Spectrum - Summer 2023

SPECTRUM

Latest News | 2-3

* Making Organ Donation the Norm in Ireland

Brendan's Doodles | 3

Member Services | 4-9

*Kerry to Kilimanjaro - Everything you need to know for travelling as a PWCF

*Travel Diary - Chile: The Biggest Adventure of All

Spotlight | 10-18

Emigration and CF

*Cruising on the Costa Brava

*Ambitions for Australia

*Transferring to Texas

CFRI | 19

*Using Data for Good

CFI Conference in Photos| 20-21

Research Update| 22-25

* Covid-19 CF Study Results Summary

* Irish Comparative Outcome Study of CF - Part 2

* Pregnancy Study Update

ECFS Report |26-30

Fundraising| 31-37

* Challenges and Events

* Thank You

CFI Branches |38

NEW TO SPECTRUM!

Last month, Brendan Lonergan, artist and Board Member, contributed to Spectrum and told his story with a series of creative doodles. The community loved these so much that we thought we'd make them a regular feature.

Keep an eye out in coming editions for 'Brendan's Doodles' with his animated thoughts on life as a PWCF.

Welcome to the summer edition of Spectrum

This is a great edition if you are interested in some of the most recent CF research developments, with a report from the European CF Society meeting in Vienna held in early June.

For those of you who have an interest in-post transplant CF care, we also welcome the advertisement by the Mater Hospital of the first Consultant specialising in post-transplant CF care. This has been an important ambition of CFI and is consistent with the Model of Care published in November 2022. The Human Tissue Bill will shortly come into law, we have been told, bringing in the much anticipated ‘Soft-Opt-Out’ organ donation consent provision.

The Kaftrio 35 dispute was not only very challenging for the families impacted, but it also highlighted the gaps and weaknesses in the overall drug reimbursement system in Ireland. The recently published Mazar’s Report highlighted some of these issues, including inadequate communication with patient groups and lack of transparency.

Most new high-tech drugs in Ireland are delayed by between 2436 months after approval by the European Medicines Agency; there is no accelerated access for unmet need and there is no orphan drug designation, which means that we have less or delayed access to drug therapies for those with rare diseases in Ireland than most other European countries.

Thanks to all who attended our annual conference in April. It was an informative and lovely event and it was so good to meet up after the enforced COVID-19 gap. The recordings of the conference will shortly be on our website for those that missed any of the talks. If you have not seen our 60th anniversary documentary launched at the conference, I would strongly recommend - very moving. It is available on our website at https://www.cfireland.ie/aboutus/60-years-of-cfi

We hope you have a lovely summer as together we continue to make progress in CF care in Ireland – much done, more to do.

Philip WaPhilip Watt Philip WatCFIC CEO, CFI

LATEST NEWS

Making Organ Donation the Norm in Ireland

We hear that the Human Tissue Bill 2022 may be passed before the end of the summer. This is great news for those with CF awaiting any form of transplant, with the introduction of a new organ consent that has proved so effective in other jurisdictions including the UK and Spain. CFI has been at the forefront of this change as part of the Irish Donor Network.

Despite much public goodwill towards organ donation in Ireland, second to no other country in Europe, combined with excellent clinical expertise and dedication, organ donor and transplant rates in Ireland are below average when compared with our European neighbours. Ireland was only in 18th place out of EU28 for transplants in 2020 and only in 17th place for deceased organ donation that same year.

The national transplant programmes in Ireland are undertaken in three hospitals - the Mater Hospital (heart and lung); Beaumont Hospital (kidney) and St Vincent’s Hospital (liver and pancreas). These programmes further rely on the support of all major ICU hospitals around Ireland and for coordination by the Office for Organ Donation and Transplantation Ireland (ODTI).

Action is needed to move Ireland into the top 10 EU countries for donation and transplantation. In this context, The Irish Donor Network supports the introduction of Soft-Opt-Out organ donation consent in the Human Tissue Bill.

Consent and Consultation

Currently, the decision on organ donation in respect of a deceased person rests entirely with the next of kin, including where the deceased person held an organ donor card or had indicated their wish to be an organ donor on their driving licence. These efforts have been worthy and should continue as awareness initiatives, but they are no longer adequate on their own.

Under the proposed opt-out system, all adults in Ireland will be considered to have agreed to be an organ donor when they die unless they have recorded a decision not to donate on an opt-out register, or are in one of the six identified excluded groups, which provides important protections for vulnerable people.

This consent system will apply to the donation of the heart, lungs, liver, kidneys and pancreas (solid organs). A separate consent process will be undertaken for the donation of other organs or for tissues and cells from deceased donors.

The next of kin will continue to be consulted before any action is taken, and the wishes of the deceased should be central to this decision. Soft-Opt-Out differs from Hard-Opt-Out in relation to the point of consultation with next of kin. In the Hard-Opt-Out approach (not envisaged for Ireland) next of kin would not be consulted.

A Soft-Opt-Out Organ donation system relies on people knowing about their options and being able, easily and quickly, to opt out if that is their wish through a secure Online Register. It is not about forcing people into donation. It is about making it as easy as possible for an individual’s willingness to donate organs after their death to be acted upon.

One organ donor can potentially save seven lives. There are also benefits to recipient families and Irish society in general, including the Irish health system and the wider economy in promoting transplantation and working towards the most effective organ donor system possible. As Minister Donnelly stated to the Oireachtas at the second stage of the Human Tissue Bill in January 2023:

"In all scenarios, family members will continue to be consulted before donation takes place. The wishes of the deceased should be central to the decision but if the family objects to the donation, then it will not proceed. I want to be absolutely clear that in no way does the move towards an opt-out approach mean that individual choice on this most sensitive issue is being threatened or eroded. Those who object to organ donation, for whatever reason, will be able to record their objection on a new register that will be established under the legislation. In such cases, their wishes will be respected and their family will not be approached on the issue of organ donation. It will be possible for individuals to remove their name from the register at any time.

"The Bill also provides a framework for living donation."

The Bill is now heading to the report stage and CFI will be active in briefing Oireachtas members on why this important legislation should be supported.

Brendan's Doodles

by Brendan Lonergan @BOB_NARROW

Philip Watt CEO CFI

MEMBER SERVICES

Travel for me has always been special. From birth, it meant visiting my grandparents in England. These trips became second nature to me whether it was by air or water, or took five or fifteen hours door to door, packing a suitcase had a very special meaning.

As a PWCF, it also meant that I learnt early on to be aware of ordering my medications and making sure I had enough for a trip, or for unforeseen events like an unexpected train strike. Over the years I have travelled, all through the UK, mainland Europe, Eastern Europe, USA and had the trip of a lifetime to the Caribbean. With each destination, as long as I had the correct plug attachment and voltage for my nebuliser, back-up antibiotic and hospital letter confirming my medications, I was ready to go.

My next trip is more extreme than any trip I have taken before. I will aim to summit Kilimanjaro in July as part of a Guinness World Record Attempt in aid of CFI. Alongside me will be Siobhan Brady who hopes record by playing the harp for eighteen minutes at the top, while I recite a poem given to us by President Higgins. This level of endurance activity comes with the possibility of extreme weather conditions which means I have more organisation to do than ever before. So whether you are travelling to Kerry or Kilimanjaro this summer, read on for my top travel tips, along with handy resources, to make any trip a success.

Medication supply…

First question – have you enough medication for your trip? If you are taking a short hop, make sure you bring enough medication to last the time and even consider pre-ordering your next month’s supply before you leave. That way you are good to go when you come home and you won’t find yourself short a few day’s supply when you get back.

