Spectrum - Summer 2022

SPECTRUM Summer 2022

10 years shoulder to shoulder at the Head2Head walk Register for the 2022 walk at www.cfireland.ie

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CONTENTS Latest News | 1 - 2 *FAQs on The Exclusion of 35 Children with CF from Kaftrio

Member Services|3-5 *New 'Additional Needs' Payment - DSP

CEO’s Message

‘Health care delayed is health care denied’

*Travel Diary: Disneyland Paris

Spotlight | 6-14 Sandy Toes and Salty Kisses - Parenting with CF *Rory and Florence *Carol and Abigail *Caroline, Anna and Jamie

Teen Titans| 15-20 *Keep It Up Ava! *Secondary School and CF by Darcy Grainger

TLC4CF | 20-21 *Redmonds of Roscrea - Happy Days *Limerick Panto Society

CFRI | 22-25 *CFRI Report 2020 *The CFRI

Conference Review| 26-31 *ECSF 2022 Review *CFI Conference 2022 Review

Hospital Hub | 32 *CHI Crumlin - CF Exercise Hub

Fundraising| 33-39

Philip Watt, Chief Executive

There is an well-known saying in legal circles that ‘justice delayed is justice denied.’ The same can be applied to healthcare, particularly where there is a human rights dimension.

The exclusion of 35 children with CF from Kaftrio is very much a human rights issue. The inequity of the pricing dispute that caused this problem is further underlined by the fact on the same day that 35 children were denied access, a further 140 other children with CF successfully gained access to Kaftrio.

CFI continues to say to those impacted children and their families, ‘we are here with and for you. We will not give up until your rights are vindicated’.

CFI is also striving for ‘future proofing’. No other child with CF should endure the same devastation of being excluded from a drug therapy that has been recognised as revolutionising CF care in Ireland and many countries worldwide.

Ukraine CFI is pleased to let you know that 3 people with CF from Ukraine are now in Ireland receiving help and care. We have also donated €10,000 to date to support coordinated efforts at a European level, including the direct delivery of medications and support for the Ukraine CF Association. CFI thanks everyone for their support including during 65 Roses week and we will be making a further donation soon.

*Challenges and Events *Thank You

EDITORS: Samantha Byrne & Nicola Delaney Foxe

Philip Watt, CEO, CFI

COVER: Graham and Janice at 2021 Head2Head Walk 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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Spectrum / Summer 2022

LATEST NEWS FAQs on The Exclusion of 35 children with CF from Kaftrio

What is the current status of the pricing dispute between the HSE and VERTEX? There is still no breakthrough on this issue at time of writing (27 June 2022). It remains completely unacceptable that 35 children continue to be excluded from access to Kaftrio, particularly when expectations were raised that these children would gain access at the same time as 140 other children with CF in May 2022.

However, talks between the HSE and VERTEX continue and the matter has still not been referred to the NCPE for a full HTA, a move that would further delay access. The inequity of this dispute is further underlined by the fact that children with CF aged 12 and over with the same genetic alterations, presently get access to Kaftrio.

Why were 35 children excluded from Kaftrio?

The excluded 35 children all have a particular genotype combination that includes F508del and a minimal or unclassified altered gene. This group were not included the 2017 Orkambi-Plus Portfolio deal (as amended in 2019).

According to the HSE, ‘What we tried to achieve is that we wanted access to all of the VERTEX portfolio within the resources available to the HSE. We sought to include every drug in the portfolio deal. Vertex was unwilling to do so’ (Examiner, 22 June).

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According to VERTEX ‘Since the beginning of this year, we have been working with the HSE to update the reimbursement agreement to ensure the broadest possible access for our medicines today and in the future.

Our focus has been to provide the authorities with all the information required and we have put forward several solutions that would allow rapid access for these 35 children and other patient populations who are not explicitly currently included in the agreement, who may become eligible for our medicines in the future. This has included extending the agreement in a manner consistent with the existing terms’ (26th May 2022, Open Letter).

Vertex added in a later statement ‘The 35 patients in the 6 to 11-year-old patient population with specific genotypes were not included in the original agreement. We have offered to include these additional patients at the same cost per patient as the existing agreement’ (22 June, Statement to The Examiner newspaper).

What is CFI’s approach to advocacy in support of the 35 children?

CFI has adopted the following approach, in partnership with our members and approved by the CFI Board: •

To inform CFI members, based on the most up to date and available information that we have, but also noting that CFI is not party to these negotiations so we will never have all the detail and have to make our own assessments accordingly

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To advocate for and with those families impacted by this decision and to seek broader support from the whole CF community through a Campaign for Kaftrio working group

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To flag this issue as an urgent matter of concern that needs resolved by VERTEX and the HSE/Department of Health.

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To ensure that the general public is aware of what we contend is a human rights issue

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To seek meetings with VERTEX and the Irish Government to highlight the concerns of parents

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To underline that the responsibility for resolving this dispute lies between the Government and VERTEX

What happens next in the process?

CFI understands that at the time of writing that negotiations continue, which is a hopeful sign but then again does not necessarily determine a positive outcome.

If the outcome of the present talks is ‘no agreement’, the dispute will be referred to the National Centre For Pharmaco- Economics (NCPE), the body that decides on the effectiveness of drug therapies in Ireland. The NCPE has already undertaken a rapid review on the genotype sub set impacted by the dispute and recommended on 10 May that a full Health Technology Assessment (HTA) should be undertaken. It is now up to the HSE whether to follow this route. There are basically 3 options that can emerge from an HTA in Ireland: ‘Yes’ ‘No’ or ‘No- not at this price’. The latter outcome usually triggers more prolonged negotiations. The HTA takes 90 working days but in practice this process can be much longer.

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MEMBER SERVICES New 'Additional Needs' Payment from the Department of Social Protection On the 6th June 2022, the Department of Social Protection launched a new 'Additional Needs' payment. This payment is available if a family have expenses that cannot be paid from the weekly income. A request can be made to the Community Welfare Officer and you can find more relevant details below.

What is the Additional Needs Payment An Additional Needs Payment is a payment available to you if you have expenses that you cannot pay from your weekly income. You may get this payment even if you are not getting a social welfare payment. It also may be available to you if you are working and on a low income.

The DSP can provide Additional Needs Payments for: •

the increased cost of fuel and electricity

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essential repairs to property, including motor vehicles and replacing household appliances and furniture

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assistance with deposits for private rented accommodation

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furniture, bedding and other items if you are setting up a home for the first time

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funeral costs

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recurring travel expenses such as for hospital appointments or visiting relatives in hospital or prison

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food, clothing or shelter in the aftermath of a fire, flood or other emergency event

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other additional exceptional needs as they happen

Additional Needs Payments are paid under Supplementary Welfare Allowance and are administered by the Community Welfare Service (CWS) of the department.

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How to qualify Anyone on a social welfare payment or working and getting a low income may qualify for an Additional Needs Payment. Your weekly household income, expenses and the type of assistance you need will be taken into account.

To get an Additional Needs Payment, your application will be reviewed by a Community Welfare Officer and they will look at your circumstances before deciding the payment you can get.

Rate of payment There is no set 'rate of payment' for the Additional Needs Payment. The payment you get will depend on your weekly household income and the type of assistance you need.

Examples of applications for the Additional Needs Payment

How to apply You can fill out the form below and send it to your local Community Welfare Service or you can get the form in your local office and fill it out there. There is no need to make an appointment. The application can be submitted by post.

Find your local Community Welfare Service.

