SPECTRUM Autumn 2021
Going Head 2 Head! Cystic Fibrosis Ireland's first live event of 2021, our Head2Head Walk takes place on Sunday, September 5th
www.cfireland.ie
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CONTENTS Latest News | 1 - 5 * COVID-19 Guidance * Beaumont Hospital CF In-Patient Unit * Extension of Kaftrio * Update on the work of CFI * Congratulations from CFI
Spotlight | 6 - 15
Independent Living * Education: College and CF * Fertility & Pregnancy: My Journey to Motherhood
Member Services|16 - 19 * Back to School * Flu Vaccine
Research| 20 -25 * PPI in Research * Research Interview: Dr. Audrey Tierney Exploring Diet Quality in CF * ECFS Conference Report
Legacy| 26 - 27 * My Legacy Month - November
Fundraising | 28 - 38 * Challenges and Events * Thank You * Length x Breath Challenge * Christmas Cards
CEO’s Message Hi everyone, welcome to the Autumn edition of Spectrum. As always, this issue features a number of positive news items and personal stories which we hope you will enjoy.
Philip Watt Chief Executive
We hope you all had a good summer, while understanding for many it remains a challenging time, including for those who are seeking to return to education, training and employment.
While we cannot be complacent, it is clear the vaccination programme, including the extension to 12-15 year olds, is making a huge positive difference. Recent research in Ireland (North and South) indicates that 80% of people under 60 years of age in hospital with COVID-19 are not vaccinated – clearly indicating how the vaccinations have prevented both illness and more serious illness arising from COVID-19, though the ongoing impact of the variants remains a worrying factor. CFI is urging the Government to now think about supports for those with CF and other conditions who are planning to transition from ‘cocooning’ to part-time and full-time employment. This issue has been flagged by people with CF within CFI. Practical support from Government for those reentering the workplace would be most welcome and CFI will further consider how we can ‘do our bit’ in this regard. As part of the forthcoming Human Tissue Bill, CFI will continue to urge the prioritisation of a new form of organ donor consent ‘soft opt out’ that has proved so successful in Wales, England and more recently in Scotland, as well as many other parts of Europe. Finally, a huge thanks to the ongoing guidance and support of the CFI Board ably chaired by Keith McCabe, our many CFI branches and to my staff colleagues in CFI whose work has been unstinting over the summer. Thanks as always to those providing dedicated CF clinical services, many of whom are involved in the CF research that is vital for the emergence of further new and innovative therapies and shaping the way CF services are delivered in Ireland.
DISCLAIMER: The views of contributors, when expressed in this publication, do not necessarily reflect the position or policy of Cystic Fibrosis Ireland. COVER: Participants of the 2019 Head 2 Head Walk
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Philip Watt, CEO, CFI
Spectrum / Autumn 2021
LATEST NEWS: COVID-19 Guidance for people at higher risk of severe COVID-19 People with cystic fibrosis are urged to continue to follow the HSE Guidance to reduce their risk of exposure to COVID-19. Transplant patients should take additional precautions to further prevent risk.
Coronavirus COVID-19
Helptheir prevent coronavirus The HSE has updated guidance for people at higher risk from COVID-19 to reduce risk of exposure to COVID-19. The guidance recognises that people at high risk of severe disease from COVID-19 have the right to make their own decisions and the responsibility to look after their own health and the health of others in line with the legal Cover mouth touching Wash your obligations that apply to everyone. This involves making their own choices about how they keep aifbalance Avoid between coughing or sneezing your face hands doing the things they need to do and want to do to while keeping the risk of harm from COVID-19 as low as is practicable. People at higher risk from severe COVID-19 are advised to wear a surgical or medical face mask instead of a cloth face covering as it gives you better protection. Keep surfaces clean
Stop shaking hands and hugging
Keep a safe distance
The transplant team at the Mater Hospital have produced an information leaflet The virus spreads in sneeze and cough droplets, so regularly taking for Vaccinated transplant recipients on how to continue to avoid COVID-19 the above measures helps reduce the chance of it spreading. infection. You can download a copy of this leaflet from www.cfireland.ie COVID-19 vaccines are safe and effective at preventing severe illness and death immunocompetent people. VisitinHSE.ie For updated factual information and advice However for people with weakened immune systems, such as those after a heart and/or Or call 1850 24 1850 lung transplant, the vaccine is less effective. Transplant patients are more at risk of a severe infection than those who are not immunocompromised so it is important to take additional steps to avoid infection Protectionregardless from coronavirus.of easing of restrictions It’s in our hands. for the general public. This involves making choices about what you need to do and want to do to keep the risk of becoming infected with COVID-19 as low as you can. Full details are available on our website www.cfireland.ie or on the HSE website www.hse.ie While COVID-19 is still with us, there is a legacy of Covid-19 that needs to be acknowledged. Adjusting to the "new normal" can be an anxious time This is to be expected after a year of living with restrictions in a pandemic, we have come to associate staying home with staying safe, limited our social interactions and kept our close contacts small to prevent transmission. We would like to remind members that our Member Services team are available to speak to and that supports are available for you and your family. Children aged 12-15 years now being vaccinated As most will know at this stage, vaccine registration for children aged 12-15 opened on Thursday August 12th. Children will be offered the Pfizer or the Moderna COVID-19 vaccines, in line with the recommendations of the European Medicines Agency that mRNA vaccines were safe and effective for those aged 12 to 15. The HSE particularly recommends vaccination for this age group if they: •
have a health condition that puts them at higher risk of severe illness from COVID-19
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live with someone who is at higher risk of COVID-19
At present the places of vaccination for all children 12-15 years old, including those with CF, is a vaccination centre or through your pharmacist. Some GP’s may also provide vaccinations. www.cfireland.ie
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Beaumont Hospital CF In-Patient Unit Gets Go Ahead from HSE - CFI seeks Start and Completion Date Cystic Fibrosis Ireland warmly welcomes the news that the HSE has allocated €2.5 million towards the building of the long awaited 20-room in-patient ward in Beaumont Hospital, Dublin. CFI has received confirmation that this funding will be provided as part of the HSE Capital Plan for 2021. This news is very welcome and CFI is seeking a meeting with Beaumont Hospital Management in early September to discuss a start and completion date. The ambition is to build a very high specification 20 room in-patient building for adults with CF in Beaumont Hospital following on from the out-patient unit completed in 2011. This ambition is an important one, not just for adults with CF North Dublin, but for all PWCF living in North Leinster and Ulster whose main specialised centre is Beaumont Hospital.
Beaumont Hospital's Cystic Fibrosis Unit
There are more than 160 adults with CF attending Beaumont Hospital. The number of patients with CF attending Beaumont is projected to increase because of longer survival ages arising from a combination of access to Patient Meeting innovative Engagement medications, improved CF facilities and the impact of the lung transplant programme in Ireland. CFI has worked in partnership for many years with local charity CF Hopesource Foundation and we have jointly 20th August 2020 committed to raise €1m towards the overall costs, estimated at around €12m. Commitments to build this unit was included in the Programme for Government 2016 and more recently in the HSE Service Plan, 2021 and the HSE Capital Plan 2021.
Consultation A high level steering group to oversee the project was established in 2018 and includes representatives from CFI and CF Hopesource; hospital management; clinicians and the design team. In 2020 architects Scott, Tallon and Walker were appointed to draw up detailed plans and August 2020 a detailed consultative process was undertaken by CFI and CF Hopesource with those attending the hospital, see cfireland.ie for more details on this. This consultation considered issues such as exercise equipment; facilities for studying while in hospital and other design considerations. The results of this survey were discussed with the Hospital management/architects and much was subsequently incorporated into the final plans. As with all major building developments, COVID-19 had a major impact on the pace of this project. The resumption of major building work in Ireland and the commitment of €2.5 gives us renewed hope that the building phase of this project will commence soon.
