SPECTRUM Let me tell you a story - The launch of "Our Baby has Sixty-Five Roses" - Cystic Fibrosis Ireland
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SPECTRUM Summer / 2019 Let me tell you a story... The launch of "Our Baby has Sixty-Five Roses" www.cfireland.ie i
CONTENTS
CEO’s Message
Latest News | 1 - 3
* Hospital Updates Welcome to the summer issue of
* Travelling with CFTR Medicines Spectrum. We have a bumper issue that
includes a report from the European
* Community Events
CF Conference in Liverpool; our work
* The Hyde Family
with other patient groups to improve
* The Irish Donor Network Philip Watt
services; our regular hospital update
* Working with other Patient Groups and much more. Chief Executive
* Thank You Erin
It has been a busy few months for the CF Community
beginning in March with the Annual Conference, we then
Spotlight | 4 - 6 had 65 Roses Day in April. The Malin 2 Mizen Cycle, the
Gastrointestinal Issues & CF 2nd annual CF Art Exhibition in Limerick and the launch of
* A Parent's Perspective the Helium Arts Project in Waterford all took place in May
and so far in June we have had the One in 1,000 campaign
* Personal Experience
and launch of "Our Baby has Sixty-Five Roses."
Hospital Hub | 7 The big news from this year’s European conference was
the very promising forthcoming Vertex triple combination
Member Services| 8 drug therapy. CFI understands that this drug therapy is
being fast-tracked, but it may still take up to 18 months
before it is approved by the European Medicines Agency
Community Events| 9 - 14 (EMA).
* Seven Summer Stars
* Our Baby has Sixty-Five Roses We also include a report on our own conference held
in Carlow in March – many of the podcasts are now
available on our website.
Kids Corner | 15
There have been a number of positive developments
Youth Reach | 16 with the hospitals and we continue to advocate for our
members both here and abroad on improving CF services
Conference Reports| 17- 23 and care.
* CFI Annual Conference Finally, we would like to say farewell and many thanks to
* ECFS our Regional Officer, Erin Sugrue, who has contributed
much over the past 5 years to improving CF services in
Research | 24 - 26 Limerick Hospital in partnership with TLC4CF.
* Phages as a therapy against infection
* PERT Pilot Study
* Study update on treatment of lung infections Philip Watt, CEO, CFI
* Superbugs Webinar
Samantha Byrne & Nuala McAuley, Editors
Fundraising |27 - 37
* Challenges and Events DISCLAIMER:
* Thank You The views of contributors, when expressed in this publication,
do not necessarily reflect the position or policy of Cystic Fibrosis
Ireland.
ii Spectrum / Summer 2019
LATEST NEWS:
Beaumont Hospital Update
The proposed new 20-room in-patient unit in Beaumont Hospital has received planning permission and the hospital
is currently in the process of inviting tenders for the building of the unit. This means that contractors will be invited
to submit bids to undertake this major building project. We have been told this process will be completed by July /
August 2019 and a decision to appoint a preferred contractor will require a further number of weeks – so it is likely a
decision will be made in early Autumn.
The final decision on when this project receives funding is still to be taken by Government, but we will keep up the
pressure. CFI undertakes this work in partnership with local charity CF Hopesource. We have pledged to fundraise
€1m towards the cost of this project.
In short, there is good continuing progress which follows on the decision by Dublin City Council to approve planning
permission in January 2019.
Senior Registrar in Beaumont
CFI and CF Hopesource have funded a CF Registrar (senior doctor)
specialist post in Beaumont Hospital for approximately two years
at a cost of €100,000 per annum.
We were recently told that this post will now be funded by the HSE
/ Beaumont Hospital, which is welcome news.
Mater Hospital
Following representation from CFI over many months, the Mater Hospital have indicated their intent in converting 7
additional pre and post-operative rooms in MMUH. Some of these rooms are presently used for out-patient clinics
and admin duties so this process is likely to be incremental over the next few months.
We are delighted to confirm that the post created and funded by CFI, Post-operative Clinical Nurse Specialist (CNS)
has been made permanent and from now on the post will be funded by the Mater Hospital.
In partnership with our branches in Galway and Sligo, we also continue to fund posts in their local hospitals.
Clinical Genetic Services
CFI along with other patient groups recently met with senior staff in the HSE
about resourcing and other concerns in relation to Clinical Genetic Services
(based in Crumlin). This service plays a vital role in diagnosing, advising treatment
and providing genetic counselling, including to CF patients. Following lobbying
over many months, 2 key staff are being appointed including a new Professor of
Genetic Services. The appointments will be made circa August 2019.
Travelling with CFTR Medicines
A recent issue that has arisen is the challenges for PWCF travelling abroad for work or extended tourism if they are
on a CFTR Drug (Kalydeco, Orkambi, Smykevi). We understand clinicians/hospitals are reluctant to give more than a
3-month supply because of the very high cost of the drugs and fears that the drugs will deteriorate or may be lost.
Cystic Fibrosis Ireland will prioritise this issue over the coming months.
www.cfireland.ie 1
Community Events
"Our Baby has Sixty-Five Roses" is the title of a new book just
launched by Kildare mums Eilís Moroney and Ruth Cahill to
help explain cystic fibrosis (CF) to other children in an age-
appropriate way. The book is about helping siblings and
relatives better support the person with CF in their lives, while
also giving comfort to parents at a very difficult and challenging
time.
We were very happy to host the launch of the book in
Rathmines on June 19th.
On May 29th, CFI were delighted to attend the launch of
"Seven Summer Stars", an exhibition of art work from a group
of young teenagers with CF who took part in a pilot programme
led by Helium Arts, in the Waterford Healing Arts Trust centre.
The project took place in spring 2019 and saw seven young
people with CF aged 12-16 using technology for mediated
meet-ups. Over the six online sessions the teenagers took
part in a group art journaling experience and had access to
professional artist mentors.
Separately, the second annual art exhibition for people with CF
took place in the CB1 Gallery in Limerick in June. Over fifteen
pieces by eight artists were on display. A huge congratulations
to all those who took part in the exhibition and to Sara Cross,
curator for organising the exhibition once again.
It is great to see the CF community using their creativity to help
raise awareness and understanding of cystic fibrosis.
