18

CCHHAARRIITTYY MMAATTCCHH

PPAATTIIEENNTTSS WWIITTHH CCFF VV SS TTHH EE SSTTAAFFFF OO FF

SSTT.. VVIINNCCEENNTT’’SS HHOOSSPPIITTAALL

DDAALLYYMMOOUUNNTT PPAA RR KK 1166 SSEEPPTTEEMMBBEERR 22000066

The Stars:Lar Brennan & Dave Duffy

The Team

The Others The Fans

The Medals

The Score: Who Cares!

The Game: Football

Future ForceFuture ForceAUTUMN 2007

MAGAZINE OF THE CYSTIC FIBROSIS MAGAZINE OF THE CYSTIC FIBROSIS ASSOCIAASSOCIATION OF IRELANDTION OF IRELAND

by pwcf for pwcfmy little miracleadvantage of cf

CONTENTS

Introduction ............................................................ 1Editorial - Rory Tallon

E-Interview .............................................................. 2Rosie Fitzgerald - Post-tranplant Rosie tells usall about Having the Word

Web Watch ............................................................... 3CF pregnancy, genetic counselling and nutrition

Its a Rap .................................................................. 4The Rap of Vinny

Decision to Transition ........................................... 5The all important timing of the move to adult care

Things to do on your Neb ...................................... 5Don’t just twiddle your thumbs- put your nebtime to good use

What Ails Ye ............................................................. 6O Mother Where art thou and thoust fellow MPsNathan Swan

Photo Gallery ................................................. ......... 8Christmas Party 2006, Carol Brady

29th European Conference ...................................... 10More on the Copenhagan Conference 2006Deirdre Meredith

Patient Liaison Group .............................................. 12Update on St Vincent’s working group

Bulletin Board .......................................................... 12Keeps you updated

My Little Miracle ...................................................... 13To be a mum with CF is a major undertaking- but it’s all worth it, Sinead Sheehan

The advantages of a CF Gene ................................. 15CF has its benefits!, Maria Daly

My Dream ................................................................. 16Jean Byrne

Tribute To Jean ......................................................... 17Future Force remembers our most sadly missed friend, editor and PWCF representative

Dalymount Park Charity Match ............................... 18Were you there?- forget Ronaldo, we had Anto

pg 2

pg 5

pg 13

pg 18

pg 15

INTRODUCTION

ello readers! We’re back. After a muchregrettable skipped edition, apologies. Your

very own editor and designer and PWCF repre-sentative Jean Byrne sadly passed away inMarch of this year- mid way through compilingthis current edition. Future Force will never bethe same again. I know I won’t too. Jean tookFuture Force to a whole new level. I hope the cur-rent committe can live up to your brilliance Jean.We miss you. Thanks for everything you did forCF in Ireland. You can read a little more aboutJean in her tribute.

What a year it has been so far! The grand electionand the grand pre-election publicity campaign byCFAI- well done people. A lot of you out thereplayed a huge part in putting CF on the politicalradar - doing TV slots, national paper interviewsand articles, radio shows and hard-slog lobbyingthe local politicians- well done to all and welldone Orla Tinsley - your eleventh hour plea toBertie and Enda in the Irish Times was inspira-tional. A pity Bertie didnt know his osteoporosisfrom his cystic fibrosis for the leaders debate,but then he is having a bit of memory trouble oflate so maybe all that basic school biology evad-ed him too. I hear the CFAI delegation set himstraight at their meeting with an Taoiseach inSeptember. Maria Daly was your PWCF represen-tative at that meeting and explained to the HSE,the Taoiseach and the Minister for Health whyexactly we need a designated CF unit at thenational referral Centre in St Vincent’s and inCork and Galway and other regional centres asper the 2005 Pollock Report. I understand thelaunch of the HSE working group report on CF isimminent, or at least it was last July 2006 anyway.

Congrats to Paul Minchin, the first PWCF inIreland to have a double lung transplant in theMater. Best wishes Paul. Lets hope the Mater cancontinue the good work that they have now start-ed. Unfortunately organs are the limiting factorso please don’t forgot to dicuss organ donationwith your friends and extended families. Rosietold Future Force exactly what it was like havingthe word, thanks for the interview Rosie.

We have loads for you as usual in this edition ofFuture Force. Don’t waste your time while onyour neb- you can practice those all importantmulti-tasking skills of yours. Why not write arti-cles for Future Force too? Nathan goes bashing,Carol reports on the 2006 Christmas Party whileMaria explains why having CF genes has its

advantages! We game em a run for their moneythat St Vincent’s team- no not your friendly CFtreatment team - I speak of the makeshift ‘foot-ballers’ they fielded at Dalymount park - see thepics on the back page. Next time we wont go soeasy on you lads. Thanks very much Dave Duffy- you made the day.

Future Force needs your thoughts, views, arti-cles, wit and wisdom to continue. In fact we’lltake anything you feel like sharing! Any techiesout there send us graphics and cool clipart too!Thanks so much to all who contributed to thisone- we got there. Lets keep Future Force going-for Jean, for Robbie, for Linda, for Davin and forourselves.