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Head2Head Walk In the meantime we would urge support for the annual Head2Head Walk organised by the Eastern Branch of CFI from Howth to Bray which takes place on Sunday 5th September. This is our first in-person event since 2019 and we are excited to welcome participants back. After a very successful Head2Head Virtual Walk in 2020, we are encouraging everyone that can to participate by completing a 21km or 42km walk – the distance from Howth to Sandymount (21km), Sandymount to Bray (21km), or from Howth to Sandymount (42km) on Sunday 5th September. For further information or to register for the event visit www.cfireland.ie Kaftrio Extension It is welcome news that more people with CF in Ireland will gain access to Kaftrio, commencing August 2021. People with CF, aged 12 and over, who have one copy of the F508del mutation, regardless of their other mutation type, will now be eligible for Kaftrio. This is a result of the Portfolio deal agreed between Vertex and the HSE in 2017 as part of the ‘Orkambi Agreement’ which is supported by CFI. CFI estimates that around 30-40 patients who previously had no access to one of the 4 existing Vertex CFTR modulators (Kalydeco, Orkambi, Symkevi and Kaftrio) will now have the potential to access Kaftrio. Others who are already on an existing CFTR modulator may also gain access to Kaftrio for the first time. It is important to note once again that a small percentage of patients with CF in Ireland with other rare/nonsense mutations will never be able to benefit from a CFTR modulator because they produce no CFTR protein. CFI will of course strive to continue to keep everyone informed of on-going progress in health research/clinical trials for future therapies to seek to ensure ‘no one left behind’. CFI Awarded Triple Lock Standard for 2021 CFI are delighted to confirm that we have once again been awarded the Triple Lock standard from the Charities Institute Ireland for 2021. The Triple Lock demonstrates Cystic Fibrosis Ireland's openness, transparency and integrity operating to Triple Lock Standards of transparent reporting, good fundraising and governance.
www.cfireland.ie
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Webinars on Housing and Wellbeing We held two webinars over the Summer as part of our ongoing webinar series. In June, we hosted “Where Are We Now? In Conversation with Psychologist Dr. Eddie Murphy’”, a webinar for the CFI Community focusing on mental wellbeing and adjusting to the emotional impact of COVID-19. The interactive session included inputs from our CF Advocates and Lorraine O'Neill, Parent of a child with CF. “A Place to Call Home”, a webinar on housing, took place in July. The webinar had a particular focus on the mortgage journey, exploring the supports available for social housing and discussing some of the other challenges facing people with CF and their families in accessing home ownership or rented accommodation. We thank all who contributed to the webinars, especially Lorraine O'Neill and Colm Priestley who shared their own experiences and insights. Thanks also to the expertise of CFI staff who organised and contributed to these two excellent webinars. Recordings of the all the webinars held to date are available to watch back on our website www.cfireland.ie. We hope to announce details of the next series of webinars in September, make sure to check out the website for details and to keep up to date with the latest news from CFI. New Housing Cost Rental Scheme Those earning up to €82,000 per year before tax will be eligible for the Government’s cost rental scheme, under new regulations (forthcoming). The cost rental scheme seeks to bring down rents in developments that will generally be run by housing non-profits, developed with no profit margin built in. Eligibility for the scheme will be capped at a household income of €82,273, or about €53,000 at a net level, with single people as well as dualincome households earning up to this level eligible to apply. Different housing types – such as single-bed or larger apartments – will be allocated depending on the number of people in each household. E-Bike scheme Many thanks to those that applied for the e-bike pilot scheme. It was a difficult process to decide on the 20 participants. If the pilot scheme is successful CFI will seek sponsorship and funding for an ongoing scheme. Exercise Grant Second Round Thanks to a decision by the CFI Board, there was an increase in funding provided for the second round of the exercise grant in June 2021 which enabled more of our members to avail of this important support. Good Luck Aisling Many thanks to Aisling Tierney who served as Digital Marketing Co-ordinator in the fundraising unit of CFI and has recently secured a new role. Among the contributions Ash made during their time with CFI was to coordinate much of the 65 Roses Concert. We will miss you Ash and best of luck in your new role!
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Congratulations Dr. Twomey Dr Michael Twomey graduated from University College Cork on a sunny 13th July 2021 after obtaining a Ph.D. with his project “A Check List to Aid Patient/Carer Memory Recall within the Medical Appointment: An Action Design Research Journey”. Michael’s work has already made a difference to people living with CF and Cystic Fibrosis Ireland looks forward to working with Michael in the future to further this. Congratulations Michael, we are all very proud of you.
Tallaght Team Triumph Congratulations to the CF Team in CHI Tallaght who won the CHI Progressive Team award for 2020. The Awards recognise the hard work, dedication and innovation of staff throughout the four CHI Paediatric Hospitals - Connolly, Crumlin, Tallaght and Temple Street. Pictured right receiving the award are (L to R) Gerardine Leen (CNS), Prof Basil Elnazir, Dr. Fiona Ringholz, Muireann Dee (Physiotherapist), Jessica O’Driscoll (Dietitian), Emma Kilbride (Respiratory Physiologist) and Geraldine Connell (CNS) Not everyone could attend the presentation of the award so we would also like to mention the other members of the CF Team Prof Peter Greally, Helen Gibbons (Psychologist), Lynda Thompson (Respiratory Physiologist), Ruth Jennings (Social Worker), Gillian Doyle (Pharmacist), Jacinta Greene (Dietitian), Anna-Rose Prior (Microbiologist) & Stephanie Hynes (Department Secretary).
www.cfireland.ie
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Spotlight Series: Independent Living
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ccording to the CFRI 2019 Annual Report (www.cfri.ie) there are more adults (aged 18 years or older) living with CF in Ireland than children.
With improved life expectancy and a better quality of life, there is an increased ambition for people with cystic Fibrosis (PWCF) in Ireland to live more independent lives. To support the changing demographic of our membership, Cystic Fibrosis Ireland established an Independent Living sub-group in 2016. The group commissioned research which led to the publication of the CFI Independent Living Report in 2018. The report compared data from two surveys, the first was undertaken in 1998 and the second in 2017, exploring how life for adults with cystic fibrosis has changed over two decades. The results were generally very encouraging, showing that with sustained investment in better health services and with a good model of care and access to new and innovative therapies, the lives of people living with a long-term and life-limiting disease can be changed for the better in a relatively short period of time. The report focused on five key areas that make Independent Living possible; • Housing and Living Arrangements, • Education, • Employment and Social Protection, • Mobility and Transport • Supports for beginning a family These continue to be key areas of focus for CFI and account for several key objectives in our strategic plan. The report was not about highlighting problems, but identifying solutions to ensure that PWCF will continue to have access to opportunities to realise their full potential. Housing was the Spotlight in the Summer issue of Spectrum and in July we held a webinar on access to housing for people with cystic fibrosis and their families. CFI recently launched a pilot e-bike scheme to examine the potential benefits of an e-bike to quality of life for PWCF. In this issue of Spectrum, we are delighted to Spotlight two areas of Independent Living - Education and Parenthood. A huge thank you to the two contributors for sharing their personal experiences. If you would like to discuss any of the issues raised, supports are available. You can contact the member services team on 01 496 2433 / email info@cfireland.ie
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Education: CF and College Education is key to Independent Living. Not only does it increase potential employment opportunities and income, but it helps build a variety of social and key life skills. Cystic Fibrosis can impact on education at any stage of life through a variety of formats including missed time due to sickness, hospitalisations and appointments, reduced energy levels, missed social interaction, deferred exams or repeated years. For people with CF, COVID-19 was also a disrupting factor on education with many students remaining in home schooling / online modules longer than their peers due to health guidance. It is important to remember there are many routes to education and that if the plan you have does not work out, it does not mean that there isn't another way to continue your education. This Spotlight is specifically focusing on college and third level education. CF Ambassador, Aileen Henderson shares her experience from choosing a course to completing her degree. If you are a person with CF starting college or considering a return to education and would like to discuss supports available, please contact CFI for advice on 01 496 2433 / info@cfireland.ie
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i, my name is Aileen Henderson, I’m 25 years old and I have Cystic Fibrosis. I recently graduated from college so I understand the added pressure CF can often bring when entering this new chapter in life.