But what if you are going for a longer stint? If you are travelling for more than two months, it takes a bit more planning. Your CFTR meds are prescribed via the high-tech hub system and the prescription must be renewed every three months, while most other medications are every 6 months. Chat to your CF Team about practical ways to renew your CFTR medication prescription while you are away (if this applies to you), and once you’ve figured that out, consider how you will get the medication ordered, collected and shipped to you, whichever beach you are on or whichever mountain you are climbing!

Taking your meds with you?

I’d say bring your meds in your carry-on luggage. You don’t want your meds to be knocked about, lost or even frozen in the cargo of a plane. But just in case something does happen, bring copies of your prescription with you

Kerry to KilimanjaroEverything you need to know for travelling as a PWCF

and a letter from your CF team detailing what medications you have to take. Not only is this helpful if you need meds while you are away, you might also need it going through security with a bag full of medication in your bag! You could also bring your CF medical ID card for this reason too (available free from CFI)…

For Kilimanjaro, I will be adapting my medications for the climb. I have discussed with my team the meds I definitely have to take on the mountain and which meds I can swap in and out, e.g. two of my nebules can be changed to inhalers shortening my time doing treatments, giving me more time to sleep. I will bring at least two back up antibiotics in case of an emergency.

Nebulisers

Don’t forget to check the voltage and plug requirements of the region you are going to and pay attention to your 220-240 volt box nebulisers. These don’t work on US 120 volt electricity supply, so make sure you arrange a travel nebuliser, source a nebuliser or maybe arrange a nebuliser rental depending on where you are going.

For Kilimanjaro, I will be changing my nebuliser to a battery-operated travel nebuliser which we are hoping will work best on the mountain. I was told batteries have a shorter life at altitude therefore having less medication to nebulise makes more sense as I don’t want to be carrying loads of batteries. I hear you saying “but batteries are light? Surely it wouldn’t be hard to carry!” But like feathers, a tonne is a tonne no matter what it is made up of and we are restricted with weight.

The 'cleaning your nebuliser' decision may be controversial to some, but believe me when I say, I had a long debating discussion with the best microbiologists, weighing up the pros and cons of water quality, even when boiled. We concluded that I do my treatments and seal my neb mask and chamber into a zippy for the period of time on the mountain. Please don’t freak out anyone :)

Hydration

And while we mention water - hydration is, as you know, so important. Don’t forget to pack electrolyte rehydration tables or sachets, such as Dioralyte. You can buy these over the counter in your local pharmacy. Drink plenty of water both on holiday and travelling through airports and on the plane. Air-con is not a friend to hydration.

As the trip in July is an endurance event, I have had to look into best hydration and eating/fuelling for me as a PWCF (I will need to eat and drink more). This wasn’t as daunting for me as you may think as I’ve practiced eating and drinking routines on the bike for M2M so I’m confident I’ll be able to replenish hydration/fuelling on Kilimanjaro. The trick is not to get to the stage of being hungry or thirsty. With CF you don’t always feel thirsty even when your body might crave water due to an impaired thirst response that comes with CF.

Vaccines and Insurance

This one is pretty simple. Make sure your travel vaccinations are up to

date and correct for the country you are visiting. If you aren't sure, ask your GP or call the Tropical Medicines Bureau. For my trip, my travel vaccinations have all been arranged now so the last item to sort is travel insurance.

Travel insurance is extremely important for trips, (if you have private health insurance it is easier and cheaper to get travel insurance). I needed to get extra insurance to cover the climb of 6000m and to cover my CF “just in case”. I have also been in touch with an Irish lady who can help me navigate the hospital system in Tanzania and point me in the right direction if required. It’s better to have the information rather than looking for it in an emergency. Within the EU, EEA area or Switzerland you can also apply for your European Health Insurance card. It is free and offers the same access to public healthcare as the citizens of that country with free or minimal costs attached.

Do your homework

Don’t forget to research where you are going, the conditions there that might affect you, what the voltage or electrics of the destination are, any visa requirements you might need and maybe even where the nearest Irish embassy is. If you are going far, don’t forget to register your details on the citizen’s registration with the Department of Foreign Affairs who can help with unforeseen emergencies such as lost passports or family emergencies. And it’s also a good idea to find out where the nearest cystic fibrosis specialist care centre to you will be and keep their details with you, just in case.

CF Team

If in doubt, ask your team about anything – they are best placed to advise on your specific situation. The elephant in the room for me this time is ALTITUDE, I have no control over what will happen at altitude, I’ve discussed with many people and read articles and the bottom line is it seems to come down to luck and maybe Diamox (a diuretic). Everyone, including PWCF who I have spoken to who have attempted/ summitted Kilimanjaro have said it is one of the toughest but most amazing trips of their lives. As I said at the beginning, travel holds a special place in my heart, so I’m more than happy to give this a go knowing I’ve done everything I can to be as prepared as I can.

Have a lovely summer and enjoy the holliers!

Helpful Resources

Travel Tips – available from CF Ireland

Medical ID Card - contact CF Ireland

Request a list of CF Centres around the world

European Health Insurance Card

Irish Embassies around the world

Irish Immigration Service

Department of Foreign Affairs

Tropical Medical Bureau

CFI MEMBER SERVICES TEAM

Travel Diary of a PWCF:

The Biggest Adventure Of All - Stephen Corbett

Iguess for me the adventure started long before the trip to Chile. Before Covid in fact. If anything, Chile was the end of a much longer adventure. You see after my transplant there was a lease of new life and a sense of deep gratitude for life that I could never explain in words, but there was also an emptiness.

Physically, it was beyond anything I ever imagined. I still pinch myself sometimes nearly nine years later. But mentally, things weren't quite what you would expect. Because for me having no purpose in your life beyond survival had its price. When you achieve that goal, finding an equally profound purpose can be strangely difficult. You live life fully. You go on adventures. You do all the things you always wanted to do.

Then there's... well nothing. Not having a deeper meaning in my life left me in a place that you wouldnt believe a person as blessed as I was, would go to. So for me my adventure actually started in 2018, living at home with no ambition and way more depressed than somebody in my position should be.

In classic romcom fashion, I met somebody that turned my life around. Somebody who taught me not to be afraid of my past or future, somebody who taught me to always be here right now, fearless. And she did all of this just by being who she was. With time, I felt motivated to get my life together, even though I hadn't yet figured everything out. Even when Covid hit, we simply isolated together and we grew even closer with time.

With time I knew there was nobody else I could imagine spending my life with, so I asked her to marry me. She said yes.

If nothing else, that alone made my whole life up until that moment worth it.

So going to Chile was not only a holiday. You see my wife is Chilean, and because of me, she had chosen to stay as long as she had in Ireland. Since she had stayed around three years here because of me, spending three months in Chile was the least I could do.

So we planned and we planned well.

For everything we had a plan B and a plan C. Medicines, insulin, everything. We negotiated with the pharmacy to give us four months of medicine (an extra month just in case), bought four months worth of factor 50 suncream and all the clothes necessary to stay covered in hot weather, long sleeve tshirts, long thin pants and two hats each.

On top of all that there was a lot of documentation to prepare given that this was still on the tail of the Covid crisis. The situation had been taken very seriously in Chile so you needed to be up to date on your shots and follow all the necessary procedures.

But once the preparation was done, it was really no different to anyone else travelling. Three months of meds through customs is never going to be a dull moment after all! There was the usual fun around airports, waiting times, airplane insomnia were the usual expected situations.

When we got there, we had to isolate in the hotel and that was fine too. In fact I'd say you were safer in Chile from infection than in most countries!

