Spectrum Spring 2018

SPECTRUM Spring / 2018

COMING UP ROSES!

RTÉ’s Keelin Shanley launches 65 Roses Day 2018 with Liam & Áine Hannafin.

www.cfireland.ie

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CONTENTS Latest News | 1-2 * CFI launches major new report

Hospital Hub |3 * Mater of Fact

Spotlight | 4-7 * Surviving the Storm * Online Support

Member Services| 8-11

* Noticeboard

Kids Corner | 12 Youth Reach | 13 Conference Gallery | 14-15

Research | 16 - 18

* News update * Why does Ireland have the highest incidence of CF in the world? * Research Ezine

Fundraising |19 - 29 * Challenges and Events * Thank You DISCLAIMER: The views of contributors, when expressed in this publication, do not necessarily reflect the position or policy of Cystic Fibrosis Ireland.

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CEO’s Message Welcome to our ‘new-look’ Spectrum and many thanks for the revamp to Samantha Byrne and Nuala Mc Auley of CFI. This issue features news and spotlight features and future issues will have a stronger focus on research and policy concerns.

Continue to let us know what you want to see in future issues

Philip Watt Chief Executive

We also include a focus on our annual CFI conference that was held in Dundalk, County Louth 6-8 April. Many thanks to the Dundalk branch for hosting us.

We were delighted to welcome Simon Harris TD Minister for Health to our annual conference in Dundalk and his announcement that ‘soft opt-out’ organ donation will be introduced, possibly in a stand-alone Bill, in May 2018. He also promised to work with us on the commitment in the Programme for Government on the in-patient unit in Beaumont Hospital (20 rooms) that was also promised and which is badly needed We once again call on all our supporters to help us in the immense task of fundraising. In addition to 65 Roses Day on 13 April, we need plenty of support for fundraising throughout the year. The monies raised will support our advocacy work; the many support grants we give to or members; for research and for improved CF services throughout the country including the 5 key additional clinical staff we fund in CF hospitals.

Finally we hope that you will read the Independent Living and CF Report which sends out such a strong message of hope and shows the progress over the past two decades. We cannot of course gloss over the challenges that remain and these will focus in our next strategic plan.

Philip Watt, CEO, CFI Sam Byrne, Editor, Spectrum

Spectrum / Spring 2018

LATEST NEWS: CFI launches major new report A new and ground-breaking report on ‘Independent Living’ from Cystic Fibrosis Ireland (CFI) highlights major improvements in the quality and the length of lives of people with cystic fibrosis (CF) over two decades (1998-2017). The report shows that the sustained investment in better CF services and medications in recent years has made a very significant difference to those with CF in Ireland, but there is also much more that can and should be done.

At the core of the Report ‘Independent Living and Cystic Fibrosis’ are two surveys undertaken by CFI in 1998 and 2017.

Around one quarter of the adult CF population participated both surveys. Corroborative data was also drawn from other sources such as the CF Registry of Ireland.

Dr. Martin Wildman, Patricia Duffy Barber, Chairperson of CFI and the Independent Living Sub-Group and Professor Gerry Mc Elvaney at the CFI Annual Conference 2018

Amongst the key points noted in the report are: •

In 1998 adults with CF were not expected to be in any employment. In 2017, a remarkable 54% of those surveyed were in full-time or part-time work.

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In 1998, 74% of respondents lived with their parents in their parents’ house. By 2017 this figure had reduced to 43%.

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In 1998 adults with CF were not expected to have children of their own. In 2017 (thanks to better health and the introduction of IVF) 26% of the adult CF population aged 21 or over surveyed had children

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In 1998 only 8% of those surveyed were married, compared with 26% in 2017

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The percentage of those obtaining third-level qualifications has increased from 38% in 1998 to 49% in 2017

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The median age of death in Ireland was only 17 years of age in 1998. By 2015 this had increased to 30 years for the first time.

Philip Watt CEO of CFI stated: “The findings are in many ways remarkable and send out a strong message of hope. The main message is that adults with CF are living much more fulfilled and independent lives in 2017 compared with 1998. They are much more likely to have a job, have better education attainment, to have their own home and to even have a family of their own.

“The improvements in length and quality of life are closely linked to improvement in services such as dedicated CF hospital centres which we have fought for; improved rates of double lung transplantation and more recently access to new ground-breaking drugs such as Kalydeco and Orkambi. The report shows that with sustained investment over a number of years people with a chronic and fatal disease such as CF can lead longer and better lives.”

www.cfireland.ie

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“However key gaps remain. The Government promised a dedicated CF in patient unit in Beaumont Hospital commencing in 2017. There has been no progress on this commitment in the Programme for Government and understaffing in major CF centres remains an issue.”

The ‘Independent Living and Cystic Fibrosis’ Report covers a number of key issues: Relationships and Housing In 1998, 74% of respondents lived with their parents in their parents’ house. By 2017 this figure had reduced to 43%.

In 1998, 87% of adults surveyed were ‘single’ compared with 39% in 2017.

In 1998 adults with CF were not expected to have children of their own. In 2017 thanks to better health and access to IVF, 26% of the adult CF population aged 21 or over have children.

Employment In 1998 adults with CF were not expected to be in employment because life expectancy was much shorter.

However, by 2017, a remarkable 54% of those surveyed were in full-time or part-time work. These jobs range from journalism to the caring professions, from IT to accountancy.

However, major challenges remain. 14% of all respondents in the 2017 survey remain ‘too ill to work’.

Many others said the potential loss of a medical card or sudden loss of Disability Allowance when entering a job remains a major deterrent to employment and independent living as their health comes first.

There is also evidence of improved understanding by employers about the potential contribution of people with CF in the workplace. In 2017, 92% of people with CF surveyed were prepared to let their employer know they had CF compared with only 44% in 1998. However, the report also shows those in lower paid employment can still be treated callously by employers.

Education and Mobility: In 2017, people with CF are still missing out on school and college because of their illness. However, the percentage of those obtaining third-level qualifications has increased from 38% in 1998 to 49% in 2017.