Hyde family in Australia
An Irish family in Australia who emigrated in 2009 are now
being threatened with deportation because their son, Darragh
(born 3 years ago in Australia) was diagnosed with CF and is
considered a ‘treatment burden’. The family are well integrated
into Australia – the mother is a Vice Principal and the father is a
part-time bus driver and carer.
Their appeal failed on 7th May but CFI understands that a final appeal will be considered by the Australian Minister
for Home Affairs. A letter written by CFI to support the Hyde family received much media profile in Australia.
Transplants
CFI is an active member of the Irish Donor Network (IDN) which includes 7 other patient
groups including: The Alpha One Foundation, COPD Ireland, Cystinosis Ireland, The Irish
Lung Fibrosis Association, Irish Heart and Lung Transplant Association and the Pulmonary
Hypertension Unit.
The IDN brings together like minded patient groups to campaign for soft opt out organ
donation and to provide support in our major transplant centres.
We will feature our work with MRCG, IPPOSI, Lung Health Alliance in future editions of
Spectrum.
2 Spectrum / Summer 2019
Why CFI Works Closely with Other Patient Groups
CFI plays an active role in working with other patient groups to address some of the issues that impact on all of us,
for example in relation to accessing new and innovative drug therapies.
International Research published in April 2019 shows that Ireland performs poorly in respect of:
• Speed of Access to new medicines
Ireland is 20th out of 27 European countries
• The wait time for Irish patients for new medicines
Patients in Ireland wait on average 486 days for some medicines. The average wait time across 27 countries was
413 days and Denmark, which has similar population to Ireland, has a wait time of only 146 days
• The wait times for new drugs in Ireland are getting longer
The average wait time in 2017 in Ireland was 408 days, now its 486 days
• Availability of new medicines
Ireland ranks 16th out of 27 countries. Just 42% of EMA licensed medicines in 2018 were available in Ireland
• Accessing drugs for rare (orphan) diseases
Just 29% of new rare disease medicines licensed in 2015-17 are available in Ireland by the end of 2018
Some of the drug therapies that have featured in recent media reports involving significant patient advocacy include,
for example: Orkambi (Cystic Fibrosis); Respreeza (Alpha One) Translarna (Duchenne Muscular Dystrophy) Spinraza
(Spinal Muscular Atrophy/SMA).
Part of the problem for delays or lack of availability of new drugs in Ireland may be related to the way drugs are
currently assessed and the resources for assessment. A further issue is the need for more patient and clinical
involvement in assessing drugs in Ireland. This involvement is much better in Scotland where we might look to ideas
for improving the system.
This means that access to and delays with new drugs is a problem for many patient groups and it makes sense that
we collaborate through bodies such as the Rare Disease Task Force; Medical Research Charities Group (MRCG) and
the Irish Platform for Patient Organisations, Science & Industry (IPPOSI) to address these problems jointly.
Thank You Erin
Many thanks to Erin Sugrue who is leaving CFI after 5 years as our fantastic regional
officer in Limerick working in partnership with the 3 local branches – Tipperary,
Limerick and Clare - TLC4CF. We were very lucky to recruit Erin who came with great
experience from Pobal, an agency which works on behalf of Government to support
communities and local agencies toward achieving social inclusion and development.
As part of the work of CFI / TLC4CF in the Mid-West region, Erin contributed to:
• Preparing the liaison meetings between UHL and CFI/TLC4CF
• Helping to coordinate fundraising in the region
• Supporting many of the meetings that led to the Leben building (adult CF unit) in
University Hospital Limerick
• Liaising with TLC4CF and CFI
Erin coordinated the CFI / TLC4CF office in Limerick and is a fantastic organiser and coordinator and she is a pleasure
to work with. With many interests outside CFI including in the cultural and media sphere, it is in this arena that Erin
is seeking to get further involved in the near future.
Thanks again Erin, we will miss you. You made a real difference in your work over 5 challenging years to people with
CF and their families in the Mid-West region.
www.cfireland.ie 3
SPOTLIGHT:
Gastronintestinal Issues and CF
C ystic fibrosis is widely known as a respiratory condition, however it is actually a multi-organ disease which
affects the lungs, pancreas, digestive tract, gastrointestinal system and the reproductive system.
According to the 2018 CFRI Annual Report, over 90% of people with cystic fibrosis in Ireland experience
gastrointestinal issues such as gallbladder disease, distal intestinal obstruction syndrome, gastro-esophageal
reflux disease, constipation, GI bleeds, peptic ulcers and pancreatitis.
"The first step in solving a problem, is recognising that one does exist."
As an organisation, CF Ireland are committed to increasing awareness of all aspects of cystic fibrosis. We will
continue to advocate for better understanding of the impact cystic fibrosis has on the gastrointestinal system and
improved integration of gastroenterology in CF care.
We receive a lot of feedback from members requesting more information about the gastrointestinal issues
associated with CF. We are grateful that two members of the CF Community have chosen to share their own
experiences with us. As this is a sensitive and personal subject, their experiences are being published anonymously.
We hope that these personal stories will help individuals in the CF Community recognise that they are not alone,
but also help start the process of viewing cystic fibrosis as the multi-organ condition it is.
A Parent's Perspective
Tuesday 23rd April 2013 we sat in Crumlin hospital waiting to see the
Gastroenterology Consultant. We had been referred by a locum in our GP
surgery after a long period of my son suffering ongoing abnormal bowel
movements and tummy pain. We were told it was toddler diarrhoea and
that he would grow out of it, but as he got older he would say his tummy
was sore most days and I knew something wasn't right.
I booked another appointment with his GP and there was a locum Doctor
covering that day. She took a good look over him and I told her I felt
there was something wrong. She had a friend who worked in the gastro
department in Crumlin and made a phone call. We were referred for an
appointment and had to drop a sample down to the hospital that day. I was
relieved it was getting looked into so we arranged the sample straight away.
A week later as I was driving home from work, I pulled over to take a call
from the GP surgery. The Doctor explained the sample was very abnormal
and they wanted to see us in Crumlin the following week. I was so upset
as she could not tell me anything more. I was just lucky that we saw that
Doctor on that day and am forever grateful to her.
4 Spectrum / Summer 2019
We went to the appointment and the nurse took some information
about my son's diet, explaining the pains and bowel movements. He
had a great diet and ate most foods, but he could have up to 10 bowel
movements in one day. He had chronic tummy pain every single day.