We visited many different kinds of places. We went to Elqui Valley, where I saw more stars than I ever thought possible in my life. We walked through the mysterious valleys, only half knowing where we were going, with a bottle of frozen water in the bag to keep the meds cool that we would slowly pour down our backs as it melted, and have a bottle of sports drink each that was really a saving grace in the constant 30° heat. At the end of the day we would swim in the river at the bottom of the valley and listen to the old stories about the place from the locals.

Then there was Villa Rica, the volcanic lakes to the South, which was home to the indiginous tribes of the Mapuche people. If there was ever a place truly untouched by human hands it was here, and gave me an appreciation for the beauty that can be found in the world around us. I could swim in those crystal clear lakes or walk through those valleys of covered in waterfalls every day for the rest of my life and never feel like I needed anything more.

And finally there was San Pedro in the Atacama Desert. This was the trip I had the most anticipation about for the most obvious reason. If these new lungs were ever going to be put to the test, it was going to be at 4000m above sea level! So at first we got acclimatised to a mere 3000m in the town of San Pedro. I felt fine at the time, and surprisingly I loved it. In fact the biggest change I noticed was the freshness of the air at that altitude. The only way to describe it was how clean it felt as you breathed it.

For a few days we spent our time down there. Seeing a desert lit up by stars alone is something I'll never forget. Then on the big day when we went up to the higher altitude of 4000m, something truly amazing happened. Not only was I okay at that altitude, I was actually in better shape than everyone else.

I can't explain it, but in that same moment, I understood. All that time in my life I spent surviving, that simply wasn't enough. Surviving for the sake of surviving will never be enough. However, surviving for the pupose of living a full life full of love and adventure, that's worth the fight, one-hundred times over. A full life full of love and joy is worth every moment of the indignant pain, grim hopelessness and even the blatently irrational grit that goes into surviving. But if you choose to live a good life with it, it's worth it.

So for me, getting to the top of this mountain range at 4000m was the end of a much, much longer journey. And for the past year that adventure is ongoing. I'm creating a life together with someone I love.

And I know in my heart that that's the biggest of all adventures.

SPOTLIGHT

We Irish are well known for being a nation of travelers and emigrants. With around 70 million people around the world who are Irish-born or of Irish descent, leaving Ireland has been a necessity for many at times but has become a choice or a right of passage for others. With few boundaries in place, we are welcomed into other cultures and countries to learn, to experience or to settle down.

Aquick google search throws up inspiring travel quotes such as, "Work, Travel, Save, Repeat..." but making the move out of Ireland is not as seamless as this for everybody. Many in the CF Community, face additional boundaries when making the choice to emigrate. Looking back at previous Spectrum travel diarys, highlights the organisation and meticulous planning that is required when embarking on a short holiday or trip as a person with CF, so in this edition, we speak to PWCF living in Ireland who have faced challenges in following their dreams to emigrate and what it takes to move from the Irish healthcare system to another.

Cruising on the Costa Blanca

Buenos dias, hello my name is Declan Houlihan, PWCF approaching forty one years of age. Readers in the past may have read some of my previous articles for Spectrum and it's a pleasure to be asked to share my experiences once again, this time about my relocation and subsequent life living on the Costa Blanca here in Spain for almost six years now.

If you haven't read before let me give a brief background of my story so far. A travel fanatic from an early age with a deep fascination for the world around me. At the late age of seventeen I was diagnosed with cystic fibrosis after many years of misdiagnosis, stomach pains and an annoying cough!

I was very fortunate to achieve my dream of travelling around the world and living in Australia back when I was 25 years of age, taking advantage of the one year working holiday visa and multiple round the world stops en route. Due to the difficulties obtaining a permanent visa for Australia, mainly due to having cystic fibrosis, I returned back to Ireland but didn't shake off the travel bug. In the following years I was fortunate to travel most of Europe, when possible, with my now wife, and then we made the decision back in 2014 to travel the world again for a longer period. After working hard for many years in the health service and saving some money, it was time to pack my bags and visit north, central and south America, followed

Emigration and CF: Cruising on the Costa Blanca

by another year living in Australia followed by three months travelling around Asia. During this time we documented our travels online via our website, continentchasers.com, where I blogged about places visited and wrote about life living and travelling with cystic fibrosis.

It was then time to come back to Ireland with an empty bank account and get back into the real world with full time work and responsibilities. This however was proving tricky and I simply couldn't readjust to Irish life, particularly the damp climate and grey skies.

The cost of rentals in the Dublin area was the final nail in the coffin and the stresses and strains were starting to impact my health after many years living well despite the condition. After a brief hospital stay with severe stomach pain, my wife and I made the decision to try life in Spain. We had nothing to lose at this point so in August 2017 we decided to move to the Costa Blanca in the Alicante province after some research online. Without a job, the language or much money we felt the benefits would outweigh the negatives.

I was very fortunate that my wife had started the process of getting a job with a real estate agent before we left Ireland so we weren't completely hopeless. The salary wasn't very good but it was a start. Sitting out in our affordable, rented accommodation garden under clear blue skies soaking up the sunshine bought a smile to our face which confirmed we had made the right choice.

The early days were not all sunshine, sea and sand however. Yes, we had a great few weeks upon arrival before my wife started her new job, but my lack of work and worries about receiving medical care and medication were always lingering in the background. In the early days it was simply a case of receiving my medication via courier from my pharmacist back in Ireland. I had six months of my prescription with the view to getting a cheap flight back to Ireland when needed to go for my review in St.Vincent's to obtain my updated prescription when required. As Ivacaftor was one of my prescribed medications at the time, the cost was obviously quite high,

hence sourcing a good, reliable courier was vital. For the relatively low cost of fifty euros each month DHL delivered my medication from the pharmacy direct to my door here in Spain. It was a godsend but I knew this arrangement couldn't last forever.

Before long I was receiving calls from the HSE threatening to cut off my supply as I was not now living permanently in Ireland. I won't go into my feelings but let's just say I was disappointed. I understood the official clerical standpoint, but here I was an Irish citizen living in the European Union, without a job and seeking a new life to improve my health and quality of life. If I stayed in Ireland I would probably have cost the taxpayer more with hospital stays as I aged in the colder climate. Spain was potentially giving me the opportunity to stress less and live a more fulfilling outdoor life.

I guess rules are rules and ultimately I was left without my supply of Ivacaftor for a couple of months while I got things sorted. In the end the lack of medication gave me the push to register onto the Spanish system rapidly, with the help of newly made local friends and a job contract, I was able to obtain the necessary paperwork and get myself onto the Spanish health system following many steps.

This process involved obtaining a NIE number, which is a personal, unique and exclusive number that is assigned to foreigners who, for economic, professional, or social reasons, are engaged in activities related to Spain and require identification in this country. A NIE obtained identifies the bearer to all levels of the Spanish public administration, but does not entitle the holder to reside in Spain or prove residence there. Easily obtainable from the local town hall for a fee, you then need to obtain a Padrón. In simple words, the padrón is a local certificate you receive after registering at the town hall where you live. The certificate for the Padrón in Spanish is the Certificado de Empadronamiento (a municipal register or census record).

The next step was to obtain Residencia (residence permit). Once you have found somewhere to live and have registered yourself on the Padrón, you will need to get your documentation in order so that you can make the residency application and obtain your TIE (Tarjeta de Identificación del Extranjero). This card will demonstrate that you are legally resident in Spain and have been added to the Central Registry of Foreign Nationals or ‘Registro Central de Extranjeros'.

