Spectrum Autumn 2018

SPECTRUM Autumn / 2018

Head Count Record number of participants take part in the annual Head 2 Head Walk

www.cfireland.ie

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CONTENTS Latest News | 1-3 * Organ Donation Consent * Drug Therapies Update: Symkevi * New Antibiotics Available in Ireland

Spotlight | 4 -10 Education * A Father’s Perspective * School with CF * A Class Act * Tell me I can’t, I’ll Show you I can * Roll Call - Back to School Photos

Hospital Hub |11 - 13 * My CF Story * What a Difference a Decade Makes

Kids Corner | 14 Youth Reach | 15 Member Services| 16 * Noticeboard

Research | 18 - 20 * Health Research Board Study * Science of CF Survey * Non Tuberculous Mycobacteria * Genetics Based Precision Medicine * Orkambi in Australia * Social Policy Update

Fundraising |21 - 29

CEO’s Message We have a wide range of interesting articles in this issue of Spectrum.

Philip Watt Chief Executive

The news that Minister Harris will commence the legislative process to introduce a new form of organ donor consent (Soft Opt Out) on 1 October 2018 is to be warmly welcomed.

Once this has been completed it will be presumed that most people living in Ireland will want to donate organs but next of kin will still have the right of veto. This will bridge the gap between intent and practice; 85% of us want to donate organs, but only one third of us carry an organ donor card.

The bottom line is that this system has been shown to improve donation throughout Europe and more organs will mean more have ‘the gift of life’.

Also in this edition of Spectrum is a focus on ‘back to school’, the new app developed jointly between Dr. Des Cox in Crumlin and CFI which is proving very popular; a nurse’s perspective of CF Care; a focus on research and of course an update on some of our fundraising events.

As winter approaches, don’t forget to update your ‘Flu jab’.

Our key objective this term is to ensure the commencement of the building of the long awaited 20 rooms in Beaumont Hospital.

Philip Watt, CEO, CFI Sam Byrne & Nuala McAuley, Editors

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

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LATEST NEWS: New Organ Donation Consent will improve the chance of a ‘Gift of Life’ As TDs get set to return from the summer recess, and in wake of RTE’s excellent documentary on Orla Tinsley’s transplant, CFI renewed its call on Government for the introduction of presumed consent organ donation legislation – also known as soft opt-out organ donation – to improve transplant rates in Ireland.

The call comes against a backdrop of most recent figures showing 600 people waiting for potentially life-changing organ transplants in Ireland in 2017. Ireland lies in 15th place out of 28 European Union countries when it comes to organ donation, despite very high levels of public support.

International evidence has confirmed the positive impact of opt-out consent on deceased organ donor rates. Over a three-year period, after making the change to an opt-out system, Belgium saw its rate of organ donation increase by 100 per cent. The recent experience in Wales with soft opt-out has also been very positive. The countries with the best rates of organ donation in Europe have some form of soft opt-out system, combined with sufficient infrastructure.

Introducing a soft opt-out organ donor consent system will undoubtedly save more lives and help to ensure our organ donor system is fit for the 21st century.

Ireland is one of the last countries in Europe, along with Scotland, Northern Ireland and England, not to have presumed consent when it comes to organ donation. The number of people awaiting life-saving organ transplants in Ireland is more than double the operations carried out.

Bringing in this new legislation will also bridge the gap between people’s intentions and their behaviour. 85 per cent of people in Ireland are willing to donate organs but only one third of us carry an organ donor card and many of us do not inform our next of kin of our intentions to donate or otherwise.

Organ donation will always be a ‘gift of life’. With this proposed new system, next of kin will continue to be consulted so no family will be forced to donate the organs of a loved one should they not wish to do so. There will also be an online register for opting out of organ donation.

We are delighted to let you know that Minister Simon Harris recently tweeted his commitment to implementing new legislation. www.cfireland.ie

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Drug Therapies Update: News on SYMKEVI® and an important generic anti-biotic drug therapy for CF SYMKEVI® (tezacaftor/ivacaftor) is likely to be the next drug available in Ireland under the innovative CFTR pipeline deal signed by the Irish Government and Vertex Pharmaceuticals (an agreement strongly supported by CFI).

SYMKEVI® (tezacaftor/ivacaftor) is a prescription medicine used for the treatment of CF in patients aged 12 years and older with two copies of the F508del mutation, or at least one mutation responsive to treatment with SYMKEVI® (tezacaftor/ivacaftor) from the following list: 711+3A →G

A455E

D579G

E193K

K1060T

R117C

S945L

2789+5G →A

A1067T

D1152H

E831X

L206W

R347H

S977F

3272-26A →G

D110E

D1270N

F1052V

P67L

R352Q

3849+10kbc →T

D110H

E56K

F1074L

R74W

R1070W

SYMKEVI® (tezacaftor/ivacaftor) was given initial approval by a sub group of the European Medicines Agency (EMA) in July 2018 and CFI hopes it will be fully approved by the EMA shortly and may be available in Ireland as early as February 2019 (our estimate, subject to change).

“Tezacaftor/ivacaftor combination therapy for CF is another important achievement in the development of disease modulating therapies. This combination improves important clinical outcomes and may benefit those who cannot use ORKAMBI® (lumacaftor/ ivacaftor)” said Professor Stuart Elborn, Director of the Specialist Adult CF Centre at the Royal Brompton Hospital, London.

It is likely that some patients presently on ORKAMBI® (lumacaftor/ivacaftor) will be switched to SYMKEVI® (tezacaftor/ivacaftor), but the criteria for switching is not yet clear and may require written guidance from the National Clinical Programme. This is vital but may delay the drug by a few weeks so we ask our patients and families to take this into account when an announcement is made.

Two triple combination therapies, VX-659 plus tezacaftor and ivacaftor and VX-445 plus tezacaftor and ivacaftor, are both in Phase III clinical trials at present following very promising results from Phase II. The target genotypes are people with one F508del and one minimal function gene alteration who currently do not have a CFTR precision medicine. We estimate that 100-150 patients with CF (about 10% of the total Irish CF population) fall into this cohort.

These two therapies are presently outside the pipeline deal but CFI will seek to have them included once the results of the Phase III trials are verified. Phase II trials showed a fantastic increase in FEV1 of 13 percentage points from baseline, which would put this drug therapy in the Kalydeco® (ivacaftor) range of effectiveness for that marker, although it will also be important to use other markers to assess its full impact.

In short, CFI envisages that up to 90% of people with CF will be on one of the above ground-breaking CFTR drugs in the next few years. We predict that, all going to plan, this will bring a further major boost to life expectancy and quality of life for people with CF in Ireland. Important as these new and innovative drugs are, CFI reminds our patients and families that they must continue to adhere to their full range of prescribed therapies. CFI thanks the CF centres in Ireland, involved in clinical trials and other research related to CFTR drug therapies, for their significant contribution.

Antibiotics: New Colobreathe Capsule and Tymbrineb available in Ireland There have also been some important recent developments in relation to antibiotics for people with CF in Ireland Teva Pharmaceuticals Ireland have launched a new Colobreathe (colistimethate sodium) capsule which is now available in Ireland. This new capsule is designed ‘to help minimise capsule fracture on administration when used with the Turbospin inhaler’.

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Spectrum / Autumn 2018

CFI have been informed by Teva, that when used with the Turbospin inhaler, the new capsule ‘adds to Colobreathe’s existing benefits of portability and simplicity of use’. For people with CF, this can mean less time spent in preparing and administering medication and as with similar time saving devices and it therefore supports adherence.

A nurse support programme associated with Colobreathe can be accessed by calling 1800 804494 or by emailing colebreathesupport@ashfieldheathcare.com Teva Pharmaceuticals have launched a new generic medicine called TYMBRINEB (tobramycin) nebuliser solution.

TYMBRINEB is indicated for the long-term management of chronic pulmonary infection due to Pseudomonas aeruginosa (in patients 6 years and older).

CFI understands that this is a generic form of tobramycin and is an outcome of the patent for tobramycin expiring in Ireland, thus bringing competition into the market for this important and widely used anti-biotic. CFI wishes to acknowledge the ongoing role of Novartis in providing tobramycin for two decades and counting and of course for their vital continuing support of the CF annual medical and scientific meeting in Killarney over many years.

