SPECTRUM Head Count Record number of participants take part in the annual Head 2 Head Walk - Cystic Fibrosis Ireland
←
→
Page content transcription
If your browser does not render page correctly, please read the page content below
SPECTRUM Autumn / 2018 Head Count Record number of participants take part in the annual Head 2 Head Walk www.cfireland.ie i
CONTENTS
Latest News | 1-3
* Organ Donation Consent
* Drug Therapies Update: Symkevi
* New Antibiotics Available in Ireland CEO’s Message
Philip Watt
Spotlight | 4 -10 We have a wide range of interesting Chief Executive
Education articles in this issue of Spectrum.
* A Father’s Perspective The news that Minister Harris will commence the
* School with CF legislative process to introduce a new form of organ donor
* A Class Act consent (Soft Opt Out) on 1 October 2018 is to be warmly
welcomed.
* Tell me I can’t, I’ll Show you I can
* Roll Call - Back to School Photos Once this has been completed it will be presumed that
most people living in Ireland will want to donate organs but
Hospital Hub |11 - 13 next of kin will still have the right of veto. This will bridge
the gap between intent and practice; 85% of us want to
* My CF Story donate organs, but only one third of us carry an organ
* What a Difference a Decade Makes donor card.
The bottom line is that this system has been shown to
Kids Corner | 14 improve donation throughout Europe and more organs will
mean more have ‘the gift of life’.
Youth Reach | 15 Also in this edition of Spectrum is a focus on ‘back to
school’, the new app developed jointly between Dr. Des
Member Services| 16 Cox in Crumlin and CFI which is proving very popular; a
* Noticeboard nurse’s perspective of CF Care; a focus on research and of
course an update on some of our fundraising events.
Research | 18 - 20 As winter approaches, don’t forget to update your ‘Flu jab’.
* Health Research Board Study Our key objective this term is to ensure the
* Science of CF Survey commencement of the building of the long awaited 20
* Non Tuberculous Mycobacteria rooms in Beaumont Hospital.
* Genetics Based Precision Medicine
* Orkambi in Australia
* Social Policy Update
Philip Watt, CEO, CFI
Fundraising |21 - 29 Sam Byrne & Nuala McAuley, Editors
* Challenges and Events
* Thank You
DISCLAIMER:
The views of contributors, when expressed in this publication, do not necessarily
reflect the position or policy of Cystic Fibrosis Ireland.
ii Spectrum / Autumn 2018
LATEST NEWS: New Organ Donation Consent will improve the chance of a ‘Gift of Life’ As TDs get set to return from the summer recess, and in wake of RTE’s excellent documentary on Orla Tinsley’s transplant, CFI renewed its call on Government for the introduction of presumed consent organ donation legislation – also known as soft opt-out organ donation – to improve transplant rates in Ireland. The call comes against a backdrop of most recent figures showing 600 people waiting for potentially life-changing organ transplants in Ireland in 2017. Ireland lies in 15th place out of 28 European Union countries when it comes to organ donation, despite very high levels of public support. International evidence has confirmed the positive impact of opt-out consent on deceased organ donor rates. Over a three-year period, after making the change to an opt-out system, Belgium saw its rate of organ donation increase by 100 per cent. The recent experience in Wales with soft opt-out has also been very positive. The countries with the best rates of organ donation in Europe have some form of soft opt-out system, combined with sufficient infrastructure. Introducing a soft opt-out organ donor consent system will undoubtedly save more lives and help to ensure our organ donor system is fit for the 21st century. Ireland is one of the last countries in Europe, along with Scotland, Northern Ireland and England, not to have presumed consent when it comes to organ donation. The number of people awaiting life-saving organ transplants in Ireland is more than double the operations carried out. Bringing in this new legislation will also bridge the gap between people’s intentions and their behaviour. 85 per cent of people in Ireland are willing to donate organs but only one third of us carry an organ donor card and many of us do not inform our next of kin of our intentions to donate or otherwise. Organ donation will always be a ‘gift of life’. With this proposed new system, next of kin will continue to be consulted so no family will be forced to donate the organs of a loved one should they not wish to do so. There will also be an online register for opting out of organ donation. We are delighted to let you know that Minister Simon Harris recently tweeted his commitment to implementing new legislation. www.cfireland.ie 1
Drug Therapies Update: News on SYMKEVI® and an important generic anti-biotic drug therapy for CF
SYMKEVI® (tezacaftor/ivacaftor) is likely to be the next drug available in Ireland under the innovative CFTR pipeline
deal signed by the Irish Government and Vertex Pharmaceuticals (an agreement strongly supported by CFI).
SYMKEVI® (tezacaftor/ivacaftor) is a prescription medicine used for the treatment of CF in patients aged 12 years
and older with two copies of the F508del mutation, or at least one mutation responsive to treatment with SYMKEVI®
(tezacaftor/ivacaftor) from the following list:
711+3A →G A455E D579G E193K K1060T R117C S945L
2789+5G →A A1067T D1152H E831X L206W R347H S977F
3272-26A →G D110E D1270N F1052V P67L R352Q
3849+10kbc →T D110H E56K F1074L R74W R1070W
SYMKEVI® (tezacaftor/ivacaftor) was given initial approval by a sub group of the European Medicines Agency (EMA)
in July 2018 and CFI hopes it will be fully approved by the EMA shortly and may be available in Ireland as early as
February 2019 (our estimate, subject to change).
“Tezacaftor/ivacaftor combination therapy for CF is another important achievement in
the development of disease modulating therapies. This combination improves important
clinical outcomes and may benefit those who cannot use ORKAMBI® (lumacaftor/
ivacaftor)” said Professor Stuart Elborn, Director of the Specialist Adult CF Centre at the
Royal Brompton Hospital, London.
It is likely that some patients presently on ORKAMBI® (lumacaftor/ivacaftor) will be
switched to SYMKEVI® (tezacaftor/ivacaftor), but the criteria for switching is not yet clear
and may require written guidance from the National Clinical Programme. This is vital but
may delay the drug by a few weeks so we ask our patients and families to take this into
account when an announcement is made.