I finished secondary school in 2015 with very little idea of what I wanted to do next. I got offered a place in science in my hometown of Galway, but decided to turn it down as my worst fear was to be stuck in a course that I wasn’t 100% certain of. Having always had a great passion for music throughout my life, I decided to go with my heart study music. Rather than move away from home, which would have been a big step for me straight after school, I decided to do a year long PLC in ‘Music and Composition’ in Galway. At the beginning of the year, I typed out a letter informing my tutors about my CF and how it would affect my work to some extent throughout the year. I handed this to them individually to avoid it getting lost in any emails. For me, I had no issues with them knowing about my condition as I have always been open with my friends, although it may be a bigger deal for others opening up about their CF. I never wanted special attention, just for them to understand that I may miss days dues to being sick or for appointments. As long as it has the most important information, specific to you, that you feel would be beneficial for them to know, that’s all that’s needed. I also mentioned in the letter that if another student came into the college with CF that both of us would need to be informed for crossinfection precautions. www.cfireland.ie
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My PLC course hours were similar to school hours with one day a week off. I found it a great transition from school, enjoyed meeting new people and I have still have great friends from it. I was able to keep up with my classmates the majority of the time and I noticed that I felt much better overall than I did in school - maybe this was due to smaller class numbers, no homework and generally a much more relaxed environment. However, I did miss several days due to chest infections or colds that I was almost guaranteed to pick up if they were going around at the time. I also had a 2 week admission in hospital for antibiotics. During that year I did lack some energy and would get tired very quickly. I’d often find myself falling asleep when I came home - naps were a big thing for me back then! I’m also a major night owl so maybe if I went to bed before 2am that wouldn’t have been an issue. (Let’s not blame everything on CF!). I did have to watch myself carefully and make sure I didn’t overdo it, or I would easily get run down and sick. While thankfully admission for IV antibiotics is rare enough for me, I was on several courses of oral antibiotics throughout the year to keep me at my baseline as much as possible. Once I decided that I wanted to continue studying music. I did some research on what colleges would suit. I had heard of the ‘Brighton Institute of Modern Music’ (BIMM) from some friends which is a college that is situated in many parts of the world with the Irish one in Dublin. BIMM is affiliated to TU Dublin (formerly known as DIT). It looked like the ideal college for me, however, the fact it was in Dublin was a slight issue for me at the time. It meant I would have to move away from home and at the time that was something I wasn’t fully comfortable with. Although my CF didn’t hold me back too much, it did get in the way quite often and I felt I wouldn’t be able to manage the uncertainty of my condition by myself in Dublin. After several back and forths on what would be the best approach, I decided to do the diploma course in ‘Professional Musicianship’. This course was only one day a week and would allow me to decide if I wanted to move on and do the full time Level 4 course the following year. Initially I had planned to commute and stay up in Dublin one night a week, but college life soon took over. I met lots of new friends and was involved in many bands and projects in college that I enjoyed a lot. I ended up being there most of the time and stayed in student accommodation with 3 other girls that I got on really well with. As it was the first time I had moved away from home, things like washing and food shopping were a new novelty! It was during this year that my health had declined a little. My lung function was at a new baseline around the 50s with it dropping to the high 40s if I had an infection. I often got my shopping delivered once a week as I found it a bit of a struggle carrying it home. My mum had suggested one of the shopping trolleys but not a chance would I be seen going to the shops like that back then! (wouldn’t bother me in the slightest now!). As the end of the year approached, I had already decided I was going to go on and do the full-time degree for 4 years. Because I had moved up to Dublin gradually during the diploma year, when I moved back up after the summer to start my degree it didn’t seem like a big deal. I had figured out how to look after myself up there and go about my day. I was the same as any other person just with some added adaptations to be made. I had my medications in my room and also had a mini fridge in my room to store anything that needed to be refrigerated (e.g. Cayston & Pulmozyme), so I wasn’t taking up the whole fridge in the kitchen! I also sorted out a local pharmacy. They were very good to me and it saved me having to bring everything up from Galway. If I needed anything I was able to call in on the way to college. College life was fairly heavy going. I had classes during the day and most days I had rehearsals until late at night. My course was a BA in Commercial Modern Music and I specialised in vocals. My course covered a broad range of topics including music theory, music business and song-writing. Some of my friend specialised in other areas such a guitar, drums and sound technology so I got to experience all aspects of the music industry.
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I had to make sure to eat properly and get my dinners in as my weight wouldn’t have been as stable back then as it is now. For the most part I was able to manage well. I had registered with the disability services in my college and I would highly recommend you make yourself known to this department in your college. At first, I was reluctant, as again, I didn’t want to be treated any differently to my classmates, but as time went on, I became more comfortable and by final year I was making use of all the accommodations available to me. These included extra time/extensions for exams and I was also able to avail of a separate room for myself for written exams. I used this once so that I could eat during the exam and keep my sugars up! I need to eat regularly due to borderline CF related diabetes but thankfully that’s stable and I don’t need insulin. In my first year of college, I got to start the new modulator drug Orkambi, but unfortunately, I had a bad reaction to it and had to stop. I seemed ok on it at first but then developed an all-over rash after approximately 2 months. This was a hard thing to come to terms with as I felt if that didn’t work for me none of the up-and-coming drugs would work either. In 2nd year I went through another bad patch with my health which involved several haemoptysis (coughing blood) episodes. I tried some different oral antibiotics and was admitted for IV antibiotics. This was also the wakeup call I needed to get back into exercise. I had been good at exercise before and would have been very active as a kid although this time I needed to find something that I liked and stick to it. I started going to the gym in my student accommodation almost daily, and became quite addicted (never thought I would say that!). To start with, I did light weights and some cardio. I eased myself in gradually as I would have been very prone to coughing up blood during strenuous exercise. I couldn’t run for more than 30 seconds without it happening but bit by bit I improved. In January of my second year of college I started the newer CF drug ‘Symkevi’. I had heard people were tolerating it much better, but I still didn’t let myself get too hopeful about it working for me after the bad experience I had on Orkambi. I remember the day I started Symkevi very clearly as I was singing at a gig in Dublin that night and I was adamant not to miss it. I started the drug that morning in Galway, and my mum drove me up to Dublin and stayed around until the gig was finished just incase I had any reaction to it. She then drove back to Galway that night once everything seemed to be going ok. Such dedication to your daughter! I had no side effects straight away but was still nervous for a good while after I started Symkevi as it was 2 months into Orkambi that I had the drug reaction. Once I was over the 2-month mark on Symkevi and everything was going well, I could relax a bit and realise that my body was tolerating it well. Such a relief! I continued to work on my fitness and started to introduce short running sessions which I have built up hugely over time with no episodes of coughing blood. I celebrated by running the 10km for CF during the first 2020 Covid lockdown. I have to say, my fitness improved greatly that summer as I was in lockdown and relying on zoom exercise classes and just about everything else on zoom to keep me entertained! I didn’t see any major change within the first couple of months of Symkevi, it was definitely a slow improvement for me, however, looking back it has transformed my life. In my final year of college, I had a brief break from all the fun and games. I was lucky enough to be in the small minority of people with CF who have liver problems. This was picked up when I was very young and remained stable up until recently. www.cfireland.ie
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In November 2020 I started having a lot of stomach problems and it was recommended by my CF team to go into A & E at the time. I didn’t think too much of it, sending snaps to my friends of me waiting thinking I was absolutely gas! I was soon admitted to Galway hospital where they eventually discovered some fluid build-up around my liver meaning it was under some pressure. I was transferred to Vincent’s hospital in Dublin by ambulance in the height of Covid and admitted up there where I stayed for a week. It was definitely the hardest experience I’ve had to go through and even more scary during Covid as I was alone a lot of the time. My mum stayed nearby and managed to get in a few times to visit. My family, friends and boyfriend where great throughout and kept me occupied as best they could. During my time in St. Vincent’s, the topic of liver transplantation was mentioned. This wasn’t completely new to me as I had been assessed for transplant previously, but the decision was eventually made that I was too well at the time. I started diuretic tablets to get rid of the fluid on my liver and was also assessed for transplant again. I was discharged and had to wait for a call over the following few weeks to see if the team in Vincent’s had decided to put me on the active transplant list or if they thought I still wasn’t ready. I feel like I really wasn’t myself during that time although didn’t let it on too much! I was fully convinced it was time to be put on the list. But the tables turned again and the liver team decided I was still too well to be put through a transplant. The fluid was gone and aside from my liver being unstable for a while, I was still the healthiest I had ever been in my life. Dodged the bullet again! Because I was fully convinced I was going to be put on the liver transplant list, I had come to the realisation that I might have to defer my final year of college which wasn’t an easy thing to come to terms with being so close to the finish line. Thankfully this wasn’t the case, and I got all my assignments and thesis submitted on time with the help of a few extensions (highly recommend if you need them!). With all lectures and tutorials gone online due to Covid, this actually suited me and I managed to catch up on anything I missed although I did miss the social life! Now that I’m finished college, I’m back to how I was before my liver set off and doing better than ever. I moved back to my family home in Galway during the start of Covid and have recently been accepted into a ‘Digital Marketing’ masters in Galway which I’ll be starting in September. My best advice to someone nervous about moving away for college or just college in general is not to let CF stop you from taking the leap. It’s very do-able if you figure out the best approach for you and take advantage of the disability and student services offered to you. There are so many ways to adapt college life for you, and if you don’t ask you don’t get! The extra time during exams and extensions, even changing timetables around to suit my schedule, all made a huge difference to my college experience. The future of CF is so much brighter than ever before so take the bull by the horns and live your best life!
Aileen As well as being an Ambassador for Cystic Fibrosis Ireland, Aileen is also part of an online cystic fibrosis group, Salty Soldiers. The group is made up of 12 people with CF who share their experiences of living with cystic fibrosis to raise awareness of the condition and inspire the next generation. Follow Aileen and the Salty Soldiers Group on Instagram @SaltySoldiers
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Fertility & Pregnancy in CF
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My Journey to Motherhood ne of the lesser-known side effects of cystic fibrosis is infertility. Around 98% of men with CF are infertile because of Congenital Bilateral Absence of the Vas Deferens (CBAVD).