Many thanks to Peter Minchin Many of you will have gotten to know Peter Minchin over the past four years in his role as Fundraising Coordinator, he was hugely popular with everyone he worked with. We are delighted for Peter that he has recently found a new career in An Garda Síochána and as will soon begin his basic training in Templemore.

Many of you will also know that Peter had a special connection to CF because his brother Paul Minchin was the first PWCF recipient of a double lung transplant in the Mater Hospital in 2007. Sadly, Paul passed away in May 2011. We know that Paul’s death had a profound effect on Peter and was a key factor in his moving from the Bank where he worked to join CFI in 2014.

I don’t think I have ever attended a larger funeral than that of Paul Minchin in his home near Bagenalstown in County Carlow, which is an indication of the very high regard there is for the whole Minchin family.

We will miss Peter and we wish him all the best in his new career, we know he will make an excellent Garda. I will have particularly fond memories of him from a fundraising walk that Peter helped organise in Flanders, Belgium in 2015 to mark a forthcoming anniversary of Irish soldier’s involvement in the First World War battle of Messines.

While it was was a very poignant and moving couple days it was also very enjoyable because of Peter’s company and I remember that Peter and our colleague Jolyn carried half the mud from Messines back to Ireland on our shoes - I don’t think the car hire firm was too amused!

Peter loved helping to organise the Malin 2 Mizen cycle, the Head 2 Head walk and indeed all the fundraisers he was involved in. Best of luck in your new career Peter, from all of us in CFI.

Good luck Caroline!

CF Advocate Caroline Heffernan will take on her biggest challenge to date when she takes part in the Barcelona Ironman on October 7th. Comprising of a 3.8km swim, 180km cycle and a 42.2km run, an Ironman is a huge challenge for anyone. For a person with cystic fibrosis to take it on is a major achievement. We are sure you will join us in wishing Caroline the best of luck in the event. You can follow her progress on Facebook: Ironman Barcelona Challenge for Cystic Fibrosis Ireland.

www.cfireland.ie

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SPOTLIGHT: Education The recent Independent Living Report produced by Cystic Fibrosis Ireland highlighted that education is still an area where people with cystic fibrosis (PWCF) face enormous challenges due to their chronic illness. The report showed that there has been a significant increase in PWCF obtaining third level qualifications. However, cystic fibrosis continues to cause significant periods of absenteeism from school or college which causes an associated impact on the ability to perform.

For this Spotlight segment we hear from members of the CF community at different stages of education who tell us the impact CF has on their education and how they manage study and treatment.

The FIrst Day of School from a Dad’s Perspective.

Our first born, Caoimhe, started ‘big’ school in Gaeilscoil Uí Fhiach on the 29th August.

She looked so young and small in her oversized uniform, but she was beaming from ear to ear to see all her friends again.

Caoimhe had attended the Naoinra pre-school for two years prior to beginning primary school and she already knew a number of other children beginning their first year along with her. This, for us as parents, was a huge relief as my wife Grainne already knew so many of the parents and they also knew and understood our ‘situation’. Both of our girls Caoimhe and Fiadh have cystic fibrosis.

Although Caoimhe was excited, I could see that she was also very anxious. This was a huge milestone in her life and also a big change for her. As a father letting go of my baby girl’s hand and watching her walk into the classroom and sit on her tiny chair was very overwhelming especially as we have the added stress of her condition but she took to it like a pro. Caoimhe has been assigned a special needs assistant (SNA) to make sure she eats her lunch, takes her creon, washes her hands etc. Although Caoimhe isn’t aware that she has someone watching over her it gives us some sense of reassurance when she isn’t with us.

I know as a family we will face so many challenges with our two girls but as school is going to be a major part of their lives we will strive to keep everything as normal as possible for them.

I am generally a very positive person and I am very optimistic for the future of our kids health and education.

Caoimhín Ó Lúing

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School with CF The return to school can be difficult for everyone, especially those with Cystic Fibrosis. For these people, this means the return of waking up early to do physiotherapy, nebulisers and having to take all sorts of medications. And this is all before they leave to face a tough day of school ahead.

My own experience as a person with Cystic Fibrosis in school wasn’t the best. I started school at the age of 5 and a half because I wasn’t able to eat solid foods then, I was peg fed until this age. Once I started eating solid foods, I began to take creon. As I was only around 6, I needed help with this.

I had an SNA in school which I found very helpful and beneficial for me.

My typical morning would consist of waking up at 7. I would take all my tablets and then I’d head downstairs to do my nebulisers and my physio. I used to use the vest which was actually nice, kind of like a little massage before school! I would then get out all of my clearance and head off to school. I did this routine again that evening which was difficult when you’re only a child.

At times, it was quite hard to balance my school life and my medical life, but I managed it with the help of my friends. For me, the fact my friends knew about CF made it easier to manage in school. If I was ever on IV antibiotics, they would help me carry my books and bag and if I was out sick or in hospital they would help me catch up with everything I had missed which made me feel included and a part of school life even when I couldn’t be there.

The transition from primary to secondary school was good for me. My big sister Lauren helped me settle in. First year was a good enough year for me health wise. Of course, I got a few infections and was admitted to hospital a few times over the year but overall, I was well. I started the drug Orkambi that year but unfortunately it didn’t work for me.

Second year wasn’t as good for me. By this time, I was on the transplant list in Newcastle. I got too sick to return to school after Christmas in second year. I had to stay at home on permanent oxygen. I started home schooling with four brilliant teachers in March. I really enjoyed it, but of course I missed my classmates and especially my friends.

In the summer of 2017 I moved to Newcastle to wait for my transplant which I thankfully received on the 11th of August 2017. I focused on my recovery for the next few months and I moved back to Ireland at the end of September.

As I was still recovering I wasn’t able to go back to school yet. I continued with my home schooling until November and then went back to school. I was delighted to be back in school.

I’m someone who really enjoys school and I missed it a lot.

I was now in Third year which was an important enough year. As I had missed so much school, I only did six subjects which was much easier to manage than 11.

My principal was very kind to me and he helped with my return in any was he could. I did the Junior Certificate this summer and I got all honours in my subjects.

I started Fifth year in September which I’m excited about.

My life in school after my transplant is so much different to before. I now wake up at 7:30 and I eat breakfast with my family every morning which I never had time for before. I take my anti-rejection tablets every day at 9:30 to make sure I stay feeling this good and that I’m able to continue working hard in school.

For now, I’m looking forward to the school year ahead!

Siobhan Murrary www.cfireland.ie

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A Class Act Hi, my Name is Laura Carrick and l have Cystic fibrosis, l am 16 years old and in 5th year in secondary school.

When l was in primary school, to help my teacher and class understand my illness l showed them a short cartoon video about CF and l answered any questions that they had. My Mam also sent the school a letter written with the help of the hospital, as far as l remember explaining my illness, the importance of washing hands and emphasising the importance that if any of my classmates were sick or had a cold that they should not be around me as it could cause me to be very ill. I always remember my Mam would stick the Creon to the lid of the lunch box with a note and a smiley face to remind me to take it.

By the time l moved to my new school to start secondary, most of the girls knew l had CF. l informed the new school again of my illness and made sure no one else had CF in my class.

My teacher and the receptionists knew, but l did not tell the whole class as l felt differently about CF now that l was older. I did not feel l wanted everyone to know yet as l had enough to cope with, with my transition to the new school so I only told my close friends, although most people from my old school were in my new school and knew anyway.

It was a lot tougher as l have to get up a 6.30 to do my meds before school as my school starts at 8.35am so it’s a challenge sometimes. l do get tired and the term goes on and at one point l was missing a lot of school as l was sick, but my mom got me a home tutoring for a bit so l could catch up with my classes which helped me over that bad period as my Junior Cert was coming up at the time. l do find that if the teacher understands why I’m out they don’t pressure me and my Mam and Dad don’t either, but they do want me to do well in exams so l do try my best, but it’s not the most important thing to me. My Mam always says your health is more important so l now have to agree; if lm not well l will miss school and then my school work will suffer so l try to stay well so l won’t miss school.

My school has been very good to me with regard to my CF. If l am late, I get excused so l won’t end up with a million pink slips and detention. They allow me to drink in class and eat and l can go to the bathroom whenever l have to. I always have a hand sanitiser with me and l leave a spare bottle of Creon in my locker in case l forget it in my school bag. I am lucky that if l do forget anything, l am not too far from home and I can get my Mam to drop it up to me.