Two triple combination therapies, VX-659 plus tezacaftor and ivacaftor and VX-445 plus tezacaftor and ivacaftor,
are both in Phase III clinical trials at present following very promising results from Phase II. The target genotypes
are people with one F508del and one minimal function gene alteration who currently do not have a CFTR precision
medicine. We estimate that 100-150 patients with CF (about 10% of the total Irish CF population) fall into this cohort.
These two therapies are presently outside the pipeline deal but CFI will seek to have them included once the results
of the Phase III trials are verified. Phase II trials showed a fantastic increase in FEV1 of 13 percentage points from
baseline, which would put this drug therapy in the Kalydeco® (ivacaftor) range of effectiveness for that marker,
although it will also be important to use other markers to assess its full impact.
In short, CFI envisages that up to 90% of people with CF will be on one of the above ground-breaking CFTR drugs
in the next few years. We predict that, all going to plan, this will bring a further major boost to life expectancy
and quality of life for people with CF in Ireland. Important as these new and innovative drugs are, CFI reminds our
patients and families that they must continue to adhere to their full range of prescribed therapies. CFI thanks the CF
centres in Ireland, involved in clinical trials and other research related to CFTR drug therapies, for their significant
contribution.
Antibiotics: New Colobreathe Capsule and Tymbrineb available in Ireland
There have also been some important recent developments in relation to antibiotics
for people with CF in Ireland Teva Pharmaceuticals Ireland have launched a new
Colobreathe (colistimethate sodium) capsule which is now available in Ireland. This new
capsule is designed ‘to help minimise capsule fracture on administration when used with
the Turbospin inhaler’.
2 Spectrum / Autumn 2018
CFI have been informed by Teva, that when used with the Turbospin inhaler, the new capsule ‘adds
to Colobreathe’s existing benefits of portability and simplicity of use’. For people with CF, this can
mean less time spent in preparing and administering medication and as with similar time saving
devices and it therefore supports adherence.
A nurse support programme associated with Colobreathe can be accessed by calling 1800 804494
or by emailing colebreathesupport@ashfieldheathcare.com
Teva Pharmaceuticals have launched a new generic medicine called TYMBRINEB (tobramycin) nebuliser solution.
TYMBRINEB is indicated for the long-term management of chronic pulmonary infection due to Pseudomonas
aeruginosa (in patients 6 years and older).
CFI understands that this is a generic form of tobramycin and is an outcome of the patent for tobramycin expiring
in Ireland, thus bringing competition into the market for this important and widely used anti-biotic. CFI wishes to
acknowledge the ongoing role of Novartis in providing tobramycin for two decades and counting and of course for
their vital continuing support of the CF annual medical and scientific meeting in Killarney over many years.
Many thanks to Peter Minchin
Many of you will have gotten to know Peter Minchin over the past four years in his role as
Fundraising Coordinator, he was hugely popular with everyone he worked with. We are
delighted for Peter that he has recently found a new career in An Garda Síochána and as
will soon begin his basic training in Templemore.
Many of you will also know that Peter had a special connection to CF because his brother
Paul Minchin was the first PWCF recipient of a double lung transplant in the Mater Hospital
in 2007. Sadly, Paul passed away in May 2011. We know that Paul’s death had a profound
effect on Peter and was a key factor in his moving from the Bank where he worked to join
CFI in 2014.
I don’t think I have ever attended a larger funeral than that of Paul Minchin in his home
near Bagenalstown in County Carlow, which is an indication of the very high regard there is
for the whole Minchin family.
We will miss Peter and we wish him all the best in his new career, we know he will make an excellent Garda. I will
have particularly fond memories of him from a fundraising walk that Peter helped organise in Flanders, Belgium in
2015 to mark a forthcoming anniversary of Irish soldier’s involvement in the First World War battle of Messines.
While it was was a very poignant and moving couple days it was also very enjoyable because of Peter’s company and
I remember that Peter and our colleague Jolyn carried half the mud from Messines back to Ireland on our shoes - I
don’t think the car hire firm was too amused!
Peter loved helping to organise the Malin 2 Mizen cycle, the Head 2 Head walk and indeed all the fundraisers he was
involved in. Best of luck in your new career Peter, from all of us in CFI.
Good luck Caroline!
CF Advocate Caroline Heffernan will take on her biggest challenge to date
when she takes part in the Barcelona Ironman on October 7th. Comprising
of a 3.8km swim, 180km cycle and a 42.2km run, an Ironman is a huge
challenge for anyone. For a person with cystic fibrosis to take it on is a
major achievement. We are sure you will join us in wishing Caroline the
best of luck in the event. You can follow her progress on Facebook: Ironman
Barcelona Challenge for Cystic Fibrosis Ireland.
www.cfireland.ie 3
SPOTLIGHT:
Education
The recent Independent Living Report produced by Cystic Fibrosis Ireland highlighted that education is still an area
where people with cystic fibrosis (PWCF) face enormous challenges due to their chronic illness. The report showed
that there has been a significant increase in PWCF obtaining third level qualifications. However, cystic fibrosis
continues to cause significant periods of absenteeism from school or college which causes an associated impact
on the ability to perform.
For this Spotlight segment we hear from members of the CF community at different stages of education who tell
us the impact CF has on their education and how they manage study and treatment.
The FIrst Day of School from a Dad’s Perspective.
Our first born, Caoimhe, started ‘big’ school in Gaeilscoil Uí Fhiach on the 29th August.
She looked so young and small in her oversized uniform, but she was beaming from ear
to ear to see all her friends again.
Caoimhe had attended the Naoinra pre-school for two years prior to beginning primary
school and she already knew a number of other children beginning their first year along
with her. This, for us as parents, was a huge relief as my wife Grainne already knew so
many of the parents and they also knew and understood our ‘situation’. Both of our
girls Caoimhe and Fiadh have cystic fibrosis.
Although Caoimhe was excited, I could see that she was also very anxious. This was a huge milestone in her life and
also a big change for her. As a father letting go of my baby girl’s hand and watching her walk into the classroom and
sit on her tiny chair was very overwhelming especially as we have the added stress of her condition but she took to
it like a pro. Caoimhe has been assigned a special needs assistant (SNA) to make sure she eats her lunch, takes her
creon, washes her hands etc. Although Caoimhe isn’t aware that she has someone watching over her it gives us some
sense of reassurance when she isn’t with us.
I know as a family we will face so many challenges with our two girls but as school is going to be a major part of their
lives we will strive to keep everything as normal as possible for them.