Application Form: Supplementary Welfare Allowance (SWA1) Claim Form for Supplementary Welfare Allowance/ Additional Needs Payment.

Download

What you need to provide for us to help you with your claim To help process your claim, you should have the following: •

Personal Public Service Numbers (PPSNs) for yourself, your spouse, civil partner or cohabitant and your children

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proof of identity, for example: a Public Services Card (if you have one), a passport, driving license, work permit, immigration (GNIB) card

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documents to show your income, expenses and financial situation, such as pay slips and bank statements.

If you have any questions about CFI grants or services, state benefits or require information about Cystic Fibrosis, please get in touch with our Member Services Team.

Sam Byrne sbyrne@cfireland.ie

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Rory Tallon rtallon@cfireland.ie

Caroline Heffernan cheffernan@cfireland.ie

Spectrum / Summer 2022

Travel Diary of a PWCF: Disneyland Paris Following on from the travel tips provided in the last edition of Spectrum, CFI Member Thomas Henry tells us about a recent European trip he took and what travel looks like for him as a post-transplant PWCF.

For years I had been trying to plan a trip to Disneyland Paris as I'm a huge Disney fan. I'm a big child at heart really anything Disney I just love. I had heard people talking about how expensive it was so I just kept putting off planning it myself. Last Christmas morning, as one of my surprises, my boyfriend had bought us a trip to Disneyland Paris. You can imagine the surprise I got, I was ecstatic! We went over on the 21st of March this year for 4 days and 3 nights.

Going away anywhere, I always pack a few days beforehand as there is quite a lot of medication I need to bring, even more now than before I had my transplant. I have a specific tablet box that I fill, which holds four days of my tablets at a time in morning and night time compartments. If I'm going away for a few days or a week I would fill maybe two boxes so I have them made up for me ready to take. Then I also bring a week or two supply in their blister packs and leave them in my bag.

I'm always afraid I will lose my medication so I have some in my hand luggage and some in my big case, just in case one goes missing. If it does, at least I have the other stash. As I always say 'its better to be looking at them than looking for them'. My nebuliser goes into my backpack carry on too and nebules are in a cooling bag inside my little backpack. My backpack is like Mary Poppins handbag when I'm traveling anywhere :) I normally get a letter from my GP to say that I'm carrying medication with me but nine times our of ten I never have to show it and they never search my carry on unless I have my sinus spray which is a glass bottle but usually I pop that into my big case.

We had the most amazing time in Disneyland. We were like two big kids amazed at everything over there. It was just something else and we're planning on going back over in a couple of years again. If you get the chance to go then you really should! Its not just for children but it will make you feel like a child again!

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SPOTLIGHT With an increasing number of PWCF becoming parents, some may wonder how to explain their Cystic Fibrosis to their child. But parenting generally everyone will have different perspectives, In this Spotlight, we hear from three CF parents about how they introduced their young children to their CF and hear from the children about how it was for them growing up with a parent who has CF.

Rory, Sarah, Florence (10) and Aine (8) Rory - PWCF Bed time stories – what magic family time with our two daughters. One of their favourite infant books centred on 10 little fingers and ten little toes but where do you start explaining to a three-year-old that cystic fibrosis is primarily a chronic respiratory illness genetically inherited from birth caused by a faulty gene encoding for the CFTR protein that results in chloride ion transport dysfunction? These little bundles of energy toddling around the place were still getting to grips with their ABC’s.

In truth I didn’t have a strategy other than a determination to include CF in their learning of the world around them as they experienced it with me in it. So, when they got past the ABC’s and began to ask questions about everything and anything from their preschool day to the moon and everything in between at that point we focussed on their favourite infant book they used to love about ten little fingers and ten little toes and took it from there really.

The Proof is in the Skin We explained that every child and every person is different in some way that no one is exactly the same. Some people are big, some are small, some thin, some not so thin and some people are salty and that I was their salty Daddyfollowed by very curious and scientific skin licking proof of concept versus Mammy as control. That sparked the why and how and we explained that’s just the way I was born.

Pac-Man Explanations for things they then began to notice followed over their early school years based on what they saw day to day. So, they would see me taking enzymes and taking insulin. Here we explained about the food

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Sandy Toes and Salty Kisses: Parenting with CF they eat making their body grow and needing vitamins and vegetables and fruit in their meals.

We explained that the body digests food when they eat it- breaks it down into little pieces that the body uses the build their bones and muscles while they grow big and strong and for energy to keep them running around a bit like battery power when the batteries run out in their toys -they need more energy or new batteries. The enzymes I take digest my food for me because my CF, that thing that makes me salty, also means my body doesn’t produce enzymes like their body does. My enzyme tablets are a bit like Pac-Man gobbling up my food for me in my belly. Insulin was sort of the same deal too.

"Hello my name is Áine. I'm 8 years old and my dad has CF. My Dad is a hard working man. When he goes to the hospital I miss him very much.

It is probably very hard for him to do some things like going on long walks or long runs.

So if you know anyone with CF please support them.

Thank you"

Good enough for them – Pac-Man was a well-established concept.

Nebuliser Cuddles They also enjoyed nebuliser cuddles. I explained about the mist helping me clear out sticky mucus from my lungs and this helped me to do my exercise too so I could stay strong and fast to try keep up with them for their chases around the playground.

That was the easy part. It was much harder explaining why I had to leave them and sleep over in the hospital for a few weeks one summer. We explained that I needed to go in for antibiotic medicines to help my cough. Oh and they don’t need to worry when they hear me coughing a little bit– that’s sort of normal for me but I need extra medicines if I cough a lot. We do have to worry though if we hear them coughing because its different for them if they are coughing and we would mind them and get them better if they have a cough.

On a Marmite Mission They did come to visit me in hospital, they were so very nervous but they had a very special top-secret mission (ssshh - to smuggle me in my favourite jar of marmite spread). The hospital visit resulted in loads more questions but after a good play on the snazzy remote controlled bed they left feeling a bit more assured for my sake.

Thankfully our daughters haven’t ever seen me very ill. They have seen me a bit

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miserable and sleeping on the couch when I have had a winter cold and have been coughing through the night.

They were very worried about COVID as many children were, trying to negotiate when to worry and when not to worry but we explained we would mind us all very carefully if any of us did get it, which we all eventually did.

Thanks to the vaccines the experience was mild for us all.

Sandy toes and salty kisses So that’s sort of how we have explained my CF to our daughters and continue to explain to them. As they are getting older and wiser and their knowledge base increases so too will the level of detail of our explanations for their salty Daddy.

My most cherished father’s day gift hangs close at my desk - a holiday souvenir that means so much more to us and the world to me: “Sandy Toes and Salty Kisses”.

Carol, Nigel and Abigail Carol - PWCF I always knew I wanted to be a Mam but because I have CF, myself and Nigel (my husband) decided he should go for genetic testing. But as things turned out, I was pregnant before the results came back!

People used to ask me if I was worried that the baby would have CF. Of course I was worried but I couldn’t let that over take me. I used to say ‘sure if the baby has CF, who better to be her parents than us!’

Abigail arrived a bit early and was a tough little one from the beginning. Unfortunately, I became quite ill when she was a week old and had to go to hospital, so Nigel was alone with a one-week-old premature baby who needed feeding every three hours and took an hour and a half to feed! It was tough but it was the start of a very close relationship between the two of them.

