quality of life for either group.

What does this mean and what’s next?

Our research shows the possibility that if CF centres use wearable fitness watches with their patients and send text message feedback based on that data, then we could see an increase in daily step count and fitness levels in people with CF. This was evident at 12 and 24 weeks in our study participants.

This type of intervention may be a future tool to encourage regular physical activity in people with CF. However, it is important to note that longer-term studies are required examine this further.

Why is it important that we do innovative research like this in Ireland?

As Ireland has the highest incidence of CF in the world I think it’s important that we keep Ireland at the forefront of advancing CF care. We also need to consider new innovative ways of CF management, to try to determine optimum strategies for adhering to exercise/medication and ultimately strive to achieve the best outcomes for our patients. .

26

Spectrum / Spring 2023

Conference Report 2023 The Cystic Fibrosis Ireland (CFI) Annual conference was held this year on 31st March – 1st April in Dunboyne, County Meath. The conference brought together some of the brightest minds in the field of cystic fibrosis (CF) to exchange ideas, share insights, and discuss the latest trends and innovations. Over the course of two days, attendees participated in a variety of keynote speeches, panel discussions, and interactive workshops both in-person and virtually. The hybrid conference covered a range of topics, from the challenges with treatment adherence to CF microbiology considerations for independent living. With a diverse array of speakers from around the world, the conference was a truly unique and valuable opportunity to reflect, learn, and connect.

A reflection of the history of CFI and CF care It is widely recognised that there has been more progress in cystic fibrosis (CF) care in Ireland over the past decade than any other decade since the formation of Cystic Fibrosis Ireland in 1963. For many people living with CF, CF has become a more manageable disease with better therapies and improved CF centres across Ireland. Philip Watt, CFI CEO, used his opening address for Saturday’s conference to celebrate the progress that has been made in the past 60 years by the organisation, but to also recognise and remember the challenging history of CF care in Ireland and those who fought for better care and services. A specially commissioned documentary film was shown in honour of CFI’s 60-year anniversary, which truly emphasised the journey of the CFI association within the context of challenging CF care in Ireland. This short documentary film was developed by CFI and is be available to view on the CFI website and through social media channels.

It’s well worth a watch.

The conference keynote speaker, Professor Charles Gallagher, took the stage to further outline the progress made in CF clinical care in Ireland. Prof Gallagher walked the audience through the history of CF care, from the virtually non-existent CF care pre-2005, to what he referred to as “the Pollock years” of 2005 – 2016, which were the outcome of the Pollock Report commissioned by CFI in 2005. This monumental era saw a shift in the understanding of what CF clinical care should look like within the HSE, including the introduction of dedicated CF consultants within a small number of specialist CF centres across Ireland, and the start of the newborn CF screening programme. This period also saw a crescendo of public campaigning for CF in Ireland. Prof Gallagher highlighted the 2007 “Sick Waiting” campaign lead by CFI (then known as CFAI) which demonstrated the unacceptable wait-times for admissions, safe ward facilities and CF staffing levels at the

www.cfireland.ie

27

time.

In 2016 a significant milestone was reached with the creation of the National Clinical Programme for Cystic Fibrosis (NCPCF). Prof Gallagher, who chairs the NCPCF, explained the intention of the NCPCF is to enlist the broad expertise of CF care across Ireland from all disciplines to make real and effective changes in CF care in partnership with the HSE. Most crucially, according to Prof Gallagher, is to ‘future-proof’ the guidance delivered by the NCPCF in recognition that the CF population will continue to grow and evolve in years to come. 2019 saw the launch of the NCPCF Model of Care which outlined the minimum standards for dedicated CF centres, including infection control procedures and staffing ratio. Most recently, the Post- Lung Transplant & CF Model of Care was launch by the NCPCF in 2022, another milestone for CF care in Ireland.

Prof Gallagher ended his keynote address by highlighting the future priorities for the NCPCF. There was a real emphasis on the crucial importance of CF psychology services and his desire to both ensure a CF psychologist is available in every CF centre and to develop a National CF Psychology Service. Resourcing of post-Transplant care at the Mater, Cork and Galway hospitals is also a key priority. Prof Gallagher listed the key evolving issues for CF care in Ireland, including CF and ageing, fertility and pregnancy, evolving complications of CF, CF diagnosis, recruiting and retaining expert clinical staff in CF centres, and the continual international development of new CF drugs and how these may impact CF care in Ireland.