The effect of CF on the fertility of women is more complicated. Even in women without cystic fibrosis, pregnancy carries an element of risk for mother and child and requires lifestyle changes such as carefully managed nutrition and improved fitness. This is especially true for the woman with CF, as there are simply more things to consider. It is recommended to speak to your CF team if you are planning on starting a family. This is particularly important for a woman with CF who is planning to become pregnant. It is important that you and your partner discuss the implications of becoming pregnant and having a child. Talking to your CF team provides the opportunity to discuss the medical facts, the probable course of the condition, the effect of CF on the pregnancy, the effect of pregnancy on CF and the impact of caring for a baby on treatment and care. Notwithstanding this, all women and all pregnancies are unique and ultimately having a child is a very personal decision for the partners concerned. The journey to parenthood is very rarely straightforward for a person with cystic fibrosis and their partner. Cystic Fibrosis Ireland would like to remind members that supports, both financial and emotional, are available for any person with CF considering starting a family. In this Spotlight, one woman with CF shares her journey towards motherhood and looks back at how cystic fibrosis has impacted her life to date. Hi my name is Edel and I am 34 years old, I have cystic fibrosis and I am recently welcomed my first child, a daughter named Shelby- Rose. I am delighted to share my journey to motherhood with you. I was diagnosed with cystic fibrosis at 6 weeks old. Both of my parents are carriers of the CF gene, but never knew until my brother Seamus was born and was diagnosed. Unfortunately, Seamus passed away from CF at 10 months old. When I was born a couple of years later, I was tested straight away due to the family history of CF. I have 4 older brothers, including Seamus, but none of my other brothers have CF, although one of them does carry the gene. For me, life with cystic fibrosis has been tricky to say the say the least. www.cfireland.ie
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Growing up, my parents managed everything relating to my CF to a really high standard. Looking back now, I can understand their anxieties, they have already lost a child to cystic fibrosis and they were afraid to lose me too. Growing up in the 80s with CF, the future was always uncertain and at often times felt bleak, but my parents never let me worry too much about my condition, they took on that responsibility for me. They took everything that the doctors told them as gospel, made sure they understood everything and kept up to date on the latest treatments and care advice. They were very strict with my routine of medications, exercise and nutrition, making sure I never missed a dose. Their diligence has helped get me to where I am today, I will never be able to thank them enough for all they have done for me over the years. Thanks to their guidance my CF was under control and growing up I was healthy enough. When I was seven, I caught my first bug. Cystic fibrosis was a part of our family, not just a part of me, and my parents talked to me and my brothers about CF and encouraged us to ask questions. So when I did get sick, I knew what to expect. Thankfully I recovered well from this episode. As a child, I had three hospital admissions for IVs or issues relation to my sinuses. CF didn’t impact me much until I was a teenager. At 16 in particular, is when I noticed CF was becoming more demanding. I caught my second bug around this age, but this time my recovery was slower. My treatment regime was expanded to include nebulisers and clearance masks twice a day, every day. I won’t lie, it was a tough adjustment, I would have to get up at 6am to have all my treatments done before school at 9am, but I knew how important the treatments were. I didn’t want to be sick again, I didn’t want to miss out on school and potentially fall behind, I didn’t want to miss spending time with friends and family so I battled through. I think attitude is a huge part of treating CF, it can be tough and draining, but you need to have a mindset of “let’s do this, let’s get it done, kill any bugs that may be lingering and get back to living life”. That was how I approached things back then, and I have carried this with me my whole life. Every person with cystic fibrosis is different, we may all have the same condition, but how CF affects us physically is unique to each individual. But one thing I believe for sure is that we all feel similar thoughts and feelings and have a similar attitude. When I finished school, I was ready for the next stage of my life, I took ownership of my CF care and started to look towards the future. I qualified as a beauty therapist and began working full time. At 23 is when I started to feel the full wrath of CF, I was at a stage where I needed treatment every 8 to 12 weeks. I was no longer in a position to work full time and was forced to give up my job, I was heartbroken. Not only did I love my job, it was it was a key aspect to me living independently. I felt like CF was taking something from me. CF took a lot from me, but it also helped me find what I really want from life I also developed suspected reflux, which the CF team thought was contributing to a steady decline in my lung function. In 2011, I underwent reflux surgery. At the time of the operation, my lung function had decreased by 30%. The operation itself was a success, and to this day I have never had an issue again with reflux. However, the recovery from the procedure was extremely tough, I couldn’t eat or swallow any solid good and had a feeding tube in my stomach to ensure I was receiving sufficient nutrients to aid my recovery. For me, the feeding tube and recovery was worse than the surgery itself, but no matter how painful it was I did everything that was asked of me to recover. Four weeks after the surgery, I had a check-up and was given the all clear. The struggle had been worth it.
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While the issues with my reflux were resolved, my health remained unstable. I was having frequent and recurring chest infections and nothing seemed to be helping. In 2012, I had to have a portacath fitted in my chest. This was a particularly low point for me, I felt like not only was my CF progressing, but it was beating me. I was gutted at the time. However, looking back now I can saw the port was the best decision I ever made. It lessened all the suffering I would have had to endure every few weeks trying to find strong veins, as I’m sure most people with CF will understand you tend to run out of “good veins” after multiple sets of IVs. After a course of IVs, I would feel amazing but the feeling wouldn’t last long. The cycle of good health followed by lows with treatment in between can be draining and it definitely began to take a toll on me, but it was almost something I became accustomed to, it was part of my CF journey and part of my regime. Unbeknownst to me, a change was on the way. Around this time the first CFTR modulators started to arrive and I was put into a trial on compassionate grounds to see if it would help with the constant infections. And it definitely did. My energy increased, I gained strength back and in turn my overall health began to improve. While I was having less infections, I still required frequent course of IVs to help me out, but the time between courses began to grow longer. As I grew stronger, my confidence returned and I was able to start living my life again. Unfortunately, I couldn’t return to the beauty industry due to my health, but I decided to follow another passion of mine, fashion design and I enrolled in a two-year fulltime course. It was a tough adjustment, but CF has taught me that I am stronger than I know. Around the same time, my friend (now husband) Paul moved home from Australia. We have known each other since we were teenagers, we started dating in 2012 and the rest, as they say is history.
He is the most amazing man that I have ever known, and he chose me to spend his life with. He chose to carry the battle of CF in his life, I had no choice. I was born with CF, but Paul choose to love me anyway no matter what the future held for me and us. He is the most gentle, caring, kind, empathetic and loving person I have ever met. When he asked me to marry him, I didn’t hesitate. We married in 2016. In 2014, I qualified with a Fetac level 5 in fashions design. As part of the final showcase, I created my own fashion line which was featured in the college fashion show. I was achieving things I had never imagined possible and felt like I was finally back on top. Just a year later my world was turned upside down, but it wasn’t my health that was the cause. My nephew Adam, aged 22, passed away in 2015 after a hard-fought battle with cancer. His diagnosis came as a huge shock, but I wanted to support him as much as I could. I spent every day with him through his 10 month battle and I will cherish that time forever. Adam was and always will be my hero. www.cfireland.ie
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Grief is the most painful experience and has been the toughest thing I have faced in my life. Losing Adam changed me as a person in every way. I realised more than ever how precious life was. I think psychologically CF had a big impact on my mental health growing up and also personal stuff that went on in my life made me quite fragile in my mind. I think most people with illness will relate to that also. I chose to start working on myself, I began yoga, meditation and journaling and I found it so helpful. All the years I had spent working on my CF treatments and ensuring my body was healthy, I discovered I also needed to take care of my mind. As I worked on my inner self my body started to heal also and my health improved. With a new outlook on life, I decided to follow my dreams once again. My whole life I wanted to be a mother and to carry my own child. However, I knew it wasn’t going to be straightforward for me. I felt stronger than I had in years thanks to CFTR modulators so I felt it was the right time for me so I spoke to my CF team. I will admit I was taken aback when they said I wasn’t strong enough to carry my own baby, but they went through everything with me and they were right. My lung function was less than 60% and while I was feeling well at the moment, I knew the pattern of CF could be unpredictable. It was tough, but dreams aren’t always east. I was determined and set out to improve my health, I changed my diet, introduced cardio to my exercise regime, made sure to drink more water, continued to work on my mental health and used yoga and meditation to work on my breathing techniques. The results didn’t happen overnight, but I trusted the process. My health continued to improved and slowly, but surely my lung function began to increase and I got to almost 70%. I was regularly checking in with my CF team and we reached a point where they were happy that it was safe for me to carry a child. Paul and I were over the moon. We started trying to conceive naturally, but after two years with no success and we decided to get some help and applied for the CFI Fertility Grant to help with the costs. We underwent three cycles of medicated IUIs, but they were all unsuccessful. It was heart-breaking. On top of that the hormone injections caused a flare-up in my lungs, one particular round was so bad that I needed to go on antibiotics. IUI wasn’t working for us. Our next step was IVF. Paul sat me down and said Edel, “I don’t think IVF is for us.” IVF is hard for women who are in full health and Paul had concerns about what the process would be like for me. The process would involve a lot more hormones than the IUI and we weren’t sure how I would handle this. It was a difficult conversation, but it was necessary. The pregnancy journey can be hard, so communication is key. We decided not to go ahead with IVF, but to believe and hope it would happen naturally for us. In 2020, the pandemic turned life upside down. I, like most people with CF, spent the majority of the year cocooning. It was definitely a tough time and I was looking forward to things going back to normal. When restrictions began to ease, I was delighted to start a new job as a courier. A completely different career path than I was used to, but it was a job I took to quickly. Working by myself, it gave me all the protection I needed in terms of COVID-19, but I was delighted to be back working and keeping busy after months of being at home. In November 2020, I began on the latest CFTR modulator Kaftrio.