Nigel and I always took the approach that we would ‘see how we go’ so we never had a sit-down to discuss how or if we should tell Abigail that I had CF. CF was, and is, a part of our lives. It was just natural that it be a part of Abigail’s too. We weren’t going to hide it from her though I will admit I tried to protect her from elements of it by keeping her out of the clinics and hospitals. I was afraid it might frighten her. But as she got older, she started to come and get familiar with the medical environment.

And that is something we were very aware of - making sure we were giving her information that she could digest and understand. It had to be appropriate for her age. Everyone knows their own child and what they can handle, and it is important to know what to say and what not to say.

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I also read books to her about CF when she was little to help her understand and I remember teaching her how to do IVs with me too. I showed her how to do it properly, with the push/pause technique and everything and I ordered special gloves for her to wear. I made sure she was involved in every aspect of CF from a young age to take the fear factor out of it. My thinking was that I could show her that IVs are what would make me better again and make me be able to be Mammy again, they were a good thing.

Of course, when I was in hospital, we needed a hand and either my or Nigel’s Mam were there to help us out. We tried our best to keep Abigail in her usual routine, her own bed, her own space for the times when I wasn’t around, and tried to make sure those periods weren’t upsetting for her.

So, I suppose, we made it a part of life, because it is a part of our life. I never realised though how relaxed we were about medication and things like that until other children came into the house. I’d suddenly realise ‘Oh goodness, there are syringes over there’ or something.

Abigail was just so used to it, I’d forget sometimes that other children weren’t!

And I think all those things are why she is so good with it. Last year, I collapsed in the sitting room. Nigel was at work so Abigail had to take up the reins. She rang my Mam and asked what medicine she could give me.

Then she rang our neighbour who came down and rang the ambulance. Abigail was brilliant. She didn’t panic, she stayed strong and wasn’t afraid.

I know other families will do it differently but I suppose that showed me that for us, it was the right way to approach it.

We are a very tight family and when the chips were down, you really gel. You have to pull together and be there for each other and get each other through the rough patch to come out the other side. In a family, everyone is in it together so to other parents with CF out there, I would say involve your children as much as you can. For us, it took the fear out of CF and made us closer in the process.

Abigail My name is Abigail McGrath and I am 13 years old. My Mam is Carol and she has CF. I always knew Mam had CF. I just grew up with it - it was just another part of our life.

When I was little I remember Mam reading stories to me. We read all sorts of stories but Mam read books about CF to me too. I can’t remember the name of them now – I think they were written by a girl who had CF - but I remember them well.

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I grew up knowing that Mam would be sick sometimes and would need to do IVs or go to hospital. I went for some check-ups with Mam but not many - mostly I stayed home. But if Mam was in hospital for a long time, I’d go in and visit.

Actually, it was kind of fun when she was in hospital, because my Nanny would come and help me and Dad out and I loved that. I have two Nannies and one doesn’t drive, so when that Nanny came, it was even more exciting.

I remember one time Mam was in hospital for a few weeks. I was in first class and it was my turn to bring Fergal the Frog home from school. We had to take turns to bring home this frog teddy and bring him out and take photos or write in a copy what we had done with him. Because we were visiting Mam, I took him to the hospital. He got a wristband and everything! It was so funny! And the next day when I brought him back to school, sure no one had a story like mine to tell.

But when Mam was at home, I always wanted to help her. She taught me how to do IVs properly when I was little so I could help. I remember practicing with the needles – I had to inject an orange! I loved when Mam had to do her IVs because I loved helping her. I used to get out my gloves and they’d be hanging off me, even though they were the smallest size Mam could get, and me and Mam would have fun doing them.

CF being part of life meant I was never scared of it. Mam and Dad had taught me what I needed to do to help Mam or if an emergency happened and so last year, when Mam collapsed in the sitting room and I was at home, I knew what to do. I didn’t panic. I wanted to ring an ambulance and kept saying that to Mam but in the end, I rang my Nanny to find out what to give Mam, and our neighbour rang the ambulance for us.

The hardest thing that day was waiting for the ambulance because it took so long to get to us. I was on the phone to the CF nurse giving out about it – Mam needed more help than what I could give and I was cross that the ambulance wasn’t there. My Dad even got back from work quicker than the ambulance. So yeah, I was worried, but not scared.

That summer Mam was in hospital and everyone jumped in to help. I was at Pony camp and had loads of activities to keep me busy. Mam says I handled things around then ‘like a boss’ that day and I think that was because CF wasn’t anything different to me. Mam and Dad always taught me about it and so even when you’d see medicines around the house or syringes or something, I wasn’t interested in them. Other little kids might think they were sweets and that kind of thing, but even though I was little, I knew not to touch them.

The only time I did was to bring them to Mam! So, I think the way Mam and Dad involved me

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made me curious, not afraid. I was definitely curious - always asking the nurses questions and even when Mam was in ICU that time, it was the same. I asked lots of questions because this is my life. And that’s what CF is to me - just an everyday part of life.

Caroline, Fran, Jamie and Anna Caroline - PWCF Deciding to have children is a big step for all couples but when you have CF it makes the decision a little more complicated. Many questions had to be asked.

Will I have difficulty getting pregnant?

Is my health good enough for a pregnancy?

What impact will a pregnancy take on my health?

Will I be able to continue taking my regular medications when pregnant, includes antibiotics?

Is it fair bringing a child into the world when I potential have a shorter life expectancy?

The list is endless, so what do you do?

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In 1997 I went with my gut instinct and decided the time was right and hoped to be successful. To answer the question, did I have trouble getting pregnant? no, it happened within two months so we didn’t get much time to reconsider with Jamie arriving in May 1998.

For my second child Anna, the doctors were putting a push on me to decide if I was going to have another child as I was now in my 30’s (how ancient, but in fairness for a PWCF at the beginning of this century it probably was).

I never actually sat down with the kids and said “Mammy has CF”, it happened more organically. My treatments were always done in front of the kids, beacuse if I was at home alone I had to supervise while doing treatments.

I was actually on the treatment table getting a long line in my arm and breastfeeding Anna when she was only 2 weeks old (taking multitasking to a new level). It became normal routine life, they knew no different. At times when I refused to get up until treatments were complete we had a few disagreements and the odd tantrum but they soon realised Mam's neb time wasn’t to be interrupted. It's exactly the same with Milo (my grandson) now.

As they got older I included them in my treatments, they would get meds from the fridge and fill my nebuliser for me, recycle the plastic vials, put my IV’s in the fridge when they arrived from Baxter etc.

I was always very honest about CF (in an age appropriate way), however over the years they knew I attended funerals of friends so we would have a chat about Mam doing her treatments and keeping as fit as possible, doing everything in my power to stay well, to increase my chances of being alive for as long as possible. But I do think you have to balance the conversation and appreciate that with life comes death and we can only do our best with what we are dealt. No one knows what life will hold so we can’t live our lives being afraid that the end is near.

For me going into hospital broke my heart for many reasons; being away from the kids is always hard (even now), but it also meant Fran had to be a single parent for that period of time, trying to work, mind the kids, worry about me, come to visit etc.

We are lucky to have a very supportive family but I know these times were particularly hard for everyone; Fran, Jamie and Anna,

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my parents, in-laws. You have to remember my kids were young when medications weren’t as good as they are now, so with each exacerbation the thoughts of life expectancy crept into the heads of the adults.

By living with CF as a family I hope I have taught my kids, that even if you don’t have an equal start in life, with a little extra effort, hard work and determination you can come someway to achieving your dreams. It's not about winning the race of life but travelling the route of your choosing and enjoying the ride even if it is a little like being on a roller coaster.