After this process, which took quite a few weeks and involved mountains of photocopying and filling in of forms, I made an appointment with the local hospital and took along a local friend to translate. After my initial visit the doctor referred me to the nearest specialist located in Valencia which is a two and a half hour drive away. I was then entitled to get my medication each month from my local hospital here in Torrevieja, some ten minutes drive from my home. That was that and from here I attend the specialists every three months in Valencia. The first thing my doctor required was a transfer of my medical records from Ireland, after some initial issues they were transferred electronically which gave my new medical team my history to shed light on my background. Since this period my Spanish has improved, I have been put on the wonderful new drug, Kaftrio, and I am living life to the fullest.

I find the facilities in Valencia to be excellent and great attention to detail is paid to all my needs. Added to this I play football at least once a week and attend the gym four days per week alongside working part time. Combined with the sunshine, good food and sea air it all seems to be keeping me in good shape as I approach my forty first birthday.

People usually ask me about travel insurance which is a hot topic for many with cystic fibrosis who wish to venture abroad. My honest answer is I never purchased cover throughout my travels!! This isn’t for everybody but I have been lucky, if there is such a thing after being diagnosed with CF!! I always felt that I could look after myself and that the minefield of obtaining travel insurance was one mountain I didn’t wish to waste time climbing. I witnessed many so called "healthy individuals" easily obtain travel insurance over the years despite smoking, eating poorly and not exercising. I don’t smoke, try to be careful what I eat, exercise regularly, yet because of CF, travel insurance companies penalise me heavily and make it impossible to obtain at a reasonable cost. I have been fortunate not to fall ill in any country on my travels, well not so ill that I needed hospital care, so managed without. It’s not for everybody so I don’t wholly recommend not having travel insurance.

Ultimately I have no regrets and it's how I wish to live my life. I don't want to be on my deathbed looking back on the things I should have done. Of course I have needed some luck along the way and with the help and support of my wife and family it has allowed me to be in this position today. With advanced treatments continually being introduced the life expectancy for people born with CF is rising. When I was diagnosed I didn't think I would live to see thirty, never mind forty years of age!! For now I continue to dream and who knows where I will be in the next five years, if I even am here at all! We all have our own paths to follow and there will be twists and turns along the way. Just go with the flow and you never know where you may end up! Ireland will always have a special place in my heart but for now Spain soothes my soul.

If you need any advice about the move please do not hesitate to contact me. Alongside my wife we run our own real estate business here in Spain so aside from finding properties we have a vast array of knowledge for people who wish to move to Spain, many of whom come from Ireland. Find us at www.agentreddevil.com for more.

¡Hasta luego!I as they say here, see you all soon!

Emigration and CF: Ambitions for Australia

My name is Jodie and travelling the world has always been my ultimate goal. I want to experience new cultures, meet new people and of course spend more than one week a year basking under the sun.

Australia is a place I have had in mind when asked “where would you see yourself living in a few years” since I was a teenager. Having CF it was always a slight concern when it came to travelling as I had learned it could be a difficult process.

In September 2022 my boyfriend and I applied for a 12-month Australian working holiday visa (subclass 417). He was granted his visa within five minutes. I received an email requesting a medical exam, which involved a chest x-ray and consultation with an Australian board approved doctor. The earliest appointment I could get was in Cork for November.

The doctor I met with was very pleased with me and all my medical records, which I needed to gather from my CF clinic team prior to the examination.

Although he couldn’t give me an answer, he was quite hopeful as I am luckily extremely fit and healthy, with a lung function consistently above 105% over the last 6 years and in the 120% range in the last two years. I also haven't needed hospital admission in 12+ years for IVs or any other reason.

It wasn’t until January that I heard back from Australia, after some unhelpful phone calls about the amount of time that had passed, that they would be requesting some additional information to support my case.

I was initially disappointed as I was told in this email that my visa was looking unlikely due to the potential cost risk associated with travelling with a chronic condition. This turned to optimism as I contacted my CF team, my GP, my chemist, CFI, the clinic from my visa medical exam, my local TD and VHI to organise a purchase of an extensive health and travel insurance policy.

All of the medical professionals I contacted provided me with very positive letters proving that I am in excellent health and should cause no concern in relation to my health for extended travel. My pharmacy assured me I would be able to bring medication with me from Ireland, as many people have done before whether they are working, studying or travelling. CFI provided a letter which referenced the reciprocal health agreement between Ireland and Australia to help my case.

My local TD made contact with a representative in Australia who advised me to set up a health travel insurance policy to cover all costs and that should be enough if I was admitted into hospital the cost would be covered by my insurance. I sent my letters in February.

After patiently waiting I made a few more phone calls, one of which told me my application would be prioritised. Three days later my visa was denied for not meeting the health requirements.

I was extremely disappointed and frustrated with this decision. CF is a very individualistic condition which affects people in many different ways. At this stage in my life I feel as capable, fit and healthy as ever.

It is a big concern of mine that now that I have been refused once it will have a negative impact on my ability to travel as it is something that will have to be highlighted on all future visa applications for other countries too.

At this stage I am unsure if an Australian tourist visa is possible as I was informed by an Australian immigration agent, it would require a second application and potentially a second medical exam which is another huge cost to undertake again and potentially be refused.

Seven months of waiting for an answer been stressful. When I first applied I thought “at least by the new year we’ll have a plan in motion”. It was difficult trying to make big life decisions while waiting for a reply. Putting off holidays and events with friends and family because I felt like I couldn’t commit to plans, moving back home “for a short period of time” I thought.

It was difficult telling family and friends who were so excited for me that I was denied my visa, each time I was met with shock and sadness. I believe that each individual is unique and deserves to be seen independent of assumptions and prejudices. I am aware that on paper all I seem to be is a “person with CF”, but there is so much more.

My next step involves looking at something closer to home. I will hopefully be able to experience the joys of travelling and living abroad in Europe in this next chapter of my life. Although Australia hasn’t worked out this time, I will not let this refusal hold me back. Hopefully there will be other opportunities for me to see what Australia has to offer and to be viewed as the individual I am. For now though, there is plenty of the world to see.

Emigration and CF: Transferring to Texas

Hello, my name is Anastasiia and my son, Timo, is a person with CF. We recently moved to Texas from Dublin where we arrived after leaving Ukraine because of the war last year. This is our story.

We started to plan moving to the USA nearly 4 or 6 years ago, when Tim was about 5 years old and when I got the first information about Kaftrio. I understood that Ukraine can't give even basic free treatment for its patients, so we need to move. My husband is an IT manager in a USA company, so we had a real plan.

In Autumn 2022 we received confirmation of relocating and started the visa process. Then the war started in February and we moved to Poland to be safe and continue our son's treatment. Then we received an invitation to the appointment in the Dublin visa centre. In Poland there were long queues and my husband’s employer

looked for the fastest way to get us to the USA but the war and processes made it difficult. When we came to Dublin, we found that there was a mistake with that appointment. It was scheduled for another type of visa and we had to wait for an undefined time. Luckily, we met an Irish family that hosted us and helped a lot. We lived in their house for 6 month while we waited for a visa. The appointment took 5 months from when we arrived and then there was one more month to wait for visas and buy tickets and get organised.

The CF care my son received in the Dublin centre was at the highest level. He is stable so he didn’t need hospital treatment. We just visited the CF centre for checks, tests, prescriptions. All doctors and staff were very qualified and understanding. Also, I mentioned that the quality of medicines is better than in Ukraine. We have a very similar Irish health system in Ukraine-social insurance. The problem in Ukraine-it doesn’t

have enough money to cover all promised by government services. CF medical help in Ukraine was very bad, but in the last few years it has got better and even the war. We have a strong patient community there and doctors-enthusiasts who really care about their patients. Thankfully to those doctors, my son is in good condition. Ukraine uses European CF treatments protocol and it’s not differing much from Irish one. But the opportunities of the system differ. The one complaint about the Irish system-is the long waiting time for specialists (not CF doctors, for example for paediatric endocrinologist the queue was more than 1 year!!!)