SEND ALL LETTERS/ARTICLES TO

The Magazine GroupFuture ForceCF House24 Lower Rathmines Rd.Rathmines,Dublin 6

Email: futureforcemag@hotmail.com

CREDITS

Future Force is the property of:The Cystic Fibrosis Association of Ireland,CF House, 24 Lr Rathmines Road,Dublin 6

Tel: 01-4962433 Fax: 01-4962201Lo-call: 1890-311-211Email info@cfireland.iewww.cfirleand.ie

EDITING STAFFJean Byrne Eleanor WalshRory Tallon

DESIGNJean Byrne Rory Tallon

MAGAZINE COMMITTEEEleanor Walsh, Nathan SwanMaria Daly, Rosie Fitzgerald,Orla Tinsley, Carol Brady

PHOTOGRAPHSCarol Brady, Jean Byrne

The views and opinions expressed in thismagazine are not necessarily the views ofThe Cystic Fibrosis Association of Ireland.

1

H

Rosie Fitzgerald got the life-saving call for transplant.Future Force caught up with Rosie to tell her story.Thanks Rosie- quite a story- well told...

FF: How did you first feel about transplant?Rosie: Having the word 'transplant' uttered for the firsttime at clinic made my stomach lurch. I knew I wassick, my lung function was poor and very, very slowly Iwas losing ground. Despite this, I had always thoughtof transplant as something "in the future.." and notnow.

FF: When did tranplant become a reality foryou?Rosie: I was in 6th year and preparing to do my leav-ing cert when I went over to the Freeman hospital inNewcastle to have an assessment for a double lungtransplant. I kept telling myself how silly this was, but inthe back of my mind, the reality of how sick I must beto reach this stage was sinking in. Back then, I was toldI was a suitable candidate for transplant but was toowell.FF: So how did that decision affect you?Rosie: I was happy to put it all behind me and get backto living a "normal" life. Definitions of normal had slow-ly changed over time, and being dropped at the door ofevery place I visited became the norm, as did doingeverything possible to avoid rendering myself breath-less at every turn. This meant as few trips upstairs aspossible, going shopping and "out" in general less andless as well. Being on ivs every two- three weeks or soalso became normal for me.FF: What happened with your Leaving Cert?

Rosie: The summer of my leaving cert, I became a lotsicker than I had ever been. I ended up doing my leav-ing cert in Crumlin Hospital. I felt myself tired muchmore, and I would require oxygen at night and duringthe day when I was sick. My weight was at its lowest at39kg despite being on night and day feeds and I wasgenerally just sick.

FF: Were you re-assessed since you felt somuch worse?Rosie: In November 2006, it was decided that I need-ed to go on the waiting list for a transplant. When thedoctor told me, I was shocked. It was then I had to faceup to the fact that this wasn't "just a phase" of badhealth; my lung functions were low and not gettingback up again. My quality of life was getting that littlebit worse. I had to defer my college course becausecaring for my health became my number one goal. Iconcentrated on getting my weight up and with the helpand hard work of all dieticians involved, in February itwas high enough to get on to the list. And so the waitbegan…!

FF: How did you manage waiting - were youanxious?Rosie: I knew from other people's experiences that Icould be called at any time. It may be days, weeks,months or even years. I was also aware that my callmay never come. I think it was at times like this, wherekeeping a positive head helped me most. What wouldbe would be, and there was literally nothing I could doabout it except enjoy the health that I had when I hadit. This meant hanging around with friends and justdoing things that made me happy. I wasn't prepared toput my entire life on hold and sit by the phone; life wasstill going on and I was determined to enjoy it.

FF: So tell us about your operation?Rosie: 6 months after waiting, I got my call and flewover to Newcastle. I was scared, excited, nervous andelated all at once. The night before I was able to go upon to the transplant ward and say hello to a fellow Irishtransplant patient Amanda who had had her transplant10 days before me. I couldn't believe it when shewalked to the door and I saw how well she looked sosoon after. It was both comforting and as cheesy as itmay sound, inspiring to see a friendly face that had justbeen through what I was about to face and to know thatshe had made it out the other side. The next day, I saidgoodbye to my parents and went into theatre. Mywhole body was tense, and then I knew that I just had

E-INTERVIEW

2

E-Interview with Rosie FitzgeraldHaving the Word

E-Interview with Rosie FitzgeraldHaving the Word

WEB WATCH

3

to relax and go with it. I took a few deep breaths as thesedation went in and closed my eyes.When I awoke briefly that evening, it only took me sec-onds to realise I had had my transplant and that I wasthrough. I knew the road ahead would have many hur-dles, but I was so thrilled and excited at what lay beforeme. I came off the ventilator and drifted in and out ofsleep. I didn't notice any new fantastic ability to draw abig breath, but when I went to tell this to the nurse, Inoticed that my sentences went on and on without megasping for air. That feeling was magical.

FF: How was your recovery?Rosie: I spent one and half days in ICU and I was thentransferred up on to the transplant ward, 27a. Initiallythings began to go very well; I felt great despite what Ihad gone through, but then a combination of problemscame together which had us all (me included) a bit wor-ried. My kidneys began to cause problems and therewas too much fluid on my lungs making it harder tobreathe. They had a cosy bed booked back for me inICU should things not improve, but we decided to tryCPAP (a non-invasive ventilator) machine first. I thinkthe fright of it all and my emotions having built up,resulted in me deciding that this whole transplant waspossibly not one of my best decisions; I wanted my oldlungs back and I wanted them now! I explained this tomy mom through my tears but after a few minutescalmed down again. Things improved greatly thatevening and I was off the CPAP and back on the rightroad again, nicely avoiding a trip to ICU. It was areminder that despite having beautiful new lungs in me,I wasn't ready to jump out of bed and do somersaultsjust yet! There would be some fantastic highs on myrecovery, but every so often there would be the odd lowor hard moment too. Whenever I had a tough couple ofhours, I had to focus that the next couple would bemuch better.