As I said before l did not want everyone to know about my illness but there came a point that l felt l needed my class to know as not everyone understood what l had and at one stage l felt girls avoided getting to know me because l had CF. In English, l had to do a presentation so l decided to do it on CF to explain why l coughed, why l drank a lot of water and why l was out a lot. Some girls thought l was making up excuses sometimes so l felt l needed to explain myself, but to be honest if you don’t have CF how could you possibly understand how l feel? Anyway l did the presentation and it went really well. l was feeling so much happier and some girls in my class were shocked that l had CF. l actually made some friends so l think it was a good move to be open about CF and l am happier in school in now.

Since they know now, l decided to fundraise for CF and l brought in the roses on 65 roses day to sell to my friends and classmates in school and l was allowed to sell at the school door in the morning before classes. School is a challenge

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but my Mam and Dad always wanted me to have a good experience and l did everything all the other kids did, of course sometime I have missed school trips to places because I am feeling unwell or cannot do it because of my CF but for the most part I am like any other kid in school. l have gone on trips with the school which l loved even if l had to get up earlier than everyone else to do my airway clearance it was definitely worth it.

Some advice I would give you is to approach the teacher and ask if you could be in a room with your BFF. I know that on some of my trips there can be up to six girls in a room but still having my closest friend in the room was great as she understood much better about my medications. Also tell the teacher (if they don’t already know) that you have cystic fibrosis and explain the medications you have to do, so if you’re not feeling well on the trip they will understand.

When it’s exam time it can be exhausting because you get a lot of homework and you also have to study for the exams, so try to get into school as much as possible during that time because it’s extra important. A tip I would give you is to talk to your exam coordinator and ask them if you can take the exam in a separate exam centre. This way I feel more comfortable coughing and also I am allowed to bring in a bottle of water. Also, if I get dizzy or feel sick and have to leave, I won’t disturb everyone by leaving the exam early. l love photography and watching YouTube, so this summer I was lucky enough to have the opportunity to meet my favourite YouTuber Alisha Marie with Make A Wish. Make A Wish is an amazing organisation that flew me, my sister Louise and my Mam and Dad to California. We had the most amazing time there, and I would love you go back someday. They got us creator passes to Vidcon so I got to see more of my favourite YouTubers there. It was an experience I will never forget. I took so many photos over there and got to go to some places that I had seen my favourite YouTubers go.

Last year in my school I applied to be a mentor to the first years which is basically where we get a small group of first years usually 3 or 4 and we welcome them to the school on their first day and give them a tour and make sure they are getting on ok in the school, making friends and socialising with the other girls in their year. So I applied and then we found out this year who got the places. I was so happy that I was chosen to be a mentor.

I have a couple of ideas of what I want to do in the future. I am thinking about photography (possibly at DIT). I would also love to start my own YouTube channel and see where that takes me, but I have also always loved fashion so maybe I’ll go into some aspect of fashion I particularly love fashion styling. As you can see I have lots of ideas and options for my future but I’m only in 5th year so a lot can change before I leave school. Who knows maybe I’ll find something else I love.

Tell Me I Can’t, I’ll Show You I Can My Name is Sarah, I am 23 years old. I was diagnosed with Cystic Fibrosis when I was nine months old. I have one brother and two sisters but I am the only one that has CF. When my parents discovered I had cystic fibrosis they worked really hard to understand the condition and to keep me as healthy as they possibly could. My Mam and Dad came up with fun ways to do my physio, like buying me a mini trampoline and jumping to music every morning and evening (Rotterdam - Beautiful South and Spice girls of course were my favourite trampoline songs). They got my older brother involved in the games too, this way I didn’t feel like anything was different. My Parents never wrapped me in cotton wool and I got to experience all the different milestones of my life, first day of school, first day of secondary school and first teenie bopper disco just like everybody else. I am very close to all of my family and in some ways me having CF brings us all closer together. www.cfireland.ie

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When I was young I didn’t have many hospital admissions. I was a very active sporty young girl, I was part of school football and camogie teams, I even played for County Galway ladies football. Then at the age of 17 I was offered an amazing opportunity from a really inspirational man called Ian McKeever (RIP), to climb Mount Kilimanjaro, the highest Mountain in Africa, 5,895 meters! Without hesitation I accepted, not once did I believe I couldn’t do it! So in 2017 I climbed Kilimanjaro and raised money for CF, Chernobyl children and Crumlin hospital. But believe me when I say it took a lot of training! I climbed all the mountains in Ireland with my Dad and some more than once!

It was an incredible journey and one I will never forget! There were a lot of preparations to be done with vaccines and medications which took a long time to get sorted but with the help of my pharmacy and CF team in Galway it wasn’t a huge problem. This really was a huge achievement for me and for my family to see that I wasn’t letting CF or anyone get in my way of achieving my life goals.

When I turned 18, I had a few problems and was hospitalised a couple of times. During my Leaving Cert year I got sick and did a couple of weeks study in hospital and at home. I even left hospital for a few hours to sit my Irish Oral exam with cannulas in both hands!

Nevertheless I was determined to get my leaving cert done that year! I completed my leaving certificate in 2013 and secured a place in University College Dublin, to study Agricultural Science/ Animal Science.

I started College in September 2013, I lived on campus for the first two years and then in a house with my friends for the final two years. I used to use public transport, the train to travel up and down from Dublin to Galway, but it wasn’t exactly ideal at winter time or high flu seasons and it was also difficult for me to bring suitcases full of medication with me, because of this I was determined to get my driving licence. So I started driving lessons and within a few weeks completed and passed my driving test and bought a car! This also came in handy when it came to college.

As part of my Degree I had to complete 6 months farm placement in a sheep, dairy, swine, beef and poultry enterprise. I chose to travel to Northumberland UK, for my sheep placement, I spent four weeks there in the Cheviot Hills with 800 ewes during lambing season! I went home between this and my next placement as I got an exacerbation and needed two weeks IVs. After this I celebrated my 21st Birthday and the day after travelled to Brandenburg, Germany and worked for four weeks in a large scale swine farm with approximately 60,000 pigs and then four weeks more on a large scale dairy farm in the same area in Germany. Finally, I came home and worked on a poultry farm and beef farm. I also did a DIY artificial insemination course.

This was a really big step in my life as I was working and managing all my CF routines by myself hundreds of miles away from home! I think this was the moment I really started to feel like an adult! No, who am I kidding I’m always going to be a big child! In my final year I got a really bad infection and was out of college for approximately six weeks, this was really tough for me to try catch up! I was lucky to have a good relationship with my lecturers in UCD and friends and they helped me a lot by sending me notes and assignments and giving me extensions on projects!

When I started college I was really insecure about my cystic fibrosis and didn’t want anybody to know about it in case I would be treated differently. I built up the courage to tell lecturers just because I had to because I was missing some lab practicals or lectures and needed a little more time or help with certain things, but it took me until my final year to tell many friends and colleagues about my cystic fibrosis and I was absolutely honoured when my year representative asked me if we could choose cystic fibrosis as the chosen charity that our year would raise money for!

Some of my classmates even came to the Dáil with me to protest for Orkambi which was very special!

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And after all that it really wasn’t that big of a deal after all so I would highly recommend anyone going to college to make sure you make good relationships with lecturers and friends but also make sure the college and lecturers know your circumstances as it’s easier for them to understand why you may miss college and they can help you! But most importantly don’t miss out on the college life, social nights out and college activities. I found these were really important to keep your head in a good place and I won’t lie they are a lot of fun!

In 2017, after four very quick years, I graduated from Agricultural Science- Animal Science in UCD with a 2.1 degree.

After this I decided that although I really loved the degree I received, I wasn’t fully satisfied and decided to continue my studies to get my dream job and I am currently studying in my 3rd year out of 5.5 years to be a Doctor of Veterinary Medicine. I really love the course and I am extremely excited to achieve my childhood dream of being an “Animal Doctor”. I have also been taking Orkambi for just over a year now and have not been hospitalised once since I started! It was tough on my body to begin with but with the help and support of my excellent cystic fibrosis team in Galway Dr. O’Mahony, Orela, Irene, Mary and Fiona, it made things somewhat easier to adjust and I haven’t looked back since. It seems to be working very well for me; I’m finding it easier to stay healthy, I have better energy levels and I seem to be able to keep a stable weight, which I found difficult to do before! So I would say it has changed my life in a very positive way!