Real World Data for Kaftrio in Adults and Children Professor Ed McKone, consultant Respiratory Physician, joined the conference to share an outline of recent international research and an update on the real world data for Kaftrio use in children and adults with CF (as part of the RECOVER study). Prof McKone guided the audience through the research outcomes from various Kaftrio clinical studies.

Prof McKone used his speech to emphasise the difference in what he refers to as “un-real world data”, which comes from clinical trial environments, compared to important “real world data” which comes from the everyday use of drugs like Kaftrio and studies like the RECOVER study. According to Prof McKone, ‘Real world’ studies are important because they can show the value and validity of drug treatments and the real-world experience of patients.

Clinical trials, on the other hand, only examine a drug in a very clinical, strict environment where certain populations may be excluded.

Real world data is collected either through real world clinical trials, or through national CF registries.

Prof McKone broke down the local experience of Kaftrio use in Ireland by examining the RECOVER Study.

28

Spectrum / Spring 2023

RECOVER is ongoing at the time of discussion, and involves 181 adult and paediatric CF patients across Ireland and the UK. Preliminary data presented shows improvements in sweat chloride levels, FEV1, LCI (a measure of lung function) and CF Quality of Life measures.

Interestingly, the RECOVER study has shown a dramatic decrease in both IV and oral antibiotic use which has not been thoroughly examined in previous research and this in itself may be a marker of improved quality of life and reduced admissions.

RECOVER also provided evidence of reduced adherence to inhaled medications and the implications of this needs to be researched further.

The impact of Kaftrio of the progression of lung disease was discussed in the presentation. The ideal goal for modulator therapy will be to have a drug that can both increase FEV1 and simultaneously slow the rate of decline of lung function. The impact of Kaftrio on both markers has been reviewed in a 2022 study in the Journal of Cystic Fibrosis (Lee et al.) where the authors evaluated the impact of Kaftrio on the rate of lung function decline over time by comparing with a matched group of PWCF from the US CF Registry. This study showed that Kaftrio is the first CFTR modulator therapy shown to stop lung function decline over an extended time period.

All of the clinical trials which examined Kaftrio in the “un-real world” setting, according to Prof McKone, focused on lung disease outcomes only. But what about the other conditions in CF? Three studies have so far shown a reduction in abdominal symptoms in patients who receive Kaftrio, such as nausea/vomiting, fullness, bloating, reflux and pain. An additional three studies have also shown a reduction in sinus disease for patients taking Kaftrio. There has been less research so far into CF-related diabetes and the impact of Kaftrio, however a small study of 33 people in Switzerland has shown an improvement in Oral Glucose Tolerance Testing (OGTT) in patients who are prescribed Kaftrio. Questions remain about whether these improvements are a direct impact of Kaftrio itself or secondary impact of the improved lung function observed in patients taking Kaftrio.

Clinical trials and real-world studies have identified side effects of Kaftrio. The most common side effects are rash and abnormal liver function tests (LFTs), and in some cases Kaftrio has been ceased due to uncontrollable LFTs. Further side effects that have come from the real world data studies include mental health changes, testicular pain and cysts, abdominal pain and constipation.

The use of Kaftrio in post-lung transplant patients was also discussed, although the jury is still out on this decision. There is the need to examine the risks and benefits for a post-lung transplant patient to begin Kaftrio, considering the benefits of Kaftrio are mostly shown in lung disease markers. A risk/benefit analysis is also required in these cases due to the risk of side effects and drug interactions within an already complicated transplant drug regimen.

Pregnancy and the impact of Kaftrio has been examined in one US-based survey study. It is believed that there may be substantial benefits for the mother to remain on Kaftrio, including preserving lung function,

www.cfireland.ie

29

reduced exacerbations and improved nutrition status during pregnancy, however more research is required to confirm the safety and efficacy of Kaftrio in this population. The MAYFLOWER study is currently monitoring the safety of Kaftrio use for both mothers and infants up to two-years post-birth in the US.

Treatment Adherence, Mental Health and Quality of Life International guest speaker, Dr Alexandra Quittner, Clinical Psychologist and Epidemiologist from Joe DiMaggio Children’s Hospital, Florida, presented a passionate and knowledgable talk on her work in self-management behaviours and quality of life for people with CF. Dr Quittner has over 30 years’ experience working in the field of CF. She strongly feels the new era of modulator therapies is bringing new opportunities for improving adherence and managing mental health.