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Spectrum / Autumn 2021
A couple of weeks after commencing Kaftrio, I discovered I was pregnant. We were so excited. I have no doubt that Kaftrio helped me to achieve this, it made me healthier. But COVID-19 also played a part, cocooning forced me to slow down and take stock. I don't think I admitted it at the time, but the fertility process had taken a toll on me mentally. It was something I wanted so badly and I was putting myself under a lot of stress. It was also a rollercoaster of emotions. I was disheartened every month, but tried my best to stay positive in the hopes that our dream would come true. Taking the time to slow down, accept what was and reflect was something I needed and the pandemic gave me that opportunity.
Being pregnant during a pandemic has been stressful. Having had such a long journey to conceive, I wanted to take every precaution so I gave up my job to focus on my pregnancy. It wasn’t an easy journey, but one thing I never stopped was believing that I would carry and have my own baby. And here I am. Our daughter Shelby-Rose was born on Wednesday, August 11th and we are so in love with her. I was lucky enough to have a natural birth and my lungs felt so strong throughout the labour. My life has had very difficult times and it has also been absolutely amazing but I wouldn’t change a second of it because it led me to here. I believe I am who I am and am where I am because of an abundance of love and support from Paul and my family Thanks for taking the time to read this Love,
Edel
Congratulations Edel and Paul on the birth of your beautiful daughter. Welcome to the world Shelby- Rose. We will have a follow up on Edel’s journey with motherhood on our website in the coming months, but you can continue to follow Edel’s story on Instagram @Bravely_battling_ www.cfireland.ie
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Back to School W
ith return to school preparations underway, Cystic Fibrosis Ireland are aware that for children with CF and parents of children with CF this may be a particularly anxious time. Many children with CF have not been in school full time since March 2020. While home schooling ensured education continued, absence from the classroom may have resulted in the loss of some social skills and with COVID-19 restrictions and precautions in place some children may have developed anxiety about being in a crowded setting. If you feel your child could benefit from speaking to someone to help deal with their anxieties, CF have a counselling grant that you can avail of. For further information on this please contact Samantha Byrne, Senior Member Services Coordinator on 01 496 2433 ext. 201 or email sbyrne@cfireland.ie Aside from COVID-19, cystic fibrosis itself can play a part in anxiety returning to school. Children with CF may be more ‘noticeable’ in school because they may cough more often than others. In particular with the pandemic, this may be a cause of stress for your child. CFI would recommend talking to your child and to the teacher on how to best manage this. The teacher may wish to address this with the other children in the class, with the child’s and their Parent/guardians’ permission. For children who are transitioning from primary school to secondary school, there may be added worries. Adjusting to a new setting, potentially new classmates and a new schedule with longer hours can feel overwhelming. Unlike primary school, your child / children will have more than one teacher and as such it is important the school is aware of your child’s condition so allowances can be made for any additional supports that may be needed. Many schools would have excellent and well-established procedures for passing on information, but it is always worthwhile checking. As parents of children with CF you have learned to cater to your children’s needs. For many children / teenagers secondary school is the beginning of their independence. As a parent this can be an uncertain time as your child adjusts to a new setting while also taking control of their condition. Teachers can provide invaluable reassurance by making a special effort on a regular basis. PRACTICAL HELP In cases of advanced chronic lung damage and also during exacerbations, the level of lung function can drop significantly. Accessibility may become a permanent obstacle and concern for children with CF where climbing the stairs and walking long distances may become too tiresome. For example, an alternative to the use of stairs such as a lift or ramp should be in place where classrooms are located on second and third stories. Alternatively, arranging class time tables to ensure all classrooms are relatively close to each other can also help. Frequent coughing fits will occur, even when a child is in good health. Teachers should be understanding in allowing the student to access to cool drinking water in the event of such happening. There are often small ways to limit the amount of coughing by a student. These could include having suitable seating arrangements where they are away from open windows and radiators. Also, ensuring that the classroom is as clean as possible to prevent the accumulation of dust particles is a great way to limit the large amount of coughing. An allowance should also be made for frequent bathroom breaks for students with CF who may experience sickness from an upset stomach as a result of pancreatic insufficiency or related CF problems.
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HOME TUITION A number of children with CF will, at times, experience unavoidable absenteeism from school due to illness and hospital stays on occasion. Even children with CF who are well, will need to attend regular outpatient clinics and annual review assessments. For this reason, they may need additional home tuition in order to catch up with work they have missed. Home tuition is intended to provide education for children with a significant medical condition causing major disruption to their attendance at school. An application for home tuition can be made through the school at which the child is present. Forms are available from the school principal or from the Department of Education and Skills. You can also contact the Special Education Section of the Department at special_education@education.gov.ie or at (090) 648 4187. FLU VACCINE As your child prepares to return to school, you should consider taking up the offer of a free ‘flu vaccine to protect them over the winter months. All children aged 2 to 17 in Ireland will be offered a free vaccine from their GP or pharmacy. It is important that your child avail of it in order to protect themselves and other high risk members of the family. Flu spreads easily and infects both children and adults. Most children who get the flu have mild symptoms. But every winter some children can get complications of flu. Children are more likely than adults to get severe complications of flu. Children who are sick with flu miss days in crèche, childcare and school. They also miss out on their usual activities such as hobbies and sports. The flu vaccine will help protect your child against flu and reduce the spread of flu to others. The vaccine is given as a single spray in each nostril of your child's nose. Your child can breathe normally while getting the vaccine. There is no need to take a deep breath or sniff. The vaccine is not painful and is absorbed quickly. It will work even if your child has a runny nose, sneezes or blows their nose after the vaccination. Most children need only 1 dose of the vaccine each year. Some children aged 2 - 8 years with chronic health conditions like chronic heart or lung conditions may need 2 doses if they have never had a flu vaccine. The doses are given 4 weeks apart. If your child is 6 months to 2 years of age and is in a high-risk group for flu, they'll be offered a flu vaccine injection. This is because the nasal spray is not licensed for children under the age of 2. Your child can get the vaccine at your GP or pharmacy from October until the middle of February 2022. FURTHER INFORMATION CFI have published a leaflet on School and CF which you can download from www.cfireland.ie. If you would like a hard copy sent to you or your child's school by post, please contact the office on 01 496 2433 or email info@cfireland.ie We will also be producing a 1-page insert on School and Covid-19 over the next couple of weeks. Please check our website and social media channels for more information on this. Some general advice on returning to school in September 2021 can be found on the gov.ie For information on the flu vaccine please visit www.hse.ie/flu Cystic Fibrosis Ireland can be contacted by parents or teachers who may need further support and advice. www.cfireland.ie
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Back to School Advice for Parents of children with CF Most students will be looking forward to going back to school and will feel happy and excited. Some may feel a little anxious about returning. This is a normal response during a time of change. Here are some tips and advice on helping your child / children adjust to the return to school.
Establish A Routine Return to a set bed time and try to get up earlier. This way it won’t be such a shock to the system when school starts back and will ensure everyone has enough rest for the day ahead.
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Pack It Up
Talk
Make a list of everything that is needed for going back to school. Get everything ready in the week / days before school starts so that it becomes something to look forward to.
Make time to speak to your child about how they are feeling about returning to school. Explain to them some of the changes that may be in place including social distancing, masks and increased hand hygiene.
Be Informed
Plan Your Journey
Contact the school in advance of your child returning to inform the staff of your child's CF and learning about the school procedures around COVID-19. Keep up to date on advice for children with CF on www.cfireland.ie
If possible, encourage your child to walk or cycle to school. If public transport is needed ensure they have a face mask. Check transport times & capacity as some services may be operating at reduced occupancy.
A New Way to Say Hello
Social Distancing
A Helping Hand
The school may have additional measures in place to protect students & staff including no contact. Let your child know this in advance so they aren't confused as to what is happening.
Your child may be placed in a pod within their class and / or social distancing in the classroom may be in place. You can contact your school in advance so you know what safety measures are in place.
Remind your child about the importance of hand hygiene. You can provide your child with their own hand sanitiser, but there should be stock in the school also.
Spectrum / Autumn 2021
Why do you need flu vaccine? Flu isn’t just a cold
Flu can cause pneumonia and bronchitis and can make chronic health conditions worse.
Flu is serious
Up to 500 people die from flu every year in Ireland.
Flu vaccine protects you
You should get the flu vaccine if you: • are 65 years and older or • have a chronic health problem such as heart or lung disease or • are pregnant
Flu vaccine protects others too
You should get the flu vaccine if you are a healthcare worker or a carer or if you live with someone with a chronic health condition.