I am generally a very positive person and I am very optimistic for the future of our kids health and education.
Caoimhín Ó Lúing
4 Spectrum / Autumn 2018
School with CF
The return to school can be difficult for everyone, especially those with
Cystic Fibrosis. For these people, this means the return of waking up early to
do physiotherapy, nebulisers and having to take all sorts of medications. And
this is all before they leave to face a tough day of school ahead.
My own experience as a person with Cystic Fibrosis in school wasn’t the
best. I started school at the age of 5 and a half because I wasn’t able to eat
solid foods then, I was peg fed until this age. Once I started eating solid
foods, I began to take creon. As I was only around 6, I needed help with this.
I had an SNA in school which I found very helpful and beneficial for me.
My typical morning would consist of waking up at 7. I would take all my tablets and then I’d head downstairs to
do my nebulisers and my physio. I used to use the vest which was actually nice, kind of like a little massage before
school! I would then get out all of my clearance and head off to school. I did this routine again that evening which
was difficult when you’re only a child.
At times, it was quite hard to balance my school life and my medical life,
but I managed it with the help of my friends. For me, the fact my friends
knew about CF made it easier to manage in school. If I was ever on IV
antibiotics, they would help me carry my books and bag and if I was out
sick or in hospital they would help me catch up with everything I had
missed which made me feel included and a part of school life even when I
couldn’t be there.
The transition from primary to secondary school was good for me. My big
sister Lauren helped me settle in. First year was a good enough year for me
health wise. Of course, I got a few infections and was admitted to hospital
a few times over the year but overall, I was well. I started the drug Orkambi
that year but unfortunately it didn’t work for me.
Second year wasn’t as good for me. By this time, I was on the transplant list in Newcastle. I got too sick to return to
school after Christmas in second year. I had to stay at home on permanent oxygen. I started home schooling with four
brilliant teachers in March. I really enjoyed it, but of course I missed my classmates and especially my friends.
In the summer of 2017 I moved to Newcastle to wait for my transplant which I thankfully received on the 11th of
August 2017. I focused on my recovery for the next few months and I moved back to Ireland at the end of September.
As I was still recovering I wasn’t able to go back to school yet. I continued with my home schooling until November
and then went back to school. I was delighted to be back in school.
I’m someone who really enjoys school and I missed it a lot.
I was now in Third year which was an important enough year. As I had missed so
much school, I only did six subjects which was much easier to manage than 11.
My principal was very kind to me and he helped with my return in any was he
could. I did the Junior Certificate this summer and I got all honours in my subjects.
I started Fifth year in September which I’m excited about.
My life in school after my transplant is so much different to before. I now wake up
at 7:30 and I eat breakfast with my family every morning which I never had time
for before. I take my anti-rejection tablets every day at 9:30 to make sure I stay
feeling this good and that I’m able to continue working hard in school.
For now, I’m looking forward to the school year ahead!
Siobhan Murrary
www.cfireland.ie 5
A Class Act
Hi, my Name is Laura Carrick and l have Cystic fibrosis, l am 16 years old and in
5th year in secondary school.
When l was in primary school, to help my teacher and class understand my
illness l showed them a short cartoon video about CF and l answered any
questions that they had. My Mam also sent the school a letter written with the
help of the hospital, as far as l remember explaining my illness, the importance
of washing hands and emphasising the importance that if any of my classmates
were sick or had a cold that they should not be around me as it could cause me
to be very ill. I always remember my Mam would stick the Creon to the lid of
the lunch box with a note and a smiley face to remind me to take it.
By the time l moved to my new school to start secondary, most of the girls knew l had CF. l informed the new school
again of my illness and made sure no one else had CF in my class.
My teacher and the receptionists knew, but l did not tell the whole class as l felt differently about CF now that l
was older. I did not feel l wanted everyone to know yet as l had enough to cope with, with my transition to the new
school so I only told my close friends, although most people from my old school were in my new school and knew
anyway.
It was a lot tougher as l have to get up a 6.30 to do my meds before school as my school starts at 8.35am so it’s a
challenge sometimes.
l do get tired and the term goes on and at one point l was missing a lot of school as l was sick, but my mom got me
a home tutoring for a bit so l could catch up with my classes which helped me over that bad period as my Junior
Cert was coming up at the time. l do find that if the teacher understands why I’m out they don’t pressure me and
my Mam and Dad don’t either, but they do want me to do well in exams so l do try my best, but it’s not the most
important thing to me. My Mam always says your health is more important so l now have to agree; if lm not well l
will miss school and then my school work will suffer so l try to stay well so l won’t miss school.
My school has been very good to me with regard to my CF. If l am late, I get excused so l won’t end up with a million
pink slips and detention. They allow me to drink in class and eat and l can go to the bathroom whenever l have to. I
always have a hand sanitiser with me and l leave a spare bottle of Creon in my locker in case l forget it in my school
bag. I am lucky that if l do forget anything, l am not too far from home and I can get my Mam to drop it up to me.
As I said before l did not want everyone to know about my illness but there came a point that l felt l needed my class
to know as not everyone understood what l had and at one stage l felt girls avoided getting to know me because
l had CF. In English, l had to do a presentation so l decided to do it on CF to explain why l coughed, why l drank a
lot of water and why l was out a lot. Some girls thought l was making up excuses sometimes so l felt l needed to
explain myself, but to be honest if you don’t have CF how could you possibly understand how l feel? Anyway l did the
presentation and it went really well. l was feeling so much happier and some girls in my class were shocked that l had
CF. l actually made some friends so l think it was a good move to be open about CF and l am happier in school in now.
Since they know now, l decided to fundraise for CF and l brought in the roses on 65 roses day to sell to my friends and
classmates in school and l was allowed to sell at the school door in the morning before classes. School is a challenge
6 Spectrum / Autumn 2018
but my Mam and Dad always wanted me to have a good experience and l did everything all the other kids did, of
course sometime I have missed school trips to places because I am feeling unwell or cannot do it because of my CF
but for the most part I am like any other kid in school. l have gone on trips with the school which l loved even if l had
to get up earlier than everyone else to do my airway clearance it was definitely worth it.