In 1998, 82% of those surveyed were reliant on public transport. By 2017 only 19% of journeys made by people with CF were on public transport. This reflects a much greater access to/ownership of cars and an increased wariness of the risk of infections in confined places, such as buses.

Foreign travel: For leisure is increasingly the norm for people with CF, through travel insurance can be difficult to access at times.

Starting a family: A review of branches will be completed early in 2018 and will seek to enhance our branch structure.

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Spectrum / Spring 2018

HOSPITAL HUB Mater of Fact T

he Mater Lung Transplant Unit welcomed 40 representatives from Cystic Fibrosis (CF) centres from across the country, and 10 in-house staff members to a study day entitled “Lung Transplant for People with CF-The Journey”.

Susan Talbot Towell (CNS Lung Transplant/Cystic Fibrosis)with the help of Sara Winward (ANP Lung Transplant) and Iain Lawrie (CNS Lung Transplant) organised the day to share information and experiences with CF professionals. The main aim of the day was to share knowledge about lung transplant for PWCF to empower CF professionals. The take home message was to encourage patient education to begin earlier in referring centres to aid realistic expectations about lung transplantation and its outcomes.

Speakers on the day included several members of the Mater Lung transplant team, including a surgeon, dietician, social worker, pharmacist, co-ordinator, psychologist and psychiatrist. The programme covered all aspects of transplant care, from the assessment process to optimisation of health prior to listing, through surgery and post-transplant care regarding diet, lifestyle, medicines and psychosocial considerations. The Mater Programme, led by Professor Jim Egan, is now the third busiest in Europe and the team showcased the excellent work being done there.

The feedback from the day was extremely positive, with many participants expressing an interest to further their learning about the transplant process.

Our team recognise the importance for transition from referring centres to the transplant clinic to become more streamline. Susan plans to form a group of link multi-disciplinary team around the country to continue education and has begun to design a pathway designed consolidate the learning and support transition. This day was the first of its kind in Ireland with future plans to run annually.

Susan and the lung transplant team would like to thank CFI for all of their support with this development.

Left to right: Katie O’Brien (Clinical Psychologist) Patricia Ging (Transplant pharmacist) Susan Talbot Towell (CNS Transplant/Cystic Fibrosis) Sara Winward (ANP Lung Transplant) Sandra Murphy (Senior dietican) Maria Love (Medical Social Worker)

www.cfireland.ie

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SPOTLIGHT: Surving the Storm T

he biggest snow fall since 1982. The beast from the east!

Memories being made building igloos, giant snowmen and enjoying sleigh rides.

Schools closed and a red warning with a no travel ban.

Jamie was in St. Michael’s ward in Crumlin Children’s Hospital for it all.

We enjoyed watching all the activities on TV and looking at our family and friends pictures. All the while, trying our upmost to make the best of a bad satiation and make our own memories.

On Wednesday February 14th, Jamie got a call from Crumlin, they had a bed for him. He had done 4 oral antibiosis since Christmas and his cough was not clearing so we knew it was time for IV’s. Jamie was not happy about going in this time as this was his third admission since September and he just had enough at that stage which is understandable, he is only 10. Again we both packed up and the family was separated.

When we walked onto Michael’s ward we were met with warm smiles from all the nurses it is so nice and settled Jamie straight away. A big fuss was made about him and they all wanted to know how he was and us too. Every person that walked through his door made a big impact on his stay.

Jamie started his IV’s on the Wednesday and was doing well.

On his 3rd set, Lorraine the play specialist called down, as she did every morning, to check on him. She was asking if he liked star wars Jamie jumped up to show her he was actually wearing a star wars t-shirt, they had a good chat about it and she went off. Two hours later she came back and opened the door and told Jamie to get ready for a surprise, she arrived in with Chewbacca, it was like all his dreams came true. The excitement he was showing him all his star wars Lego and talking about the films it really made his day.

That was not the only surprise visit Jamie had, the day after the Irish rugby team won over wales they popped in to the hospital and Jamie got to meet them and get their autographs. He had a great time chatting to them about the game and telling them about his CF. There was a great buzz around the ward that morning, everyone was so excited to have them there.

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Spectrum / Spring 2018

Jamie was all excited to tell all his friends who he met and was so nice for him to walk away after 17 days with his great memories of them.

On his second week in hospital, the weather started to change, Storm Emma was coming! The weather was too bad for anyone to travel and visit us. I was so lucky and met 4 amazing CF mammies and a daddy that kept me sane, in truth we kept each other going as none of us had seen our family in days.

Jamie was in isolation in suite 3 which has a big window looking out at a fence and a hedge. Over the few days of snow we had a great laugh watching our fence disappear, with no clue as to what was going on beyond the hedge, we were in our own bubble.

The staff in Crumlin over the days of the storm were just amazing. Many of the nurses arrived to work 18 hours before their shift started the next day when the red alert was on and slept on makeshift beds and staying as long as required. Others got snowed in and some just chose to stay and help. Everything was done with a smile still on their face. The nurses not only looked the patients, but the parents too. During the red alert the hospital provided food for all parents, even the ladies serving us the food were asking us if we were ok.

It was hard as I had not seen my other kids in a week, but I had to focus on the reason I was there, to get my other child better. I also has to remember that many of the nurses, doctors, cleaning and catering staff that were there had not seen their kids too.

On the Friday myself and the other CF mammies said we would brave the snow and see what was going on beyond the hedge and venture to the shop for a few bits for ourselves, the children and to pick up some chocolates to say thank you to the nurses.

Nicky a nurse on Michaels stopped me in the hall and gave me her snow boots, hat gloves and snow trousers going out.

Like her, others really went above and beyond their job over them days, more worried about us then themselves. I know I speak for the other parents when I say we will be forever grateful to them all for everything they done for us. We were in good hands with them all.

Jamie was discharged on the Friday, but we were unable to leave until Saturday. I came away from Crumlin with so much respect for each and every person up there. Heroes the lot of them.

Sadie Kerrigan CF Mammy

www.cfireland.ie

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SPOTLIGHT: Online Support A

s every person with CF knows cross infection is a huge risk and can inhibit personal interaction with fellow members of the CF Community.