He was very tired and pale and never slept very well. The consultant
came in and immediately asked if anyone in the family had cystic
fibrosis, we had just lost my husband's friend to CF the year before
so we were really shocked and not expecting to hear those words as I
always thought CF was lung disease. He arranged a sweat test for the
following day and those 24 hours were an agonising wait.
24th April we arrived back in to Crumlin hospital for the sweat test, not knowing what to expect. The test was really
easy and not distressing for my son who was only three at the time. One of the nurses told me she would phone us
later that day with the results. It was an awful wait but we got the phone call that evening confirming a diagnosis of
cystic fibrosis. We were heartbroken. I will never forget that day.
Four days later we met the whole team and the wonderful Consultant, Dr Cox. They answered all our questions, but
most of it was a blur to be honest we were still in shock. We had to change up our routine and teach him how to
take all these new medications and make sure they were given correctly. He was a dream, he took to everything so
well and was swallowing capsules within a couple of weeks!
My son was in playschool at the time he was diagnosed and was settled there and really enjoyed it. He went back
to school a few weeks after being diagnosed and when I had a chat with the manager, I found out that another
member of staff had CF. I remember being told in Crumlin hospital that he was not allowed mix with anyone else
with Cystic Fibrosis and I panicked. His teacher who had it was absolutely amazing, swapping his days so that my
son could still attend a couple of days a week and gave me some information packs from CF Ireland. He was so
nice about it and we were so thankful to him that we did not have to change playschools as my son had settled
and made lots of friends. However, he got on so well with the teacher and really missed having him around. It was
really sad but we had to stick by the rules to keep everyone safe. They were happy to give him his Creon tablets at
lunchtime and encourage hand hygiene.
It was a long road getting the dosage right and his bowel movements settled, but the constant stomach pain went
on for many more years and unfortunately is ongoing. He has had endoscopes, colonoscopy, bronchoscopy, coeliac
testing, all of which came back fine. He has learned how to cope with the pain and is a very happy young boy. He
never complains and takes all the advice he gets from his team. He has seen the psychologist on the CF team for
some coping strategies.
I find it hard to get answers and nobody is sure why exactly he is suffering with his stomach so
much. His lung function is great and he has been well in relation to his lungs. People always
associate CF with the lungs, but it is so much more. I never found much CF gastro related
information when researching on google etc. about stomach pain, it mentions frequent bulky
stools, but not to the extent we had experienced.
He has a fantastic teacher who understands his needs. He takes
his medication during school and is allowed toilet breaks without
asking. His friends in school always look out for him and will sit
with him in yard if he is having a bad day with his pains.
It never affects his confidence. He deals with it all really well. He is
such a happy, polite, outgoing young boy and makes the most of
every single day. He makes us so proud and we are so grateful to
his team in Crumlin who look after him so well.
www.cfireland.ie 5
A Personal Experience
Back in 2014 I spent my summer off college in Chicago, it was so fun. I was waitressing part-time, going to the beach
and exploring the city! I was also training for the New York City marathon. My health was good, my CF was behaving
itself. But on my last week there I became very unwell – terrible headaches, dizziness and nausea. I wasn’t sure what
the problem was, I had to spend the time in bed. I also didn’t like the thought of the flight home when being so
unwell.
Once back in Ireland I rested up for a while and felt OK again. I took part in the marathon that November and was
delighted with the achievement. But I kept getting the dizziness and nausea. I was so sick that Christmas, having to
spend most of the time in bed. Finally the doctor sent me for an x-ray of my abdomen and it showed I was all blocked
up – this was strange as I had been having bowel movements all along. I had to go home and take a combination of
laxatives to try sort it out. It was a very slow process, the doctors told me to have patience that it would take time for
the medication to work.
I have always had bowel problems due to my CF, but this was
different, it was a lot worse. I was sent for numerous tests, scopes
and of course tried out different diets. The special diets didn’t
make any difference and the tests didn’t show up anything unusual.
As well as the dizziness and nausea, I was now having problems
with a distended stomach and terrible pains. I was referred to the
gastro surgical team in Beaumont, they were great, so nice and
understanding to how difficult it all was. I felt the CF team never
had the interest or understanding with my gastro problems – their
answer to it was always that they only deal with respiratory issues.
At this stage I was very unwell, I had to do my college work from home. I ate only plain food, I had lost a lot of weight
and my brain function wasn’t great. I spent most of my time at home drinking Movicol! My routine was 12 Movicol
throughout the day and 2 dulcolax tablets at night – I tried Lactulose as well but it kept fermenting in my stomach
causing it to be extra distended and painful.
The surgical team say my gastro issues are to do with producing too much mucus,
causing mucus plugs, which are stopping things being able to work. After 2 years
of trying out numerous combinations of laxatives and medications for my bowel
nothing was working, so the surgical team said it was time for me to get a stoma (an
ileostomy). I knew this was something I wanted to put off for as long as possible, I
asked them could I wait until it was completely necessary – my thoughts were that
I’d prefer to feel sick than to have to deal with that just yet! So I was referred to the
constipation nurse – her label actually read ‘constipation nurse’ which I found so
funny!
The constipation nurse was a great help, she discussed alternative treatments – she organised for a nurse to come to
my house to show me how to irrigate my bowel (not exactly something I’m dying to talk about but that’s CF life).
Nowadays my daily routine is to irritate my bowel every morning using Qufora IrriSedo Cone system, drink 6
Movicol sachets throughout the day and take 2 dulcolax tablets every night. This works for me some days and then
sometimes it doesn’t, but the difference is that I can cope with it. I no longer panic when I don’t have a bowel
movement for a few weeks, I know if I continue with my meds it will be OK eventually. I also drink a minimum of 3
litres of water a day, walk most days and eat a healthy balanced diet.
I hope that in the near future gastro will become more involved in the treatment of CF. Whether that’s better
communication between the CF team and gastro surgical team, or ideally each CF Team should include a gastro
doctor.
6 Spectrum / Summer 2019
Hospital Hub
Noticeboard
T
he summer months are upon us, and even the most pessimistic of us will surely still be holding out a little
hope for a return of last year’s glorious sunny summer days.
Whether the summer sun does arrive in Ireland this year, or you’ve hedged your bets and booked a sun
holiday, maintaining hydration in hot weather is essential.