FF: Must have seemed like you would neverrecover - was it tough leaving the bed?Rosie: After that, things really began to pick up. I haddrains and lines removed day by day and was able toget out of bed and walk a few steps. I had a lot of fluidin my body which made it hard to get around quickly but daily trips to the gym and cycles on the exercise bike(all without so much as a cough) helped all this.I suffered some acute rejection after a couple of weeks,but this was fixed with a brief increase of my steroidsand a change in my anti-rejection medication.

For anyone considering genetic counselling,you may find this website useful. It gives anexplanation about genes, genetic counselling,who the genetic counsellors are, who shouldsee one and a family's risk for CF. (full addressfor this page www.kidshealth.org/parent/sys-tem/medical/genetic_counseling.html)

We’ve featured this site before but deservesanother mention because it contains a minefield of information about CF and pregnancyincluding personal pregnancy stories, pregnan-cy in lung transplant recipients, metaboliceffects of pregnancy in cystic fibrosis, pregnan-cy and fertility and the impact of pregnancy onthe CF patient. It's worth a look for anyone con-sidering starting a family!!

This website contains advice about weight gain,including tips for adding protein and calories tothe diet. It also has advice for parents, aboutnutrition for teenagers, infants and toddlers withCF. It contains a food pyramid and a vitaminchart.

IF YOU FIND A REALLY USEFUL WEBSITE YOU THINKOTHERS SHOULD CHECK OUT DONT FORGET TO EMAILUS THE LINK AND TELL US WHY IT WAS USEFUL-EMAIL TO FF UU TT UU RR EE FF OO RR CC EE MM AA GG@@HH OO TT MM AA II LL..CC OO MM

EB ATCH

www.kidshealth.org

www3.nbnet.nb.ca/normap/cfpregnancy

www.cystic-fibrosis-symptom.com/nutrition

by Eleanor Walsh

IT’S A RAP

4

Was feeling like some tlcHeaded right over to St VNot looking so wellHad trouble with my adl'sWas pale like a vampCouldn't get up that ramp

Nurses checked my pftsNext there was the fbcWe had to call the MIOCB'cos I needed an abgThey don't look so hotthose O2 satsHey!.. ouch! That hurt, I'm not down with that

((AAuuddiieennccee tt iimmee……))

All my homie VIPson theirhome IVee'sAll my homie OP's in the OPDAll my peed off peopsin the A&ECome rap with me… & the Doc SVee

They found me a bedWhere I could lay my headInserted a lineTo start my timeReading my chart - see IV's bdRead it again, no sorry, td

Then came the MROCTo check on meI said doctor,doctor, can't you see I'm burning, burning?He said ‘is there agenius in da house?’ I said "who me…?"Got me a temp 39 degrees C

Dietician wants the low onmy situation kgee'sI hollered no way that ng tube’s not for meBut I let her check myHb1ACIn relation to my CFRDTeam came round for an‘intimate’ r/vee

Intimate my ass - I counted23!

((AAuuddiieennccee tt iimmee……))

All the studentslookin' scared say haaayAll the gerie's on theward say hoooHay ho, hay hoGive it to mestraight, do I have a DIO?

Bmi, bmd, crp, bp, what ongod's earth

are ye doin' to me?Fev, ecg, ultrasound, ct, hey doc Vee you just messingwith me?

2 weeks on and i’m feelingmuch better, Got Doc Vee towrite a work letterSlept well last nite, chest not so tightDid a morning on the bike

with no sobSo thumbs up y'all, I'll behome for tea!

((AAuuddiieennccee tt iimmee……))

All the porters in theplace say haaayAll the nurses on theward say hooHay ho, hay hoSpike a high, hit a low You checked my satsnow let me go

((EExxiitt ssttaaggee lleefftt .. .. .... .. .. .. .. .. .. .. .. RRaappiidd !! ))

In between discussing "Big Brother" and "CelebrityLove Island", the staff on 27a educated me on how torecognise symptoms of infection and rejection; on theimportance of taking my medication as well as givingpractical advice on a whole range of issues. Theyhelped me to keep my sanity during my recovery,although what I did to theirs' is questionable (!) with myfeeble attempts at the Geordie accent amongst otherthings!Exactly one month after I arrived in Newcastle, I wascatching a Ryanair flight home. I couldn't wait to runabout and show off my new lungs and everything Icould do with them. I had to be careful around crowdsinitially due to infection risks, and still avoid (or try to)people who are sneezing everywhere! Since trans-plant I no longer require any feeds and my weight ishigher than it's ever been. I work a few days a weekand try to play sport or exercise of some sort on mydays off. I am planning to return to college next year toget my degree in Psychology Applied to IT. It's the lit-tle things like not having to leave extra time to get to aclass (so I can take my time on the stairs!) that I lookforward to. In the meantime (in between work andsocial life) I hope to raise awareness about the impor-

tance of organ donation and amongst pwcf I shall berecruiting people for the transplant games! In the sum-mer I plan to take part in the British Transplant Gamesas part of the Freeman team. I have had my three and six month check-ups andthankfully everything is going great. I attend the MaterHospital every so often to have blood levels checkedand to make sure everything continues to go in theright direction. There have been so, so many enjoyable and fantasticdays and moments since my transplant, but none ofthis would have been at all possible without my donor.Through my donor and their family's kindness andgenerosity, I have been able to do things I would neverhave thought possible. I will always be eternally grate-ful to them for this precious gift of life.Rosie: Can I just add: If anyone who’s had a trans-plant in the Freeman Hospital would like to participatein transplant sports please contact me at fitzger-alb@yahoo.com. Its all about fun and getting intoexercise so you don't have to be sporty- absolutebeginners fit right in!FF: Thanks so much Rosie, good luck in thosegames and best of luck with your new life!