Outside studying I also like to live an adventurous and interesting life! I love taking part in charity events: I have run the VHI Mini Marathon twice with my Mam and done many 30km charity cycles with her! I love to go out with my brother, sister and her partner on nights out. I love to travel and have visited many countries and unique places. I have met a really understanding and supportive boyfriend, who also studies veterinary medicine so we have a lot in common and together we travel, hike, run, cycle, study, cook and just enjoy every day we have in this amazing world. I find it really difficult to tell people about my cystic fibrosis (well especially people I really like), I am afraid of what they will think or say or if they will treat me differently. I told my boyfriend early on in our relationship about my cystic fibrosis, he had never heard of the condition and was curious, I gave him a few days to digest what I had told him and when we met again he was very understanding and had even researched about cystic fibrosis! This meant a lot to me and I knew he was going to be a very special person in my life! This summer we took part in a pilgrimage with his family and walked 288km from Hungary through Slovakia to Krakow in Poland over eight days, walking between 28 and 35km each day! It was tough trying to make sure I kept up with all my treatments and ate enough food for the amount of energy I was burning, but with the help of my boyfriend we managed very well. Also over the summer we helped out in his father’s veterinary clinic and gained a lot of valuable knowledge and experience.

Finally, I would just like to say, my motto in life is “Tell Me I Can’t, I’ll Show You I Can”, this motto has given me a lot of strength and determination to carry on and do everything I want to do or at least try my best and I’m happy!

Everyone with Cystic Fibrosis is different and I think we are all strong in our own ways! Two pieces of invaluable advice from two very wise people: Daddy said to me “Every day your biggest achievement is waking up breathing and telling yourself today is going to be absolutely fabulous!” and Mammy’s “It’s not the end of the world, don’t worry about it, there’s always tomorrow”. www.cfireland.ie

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Back to School: Roll Call for the CF Community

Hands up who is excited to start school?

Adam Munnelly couldn’t wait for his first day in National School!

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Adventure Awaits!

Model Student

Time for TY

Ciaran Kimurray, aged 3 all set for his first day of Montessori.

Jayden Chambers, aged 5 ready for his first day in junior infants.

Mark Kerins on his first day of Transition Year. Mark’s two older bothers also have CF and are currently in college.

Brothers in arms!

Moving on up!

Big brother is watching!

Senan McCabe, PWCF, aged 4 was excited for his first day of Junior Infants. Big brother Rian was on hand to help him settle in at school.

Mason Merrigan, aged 6 on his first day of senior infants.

Ruth Forster, PWCF, aged 4 was happy to be joining big brother Tom in school and was looking forward to her first day of junior infants.

Spectrum / Autumn 2018

HOSPITAL HUB My CF Story

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s part of a quality improvement project at Our Lady’s Children’s Hospital Crumlin (OLCHC) in 2016, our CF team created a patient handheld record for families and their children with cystic fibrosis (CF). These records have been shown to encourage and improve self-management in people with chronic illnesses. Many parents of children with CF come to clinic with their own diary or record of their child’s CF treatments. We wanted to develop a record that would provide families with a single place to store their clinical data and log questions about CF. We initially piloted a paper handheld record with a number of families of younger children with CF. The pilot was a great success and every parent felt that the handheld record was user friendly and that it would improve communication between them and the CF team. Many parents switched over to using the handheld record instead of their own diaries. Many also requested that an electronic version be developed. Our pilot study demonstrated that there was a real demand from families for this valuable tool. CF Ireland kindly agreed to fund the project so we set about developing an app to incorporate the requests and feedback from parents and staff collected during the pilot study.

The app provides children and their parents with their own personal healthcare record which contains information about their CF. The app has been designed to allow children and their parents to keep together all the information that they may need to help manage their CF care. The app has three different formats depending on the child’s age, namely 0-5, 6-11 and 12-18 years, and adjusts the phrasing and content to suit the child’s age.

As the app was developed by the OLCHC CF unit, much of the content directly relates to how children with CF are managed at OLCHC. We envisage that parents and older children with smart phones will open the app while at CF clinic to access any clinical information or questions which have been logged over the past few months. We think of the app like a storybook of lots of important information about CF that families can access whenever they need and this is why we called the app “My CF Story”. We hope that families will find this app empowering and that it will become an integral tool in managing their child’s CF. If the app proves successful, we would like to roll out a national version incorporating the needs of parents and children with CF across the country.

We would like to give a special thanks to all the families who contributed their time to the pilot study and evolution of the app. We must also recognise the contribution of all the staff at the OLCHC CF unit who assembled the main structure and content on the app. Finally, a big thanks must go to CF Ireland who generously funded the development of this app and it would not have been possible to bring this project to fruition without their help and support.

Dr. Des Cox, Consultant in Paediatric Respiratory Medicine, Our Lady’s Children’s Hospital, Crumlin

www.cfireland.ie

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HOSPITAL HUB What a difference a decade makes!

Geraldine Connell CF Clinical Nurse Specialist at the National children’s Hospital, Tallaght University Hospital

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ver the centuries cystic fibrosis (CF) had many aliases before becoming a recognised condition – mucoviscidosis or fibrocystic disease of the pancreas were the main contenders! Indeed for many decades CF had been categorised as a childhood condition and yet today the number of adults with CF outweighs the number of children with this condition. The latest available CF Ireland Registry statistics show that in 2016 there were 701 adults compared to 565 children with CF on the register – with well established adult CF centres continuing to provide care. The potential implications facing a parent when their child has been diagnosed with CF are life-changing. While this is true, the progress in CF care and treatments now in comparison to when the CF gene was first discovered in 1989 is remarkable.

I have worked as a CF Clinical Nurse Specialist in paediatrics in the National Children’s Hospital for well over a decade and in this time have observed many changes in CF treatment and their outcomes. There has never been more hope of major progress in CF care than the present. I have been lucky enough to work alongside the first CF nurse in Ireland, Gerardine Leen, who has a wealth of experience in CF (almost 3 decades!). As Clinical Nurse Specialists we play a pivotal role that is central to the care and management of CF. We are the first point of contact for patients, staff and our community colleagues. With the advancements in CF care our role has expanded significantly.

In 2005, the Pollock Report identified CF service needs and provided a benchmark for improved quality and access to care. The requirement of designated CF staff, CF centres and facilities was asserted. Now in 2018 there is a new paediatric hospital on the horizon hoping to provide the centralisation and alignment of services.

Since 2014, the availability of International Standards of Care (European Consensus Guidelines) has provided centres with guidelines to deliver the highest quality of care to our patients. The establishment of National Clinical Programme for CF (NCPCF) in 2016 (led by Prof. C. Gallagher St. Vincent’s University HospitalUH – G. Leen Nurse Rep) will provide a national framework which will ensure people with CF (pwcf) receive optimal care to preserve and enhance their quality of life, improving health outcomes and well-being.

There has been a tremendous deal of change in the past decade for pwcf.

In more recent years emphasis has been placed on strict infection control and rigorous segregation policies. Gone are the crowded open wards, waiting rooms and holiday camps! In its place are single en-suite isolation rooms, emphasis on handwashing, infection control, strict patient segregation and masks! Astonishingly in 2006, 23% of paediatric patients were chronically colonised with Pseudomonas aeruginosa in comparison to 8% in 2016 which speaks volumes in terms of management impact.

Since 2000, The National CF Reference laboratory at Tallaght Hospital provided a national service for the early identification and susceptibility of CF pathogens. Early identification of CF pathogens and early treatment and eradication is paramount for the continued wellbeing of pwcf.

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Spectrum / Autumn 2018

Although having CF requires lifelong treatment to maintain health, recent advancements in medications have simplified many treatments. With the advent of inhaled antibiotics v’s nebulised e.g. Tobipod and Colobreathe, the treatment burden and patient compliance has improved. We now not only have mucolytics such as pulmozyme to offer, but hydrators such as hypertonic saline and mannitol are available to restore CF airway surface liquid (ASL). We have seen a reduction in the need for IV antibiotics and less use of our home IV service which can only be a positive sign! The CF registry reflects this with 20% of paediatric patients having received IV antibiotics in 2016 in contrast with 33% in 2006!