Some advice I would give you is to approach the teacher and ask if you could be in a room with your BFF. I know
that on some of my trips there can be up to six girls in a room but still having my closest friend in the room was
great as she understood much better about my medications. Also tell the teacher (if they don’t already know) that
you have cystic fibrosis and explain the medications you have to do, so if you’re not feeling well on the trip they will
understand.
When it’s exam time it can be exhausting because you get a lot of homework and you also have to study for the
exams, so try to get into school as much as possible during that time because it’s extra important. A tip I would give
you is to talk to your exam coordinator and ask them if you can take the exam in a separate exam centre. This way I
feel more comfortable coughing and also I am allowed to bring in a bottle of water. Also, if I get dizzy or feel sick and
have to leave, I won’t disturb everyone by leaving the exam early.
l love photography and watching YouTube, so this summer I was lucky enough
to have the opportunity to meet my favourite YouTuber Alisha Marie with Make
A Wish. Make A Wish is an amazing organisation that flew me, my sister Louise
and my Mam and Dad to California. We had the most amazing time there, and I
would love you go back someday. They got us creator passes to Vidcon so I got
to see more of my favourite YouTubers there. It was an experience I will never
forget. I took so many photos over there and got to go to some places that I
had seen my favourite YouTubers go.
Last year in my school I applied to be a mentor to the first years which is
basically where we get a small group of first years usually 3 or 4 and we
welcome them to the school on their first day and give them a tour and make
sure they are getting on ok in the school, making friends and socialising with
the other girls in their year. So I applied and then we found out this year who
got the places. I was so happy that I was chosen to be a mentor.
I have a couple of ideas of what I want to do in the future. I am thinking
about photography (possibly at DIT). I would also love to start my own
YouTube channel and see where that takes me, but I have also always loved
fashion so maybe I’ll go into some aspect of fashion I particularly love fashion
styling. As you can see I have lots of ideas and options for my future but I’m
only in 5th year so a lot can change before I leave school. Who knows maybe
I’ll find something else I love.
Tell Me I Can’t, I’ll Show You I Can
My Name is Sarah, I am 23 years old. I was diagnosed with Cystic Fibrosis when I was nine
months old. I have one brother and two sisters but I am the only one that has CF. When my
parents discovered I had cystic fibrosis they worked really hard to understand the condition
and to keep me as healthy as they possibly could. My Mam and Dad came up with fun ways
to do my physio, like buying me a mini trampoline and jumping to music every morning and
evening (Rotterdam - Beautiful South and Spice girls of course were my favourite trampoline
songs). They got my older brother involved in the games too, this way I didn’t feel like
anything was different. My Parents never wrapped me in cotton wool and I got to experience
all the different milestones of my life, first day of school, first day of secondary school and
first teenie bopper disco just like everybody else. I am very close to all of my family and in
some ways me having CF brings us all closer together.
www.cfireland.ie 7
When I was young I didn’t have many hospital admissions. I was a very active sporty young girl, I was part of school
football and camogie teams, I even played for County Galway ladies football. Then at the age of 17 I was offered
an amazing opportunity from a really inspirational man called Ian McKeever (RIP), to climb Mount Kilimanjaro, the
highest Mountain in Africa, 5,895 meters! Without hesitation I accepted, not once did I believe I couldn’t do it! So
in 2017 I climbed Kilimanjaro and raised money for CF, Chernobyl children and Crumlin hospital. But believe me
when I say it took a lot of training! I climbed all the mountains in Ireland with my Dad and some more than once!
It was an incredible journey and one I will never forget! There were a lot of preparations to be done with vaccines
and medications which took a long time to get sorted but with the help of my pharmacy and CF team in Galway it
wasn’t a huge problem. This really was a huge achievement for me and for my family to see that I wasn’t letting CF or
anyone get in my way of achieving my life goals.
When I turned 18, I had a few problems and was
hospitalised a couple of times. During my Leaving
Cert year I got sick and did a couple of weeks study in
hospital and at home. I even left hospital for a few hours
to sit my Irish Oral exam with cannulas in both hands!
Nevertheless I was determined to get my leaving cert
done that year! I completed my leaving certificate in
2013 and secured a place in University College Dublin,
to study Agricultural Science/ Animal Science.
I started College in September 2013, I lived on campus for the first two years and then in a house with my friends
for the final two years. I used to use public transport, the train to travel up and down from Dublin to Galway, but
it wasn’t exactly ideal at winter time or high flu seasons and it was also difficult for me to bring suitcases full of
medication with me, because of this I was determined to get my driving licence. So I started driving lessons and
within a few weeks completed and passed my driving test and bought a car! This also came in handy when it came to
college.
As part of my Degree I had to complete 6 months farm placement
in a sheep, dairy, swine, beef and poultry enterprise. I chose
to travel to Northumberland UK, for my sheep placement,
I spent four weeks there in the Cheviot Hills with 800 ewes
during lambing season! I went home between this and my next
placement as I got an exacerbation and needed two weeks IVs.
After this I celebrated my 21st Birthday and the day after travelled
to Brandenburg, Germany and worked for four weeks in a large
scale swine farm with approximately 60,000 pigs and then four
weeks more on a large scale dairy farm in the same area in
Germany. Finally, I came home and worked on a poultry farm and
beef farm. I also did a DIY artificial insemination course.
This was a really big step in my life as I was working and managing all my CF routines by myself hundreds of miles
away from home! I think this was the moment I really started to feel like an adult! No, who am I kidding I’m always
going to be a big child! In my final year I got a really bad infection and was out of college for approximately six weeks,
this was really tough for me to try catch up! I was lucky to have a good relationship with my lecturers in UCD and
friends and they helped me a lot by sending me notes and assignments and giving me extensions on projects!
When I started college I was really insecure about my cystic fibrosis and didn’t want anybody to know about it in
case I would be treated differently. I built up the courage to tell lecturers just because I had to because I was missing
some lab practicals or lectures and needed a little more time or help with certain things, but it took me until my
final year to tell many friends and colleagues about my cystic fibrosis and I was absolutely honoured when my year
representative asked me if we could choose cystic fibrosis as the chosen charity that our year would raise money for!
Some of my classmates even came to the Dáil with me to protest for Orkambi which was very special!