Peer support is as important to self management of a condition as any other aspect of care.

To this length a group of CF’ers came together to start a an online group for people with CF to act as an outlet, a support and a meeting point for the community. While we recognise the CF Community is made up of nonPWCFs (parents, siblings, partners, children) it was important for patients to have a place where they could speak openly.

The PWCF group on Facebook is a closed group which was established in 2008 The group is monitored by administrators Antony Dempsey and Brendan Lonergan, but content on the group is not filtered. Members discuss their support frustrations, fears and formulas for maintaining good health as well as discussing topics that affect people with CF such as travel, education, mortgage and insurance sharing their own experiences and tips.

If you would like to join the group you can do so by searching PWCF Group Ireland, The only criteria is that you are a person with CF residing in Ireland and over 16 years of age There is no obligation on individuals to take part in discussions or raise issues, but interaction is encouraged.

While you may not feel you can add anything, you will be surprised as to what you can bring and indeed take away from the group. We, as patients, are the best advocates for ourselves. While isolation is advised by medical profesionals as best practice to maintain good health, peer support and communication is the recommendation by patients. So get involved, get connected, get in touch.

You can contact Antony Dempsey if you have any questions on 086-8230140 Remember you are not alone.

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Spectrum / Spring 2018

T

he CF Mammies Facebook page was born at a CF conference in Kilashee castle, Kildare in 2012 where four Mums met each other for the first time. We sat and spoke about all our experiences with our children (some more experienced than others in the CF journey) but brought great comfort to know we were not alone. When it was time to leave on the Sunday afternoon we decided to set up a facebook group with each other that we could continue to chat and offer advice & support when needed.

The page admins are Marie Duffy, Maire Gallagher, Marion O’Donohoe and Louise Byrne. The page has grown from those 4 Mums to 150 members. The page is devoted to Mums living in Ireland who have 1 or more children with Cystic Fibrosis. The page is a secret group so people cannot find it in the search settings – to join you must either be recommended by another CF mammy that is already a member of the group and then the admin can approve her or contact one of the administrators direct.

The Mums find it great for getting advice and support from people who know exactly how you are feeling. Some have found other Mums who live close to one another now meet for coffees every now and again and once a year we have a CF Mammies weekend away. It’s a very close community and anyone that is a member finds great comfort in it.

CF Mammies Maire Gallagher, Rachel Mellon & Linda Whitmarsh at the CFI Annual Conference in Dundalk

Here are a couple of words from some of the members that use the page: “I love being part of the CF Mammies group. There is nothing quite like a mums gut feeling, and with CF being so complex yet individual being able to bounce your thoughts and worries off other Mums is so important for me. Sometimes I'm the novice and other times I'm the expert in our various chats but what we can give is knowledge and support to each other whether your a new parent or you an old hand. It's very much all for one and one for all.”

Ruth “ I joined the CF Mammies Facebook group about 2 years after our diagnosis and I remember instantly feeling a sense of belonging, a weight lifting off my shoulders. Although I had family and friends who were there to support me, I never realised how isolated I felt before joining the group, it was only in retrospect that I recognised it. The relief of being part of a community where everyone understood what I was going through was incredible. I never had to pretend everything was fine or explain why it wasn’t, these women just got it. The mammies are always there to lend an ear or to answer any question, no matter how big or small. They are the friends I wish I never had to meet, but the friends I couldn’t survive without. They remind me of a pride lionesses, fierce protectors who champion each other and our children. My pride.”

Chris

By Marie Duffy, CF Mammy

www.cfireland.ie

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MEMBER SERVICES Noticeboard Did you know?.....

Free Travel Pass “Make Work Pay” Initiative – anybody who, from the 6th April 2017, moved from a long term disability payment to a job, will be eligible for a 5 Year Extension to their Free Travel entitlement, providing all other qualifying conditions for the free travel scheme are satisfied. http://www.welfare.ie/en/Pages/780_FreeTravel.aspx Property tax exemptions and reductions If you have had your home adapted for a person with a disability, you may be entitled to a Local Property Tax reduction/exemption. Please find details on https://www.revenue.ie/en/property/documents/lpt/ guidelines-reliefs-disabled-incapacitated.pdf Domiciliary Care Allowance and Medical Card Any child for whom Domiciliary Care Allowance is paid is also entitled to a full Medical Card, regardless of the means of the family. You can register for the medical card using the following link: https://www.sspcrs. ie/portal/free-gp-care-dca-web/pub/home Book Buds scheme to bring the wri�en word to CF kids in hospital The distribution of books to the paediatric units around the country has commenced. Thanks to Lorraine Levis, Dubray Books, O’Brien Press and Big Bang Comics for their conbribution to this scheme.

CFI Medical ID Card We have improved the look and feel of the card. Please apply for yours at www.cfireland.ie or email sbyrne@cfireland.ie for an application form.

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Spectrum / Spring 2018

Art Exhibition for CF Artists Monday May 21st to Friday 1st June 2018 CB1 Gallery, O’Connell Street Limerick 11am to 5pm daily Reasons for the Exhibition This is the first art exhibition to feature exclusively, artists with Cystic Fibrosis in Ireland. The exhibition is about connecting people with CF together with art, to expand our narrow horizons with something that is limitless. It is freedom of expression, to show our communities who we are, what we can do and where we can go. It’s about giving adult artists with CF who might have confidence issues, the opportunity to display their art or craft. It is also about inspiring the younger generation to be creative, branch out and find their own place in the world, whatever that might be.

Exhibition theme Exploring the personal experience of life with CF and life beyond CF. The event organiser is Sara Cross, a person with CF aged 34yrs and living in Limerick City. Sara is a craftivist – a talented crafter and knitter, who is passionate about supporting people to see their own potential through creative means. As a self-taught artist, she makes her living by taking on commissions for hand-crafted pieces. Having founded ‘Knitters 4 life’ as a voluntary knitters circle aimed at developing skills, confidence and friendship, Sara also teaches crafting.

An American by birth, Sara is a mother of one delightful daughter named Emma.