DECISION TO TRANSTIION

5

Ya ever hear the song “Should I Stay orShould I Go”, well have you ever had that reallife dilemma?

I'm sure the adult CFers know what I'm onabout. That's right ladies and gentlemen, theprocess of Transition. For me, its like some-one's slapping me in the face and telling meto wake up, I'm now an adult. I, like many ofye, want to stay in Paediatrics forever, yep...forever! But I have to go. I'm sure they wannasee the backside of me by now. But no, I'mstaying until my exams are over because ofthe stress level involved in exams nowadays,I'm sure ye all can get that choice at least.

These are the factors I looked at in determin-ing where to go for Adult Care

Where to Go? Well firstly, look at youroptions i.e. does your paediatric hospital offerAdult care, or is the Mammy making ya go

to a credited CF centre like St Vincent's orCork University. Also look at practicality for thisone, which is closer and the like?

Facilities: Which hospital has the better facili-ties? The thing I did was asked people who areattending the different hospitals. Go towww.cfireland.ie (publicity n'all) and ask on theforums.

Mollycoddling: Now I for one don't mind a bitof mammying now and again but enough isenough, especially when the Doctor's andNurses are doing it too. I am also looking for-ward to the independence I'll receive once Itransfer, either it be going to clinic by myself orat least just not being told goo-goo-gaa-gaanonsense.

So to all of those out there about to transition,I hope ye have read my "protocol" ha-ha forchoosing an Adult hospital!!

THINGS TO DOTHINGS TO DO WHILEWHILE DOINGDOING YOURYOUR NEB !NEB !

FFiillee yyoouurr nnaaiillss

WWoorrkk oonn ccoommppuutteerr- cchheecckk oouutt CCFFIIrreellaanndd wweebbssiittee

Catch up on

text mmessages

BBlooww dry yoour haairEEmmaaiill ffrriieennddss- eemmaaiill ffuuttuurree-

ffoorrcceemmaagg@@hhoottmmaaiill..ccoomm

WWaattcchh TTGG44 - ffoorr tthhee ssuubbttiittlleessooff ooccuurrssee ((nnooiisseeyy nneebbss))

RReeaadd aa bbook- orr neewssppaappeerr

SSttuuddyy - ((ssoorrrryy!!))DDoo aa jjiiggssaaww

KKnniitt aa ssccaarrff ffoorr tthhee wwiinntteerr

MMaakkee uupp yyoouurr ccoolloommyycciinn

MMaakk

ee uupp TTOODDOO

lliissttss !!

BBee ccrreeaattiivvee - ddrraaww ppiiccttuurreess

CCuuddddllee yyoouurr ppeettss

DDoo yyoouurr kkiiddss hhoommeewwoorrkkDDoo oouutt MMeedd lliisstt ffoorrCChheemmiisstt

GGeett ssoommee zzzzzzzzzzzzzzzzzzzzzzzzzzzzzzzzzz’’ss

WHAT AIL’S YE

6

WHAT AILS YE?Is there something that’s been bugging you for a while? Got something to get off your chest?Future Force invites you to have a good gripe and asks “What Ails Ye”? Watch out parents, Nathan goes bashing!

n the last few years one of the topics which gets dis-cussed on a more regular basis than I would have

thought it deserved is the action, reaction and thesometimes down right strange behaviour which occurswhen the parents of PWCF are confronted with theirviews on cross infection and general contact with otherpeople of the C.F. persuasion.

I hated the way that these parents I refer to wouldpreach about how "they" treat their child's C.F. - someof these "children" being in their late teens and earlytwenty's. Not all parents are like this of course, it is thefew, the brave and the proud that I refer to and in noway do I wish to offend anyone; although I fear I will asit seldom ceases to offend when people's views areput under the microscope.

How about we generalise the group I think I wouldbelong to and the group the parents I am talking aboutinto that oh so great invention of the spoken word, anacronym.

mmmm what shall I refer to the slightly mad parentsas; how about MPs {mad parents} and those that dis-

agree MPBs {mad parent bashers}.

So what is an MPB?Well one is a MPB when the stories about MP's filterdown to the general populous, through the c.f. forum,A.G.M's or any other get together, have you seeing redand make you slap your head and cry out "where dothey get these people?"

For years I have been one of these people and wouldlook at these parents in utter disgust, so one day whileperusing the c.f. forum I came across a string wheresomeone was discussing their children and about howthey wouldn't even let them come to Tesco's with themwhen they do the shopping, for fear of contact with notjust other PWCF but anyone that might be carrying acold, infection or even just a slight case of the plague.

This left me reeling; if you are to be afraid of takingyour child to the shops how will you handle it whenthey start school {if they haven't already}What about when they start dating, are they going tohave to kiss their girlfriend/boyfriend through clingfilm?And of course what will happen to the poor little mitewhen he or she has to go into hospital for treatment.

his is only one person and only one case in point.Another much more disturbing story that I feel gets

to the root of the problem was told to me by a fellow PWCF who can relate to the children of this piecebecause his mother would have been an MP.Over a glass of vodka and orange he told me this taleof one mother, two children and a doctor.It started with the mother having a meeting with herdoctor about her new born child who had just beendiagnosed with C.F. Her other child, a few years older,had also been diagnosed with C.F. when he was aninfant, so she was already aware of C.F. and what itcan mean for the young child. What she wasn't awareof was what the doc hinted she should do with theolder child. Put quite simply the doctor was trying toconvince the mother that it would be best if the older ofthe two moved out of their house and went to live withthe mother's parents.