8 Spectrum / Autumn 2018And after all that it really wasn’t that big of a deal after all so I would highly recommend anyone going to college to make sure you make good relationships with lecturers and friends but also make sure the college and lecturers know your circumstances as it’s easier for them to understand why you may miss college and they can help you! But most importantly don’t miss out on the college life, social nights out and college activities. I found these were really important to keep your head in a good place and I won’t lie they are a lot of fun! In 2017, after four very quick years, I graduated from Agricultural Science- Animal Science in UCD with a 2.1 degree. After this I decided that although I really loved the degree I received, I wasn’t fully satisfied and decided to continue my studies to get my dream job and I am currently studying in my 3rd year out of 5.5 years to be a Doctor of Veterinary Medicine. I really love the course and I am extremely excited to achieve my childhood dream of being an “Animal Doctor”. I have also been taking Orkambi for just over a year now and have not been hospitalised once since I started! It was tough on my body to begin with but with the help and support of my excellent cystic fibrosis team in Galway Dr. O’Mahony, Orela, Irene, Mary and Fiona, it made things somewhat easier to adjust and I haven’t looked back since. It seems to be working very well for me; I’m finding it easier to stay healthy, I have better energy levels and I seem to be able to keep a stable weight, which I found difficult to do before! So I would say it has changed my life in a very positive way! Outside studying I also like to live an adventurous and interesting life! I love taking part in charity events: I have run the VHI Mini Marathon twice with my Mam and done many 30km charity cycles with her! I love to go out with my brother, sister and her partner on nights out. I love to travel and have visited many countries and unique places. I have met a really understanding and supportive boyfriend, who also studies veterinary medicine so we have a lot in common and together we travel, hike, run, cycle, study, cook and just enjoy every day we have in this amazing world. I find it really difficult to tell people about my cystic fibrosis (well especially people I really like), I am afraid of what they will think or say or if they will treat me differently. I told my boyfriend early on in our relationship about my cystic fibrosis, he had never heard of the condition and was curious, I gave him a few days to digest what I had told him and when we met again he was very understanding and had even researched about cystic fibrosis! This meant a lot to me and I knew he was going to be a very special person in my life! This summer we took part in a pilgrimage with his family and walked 288km from Hungary through Slovakia to Krakow in Poland over eight days, walking between 28 and 35km each day! It was tough trying to make sure I kept up with all my treatments and ate enough food for the amount of energy I was burning, but with the help of my boyfriend we managed very well. Also over the summer we helped out in his father’s veterinary clinic and gained a lot of valuable knowledge and experience. Finally, I would just like to say, my motto in life is “Tell Me I Can’t, I’ll Show You I Can”, this motto has given me a lot of strength and determination to carry on and do everything I want to do or at least try my best and I’m happy! Everyone with Cystic Fibrosis is different and I think we are all strong in our own ways! Two pieces of invaluable advice from two very wise people: Daddy said to me “Every day your biggest achievement is waking up breathing and telling yourself today is going to be absolutely fabulous!” and Mammy’s “It’s not the end of the world, don’t worry about it, there’s always tomorrow”. www.cfireland.ie 9
Back to School:
Roll Call for the CF Community
Hands up who is excited to start Adventure Awaits! Model Student Time for TY
school?
Ciaran Kimurray, aged 3 Jayden Chambers, aged 5 Mark Kerins on his first day of
Adam Munnelly couldn’t wait for all set for his first day of ready for his first day in junior Transition Year. Mark’s two older
his first day in National School! Montessori. infants. bothers also have CF and are
currently in college.
Brothers in arms! Moving on up! Big brother is watching!
Senan McCabe, PWCF, aged 4 was excited for Mason Merrigan, aged 6 on his first day Ruth Forster, PWCF, aged 4 was happy to
his first day of Junior Infants. Big brother Rian of senior infants. be joining big brother Tom in school and
was on hand to help him settle in at school. was looking forward to her first day of
junior infants.
10 Spectrum / Autumn 2018HOSPITAL HUB
My CF Story
A s part of a quality improvement project at Our Lady’s Children’s Hospital Crumlin (OLCHC) in 2016, our CF team
created a patient handheld record for families and their children with cystic fibrosis (CF). These records have
been shown to encourage and improve self-management in people with chronic illnesses. Many parents of children
with CF come to clinic with their own diary or record of their child’s CF treatments. We wanted to develop a record
that would provide families with a single place to store their clinical data and log questions about CF.
We initially piloted a paper handheld record with a number of families of younger children with CF. The pilot
was a great success and every parent felt that the handheld record was user friendly and that it would improve
communication between them and the CF team. Many parents switched over to using the handheld record instead
of their own diaries. Many also requested that an electronic version be developed. Our pilot study demonstrated
that there was a real demand from families for this valuable tool. CF Ireland kindly agreed to fund the project so we
set about developing an app to incorporate the requests and feedback from parents and staff collected during the
pilot study.
The app provides children and their
parents with their own personal
healthcare record which contains
information about their CF. The
app has been designed to allow
children and their parents to keep
together all the information that
they may need to help manage their
CF care. The app has three different
formats depending on the child’s
age, namely 0-5, 6-11 and 12-18
years, and adjusts the phrasing
and content to suit the child’s age.
As the app was developed by the
OLCHC CF unit, much of the content
directly relates to how children with
CF are managed at OLCHC.
We envisage that parents and older children with smart phones will open the app while at CF clinic to access any
clinical information or questions which have been logged over the past few months. We think of the app like a
storybook of lots of important information about CF that families can access whenever they need and this is why
we called the app “My CF Story”. We hope that families will find this app empowering and that it will become an
integral tool in managing their child’s CF. If the app proves successful, we would like to roll out a national version
incorporating the needs of parents and children with CF across the country.
We would like to give a special thanks to all the families who contributed their time to the pilot study and evolution
of the app. We must also recognise the contribution of all the staff at the OLCHC CF unit who assembled the
main structure and content on the app. Finally, a big thanks must go to CF Ireland who generously funded the
development of this app and it would not have been possible to bring this project to fruition without their help and
support.
Dr. Des Cox, Consultant in Paediatric Respiratory Medicine, Our Lady’s Children’s Hospital, Crumlin
www.cfireland.ie 11HOSPITAL HUB
What a difference a decade makes!