Flu vaccine is free for people at risk
Talk to your GP (doctor) or pharmacist today about getting the flu vaccine.
Vaccine to boost the immune response in adults aged 65 and over
As we get older our immune system may not respond to vaccines to the same extent. The adjuvanted flu vaccine (available specifically for adults aged 65 years and over) contains an ingredient to create a stronger immune response.
hse.ie/flu Public Health Advice www.cfireland.ie Order Code: HNI01089
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Research News Research content by Dr. Sarah Tecklenborg, Senior Research & Policy Coordinator, CFI
Public and Patient Involvement (PPI) in Research
Your Voice Matters
Cystic Fibrosis Ireland invites people with cystic fibrosis to have an active say in the research and initiatives which impact them. CFI will be recruiting a panel of PPI contributors to support the work of the research team and research groups around Ireland. The panel is open to people living with CF aged 12 and over and the family members of people with cystic fibrosis. PPI contributors will be reimbursed for their time where possible. What is PPI? Public and Patient Involvement (PPI) in CF research is research which is carried out ‘with’ or ‘by’ members of the CF community rather than ‘to’, ‘about’ or ‘for’ them. PPI activities recognise that the lived-experiences of people with CF and their families can contribute additional, valuable expertise and bring novel insights to research. The inclusion of people living with CF in the design of research allows researchers the opportunity to gain different perspectives and identify questions which matter the most to the CF community. This improves the quality and relevance of the work ensuring it will have the greatest impact on those living with the disease.
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What might PPI contributors be asked to do? PPI contributors are not participants in a research study, they are actively involved in the decisions made about the research. PPI contributors are valuable members of the research team bringing a wealth of expertise from their lived-experience of Cystic Fibrosis.
PPI contributors can be involved in the planning, design, implementation, management, evaluation and dissemination of research projects and may be asked to: • Help inform research priorities or identify research questions of interest to the CF community • Help to clarify the research question • Co-design the project or comment on the suitability of chosen research methods • Develop research material such as leaflets, information sheets, survey tools and questionnaires, ensuring the language used is easy to understand and that all the necessary information is provided • Assist with the recruitment strategies for a project • Assist in collecting data or carrying out interviews or focus groups • Represent the CF community on project steering groups • Assist the research team to interpret data and identify themes • Help to write up dissemination materials to share the research findings back to the CF community • Share their knowledge and views on where and how best to share the findings There is a growing interest in public and patient involvement in research by both funders and research organisations. Cystic Fibrosis Ireland is committed to ensuring the voice of people living with CF is at the forefront of research we are involved in. How can I get involved? If you are interested in becoming a member of the panel or would like further information please contact Sarah on stecklenborg@cfireland.ie or call 01 4962433.
www.cfireland.ie
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RESEARCH UPDATE Cystic Fibrosis Ireland caught up with Dr. Audrey Tierney, Senior Lecturer and Discipline Lead in Human Nutrition and Dietetics in the school of Allied Health University of Limerick, to hear how her work on diet quality in CF is going. Dr. Tierney was awarded a grant through the Health Research Charities Ireland/Health Research Board Joint Funding Scheme in partnership with CFI to investigate diet quality in people living with CF in Ireland.
What is the Title of your Research? The title of the research project is ‘Exploring diet quality in Cystic Fibrosis – enablers and barriers to eating a healthy diet in CF’ Who are your co-authors/collaborators? The project is being funded by Cystic Fibrosis Ireland and the Health Research Board and it involves researchers in the University of Limerick. We have CFI ambassadors as collaborators on the project also to guide the research and to make sure that the findings get reported to the CF community. What are your research questions for this study? The research project is aiming to answer these questions: • What are the typical diets consumed by people with CF living in Ireland? • How is diet related to overall health in CF? • What are the barriers for people with CF to eat a healthy diet? How is this question important to PWCF? People with CF have typically been encouraged to consume a diet that is high in energy to meet nutritional requirements. Trying to eat a diet high in energy can often mean that diets can be high in sugar and fat, or that the quality of the diet is not as good as it should be for overall health. With advancements in care and treatments in CF we want to assess the need for this high energy diet in CF and if weight, lung function and overall better health can be achieved and maintained with a healthy eating diet that is advised for the general population. But we don’t have Irish or even European data on what people with CF are currently eating and how this relates to health and wellbeing. This research is really important to inform practice guidelines and next steps in terms of what we advise for people with CF in terms of best types of diets to consume.
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What have you done so far and what did you find? We have completed a review of the literature which shows that people with CF all around the world have tended to eat a diet that is high in energy and fat to meet the advised requirements. In some instances the literature has shown that adults with CF eat a diet that is high in sugar and low in fibre, fruits and vegetables to meet these requirements. However, these studies are quite old. We are aiming to get approximately 150 people with CF to complete this research study where we look at current dietary patterns. It is a short study that takes participants about 30 minutes to complete. What are the potential benefits that this research may bring to PWCF? We hope that this research will allow us to relook at the dietary recommendations for adults with CF. We are interested to see if people with CF can eat a healthy diet and to find out what are the barriers to eating healthy with CF so that we can focus on efforts to address this. How do you find working in the field of CF research in Ireland? I have worked in the area of CF for the past 12 years both clinically and in research. It has been great to see the advancements in CF in the area of new medications and new treatments coming on board. I think we need to focus on the person as a whole and to ensure that quality of life and all aspects feeding into quality of life are equally considered i.e. diet, physical activity, mental health, sleep etc What are the main challenges? The main challenges are to ensure that the person with CF’s voice is heard in research and practice. That is why we have partnered with CFI for this research project and we are hoping as many adults with CF take part to ensure we capture the most relevant data. What does the future hold for you? After this research project I would love to look at carrying out an intervention study to see if people with CF could eat a Mediterranean type diet here in Ireland (this was the focus of my research in Australia with people with other chronic diseases) and to see if it improves some symptoms and overall quality of life. Watch this space!!
GET INVOLVED To participate in this research study register your interest at www.cfireland.ie The survey will take approximately 30 minutes to complete. Cystic Fibrosis Ireland encourage members to take part in this research project if they can. It is imperative we support research that aims to improve current methods of treatment and management of the disease as well as enhance the quality of life of those living with the condition.
www.cfireland.ie
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CONFERENCE REPORT
The impact of Basic Science on the development of new therapies for CF At the 44th European Cystic Fibrosis Conference, held digitally from the 9th-12th of June 2021, Prof. Luis Galietta, Associate Professor of Medical Genetics, University of Naples gave the closing plenary lecture discussing the impact that basic science developments have had for new therapies for CF. Basic research, also known as fundamental research, is a type of scientific research which aims to get a better understanding, or to increase the scientific knowledge base, of a subject. It is undertaken primarily to acquire new knowledge about the underlying foundations of something without any particular application or specific use in view. Basic research has provided the important scientific knowledge and technological developments that have led us to the CFTR modulator era and offers potential new future therapies for Cystic Fibrosis. Orphan mutations and precision medicine Despite the availability of modulator therapy there remain a substantial number of people living with CF who have rare mutations with unknown sensitivity to correctors and potentiators. It is not possible or financially feasible to develop specific drugs for each one of these mutations. A method of precision medicine, which uses cells derived from an individual patient makes it possible to evaluate, in vitro, if CFTR function can be rescued using existing available treatments. This approach has been made possible by basic science studies which have provided information on how to adequately culture cells from intestinal samples. Prof Galietta discussed studies which have identified the important biological pathways, requiring moderation by cofactors and pharmacological agents, to obtain the effective growth of sufficient cells from a sample. These cells can then form organoids, organoids are 3D multicellular tissues which mimic the corresponding organ that they came from and allow it to be tested in a lab environment. CF researchers have shown that these organoid cell models can be used to evaluate CFTR function, the organoids will visibly swell if the tested CFTR modulator has successfully rescued CFTR function.