Since the establishment of the National New Born Screening programme in July 2011 managing the care of an infant with CF from three weeks of age is now the ‘norm’. We are typically seeing ‘well’ babies from diagnosis rather than symptomatic children. Early diagnosis permits prompt expert education and an opportunity to maximise nutrition, implement excellent infection control measures and allows optimal treatment of chest infections. Ultimately our aim is to empower parents from the very beginning with the skills and knowledge to manage their child’s condition – in turn empowering the patient to manage their own health care needs in the future.

Measures such as physiotherapy have also evolved over the years – with emphasis placed on fitness and body conditioning day to day and active chest physio when respiratory symptoms arise. Patients are taught from an early age to manage their condition with the aim of promoting independence and confidence.

Cystic Fibrosis can now be described as a life-long versus a life-limiting condition. The prognosis for pwcf continues to improve and the prospect of specific gene treatment is now a reality.

When the CF gene was identified back in 1989 a real opportunity to treat the underlying cause of CF arose.

Many years later and with numerous clinical trials this has now become a reality! After a long fight led by the CF community, funding for Kalydeco and Orkambi was secure, with other CFTR modulators in the pipeline. These have had a dramatic effect on the nature of the condition, in some cases normalising sweat tests! It cannot be over emphasised the clinical benefits of these modulators for pwcf. We are only at the beginning of this journey and as more and more mutation specific treatments become available the nature of CF will change. Maybe in time we may start CFTR modulator therapies from diagnosis once longer term safety and efficacy data has been established.

Social media has provided a platform for families and pwcf to communicate and share knowledge – almost like the ‘waiting room’ conversations in the years before. Information and discussions are now available at our fingertips. The CF community is well established and continues to evolve and our role as professionals will continue to expand and adapt to medical advancements in the management of our patients.

As we enter the next decade isn’t it inspiring to look back on the last?

And who knows what the future has in store …….

www.cfireland.ie

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KIDS CORNER C

ystic Fibrosis is a genetic condition. Genetics are what make us who we are.

We inherit our genes from our parents, you might have the same hair colour as your Dad or the same eye colour as your Mam. Genetics link a family so you could be tall like your cousin or have the same smile as your Aunt.

Here are some things that you may share with your parents, siblings, cousins, grandparents, aunts and uncles:

You

Mam

Dad

Sibling

Hair Colour SPOT THE DIFFERENCE There are seven differences in the Halloween pictures above and below. Can you find them all?

Eye Colour Freckles Dimples Smile Rolling your Tongue The hand your write with Cystic Fibrosis While genes are what link us they are also what make us unique.

CF can sometimes make us feel different to everyone else, but it is important to remember that cystic fibrosis is just one part of you, and helps to make you unique, but it is not who you are.

Being the best is great, you’re the number one.

Being unique is greater, you’re the only one.

Jesscia Cassidy, aged 8, who recently performed with her dance troupe and Riverdance for the Pope in Croke Park.

MAZE SOLUTION Summer 2018

Congratulations to PWCF

Jessica is pictured left 4th from the front!

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Spectrum / Autumn 2018

YOUTH REACH E

xercise is of extreme importance for people with cystic fibrosis (PWCF) as it is a natural form of chest physiotherapy that increases lung capacity and facilitates airway clearance. Not only is exercise good for the body, but it is also a great way to improve your mental health. PWCF Niamh Doyle recently won the Gold Medal for the vault in the Gymnastics Ireland Super Series Competition and took time out to speak to Cystic Fibrosis Ireland on the role exercise plays in her life.

I was diagnosed with CF at 13 weeks so I don’t remember much of those early years but I do remember as I got older my treatments hospital visits and bouts of sickness became a normal part of my life. My parents tell me I was a very active child over active in fact and as soon as I was old enough they got me involved in sport.

I have been doing gymnastics for the past almost 7 years with Portlaoise gymnastics club. It is something I feel has been a huge part in keeping me well. In the early days when I was a junior gymnast we did a lot of floor tumbling and trampoline work and as I have gotten older and I’m doing more difficult things I feel I am being challenged physically. I compete on four pieces of apparatus, floor forward beam and bars. The vault is my favourite and I think the one I am best at. What I love about gymnastics and my club is that my coaches are very understanding, they push me as much as I can be pushed but if I am feeling sick or tired they will let me sit out, have a drink and then let me come back in when I am ready. Sometimes I get out of breath or if I am getting sick I can be hot and tired, but I always try and finish the session even if I just sit and do some stretches. At the time it feels tough but then I go home and have a good rest and a lot of the time I feel the better of it .I am lucky that I have not been so sick that I could not commit myself to a sport like gymnastics, I make 70% of my training sessions, sometimes I am just too tired and other times I am unwell. I do not give up because I love the sport. I am strong and healthy as a result of doing it and I also love the social aspect we compete individually and as part of a team.

Gymnastics can be physically demanding for flexibility and stamina. I feel doing the sport has given me great strength and muscle strength which has helped to keep me well.

I also play rugby and again I have been very lucky to have extremely supportive coaches who do the same and mind me particularly if the weather is bad they will text my mum and say don’t bring Niamh to training tonight the weather is too bad!

This means a lot because it shows that they care and are trying to help keep me well enough to play my matches which is very important to me because I love match day.

www.cfireland.ie

Summer 2018

Niamh Doyle

WORLD LADDER SOLUTION

I feel sometimes parents of kids with any illness are afraid to push them or allow them to challenge themselves and sometimes unfortunately if the child is sick enough the parents are right to do this but if it is possible all children especially those with lung related illness should do sports: swimming, gymnastics or whatever it is the child likes because from experience I can definitely say that sport and exercise in general play a huge role in keeping me well.

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MEMBER SERVICES Noticeboard Schools and Cystic Fibrosis Booklet Back to School Offer!

Get your copy of CF Ireland’s latest publication “School and Cystic Fibrosis”.

This booklet is a useful resource for schools, teachers and parents whether your child is starting school, changing schools or has a new teacher. While recognising that every child with CF is different, the booklet provides information about varying aspects of CF and its implications at school.

Order your copy by phoning 01 496 2433 or emailing Nuala at nmcauley@cfireland.ie. Booklets can be sent to parents or directly to the school and are also available to download from our website https://www.cfireland.ie/cfi-services/publications/item/387-school-andcf

Did you know?

From the 1st September 2018 all persons in receipt of Carer’s Allowance or Carer’s Benefit are able to attend a GP free of charge. Registration is now open and applies to carers who are currently in receipt of either full or half rate Carer’s Allowance or Carer’s Benefit, and who do not currently hold a Medical or GP Visit card.

“We’ll be our own lifesavers.

We’ll get the flu vaccine.”

All carers who already hold active Medical Card or GP Visit Card eligibility from a previous application retain this eligibility. There is no requirement for them to also register for eligibility on the GP Visit Card for the Carers scheme.

Further information on the scheme is available on www. medicalcard.ie or by calling 1890 282 919.

To register: https://www.hse.ie/eng/cards-schemes/ gp-visit-cards/gp-visit-card-for-carers/gp-visit-card-forcarers-english-registration-form.pdf

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The flu vaccine is a lifesaver for older people and those with long-term health conditions.

CONTACT YOUR GP OR PHARMACY TODAY TO GET THE FLU VACCINE.

www.hse.ie/flu Order code HNI00717

Spectrum / Autumn 2018

My Legacy Week October 29 th - November 3 rd

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y Legacy Week is as good a time as any to make your Will and consider leaving a legacy gift to a cause you 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 you are encouraged to take that first step in writing your Will by making an appointment with a solicitor. Cystic Fibrosis Ireland are a member of MyLegacy.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 you might think and your solicitor will discuss any fee for drawing up this important personal document when you make your 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. It won’t cost anything in your lifetime, but will ensure your generosity. Your legacy will live on to support future generations, and help us to make long-term plans in support of CF care throughout Ireland.