On the 1st of June Fran and I celebrated 26 years of marriage with a further nine years to boot, OMG 35 years!

I have surpassed all life expectancy predictions, we have two amazing children and the dream that I never let myself dream, a grandson, Milo. I’m pretty much floating on air.

Anna and Jamie When mam asked us both to write a paragraph on being a child of a person with CF, we both looked at each other and said, sure we don’t know anything different. Living with CF is our normal family life.

As we grew up we never felt the need to look up or Google CF as I think we have always known about it, it is our norm. Whenever we had a question, we would just ask and Mam would tell us what we needed to know.

Thinking back though the one thing we both hated was Mam's neb time, but not necessarily for the reason you’d think. It was the noise of her old porta neb, the buzzing drove us mad as we watched TV in the evening with no escape to another room to watch our programs. Now with the Eflow we don’t even realise she is doing her nebs, and thankfully we don’t all have to watch the same TV either!

Now Jamie and I watch on as Milo squeezes the liquid into Mams neb just like we did. When Milo wants Mam to get up and play, we jump in and tell him 'Nanny can’t get up as she is doing her nebs', and at times we have to distract him just like Mam and Dad had to with us.

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On the occasions when we visited Mam in hospital, the children’s ward in Castlebar was always a great place to visit. Sometimes we were mistaken as the patients as Mam and the other CF adults were the only adult patients on the ward. Hospital visits always meant treat time after so Dad could bring us home without too much of a fight!

Mam didn’t have a lot of hospitalisation that we remember as she mostly does home IV’s. We could tell when it is time for IV’s as a couple of weeks before hand you might find her asleep on the couch in the afternoon and, dare I say it, was usually the only time she could be a little ratty or sharp with us as she knew a set of IVs’ were coming and it obviously annoys her.

Mam has a very distinct voice which can be husky/hoarse when she isn’t feeling the best. I think it must be a trait as her CF friends sound similar. Mam doesn’t cough as much anymore since starting the Kalydeco 6 years ago, which really does mean there isn’t a daily constant reminder of CF. When Mam started going to Beaumont more often then Castlebar we were worried and more concerned and thought it seemed more of a big deal. When Mam explained that Beaumont was just the same as Castlebar in regards to appointments we relaxed about these appointments. Trips to Castlebar, were our norm so Beaumont was a little bit of a curve ball.

We do worry that Mam sometimes over does it, especially when she takes on big events. Having said that we are very proud of her commitment and completion of everything she does. She doesn’t take on events lightly and makes sure she is properly prepared for every event no matter what she is doing.

CF isn’t an easy life but Mam always tries to be the optimist in life, living life to her fullest.

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CFI

Team

Update

Welcome back to Liz Jacques, who has returned to CFI as Mid-West Regional Development Office/TLC4CF based in our Limerick Office. Liz went on maternity leave in August 2021 and has been taking care of baby Finn since his arrival. soon after. He is not a newborn anymore and Liz cannot believe how fast the year has flown by!

Congratulations again Liz, we are delighted to welcome you back.

Nicola joined CFI as Public Awareness and Social Media Officer in January 2022. For eight years Nicola was self-employed, partnering with charitable, not-for-profit, public and private sector organisations to work with children with and without additional needs. Following her experience as a volunteer PRO for a local GAA club, Nicola decided to change careers, and graduated from DCU’s Masters course in Public Relations and Strategic Communications in 2021. Aside from work Nicola is Mammy to three energetic young children and enjoys reading and cycling.

Miriam began working with CF Ireland in February 2022. Miriam is a part of the Fundraising team and is the lead coordinator for Malin2Mizen, Cycle4CF, 1in1000 and The Head2Head Walk. Previous to this role, Miriam worked in Philantrophy Ireland as Communications Officer. A graduate from UCD's MA in Arts Management & Cultural Policy and TU's BA in Contemporary Art & Critical Theory, Miriam's background is a combination of cultural, as well as not-for-profit institutions. Outside work, Miriam enjoys discovering new cooking recipes and horror movies.

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Teen Titans ‘Keep It Up’ Ava ‘Keep It Up’ is a recent RTE show which brought nine teenage girls together who either gave up or had never played sport. The girls were from all different backgrounds and experiences and formed a basketball team. The programme followed them as they re-engaged with sport with the help of their coach, Emer O’Neill.

One of the nine girls is Ava Li Blount, a PWCF and CFI Member from Wicklow. CFI caught up with Ava to find out all about her experience....

How did you end up on the show?

I am part of an all-girl running club called Go

Girls in

Wicklow. My running coach suggested I put myself forward for it - that would be a good fit.

First, I sent in an introductory video explaining my story. I told them about my experience with exercise growing up, how I felt I could be judged because I wouldn’t have been at the same levels of others because of the CF. After that, I had a follow up call with the producer who had even more questions for me, and then I got the call telling me that they thought I’d be a perfect fit too.

We filmed for two full weeks last summer (2021) but I didn’t tell any friends at the time. We had to keep it to ourselves and any photos we had from it had to be kept secret till the trailer officially launched. When it did, we covered our social media accounts with it!! It aired recently and everyone knew then!

Why did you go on the show?

Growing up with CF meant I had to work harder than others physically.

When I lived in Dublin, I didn’t really do sports – I more just went for walks. I used to get red in the face quicker than all the others because

my body had to work much harder and I would cough and cough and cough.

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Spectrum / Summer 2022

When I hit my tween years, I began to get a bit embarrassed about that and it put me off sport a little.

That’s why I joined the girls running club – to be more active but in a comfortable environment. I’m over that insecurity now and the show just seemed to fit my experiences and I also had a story to tell. And it was an amazing opportunity and experience.

What were your team like?

The group were amazing - euphoric even!

We were from all different backgrounds and all had different reasons why we weren’t really into sport but one thing we had in common was that our experiences with sport had hurt each of us and left us a little insecure about playing sport.

was

On day one, we were all finding our feet. We went in strangers but we were all on the same level. We didn’t have to worry about being or looking perfect, we were just playing sport. From the start, we were all sweaty and not worrying about how we looked. Sport our connection and by the end of the first day, we were friends.

We were together all day every day for 2 weeks and like any team, we got on each other’s nerves at times but we always overcame any issues.

How did it feel to tell the team about your CF and have it televised?

CF Is part of me. It makes me who I am. I am definitely not ashamed of it because

without it I wouldn’t be Ava. I’m Ava who loves drama, Ava who loves English, but also Ava who has CF. So telling people depends really.

Sometimes I don’t hold back and other times I feel I don’t need to tell people about it at all. My close friends know but not everyone needs to.

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The main thing is that I’m

not treated any

differently if someone does know. And the girls on the team were amazing. They were so supportive and we had such a good chat about it. Actually, I said so much more to them during filming than what was aired on the programme! That was just a tiny part of it. And they definitely didn’t treat me any differently after they found out.

What was it like to see yourself on telly?

It was so weird. It got worse before it got better! When I was watching the first episode, I didn’t really believe it. Then on the second episode, I started saying ‘oh God’, then questioning myself asking ‘do I really sound like that?’ and that sort of thing. But by the end of the show, it was just really cool.

What was the experience like?

It was a magic experience.

It was so good. We became stronger, faster and had more stamina.

I’m on Kaftrio for a year now and I’ve been doing really well on it, but to go out and train with a team like this was incredible.

At the end of the series, the coaches had organised a match with another team for us. We played in the National Basketball Arena and it was a perfect way to finish things off. We didn’t win but that didn’t matter.