Geraldine Connell CF Clinical Nurse Specialist at the National children’s Hospital, Tallaght University Hospital
O ver the centuries cystic fibrosis (CF) had many aliases before becoming a recognised
condition – mucoviscidosis or fibrocystic disease of the pancreas were the main
contenders! Indeed for many decades CF had been categorised as a childhood condition
and yet today the number of adults with CF outweighs the number of children with this
condition. The latest available CF Ireland Registry statistics show that in 2016 there were
701 adults compared to 565 children with CF on the register – with well established adult
CF centres continuing to provide care. The potential implications facing a parent when their
child has been diagnosed with CF are life-changing. While this is true, the progress in CF
care and treatments now in comparison to when the CF gene was first discovered in 1989 is
remarkable.
I have worked as a CF Clinical Nurse Specialist in paediatrics in the National Children’s Hospital for well over a decade
and in this time have observed many changes in CF treatment and their outcomes. There has never been more
hope of major progress in CF care than the present. I have been lucky enough to work alongside the first CF nurse in
Ireland, Gerardine Leen, who has a wealth of experience in CF (almost 3 decades!). As Clinical Nurse Specialists we
play a pivotal role that is central to the care and management of CF. We are the first point of contact for patients,
staff and our community colleagues. With the advancements in CF care our role has expanded significantly.
In 2005, the Pollock Report identified CF service needs and provided a benchmark
for improved quality and access to care. The requirement of designated CF staff, CF
centres and facilities was asserted. Now in 2018 there is a new paediatric hospital
on the horizon hoping to provide the centralisation and alignment of services.
Since 2014, the availability of International Standards of Care (European Consensus
Guidelines) has provided centres with guidelines to deliver the highest quality
of care to our patients. The establishment of National Clinical Programme for CF
(NCPCF) in 2016 (led by Prof. C. Gallagher St. Vincent’s University HospitalUH – G.
Leen Nurse Rep) will provide a national framework which will ensure people with CF
(pwcf) receive optimal care to preserve and enhance their quality of life, improving
health outcomes and well-being.
There has been a tremendous deal of change in the past decade for pwcf.
In more recent years emphasis has been placed on strict infection control
and rigorous segregation policies. Gone are the crowded open wards,
waiting rooms and holiday camps! In its place are single en-suite isolation
rooms, emphasis on handwashing, infection control, strict patient
segregation and masks! Astonishingly in 2006, 23% of paediatric patients
were chronically colonised with Pseudomonas aeruginosa in comparison
to 8% in 2016 which speaks volumes in terms of management impact.
Since 2000, The National CF Reference laboratory at Tallaght Hospital
provided a national service for the early identification and susceptibility
of CF pathogens. Early identification of CF pathogens and early treatment
and eradication is paramount for the continued wellbeing of pwcf.
12 Spectrum / Autumn 2018Although having CF requires lifelong treatment to maintain health, recent advancements in medications have simplified many treatments. With the advent of inhaled antibiotics v’s nebulised e.g. Tobipod and Colobreathe, the treatment burden and patient compliance has improved. We now not only have mucolytics such as pulmozyme to offer, but hydrators such as hypertonic saline and mannitol are available to restore CF airway surface liquid (ASL). We have seen a reduction in the need for IV antibiotics and less use of our home IV service which can only be a positive sign! The CF registry reflects this with 20% of paediatric patients having received IV antibiotics in 2016 in contrast with 33% in 2006! Since the establishment of the National New Born Screening programme in July 2011 managing the care of an infant with CF from three weeks of age is now the ‘norm’. We are typically seeing ‘well’ babies from diagnosis rather than symptomatic children. Early diagnosis permits prompt expert education and an opportunity to maximise nutrition, implement excellent infection control measures and allows optimal treatment of chest infections. Ultimately our aim is to empower parents from the very beginning with the skills and knowledge to manage their child’s condition – in turn empowering the patient to manage their own health care needs in the future. Measures such as physiotherapy have also evolved over the years – with emphasis placed on fitness and body conditioning day to day and active chest physio when respiratory symptoms arise. Patients are taught from an early age to manage their condition with the aim of promoting independence and confidence. Cystic Fibrosis can now be described as a life-long versus a life-limiting condition. The prognosis for pwcf continues to improve and the prospect of specific gene treatment is now a reality. When the CF gene was identified back in 1989 a real opportunity to treat the underlying cause of CF arose. Many years later and with numerous clinical trials this has now become a reality! After a long fight led by the CF community, funding for Kalydeco and Orkambi was secure, with other CFTR modulators in the pipeline. These have had a dramatic effect on the nature of the condition, in some cases normalising sweat tests! It cannot be over emphasised the clinical benefits of these modulators for pwcf. We are only at the beginning of this journey and as more and more mutation specific treatments become available the nature of CF will change. Maybe in time we may start CFTR modulator therapies from diagnosis once longer term safety and efficacy data has been established. Social media has provided a platform for families and pwcf to communicate and share knowledge – almost like the ‘waiting room’ conversations in the years before. Information and discussions are now available at our fingertips. The CF community is well established and continues to evolve and our role as professionals will continue to expand and adapt to medical advancements in the management of our patients. As we enter the next decade isn’t it inspiring to look back on the last? And who knows what the future has in store ……. www.cfireland.ie 13
KIDS CORNER
C ystic Fibrosis is a genetic condition. Genetics are what make
us who we are.
We inherit our genes from our parents, you might have the same
hair colour as your Dad or the same eye colour as your Mam.
Genetics link a family so you could be tall like your cousin or
have the same smile as your Aunt.
Here are some things that you may share with your parents,
siblings, cousins, grandparents, aunts and uncles:
You Mam Dad Sibling
Hair Colour
Eye Colour SPOT THE DIFFERENCE
There are seven differences in the
Freckles
Halloween pictures above and
Dimples below. Can you find them all?
Smile
Rolling your Tongue
The hand your write with
Cystic Fibrosis
While genes are what link us they are also what make us unique.
CF can sometimes make us feel different to everyone else, but
it is important to remember that cystic fibrosis is just one part
of you, and helps to make you unique, but it is not who you are.
Being the best is great, you’re the number one.
Being unique is greater, you’re the only one.
Congratulations to PWCF
Jesscia Cassidy, aged 8, who
MAZE SOLUTION
Summer 2018
recently performed with her
dance troupe and Riverdance
for the Pope in Croke Park.