The event is kindly supported by TLC4CF and CF Ireland.

An invitation has been extended to all adult artists who have CF in Ireland, to submit two pieces of art or craft that have a personal relevance. We hope to feature up to 8 adult artists, who are either professional artists or talented amateurs. Art is defined as any medium with which the artist works. That might include for example: paintings, photography, sculpture, ceramics, crafts, knitting/crochet, quilting, fashion, wood work, multi-media etc. Some size limitations may apply and a narrative is optional.

Details of the pieces must be emailed to Sara Cross by end March and all pieces must be delivered and removed by the artist within a week of the exhibition. Should artists wish to sell any of their work at the exhibition, no commission will be charged and the artists will keep the proceeds.

Children and young artists with CF will be invited to submit their own work of art for a special display. Perhaps the piece will be about your experience of having CF or maybe it will be about what your hopes and dreams for the future might be. A poster seeking entries from young people will be circulated by TLC4CF, CF Ireland and social media by end February.

Viewings by PWCF can be accommodated on a scheduled basis by contacting Sara.

www.cfireland.ie

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An official opening will take place on Monday 21st May from 4pm to 8pm. The speakers will likely include the event organiser (PWCF), a representative from CF Ireland and an invited guest. Local musicians will be asked to play and media coverage will be sought. Limerick’s Live 95FM have indicated that they may do an interview and the event organiser featured on a recent ‘Nationwide’ programme.

The organiser would like the event and the reaction of the public to be recorded so that PWCF who cannot attend may view the proceedings online. A free or inexpensive way of doing this is being investigated. CF Ireland will be invited to feature the event on social media and film via Facebook live.

A coffee and canapes reception will be offered as the venue prohibits alcohol. Family members, local CF branches, medical staff and other guests from the cultural world will be invited to attend the official opening. Space limitations may limit attendance numbers at the official opening addresses but the venue will remain open that evening until 8pm.

Public opening times The exhibition will be open to the public on weekdays for two weeks, from Tuesday May 22nd to Friday 1st June, from 11am to 5pm daily. Entry is free to all. There will be a donation bucket for CF Ireland or TLC4CF. Funding of the event This event is not likely to incur significant costs. TLC4CF and CF Ireland have offered to sponsor the event.

TLC4CF will cover the donation to the Gallery for hosting the event (€200), along with the printing costs.

CF Ireland will sponsor the reception for the official opening and will provide support with social media.

Contact details for further information If you, your child or young adult has CF and you are interested in participating in this exhibition, please contact Sara or Erin for further information. We will need to have your contact details, the type of piece you hope to submit and approximate size. Scheduled visits to the exhibition are possible for PWCF and their families. Priority times may be given to people travelling long distances.

Sara Cross 085-1533968 Email: Knitters4lifegroup@ gmail.com Erin Sugrue 087-1954213 Email: tlc4cf@gmail.com Press invitations and a press release are available now,

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Spectrum / Spring 2018

Benat’s Broadcast!

CFI would like to acknowledge Benat Broderick for his advocacy and fundraising work for Cystc Fibrosis Ireland which has included TV interviews, radio interviews, an online blog, meeting with Ministers, giving talks on CF to his school and organising 65 Roses Day collections!

A great Ambassador for CF, we look forward to seeing what the future holds for this talented teenager.

Follow Benat on Twitter: @BenatBroderick

Photo Finish for Race Night Fundraiser

Star CF‛er A huge thank you to Darcy Grainger, aged 10, who donated €625 raised from a collection in memory of her grandad Christy Grainger.

Darcy has also been supporting CFI by raising awareness for 65 Roses Day!

www.cfireland.ie

CFI would like to say a massive ‘thank you’ to Siobhán Murray who, with the help of her parents Joe and Sue, raised a whopping €10,000 for Cystic Fibrosis Ireland organising a Race Night in Swords.

In addition the event raised funds for Temple Street Hospital and TBC in the UK. Siobhan underwent a double lung transplant in the UK in August 2017 and is back at school preparing for her Junior Cert.

Well done Siobhán!

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KIDS CORNER Name: _________________________________________________ Can you solve the CF wordsearch below?

Words can be found across and down!

CFI

COUGH

CYSTIC FIBROSIS

DOCTOR

EXERCISE

HOSPITAL

LUNGS

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NEBULISER

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Did You Know?

Find the following words in the puzzle.

65 Roses is how most children Words are hidden and learn. how to say Cystic Fibrosis!

Here are some other ways to learn words associated with your CF

COUGH HOSPITAL Physiotherapy (FIZZ-E--O-TEAR-UP-E) CYSTICFIBROSIS LUNGS DOCTOR MUCUS Orkambi (OR-CALM-BE) EXERCISE NEBULISER

NURSE SIXTYFIVEROSES

Kalydeco (CAL-I-DECK-OH)

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Spectrum / Spring 2018

YOUTH REACH S

elf-management is key in CF Care. CFI encourage members to become involved in their own care from a young age as this helps to inform and empower patients to have the knowledge skills and confidence to manage their own health and healthcare, so that they can adhere to treatment regimes, make healthy lifestyle choices and make informed and personally relevant decisions about their treatment and care It’s time to put your CF knowledge to the test. Can you complete our CF crossword below?