For the safety of the baby the older child should not beallowed near the youngster.Granted in a crazy way this can make sense; the babyis still developing its immune system and if it was tocatch something from the older boy then it could causea problem.

ut to move a child out of its home to make way for thenew baby as far as I can see would cause far greater

problems in the future, not to mention that the

When parents go mad....

By Nathan Swan

O Mother Where Art Thou

7

O MOTHER,O MOTHER,WHEREWHERE ARTART THOU?THOU?

granparents were both old and retired and to ask themto take on the full time care of a youngster would bedespicable.But the doctor persisted.

Apparently this sort of coaxing by doctors goes onmore than I would have thought.

hese and many others are the stories that are toldthat get myself and other MPB's so infuriated with

MP's who hang on every word that the doctors tellthem and try to keep their children wrapped up in cot-ton.

It is a well know fact between PWCF that the majorityof information about our own illness is gained fromother PWCF. I have known doctors to give me advicethat PWCF have told me was untrue or down rightwrong. I have also handed out more than my fair shareof info to PWCF which they have in turn passed on toothers.

I aint saying that we know everything but I would liketo think we have picked up some info along the way.Recently I have talked to a fellow PWCF who hasnever been in direct contact with any other PWCF fortheir entire life. This might sound to some as an idealsituation, but from what I was told by this person it wasfar from ideal. They felt so uninvolved and alone withtheir peers in the CF world that they were delighted tofind the CF forum and have the opportunity to talk toother people who have to deal with the same problemsthat they do.

or reasons I shall not divulge this person had neverbeen even in the same room as another PWCF. Madparents would like it if the situation described abovewas the same for their children but I do not think thatthey can perceive just how lonely it can sometimes beto have CF. If we were never able to commune witheach other except over the internet then I fear it wouldbe a sad day.

You can not look into the eyes of a screen and seerecognition in the face of a person who has an under-standing of the same problems that effect you. It can not make you laugh and smile when you need cheer-ing up, a screen does not feel.

I get scared when I think of what the future might holdfor PWCF - bad information and common sense seemto have switched sides at some point and the MP’s arefeeding off it like locusts at an all you can eat buffet.I understand the need for segregation and isolationwhen it comes to treating CF- having had my own boutof MRSA a few years back I know what its like to beunable to leave a room and have to sit there watchingRTE or TV3 all day long because you're a hazard toother PWCF.

I could waffle on here for ages and it would do no good,but if I get through to just one parent and make themunderstand that in 'protecting' their kid they could beharming them too, it will be worth it.

augh and grow fat.

Editor’s Note:

Future Force anticipates that this will provoke emotivediscussion out there. The issue with cross infection inCF is in no way trivial and cross infection needs to berespected. To respect cross infection however youneed to understand risk. Educate yourselves aboutthe risks of cross infection. Cross infection preventioncomes down to risk management and risk manage-ment comes down to common sense. Paranoia and'pseudo-fear' are hugely negative and damaging psy-chosocial concepts. They cause you to view otherpeople with CF with fear, suspicion, accusation andnegativity before you even speak to them or assessthem as persons in their own right. Be safe, beinformed, be sensible but above all be humane.

8

PHOTO GALLERY

This years Christmas party was in the TalbotHotel in Carlow. It is located about a mile out-side Carlow town and right next door to thehotel is the dome with bowling, pool andgames.

I have to say the hotel staff had everythingunder control and were very friendly, the roomswere great and everyone seemed to be veryhappy with the hotel.

So it was off to the beauticians and hair-dressers for the girls and to the bar for the boysto hold it up as best they could.

After hours of beautifying ourselves and awash and a shave for some of the lads we allmet down in the bar of the hotel to getacquainted with some new and old faces.

After a few bevies it was into the room to sitdown for our meal, some of us had been sav-ing ourselves all day for this meal.

The atmosphere was brilliant with some of uswearing Santa hats, ties and whatever else wecould put on our heads... in some cases evenbits of the Christmas tree (Joy)!.

Then it was on to the dance floor to dance thenight away to the band Applecore and a discoafter. Some of us managed to win a weekendaway we wont say who (Derek) and it all didn'tend there as it was in to the residents bar toend the night on a high.

PWCF Annual Christmas Party

2006 by Carol Brady

9

CHRISTMAS PARTY 2006

EUROPEAN CONFERENCE

10

Every year there is an International Cystic FibrosisConference which takes place in a different country. InJune 2006, Carol Brady (PWCF Delegate) and I(PWCF Delegate) set off for Copenhagen, Denmarkfor the 29th International Conference. GodfreyFletcher, CEO of CFAI, accompanied us on this trip. Copenhagen is Denmark's capital city with a popula-tion of approximately 1.5 million. It has lots of charm,elegance and a relaxed atmosphere. It is Europe'sdesign capital which was evident in the contemporaryconference centre we visited. It also appeared to be asafe city with a friendly population. Carol and I had thepleasure of visiting the Tivoli Gardens, a large touristattraction in Copenhagen, well worth a visit for any ofyou planning to go to this city. Our Conference took place in a location outside thecity of Copenhagen in the Bella Conference Centre.This is a beautiful, spacious modern building which isvery well equipped to facilitate a large number of peo-ple. Public transport is extremely efficient in this city sowe had no excuses to miss the early starts!!