In CF, high salt losses from sweat can lead to salt levels in the blood becoming too low. This can cause a dulling
of the sensation of feeling thirsty and can quickly lead to dehydration if not addressed.
To maintain hydration safely in hot weather you therefore need to drink plenty of fluids
without waiting to feel thirsty, and replace the salt you lose in sweat by eating salty snacks
(like pretzels, salted nuts or popcorn) and by adding a pinch of salt to food and drinks. If
you are heading somewhere very hot, exercising in hot conditions, or if you perspire a lot
normally speak to your CF team about getting some salt supplements on prescription to
take away with you.
Roisin Fitzpatrick, Senior Dietician, St. Vincent's University Hospital
T
LC4CF and the CF community of the mid-west region were delighted to
present a ‘special contribution to CF’ award to Peig Harnett, to mark her
retirement from nursing. Peig has been caring for people with CF for
over 30 years and we have been honoured to have her as the Clinical Nurse
Specialist in Limerick.
Over the past three decades she has been trusted and loved by her patients;
relied upon by their parents; esteemed by her colleagues and feared by hospital
management!
She worked incredibly hard to get the new CF adult unit in Limerick running
properly and she has always undertaken her role with dignity, professionalism,
efficiency and most importantly compassion.
According to her patients "she cared enough to sit on the
edge of our bed to talk fashion, relationships or give us
comfort in our darkest hours".
Peig was always contactable by mobile and took calls at all
hours of the day and night, regardless of whether it was
the weekend or she was on holidays. A gesture that is rare
and valued by all for whom she was their first, last and only
resort. We congratulate Peig on her award and her well
deserved retirement.
www.cfireland.ie 7
Member Services
Exercise Grant Round 2 opens Monday 9th September
Application form available on CFI website from mid August
Round will close when funds are exhausted
Apply early to avoid disappointment
Did you know?
If you are post-transplant PWCF you may be entitled to re-imbursement
of the cost of 50+ SPF Sunscreen.
Talk to your post-transplant nurse, or call us here at CFI for more information!
Heading away in 2019? Got Questions?
Travel Insurance
Taking medications abroad
Traveling with Oxygen
CF Clinics abroad
Talking to your CF team
Call Rory Tallon on 087 932 3930 or Caroline Heffernan on 087 932 3933
Enjoy your holiday!
8 Spectrum / Summer 2019Seven Summer Stars
S
even Summer Stars are a group of young teenagers with cystic fibrosis who have been making art and
connecting creatively as part of a pilot online art programme led by Helium Arts, the national children’s arts
and health organisation, in partnership with Cystic Fibrosis Ireland, the Irish Museum of Modern Art and
Waterford Healing Arts Trust.
The first phase of the project took place in spring 2019 with seven young people aged 12-16.
Using digital tools and online mediated meet-ups along with access to professional artist mentors, the project
supported a group art journaling experience over six online sessions. Artist Rachel Tynan, who has been
collaborating with young people on Helium’s creative projects since 2012, is the lead artist. An Cosán Virtual
Community College provided the online technology and moderated the online sessions.
So how did it all work?
The young people connected via a
virtual classroom. A multiple-screen
format meant everyone could see each
other during group discussions and the
screen could also be reconfigured to just
show one person; for example, when
Rachel was sharing PDFs of artwork
and journaling processes. Participants
typed ideas and suggestions into a live
messaging box and breakout sessions
enabled smaller group conversations
which were a great opportunity for
participants to get to know each other Artist Rachel Tynan sharing examples of her artwork using the virtual classroom.
better.
A very cool Ladibug document camera allowed for high quality art demonstrations. Before each workshop, the
young people could take part in an online artist ‘drop-in’ session with Rachel to talk about what they were working
on and how they might progress their ideas.
“I thought it was really cool to meet other people with CF and have that connection. And I
like how we’re sort of all in the same room at the same time and that’s comforting.”
Each workshop was recorded for anyone who couldn’t make a session. The recordings were stored on Google
Classroom, which also became a space to connect between workshops. Participants uploaded artwork and images
from their project journals here, shared photos that ‘tell a story’ as part of a project led by guest filmmaker Linda
Curtin, and watched art tutorial videos created by Rachel. One of the videos demonstrated how to create the fairy
light roses seen in the image above.
“I think it’s really fun and I like how we can all find different ways of being creative”
www.cfireland.ie 9To add another dimension of excitement between sessions, Rachel
posted the young people art packages. Before the project started,
each participant received an art pack in the post and it was great
to see everyone using the materials in their journaling work. The
young people also received surprise packages (‘Do not open …yet!’),
postcards of artwork from the National Gallery which tied into themes
they were exploring, and mugs that they could decorate.
Fostering connections between young people with CF in an online
creative environment was one of the main aims of the project.
Participants explored connections they shared on a visual map, from
owning a dog (nearly everybody) to loving chocolate (everybody) to Surprise packages posted to participants.
playing the guitar.
“At the beginning we did that drawing where we connected what we had in common and I
think that really just started to connect us. And then as we moved on we kind of became
good friends and we haven’t even met each other in real life.”
Through a word association game and a digital poll, the young people decided to call themselves Seven Summer
Stars and designed their own group logos. Rachel combined their design suggestions into one logo and everyone
received their own Seven Summer Stars t-shirt in the post.
The project also sought to increase access to the arts for young people with CF. Participants explored artworks
from IMMA’s Freud Project ‘Gaze’ exhibition via a pre-recorded gallery experience, led by curator Mark Maguire
from IMMA’s Engagement and Learning Department with artist Rachel Tynan. Artists with CF who use their life
experiences as inspiration for their work was another area of exploration and Mayo-based artist Katie Moore
proved to be a big hit!
“I actually have realized I really like drawing things that relate to my CF
,
that’s because of you guys!”
The group were excited by the idea of sharing their artwork at the end of the project and a public showcase took
place at Waterford Healing Arts Trust on May 29th. Participants were brought on a virtual tour and took part in a
virtual ‘meet the artist’ session with project partners and showcase attendees where they discussed the inspiration
behind their work.
The public showcase at Waterford Healing Arts Trust on May 29th.
“I’m really excited to have a proper showcase and have my art in it. It’s not something you
would do every day or something that most people would get [to do].”