Across Down 1) In people with CF the body produces an unusually thick, sticky (5) 3) In addition to medication, this is the other key part of CF treatment (8) 4) Measures how much air a person can exhale during a forced breath _ _ _ 1 (3) 6) Cystic Fibrosis Ireland, in short, is a patient organisation to support the CF Community in Ireland (3) 7) Expand IVs, a therapy that delivers treatment directly into a vein. (12) 8) Self Management is key in the treatment of CF. Following advice and instructions from medical professionals is known as (9) 9) Children first learn to say Cystic Fibrosis by counting 65 of these (5) 14) In summary, people with cystic fibrosis (4)

www.cfireland.ie

2) Tablets to be taken with each meal. (7) 5) CF is an inherited condition. When something is inherited it is also referred to as this. (7) 10) When a person’s treatment moves from a children’s hospital to an adult CF unit. This process is known as (10) 11) Although CF can be managed at home, sometimes patients may need to be admitted here (8) 12) Medication to help manage CF can be inhaled, the device used for this is a (9) 13) People with CF should not interact with other people with CF, especially when unwell, as there is a high risk of (5, 9) 15) Although there is no cure for CF, studies are ongoing to improve treatments and understand the condition more. This is known as (8) 16) Cystic Fibrosis is a condition that mainly affects these organs (5)

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CFI Conference

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Spectrum / Spring 2018

2018 Gallery

www.cfireland.ie

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RESEARCH News Update On the 1st and 2nd February 2018 I attended the 18th National Cystic Fibrosis (CF) Clinical Meeting in Killarney, Co. Kerry. On day one there were two symposiums: One on nutrition in adolescents with CF and one on fungal infections affecting the lungs in CF. On the second and longer day, leading CF clinicians and researchers from around the world each outlined their work to address a particular issue in CF care.

Speakers included: Dr. Lisa Opipari-Arrigan, Clinical Psychology, Cincinnati Children’s Hospital, US Dr. Mandy Bryon, Clinical Psychology Great Ormond Street Hospital, London, UK Dr. Niamh O’Sullivan, Consultant Microbiologist at OLCHC Professor Derek Sullivan, Professor of Microbiology, School of Dental Science, TCD Professor Pierre-Régis Burgel, Respiratory Medicine, Cochin Hospital, France Dr. John Waterstone, Consultant Gynaecologist, Waterstone Clinic Professor Barry Plant, Respiratory Consultant, CUH Dr. Nico Derichs, Respiratory Medicine, Kinderarztpraxis Derichs, Germany Dr. Jeffrey Beekman, Professor of Nephrology, University Utrecht, Netherlands Dr. Carlo Castellani, Medical Geneticist, Hospital Trust of Verona, Italy Dr. Martin Wildman, Respiratory Medicine, Sheffield Adult CF Centre, UK Professor Charles Gallagher, Clinical Lead, National Clinical Programme for CF Dr. Peter Barry, Respiratory Med, Manchester University NHS Foundation Trust Professor Liam Plant, UCC, CUH and HSE National Renal Office.

Throughout the clinical meeting I was struck by the sheer volume of work undertaken by and the dedication shown by these individuals to bring CF care into the future.

Certain areas of research and presentations stick out in my mind including talks on: The treatment of Aspergillus fumigatus infections in the airways of patients with CF, including the pros and cons of using oral antifungal drugs Advances in fertility care in CF which have allowed men with CF to become fathers by virtue of testicular extraction and IVF An international consortium project lead by Professor Barry Plant using new DNA based technologies, as opposed to traditional culture methods, to tailor antibiotic choices for PWCF The underestimation of drug-drug interactions in CF care and the importance of embedding a pharmacist into the CF team A project which uses recorded nebuliser data and behavioural specialists to improve adherence to nebuliser therapy The progress of the National Clinical Programme which has involved setting up six sub-groups each dealing with a different area of CF care including post-transplant care and CF and fertility The effects of CF on the kidneys caused by virtue of CF and not just through the use of aminoglycoside antibiotics or post-transplant care.

By Daniel Costigan, Senior Research & Policy Officer

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Spectrum / Spring 2018

Why does Ireland have the highest incidence of Cystic Fibrosis in the world?

Cystic Fibrosis (CF) is a monogenetic condition passed down generationally that prevents cells from being able to properly create the CFTR protein. CF is a condition which is unusually prevalent in Ireland compared to the rest of the world.

Whilst there is not yet concrete evidence for the reasons behind this and further research will be required, there are a number of theories as to why there is such a high prevalence of CF in Ireland.

The information presented in this article discusses current theories on the subject of the origins of CF in Ireland and the rest of Europe.

The most common genetic mutation that causes CF is the F508del mutation, causing around 66% of cases worldwide. A study conducted in 2015 (Farrell et al) gathered genetic samples from 182 patients with CF. These samples were used to create a historical prediction about the origin of the F508del mutation in Ireland.

The mutation was determined to have originated in Europe, most likely in France approximately 5,180 years ago in 3166 BCE. The mutation then likely spread to Ireland in 2806 BCE and continued to move mostly from West to East Europe, eventually reaching Albania in 794 CE. It travelled particularly quickly from France to Denmark and then to Ireland, spreading over a period of 360 years. It is speculated that this could have been due to mass migrations of Celts from the La Tene period. This expansion also led to the spread of the G551D CFTR mutation, dubbed “the Celtic Gene”. The combination of these two genes in Celtic populations may provide an explanation for the origin of the high rates of CF in Ireland.

The slowed spread of the F508del mutation in Eastern Europe is believed to be due to the increased genetic variability of those populations at the time, while groups in Western Europe became more homogenised. This would likely be a contributing factor to the prevalence of CF in Ireland, as it is a small land mass which contained a small island population at the time. A mass migration of La Tene Celts could have resulted in a small offshoot with a high proportion of CF carriers settling in Ireland, in a case of the “founder effect”.

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It is also interesting to note theories which associate CF genetics with resistance to infectious diseases such as tuberculosis, cholera and typhoid fever. Other theories have indicated that CF may have provided some advantage in exposure to heavy metals and toxic plants.

Farrell PM, Génin E, Férec C, et al. (2015) WS17.1 The ancient origin of F508del-CF: When and where the mutation arose. Journal of Cystic Fibrosis 14: S31.

Laki J, Laki I, Németh K, et al. (2006) The 8.1 ancestral MHC haplotype is associated with delayed onset of colonization in cystic fibrosis. International Immunology 18: 1585-1590.

Research eZine In Cystic Fibrosis Ireland (CFI), we realise that health literacy is extremely important for persons with cystic fibrosis (PWCF) and their families who want to learn more about existing and future cystic fibrosis (CF) treatments.

With this in mind, we have produced a quarterly e-zine to keep you up to date on the latest clinical research developments, which are likely to have a significant impact on health outcomes for PWCF. In this issue we present an overview of research on the safety and efficacy of existing CFTR modulators. We also examine the development of new modulator therapies and look at the potential expansion of existing CFTR modulators to a wider range of CFTR gene mutations through the use of personalised diagnostics such as intestinal organoids and nasal spheroids.