Carol wrote an article which appeared in the last edi-tion of Future Force featuring 'Work Disability in Adultswith Cystic Fibrosis and its Relationship to Quality ofLife', 'Staff Knowledge and Understanding ofSegregation Policies in a large adult CF unit' and'Increased Segregation in an Adult Cystic FibrosisCentre: the impact on patients' feelings and behav-iour'. I am choosing different topics to attempt to givethe readers a broader overview of the options avail-able at the conference.

Enhancing Family Strengths Charlotte Dawson, UK

First of all what are considered to be family strengthsin relation to children with Cystic Fibrosis? Doctors,Nurse Specialists, Psychologists, Physiotherapists,Dieticians, CF Trust, School/Community Nurse, newacquaintances (possibly CF patients themselves),physical support and emotional support are all variedstrengths that families can have. Think of how mucheasier it would be for the family if the CF patient has agood relationship with their Consultant or with their CFNurse and could turn to them to discuss things, or per-haps voice their concerns objectively to. Sharing expe-riences with other CF patients can be beneficial as thepatients can relate to each other and support eachother.

In terms of enhancing family strengths for families whohave a CF child, the first thing you need to consider

are the resources available to families. How heavy isthe burden of care on families and how much does it impact upon general family life having to care for a CFchild? Inevitably, more money would be required incaring for a CF child. Is there a fund available to helpfamilies if they cannot afford little treats for the CFchild?

In order to enhance family strengths, flexibility needsto be taken into account. Can the child use independ-ent techniques in their treatment thus taking pressureoff the family? There are many varied techniquesavailable nowadays which enable families to choosewhich suits their needs the best.

In order to enhance family strengths, families have totake account of what support is available to them.What is their main source of information? Is the hospi-tal local to them or is there an outreach service avail-able? Some hospitals organise parents evenings toprovide a forum for parents to swap information abouttheir CF child. Are there regular newsletters availablewhere people can read of others' experiences? Is theCF Nurse in the local hospital available on his/herbleep, mobile phone or email? Often it gives parentspiece of mind if CF personnel are easily contactable.

To enhance family strengths, liaison is important too.The parent-child communication is vital. Does the childlisten to the parent about taking their medications or dothey rebel thus jeopardising their treatment? The fam-ily-doctor relationship can be important also.Sometimes the GP might be the first point of contact.It is vital that the child's GP understands the conditionand best forms of treatment. In this respect it is impor-tant that the child's Hospital/Consultant keeps the fam-ily GP up-to-date, especially if the child has spent aperiod of time in hospital. The GP needs to beinformed of the treatments the child received and whatworked best etc. It is essential that there is good com-munication between the Medical Teams in the hospital.Often children with CF may need different teams tosee them, for example it would be important that if thechild suffered liver problems that the Liver Specialist

By DeirdreMeredith

COPENHAGAN 2006

11

liaises regularly with the Chest Consultant to ensurethat the patients treatments gel together.

Is Living with CF a Painful ExperienceDavid Stock, Specialist Registrar, Adult RegionalCF Unit, Liverpool, UK

When I saw this topic on the agenda I have to say Iwas intrigued. In what respect did David Stock mean apainful experience? Did he mean physical pain or didhe mean how a CF patient's lifestyle might be difficult.

The answer lies somewhere in between. Physically,pain is a common problem with any chronic illness.Often with CF there is uncertainty regarding optimaltreatment. It can be a vicious circle where somethingtreats pain but may cause other problems, for examplesome painkillers are known to contribute to bowelblockages so while they may be the best treatment forthe pain they cannot be given due to the side effectsthey have. Specifically, pain in patients with CF cancome from multiple sites.

David Stock carried out a study using a locallydesigned questionnaire. He surveyed a sample ofregional CF cohort in hospital and out patients inJanuary 06. He also held face-to-face interviews withDoctors or Specialist Nurses in CF. He surveyed 30people in total including 10 who were in employment,4 who were in education and 16 who were unem-ployed. 14 males and 16 females were questioned.The mean average age was 23. 10 of these CFs werevery active, 14 were moderately active and 6 were un-active. He quoted the most common sites of pain fromthis study as being headache, backache and joint pain.

In conclusion, half of CF Adults experience pain everyweek which can lead to a marked limitation of activity.Analgesics are generally quite effective and well toler-ated. But each CF patient may find different analgesicssuit them better.

Three Methods of Measuring Adherence in a LongTerm Trial in Cystic Fibrosis. M. Elkins, M Robinson, C. Moriarty, J. Sercombe, B.Rose, C. Harbour, P. Bye, NHSCF Study Group -Royal Prince Albert Hospital, Woolcock Institute &University of Sydney, Sydney, Australia

A trial was carried out with children over the age of 6 inconnection with the inhalation of saline twice daily overa period of 48 weeks. The three methods used to mon-itor this were:

"Diary Cards completed weekly"Return of used and unused ampoules at scheduledvisits" A devise which logged nebuliser use (subjects were

only informed of logging devices after the trial andgiven the opportunity to decline consent to have theirdata downloaded)

The results showed that no subject declined to havetheir results downloaded in the case of the logged neb-uliser use. Complete adherence datasets wereobtained from 90% of subjects monitored using thelogging device. This was better than the proportion ofsubjects using diary cards and return of ampoules.Results from diary cards were more comprehensivethan the return ampoules. Adherence was significantlyhigher in children than in adults on diary cards andreturn of ampoules but the difference was non-signifi-cant with the logging device. It is interesting to notethat adherence declined over the year of participation,via all three methods.

Poor adherence and insufficient behaviour leads to afailed patient! Therefore, the CF Patient and their Medical Teamneed to reach a time limited mutual agreement whichin effect is team working.