Organoid Generation
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CFTR function testing in organoids
Spectrum / Autumn 2021
There are several CF studies in progress now with the goal of obtaining precision medicine in CF. These projects use nasal epithelial cells obtained by nasal brushing, or intestinal epithelial cells obtained by rectal biopsy to generate tissue models in the lab. These tissue models can then be used to test the efficacy of various therapies to give an indication of the most appropriate treatment for an individual patient, personalised medicine. Even with these new approaches however, there will still be some patients who do not benefit from currently available CFTR modulators, some of these people will have nonsense CFTR mutations. There are indications that it may, in the future, be possible to rescue CFTR in these people however safer, more effective compounds need to be identified. Basic science will be needed to clarify the molecular mechanisms underlying the pathways by which nonsense mutations impact CFTR. It is likely that people with nonsense mutations will require support from numerous therapeutic strategies including correctors, potentiators and amplifiers. CFTR interactome Basic science is being used to investigate the CFTR interactome. This is a very active field of research in CF. It refers to the complex array of proteins inside a cell which are acting in a positive or negative way on the processing, transport and degradation of the CFTR protein. Identifying and understanding how these proteins interact in the cell may help to find new ways to improve the rescue of mutant CFTR protein. CRISPR Gene Editing The discovery of CRISPR/Cas9 by Emmanuelle Charpentier and Jennifer Doudna has revolutionised gene editing. CRISPR is a group of DNA sequences which play a key role in the antiviral defense mechanisms of bacteria. Dr Charpentier discovered CRISPR while studying the bacteria Streptococcus pyogenes. The bacteria use it as a form of acquired immunity, to recognise and prevent reinfection from bacteriophage (viruses that infect bacteria) by cutting or cleaving the DNA sequence of the invading virus. Charpentier and Doudna, who won a Nobel prize for their work in 2020, recreated the bacteria’s genetic scissors in the lab and showed it could be controlled to cut any DNA molecule at a predetermined site. CF researchers hope that in the future it may be possible, using CRISPR/Cas9 to permanently fix the CFTR mutation in patients. There are however many obstacles to this. In order to permanently fix the genetic defect researchers must target the basal stem cells in the epithelia. Basal stem cells are the progenitors of airway epithelium. These cells can differentiate and replenish all of the epithelial cell types as necessary. Fixing the genetic mutation in stem cells means that every cell that derives from these stem cells thereafter will have a functioning CFTR. These stem cells are deep within the epithelium and are difficult to reach. Gene editing may require the correction of these stem cell in vitro (outside of the body) and then the engraftment of these edited stem cells into the epithelia of patients. Alternative Targets in CF Basic science is also working to identify alternative targets which may be treated in CF. Transepithelial ion transport involves the coordinated activity of other channels, not just CFTR. As basic science provides a greater understanding of these other ion transport mechanisms researchers may find ways to bypass or compensate for the defect in the CF transmembrane conductance regulator. This strategy may be particularly beneficial for people with CFTR mutations for which the CFTR modulators will not work, such as those with frameshift mutations or gene deletions. www.cfireland.ie
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My Legacy Month - November My Legacy month is as good a time as any to encourage others to make a Will and consider leaving a legacy gift to a cause they care about such as Cystic Fibrosis Ireland. Having a Will is very important for so many good reasons and particularly during My Legacy Week, when people are encouraged to take that first step in writing their Will by making an appointment with a solicitor. Cystic Fibrosis Ireland are a member of My Legacy.ie who have the support of hundreds of solicitor firms all around the country who can offer expert guidance and advice about making a Will at any time of the year. It is usually a much more straightforward and cost effective process than people might think and a solicitor will discuss any fee for drawing up this important personal document when you make an appointment. Once family and friends have been looked after and all other important personal decisions have been made, deciding to leave a legacy gift to a charity is a wonderful way to support a favourite cause such as Cystic Fibrosis Ireland in the future. Large or small, every legacy is a generous gift of hope and trust for the future. We understand that leaving a legacy is a very private decision. Some people choose to let us know that Cystic Fibrosis Ireland has been remembered in their Will. Others prefer to keep their desires private, leaving it to their executor or solicitor to let us know when the time comes.
1. Make An Appointment Avail of expert advice and support. Take the first step to discuss your wishes and decisions for the future. 2. Look After Loved Ones First Why a Will is important A Will provides for loved ones, assigns guardians, protects your assets and helps reduce inheritance tax. 3. Consider A Legacy To Charity Your gift may be big or small and is tax free. If you have a cause close to your heart such as Cystic Fibrosis Ireland, please consider leaving a gift to that charity in your Will. Many people can afford to give far more in their Will than they ever could when they are alive.
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By leaving a gift to Cystic Fibrosis Ireland people can help us to; • Fund pioneering research, so that one day CF can stand for ‘Cure Found’ • Fund specialist Cystic Fibrosis multidisciplinary posts in hospitals throughout Ireland • Provide funding for Cystic Fibrosis units around the country, including dedicated in-patient, day care and outpatient facilities • Help people with Cystic Fibrosis directly with Exercise, Transplant, Fertility Counselling and Bereavement Grants • Provide advice, information and advocacy services to help people with Cystic Fibrosis
A legacy gift will live on to support future generations. For more information on leaving a gift in a Will, please see the enclosed leaflet “Your Legacy Could Be Life Changing”, visit our website at www.cfireland.ie or the My Legacy website www.mylegacy.ie. Alternatively contact Cystic Fibrosis Ireland on 01 496 2433/ info@cfireland.ie for more information.
Take the time to make a Will
www.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) 496 2433 or email fundraising@cfireland.ie September 5th: Head2Head Walk Thanks to the amazing support from walkers across Ireland and Overseas our Virtual Head2Head walk in 2020 raised over €30,000 for Cystic Fibrosis Ireland! We are delighted to confirm we are returning to our actual Head2Head walk this year and encourage everyone that can to join us on Sunday 5th September and help raise vital funds for Cystic Fibrosis Ireland? The walk will begin at Howth Dart station at 9am and finish in Bray between 4 and 6pm. Walkers have the option of completing the full walk from Howth Head to Bray Head or completing half the walk, either the first half from Howth Head to Sandymount Strand or Sandymount Strand to Bray Head. For anyone starting at Sandymount Strand, the walk will commence at 12.30pm. Due to Covid-19 guidelines we will not be using the hotel in Bray this year and the walk will finish beside the hotel along the sea-front. Light refreshments and snacks will be available in Howth, Sandymount and Bray.
With your walk registration you will receive your registration pack including your stylish Head2Head Walk Shirt and everyone who completes the walk will receive an exclusive Head2Head Walk medal. Register now to secure your place at cfireland.ie. A huge thank you to everyone who has registered and fundraised for the event to date.
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September 10th to 16th: Paris2Nice Cycle This 700km charity cycle always proves to be an enjoyable and rewarding challenge! This is a wonderful way to make new friends, get fit and have a holiday of a lifetime all the while raising much needed funds for Cystic Fibrosis Ireland. The cycle will pass through amazing scenery as it makes its way from Paris along the quite country roads towards Nice. The 2021 event is now fully booked! For further information or to register your interest for the Paris2Nice Cycle 2022 log on to cfireland.ie or contact fundraising@cfireland.ie
September 16th to 19th: Malin2Mizen Cycle4CF Our annual Malin2Mizen Cycle4CF will take place on Thursday 16th to Sunday 19th of September 2021. We would like to thank everyone who has signed up and fundraised so far! Malin2Mizen Cycle4CF will begin at Malin Head in Co. Donegal on Thursday 16th September and will finish in Mizen Head in Co. Cork on Sunday 19th, September. The event requires plenty of training as we will be covering an incredible 640km in 4 days! Registration for our 2021 event has now closed, but If you are looking for a rewarding fitness challenge and are a keen cyclist then this is the event for you! You can register your interest for our 2022 cycle which will take place from Thursday 5th to Sunday 8th May by contacting us on 01 496 2433 or email fundraising@cfireland.ie. All participants must provide a deposit of €250 to secure their place and are asked to raise €2,000 in total. This will cover your food, accommodation and insurance for the 4 days of the cycle. All funds raised will go to Cystic Fibrosis Ireland to help us continue to support people living with Cystic Fibrosis. www.cfireland.ie
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September 27th to 30th: Donegal & Derry Walk As we were unable to hold an International Walk again this year due to Covid -19, we are delighted to inform you that the International Walk Committee have organised a walk in Ireland for Cystic Fibrosis Ireland in September. The walk will take place in beautiful Donegal and Derry from September 27th to the 30th.The walkers will go visit the Ulster American Folk Park, Glenveagh Castle, Glenveagh National Park, Grianan Aileach, Derry and many more wonderful sites during their time in the Northwest. We are delighted to confirm that the International Walk will return in September 2022 and anyone interested in joining the walk in Bulgaria should register their interest now at www. cfireland.ie, email fundraising@cfireland.ie or phone us on 01 496 2433.
October 3rd: Virgin Money London Marathon
October 24th: KBC Virtual Dublin City Marathon
The London Marathon is known as the "Marathon of a lifetime" and is a hugely popular event and takes place on Sunday, 3rd October! Those who have run the Marathon in the past describe it as a life changing experience. The Virgin Money London Marathon takes participants past many of the amazing landmarks in London including crossing Tower Bridge, passing the London Eye and Big Ben, and finishing in front of Buckingham Palace. Places are now fully booked for the Virgin London Marathon 2021!
The Dublin City Marathon takes place virtually again this year on Sunday, October 24th. Our runners will complete the marathon in a venue of their choice to help raise funds to improve the lives of people living with CF in Ireland.
The London Marathon is one of the world’s greatest participatory events, so we would encourage you to register your interest at www.cfireland.ie as soon as possible if you wish to secure a place in the London Marathon 2022! Good luck to all our participants in the London Marathon this year!