We received notice that our visa was ready and we moved. But before arriving in the USA, I checked which CF centres are closer for our place. Then I read reviews and ratings for those centres and also I found some information on social media. It was possible to choose which one we want but only in the insurance network from the health insurance.

When I chose the centre, I immediately wrote to them, informing that we are transferring from Dublin to Dallas, and would like to receive care in their hospital. I asked what I needed to do to make my son receive care there. First of all it was insurance. Actually it was a struggle to figure out what plan is better, what's the difference etc. We spent a lot of time speaking with HR, trying to select the best option.

The issue was that we couldn't get insurance without being in the USA and passing some documentation process. The nurse coordinator understood that and asked transferring documentation from Dublin hospital. In a couple days all documentation was sent. As soon as they received it, they scheduled an appointment. But our responsibility was to come to that appointment only with insurance. Thank God, all these factors came together and we succeeded in attending the CF doctor during our first month in The USA. In the hospital I received a lot of information about different support programs for basic medications.

I installed the mobile app where I can schedule appointments, get consultation, and all information about tests and visits is there.

Now, Tim is doing good at school. He goes for swimming, basketball and coding classes. He has friends here and I hope a happy childhood. We live in quite suburban Dallas and life is more quiet and calm, like for

Europeans. You can’t just leave home and go for a walk. You need to drive. But the place is nice, safe.

We are trying to travel, but there are long distances!! The USA is huge! Everything is bigger in Texas: cars, distances, houses, food and drinks. But this place is not as safe as they look. A couple weeks ago it was shooting in the mall in 15 minutes from us. And even if you are living in a good and safe place with great schools, you can’t feel fully protected from the guys with weapons. It’s a big problem here. However, we have a big Ukrainian community, attending Ukrainian fundraising meetings, and volunteering, like I did in Dublin a little bit.

After my son started Trikafta, I have a lot of free time. I haven't had that feeling since he was born. I can study, can have a full-time job, and stop worrying about his health every minute. It's a great feeling. I am finishing a software testing course and will look for job opportunities soon.

Actually, recently we had a check at the hospital, and I had some conversation about work for The CF Foundation. So cross fingers!

Cystic Fibrosis on the TV....

Our CF Advocates, Caroline and Rory, were recently involved in providing insight to the writers of new adult crime drama, Clean Sweep, on life with CF. Well done to the writers for an excellent portrayal of CF and to Aidan MCann, the young actor who plays Niall Mohan, a young boy navigating everyday life as a PWCF. The series is available to stream on RTE player.

The CFRI

Using Data for Good

Connecting with international colleagues in CF research and practice allows CFRI to maintain its high quality approach to data collection and research through learning and sharing best practice from both researchers and registries internationally.

From 7th – 10th June 2023, a team of three CFRI staff members travelled to to Vienna, Austria to participate in the European Cystic Fibrosis Society Conference 2023. We attended meetings relating to international collaboration projects including the global CF collaboration and the Pharmacovigilance group meeting as well as attending the European Cystic Fibrosis Society Patient Registry (ECFSPR) steering group meeting. In between meetings, we got to learn from international research and practice across a broad range of topic areas on the impact of modulators, pulmonary exacerbations and microbiology, and pregnancy and fertility amongst others. Att4ndin Attending the Attending the conference and learning from experts in the field ensures we can stay up-to-date on the latest research and remain fit-for-purpose.

We also presented a poster illustrating the value of 20 years of CFRI data (our report will be out soon so keep an eye out). ECFS is a highlight of our year and certainly demonstrates the value for CFRI in engaging and learning across borders. CFRI work across borders through engaging in the ECFSPR. Every year, we submit anonymised and summarised data to the ECFSPR, along with over 40 other countries. The ECFSPR includes summary data from over 50,000 PWCF across Europe.

“The information is used to measure, survey and compare aspects of CF and its treatment in participating countries, to deepen our understanding of CF, to improve standards of care, to provide data for epidemiological research and to facilitate public health planning1”

The ECFSPR is a powerful source of data on PWCF across Europe and is used to ultimately improve the outcomes of PWCF through informing research, clinical practice and public health planning. ECFSPR also publish an annual report and associate country posters which can be found here: https://www.ecfs.eu/ ecfspr/posters. Updated versions are currently being prepared by the ECFSPR team. The 2021 report has just been published and can be found here: https://www.ecfs.eu/projects/ecfs-patient-registry/annualreports.

Want to get involved? Consent

If you would like to participate in the registry, please contact your CF Centre of the CF Registry at info@cfri.ie Participation is entirely voluntary. You are free to revoke your consent and withdraw from the registry at any time. If you have any questions about participating in the registry or about the data we collect, get in touch with us at info@cfri.ie.

1 https://www.ecfs.eu/ecfspr

CFI ANNUAL CONFERENCE

2023 , DUNBOYNE CASTLE

COVID-19 CF Study Results Summary

Dr Rini Bhatnagar

During the Covid-19 pandemic, CFI partnered with a research team led by Prof Patricia Fitzpatrick in UCD to investigate the impact the pandemic was having on people living with CF. Here, Dr Rini Bhatnagar, researcher with the team, describes what we learned from that study.

The COVID-19 pandemic significantly affected children with Cystic Fibrosis (CWCF) and people with Cystic Fibrosis (PWCF). Disruptions in healthcare, school closures, and changes to employment were a few of the many challenges faced. These challenges were felt by the whole population but potentially more so by those living with CF and their families. The aim of this study was to quantify the impact of the first six months of the pandemic on the lives of PWCF and parents of CWCF in terms of hospital visits, telemedicine use, education, employment, and mental well-being.

An online survey was conducted among PWCF and parents of CWCF, asking about their experiences during the pandemic in terms of hospital visits, precautions taken, impact on education, impact on employment, telemedicine, and mental health. A total of 119 PWCF and 123 parents of CWCF participated. The study was approved by the National Research Ethics Committee for COVID-19 (20-NREC-COV-090).

Results showed that almost half of PWCF (47.5%) and over half of parents of CWCF (55%) deferred hospital visits due to fear of encountering COVID-19. Some units were closed (19.4% parents of CWCF; 11.5% PWCF). This deferral of hospital visits is concerning, as routine examinations are essential for early intervention in infective respiratory exacerbations and other CFrelated diseases.

The pandemic also led to a shift in how adults with CF and parents of CWCF connected with CF care staff, with online consultations and prescriptions via email being introduced. These methods were new to 47.6% of PWCF and 56% of parents of CWCF, and >80% found them helpful. Small numbers of both PWCF and parents of CWCF used online education or online physiotherapy classes, but those who did “found it very helpful”.

Employment of PWCF was also impacted, with many working from home or not working while cocooning. Over one-third of employers (44.3%) were perceived as unsympathetic towards PWCF who were not working during the pandemic. 45% per cent of parents of CWCF of children aged up to 4 years were hesitant to send them to crèches or playgroups, whereas a majority of parents (46% of primary and 45% of senior school CWCF), agreed that their children completely comprehended the extra measures to be taken during COVID-19.

The pandemic greatly impacted the mental health of both PWCF and CWCF. Younger PWCFs experienced more mental distress than the older group, experiencing increased levels of nervousness and tiredness. CWCF reported increased anxiety (26%), stress (33%), insomnia (11%), and irritability (11%). Families turned to activities such as watching TV, doing art and craft, gardening, and communicating with loved ones to cope with these challenges.