10 Spectrum / Summer 2019“Thank you for such inspiring, insightful and revealing artworks! Love them!” –
showcase visitor
For a lot of the young artists, their CF experience was
a key theme, with purple roses and hues populating
their pictures, thorns protruding from a woman’s
body, and roots mimicking the double helix. The
artwork was positive (“My illness doesn’t define me”);
poignant (“Cystic Fibrosis grew roses in my lungs. Yes,
they’re beautiful but, I can’t breathe”); and optimistic,
with a purple butterfly signalling change and hope.
The showcase also included a project film created by
Linda Curtin with animator Dave Lawless, featuring
some of the young people’s project work and audio
reflections, and a response artwork by Rachel inspired
by the theme of connections and featuring the young
The artwork created by the Seven Summer Stars people’s hands waving to each other during the
online sessions.
Since the showcase, Seven Summer Stars have had their artworks exhibited at the CFI celebration for the VHI
Women's Mini Marathon and the book launch for Our Baby has Sixty-Five Roses.
Many thanks to the seven talented young artists for your creativity, imagination and dedication, and showing us that
even if we can’t always connect in person we can create amazing connections in other ways.
Left: Guests at the launch of "Our Baby has Sixty-Five Roses" admiring the art work of the Seven Summer Stars.
Right: Participants in the One in 1,000 Campaign showcasing the work of the Seven Summer Stars
Join us this Autumn!
The next phase of the online art programme will take place this Autumn and is open to young people with cystic
fibrosis aged 12-16. The programme is free for participants and no art experience is necessary. If you would like
to get involved, register your interest with Samantha Byrne at CFI: sbyrne@cfireland.ie or 01 4962433
The Summer Stars online art programme is supported by The Arts Council, The Ireland Funds, Dublin City Council
and The Health Services Credit Union.
Helium Arts is an award-winning charity supporting the social and emotional needs of children facing long-term
medical conditions through arts-based creative projects in hospital, community and public settings.
www.helium.ie
www.cfireland.ie 11Book Launch
I
’ve always wanted to write a book. As the saying goes, ‘There’s a book
in us all.’ I’ve toyed with short stories before. I’m a primary school
teacher, and I’ve often used unassuming classes as guinea pigs as I’ve
read aloud extracts from different stories that I’ve dabbled with. Children
are the harshest of critics so I could never ask for better feedback!
My beautiful daughter Aibhín was born last October. My husband and I
were so excited to meet her, and thankfully she only kept us waiting for
one week extra after her due date! Our family and friends celebrated
Aibhín’s arrival with visits, dinners, presents and lots of new baby
snuggles. Aibhín is a very calm and happy little lady. She never complained
throughout her first three weeks. She drank her milk, produced dirty
nappies and slept, just like any other baby. Except for one big difference to
her peers –she drank a lot of milk and produced a lot of dirty nappies too.
Being my first child, I didn’t think that her feeding and toilet habits were anything out of the ordinary. But all that
changed when I received a phone call from Tallaght Hospital and the dutiful nurse kindly and sensitively asked me to
bring my daughter in to meet the doctors the following morning, amid their concerns for Aibhín and the possibility
that she might have CF. And she did.
Anyone who has been on that journey will know how painfully long those 24 hours are between getting that fateful
phone call and receiving your child’s diagnosis. All I wanted was to go to the hospital there and then and find out one
way or another. The wait was excruciating.
My husband and I feel so privileged that Aibhín is under the care of the unbelievably dedicated CF staff of Tallaght
Hospital. It was strange to think that this team of people had such important knowledge about our daughter, that we
as her parents were completely unaware of it, and that they were waiting to take us under their wing, and mind us
and guide us. And that’s just what they did and continue to do.
When I first met Ruth, I didn’t know that she was a storybook illustrator. Fate definitely played a part in our
friendship and business endeavours, and our book was born!
I wrote this storybook, as I found myself overwhelmed with the words ‘Cystic Fibrosis’ in the initial weeks and
months that followed Aibhín’s diagnosis. It crept up everywhere that I looked, in the drains of the sink in the
bathroom and kitchen, in the exercise ball that I thought I’d be rid of after my pregnancy, in the baby weaning spoons
that appeared in our home all too soon, in the multivit that destroyed all of my beautiful bibs for Aibhín!
This story popped into my head one day when I found myself thinking about what Aibhín would say if she had a
voice. She would probably have told us all to stop being upset, that she was and is doing mighty, and that she has so
much enthusiasm and excitement for life and she wants to share it with everyone that she meets! I wrote this story
to remind myself to see the bigger picture - which is that anyone, anyone at all with a medical condition, or any type
of condition, is not and should not be defined by it. We all have something in our lives, nobody is perfect. I wrote
this book to really encourage families, friends and society to detach a diagnosis from an individual and to recognise
that everyone on this planet has so much to offer, achieve, celebrate, share, see, smell, experience, learn…the list is
endless.
Thanks to the inclusion of CF testing in the heel prick test over the past seven years, I also believe that this book will
be of benefit to families who have other young children in the home, be they siblings, cousins or neighbours.
12 Spectrum / Summer 2019The words and pictures gently explain a day in the life of a baby with CF,
and subtly refer to ways in which the entire family can get on board with
physiotherapy and essential attention to germ control through hand
washing etc.
Ruth’s drawings are sensitive, humorous and heartwarming. They
perfectly compliment the words of the story. Though these words are
the story of my family, I firmly believe that my words are the story of
many others too. And I hope that our book, ‘Our Baby has Sixty-Five
Roses’ will help others to take the paralyzing fear out of a CF diagnosis
for a newborn baby. Love them, and enjoy them and go easy on yourself
too.
"Our Baby has Sixty-Five Roses"
is available to buy on www.cfireland.ie Eilís Moroney
S
ince I was a child I loved to draw and paint, endlessly doodling in my homework
journal instead of studying. My passion for art brought me to study Graphic
Design in the Dublin Institute of Technology followed by a Postgraduate
Diploma in Advertising.
In 2009, following the birth of my first child Harry, and his diagnosis of CF, I
established Duck Blue, an illustration and design studio based in Maynooth, Co.