If you wish to receive the quarterly e-zine which will provide you with up-to-date, accessible summaries of clinical research in CF from leading clinicians in peer reviewed journals, you can sign up with us. You will receive an email confirming your subscription and you can unsubscribe at any time using the link at the bottom of each e-zine.

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Spectrum / Spring 2018

CALENDAR OF FUNDRAISING EVENTS 2018 April Friday 13th

65 Roses Day, Nationwide

Saturday 14th

65 Roses Skydive, Offaly

Sunday 22nd

Virgin Money London Marathon

May 10th to 13th

Malin2Mizen Cycle4CF

June Sunday 3rd

One in 1000 - VHI Women's Mini Marathon, Dublin

September 14th to 20th

Paris2Nice Cycle, France

Sunday 16th

Head2Head Walk, Dublin & Wicklow

Sept 28th -Oct 7th

Paddy Kieran’s International Walk, Romania

October Sunday 28th

SSE Airtricity Dublin City Marathon

November 4th (Sun)

TCS New York Marathon

For further details on any of our fundraising events, visit our website www.cfireland.ie, contact the CFI Fundraising Team on (01) 4962433 or email fundraising@cfireland.ie

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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) 4962433 or email fundraising@cfireland.ie Apr 13th: 65 Roses Day Cystic Fibrosis Ireland 65 Roses Day will take place on Friday 13th April 2018. We are looking for your support to help raise funds and increase awareness of Cystic Fibrosis across the country.

There are 3 ways you can help support Cystic Fibrosis Ireland on 65 Roses Day. 1) 65 Roses Day – Friday 13th April 65 Roses Day is our National Flag Day and our emblem, the CF Purple Rose will be on sale across the country for €2. Thank you to everyone who has so far offered to get involved by helping to sell purple roses and those who are organising events as part of 65 Roses Day. If you feel you can help out then please get in touch with our office as soon as possible so we can arrange to send you a fundraising pack. You can contact us by calling our office on 01 496 2433 or emailing fundraising@cfireland.ie. Our 65 Roses Day campaign will be featured across TV, Radio, Cinema, Press and Social Media in the run up to Friday 13th!

2) 65 Roses Challenge The 65 Roses Challenge is to organise a ‘65’ themed fundraising event in support of the 65 Roses campaign. Previous examples of challenges include a 65 Roses Tea Party, a gym doing 65 exercises in 65 minutes and schools holding a ‘No Uniforms Purple Day’. The challenge can be as simple or as challenging as you wish, so get your thinking caps on. For more ideas, check out our 65 Roses Challenge Event on our FB Page. If you would to discuss an idea or need fundraising materials then please call us on 01 496 2433 or email fundraising@cfireland.ie 3) 65 Roses Text Donate Support 65 Roses Day by texting 65 Roses to 50300 to donate €2 to Cystic Fibrosis Ireland or donate online at www.cfireland.ie.

Text costs €2. Cystic Fibrosis Ireland will receive a minimum of €1.80. Service Provider: LIKECHARITY. Helpline: 076 6805278.

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April 14th: 65 Roses Skydive The 65 Roses Skydive Challenge is now fast approaching so if you want to take part make sure to contact our office as soon as possible. The event will take place on Saturday 14th April in The Irish Parachute Club, Clonbullogue, Co. Offaly.

The cost of registration is €20 with a fundraising target of €500. Your fundraising amount needs to be raised and paid to CFI by Friday 30th March.

Registration forms can be downloaded from our website www.cfireland.ie or contact our office on 01 496 2433 or email fundraising@cfireland.ie.

June 3rd: 1 in 1000 – Vhi Women’s Mini Marathon On Sunday June 3rd, over 40,000 women will take to the streets of Dublin to walk, jog, or run the Vhi Women’s Mini Marathon. Registration for this year’s event is now open! You can register now at www. vhiwomensminimarathon.ie. Once you’ve done that, why not sign up to be the One in 1,000 for Cystic Fibrosis Ireland? When you sign up with Cystic Fibrosis Ireland, we will send you a fundraising pack including your T-Shirt.

We will look after you on the day when the atmosphere is electric. By being part of the One in 1,000 team, you will have access to cloakroom facilities and refreshments at D2 Harcourt Street, as well as postevent entertainment to celebrate your triumph!

You can sign up as part of a team, individually, or you can join one of our celebrity ambassador’s teams #TeamKaren, #TeamEvanne, #TeamLouise, #TeamDenise or #TeamJenny! Sign up with us to be the One in 1,000 for CF at www.onein1000.ie to get your fundraising pack, or contact Rachel at rbyrne@cfireland.ie / 01 496 2433 for more information.

Malin2Mizen Cycle4CF Preparations are now well under way for Malin2Mizen Cycle4CF 2018. We are pleased to say that 40 cyclists have registered to take part this year. The event will take place over 4 days from May 10th to 13th beginning at Malin Head in Donegal and finishing in Mizen Head in Cork.

Our cyclists are currently busy with both their fundraising and training ahead of the event itself with each cyclist aiming to raise a target of €2,000. We ask everyone where possible to support the cyclists with their fundraising and during the cycle along the route.

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Sept 14th to 20th: Paris2Nice Cycle There is still time to sign up for Paris2Nice 2018. This is always a memorable event for all those who take part so if you are considering it we would highly recommend signing up for this event sooner rather than later.

Cyclists will fly to Paris on Friday 14th September and begin cycling on Saturday 15th . The cycle makes its way along beautiful country roads with plenty of food stops in the scenic villages and towns along the route. The cycle will finish on the Promenades Des Anglais on Thursday 20th where you will be greeted by the welcoming team followed by a celebration dinner that night. Cyclists are free to fly home the following day or some choose to make a weekend of it in the beautiful city of Nice.

We would encourage anyone thinking of taking part to let us know here in the CFI office by calling us on 01 4962433 or email fundraising@cfireland.ie. We also suggest you sign up for Paris2Nice mailing list on the website www.paris2nice.com where you can also find details of the upcoming information evenings.