Jessica is pictured left 4th
from the front!
14 Spectrum / Autumn 2018YOUTH REACH
E xercise is of extreme importance for people with cystic fibrosis (PWCF) as it is a natural form of chest physiotherapy
that increases lung capacity and facilitates airway clearance. Not only is exercise good for the body, but it is also a
great way to improve your mental health. PWCF Niamh Doyle recently won the Gold Medal for the vault in the Gymnastics
Ireland Super Series Competition and took time out to speak to Cystic Fibrosis Ireland on the role exercise plays in her life.
I was diagnosed with CF at 13 weeks so I don’t remember much of those early years
but I do remember as I got older my treatments hospital visits and bouts of sickness
became a normal part of my life. My parents tell me I was a very active child over
active in fact and as soon as I was old enough they got me involved in sport.
I have been doing gymnastics for the past almost 7 years with Portlaoise gymnastics
club. It is something I feel has been a huge part in keeping me well. In the early days
when I was a junior gymnast we did a lot of floor tumbling and trampoline work and
as I have gotten older and I’m doing more difficult things I feel I am being challenged
physically. I compete on four pieces of apparatus, floor forward beam and bars. The
vault is my favourite and I think the one I am best at. What I love about gymnastics
and my club is that my coaches are very understanding, they push me as much as I
can be pushed but if I am feeling sick or tired they will let me sit out, have a drink and
then let me come back in when I am ready. Sometimes I get out of breath or if I am
getting sick I can be hot and tired, but I always try and finish the session even if I just
sit and do some stretches. At the time it feels tough but then I go home and have a
good rest and a lot of the time I feel the better of it .I am lucky that I have not been
so sick that I could not commit myself to a sport like gymnastics, I make 70% of my
training sessions, sometimes I am just too tired and other times I am unwell. I do
not give up because I love the sport. I am strong and healthy as a result of doing
it and I also love the social aspect we compete individually and as part of a team.
Gymnastics can be physically demanding for flexibility and stamina. I feel doing the
sport has given me great strength and muscle strength which has helped to keep me
well.
I also play rugby and again I have been very lucky to have extremely supportive
coaches who do the same and mind me particularly if the weather is bad they will
text my mum and say don’t bring Niamh to training tonight the weather is too bad!
This means a lot because it shows that they care and are trying to help keep me well
enough to play my matches which is very important to me because I love match day.
I feel sometimes parents of kids with any illness are afraid to push them or allow them to challenge themselves
and sometimes unfortunately if the child is sick enough the parents are right to do this but if it is possible all
children especially those with lung related illness should do sports: swimming, gymnastics or whatever it is the
child likes because from experience I can definitely say that sport and exercise in general play a huge role in
keeping me well.
Niamh Doyle
SOLUTION
Summer
LADDER
WORLD
2018
www.cfireland.ie 15MEMBER SERVICES
Noticeboard
Schools and Cystic Fibrosis Booklet
Back to School Offer!
Get your copy of CF Ireland’s latest publication “School and Cystic
Fibrosis”.
This booklet is a useful resource for schools, teachers and parents
whether your child is starting school, changing schools or has a new
teacher. While recognising that every child with CF is different, the
booklet provides information about varying aspects of CF and its
implications at school.
Order your copy by phoning 01 496 2433 or emailing Nuala at
nmcauley@cfireland.ie. Booklets can be sent to parents or directly to the
school and are also available to download from our website
https://www.cfireland.ie/cfi-services/publications/item/387-school-and-
cf
Did you know?
From the 1st September 2018 all persons in receipt of
Carer’s Allowance or Carer’s Benefit are able to attend a “We’ll be our own lifesavers.
GP free of charge. Registration is now open and applies
to carers who are currently in receipt of either full or half
We’ll get the flu vaccine.”
rate Carer’s Allowance or Carer’s Benefit, and who do not
currently hold a Medical or GP Visit card.
All carers who already hold
active Medical Card or GP
Visit Card eligibility from a
previous application retain
this eligibility. There is no
requirement for them to also
register for eligibility on the
GP Visit Card for the Carers scheme.
Further information on the scheme is available on www.
The flu vaccine is a lifesaver for older people
medicalcard.ie or by calling 1890 282 919. and those with long-term health conditions.
To register: https://www.hse.ie/eng/cards-schemes/ CONTACT YOUR GP OR PHARMACY TODAY TO GET THE FLU VACCINE.
gp-visit-cards/gp-visit-card-for-carers/gp-visit-card-for-
carers-english-registration-form.pdf www.hse.ie/flu
Order code HNI00717
16 Spectrum / Autumn 2018My Legacy Week
October 29 th - November 3 rd
M y Legacy Week is as good a time as any to make your Will and consider leaving a legacy gift to a cause you
care about such as Cystic Fibrosis Ireland.
Having a Will is very important for so many good reasons and particularly during My Legacy Week, when you are
encouraged to take that first step in writing your Will by making an appointment with a solicitor. Cystic Fibrosis
Ireland are a member of MyLegacy.ie who have the support of hundreds of solicitor firms all around the country
who can offer expert guidance and advice about making a Will at any time of the year.
It is usually a much more straightforward and cost effective process than you might think and your solicitor will
discuss any fee for drawing up this important personal document when you make your appointment.
Once family and friends have been looked after and all other important personal decisions have been made,
deciding to leave a legacy gift to a charity is a wonderful way to support a favourite cause such as Cystic Fibrosis
Ireland in the future. Large or small, every legacy is a generous gift of hope and trust for the future.
We understand that leaving a legacy is a very private decision. Some people choose to let us know that Cystic
Fibrosis Ireland has been remembered in their Will. Others prefer to keep their desires private, leaving it to their
executor or solicitor to let us know when the time comes.
1. Make An Appointment: Avail of expert advice and support.
Take the first step to discuss your wishes and decisions for the future.
2. Look After Loved Ones First: Why a Will is important.
A Will provides for loved ones, assigns guardians, protects your assets and helps reduce inheritance tax.
3. Consider A Legacy To Charity: Your gift may be big or small and is tax free.
If you have a cause close to your heart such as Cystic Fibrosis Ireland, please consider leaving a gift to that
charity in your Will.