The COVID-19 pandemic has had a significant impact on the lives of PWCF, CWCF, and their families, and ongoing attention and support are needed to address the challenges they face. The study highlighted the likely need for more psychological support in future. Online consultations were found to be effective during the peak of the pandemic and could be useful to some extent in the future. Prescription via email was introduced successfully during the pandemic and could be continued for repeat prescriptions.

Visit the CFI website to read the full research paper publications: https://www.cfireland.ie/research/research-news/research-findings-the-impact-of-covid-19-on-childrenand-adults-with-cystic-fibrosis

Cian Greaney, PhD student from the University of Limerick, was awarded the Best Poster Prize at the European Cystic Fibrosis Society Conference in Vienna for his poster "Diet quality assessment in adults with Cystic Fibrosis - comparison to population dietary guidelines. A cross-sectional study". Cian works with Dr Audrey Tierney in UL on the Cystic Fibrosis Ireland and Health Research Board funded study. The team are in the final stages of analysis and Cian has shared the findings of his work with a presentation and poster at ECFS. Cian also recently presented at the CFI annual conference.

It was great to see Dr Rini Bhatnagar from UCD presenting results from the Irish Comparative Outcomes Study in CF (ICOS) at ECFS in Vienna. Rini gave two presentations highlighting the challenges of living with CF.

IRISH COMPARATIVE OUTCOME STUDY OF CYSTIC FIBROSIS (ICOS)– PART 2

The ICOS study Part 2 is an ongoing study since 2020, addressing the key outcomes of the Cystic Fibrosis Newborn Screening Programme introduced in Ireland in 2011. This study is a continuation of ICOS Part 1, conducted from 2013 to 2017.

Who are the research team?

This study is led by Professor Patricia Fitzpatrick, Full Professor of Epidemiology and Biomedical Statistics at University College Dublin (UCD), and her research team at UCD, in collaboration with Paediatric Cystic Fibrosis Consultants and nurses from five specialist CF hospitals: University Hospital Limerick, University Hospital Galway, Cork University Hospital, Children’s Health Ireland at Crumlin and Children’s Health Ireland at Tallaght. This study is funded by the HRB.

We have 3 main objectives:

1. To compare the health outcomes and the impact of newly introduced CFTR modulators on children who were screened and those who were not screened for CF.

2. To look at the childcare costs incurred by parents and by the health services related to CF care

3. To look at the impact on quality of life and the challenges faced by the parents of children with CF.

What does this study require from you if you participate?

The study requires consenting parents to allow the researchers to access their child’s medical charts and CF Registry data, and to complete cost and quality of life questionnaires over the telephone/by post/email. All data is totally confidential.

What are the benefits to me and my child?

1. Better understanding of the effects of newborn screening for Cystic Fibrosis.

2. This study is a great opportunity to look at the effectiveness of newly introduced CFTR modulators at a national level, which may inform future medical practice.

3. We will be using the Challenge of Living with CF-Short Form questionnaire, specifically designed for parents of children with CF. This will allow us examine the key challenges faced by parents and identify what supports are needed.

We are inviting all parents of children with Cystic Fibrosis in Ireland that were born after July 2008 to take part in this study with their children.

In order to participate: Please ask for the “ICOS CONSENT FORM” from your child’s CF consultant or specialist nurse.

CF Expectations

What are we currently doing?

Jen Balfe, PhD candidate, supported by CFI, RSCI and the Irish Research Council, is conducting interviews with women with CF and the healthcare professionals involved in their care as they access fertility and maternity services in Ireland. The interviews take place online and help to inform our knowledge and understanding of the experience of fertility and pregnancy in Ireland.

What do we want to learn?

We want to learn from the voices of experience about how people with CF and their healthcare teams navigate fertility and pregnancy journeys. Women with CF who have been pregnant or have accessed fertility services in Ireland are the people with the lived experience who can help us learn about what being pregnant and becoming parents with CF in Ireland is like.

We also hope to learn from the many different healthcare teams/professionals involved in the care of women with CF during this important stage of their life about how they support women with CF and their families through fertility and pregnancy journeys. This is the first study of its kind in Ireland. Given the growing number of women with CF becoming pregnant in Ireland and internationally it is a timely study which will hopefully shed light on the enablers and barriers to care. Interviewing and listening to people who have first-hand knowledge and experience will hopefully lead to better future knowledge, awareness and supports for women with CF, their families and their healthcare teams.

What do we hope to achieve?

We hope that the findings of the study will help to inform a resource booklet/companion guide for women with CF, their families and their teams. We want to produce something tangible that women can hold in their hand to help support them while they access fertility and maternity services. We will also use the findings from the study to make recommendations for the future care and service provision for women with CF in Ireland who may become pregnant in the future.

Why is it important to take part?

Your voice is important and can make a difference for how women with CF and their healthcare teams experience accessing and providing fertility and maternity services in the future.

How can you get involved?

If you are a woman with CF in Ireland who has accessed fertility/maternity care or a healthcare professional who has cared for women with CF during this period you can find out more about the study or register your interest by contacting jenbalfe21@rcsi.ie or on twitter @CFExpectations.

Julie Bernard: Parent of child with CF

After a rollercoaster year with the Kaftrio 35 campaign I was very grateful for the opportunity to attend the ECFS Conference. The conference was excellent, with a really well-designed programme and sessions and the culture is one of openness and accessibility. Based on the sessions I attended and poster presentations, ten recurring themes emerged:

1. Diversity of patient groups with CF and people impacted by CF screening and genetic issues

At the opening plenary, it is noted that there are now four different groups of people with CF: those who started new CF drugs later in life; those who started modulators at an early age; those who are eligible but can’t tolerate modulators and those who are not eligible for existing modulators. During sessions about CF screening and genetics, it is clear there are other groups of people who are also impacted by some aspect of CF screening and genetic issues including CFSPID/CRMS (CF Screen Positive, inconclusive diagnosis; CFrelated metabolic syndrome) and CFTR-related disorders; those who receive a false positive test; those who are carriers or potential carriers of CF variants. I find myself wondering who is the CF-community? How are so many diverse groups supported?

2. How best to support the changing needs of diverse groups of people with CF?

There were discussions about how diversity was always a feature of CF but that the diversity of impact and need is now wider. So much is improving for many on modulators, but there are also new issues emerging, ongoing learning about CF genetics, so much is still unknown about the impacts of modulators and living longer with CF, ongoing research is required to enable all people with CF to avail of new and better treatments and for some of the patient groups, traditional CF impacts and care apply. Thus, it is often a case of additional needs.

3. Mental health impacts of CF

There was a clear recognition of the need for better psychological supports for both people with CF and parents and family members of children with CF, with presentations on the value of and approach to psychological screening of pwCF and parents/guardians as a standardised part of care.

4. Understanding of transitions and identity change

The case was made for a stronger recognition and understanding of the nature of the transitions and identity changes that are emerging for people with CF and parents of children with CF arising from the

The 46th European Cystic Fibrosis Conference was held in Vienna in June 2023, here some attendees share their thoughts and experiences of this year’s conference.

use of modulators. In additional to psychological support, there was a need for specialised career and education supports, including access to lifelong learning for changing life circumstances, an ongoing need for financial supports and a requirement for flexible work models.

5. Living with CF and life stages

Research was presented on key life stages and CF including: the importance of early and dedicated support for discussions about issues of sexual health; the menstrual cycle and CF; reproductive options; pregnancy and modulators; aging and CF and the need for further research about menopause and CF.

6. Adherence

Many papers addressed the issue of adherence but often focussed either on only one aspect of treatment adherence, which does not capture the reality of treatment adherence with CF, or when various aspects of CF treatment adherence were addressed in research studies, the focus was more on the barriers to adherence with less attention paid to the bridges to adherence and the success factors.