The rise of Kaftrio and other modulator therapies may bring increased opportunity for personalised prescribing and a reduction in treatment burden for PWCF. A recurrent theme of both Dr Quittners and Prof McKone’s presentations was the reduced adherence to inhaled medications in patients who are prescribed Kaftrio. Factors that influence adherence to any medication are varied but may include age and developmental stage, knowledge of treatments, skill level, a patient’s mental health status and the overall treatment burden - the latter of which is well recognised to be high in CF. Facilitating treatment adherence requires a multifaceted approach, taking into account an individual’s knowledge of disease and management, skill level, treatment plan, barriers to adherence and problem solving ability. Among the key tips for improving adherence is problemsolving. A person’s problem solving skills can be developed as a tool for adherence, with problemsolving strategies becoming part of psychological interventions provided during clinic delivered within cognitive behavioural therapy (CBT). Dr Quittner regularly facilitates problem-solving sessions with her patients, parents or partners in her clinic and speaks passionately about their positive impact.

Mental health is particularly emphasised as being a crucial component of a person with CF’s overall health and wellbeing. Dr Quittner’s research shows that rates of depression and anxiety are 2-3 times higher for people living with CF compared to people without CF. Additionally, a person with CF who reports one single elevated depression score is thought to have a doubling of mortality risk within 5 years, possibly due to patients depressed mood leading to reduced treatment adherence and increased risk of lung function decline (further research is ongoing).

Dr Quittner described this as being a “call to action” for European and US researchers torebalance and refocus on the impact of mental health and adherence on physical wellbeing in PWCF.

30

Spectrum / Spring 2023

As a result, in 2015 the Cystic Fibrosis Foundation and ECFS collaborately developed a consensus statement and clinical care guidelines for preventing, screening and treating anxiety and depression in individuals with CF and their parent caregivers.

The future direction of mental health in CF has been identified through patient, carer and healthcare professional surveys. Dr Quittner identifies the top research priorities are depression, anxiety, reducing treatment burden and the effects of modulators on physical and mental health. The reported mental health side effects of Kaftrio vary and are not well reported, but include new onset of anxiety, depression, brain fog, memory, negative body image and identity crisis with the rapid benefits of Kaftrio. Despite this long list, increased awareness of mental health side effects by clinicians, patients and carers is growing, and the importance of mental wellbeing overall is being prioritised.

CF Registry of Ireland celebrate 20 years The Cystic Fibrosis Registry of Ireland (CFRI) has celebrated its 21st year anniversary in 2023. The registry was developed as a response to the lack of centrally collected data on CF patients, treatments and health outcomes in Ireland and was originally aligned with, and located within, the CFAI. To celebrate this important milestone, Laura Kirwan from the CFRI gave us an overview of the CFRI and its achievements.

The CFRI was established in 2001. According to Dr Kirwan, at that time the initial major challenge for the CFRI team was receiving consent and registration from as many CF patients as possible. The first CFRI report was published in 2002, and within 8 years almost 90% of all CF patients in Ireland had consented to be part of the registry. More recently, the introduction of GDPR legislation in 2018 has been a challenge for CFRI staff as they have been required to re-consent and re-register all CF patients in Ireland to comply with legislation.

This re-consenting process can be completed with your CF clinical teams, so please ensure you have given updated permission to be part of the CFRI. The CFRI is a centralised healthcare data registry. A team of data collectors visit each of the 13 CF centres across Ireland to collect patient data from electronic and paper medical records. The data collectors only collect anonymous data from patients who have consented to this process, which again emphasises the crucial importance to ensure both children and adults with CF have consented themselves, or via their parents, to be part of this important registry. Each time a CF patient visits their CF centre (including telehealth), this data is recorded within the CF registry.

www.cfireland.ie

31

By 2022, 90.3% of CF patients were registered with the CFRI, which represents 1,328 adults and children with CF. According to Dr Kirwan, the 10% who are unaccounted for include newborns and patients who have chosen not to participate in the registry.

By reviewing CF data for over 20 years, we can clearly see the impact that improved healthcare treatments, technology and policy have had in Ireland. A stark example of this is the introduction of the National Newborn Screening Programme for CF in 2011. Dr Kirwan presented a dramatic graph which showed the immediate drop in median age at diagnosis for children with CF after 2011.