Sept 28th to Oct 7th: Paddy Kierans’ Memorial Walk ‘Flavours of Romania’ Places are filling up fast for our annual Paddy Kierans’ Memorial Walk, which will this year explore unique and beautiful Romania.

The ten day trip taking place from September 28th to October 7th will cater for various levels of fitness, so that each walker can take part at a pace they are comfortable with.

The 2018 walk, which starts in Transylvania and finishes in Bucharest, sees participants walking an average of 10km each day and taking in such sights as the incredible neo-renaissance Peles Castle, the stunning Southern Carpathian Mountains, and the historical UNESCO protected Village of Viscri.

Are you interested in visiting new and exciting places, making friends forever and memories that will last a lifetime, all the while raising much needed funds for people living with CF in Ireland?

Join our celebrity ambassador, Operation Transformation’s Dr. Eddie Murphy, and our walkers on the experience of a lifetime. Contact Rachel on rbyrne@cfireland.ie / 01 496 2433, or visit www.cfireland.ie.

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Spectrum / Spring 2018

Oct 29th: SSE Airtricity Dublin City Marathon Did you know that the SSE Airtricity Dublin Marathon is known as the ‘Friendly Marathon’ because of all the supporters that come out to cheer the runners on?

If you are thinking of taking the leap and signing up the Dublin Marathon this year, why not fundraise for Cystic Fibrosis Ireland while doing it?

Registration costs €83 (this is the tier 2 fee – places are filling up fast!) and race entry is guaranteed upon payment. The 26.2 mile route is mostly flat and is a single lap which starts and finishes near Dublin city centre.

Once you are registered to take part in the SSE Airtricity Dublin Marathon, contact Rachel at rbyrne@cfireland.ie / call 01 496 2433, and she will send you out your fundraising pack and let you know how to get started on your fundraising.

Contact Rachel at rbyrne@cfireland.ie / 01 496 2433 or visit the below link to find out more.

Nov 4th: TCS New York City Marathon Registration is now open for the TCS New York City Marathon 2018. CFI are pleased to say we can offer you a guaranteed entry as part of a tour package. The marathon starts on Sunday 4th November 2018 and runs through all 5 city boroughs (Staten Island, Brooklyn, Queens, The Bronx and Manhattan). As places are limited we advise anyone thinking of taking part to register as soon as you can.

You can sign up with CFI to take part and the package includes: •

Direct return flights from either Dublin or Shannon to New York

•

4 or 5 nights accommodation

•

Return airport transfers

•

Private coach from the hotel to race start on Staten Island

•

Guaranteed race entry

•

Services of Sports Travel guides

If you would like to sign up or find out more then please contact the fundraising team on 01 496 2433 or email fundraising@cfireland.ie

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All Year: Kilimanjaro CFI facilitate treks to Kilimanjaro, so if you are thinking of taking part in a trek, we advise that you book your place early.

For more details and a full list of tour dates, please see our website www.cfireland.ie or contact Peter on email at pminchin@cfireland.ie

Schools Fundraising Resource Pack CFI have issued our ‘ChariTY for CF’ school fundraising pack to all Secondary Schools, with a focus on Transition Year students.

By getting involved, not only will ChariTY for CF help schools raise awareness of cystic fibrosis and help raise much needed funds to help support people with CF, it will also help students to develop and strengthen their involvement in community and charity work.

To request a

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pack or to find out

Spectrum / Spring 2018

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 be�er quality of life for people with CF and please keep up the great work. Here is a short summary of some of the remarkable efforts: Spar Our Charity Partnership with Spar continues to go from strength to strength. Cystic Fibrosis Ireland attended the Spar Guild Meetings in Cork and Dublin at the end of January where we were presented with a cheque for €160,000 from Spar. This money was raised across their stores since our partnership started last May from sales of Spar Own Brand product and Collection Boxes and €20,000 raised from the Spar Twitter Tree Campaign at Christmas.

Spar confirmed their commitment with all their stores at the Guild Meetings to sell our Purple Roses and Rose Bags on 65 Roses Day. They have confirmed their support for our other upcoming events including Malin2Mizen Cycle4CF in May, 1 in 1000 in June and the Head2Head Walk in September.

We are delighted to be included ‘under the tree at Spar’ for ongoing fantastic fundraising by Spar to support people with Cystic Fibrosis in Ireland.

Spar cheque presentation of €160,000 to Cystic Fibrosis Ireland at Spar Guild Meetings in February from funds raised by Spar in 2017. Pictured L to R; Colin Donnelly, SPAR Sales Director; Fergal Smyth, Fundraising Manager, CFI; Shane Cantillon, SPAR Mount Oval and SPAR Little Island, Chairman of the National Council of SPAR Retailers.

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Head2Head Walk 2017 A huge thank you to Mary McCarroll, Jem & Lorrie Downes and everyone in the Cystic Fibrosis Ireland Eastern Branch who organised the most successful Head2Head Walk to date in September last year with over 500 walkers raising an amazing €48,445.40. This was also made possible by our Charity Partner Spar who turned out in force on the day to support the event, led by Mark Jones from Spar Carpenterstown who completed the 26km walk in an unbelievable 5 hours raising €3,000 with the support of everyone at the store.

We are already ready looking to the Head2Head Walk 2018 on Sunday 16th September.

Pictured at the cheque presentation at Spar Carpenterstown are Fergal Smyth, Fundraising Manager, CFI; Mark Jones, Spar; Mary Mc Carroll, Lorrie & Jem Downes – CFI Eastern Branch

Hospital Saturday Fund Cystic Fibrosis Ireland would like to thank The Hospital Saturday Fund who invited a representative to attend a reception and cheque presentation for €4,000 in the Mansion House on February 15th.