We put our absolute best effort in. We were so happy to show off our new skills and showcase our abilities. Our families came along to support us and everything. I definitely went into the camp a bit insecure and came out so much more confident.

What’s next for Ava? Well, I’m still training with Go Girls and am also doing a bit of coaching with some of the girls at the club. I help pace them and encourage them in their training.

Myself and the basketball team are still in touch too. We have a WhatsApp group and have met up a few times since. But my Junior Cert is just around the corner so I have to focus on that for now! But maybe afterwards, I’ll go out with the 'Keep It Up' girls to celebrate the programme and our results!

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Spectrum / Summer 2022

Moving to Secondary School with CF? Not a problem for Darcy!

Hi, my name is Darcy.

I’m 14 and I’m in second year. I didn’t get to properly finish 6th class as Covid-19 had just broken out. I had to live out the rest of the year on Zoom. When I started secondary school it was a completely different environment than primary. With the masks in school it was very hard to make new friends.

As a student with Cystic Fibrosis the masks did benefit me by preventing me from getting ill. The school was aware of my CF as my Mam had a meeting with my form teacher and the home school liaison, so my form teacher would always look out for me.

With other teachers I only told them about my illness if I had to or if they asked, otherwise it's none of their business.

I am extremely lucky to have a close friend of mine in the same secondary school as we went to primary together.

She will always help me with anything like taking my Creon or telling others about CF. It's always good to have your close friends educated about Cystic Fibrosis. Having people in your corner to support you anytime is important.

The first little while in secondary school is scary but everyone settles in eventually. I've had some of the best times of my life in secondary school already and I'm only in my second year!

Advice I would give to people with CF moving to secondary school is not to be afraid of missing days with your illness and not to be self conscious about taking tablets in front of classmates. If people ask about it all you have to do is give them a simple explanation. Have a good time and don’t care what people think!

Darcy Grainger Have you a STORY, an EXPERIENCE or an ACHIEVEMENT you'd like to share with the community?

Spectrum contributions are always welcome from our members and we'd love to include your piece in the next edition. Email Nicola on ndelaneyfoxe@cfireland.ie with your story and a photo or two and we can take it from there.

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Redmonds of Roscrea "Happy Days" Annual Portrait Fundraiser 2022 Every year, for more than 20 years, Redmonds of Roscrea - The Photographers have held their annual “Happy Days” portrait charity fundraiser and 2022 was no different. Thanks to the support and generosity of their fantastic clients, they were able to raise €3470 with 100% of all proceeds going directly to TLC4CF. To enable this, Redmonds gave their professional time and materials through a complimentary photo shoot and a beautifully finished 7’’ portrait for everyone who donated to this event and worthy cause.

Niamh Sheedy from TLC4CF (pictured) was presented with a cheque by Brian Redmond in their studio in Castle Street. Ms. Sheedy told Redmonds that the proceeds raised from this event will be used to benefit a variety of upcoming activities and needs such as research projects, staffing, clinical trials, nurses, facilities and equipment.

TLC4CF was formed in 2008, with the aim of improving hospital and treatment services, facilities and supports for people with Cystic Fibrosis (CF) in the mid-west region of Ireland. TLC4CF is a not-for-profit charity and an affiliated regional branch of Cystic Fibrosis Ireland, covering Tipperary, Limerick and Clare.

Redmonds have shared just some of the hundreds of images that were captured as part of this annual fundraising event. It goes without saying, “Whatever the weather, Happy Days are here again!”

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It’s Panto Time! Oh, no it isn’t. Oh, yes it is!

It might not actually be Panto time, but it felt like Christmas at TLC4CF when we found out that we were selected as this year's charity recepient for.... (drum roll please)..... the Limerick Panto Society! (Oh no you haven't, oh yes we have!)

The Limerick Panto Society was formed in 1985 and since then they have been keen supporters of many local children’s charities. TLC4CF are delighted to be the charity receipient for 2022 and our very own Niamh Sheedy, Regional Development Officer - CFI/TLC4CF, was only too happy to join Mayor of Limerick, Daniel Butler, and President of TUS, Vincent Cunnane to launch what we know will be a season to remember.

The Panto Society have donated tickets to TLC4CF who can use them in a fundraising capacity. To top that off, on one night of the season all profits will go directly to TLC4CF. The tickets will be for this years production of Cinderella which will be held in The Millennium Theatre, TUS from Dec 27th to January 8th. For more information visit https://limerickpanto.squarespace.com/ or for information on how to support us, contact TLC4CF on tlc4cf@cfireland.ie!

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CFRI Report 2020 The 2020 CF Registry Report reports on continued progress in CF survival ages but also impact of COVID evident in outpatient interactions and CF transplant programme.

The Cystic Fibrosis Registry of Ireland (CFRI) has recently published its latest annual report for 2020. A summary infographic provides an overview of some key information. The full report is available at www.cfri.ie.

The Chair of CFRI is Professor Edward McKone of SVUH and the CEO is Godfrey Fletcher, a former CEO of CFI. This year marks the 20th anniversary of CFRI. The CRFI was established by CFI and used to be based in CF House in Rathmines before becoming an independent entity based in UCD in line with best international practice.

Some of the highlights we've picked out in the CFRI Annual Report are as follows: •

The median predicted age of survival for a person born with CF has increased from 38 years in 2010 to a remarkable 51 years of age for those born 2020;

•

12.5% of the CF population is now over 40 years of age;

•

The 2020 CFRI report, not prone to exaggerated language, reports ‘CFTR Modulators have revolutionised the treatment of Cystic Fibrosis’ (p27);

•

These include the drug therapies Kalydeco (2013-present), Orkambi (2017 – present), Symkevi (2019 – present) and Kaftrio (2020 – present);

•

68% of the CF population, more than two thirds, were on a CFTR Modulator in 2020;

•

COVID-19 had a significant impact on outpatient CF care (and likely on in patient care too- CFI);

•

There was a significant decrease in in-patient treatment in 2020 compared with the previous year and 17% of all outpatient treatment in 2020 was virtual (Zoom, phone etc).

There was only one CF double lung transplant in 2020 again, at least in part due to COVID 19 (see also reports of the Irish Donor Network on the news section of this website that reported a 68% deduction in all lung transplants 2019 to 2020).

For the full report visit www.cfri.ie or contact the CFI office for a hard copy.

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2020 CF Registry Data Highlights CFRI particpants

Age%distribution of total CFRI population

1256 participants in CFRI in 2020

50

47.9%

Female 42%

60

30

Male 58%

51 yrs

40

12.5%

39.6%

40

Median survival age

20

38.3 yrs 20

10

3.3%

0 Children <18

Genotype

2016-2020

*shown as a % of total CFRI population

Heterozygous F508del

Overall

Non-F508del/ Gating

55.4%

2010-2014

Adults >40

CFTR Modulator therapy*

F508del/F508del 36.4%

0 Adults <=40

68%

20%

0

25

% eligible & taking CFTRm

Not F508del

12%

4.9%

50

% eligible & not taking CFTRm

75

100

% ineligible

N.B. Patients may be eligible for more than 1 CFTRm. Patients may have switched modulators over the course of the year.