7. What model of CF care and additional professionals would assist with this diversity and changing nature of CF care?

My own conclusion from 10+ years of dealing with CF treatment adherence as a parent was that the critical factors are full emotional - not just rational - acceptance of the diagnosis together with family-specific systems and schedules and hence that all CF teams would benefit from the addition of an OT who could visit the family home and assist with diagnosis acceptance and the development of bespoke systems and schedules. It was particularly interesting then to hear a presentation from the first OT in a CF MDT in the UK. She spoke about the benefits of different models of care and the value of moving from MDTs to Interdisciplinary teams and Transdisciplinary teams. There were other examples of additional professionals joining CF teams including a youth worker and a mental health navigator.

8. CF variants and ethnicity

There were recurring references to the issue of CF variant panels used for screening, ethnic diversity and the risk of missed diagnosis. With Ireland’s changing population, I wondered if this issue was under ongoing review in Ireland?

9. CF and health inequalities

A number of presentations drew attention to health inequalities including global inequalities regarding access to modulators and other CF treatments. Data from CF Registries showed very positive developments regarding survival rates and other outcomes but there were stark differences between high/middle/low-income countries. A presentation on access to CF care in South Africa also showed inequalities within a country. The nature of CF care and treatment adherence requires resources and access to resources varies: do we have data and have we assessed outcomes and quality of life for people with CF in Ireland based on socio-economic group/social class and by ethnicity?

10. Caregiving, language and co-creation of knowledge ‘Caregivers’ at the conference often seemed to refer to the MDTs providing care to people with CF. But people with CF are also key providers of care to themselves, as are family and friends for children with CF and likely for some adults with CF. Other professionals and patient support groups also provide care. Adopting this wider definition of care, caregiving and caregivers would provide a stronger, more inclusive basis for engaged research and co-creating the types of knowledge that are required for physical and mental health, effective care and treatment adherence and the transitions, identity changes and quality of life issues over the life course that are now part of the diversity of experiences with cystic fibrosis.

It was an incredibly positive experience to attend such an excellent conference of 2000+ people who are all committed to improving the knowledge and care for people with CF. Thanks to CFI for this opportunity and support.

Carolyn Thornton, parent of a person with CF, shares an update on Phage therapies

Due to the emergence of multi drug resistance bacteria worldwide, there has been a lot of research into the use of phage in the treatment of infection. Phage are a group of viruses that specifically target bacteria as hosts for their replication process. The end result is lysis (bursting) of the cell. This phenomena was first investigated by the Soviet Union as a method for treatment of bacterial diseases such as dysentery and gangrene in soldiers during WW2. Phage will bind to a bacterial cell but not to a human cell which makes them a useful tool. Further refinement and research has continued and phage treatment to this day is used in Russia, Georgia and Poland.

The Western World did not embrace this treatment at the time and abandoned phage therapy due to difficulties with strain specificity and purification. This was also the era when antibiotic treatment had begun to be used effectively, and multi drug resistance was not an issue.

Now the medical and scientific communities are back looking at phage with a lot of exciting research and development. At the ECFS Vienna 23, there was a Symposium devoted to the topic; the main areas being researched are how to match a phage to the bacterial host as specificity is a fundamental aspect, bacterial resistance to phage and effectiveness of phage alongside antibiotic treatment. Research is taking place in Valencia, Copenhagen, Pittsburgh, Massachusetts, Iowa & Israel.

In particular Dr.Benjamin Chan, Yale University, New Haven, USA delivered a fascinating lecture on the practicalities of phage use in Clinical practice. They have treated approximately 32 patients who have been referred to them for compassionate use of phage.

The first step is to identify the exact strain of infectious pathogen, which was a strain of pseudomonas aeruginosa (PA) in the patient case he spoke about (although they also have used phage treatment to treat MRSA, and NTM in another centre) The next step is to find a phage that matches the binding receptors on that particular bacterial strain from a library. This is called characterisation and production is then optimised. Invitro then testing occurs for sensitivity and finally there is a treatment plan. Phage treatment can be

effectively deployed by nebulisation.

In the case study referred to, a female living with CF, pan resistant PA with a FEV% decline from 90-30, had resistant strains and a high bacterial load. A course of phage was administered, and post therapy her FEV increased to 90%. Most importantly there are no evident side effects from phage and the female has been stable for 3 years.

Although Phage therapy is now considered to be another tool, there is still a lot of work to be completed in terms of susceptibility, bacterial resistance, and how this treatment works alongside antibiotics, along with a concern that neutralising antibodies may eliminate the effectiveness of phage. Nonetheless it gives a glimmer of hope to those pwCF with chronic infections.

Rory Tallon, CF Advocate CFI

It was an honour to attend the 2023 46th European CF Society Conference in the Austria Centre Bruno-KreiskyPlatz in Vienna as part of the CFI delegation. How amazing to be sitting among the leading experts listening to medical updates on the impacts of CFTR modulator therapies in CF. As I listened in, fully appreciating my 2 years of Kaftro treatment, my mind drifted back to Vienna in 2001 at the 24th ECFS where I first heard of the preclinical trial results of the Vertex VX compounds and the huge promise of these compounds on the horizon.

The optimism was palpable in the lecture halls then. So here now in June 2023 – 22 years later at the same conference again, learning of the lasting impacts of triple therapy, learning of new and possibly more effective Vertex modulator compounds in preclinical trials currently and of genetic therapies- mRNA therapies that can be delivered through a renewed focus on lipid delivery via highly developed and extremely specialised lipid nanoparticle delivery systems. These new mRNA therapies have the potential to treat everyone with CF- they are not mutation specific (mutation agnostic) The last 10% of people with CF who are ineligible for existing CFTR modulators may just have reason to hope and believe that maybe in 20 years’ time they too could be attending ECFS listening to real world experience - beyond clinical trials- of the impacts of corrective mRNA treatments.

Science and medical progress aside, it was most rewarding to meet some of the dedicated researchers CFI have funded – who were in attendance to learn and to share their research. We met Dr. Niamh Coffey from UCC Dental school investigating oral health in CF, Cian Greaney from UL researching diet quality in CF, Rini Bhatnagar from UCD studying outcome experiences of CF genetic diagnosis and Jen Balfe from RCSI researching pregnancy experience in CF.

It was wonderful to chat to them, some briefly and some over a meal out and to feel their passion for their research. It was equally rewarding to meet and get to know the parents of PWCF, Julie and Carolyn, who were also part of our CFI delegations with Philip Watt and myself.

Fascinating also to chat to the folks from CF Registry of Ireland and David from UK CF Trust. You learn so much from these conversations that you don’t always get from direct lectures. The ECFS is extremely technical, scientific and medical, it is not for a lay audience. It is where the experts meet, share and learn with our Irish CF specialists clearly right up there with the best of them.

Cian Greaney, PhD candidate, University of Limerick

The dynamic evolution of CF clinical practice and new therapies within recent years has undeniably led to remarkable advancements in health outcomes for a significant segment of individuals affected by CF. Nonetheless, alongside these commendable achievements, novel challenges have surfaced for both people with CF and the multidisciplinary teams entrusted with their care.

Given this transformative landscape, I see there being a paradigm shift in CF research, gravitating towards the exploration of preventative approaches in CF management, aiming to mitigate the onset of overweight and obesity while addressing the psychological impact and body image concerns stemming from the complex interplay of mental and physical tribulations present in the CF experience.

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 1st to 7th: Paris2Nice

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

You can now register your interest in participating in the Paris2Nice Cycle in 2024 on our website –www.cfireland.ie

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

September 10th: Head2Head Walk

On Sunday, 10th September, our 11th Annual Head2Head Walk takes place from Howth Head to Bray Head. This fabulous walk along the sea front from Howth Head to Bray Head goes from strength to strength, thanks to the wonderful organisation of the event by the Head2Head Walk Committee led by Mary McCarroll, Jem & Lorraine Downes and Glen McDonnell.