Pictured at the cheque presentation are Paul Jackson, Chief Executive, Hospital Saturday Fund; Mícheál Mac Donncha, Lord Mayor of Dublin; Fergal Smyth, Fundraising Manager, CFI; Lesley Garrett, Patron, Hospital Saturday Fund

Mylan Cystic Fibrosis Ireland would like to say a huge thank you to Mylan for their continued support of the Race Shirts for our 1 in 1000 Campaign for the VHI Women’s Mini Marathon. Mylan have once again sponsored the shirts for our 1,000 participants ensuring that there will be a sea of purple on show at the VHI Women’s Mini Marathon in support of Cystic Fibrosis Ireland.

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Spectrum / Spring 2018

Malone Group We would like to say a huge thank you to Malone Group who have come on board again as the sponsor of our Radio Adverts for the 65 Roses Day Campaign.

The adverts are running for 3 weeks on RTE Radio One, featuring our ambassador Keelin Shanley. Listen out for the advert which should ensure maximum support for all our volunteers out selling Roses and collecting for Cystic Fibrosis Ireland on 65 Roses Day. Malone Group are also organising their 65km Cycle again this year which will take place on Friday 30th March with all cyclists invited along to take part, it could be a great training spin for anyone taking part in Malin2Mizen Cycle4CF or Paris2Nice in 2018.

Thank you again to everyone in Malone Group for your continued support!

CEX We were delighted to receive a cheque for â‚Ź4009.98 from CEX which was collected in Collection Boxes in their stores across the Country. Thank you to everyone in CEX and your customers for your continued support of Cystic Fibrosis Ireland.

IQVIA Cystic Fibrosis Ireland would like to say a huge thank you to our Charity Partner IQVIA who recently presented us with a cheque for â‚Ź11,237 based on a range of fundraising events held by their staff including a sponsored walk, Open Mic night and a Christmas Fair.

It was a tremendous effort by everyone at IQVIA and will be a great help in terms of supporting people with Cystic Fibrosis in Ireland.

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GAA Cystic Fibrosis Ireland would to say a big thank you to our Charity Partner the GAA who raised €25,000 during their Tour De Thurles Cycle - a GAA and Croke Park. This was a staff led initiative which involved staff members cycling the 166km from Croke Park to the birthplace of the GAA, The Hayes Hotel in Thurles, Co. Tipperary.

The funds raised were split between Cystic Fibrosis Ireland and the four other Charity Partners.

Tayto Park A huge thank you to Tayto Park who selected Cystic Fibrosis Ireland as one of their charities to support in 2017 and have given us a donation of €10,000.

We thank them for this very generous donation and wish them the very best of luck with Tayto Park again this year, a wonderful family facility in north Meath.

Deutsche Bank We were delighted to have been chosen by staff in Deutsche Bank as a nominated charity and their recent cheque presentation for €10,000.Thank you to everyone in Deutsche Bank for thinking of people with Cystic Fibrosis!

Tara’s Palace Museum of Childhood CFI would like to say a big thank you to Tara’s Palace Museum of Childhood who recently presented a cheque for €3,000 from their fundraising efforts in 2017. Special thanks to Anne Lawless for organising this.

Pictured left are (L to R) Tara’s Palace volunteers Anna Lynch, Dilys Lindsay and Jo-Anna King-Hall presenting a cheque to Peter Minchin (CFI).

Thank you to all who donated!

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Spectrum / Spring 2018

Maeve & Helen, Dublin City Marathon CF Ireland would like to say a huge thank you and well done to both Maeve Hegarty and Helen Whearity (PWCF) who took part in the 2017 Dublin City Marathon.

They raised a fantastic total of €2,704.60 through their participation in the event.

Pictured from L to R at the cheque presentation are: Maeve Hegarty, Helen Whearity and Philip Watt (CFI).

David Crosby & Team, NYC Marathon 2018 CFI would like to say a huge thank you to David Crosby and all his team of New York Marathon runners who recently donated €11,000 to CF Ireland. David brought a team of 14 runners to the 2017 NYC marathon which included his wife Katie and mother Kathleen. David who suffered from IPF was a Double Lung Transplant recipient in 2016 so this was quite a remarkable achievement.

Well done to all involved!

North Tipperary Die cast Model & Diorama Show CFI would like to say a huge thank you to Shane Benson from Limerick who organised the North Tipperary Die cast Model & Diorama Show and donated €800 as proceeds from the event to CF Ireland. The event was held in Roscrea in December and a great day was had by all who attended. Pictured below at the cheque presentation are the Benson family presenting the cheque to Erin Sugrue on behalf of CFI. L to R: Erin Sugrue (CFI), James Benson, Georgina Benson and Shane Benson.

Doolan Family Thank you to the extended Doolan family and friends from Mount Merrion who kindly donated €500 to CFI as proceeds from the recent 90th birthday celebrations for Jennie Doolan who has relatives living with CF. It was very thoughtful and much appreciated.

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Cystic Fibrosis Ireland Branch Network Carlow Leigh Bolger 087 684 3750

Cavan Robert Dunne 087 625 8476

Clare Kenneth Flanagan 086 222 2290

Cork (Southern) Aisling O’Neill 085 863 8522

Dublin West Marie Duffy 086 237 1184

Dundalk Maire Gallagher 086 825 9656

Drogheda Cyril Gillen 087 694 4922

Eastern Mary McCarroll 087 411 9812

Galway Claire Sullivan 087 945 2004

Kerry Colette O’Donoghue 087 318 7461

Kilkenny Carmel Delaney 086 313 6029

Limerick Jackie Gaffrey 087 659 9194

Louth Marian Renaudin 087 957 2396

Mayo Billy O’Toole 086 059 6255

Meath Loretta Allen Byrne 087 205 8778

Midlands

Sligo Iris Murphy 086 825 8525

Tipperary Mandy Quigley 087 612 0848

Waterford Bernadette Power 087 679 0749

Wexford Suzanne Doyle 087 238 7904

01 - 496 2433 TLC4CF 087 967 9176

Cystic Fibrosis Ireland 24 Lower Rathmines Road, Rathmines, Dublin 6, Ireland t: +353 1 496 2433 f: +353 1 496 2201 e: info@cfireland.ie w: www.cfireland.ie Company Reg: 449954 Charity: CHY6350

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Spectrum / Spring 2018