Key stats

Adults (18+)

Children

Demographics (number of participants)

759

497

Median best ppFEV₁

66.3%

91.5%

Complications (top 3 most prevalent)

Reflux 55.1% Sinus 40.2% Diabetes 30.3%

Reflux 25.4% Sinus 6.2% Cirrhosis 5.0%

Microbiology (% chronic status)

P. aeruginosa 31.2% S. aureus 24.3%

P. aeruginosa 7.9% S. aureus 37.0%

BMI (ᵃZ-Score)

23.2

+353 1 716 3177 info@cfri.ie

www.cfireland.ie

0.41ᵃ

www.cfri.ie

Encounters 6907 encounters recorded in 2020

Other 15.7%

Unknown 0.3%

Outpatient day 36.5%

Virtual encounter 17%

Annual assessment 7.1%

CF day unit 23.5%

@CFRegistryIE

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The CFRI Who we are & what do we do?

The Cystic Fibrosis Registry of Ireland (CFRI) is the patient registry for those living with Cystic Fibrosis in Ireland.

A patient registry is a secure, centralised database, containing health data on people with a specific diagnosis or condition.

The role of CFRI is to collect and record information on the health status of people with cystic fibrosis (CF) who agree to participate. By collecting and analysing information on all people with CF in Ireland, the Registry can better understand their health and wellbeing, and the treatments they receive. We analyse this data for different purposes, all with the wider aims of strengthening Cystic Fibrosis research, improving CF care, and ultimately of improving the health outcomes for those living with CF.

What have we been up to in 2022 so far?

New staff – It’s an exciting time for CFRI. In January 2022, CFRI welcomed 3 new full-time staff to the team: Robyn Doherty (Head of Operations), Paul O’Regan (Research Analyst), and Soumya Joshy (Clinical Research Associate). We also said good-bye to Huw Rees, a Research Associate with us, who has made the exciting move to Australia. Thanks, Huw, for all your hard work over the last couple of years – we’ll miss you!

2020 Annual Report We published our annual report on 2020 data. This report summarises the data collected in the year 2020. Take a look at our report and accompanying infographic here: https://cfri.ie/annual-reports/.

Reconsenting Due to changes in GDPR, CFRI need to ask you to re-confirm your consent for the registry to collect your information. If you have not re-confirmed your consent, please contact your CF Centre or the Registry.

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ECFS We attended the European Cystic Fibrosis Society Conference and presented a poster on the impact of the COVID-19 pandemic on health outcomes for people with Cystic Fibrosis using CFRI data. We enjoyed connecting with colleagues from all over the world, and hope to build more collaborations and projects to improve care and health outcomes of those living with CF in the near future.

20 years of CFRI Did you know that 2022 marks 20 years since the publication of our first ever report in 2002? We are marking 20 years with a special edition of our annual report and an event towards the end of the year. Keep an eye out for forthcoming publications and events to celebrate 20 years!

Research projects We continue to be involved in lots of research projects relating to CF including the Recover (Real World Clinical Outcomes with Novel Modifier Therapy Combinations in Children with CF) and ICOS (Irish Comparative Outcome Study of CF – Evaluation of the clinical, psychological and economic effects of the Cystic Fibrosis New-born Screening Programme) projects amongst others. CFRI data is used in this research and our research team provide specialist statistical advice and support to the wider research teams.

Want to get involved?

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

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

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

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Conference Review ECFS Conference 2022 The 45th European Cystic Fibrosis Conference was held in person for the first time in three years in Rotterdam, the Netherlands. Philip Watt, Samantha Byrne, Caroline Heffernan and Sarah Tecklenborg participated from CFI. This is the most important CF medical and scientific conference in Europe and provides an important opportunity to learn about new developments, to see the international interest in some of the research projects we support and to network with colleagues worldwide.

In this exciting time of change for CF care, treatments and therapies, there is still lots to be achieved. The conference provided an opportunity to network, collaborate and inspire future research. The perspectives of patients, clinicians and scientists were brought together to discuss what a positive response to modulator therapy looks like.

Dr Audrey Chansard, patient advocate, researcher and person living with CF, presented a graph of her FEV1 values throughout her life and discussed the peaks and troughs in terms of the life events they corresponded to. With troughs in lung function occurring during her rebellious teenage years where she refused to attend a CF clinic and during a breakup with a boyfriend. One peak represented an exciting year living in Sweden where she was introduced to inhaled hypertonic saline for the first time. “To you this is my FEV1, to me this is my life” Audrey shared her experiences of being on Kaftrio since September 2021 and her total shock at the response, her cough stopped overnight, the burning in her lungs disappeared and her inflammation reduced. She can exercise much more easily, hasn’t needed IV antibiotics and has noticed the change in the taste of her skin.

Life on Kaftrio has been an adjustment, with less salt in her skin to stave off bacteria she has noticed a change

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Spectrum / Summer 2022

in body odour and she now has to buy antiperspirant. She sleeps 6 hours instead of 16 and has trouble managing so much energy. Audrey finds herself irritated if she can’t release it. Her weight and body image is a very sensitive subject. The weight gain needs to be constantly controlled, a topic which was highlighted throughout the conference. Audrey described how any stress was an excuse for her to go back to the hypercaloric diet she was used to. Contemplating her new future Audrey reflected on how unprepared she is for this and has to now happily consider retirement and pensions.

Poignantly, as several hundred of us enjoyed being able to sit together again for her talk, Audrey ended her presentation with a photo of herself standing with friends with CF, all masked posing for the camera “For me a cure found will mean the day we can take our masks off together and see each other’s smiles.”

Professor Barry Plant described what a positive response to CFTR modulators meant from a clinician’s perspective. Increasing lung function is a positive response to CFTR modulation, however this increase may not last forever. Prof Plant discussed his clinic’s experiences in the past where post-modulation patients were less likely to adhere to inhaled antibiotic therapies which may lead to increased risk of exacerbations.

Increasing BMI is also considered a positive response for everyone with CF on a modulator, but does it continue to go up and up and up? Prof Plant reiterated the need for weight management strategies to limit any potential cardiovascular risks from overweight and obesity. Clinicians were seeing an increase in PwCF who are overweight after starting modulator therapy.

The changing behaviours and expectations of patients could also be considered a positive response, Prof Plant highlighted the increasing numbers of pregnancies among PwCF and the increasing expectation of becoming a parent. He also noted that there is still only moderate data on the safety of pregnancy in CF with less than 100 cases in published literature.

The era of modulators has brought great excitement and hope for people with CF though Prof Plant noted that while clinicians are excited for the benefits they are also measured in their response. There is still a question around how to dose individual patients or how to adjust the dose safely if required. Lower dose triple therapy may be more beneficial than full dose dual therapy.

Prof Plant commented on issues with access to modulator therapies and how this can create disparities between different cohorts of people with CF. A clinician’s perspective is influenced by their past experience, assumptions and expectations, character traits and time. Prof Plant described how clinicians needed “to look back to move forward”

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27

with lessons learned from treating patients with mono and dual therapies now shaping how clinicians see triple therapy.

Our understanding of the impact of the modulator therapies on CF disease outside of the lungs is increasing.

The RECOVER study, led by Prof Paul McNally, utilised an abdominal symptom scoring tool developed

by Prof Jochen Mainz and team, to assess the impact of Elexacaftor-Tezacaftor-Ivacaftor, on abdominal symptoms in CF. This symptom tool is the first of its kind developed and validated in people with CF to assess the abdominal symptoms associated with CF. Unlike other studies which have used abdominal symptom tools not specific to CF, the RECOVER team presented data which shows comprehensive improvements in gastrointestinal symptoms after 6 months on Kaftrio.