Kildare. I work at my studio part time when my children are at school for a few hours
a day. I really enjoy creating bespoke cards and illustrations for all occasions from
birth announcements to keepsake wedding cards. Every Christmas I produce a range
of Christmas Cards that I sell online and at markets. I give 10% of these Christmas
proceeds to CF Ireland. Often I get repeat customers at these markets. Last December
one such customer came to buy some cards and explained to me how her cousin’s
little baby had just been born and diagnosed with CF. Having been in this position 10
years previous I gave this girl my card and said that when her cousin was ready she
could call me and we could have a chat if she wanted, especially because we only
lived a few miles from each other. And so began a wonderful friendship…
On first meeting Eilís in the beautiful Castletown House in Celbridge we chatted about her precious newborn baby,
how scary and challenging the CF diagnosis is, the heel prick test, hospitals, family and friendship. We hit it off
from the get go. During this meeting Eilís told me that she had written a short story about a newborn baby being
diagnosed with CF and asked if I would like to read it and perhaps illustrate the story. I knew instantly that I wanted
to be a part of this.
The simplicity but honesty of her writing really moved me. The detail of what she wanted portrayed in each drawing
was just what I felt was needed and over a 6 week period we worked tirelessly on creating the book that we proudly
have today.
We really feel that our book, ‘Our Baby has Sixty-Five Roses’ will become an invaluable resource not just for new
parents and families, but for nieces and nephews, pre-schools and primary schools, and of course hospitals too. I
know from my own personal experience, that for the first few years after my son’s CF diagnosis, I felt very lost and I
yearned for the life that I dreamed my child would have, and of course the life that we as a family would have.
Both Eilís and myself have experienced our firstborn child being given a CF diagnosis. We both agree that being a
parent for the first time is hard enough without a diagnosis like CF being included. We really hope that families that
face the diagnosis going forward can see our book and realise that they are not alone, that their baby does have CF
but that they are also their own individuals with so much more to offer, share and celebrate. The future is looking
bright… and together we will fight for a better future for our children and all PWCF.
Ruth Cahill
www.cfireland.ie 13The launch of "Our Baby has Sixty-Five Roses" took place in the Pot Bellied Pig, Rathmines on June 19th 14 Spectrum / Summer 2019
KIDS CORNER
E xercise is important for everyone, but especially for
people with cystic fibrosis as it helps to clear the
mucus from the lungs.
It is recommended that everyone should do at least 30
minutes of exercise every day. For people with CF exercise
is part of their everyday routine. When we do something
all the time it can sometimes become boring, but exercise
doesn't have to be boring!
Whether you are alone or with a group of your friends
there are plenty of activities that you can do to ensure you CFI would like to congratulate PWCF
are exercising and clearing mucus off your lungs, but that Evan Nealon, aged 9 and his brothers
are fun such as playing with your dog, learning to play a Dylan (12) and Alex (9) who were
wind instrument, flying a kite or a water balloon fight! recognised for their outstanding efforts
with the Overall Super Junior Award at the
Since school is UNESCO Young Environmentalist Awards.
finished for the
Since they started "Evan, Alex and Dylan’s
summer there is
Tralee Canal Clean Up" over 500 bags of
plenty of time to
rubbish have been removed from the
try out new ways
Tralee Canal and surrounding areas.
to exercise!
Well done boys!
Have fun!
Can you find all the summer sports in the wordsearch below?
MAZE PUZZLE SOLUTION
Spring 2019
Badminton Cricket Cycling Golf
Rollerskating Skateboarding Skipping Snorkeling
Surfing Swimming Tennis Volleyball
www.cfireland.ie 15YOUTH REACH
C ystic Fibrosis is widely known as a respiratory condition, but in fact it affects several organs in the body from the
lungs to the digestive system, pancreas to the reproduction system. While the impact of CF can vary from person
to person, with so many areas of the body impacted by CF it is no wonder that a lot of people with CF struggle with
their self-esteem and body image.
My stomac
e ve ry one e ls e ! h is swollen
' sho rte r than
Im !
I don't have any muscle, even though I exercise regularly!
However, you are not alone. Body confidence is something lots of people struggle with
regardless if they have a medical condition or not. Almost everyone has a part of their
body they are unhappy with. However, we should try and focus on the positives rather
than the negatives! Sometimes we can't see the beauty that is right in front of us!
What do you like most about your body?
List out what you think are your three best features:
1)
2)
3)
Then ask your parents, your best friend, your siblings etc.
While we listen to the negative thoughts about our own bodies, we very rarely take on
board the positives. Hearing someone else tell you what they love about you or writing
down your own positive comments to read back can help when we are
struggling with our confidence. This is something you can do for others
too!
Focus on what your body can DO!
There is much more to our bodies than our looks.
With CF impacting various organs it is easy to focus on what our body
can't do in comparison to others, but if we try to focus on the positives
and not the negatives we will see there is a lot our body can do that we
don't acknowledge.
What is your biggest accomplishment to date?
Standing in front of a class to deliver a presentation? Your personal
best in a park run? An A on an exam? Congratulations to Benat Broderick, PWCF
& CF Ambassador, aged 15, who was
Think about what you achieved, how you felt, both physically and
recently awarded the Pramerica Spirit of
emotionally.
Community Award in the Junior Section.
You achieved that. You, not CF. CF is a part of you, it does not define Benat was honoured for his volunteer
you, so it shouldn't define how you view yourself. work with Cystic Fibrosis Ireland and in
recognition of his efforts to help raise
Remember you don't need a perfect body to have body
awareness of CF.
confidence and a positive body image.
16 Spectrum / Summer 2019Annual Conference
The 2019 CFI Annual Conference and Annual General Meeting took place in the Mount Wolseley Hotel in Tullow, Co.
Carlow (29-31 March).
CFI CEO, Philip Watt, opened the three-day event by drawing conference delegates’ attention to the incredible
evolution in CF care which has occurred since he became CEO in 2009. This has included the development of a
network of specialised CF hospital centres, improved transplant rates, the 2011 introduction of newborn screening
for CF and the approval of a range of innovative and groundbreaking drug therapies which address the root cause of
CF including Kalydeco® (2013), Orkambi® (2017) and Symkevi® (2019).
Dr Laura Kirwan of the CF patient registry outlined the dramatic impact these improvements have had on the quality
of life for people living with CF in Ireland: Mean lung function, BMI and median age of survival have all seen marked
improvement over the last ten years.