By rigorously collecting this data, alongside other data collection points, the CFRI were able to partner with researchers at UCD on the Irish Comparative Outcomes Study (ICOS) and examine the clinical impact that early diagnosis has on the health of PWCF. The ICOS research showed that following the introduction of the newborn screening programme in 2011 there is evidence of improved growth in children with CF, reduced hospitalisation for acute exacerbations, and delayed pseudomonas aeruginosa acquisition up to age 3.

Data collected by CFRI on several other topics was highlighted at the annual conference, with an emphasis

32

Spectrum / Spring 2023

on the real-world experience of PWCF in Ireland. We have seen an increase in lung function over the last 20 years in PWCF in Ireland, with a sharp increase noted in Adult PWCF from 2020 onwards likely as a combined impact of the introduction of Kaftrio in Ireland alongside the result of increased isolation during the COVID-19 Pandemic. The impact of Ivakaftor was presented as part of an Irish longitudinal trend study which reviewed CFRI patient data before and after commencing Ivakaftor for 3 years, which included hospitalisation, IVs and oral antibiotic use.

A 20-year summary of the Median age of death, Cause of death, and median predicted age of survival data was also presented and explained. The median predicted survival age is now 51.8 years compared to 38.3 years in the 2010-2014 period. Crucially, CFRI data shows something interesting regarding the changing cause of death for PWCF – for the first time in 20 years the most common cause of death in PWCF was recorded as ‘non-CF related’ in the last year, rather than respiratory causes, transplant related, or liver disease/liver failure. This is a remarkable reflection on improvements in care and treatments for people with CF over the past two decades.

The 20 year landscape of complications of CF can also be examined using CFRI data. Dr Kirwan demonstrated in her presentation that while there has been a significant decline in chronic infections in CF (such as pseudomonas aeruginosa and staphylococcus aureus), there has been a noted increase in certain CF complications such as diabetes, liver disease and liver cirrhosis. This shows the changing landscape of CF care over the past 20 years and gives us an indication of future directions and priorities for CF care and research.

An important part of the CFRI annual data collection is to complete an annual census of patients. This is a data request that is sent to all CF centres once a year, so as to create a complete picture of data coverage of the CF registry. This also provides an excellent snapshot of CF patients each year. The 2022 data is presented below.

www.cfireland.ie

33

The future of CF data collection is important and ever-changing, especially in the era of modulatior therapy.

There is a desire from CFRI to improve their collection of data on pregnancy, discontinuation of long-term medications and chronic infection trends. This will be a focus over the next few years. Registry-based studies will be increasing to ensure CFRI are using the rich data that is collected to its full potential. There is also a clear need to collect Patient Reported Outcome Measurements (PROMs) and for the registry to share data back with patients and communities. CFRI have trialled a data collection portal, named CF View, which is a smartphone app that allows CF patients to view their clinic data in real-time and also provide information directly to CFRI. This is an exciting, ongoing piece of work for CFRI.

60th Anniversary Celebration of CF Ireland This year’s conference was also a significant event as 2023 marks the 60th Anniversary of Cystic Fibrosis Ireland. CFI used their conference platform to celebrate the progress that has been made in the past 60 years, to acknowledge the challenging history of CF care in Ireland and importantly, to remember those who are no longer with us. The Saturday evening gala dinner commenced with a poignant lighting ceremony of six candles, representing PWCF (lit by Gordan Ryan), Parents of PWCF (lit by Phil Dunphy), Partners of PWCF (lit by Fran Heffernan), CF researchers (lit by Dr Laura Kirwan), CFI Board Members (lit by Mary McCarroll) and CFI staff (lit by Samantha Byrne). A one minute silence was observed, accompanied by CFI supporter and ‘2024 Highest Harp’ challenge participant Siobhan Brady.

34

Spectrum / Spring 2023

FUNDRAISING: Challenges and Events For further details on any of our fundraising events, visit our website www.cfireland.ie, contact the CFI Fundraising Team on (01) 496 2433 or email fundraising@cfireland.ie.

April 14th: 65 Roses Day - Donate or buy a purple rose!

65 Roses Day is our National Fundraising Day for Cystic Fibrosis Ireland. This is our largest fundraising event of the year and targeted to raise €350,000 to help fund essential services needed now more than ever by people with Cystic Fibrosis in Ireland.

Please support Cystic Fibrosis Ireland on 65 Roses Day, Friday 14th April by donating online at www.65roses.ie or purchasing a purple rose from our volunteers in Dunnes Stores or other participating outlets nationwide.