Types of Adherence Monitoring:"Subjective"Overt"Concealed

In summary, monitoring via a logging device returnedsignificantly more complete datasets, and may give amore accurate assessment of adherence.

Is CF more severe in females than in males?Survival analysis from the Italian Cystic FibrosisRegistry. L. Viviani, A. Bossi, B.M. Assael & Italian CFCentres Directors - Institute of Medical Statisticsand Biometry & Verona CF Centre, Italy

According to some epidemiological studies, CF ismore severe and causes earlier mortality in femalesthan in males. The Italian Cystic Fibrosis Registry per-formed survival analysis on 5175 subjects (of which2522 were female and 2653 were male) according togender, period and mode of diagnosis.

717 deaths were related to CF, 385 of which werefemale and 332 were male. Kaplan-Meier survivalcurves were highly different between male and female.Survival probability at age 18 was 90% for females and92% for males. The date was period dependent: malesand females born in or after 1988 had not significantlydifferent survival, either those diagnosed by neonatalscreening and those by symptoms. Moreover, whilenumbers of males and females are equal for patientsborn in or after 1988, the proportion of males was high-er among those born before 1988. This could be a

PATIENT LIAISON GROUP

selection bias due to higher precocious mortality offemales. A similar number of males and femalesunderwent lung transplantations.

Taken together these data indicate that earlier death infemales should no longer be seen in the more recentcohort of patients. Analysis of lung function data in theregistry is ongoing to determine whether or not lungdisease is more severe in females although not caus-ing earlier mortality.

In summary, my experience of this conference inDenmark was a very good one on a number of levels.It was extremely informative and gave me plenty offood for thought. Overall, it is fair to say that progressis being made with regard to research into CysticFibrosis and there are more and more studies beingcarried out as time goes on, which all contribute toimproving the lives of all those worldwide who are liv-ing with Cystic Fibrosis.

12

ST VINCENT’S MEETING UPDAST VINCENT’S MEETING UPDATETE

CF Menu:CF Menu: Should be revamped - look out for greatermenu variety including panini's.

Emergency DepEmergency Department:artment: CF menu is available toyou in ED. Breakfast, hot lunch and sandwiches shouldbe provided. All PWCF in ED are identified for a specialmenu. This should be made available to you. Ensureyou let the staff know you need the CF menu.You should not have to skip meals in ED.

WWards:ards: Catering Department information leaflet avail-able on St Paul's ward advising you what to do if yourmeal is skipped. You should receive a note from cater-ing if you were away during meal times. Ask at NursesStation when you return to order a meal from catering.You should not have to skip meals on wards.

Interim Extra Respiratory Beds:Interim Extra Respiratory Beds: St Camillus’sward is now refitted with piped oxygen to serve as anextra respiratory ward. Plans are in place to merge StPaul's and St Mark's as one respiratory ward.

OutpOutpatientatients and Home IV Reviews:s and Home IV Reviews: Strict appoint-ment times now in operation. Important to respect thesetimes to allow for efficient clinic services and helpreduce the risk of cross infection with overlapping clin-ics. Please notify team in advance if you cant make anappointment to allow the team to re-allocate your slotto someone who may need a more immediate review.

Bathroom refurbishmentBathroom refurbishments:s: Plans are in place tocompletely refurbish the bathroom facilities on St Paul'sward and the wards 3 floors directly above. Time tocomplete was not specified

Issue Resolution:Issue Resolution: Please bring any issues to imme-diate attention of the nursing staff and the ClinicalNurse Manager on your ward. Please also feel free toask for Anne Henley, Assistant Director of Nurising ifyou feel any issues have not been adequatelyresolved. Anne would very much appreciate your feed-back if you have any concerns during your stay in StVincent’s.

If you have not had satisfactory resolution please con-tact the Liaison Group Representatives Orla Tinsleyand Rory Tallon through CF House - locall 1890 311211 or Email info@cfireland.ie. Alternatively pleasecontact us through FutureForcemag@hotmail.com

Notice BoarNotice Boardd

ExperExpert Patient Advisorst Patient AdvisorsWelcome Caroline Heffernan and Tomas Thompsonas your CF Advocates - they will be working toimprove things for us all- good luck guys

PWCF Delegates 2007-2008PWCF Delegates 2007-2008Nathan Swan, Brendan Lonnergan Maria Daly and Rory Tallon- anything you thinkshould be done or improved and you dont know whoto tell- well tell us and we will tell the Association.Contact us through CF House or throughFutureforcemag@hotmail.com or post your thoughtsto the CF Community Forum. We can hold chat clin-ics if anyone is interested. Post a topic to the forumand we can go from there.

CFCFAI ConferAI Conference 2007ence 2007Watch this years Galway conference on your PC.Check out conference clips on http://www.cfire-land.ie/articles.php/news/_conference_clips

CF Christmas ParCF Christmas PartytyBettystown Court Hotel, Bettystown, Co. Meath08th December 2007 - Jive Talking Party night!Accomodation is also available in the hotel at a cost of�65.00 B&B per person sharing. Cross infection guide-lines apply. Contact CF House / see CommunityForrum / contact Ger Fay - gerard71@hotmail.com

MY LITTLE MIRACLE

13

Hi my name is Sinead, I'm forty three years old and Ihave CF (which was diagnosed when I was two) Ilive in Kinsealy Co. Dublin with my seven year olddaughter Alice (pronounced Aleece as she was bornin France). I was a shy, underweight teenager withabsolutely no confidence in myself. I certainly neverimagined working, getting married (getting divorced)and having the most beautiful daughter but all this didhappen to me.