Dr Michelle Murray, Consultant Transplant Respiratory Physician at the Mater Misercordiae University Hospital,
highlighted that, as a result of these improvements, many patients are now not requiring transplant until well into
adulthood. She went on to say that transplant is not for everybody, transplant patients are a very unique group of CF
patients and they face their own particular challenges and have their own specialised needs. Ireland carried out its
first single lung transplant in 2005, its first double lung transplant in 2006 and opened its first Adult Lung Transplant
facility in the Mater Hospital in 2011.
PhD researcher, Michael Twomey is a 47 year old person with CF who is developing a patient management system
that enables people with CF to capture their medical or health history accurately between appointments and record
key information communicated by clinicians. He published a Medical Appointment Checklist Booklet in January
2019 which has subsequently been distributed to every CF patient and carer in Ireland and won a Gartner award for
innovation.
Professor Barry Plant, Director of the Adult CF Centre at Cork University Hospital, highlighted the importance of long
term, real world data on Kalydeco® and Orkambi®, in addition to the short-term benefits measured in clinical trials.
These drugs have significant “slow-burn” benefits which have the potential to dramatically alter the course of this
condition.
But then patients with CF (like Bevin Murphy, Jillian McNulty and Caroline Heffernan) and parents of children with CF
(like Bernie Martin, mother of Eva Rose Martin) are well used to taking the long view. Their presentations, covering
the past 10 years, included stories of worsening health as well as the health advances, goals and achievements
that kept them going. Their presentations also included ideas and strategies on how to cope with the added
responsibilities of either having CF or caring for a child with CF which include developing a routine and prioritising,
enlisting the help of others, talking with other individuals and families affected by CF and consulting with the CF care
team.
Nicola Hurley, PhD researcher at Dublin City University (DCU), returned to this theme in her presentation. Evidence
suggests that regular exercise training improves cardiovascular fitness (strength of your heart and lungs), which is
directly associated with an increased lifespan for people with CF.
Bernie Martin, summed up the conference well, in her presentation, when she said that, notwithstanding the great
medical advances, the lives of families affected by CF remains one of ups and downs with many conflicting emotions
and slow incremental progress.
Daniel Costigan, CFI
www.cfireland.ie 17CFI Conference 18 Spectrum / Summer 2019
2019 Gallery www.cfireland.ie 19
ECFS Conference
CFI would like to thank the following for this report and their participation in
European CF Conference in Liverpool (2019): Philip Watt; Daniel Costigan; Carolyn
Cavey; Caroline Heffernan and Nuala McAuley.
Please note that this report is based on our understanding of the research sessions
attended, but are not of course a substitute for reading the original research.
With significant improvements in cystic fibrosis (CF) care and services over the past decade, CF remains one of
medicine’s positive news stories, but there are many outstanding knowledge gaps and unmet clinical needs where
research plays a key role. That was the message of the 42nd European Cystic Fibrosis Society (ECFS) Conference
which took place from June 5-8, 2019 at the Arena and Convention Centre in Liverpool, England.
Over 2,500 doctors, biomedical scientists, healthcare professionals and patient groups from 58 countries were in
attendance.
There was a strong focus on the real lived experiences of patients, which was evident from the opening ceremony
that saw a performance from the talented dancer, Thomas Oakley (who also has CF) and a ‘virtual CF choir’ which
included both a Beatles song and ‘Take me to Church’ by Hozier. (Since 3 of the Beatles have direct Irish connections
and Hozier is from Dun Laoghaire, there was a definite Irish flavour to the opening ceremony!) This was also the
occasion to honour Professor Stuart Elborn from Belfast for his lifelong contribution to CF care and research.
CFTR Modulator Treatments
There was of course also a great deal of discussion at the conference about cystic fibrosis transmembrane
conductance regulator (CFTR) modulators. This spanned from new information on the positive effects of ivacaftor on
GI complications to updates on the new Vertex triple combination drugs.
Professor Barry Plant contended that CFTR drugs have clinical and quality of life benefits beyond lung function. Lung
function is an important (but not the only or best) sign that a CFTR modulator is having a disease modifying effect.
Focusing solely on lung function undervalues and under-predicts the long-term systemic benefits that patients with
CF derive from CFTR drugs.
20 Spectrum / Summer 2019Vertex presented on its VX-445 (elexacaftor) triple regime therapy which it is going to apply for regulatory approval of in the US and Europe over the third and fourth quarters of 2019. This triple combination is made up of VX-445, a CFTR corrector, added to tezacaftor (VX-661) and Kalydeco (ivacaftor), and it is intended for CF patients with two copies of the F508del mutation or one copy of that mutation and another minimal function mutation. Vertex has been looking at the potential of VX-445 and VX-659 triple combinations and has now decided to advance the VX-445 combination regimen for global approvals, based on final positive data from two Phase III trials - A 24 week study and a 4 week study. In the trial of the F508del / minimal function mutation population there were 403 patients (12 and over) who received either the VX-445 triple combination or a placebo during a period of up to 24 weeks. Final trial data showed that patients receiving the combination therapy had a mean improvement in lung function of 14.3 percentage points, from the study start through week 24 and a 63% reduction in the annual rate of pulmonary exacerbations when compared to placebo. Stop Mutations Eloxx Pharmaceuticals presented on novel ways to increase CFTR, not addressed by currently licensed drugs. They are developing drugs to improve ‘read through’ for nonsense or ‘stop mutations’. In these mutations, only part of the CFTR protein is made, as there is a ‘stop reading’ signal in the wrong place on the gene. Enterprise Therapeutics also discussed their programme of drug development which targets alternative proteins in the lungs. The hope would be that their new treatments could work with anyone with CF, irrespective of their genotype. 30th anniversary of CFTR Gene Discovery The CFTR gene was first discovered in 1989, this is the 30 year anniversary and so much has been discovered since. We are now in the era of precision medicine, which was a key focus of a satellite seminar organised by Vertex during the ECFS conference. There was great excitement around at this seminar about the Vertex triple combination drug, as explained by Dr Fred Van Goor of San Diego, USA, which he termed as ‘the next generation CFTR corrector’ and which involves three distinct and complementary mechanisms of action. These are: The Amplifier working at CFTR at ribosomal level, the Corrector working on CFTR mid-cell and the Potentiator improving CFTR channel on the cell membrane. This new triple combination drug therapy combines Tezafactor, Ivafactor and the new molecule VX-445. It appears in trials that PWCF homozygous for F508 del had a decrease of 45.1 mol/L of salt in sweat and an increase of 10% FEV. This drug is designed to assist not only those who are homozygous F508 del but also the heterozygous which have only one copy of F508 del and another gene with a minimum function. Indeed that is good news as still a significant group have not been eligible for modulators. Gene Editing The relatively new concept of gene editing was discussed by Patrick Harrison of University College Cork. He explained simply that all mutations are just 'spelling mistakes'. They require to be cut out of DNA (using CRISPR technique), copy donor sequence, paste and restore function. The method can be used for somatic cells (body), but more effective if in germ cells. There is interest in gene editing for Leukaemia, Huntington's disease and Muscular Dystrophy. The vision by these Scientists is that all three methods - Modulators, Editing and Gene therapy will work synergistically in long term fixing CF - watch this space for future discussion. www.cfireland.ie 21
Gene Therapy
Meanwhile new developments in gene therapy have emerged as presented by Eric Alton, London, UK. We all
thought use of phage (type of virus) as a delivery system to our lungs with CFTR genes was not going to happen, as
many efforts had no success due to the continuous need to replace the phage as expelled by the lungs. However
scientists have merged two types of phage, the Sendai Virus capsule which can attach to respiratory epithelial cell
receptors and the Lentivirus DNA which is lysogenic (will combine with our cell DNA) . This means the gene for CFTR
enters the lung epithelial cells and has a long duration of expression for up to 18 months and longer if it enters
the basal cells. There is a massive interest in this technique of using the qualities of two viruses. The idea is being
explored for delivering correction genes for other genetic diseases too.