Adherence to medication and therapies During a lunchtime symposium at ECFS, healthcare professionals voted medication adherence to be the biggest challenge facing them for managing CF patients in the next 5 years. Prof Alexandra Quittner, Senior Consultant Behavioural Health Systems Research, US, highlighted a multi-component self-management intervention to improve treatment adherence and reduce IV-treated pulmonary exacerbations in people with CF. The intervention involves a problem-solving interaction between the healthcare provider and person with CF where the group jointly define the issue with adherence, brainstorm solutions, the person with CF can vote on their favourite solution or the one most likely to work in their situation, and the group develop an implementation plan to solve the adherence issue. This patient-centred approach encourages independent living, grounded in the person’s values and preferences, while sustaining daily CF care.

The heavy treatment burden associated with daily CF care is linked to depression. Depression in turn impacts negatively on adherence. Dr Gwyneth Davies, UK, discussed studies which are aimed at simplifying CF treatments after Kaftrio. How to reduce the treatment burden associated with CF has been identified as a top research question for the CF community. Daily nebulised treatments, often taking up to two hours a day, are considered burdensome.

There are ground breaking trials underway to find out if people taking Kaftrio can safely start to reduce the number of treatments they have to manage as part of their daily healthcare routine. Two randomised studies, CF STORM (UK) and SIMPLIFY (US), aim to assess if stopping certain daily mucoactive nebulisers is safe for people taking Kaftrio. The studies will have patients actively disengage from their daily nebulised mucoactive therapies (inhaled hypertonic saline and/or dornase alfa) and assess lung function over time.

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Spectrum / Summer 2022

If people with CF choose to give up their nebulised therapies without trials like STORM and SIMPLIFY, there would be no quantified evidence for clinicians to refer to and confidently advise their patients.

Person-centred approaches to care As modulator therapies bring improvements in the health, symptom burden and experiences of people living with CF, questions are being asked by clinicians of what CF care now looks like in the era of modulator therapy.

The importance of the personal preferences PwCF when it comes to their CF care was a strong theme throughout this year’s ECFS conference. In her talk discussing the projections for the future of CF care for people who respond to modulator therapy, Professor Jane Davies (UK) spoke about how routine care for PwCF may change as people prioritise convenience and quality of life, opting perhaps for more remote monitoring as opposed to regular clinic attendance.

The important role health psychologists play in CF care was evident throughout the conference with speakers sharing their expertise in behavioural science, adherence, measuring quality of life, development of interventions and psychological support. The role of psychologists in the multidisciplinary team is changing from rescue therapy to supporting independence. Adopting person-centred approaches to care may improve adherence, quality of life, independence and agency, these factors working together can improve outcomes for all people living with CF

Ageing and CF Improving standards of care and the development of novel therapeutics mean people with CF are living longer than ever before. As the CF population ages, the comorbidities associated with CF will also change.

Ageing CF cohorts have more obesity, high blood pressure, CF-related diabetes and chronic kidney disease than those who are younger.

During a symposium on Ageing in CF, Professor Ruth Keogh, London School of Hygiene and Tropical Medicine, discussed the evolving age profile of people living with CF. In one analysis, the number of F508del homozygous adults with CF aged over 40 in the UK is expected to more than double by 2030.

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29

The updated median predicted survival age of PwCF based on the UK registry report data is now age 51.

These calculations assume that current age-specific mortality rates will hold, they don’t take into account any potential improvements in treatment or care which could likely improve this further.

As the demographics of the CF populations change, a panel discussed whether there may be a need to reconfigure the CF multidisciplinary teams in term of lead clinicians, will the CF pulmonologists be the lead member or may there be a need for a cardiologist, or renal lead, or could care move to the primary care physician?

Professor Liam Plant, Cork University Hospital, made the case for the importance of a Renal expert as part of the multidisciplinary team. Increased longevity in people living with CF means that more people are surviving long enough to develop kidney complications. People living with CF have a 200-fold higher risk of having an acute kidney injury compared to the general population. Prof Plant highlighted the importance of acting early on mild renal impairment to prevent future issues.

The scope of CF-related renal disease is broad and includes Acute Kidney Injury, progressive chronic kidney disease, Haemodialysis/Peritoneal dialysis, Electrolyte and Acid/Base problems, other Glomerular disease, Diabetic kidney disease, Kidney stones and Renal transplant. An increased awareness of the risks of renal disease, combined with prompt monitoring and treatment of renal issues by all CF teams may help to improve outcomes. The impact of the triple modulator therapy on renal disease is still unknown.

Obesity and Cardiovascular disease in CF Gaining weight has a protective effect on respiratory outcomes among PwCF up to a point. Beyond this, weight gain increases the risk of comorbidities including cardiovascular disease. Cardiovascular disease risk among PwCF is linked to a number of factors, some specific to CF disease and some related to increasing overweight/obesity. Age, CFTR function in cardiovascular cells, hypoxemia, CF-related diabetes and lung transplantation are all risk factors for cardiovascular disease linked to CF. Systemic inflammation, systemic high blood pressure, Type 2 diabetes and artherosclerosis are all overweight/obesity dependent factors. For those who can take them, the CFTR modulators may play a role in limiting the risk factors for cardiovascular disease which are not related to overweight or obesity. Improvements in the standards of care for CF may limit the risk factors of cardiovascular disease which are related to overweight or obesity. In his talk on obesity and CF, Prof Francesco Blasi, Italy, discussed the need for clinical teams to rethink their current standards of care in terms of nutrition and salt supply and to promote better lifestyle. Defining the management of cardiovascular risk factors in CF is an important starting point and improving cardiovascular monitoring from an early age may improve outcomes. In people with CF, the development of artherosclerosis, a build-up of fatty plaque inside their arteries, may be accelerated by disease-specific risk factors and years of increased chronic inflammation.

Early interventions and primary prevention strategies during childhood and adolescence, may help to reduce this risk.

As modulator therapies bring improvements in the health, symptom burden and experiences of people living with CF, questions are being asked by clinicians of what CF care now looks like in the era of modulator therapy.

Other sessions in the conference discussed the importance of exercise and physical activity, advances in gene and cell therapies for CF, alternative therapeutic approaches to CF and the psychology of CFTR modulator therapies.

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Spectrum / Summer 2022

The Conference also provided an important opportunity to network with patient group colleagues in CF Europe and worldwide. In this context it has been very useful to benchmark our progress compared with other countries, an issue we will be returning to in future articles. One of the key issues discussed was the impact of the war in Ukraine and there was very moving testimony form the Ukrainian CF association and thanks for all the support including that provided by Ireland.

CFI Conference 2022 The 2022 Cystic Fibrosis Ireland Annual Conference took place virtually over two nights, March 30th and 31st 2022.

The theme for the 2022 Conference was ‘Ambitions for Continuing Progress in Cystic Fibrosis Care in Ireland’.

Our Chairman, Keith McCabe, opened the conference and Philip Watt, CEO CFI, gave the opening address. The keynotes speaker was someone you will all know, Professor Barry Plant and we were also joined by medical and research professionals Dr. Patrick Harrison, Claire Reilly, SVUH Senior Physiotherapist and Professor Kors van Der Ent. Combined, each presentation gave attendees an insight into how physiotherapy and innovative drug therapies have turned CF into a more manageable long term disease but this was balanced by a thought provoking session on how inhaled therapies should not be forgotten and still play a pivotal role in CF management.

For the first time ever, attendees were treated to a line up including no less than six PWCF joining live. Rory Tallon, Caroline Heffernan, Brian McCarroll, Chris Coburn, Chlionadh Whelan and Patricia Duffy-Barber all joined the conference for panel discussions about Kaftrio, exercise and endurance, genetics and the HIT CF Project.