Take on a 65 Roses Challenge!

You could also take on a 65 Roses challenge to help support people with Cystic Fibrosis on 65 Roses Day! A 65 Roses Challenge can be any challenge with a 65 theme. It could be a 6.5k walk or run, 65,000 steps or a 65km cycle etc.

All you have to do is click onto our 65 Roses Day website www.65roses.ie and this will guide you through how to set up your Just Giving Page to start your challenge.

Enjoy your challenge and don’t forget to share it on your Social Media to get maximum support #65Roses2023.

For your fundraising pack so that you can complete your challenge in your purple Cystic Fibrosis Ireland shirt, please email Sarah at svalliere@cfireland.ie Pictured here is Amy Keller and friends taking on one of her park runs in her “65 Park Runs For 65 Roses" challenge!

www.cfireland.ie

35

April 30th: Duleek 10k Cystic Fibrosis Ireland and all of the team at the Duleek 10k are delighted to announce the 11th Annual Duleek 10k will take place on Sunday 30th April. Register now at www.cfireland.ie.

This Duleek 10k is aimed at people of all fitness levels and you can walk or run your 10k. The event was set up to remember and celebrate the lives of cousins Cathy O'Brien and Kelley Noone and all friends and families touched

by Cystic Fibrosis.

For further details please visit our website or email Sarah at svalliere@cfireland.ie or Ann Noone at duleekcf10@gmail.com.

April 23rd: TCS London Marathon Registration is now closed for the TCS London Marathon 2023. Good luck to all our participants in the event and we look forward to getting some really nice photos from the day.

If you are still looking to participate in a similar event, we have a number of exciting events that you can participate in instead or register your interest now for the TCS London Marathon, 2024.

1.

Head2Head Walk – Sunday, 10th September

2.

Irish Life Dublin Marathon – Sunday, 29th October

3.

TCS New York City Marathon – Sunday, 5th November

You can now register your interest in participating in the above events on our website – www.cfireland.ie. For further details call us on 01 4962433 or email fundraising@cfireland.ie.

36

Spectrum / Spring 2023

June 4th: VHI Women’s Mini Marathon / One in 1000 The Vhi Women’s Mini Marathon takes place on Sunday, 4th June. Why not become One in 1000 taking part in this very special event for Cystic Fibrosis Ireland? This is the largest outdoor women’s event in Europe and every year over the past 10 years, 1,000 women have participated in their purple Cystic Fibrosis Ireland shirts representing Cystic Fibrosis Ireland.

Join us on the June bank holiday weekend in our base at the D2 Harcourt hotel where you can relax before the start of the 10k and drop off your belongings, get your photo taken and collect some goodies to get you through the 10k. When you return afterwards, we will have the music ready and food / refreshments so you can relax with your friends SIGN UP NOW at www.cfireland.ie for this amazing event for the CF Community in Ireland and we will send you out your One in 1000 Shirt, bandana and details for the day. Don’t forget to also register with the Vhi to get your number for the 10K. For further details, please contact us on 01 4962433 or email fundraising@cfireland.ie.

September 1st to 7th: Paris2Nice Registration is now closed for the Paris2Nice Cycle 2023. Good luck to all our participants in the event and we look forward to getting some really nice photos from the cycle.

If you are still interested in an exciting cycle adventure you can now register your interest in participating in the Malin2Mizen Cycle4CF from Thursday 14th to Sunday 17th September on our website – www.cfireland.ie.

Alternatively you can register your interest now to be added to the waiting list for Paris2Nice 2024.

For further details call us on 01 4962433 or email fundraising@cfireland.ie.

September 10th: Head2Head Walk After a hugely successful 10th Annual Head2Head Walk last year, we are delighted to announce that the Head2Head Walk will return on Sunday, 10th September.

Register now to take part at www.cfireland.ie for this amazing walk along the stunning Dublin coastline.

For further details call us on 01 4962433 or email fundraising@ cfireland .

www.cfireland.ie

37

September 14th to 17th: Malin2Mizen Cycle4CF Our annual Malin2Mizen Cycle4CF takes place from Thursday 14th to Sunday 17th of September. This is a life changing experience where you get to take on an adventure you will never forget, meet new friends that will stay with you forever, improve your health and fitness and all the time raising monies for a great cause.