By leaving a gift to Cystic Fibrosis Ireland you help us to; • Fund pioneering research that aims to improve current methods of treatment and management of the condition as well as enhance the quality of life of those living with the condition • 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 out-patient facilities • Help people with CF directly with Exercise, Transplant, Fertility Counselling and Bereavement Grants • Provide advice, information and advocacy services to help people with cystic fibrosis For more information on leaving a gift in your Will, please see the enclosed leaflet “Your Legacy Could By Life Changing”, visit our website at www.cfireland.ie or the My Legacy website www.mylegacy.ie. Alternatively you can contact Rachel on 01 496 2433/ rbyrne@cfireland.ie for more information. www.cfireland.ie

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RESEARCH Health Research Board funded study called ‘Patients’ Preferences for Health’ are looking for volunteers In general, people have preferences of being in one state of health over another. This can often be a preference to have good health over bad, or to be in good health for so long and to be in less well health for a shorter period of time. By asking the public in Ireland to make these decisions or trade offs we can say something about the general preferences of people in Ireland.

Preferences gathered in this way are often used to guide choices around decisions in relation to health and using preferences from the general public is deemed to be a fair way of deciding on how to assign healthcare resources.

However, it is likely that a person’s experience of being in a health state will alter their preferences compared against those who are only imagining being in that state.

As such, this work will examine the differences between patients with CF, their valuations of health states and those of the general population to see if this is actually the case.

If you are interested in helping with this study, please contact Daniel Costigan at dcostigan@cfireland.ie

Cystic Fibrosis Ireland Invites Your Participation in the “Science of CF” Survey Cystic Fibrosis (CF), is a complex and multi-organ condition which primarily affects the respiratory and digestive systems. Other medical issues include, CFrelated diabetes, malnutrition, liver diseases and subfertility issues, particularly in males, as well as chronic lung infection. Because of this, health literacy is extremely important for people with CF, their family and friends. People with CF want to learn more about their condition and are actively seeking information on new treatments in development which may ultimately and significantly improve their health outcomes.

The challenge for healthcare professionals and scientists is finding out what people with CF want to know about their condition and how best to communicate that information in a manner that is both accessible and reliable.

In an effort to address that challenge, the Northern Ireland Microbiology Discussion Group (NIMDG) (not-for-profit) has launched a survey. The survey consists of 40 questions covering everything from your cross-infection concerns to what you consider to be your most reliable sources of CF information.

CFI invites your participation in this survey which has already taken off with over 200 completed surveys worldwide from Ireland, Northern Ireland, South Africa, Brazil, Israel, Canada and the USA. To take part in the survey go to http://www.surveygizmo.com/s3/4149836/Scientific-Communication-in-CF The survey is voluntary and completely anonymous and takes only 15-20 minutes to complete. It will run to the end of 2018 and the outcomes of survey will be available at https://cfscicomms.weebly.com/ Note: NIMDG has a strong base within CF and over the past two decades, several healthcare professional members of the Group have developed important links with the CF community through research and healthcare delivery.

The survey has been developed in conjunction with Cystic Fibrosis Australia, Cystic Fibrosis Canada/Fibrose Kystique Canada, Cystic Fibrosis Foundation (USA), Cystic Fibrosis Ireland and the Cystic Fibrosis Trust (UK).

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Spectrum / Autumn 2018

Study Points to Importance of Early Diagnosis and Treatment of Non-Tuberculous Mycobacteria (NTM) Infections in People with CF NTM are a group of bacteria that live in soil, swamps and water sources and are “cousins” to the bacteria that cause tuberculosis (TB). They are very hardy bacteria that are problematic to treat, resistant to many antibiotics and all around associated with poorer clinical outcomes for a CF patient who acquires such an infection. There has been an increasing incidence of such infections in CF patients, with recent studies reporting a prevalence of 32.7%.

A new study has also suggested that earlier diagnosis and treatment of NTM infections in patients with CF could positively affect a patient’s lung function. The study started off with 360 CF patients, 30 of whom were identified as having an NTM infection. 28 of these were further analysed and compared with 26 matched CF patients not infected with NTM. Within the NTM group, 17 patients were infected with a class of NTM known to be slow-growing, eight were infected with rapidly growing NTM and three were positive for both types.

When lung function was assessed in the two groups, researchers observed that lung function (FEV1) was significantly higher before NTM infection, regardless of the type of NTM. In relation to patterns of treatment, the team found that considerably more patients with slow-growing NTM had received penicillin/ beta-lactamase and rifampin following infection compared to before infection.

They noted that “an earlier CF diagnosis was associated with a higher isolation of slow-growing NTM and greater antimicrobial use after infection” adding that “NTM acquisition is associated with a worsening of [lung function].

Thus, both the early diagnosis and treatment of NTM infection in patients with CF may positively impact lung function.” The team believes that “increased awareness by clinicians on different NTM subtypes and more universal treatment plan for NTM infection in the CF population may positively impact patient management and outcomes.”

Vertex and Quest Genomics Collaborate to Develop Genetics-based, Precision Medicines At the end of August 2018, Vertex Pharmaceuticals and Genomics announced a three-year collaboration designed to promote the development of precision CF medicine. This approach aims to tailor treatment based on individual patient’s genetic differences and lifestyle. The partnership is extendable for up to five years and will combine Vertex’s genetic expertise with Genomics machine learning to discover and improve new therapeutic targets.

Machine learning is a nascent field of computer science which can predict outcomes based on its ability to identify patterns in previously generated information. Genomics uses a proprietary machine learning tool to predict any expected molecular, cellular, physiological or disease outcome based on genetic differences found in the human genome. Genomics says that this ‘Genomics Engine’ is the largest of its type in the world, with over 100 billion points of data to help identify genetic variations in over 14 million positions on the human genome and link them to specific outcomes.

www.cfireland.ie

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Cystic Fibrosis Ireland Welcomes Positive Australian Determination on Orkambi® Cystic Fibrosis Ireland welcomes news that Orkambi® (lumacaftor + ivacaftor), a groundbreaking medication for the treatment of cystic fibrosis (CF) will soon be subsidised by the Australian federal government, Australian Health Minister Greg Hunt has said.

This follows the Australian Pharmaceutical Benefits Advisory Committee’s August 2018 recommendation that the drug be listed on the Pharmaceutical Benefits Scheme in Australia for people ages six and over with CF who have two copies of the F508del mutation in the cystic fibrosis conductance regulator (CFTR) gene.

For the last three years the Australian CF community’s voice had been rebuffed again and again with the committee rendering rejection after rejection. It is only thanks to the relentless advocacy by people with CF, their families and friends, that people with CF in Australia now have this win.

Minister Hunt told parliament that they “will move as quickly as possible” to make the drug available to people with CF. Despite the fact that another drug for treating CF took about three months to be listed after being recommended by the committee last year, Mr Hunt is hoping that Orkambi® will become available much more quickly than that, saying that he “will move heaven and earth” to achieve this.

Social Policy Update: Cystic Fibrosis Ireland Welcomes Comments by Minister McGrath around his Commitment to Introduce Essential and Overdue Replacement Transport Schemes Cystic Fibrosis Ireland warmly welcomes the comments made in the Seanad on 04 July 2018

by Minister Finian McGrath around his commitment to introduce the transport scheme to replace the vital mobility allowance and motorised transport grant schemes which were closed to new applicants in 2013.

The Mobility Allowance Scheme was a monthly allowance of €208 which was paid to applicants, with a severe disability between the ages of 16 to 66, to contribute to their mobility needs.

The Motorised Transport Grant Scheme was a means tested HSE payment to enable a person with a severe disability to purchase or adapt a car where that car was essential to retain employment.

Both of these schemes formed a vital part of the support infrastructure to help people with CF live and be active in the community. For many with CF, private motor transport is required to enable independence and employment but for others who are significantly more ill, there are additional risks associated with physical exertion by virtue of not having access to private transport.

Speaking on the three options for alternative replacement schemes which he is deciding between for inclusion in a memorandum for cabinet and for ultimate inclusion in the Health (Transport Support) Bill, Minister McGrath said: “There is some light at the end of the tunnel. I confirm that work on the policy proposals for the new scheme is at an advanced stage. […] In regard to the memorandum, I am considering three options. I know exactly what I want in terms of the scheme. […] I was not happy with what was proposed at Cabinet and I undertook to consider other options in terms of funding. My preferred option is the most expensive. […] As far as I am concerned the Government committed to the introduction of the scheme in the programme for Government. […] I hope to bring the memorandum to Cabinet before the summer recess, and at the latest, early September. As I said, I am currently considering three options and I will decide in the next week or two which option I will bring to Cabinet. I hope I will achieve the objective of bringing it before Cabinet before the summer recess but, if not, I will do so in early September.”