However, we also welcomed recorded testimonials from many more PWCF, making for very interesting, real and lived perspectives.

From our feedback survey, we were able to determine that the majority of respondents found the conference showcased excellent guest speakers and the information provided was extremely useful. Several respondants expressed an interest in webinar topics such , ‘Ageing’ ‘Mental Health and Wellbeing’ and ‘Microbiology and Infection’. If you are interested in these webinars being hosted, please let us know at info@cfireland.ie.

CFI were delighted that so many of you registered and attended. But if you weren’t able to join on either night, you have not missed out. The recordings are available on our website: www.cfireland.ie.

www.cfireland.ie

31

HOSPITAL HUB Take a look at the development timeline of the CF exercise hub in CHI Crumlin, part funded

by CF Ireland. It began in November 2021 and is already nearing completion.

November 2021

December 2021

January 2022

February 2022

March 2022

April 2022

April 2022

May 2022

May 2022

June 2022

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

September 4th: 10th Annual Head2Head Walk On Sunday, 4th September, our 10th Annual Head2Head Walk takes place from Howth Head to Bray Head. The walk is going from strength to strength, thanks to the wonderful organisation by the Head2Head Walk Committee led by Mary McCarroll, Jem & Lorraine Downes and Glen McDonnell.

The 10th Annual Head2Head Walk will be very special and you can avail of our Early Bird discount by REGISTERING NOW at www.cfireland.ie before July 31st.

We also have a limited edition 10th Annual Head2Head shirts available for the first 400 people that register. So don’t delay, register now to avail of the early bird discount to secure one of the 400 limited edition shirts! We are really looking forward to meeting everyone in Howth on Sunday, 4th September!

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

September 9th to 15th: Paris2Nice Cycle Registration is now closed for the Paris2Nice Cycle 2022. Good luck to all our participants in the event and we look forward to getting some really nice photos from the cycle.

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33

You can now register your interest in participating in the Paris2Nice Cycle in 2023 on our website – www.cfireland.ie. For further details, call us on 01 4962433 or email fundraising@cfireland.ie.

September 18th to 25th: International Walk Our International Walk in memory of Jerry Weldon for 2022, takes place in the beautiful island Madeira.

This autonomous region of Portugal never fails to disappoint its visitor with the friendliness and outstanding hospitality of the locals, unique traditions and customs, stunning landscapes, great cuisine or the richness of its history and vegetation due to the being a major layover point during the era of discoveries.

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.

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October 2nd: TCS London Marathon There are a very limited number of places still available for the TCS London Marathon 2022 which takes place on Sunday, 2nd October.

Good luck to all our participants in the event and we are looking forward to seeing some great action photos from the day.

To secure one of the final places available, contact us or register your interest now on our website – www.cfireland.ie or contact us on 01 4962433 or email fundraising@cfireland.ie for further details!

October 30th: Irish Life Dublin Marathon The Irish Life Dublin Marathon returns on Sunday, 30th October for the first time since 2019.

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

For further information, please contact us by email fundraising@ cfireland.ie or call (01) 496 2433.

November 7th: TCS New York City Marathon Registration is now closed for the TCS New York City Marathon 2022. Good luck to all those entering, in particular those running on behalf of CFI. Don't forget to share your photos of the day and tag us on social media!

You can now register your interest in participating in the TCS New York City Marathon in 2023 on our website – www.cfireland.ie.

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

www.cfireland.ie

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December 9th: Christmas Jumper Day 4 CF On Friday 9th 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 9th December. Encourage everyone to wear their Christmas Jumper to work in return for a €5 donation to support people with Cystic Fibrosis in Ireland!

2. Register your interest to participate at 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 #ChristmasJumperDay4CF2022 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 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 2022 – Ho Ho Ho!

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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 2022.

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/get-involved/ events-and-challenges.

Skydives A skydive is impossible to describe. You have to experience it to appreciate what you are missing out on.

If you have not done a skydive yet, take the chance to gain this experience of a lifetime while supporting a great cause. This challenge is one you will never forget and all you have to do is register your interest on our website at www.cfireland.ie and we will contact you to confirm the next steps.

Now is the time. Take on the challenge and you will remember 2022 for all the right reasons. You can contact our office on 01 496 2433 or email fundraising@cfireland.ie for more information.

www.cfireland.ie

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

65 Roses Day Cystic Fibrosis Ireland would like to say a huge thank you to all our supporters who supported our national fundraising day - 65 Roses Day on Friday 8th April. So far, well over €240,000 has been raised and we would encourage anyone who still has money to lodge to please lodge outstanding funds as soon as possible. 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 thank also to every 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, coffee mornings, bake sales, 65minute walks, 65km cycle, 65 swims and a 65-hole golf classic to name just a few. If you still have funds to lodge funds, we would ask that you lodge funds to 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 2023 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 €20,000 raised to date and monies still coming in.

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Congratulations also to the first man back, Evan Scully, PWCF with an amazing time 37 minutes 10 seconds!!

Well done Evan, an inspiration to all people with CF.

Malin2Mizen Cycle4CF Huge congratulations to the 34 cyclists who completed the Malin2Mizen Cycle4CF, all arriving safely in Mizen Head after an incredible journey, cycling over 4 days from Malin Head to Mizen Head. Thank you also to the support team who helped the cyclists throughout the event. Thank you to everyone who donated towards the cycle and to all our sponsors including Maxol, Europcar, Dunnes Stores and CycleBike. To date well over €100,000 has been raised by the cyclists at what is now one of the largest events on our fundraising calendar and a testament to Bernie Priestley and the Malin2Mizen Cycle4CF organising committee.

Registration for our Malin2Mizen Cycle4CF 2023 is now open on our website www.cfireland.ie with the cycle taking place from Thursday, 14th to Sunday, 17th September 2023.

One in 1000 / Vhi Women’s Mini Marathon A huge congratulations to everyone who joined our One in 1000 Team to take part in the Vhi Women’s Mini Marathon on Sunday, 5th June. After a long 3 year wait since the last outdoor event in 2019, it was wonderful to see a sea of purple representing Cystic Fibrosis Ireland again on this very special day.

Participants joined us in the D2 Harcourt hotel where we relaxed before the start of the 10k,had a great warm up event led by Nicola and Miriam, and afterwards everyone wound down with some music, food and some refreshments.

Thank you to our sponsors Ballygowan, Fyffes and Haribo for providing the water, bananas and jellies which went down a treat! To date over €22,000 has been raised by all the amazing participants with lots of money still coming in on iDonate pages, being lodged on our website or to our bank account.

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 #Onein10002022 and we will add them to the gallery of pictures from the day on our website.

www.cfireland.ie

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

Cavan Martina Plunkett mplunkett1000@yahoo.ie

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

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

Galway Kayleigh McGuire kayleighmcguire0@gmail.com

Dublin West Rebecca Horgan cfdublinwest@gmail.com

TLC4CF Caitriona Hayes caitrionahayes@icloud.com

Eastern Branch Mary McCarroll marymccarroll1@hotmail.com

Clare TLC4CF@cfireland.ie

Carlow Leigh Bolger bolgerleigh@yahoo.ie

Limerick Niamh Harrington niamhh75@gmail.com

Cork Aisling O'Neill aislingquill@gmail.com

Tipperary Mandy Quigley mandyquigley26@gmail.com

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

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Spectrum / Summer 2022