A limited number of places remaining with registration closing on May 31st. If you would like to join us, sign up now at www.cfireland.ie as places will be allocated on a first come, first served basis.

For any queries, please email fundraising@cfireland. ie or phone us at (01) 4962433.

September 19th to 26th: CFI International Walk We are delighted to confirm that the International Walk returns in 2023 with an amazing walk in Malta & Gozo.

Malta and its sister island Gozo are part of an archipelago in the Mediterranean and boast a year-round sunny climate. Journey across 7,000 years of history, culture and natural beauty. Walk through magical landscapes, country, and coastal walks perfect for walkers of all ages and levels.

Walkers will get to enjoy diverse culinary experiences, from the traditional plate of eclectic Mediterranean food to varied International menus in local and firstclass restaurants and wine bars.

And also experience the warm and friendly Maltese culture in a safe and tranquil environment that few other destinations can rival.

If you are interested in joining our International Walk in Malta & Gozo, please contact fundraising@ cfireland.ie or phone 01 496 2433 as soon as possible.

There are a limited number of spaces still available.

This is a really well organised event with limited places available which are highly sought after.

For further details call us on 01 4962433 or email fundraising@cfireland.ie.

38

Spectrum / Spring 2023

October 29th: Irish Life Dublin Marathon

We are delighted to confirm that the Irish Life Dublin Marathon returns on Sunday, 29th October.

If you have secured a place in the Marathon and would like to be part of the Cystic Fibrosis Ireland Team taking part, register your interest NOW on our website at www.cfireland.ie. We will contact you and provide you with your Cystic Fibrosis Ireland shirt for the day once and fundraising pack!

For further information please email fundraising@cfireland or call (01) 496 2433.

December 8th: Christmas Jumper Day 4 CF Friday, 8th December if Christmas Jumper Day 4 CF. We are encouraging as many workplaces and schools as possible to wear your Christmas Jumper to school or work on the day in support of Cystic Fibrosis Ireland.

Interested in getting involved? All you need to do is…

1.

Ask your work colleagues or fellow teachers and pupils to support Christmas Jumper Day 4 CF on Friday, 9th December. Encourage everyone to wear their Christmas Jumper to work in return for a €5 donation to support people with Cystic Fibrosis in Ireland!

2.

Register your interest to participate at www.cfireland.ie and we will contact you and organise to send you a Christmas Jumper Day 4 CF pack - balloons, bah humbug badges, posters, sponsorship cards and collection buckets as required.

3.

Bah Humbug donation! If colleague does not wear a Christmas Jumper, ask them to make a donation of €10 in return for a ‘Bah Humbug’ badge! It’s all for a great cause!

4.

Ask you company / school if they are willing to match fund the monies raised!

5.

On the day, make sure to get everyone together in your place of work or on zoom and take loads of photos!

www.cfireland.ie

39

Post them to our Facebook page (facebook.com/CysticFibrosisIreland), share them using the hashtag on Twitter or Instagram #ChristmasJumperDay4CF and tag us (@cf_ireland), or simply email them to us at fundraising@cfireland.ie so that we can share your fun with everyone else.

All Year Round: International Treks

For the really adventurous looking for the challenge of a lifetime in 2023, International Treks are available across the year to climb Kilimanjaro, Machu Picchu and Everest Base Camp.

Register your interest to take part on our website www. cfireland.ie or email fundraising@cfireland.ie for more information.

Skydives If a skydive are on your bucket list, then 2023 is your chance to tick it off, while supporting a great cause. This challenge is one you will never forget and all you have to do is register your interest on our website at www.cfireland.ie and we will contact you to confirm next steps.

Now is the time, take on the challenge and you will remember 2023 for all the right reasons. You can contact our office on 01 496 2433 or email fundraising@cfireland.ie for more information.

40

Spectrum / Spring 2023

FUNDRAISING: Thank You It is not possible to list all of the Fundraising Events that have been organised since our last edition of Spectrum, but we would like to take this opportunity to thank everyone that has volunteered and raised money on behalf of people with Cystic Fibrosis. Every cent raised helps provide a better quality of life for people with Cystic Fibrosis and please keep up the great work. Here is a short summary of some of the remarkable efforts of our Volunteers:

Fundraising Awards for 2022 Huge congratulations to the winners of our fundraising awards for 2022 which were presented by Fergal Smyth, Fundraising Manager for CFI at our Annual Conference in the Dunboyne Castle Hotel. There were so many people who have done outstanding work fundraising for CFI in 2022 and we would like to thank each and everyone one of you as CFI rely on funds raised by our wonderful volunteers in order for us to be able to continue to support people with CF in Ireland.