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Registration is open for the KBC Virtual Dublin City Marathon at kbcdublinmarathon.ie. Once you secure your place and have started your training, why not consider fundraising for a charity as part of your marathon challenge? We would be delighted if you could fundraise for Cystic Fibrosis Ireland and join the CFI Team taking part! Register now at www.cfireland.ie and we will send you out a Cystic Fibrosis Ireland shirt so that you can be part of the Cystic Fibrosis Ireland Team for the KBC Virtual Dublin City Marathon 2021.
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November 7th: TCS New York City Marathon Complete one of the world’s most exciting Marathons and raise much needed funds for Cystic Fibrosis Ireland! The TCS New York City Marathon takes place on Sunday 7th November, and takes you past towering buildings, bustling streets and world-famous iconic landmarks in the city that never sleeps! If you are considering taking part in the TCS New York City Marathon, this is your last chance to express your interest at www.cfireland.ie to secure a much sought after ‘Guaranteed Entry Place’ for the event. All Year: International Treks Cystic Fibrosis Ireland facilitate treks to Kilimanjaro, Machu Picchu and Everest Base camp each year and there are a number of treks running in 2021. Taking part in of one of Cystic Fibrosis Ireland's International Challenges could be the adventure of a lifetime that you have being looking for and the perfect fundraising opportunity for adventurous spirits! To find out more, please register your interest now at cfireland.ie
December: Christmas Cards Stay in touch this Christmas with a Cystic Fibrosis Ireland Christmas Card! Our Guaranteed Irish Christmas Cards are available online at www.cfireland.ie for just €6.99 per pack of 8 cards! We have 15 different beautiful designs to choose from as well as 3 variety packs. This is a lovely way to stay in touch with your friends and family this Christmas and don’t forget during these difficult times, postage is FREE to and from Nursing & Care Homes. Best of all, the proceeds raised allow us to continue to provide much needed support and services to people with Cystic Fibrosis all across Ireland.
www.cfireland.ie
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December 10th: Christmas Jumper Day 4 CF On Friday 10th December, we are encouraging as many workplaces as possible nationwide to support Christmas Jumper Day 4 CF in their places of work or virtually again this year! Interested in getting involved? All you need to do is… 1. Ask your work colleagues to support Christmas Jumper Day 4 CF on Friday 10th 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 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 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! 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. After the event you can; •
Lodge the monies raised: IBAN: IE59 AIBK 9310 7108 5785 93 BIC: AIBKIE2D
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Post a cheque to Cystic Fibrosis Ireland, 24 Lower Rathmines Road, Rathmines, Dublin
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Donate online at www.cfireland.ie
(Please remember to include your company's name as a reference so we know who it’s from) Don’t forget register your interest to take part in Christmas Jumper Day 4 CF at cfireland.ie and we will help you organise everything for your Christmas Jumper Day 4 CF 2021 – Ho Ho Ho!
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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: One in 1000 / Virtual Women's Mini Marathon We would like to thank each and every one of you who took part in this year’s One in 1000 Virtual Women’s Mini Marathon on Sunday, 6th June. We were overwhelmed with the response and with over €40,000 so far. We are really looking forward to getting back to meeting you all in person at the actual Women’s Mini Marathon on the Bank Holiday weekend at the start of June next year. One person really can make a difference!
www.cfireland.ie
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65km Cycle for 65 Roses Day A big thank you from Cystic Fibrosis Ireland goes out to Austin Kenny and his friends for doing their annual 65km Cycle for 65 Roses Day in July. They raised a fantastic €3,325. Little Ruggers Preschool The children in Little Ruggers Preschool in Ashbourne which Darragh Lynch (PWCF) aged 5 attends, had great fun taking part in their 65 Roses Challenge, a sponsored walk for CF Ireland and the staff and parents were incredibly supportive raising an amazing €1,531.10! Pictured left presenting a cheque to our Fundraising Manager, Fergal Smyth is Darragh’s teacher Deirdre Crowley and Marese Bailey. SISK A huge thank you to the employees in SISK, Little Island, Cork for their fundraising for 65 Roses Day. They recently presented a cheque for €1,717.50 to our Fundraising Manager, Fergal Smyth on the site of one of their major construction projects in Cork.
Malin2Mizen Cycle Fundraiser A huge thank you to David, Brian and Derek who raised an unbelievable €11,603 from their Malin2Mizen Cycle in aid of CFI on Saturday 17th July in memory of Ronan “Pep” Cahill. They were joined by a great support team who can be seen in the photo below including none other than Dave “Pius” Keane, John “Rash” Bacon, Mal “Chainsaw” Mullally And Paddy “The Bull” Corkery who went above and beyond in supporting the 3 cyclists.
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Dublin Plein Air Festival
O’Flynn Group, Cork
A big thank you goes out to everyone who participated and donated to the quick paint Cystic Fibrosis Ireland charity event held on the final day of the Dublin Plein Air painting festival in Skerries on Sunday 25th July. A wonderful €1150 was raised from this annual fundraiser.
A huge thank you to everyone in the O’Flynn Group who raised an incredible €10,484 from their virtual 65 Roses Challenge.
Pictured above are Paul Darcy (Dublin Plein Air Festival) presenting a cheque to Philip Watt (CEO, CFI).
“For the month of March, O’Flynn Group decided to take part in the wonderful 65 Day Roses challenge and create our own virtual event! Not only is it a fantastic way to raise funds for such a worthy charity but it also is a great fun activity to do together as a team particularly while we‘re working remotely. We have gone above and beyond our expectations and have raised an amazing €10,484! Credit to all involved…!" Pictured below at the socially distant cheque presentation are; Front row (L to R) Fergal Smyth, Fundraising Manager (CFI) with organisers Orla Kelleher & Louise O’Sullivan Back row (L to R) Tom O’Driscoll, Kevin Lucey, Linda O’Driscoll, Paul Maher
Rush Dip 4 CF
Johnsgirl Bags
A huge thank you to Marlene Finn, Niamh Conway, Clodagh, Jimmy, Alan and Cara for organising the Rush Dip 4 CF which raised a massive €3408.10.
A huge thank you to Agnes Clarke who recently raised €1,156.11 for Cystic Fibrosis Ireland. She works tirelessly sewing bags which she then sells to raise funds for Cystic Fibrosis Ireland (and three other charities). Her nephew, Matthew O’Connor, age 14 years, is a PWCF.
It took place on Sunday 25th of July with beautiful weather and an electric atmosphere. www.cfireland.ie
Her charity bags are called ‘Johnsgirl Bags’ after her late husband, who sadly passed away this year. Follow them on Facebook for more information.
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Length x Breath
I
n July, a group of eight friends completed a mammoth fundraiser in aid of our regional branch TLC4CF. Length x Breath was a 10-day running and cycling challenge held in memory of Cathal Scanlon, who sadly passed away from cystic fibrosis in 2020 aged 21. The challenge started early on Tuesday, July 6th when organiser Padraic O’Neill set off from Sandymount on a solo run across the breadth of Ireland, the equivalent of five back to back marathons. Padraic successfully completed the run in five days before jumping on his bike to join the rest of the group for a five day cycle the length of Ireland from Mizen Head in Co. Cork to Malin Head in Co. Donegal, a distance of over 670km. The idea behind the Length x Breath challenge was to mix the pain which Cathal endured and suffered and his love of cycling. The challenge was not only completed in his honour, but in celebration of what would have been his 22nd birthday. Cathal’s parents, Sean and Carmel were involved with the establishment of TLC4CF in 2008. The regional branch was set up to improve local CF services at University Hospital Limerick. The Length x Breath team chose TLC4CF as the chosen charity to continue the legacy of the Scanlon family. TLC4CF and Cystic Fibrosis Ireland would like to congratulate Paraic O’Neill, Eoin Ryan, Bryan Curtin, Jack Canty, Evan O'Donoghue, Luke Mackessy, Sean Fitzpatrick and William Lynch on completing this magnificent challenge. On behalf of the participants, we would like to extend thanks to the people behind the scenes who helped make the event happen including the Adare Cycling Club for their support, guidance and support cyclists who joined the group on some of the journey through Limerick. Additional support services were provided from other friends and Cathal’s family, particularly his brother Darragh Scanlon. Their assistance made the planning and organisation of this event possible. Thank you also to everyone who came out to cheer the team along the way, your support and encouragement meant a lot to the team and also to Cathal’s family. To date, the challenge has raised over €50,000. A fantastic fundraiser, a gruelling challenge and a magnificent memorial for Cathal. We have put together a selection of photos which to celebrate the achievement.
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www.cfireland.ie
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Christmas Jumper Day 4 CF Friday 10th December Sign up now at cfireland.ie or contact 01 496 2433 / brendan@cfireland.ie for more information
Cystic Fibrosis Ireland 24 Lower Rathmines Road, Rathmines, Dublin 6, D06 A9P3, Ireland
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t: +353 1 496 2433
e: info@cfireland.ie
w: www.cfireland.ie
Company Reg: 449954
Charity No: CHY6350
CRA Number: 20011376
Spectrum / Autumn 2021