Cystic Fibrosis Ireland hopes that there is indeed progress on the Health (Transport Support) Bill as this would be a most welcome development for those with CF, who experience significant mobility issues and progress is long overdue.

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Spectrum / Autumn 2018

FUNDRAISING: Challenges and Events For further details on any of our fundraising events, visit our website www.cfireland.ie, contact the CFI Fundraising Team on (01) 4962433 or email fundraising@cfireland.ie Sept 28th to Oct 7th: Paddy Kierans’ Memorial Walk ‘Flavours of Romania’ CFI would like to wish all the walkers heading off on the annual Paddy Kierans’ Memorial Walk from September 28th to October 7th the very best of luck on their trip to Romania.

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

Places for this year’s walk have now been filled, but keep an eye on our website and social media channels for updates and the announcement of the venue for the 2019 walk.

Oct 29th: SSE Airtricity Dublin City Marathon CFI would like to wish all of our runners taking part in the SSE Airtricity Dublin City Marathon on October 29th the very best of luck. Places for the marathon are now all sold out, but if you have registered and you have not yet picked a charity to fundraise for, please do consider fundraising for CFI.

Nov 4th: TCS New York City Marathon CFI would like to wish all our runners taking part in the 2018 NYC Marathon on 4th November the very best of luck. The team consists of Ken Cahill, James Brett, Rita McGinn and Margaret Gannon.

The marathon starts from Staten Island and runs through all 5 city boroughs. With almost a million spectators and a carnival atmosphere the New York City Marathon is an unforgettable experience. The 2019 Tour Package will open in January. If you would like to receive information about the 2019 tour, please contact the CFI fundraising department. www.cfireland.ie

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Dec 14th: Christmas Jumper Day 4CF On Friday 14th December, we are encouraging as many workplaces as possible nationwide to support Christmas Jumper Day 4CF. Interested in getting involved? All you need to do is…

1. Ask your staff or colleagues to support Christmas Jumper Day 4CF on Friday 14th December. Encourage everyone to wear their most seasonal looking Christmas Jumper to work in return for a €5 donation to support people with Cystic Fibrosis in Ireland!

2. Bah Humbug donation! If there is a colleague who is not wearing a Christmas Jumper, they have to pay a Bah Humbug donation of €10! It’s all for a great cause.

3. Ask your company if they are willing to match fund the monies raised!

4. On the day, make sure to 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.

We can send you on some balloons, bah humbug badges, posters, sponsorship cards or collection buckets if needed – just let us know!

5. After the event you can • Lodge the monies raised: IBAN: IE59 AIBK 9310 7108 5785 93 BIC: AIBKIE2D • Post a cheque to Cystic Fibrosis Ireland, 24 Lower Rathmines Road, Rathmines, Dublin • Donate online at www.cfireland.ie (Please remember to include your company’s name as a reference so we know who it’s from) Email or call the CFI Fundraising Team to register your workplace & we will help you organise everything for the day!

December: Cystic Fibrosis Ireland - Christmas Cards It’s (nearly) that time of year again. In the run up to the festive season CFI have a full range of Christmas Cards on sale. You can select from 14 different packs of Christmas Cards, with each pack containing 8 cards. This includes 2 variety packs – includes 4 designs x 2 cards and 12 single packs – 1 design x 8 cards.

You can purchase the cards through our website using the online order link, contact our office on 01 496 2433 or email fundraising@cfireland.ie for an order form.

All CFI branches have been issued with a Christmas Card order form and we would ask that branches please return this form to the CFI office no later than 3rd November with your branch order for Christmas Cards.

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Spectrum / Autumn 2018

Apr 12th, 2019: 65 Roses Day CFI are delighted to announce that 65 Roses Day 2019 will take place on Friday 12th April 2019. Full details to follow in the next edition of Spectrum but please mark the date in your Calendar.

We are currently asking schools and companies to get involved on 65 Roses Day by helping to sell our purple roses and organising fundraising events as part of the 65 Roses Challenge.

If you would like to volunteer to sell purple roses or would like to discuss any fundraising ideas with our fundraising team then please contact us on 01 496 2433 or email fundraising@ cfireland.ie.

April 28th, 2019: Virgin Money London Marathon CFI are delighted to announce details of the 2018 Virgin Money London Marathon package where we can guarantee you an entry to this highly sought after event.

The marathon takes place on Sunday, 28th April and will start from Blackheath and runs through many of the famous London districts and past many iconic landmarks such as the Tower of London, the Houses of Parliament, Big Ben and Buckingham Palace.

The standard tour includes: • Choice of flights from selected airport, including all taxes, fees and charges • Two nights twin or double sharing with full buffet breakfast • GUARANTEED race entry • Private coach transfers to race start from the hotel (runners only) • Services of Sports Travel Guides Places are limited, so if you are interested, please contact CFI as soon as possible for further details.

May 9th to 12th, 2019: Malin2Mizen Cycle4CF The Malin2Mizen Cycle4CF will take place from Thursday 9th to Sunday 12th May, 2019. The 2019 cycle was launched in Athlone on September 22nd with the support of South Roscommon Cycling Club.

The cycle will begin at Malin Head in Co. Donegal and will finish in Mizen Head in Co. Cork, along the following route; Thursday 9th May: Malin Head – Bundoran Friday 10th May: Bundoran – Oranmore Saturday 11th May: Oranmore – Mallow Sunday 12th May: Mallow – Mizen Head Registration for the cycle is now open.

All participants are asked to raise €2,000 to take part. This will also cover your food and accommodation for the four days of the cycle. All funds raised will go to CFI to help us continue to support people living with Cystic Fibrosis.

Places are limited, so please register as soon as possible to secure your place! www.cfireland.ie

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June 2nd, 2019: One in 1000 CFI had an absolutely fantastic turnout at the Vhi Women’s Mini Marathon in 2018 and we would like to build on those numbers even further next year. One person can make a difference! You can be that one person for Cystic Fibrosis Ireland by registering to take part in the Mini Marathon as part of our One in 1,000 Campaign.

Why not get your girls together and get exercising for CFI? A great day out, with a base with food and entertainment provided, while raising vital funds for people living with CF in Ireland?

The 2019 Vhi Women’s Mini Marathon will take place on Sunday 2nd June. Keep an eye on our website and our social media channels for announcement of sign up details for One in 1,000 – 2019.

All Year: Schools Fundraising Resource Pack CFI have written to all Secondary schools as part of our School Outreach Program, which aims to educate pupils on Cystic Fibrosis and introduce pupils, especially in Transition Year, to charity work.

We are asking all schools to support our 65 Roses Challenge campaign for 65 Roses Day on Friday 12th April 2019 by: Organising a 65 themed fundraising event. This could be any event such as 6.5km walk/run to a 65 minute football match.

Holding a ‘Non-Uniform Day’ on 65 Roses Day with CFI wristbands available in exchange for a €2 donation from each pupil on the day.

Selling our Purple Roses for €2 in your school for 65 Roses Day or organising for your school to volunteer to support a fundraising collection organised by CFI in your local area.

Kilimanjaro CFI facilitate treks to Kilimanjaro, so if you are thinking of taking part in a trek, we advise that you book your place early for treks in 2019.

Skydives A skydive has to be one of the most exciting things you will ever do in your life. Now is the chance to tick this off your bucket list, while raising much needed funds for CFI. Jump from 10,000 feet, free fall for 30 seconds before the parachute opens and take in the views like you’ve never seen them before.

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Spectrum / Autumn 2018

FUNDRAISING: Thank You It is not possible to list all of the Fundraising Events that have been organised since our last edition of Spectrum, but we would like to take this opportunity to thank everyone that has volunteered and raised money on behalf of people with Cystic Fibrosis. Every cent raised helps provide a 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: Spar Ireland In May 2017, Spar confirmed CFI as their Charity Partner.

They set a target to raise €300,000 for us over the next two years. On September 22nd where Spar once again were the main sponsors of our Head2Head Walk, they presented us with a cheque for €381,000 for the funds raised to date from our partnership.

We would like to extend a huge thank you to everyone in SPAR Ireland for what has been an absolutely amazing partnership to date. SPAR stores across the country continue to proudly support CFI with Collection Boxes on display and will have our Christmas Cards on sale this Christmas. SPAR continue to support PWCF with a donation on selected SPAR own brand products and have wholeheartedly supported our events including 65 Roses Day, the Malin2Mizen Cycle4CF and the Head2Head Walk!