Our first award was presented to Gemma Dardis for her amazing work with the #AlfiesArmy Team supporting our One in 1000 campaign for the past eight years at the Vhi Women’s Mini Marathon. They are being joined each year by an ever-growing team of women who work tirelessly to raise funds and spread awareness for CFI. A huge thank you to Gemma and everyone else involved in #AlfiesArmy for making One in 1000 a great celebration of the CF community at the Vhi Women’s Mini Marathon each year and we look forward to seeing you all again on Sunday, 4th June.

Pictured below from L to R: Gemma Dardis, Fergal Smyth, Bernie Priestley – Chairperson, CFI

The second award was presented to Ken Doonan and John White, cycle leaders in our Malin2Mizen Cycle4CF 2022 and over the past number of years. Being a cycle leader is an all year round commitment with Ken

www.cfireland.ie

41

and John helping to train the cyclists in all kinds of weather in advance of this mammoth cycle. They are also a key part of the safety team during the cycle, helping to ensure all cyclists get safely from Malin Head to Mizen Head. A huge thank you to Ken and John and everyone else involve in the Malin2Mizen Cycle4CF led by Bernie Priestley and Caroline Heffernan. We have a limited number of places left in our Malin2Mizen Cycle4CF 2023 which takes place from Thursday 14th to Sunday 17th September with registration at www. cfireland.ie closing on 31st May. Pictured below from L to R: Fergal Smyth, John White, Ken Doonan & Bernie Priestley

Annual Raffle A HUGE thank you to everyone who puchased tickets for our annual raffle which raised as amazing €11,701 to help us continue to support people with CF in Ireland with the draw taking place at our Annual Conference last night. Congratulations to all the lucky prize winners!

Annual Raffle 2023 Winners 1st

2 Nights' B&B for 2 at The Gleneagle Hotel, Killarney, Co. Kerry - Hugh Leonard

2nd

2 Nights' B&B for 2 at The Great Northern Hotel, Bundoran, Co. Donegal - Louise Lynam Fitbit Sense Smartwatch - Siobhan Mawe

3rd

€250 Sunway Voucher - Ronan Balfe

5th

Garmin Venu SQ Music Edition Smartwatch - Aisling Walsh

6th

Lenovo Tab M10 HD - Caroline Donaghy

7th

Lenovo Tab M10 HD - Sarah Nolan

8th

1 Nights' B&B for 2 at The Hibernian Hotel, Mallow, Co. Cork - Helen O'Keeffe

9th

10th

1 Nights' B&B for 2 at The Maldron Hotel, Oranmore, Co. Galway - Joyce O’Driscoll

11th

1 Nights' B&B and evening meal for 2 at The Dunboyne Castle Hotel, Co. Meath Shamir Raval 1 Nights' B&B and evening meal for 2 at The Dunboyne Castle Hotel, Co. Meath - Shamir Raval Stationery Hamper - Vera Scully

4th

42

Spectrum / Spring 2023

We would also like to acknowledge our generous sponsors for all of the fantastic raffle prizes; • • • • • • • • •

The Gleneagle Hotel, Killarney The Great Northern Hotel, Bundoran Sunway Adlantic.ie EVAD IT Solutions The Hibernian Hotel, Mallow The Dunboyne Castle Hotel The Maldron Hotel Oranmore, Galway Terenure Office Supplies

Christmas Jumper Day 4 CF We want to extend a huge thank you to all the schools, companies, and everyone who participated in this years Christmas Jumper Day 4 CF! Over €40,500 raised so far with monies still coming in. This will be a great help towards funding supports and services for people for with Cystic Fibrosis in 2023!

We really look forward to seeing all your Christmas Jumpers again this Christmas!

Hospital Saturday Fund Cystic Fibrosis Ireland would like to extend a huge thank you to The Hospital Saturday Fund who presented a cheque for €4,000 to Fergal Smyth (CFI). The cheque presentation took place at a special reception hosted by the Lord Mayor of Dublin - Caroline Conroy in The Mansion House, where 28 Irish charities received support from The Hospital Saturday Fund.

Paul Jackson, Chief Executive of The Hospital Saturday Fund, stated that they were “honoured to support the efforts of such deserving charities and help in some way towards the exceptional, tireless work that they do in making such a huge and positive difference to people’s lives”.