You can avail of our Early Bird discount by REGISTERING NOW at www.cfireland.ie before June 30th. So don’t delay, register now to avail of the early bird discount to secure your spot! We are really looking forward to meeting everyone in Howth on Sunday, 10th September!

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

September 14th to 17th: Malin2Mizen Cycle4CF

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

Registration is now closed for Malin2Mizen 2023. Good luck to all our participants in the event and we look forward to supporting you over the course of the four days! Please come out and support our cyclists if you can and we will publish the route the evening before each day’s cycle as we journey from Donegal down to Cork. For any queries, please email fundraising@cfireland.ie or phone us at (01) 4962433.

September 19th to 26th: CFI International Walk

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

Walkers will get to enjoy diverse culinary experiences, from the traditional plate of eclectic Mediterranean food to varied International menus in local and first-class restaurants and wine bars. And also experience the warm and friendly Maltese culture in a safe and tranquil environment that few other destinations can rival.

We have a very limited number of places left for the walk, so if you are interested in joining, please contact our fundraising team at fundraising@cfireland.ie or phone 01 496 2433 as soon as possible.

October 29th: Irish Life Dublin Marathon

The Irish Life Dublin Marathon returns on Sunday, 29th October.

If you have secured a place in the Marathon would like to be part of the Cystic Fibrosis Ireland Team taking part, register your interest NOW on our website at www.cfireland.ie.

We will contact you and provide you with your Cystic Fibrosis Ireland shirt for the day and fundraising pack!

For further details, please contact us on 01 4962433 or email fundraising@cfireland.ie.

November 5th: TCS New York City Marathon

Another hugely popular event, the New York City Marathon, is one of the world’s greatest participatory events. For anyone who takes part it is always an unforgettable experience. The Marathon on November 5th runs through all 5 city boroughs (Staten Island, Brooklyn, Queens, The Bronx and Manhattan).

We can provide you with a guaranteed place if you book your trip with Cystic Fibrosis Ireland. Places are limited so register your interest to take place now on our website at www.cfireland.ie and we will contact you to confirm next steps as soon as details for this year’s event are confirmed.

If you would like any further information please call us on 01 4962433 or email fundraising@cfireland.ie

All Year Round: 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 2023. 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 www.cfireland.ie.

December 8th: Christmas Jumper Day 4 CF

On Friday 8th 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 8th 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 your 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

• Post a cheque to Cystic Fibrosis Ireland, 24 Lower Rathmines Road, Rathmines, Dublin

• Donate online at www.cfireland.ie

(Please remember to include your companies name as a reference so we know who it’s from)

Don’t forget to 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 2023 – Ho Ho Ho!

Thank You FUNDRAISING:

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:

65 Roses Day

Cystic Fibrosis Ireland would like to say a huge thank you to all our volunteers who supported our national fundraising day - 65 Roses Day on Friday 14th April. So far, over €278,000 has been raised. We are really grateful to all our volunteers who sold roses across the country in Dunnes Stores, shopping centres, schools and workplaces. Well done and thanks also to everyone who organised a 65 Roses Challenge. Due to the large number of events it would not be possible to list them all but they included gym challenges, school challenges, bake sales, 65km cycles, 6.5k walks and runs, 65 hill climbs, 65 park runs and 65 Roses golf challenges to name just a few.

If you still have funds to lodge from either 65 Roses Day or from a 65 Roses Challenge we would ask that you lodge the funds our fundraising account:

IBAN IE59 AIBK 931071

08578593 BIC: AIBKIE2D

or contact us by email: fundraising@cfireland.ie or phone (01) 4962433 and we can talk to you about the various options to lodge cash collected.

65 Roses Day continues to grow each year and this is made possible with the help of all of our volunteers and branches. We look forward to making 65 Roses Day 2024 even bigger and better again.

Duleek 10k

A massive thank you to Anne Noone and the organising committee for the Duleek Cystic Fibrosis 10K Remembrance Run which took place on Sunday 24th, April. Also of course thank you to everyone who took part in the wonderful event with over €23,000 raised to date and monies still coming in.

VHI Women’s Mini Marathon / One in 1000

A huge congratulations to everyone who joined our One in 1000 Team to take part in the Vhi Women’s Mini Marathon on Sunday, 4th June. Participants joined us in the D2 Harcourt Hotel where we relaxed before the start of the 10k. Afterwards everyone relaxed with some music, food and some refreshments. Thank you to our sponsors Ballygowan and Fyffes for providing the water and bananas which went down a treat!

Thank you again to everyone who took part and if you need any help lodging your fundraising monies please contact us on 01 4962433 or email fundraising@cfireland.ie. Don’t forget to share all your pictures on your Social Media #Onein10002023 and we will add them to the gallery of pictures from the day on our website.

Brian Doyle – Truck Covers

A huge thank you to Brian Doyle and all the sponsors namely Casey Brothers, Cunningham Covers, Keoghs Garage, MB Graphics, Mount Leinster Transport and Walshes Homevalue for advertising our 65 Roses Day challenges on their trucks.

Bray School Project

A big thank you to Bray School Project for fundraising an amazing €1118 for 65 Roses Day in aid of Cystic Fibrosis Ireland. It would not have been possible without the help of Aaron and the staff members!

Casey’s Centra, Rathdrum

A

A huge thank you to Mary Farrell, Liz O’Sullivan and everyone involved in this amazing fundraiser for 65 Roses Day organised by Casey's Centra in Rathdrum, which raised a massive €2,710 for Cystic Fibrosis Ireland.

Pictured below are Mary and other staff members from the store presenting the cheque to Fergal Smyth on behalf of CFI.

Vinny McLaughlin – 65 Roses Golf Challenge

A huge thank you to Vinny McLaughlin who raised a massive €15,000 from his 65 Roses Golf Challenge - '65 holes for 65 roses'.

Pictured presenting the cheque to Cystic Fibrosis Ireland from R to L are Vinny McLaughlin, Marc McCarron (PWCF) and Fergal Smyth on behalf of CFI.

TCS London Marathon

We would like to thank Kieron Bracken for taking part in and successfully completing the TCS London Marathon on Sunday, 23rd April on behalf of Cystic Fibrosis Ireland and raising a massive €1,005 - we really appreciate this!

CFI Branch Secretary Contact Details

Sligo Iris Murphy carrowcashel@eircom.net

Mayo Billy O'Toole billyotoole1@gmail.com

Galway Kayleigh McGuire kayleighmcguire0@gmail.com

TLC4CF

Caitriona Hayes caitrionahayes@icloud.com

Clare TLC4CF@cfireland.ie

Limerick

Niamh Harrington niamhh75@gmail.com

PWCF (online) Saoirse Perry saoirseperi@gmail.com

Cavan

Martina Plunkett mplunkett1000@yahoo.ie

Cork Aisling O'Neill aislingquill@gmail.com

Tipperary

Mandy Quigley mandyquigley26@gmail.com

Drogheda Frances McDonagh fmurray67@ymail.com

Dundalk Rachel Mellon rachelmell.92@gmail.com

Meath info@cfireland.ie

Dublin West Rebecca Horgan cfdublinwest@gmail.com

Eastern Branch Mary McCarroll marymccarroll1@hotmail.com

Carlow Leigh Bolger bolgerleigh@yahoo.ie

Wexford Suzanne Doyle suedoyle74@gmail.com

Waterford Ruth Carberry rcarberry02@gmail.com