Please support your local SPAR store in recognition of all they are doing for to support people with Cystic Fibrosis in Ireland!

Top Oil – Fueling Ireland CFI would like to say a huge thank you to our charity partner, Top Oil - Fueling Ireland for their cheque of €5,000. We look forward to another successful year of charity partnership!

L-R is CFI’s Rachel Byrne, and Top Oil’s Sinead Gilsenan, Niall Conway and Gerard Boylan (CEO).

www.cfireland.ie

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Head2Head Walk CFI would like to say a huge thank you to everyone who took part in the Head2Head Walk on Sunday 16th September. It was a fantastic day with record numbers taking part.

The walk began at 9.00am from Howth with the walkers making their way towards Sandymount for the half way break. The walkers really enjoyed the views around Dublin Bay as they went on their way and were provided with refreshments along the route from our fantastic sponsors SPAR. Lunch was provided by SPAR for all the walkers at Sandymount, with Classic Hits 4FM broadcasting live including an interview with one of the walk organisers Mary McCarroll and her son Brian (PWCF). The walkers then continued on their way towards the finish in Bray where a lovely reception / meal was laid on in The Royal Hotel in Bray, organised by the Eastern Branch of CFI. All participants received a goodie bag and a medal on completion of the walk.

A huge thank you to James Downes and Mary McCarroll and their team of volunteers who put an enormous amount of work into organising this event with CFI and ensuring it ran smoothly on the day. We would also like to say a massive thank you to our charity partner SPAR who sponsor this event and generously provide plenty of refreshments and the goodie bags for the walkers. Special thanks also to the Gardaí and St. John Ambulance who help out each year.

We would like to remind anyone who has sponsorship money to lodge from the event that they can do so using the bank account details below. Please remember to include your name and ‘H2H’ as a reference so we can receipt accordingly. IBAN: IE59 AIBK 931071 08578593 BIC: AIBKIE2D

Paris2Nice Cycle CFI would like thank Enda Greehy from Cork and Aoife Skehill from Galway for taking part in the 2018 Paris2Nice cycle and for raising funds on behalf of CFI. The cycle took place from 15th to 20th September and saw a large number of cyclists taking part to raise funds for a number of different charities.

Over €10,900 has been raised by the cyclists for CFI to date in what has been a fantastic effort by both Enda and Aoife. Well done to both of them.

We will confirm details of Paris2Nice 2019 in our next issue of Spectrum and on our website as soon as they are announced.

Hospital Saturday Fund The CFI Fertility Grant provides financial support to people with cystic fibrosis and their respective partners who wish to undergo fertility assessment and treatment in the hope of becoming pregnant.

With the generous support of The Hospital Saturday Fund (€2,700), one more family living with CF will be given the chance to start a family.

Pictured is CFI’s Dan Costigan and the HSF’s Mia Shepherd.

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Spectrum / Autumn 2018

Malin2Mizen Cycle4CF On September 22nd the 40 cyclists from the Malin2Mizen Cycle4CF gathered in Athlone for a 50k spin. This was followed by a presentation of medals to the cyclists by Bernie Priestley and a large cheque presentation for the total amount raised from the 2018 cycle for €141,638.61.

A huge thank you to all of the cyclists, their families, friends and supporters for all their fundraising and the commitment they had to make to cycle from Malin to Mizen in May. Thank you again to everyone else who supported the cycle as per details published in the last edition of Spectrum.

Registration is now open at www.cfireland for the Malin2Mizen Cycle4CF 2019 which will take place from Thursday 9th to Sunday 12th May, 2019. Places are limited so sign up now, to avoid disappointment.

Castletown Construction Golf Classic

National Ambulance Service – Dublin South Central

CFI would like to say a huge thank you to Teresa and Brian Delaney who raised a whopping €10,993 from the Castletown Construction Golf Classic organised at the Killeen Golf Club on 22nd June.

CFI would like to say a huge thank you to Paul and all the team at the National Ambulance Service – Dublin South Central, who raised an amazing total of €2,527.25 from their participation in Hell & Back. The team carried on from the fundraising efforts of Derek Gray (PWCF), part of the Intermediate Care Service Team within the NAS, who sadly passed away earlier this year.

Pictured L-R is Teresa Delaney, Tomás Delaney (PWCF), and Fergal Smyth, Fundraising Manager, CFI.

www.cfireland.ie

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1 in 1000 CFI would like to thank all of our One in 1,000 participants who raised a whopping €64,600. What an amazing feat!

We cannot wait to see you again next year. Here are our Lucky One in 1,000 Raffle winners: • A family pass (2 adults & 2 children) to Fota Wildlife Park, Cork – Patricia Boland • A €100 voucher for TGI Friday’s Dublin (redeemable nationwide) – Sonya Magure • 2 places for a “Hawk Walk” at Ireland’s School of Falconry, Cong, Co. Mayo – Cathy Swift • €40 voucher for Peter Mark Hair (redeemable nationwide) – Kirsty Gillard-Daly • €40 brunch voucher for Lemon & Duke, Duke Lane, Dublin 2 – Amy Kenny • 2 tickets to EPIC The Irish Emigration Museum, CHQ, Dublin 1 – Maggie Kerins • Winner of our Cocoa Brown Tan Hamper – Bernadette Hearns

Michael Carruth’s 65 Round Challenge 4CF CFI would like to say a huge thank you to former Irish Olympic boxing champion Michael Carruth for organising the 65 Round Challenge which took place on Friday 29th June in the National Boxing Stadium. The event involved 65 different people stepping into the ring to spar against Michael for one minute.

CFI would also like to thank all those who volunteered on the night to help make this event such a success. A total of €9,456.72 was raised from the event.

Michael Carruth is pictured presenting a cheque for €9,456.72 to Fergal Smyth, Fundraising Manager, CFI

Berlin Marathon Congratulations to David Crosby who received a double lung transplant in 2016 and his team of supporters – ‘The Greatness Within’, who completed the Berlin Marathon on behalf of Cystic Fibrosis Ireland on Sunday 16th September, raising an amazing €11,258 for CFI. The group previously completed the New York Marathon in 2017 and have their sights set on the London Marathon for 2019.

Pictured right is David Crosby presenting a cheque for €11,258 to Fergal Smyth, Fundraising Manager, CFI.

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Spectrum / Autumn 2018

Solo Charity Cycle – Seán Ó’Catháin

Step2Fitness

CFI would like to say a big thank you to Seán Ó’Catháin from Kerry who completed a solo charity cycle around the lakes of Ireland and donated all the proceeds which he collected to CFI. He kindly presented a cheque for €2,108.

CFI would like to say a HUGE thank you and well done to all the ladies at Step2itfitness in Causeway, Co. Kerry, who partook in the One in 1,000 campaign at the Vhi Women’s Mini Marathon and together raised a whopping €4,639.59.

Pictured in the photo are from L to R: Seamus Ó’Lúing, Berní Firtéar, Betty Hand and Seán Ó’Catháin.

Q Cafe Company CFI would like to say a huge thank you to our everenthusiastic charity partner, Q Cafe, who have raised a fantastic €4,895.45 from hosting two fundraising events.

The most recent was a charity Cycle for CF fundraising roadshow, where they brought spin bikes across the country, getting as many staff involved as they could to cycle as many kilometers as possible. They also did a brilliant job in helping to raise awareness of CF wherever they went.

www.cfireland.ie

Harrington Gold Academy CFI would like to say a huge thank you to Rebecca Codd and Tadgh Harrington of the Harrington Golf Academy who recently held a Charity Golf Scramble at Killerig Golf Club in aid of CFI and raised a fantastic €2,500!

Pictured below is Tadhg Harrington, CoFounder of the HGA, Peter Minchin, CFI, and Rebecca Codd, Co-Founder, HGA.

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CFI Christmas Cards 12 different designs available plus two variety packs.

8 cards per pack

Branches - please order via order form sent to each branch Public - please order via our website at www.cfireland.ie

Cystic Fibrosis Ireland 24 Lower Rathmines Road, Rathmines, Dublin 6, Ireland

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t: +353 1 496 2433

f: +353 1 496 2201

e: info@cfireland.ie

w: www.cfireland.ie

Company Reg: 449954

Charity: CHY6350

Spectrum / Autumn 2018