Standard “Symptomatic” Treatments
While there was understandably a great deal of excitement around the development of more new groundbreaking
medications which address the underlying causes of CF, there were also many presentations at the conference on
the need for more effective medical therapies to further prevent disease progression.
There were over 700 presentations at the conference. Some of these were intended to increase the depth of
knowledge in areas of CF research while others examined topics where researchers and clinicians know that
more information is needed. These include better treatments to deal with infection, inflammation, irreversible
lung disease and the extra-pulmonary complications of CF. More work will be required to address a host of extra-
pulmonary complications including sinus disease, CF-related diabetes (CFRD), osteoporosis, anxiety and depression
all of which are becoming more and more common and as people with CF live longer and more fulfilled lives.
It is still not clear, for example, what causes people with CF to develop CFRD. At the conference there were
updates from researchers working in this area. A better understanding could lead to better management and even
preventing CFRD from developing at all, if treated early enough.
Additionally, while we know that the lungs of CF patients are affected by thick sticky mucus, chronic infection
and inflammation, we are not sure how all these elements are linked together. During the conference there were
a series of talks looking at mucus, how it is made, how its production is affected by CF and the effects it had on
inflammation. A more detailed understanding of how the formation of mucus goes wrong in the lungs of people
with CF will help researchers to improve lung clearance in the future.
Professor Winstanley of Liverpool University also gave an update on the genetic differences and the evolution of
different strains of Pseudomonas aeruginosa. Understanding how Pseudomonas develops could give doctors the
opportunity to treat the bug earlier, when it is more responsive to antibiotics. Knowing which strain or ‘family’ of
Pseudomonas different people with CF have can also give important information on effectively managing cross-
infection in the clinic.
Poverty, Social Exclusion and CF
David Taylor Robinson of Liverpool University presented on how the socioeconomic status of patients can have a
profound impact on their health status.
His research showed that children with CF from the most disadvantaged areas in the UK have lower weight, height
and body mass index (BMI) in the first years of life after diagnosis and are more likely to have chronic Pseudomonas
aeruginosa infection and lower lung function than children with CF from less disadvantaged areas.
In addition to social determinants of health status, Dr Abi Jackson of the CF Registry of Ireland presented on the
relationship between better health outcomes and health literacy. Health literacy is defined as an individual’s ability
to access, understand and evaluate information about their own health and act on their understanding of health.
Nutrition/Anti-Oxidants
Eat lots of Strawberries! This was the message from Oana Ciofu from the University of Copenhagen. Behind this
22 Spectrum / Summer 2019message is the fact that they are full of Ascorbic Acid a part of the Vitamin C molecule. This wonderful water soluble vitamin is an Anti-Oxidant and so vital for reducing oxidative stress which causes chronic Inflammation and damage to tissues. Anti-Oxidant deficiency is a problem for PWCF especially due to malabsorption of fat soluble vitamins A and E, and Interestingly a low amount of Glutathione. This molecule is so important that it reduces intracellular oxidative stress, especially in the lungs and liver. This can be inhanced by taking N-acetyl-cysteine or NAC supplement which is an oral supplement that is a precursor of glutathione. This would of course need to be discussed with a dietician, but the more natural method is through diet, there is research into whey protein and apparently good old fashioned bone broth (or stock made from bones) is a wonderful source. A good reason to have Granny's Chicken Soup perhaps! Essential Fatty Acids: Brigitte Strandvik of the Karolinska Institut Stockholm, emphasised the need for Linoleic Acid an essential fatty acid in the diet. People without CF may require 1-2% but in PWCF it was 10-15% due to absorption issues. Linoleic Acid is a substrate for Arachidonic Acid and many lipid mediators, it is needed for synthesis of EPA and DHA, needed for metabolic and signalling processes. It is required for the pathways to produce the phospholipids in the cell and mitochondrial membranes which are 3D structures as seen in all Science and Biology books. A deficiency means that cell membrane structure is weakened which naturally affects the tissues in organs such as the Liver and Pancreas, more inflammation in the intestines and also a decrease in energy. In rats, Linoleic Acid has been seen to stimulate insulin and perhaps decrease steatosis in the Liver. So in a practical way, where do we find this Essential Fatty Acid? Rapeseed Oil and Evening primrose Oil for a start! Conclusion: Living with Uncertainty and Retaining Hope for the Future While unanswered questions may provoke curiosity for CF scientists and clinicians, families affected by CF must bear the pain and confusion of not knowing exactly what new treatments are coming down the line for loved ones. But, as we saw with the CF Community Afternoon (where eminent CF researchers gave a recap of the research highlights of the conference to the CF community), the CF community are resilient. They understand the lengthy and often frustrating process of clinical research and they still retain hope for the future of CF research and medicine. This will continue as long as the CF community are kept at the heart of CF research and the potential importance of the unanswered questions highlighted to them. www.cfireland.ie 23
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