Many people can afford to give far more in their Will than they ever could when they are alive. It won’t cost
anything in your lifetime, but will ensure your generosity. Your legacy will live on to support future generations,
and help us to make long-term plans in support of CF care throughout Ireland.
By leaving a gift to Cystic Fibrosis Ireland you help us to;
• Fund pioneering research that aims to improve current methods of treatment and management of the
condition as well as enhance the quality of life of those living with the condition
• Fund specialist cystic fibrosis multidisciplinary posts in hospitals throughout Ireland
• Provide funding for cystic fibrosis units around the country, including dedicated in-patient, day care and
out-patient facilities
• Help people with CF directly with Exercise, Transplant, Fertility Counselling and Bereavement Grants
• Provide advice, information and advocacy services to help people with cystic fibrosis
For more information on leaving a gift in your Will, please see the enclosed leaflet “Your Legacy Could By Life
Changing”, visit our website at www.cfireland.ie or the My Legacy website www.mylegacy.ie. Alternatively you
can contact Rachel on 01 496 2433/ rbyrne@cfireland.ie for more information.
www.cfireland.ie 17RESEARCH
Health Research Board funded study called ‘Patients’ Preferences for Health’ are looking for volunteers
In general, people have preferences of being in one state of health over another. This can often be a preference to
have good health over bad, or to be in good health for so long and to be in less well health for a shorter period of
time. By asking the public in Ireland to make these decisions or trade offs we can say something about the general
preferences of people in Ireland.
Preferences gathered in this way are often used to guide choices around decisions in relation to health and using
preferences from the general public is deemed to be a fair way of deciding on how to assign healthcare resources.
However, it is likely that a person’s experience of being in a health state will alter their preferences compared against
those who are only imagining being in that state.
As such, this work will examine the differences between patients with CF, their valuations of health states and those
of the general population to see if this is actually the case.
If you are interested in helping with this study, please contact Daniel Costigan at dcostigan@cfireland.ie
Cystic Fibrosis Ireland Invites Your Participation in the “Science of CF” Survey
Cystic Fibrosis (CF), is a complex and multi-organ condition which primarily
affects the respiratory and digestive systems. Other medical issues include, CF-
related diabetes, malnutrition, liver diseases and subfertility issues, particularly
in males, as well as chronic lung infection. Because of this, health literacy is
extremely important for people with CF, their family and friends. People with CF
want to learn more about their condition and are actively seeking information
on new treatments in development which may ultimately and significantly
improve their health outcomes.
The challenge for healthcare professionals and scientists is finding out
what people with CF want to know about their condition and how best to
communicate that information in a manner that is both accessible and reliable.
In an effort to address that challenge, the Northern Ireland Microbiology
Discussion Group (NIMDG) (not-for-profit) has launched a survey. The survey
consists of 40 questions covering everything from your cross-infection concerns
to what you consider to be your most reliable sources of CF information.
CFI invites your participation in this survey which has already taken off with over 200 completed surveys worldwide
from Ireland, Northern Ireland, South Africa, Brazil, Israel, Canada and the USA.
To take part in the survey go to http://www.surveygizmo.com/s3/4149836/Scientific-Communication-in-CF
The survey is voluntary and completely anonymous and takes only 15-20 minutes to complete. It will run to the end
of 2018 and the outcomes of survey will be available at https://cfscicomms.weebly.com/
Note: NIMDG has a strong base within CF and over the past two decades, several healthcare professional members
of the Group have developed important links with the CF community through research and healthcare delivery.
The survey has been developed in conjunction with Cystic Fibrosis Australia, Cystic Fibrosis Canada/Fibrose
Kystique Canada, Cystic Fibrosis Foundation (USA), Cystic Fibrosis Ireland and the Cystic Fibrosis Trust (UK).
18 Spectrum / Autumn 2018Study Points to Importance of Early Diagnosis and Treatment of Non-Tuberculous Mycobacteria (NTM) Infections in People with CF NTM are a group of bacteria that live in soil, swamps and water sources and are “cousins” to the bacteria that cause tuberculosis (TB). They are very hardy bacteria that are problematic to treat, resistant to many antibiotics and all around associated with poorer clinical outcomes for a CF patient who acquires such an infection. There has been an increasing incidence of such infections in CF patients, with recent studies reporting a prevalence of 32.7%. A new study has also suggested that earlier diagnosis and treatment of NTM infections in patients with CF could positively affect a patient’s lung function. The study started off with 360 CF patients, 30 of whom were identified as having an NTM infection. 28 of these were further analysed and compared with 26 matched CF patients not infected with NTM. Within the NTM group, 17 patients were infected with a class of NTM known to be slow-growing, eight were infected with rapidly growing NTM and three were positive for both types. When lung function was assessed in the two groups, researchers observed that lung function (FEV1) was significantly higher before NTM infection, regardless of the type of NTM. In relation to patterns of treatment, the team found that considerably more patients with slow-growing NTM had received penicillin/ beta-lactamase and rifampin following infection compared to before infection. They noted that “an earlier CF diagnosis was associated with a higher isolation of slow-growing NTM and greater antimicrobial use after infection” adding that “NTM acquisition is associated with a worsening of [lung function]. Thus, both the early diagnosis and treatment of NTM infection in patients with CF may positively impact lung function.” The team believes that “increased awareness by clinicians on different NTM subtypes and more universal treatment plan for NTM infection in the CF population may positively impact patient management and outcomes.” Vertex and Quest Genomics Collaborate to Develop Genetics-based, Precision Medicines At the end of August 2018, Vertex Pharmaceuticals and Genomics announced a three-year collaboration designed to promote the development of precision CF medicine. This approach aims to tailor treatment based on individual patient’s genetic differences and lifestyle. The partnership is extendable for up to five years and will combine Vertex’s genetic expertise with Genomics machine learning to discover and improve new therapeutic targets. Machine learning is a nascent field of computer science which can predict outcomes based on its ability to identify patterns in previously generated information. Genomics uses a proprietary machine learning tool to predict any expected molecular, cellular, physiological or disease outcome based on genetic differences found in the human genome. Genomics says that this ‘Genomics Engine’ is the largest of its type in the world, with over 100 billion points of data to help identify genetic variations in over 14 million positions on the human genome and link them to specific outcomes. www.cfireland.ie 19
You can also read