Pictured above st the cheque presentation are Paul Jackson, Fergal Smyth and Caroline Conroy.

www.cfireland.ie

43

Head2Head Walk 2022 A huge thank you to the Head2Head Walk Committee (Mary McCarroll, Jem & Lorraine Downes, Glenn McDonnell, Denise O’Brien, and Luke Kennedy), all of the volunteers and of course all of the walkers who participated in and fundraised for our 10th Annual Head2Head Walk in 2022.

We were delighted to welcome members of the Head2Head Walk Committee to CF House who presented us with a cheque for €48,054.51 raised from the walk in 2022.

Registration is now open for the 2023 Head2Head Walk which takes place on Sunday, 10th September.

Pictured – Fergal Smyth (CFI), Jem Downes, Lorraine Downes, Sarah Vallerie (CFI), Mary McCarroll, Glenn McDonnell.

Golf Classic at the Killeen Castle Golf Club 2022 A huge thank you to Brian Delaney and all at Castlethorn who helped to organise a Golf Classic at the Killeen Castle Golf Club in 2022 raising €10,000.

They are so delighted to be able to help support people with cystic fibrosis in Ireland including Tomás and Seán Delaney age 6 & 4 who are both PWCF who attend Temple Street Childrens Hospital. Brian informs us that he is running the golf event again this year on Friday, 23rd June at Killeen Castle Golf Club.

Apple Cork A huge thank you to the staff in Apple Cork who volunteered to help make up display boxes for 65 Roses Day in April. Also a huge thank you to David O'Shea, his family, friends and colleagues in Apple for organising a wonderful darts event in January, raising €4,800 for Cystic Fibrosis Ireland. Well done to all involved in a great nights entertainment and fundraising event!

44

Spectrum / Spring 2023

Joyce Country Truck Run

A huge thank you to everyone involved in The Joyce Country Truck Run which raised an amazing €8,000. Pictured below accepting the cheque on behalf of Cystic Fibrosis Ireland is Daniel Diskin from Galway.

Loreto College, Swords A huge thank you to the pupils and staff at Loreto College, Swords for raising €1,200 as part of their Christmas Jumper Day 4 CF event. We were delighted to be invited to attend a cheque presentation in the school!

St Attracta’s Secondary School, Tubbercurry A huge thank you to everyone at St Attracta’s Secondary School, Tubbercurry including students and faculty along with Apleona staff members who organised a fundraiser for Cystic Fibrosis Ireland in memory of Barry Gaffney who passed away recently. Included in photo are some of the Transition Year students, their Coordinator Martina Roddy, Apleona colleagues Norma Gray and Liam Gannon making a presentation to Rose Gaffney, Barry’s devoted mother. Also present Mary Dillon and Iris Murphy representing the local CFI branch.

Free Range Kids Preschool, Limerick A huge thank you to everyone who took part in The Superhero & Princesses day at Free Range Kids preschool, Limerick for CFI. The total raised was €1014 from all our wonderful families & staff!

www.cfireland.ie

45

CFI Branch Secretary Contact Details PWCF (online) Saoirse Perry saoirseperi@gmail.com

Cavan Martina Plunkett mplunkett1000@yahoo.ie

Sligo Iris Murphy carrowcashel@eircom.net Mayo Billy O'Toole billyotoole1@gmail.com

Drogheda Frances McDonagh fmurray67@ymail.com Dundalk Rachel Mellon rachelmell.92@gmail.com Meath info@cfireland.ie

Galway Kayleigh McGuire kayleighmcguire0@gmail.com

Dublin West Rebecca Horgan cfdublinwest@gmail.com

TLC4CF Caitriona Hayes caitrionahayes@icloud.com

Eastern Branch Mary McCarroll marymccarroll1@hotmail.com

Clare TLC4CF@cfireland.ie

Carlow Leigh Bolger bolgerleigh@yahoo.ie

Limerick Niamh Harrington niamhh75@gmail.com

Cork Aisling O'Neill aislingquill@gmail.com

Tipperary Mandy Quigley mandyquigley26@gmail.com

Wexford Suzanne Doyle suedoyle74@gmail.com Waterford Ruth Carberry rcarberry02@gmail.com

www.cfireland.ie 40 46

Spectrum 2022 Spectrum/ Summer / Spring 2023