I worked full time in Dundalk for six years, went backto Dublin for a year and then moved to London forthree years. I was attending the Brompton hospitalwhere I was informed quite bluntly that I was never tofall pregnant with FEV1 of 31%. I was devastated. Igot no counselling, no support whatsoever. We (myex-husband and I) decided to move to South France -his home country- where I lived with him for ten years.I had a bit of trouble trying to find a CF specialist buteventually I found Prof Jean-Piere Chazalette atRenee Sabran Hospital in the Var county of France,two and a half hours from where we lived. He was,and is, a great inspiration to me.

The antibiotics are the same in France as here, theonly difference being their method of giving the med-ication. No cannulas are used , instead antibiotics aregiven twice daily using butterfly needles which aretaped to your arm and removed when treatment hasfinished, so you are free for the rest of the day till thesecond dose is due. I found it great as, using butterflyneedles, I never had a problem with veins collapsing,lines kinking, swelling, infection in the site - every-thing that has happened to me since returning toIreland where the policy is cannulas. I have practical-ly no veins left now.

When doing IV's a public health nurse was assignedto come to my home twice daily where she wouldadminister the antibiotic by inserting a butterfly needleand stay with me till treatment was finished. I also hada physiotherapist come to my house twice daily fivedays a week while I was living in France. All thistreatment was covered by French Health Board. Also,there was a forty bed CF ward with single rooms forevery patient and their own separate shower facility.They had a long fight to get this ward and to keep it.

When back home in Ireland on holidays I discussedwith Prof Fitzgerald (who was consultant for CF at thetime) my wish to have a baby. He agreed with theProf in London that it would not be in my best interestto go ahead but if I insisted he would give me all thehelp he could. I also spoke at length with ProfChazalette in France and a psychologist. (I was reallyworried about haemoptysis as I had had an embolisa-tion done in 1997 but I was reassured that anotherone could be done, even if I was pregnant). Luckily Ionly had one episode of haemoptysis which passedquickly. I received excellent treatment and lots of helpfrom the CF Team in France and I'm sure it wouldhave been the same with the Irish CF Team, if I hadbeen in Dublin during my pregnancy.

I discovered I wasn't getting pregnant because thecervical mucus was too thick and the sperm couldn'tget past. So I had an insemination done in March1999 and three weeks later I discovered I was preg-nant. I have to say I was thrilled to bits and scared todeath!

I had a blood test done at three months to check if thebaby had any abnormalities. This was done on theinsistence of my ex-husband who had now decidedhe couldn't face the responsibility of a child and a wifewith an illness (it took him ten years to realise this).

I won’t go into all the detail but our marriage endedabruptly, and I have been alone with my precious lit-tle girl since. If it wasn't for the love and support ofmy family, I would have fallen apart.

I found out at three months I was having a daughteras I did an amniocentesis which I wouldn't recom-mend unless you really are the type of person whoneeds to be informed of the health of your babybefore it is born. My daughter was and is perfectlyhealthy.

I ate very healthily during the whole pregnancy andtook lots and lots of rest. Physically, I never felt sogood before. Mentally, was another thing. I was wor-ried sick that my health would go down hill but it gotbetter. Fev1 went up to 40% and my Fvc to 80% .

MY LITTLE MIRACLEMY LITTLE MIRACLEby Sinead Sheehan

MY LITTLE MIRACLE

14

My hair, skin and nails were glowing and with theextra weight I looked good too. I had over night satu-rations done as I was afraid that my baby would belacking in oxygen while I slept. But the 02 sats werefine, thank God.

I continued with my usual treatment and I only hadone coarse of IV antibiotics six months into the preg-nancy. I developed gestational diabetes and had tostay in the maternity hospital in France for the last tenweeks of the pregnancy. I put on three stone and washoping to keep at least two stone afterwards but it allfell off as soon as Alice was born.

She was born by caesarean section three weeksbefore her due date, weighing in at eight pounds. Ithought I would have a small baby and all the clothesI brought her for the hopsital didn't fit her. As you cansee even at thirty six I wasn't prepared!

The ceasarean section was done under epidural butI'll never forget the pain after because as I had bynow a chest infection, I was coughing a lot and it real-ly, really hurt!

I had two weeks of IV antibiotics in hospital afterAlice’s birth and then I went home. I returned toIreland when Alice was six weeks old. I have had fan-tastic support from my family and none from my ex-husband’s family or from him.

I have to admit it’s physically demanding bringing upa child as a single parent but I hadn't planned it thatway and wouldn't recommended anyone to do sounless in full health.

I have chased Alice in the park, rolled down hills,done handstands, danced till my head spun etc. butevery time I look at the precious little miracle that Ibrought into the world, a wave of inexplicable lovesurges through me and I will do things for her that Ithought myself physically incapable of, just to makeher happy and to make sure she feels wanted, lovedand secure ( even if I am wreaked afterwards).

Seven years have passed since her birth and I haveto say the first five, until she went to school, were themost physically difficult to date. Now I have the morn-ings free till 2.30pm. Then its homework in Irish asshe goes to an all Irish school. I bring her horse-rid-ing, swimming and to the parks with her friends. Thatbit is easy enough as it’s not physical for me. She'sthe one doing the activities!

The hardest part is when I have to stay in hospital.For the moment my mother stays with Alice and willbring her to school and at weekends one of my sis-ters or brothers will take her. My parents are notyoung so there's a lot of guilt and worry involved in my part, as I don't like asking them and I know myparents will not be physically able to do this for muchlonger.

I, personally, don't know of any support system inplace for mums with CF.

My daughter is ok now about my going into hospitalbut when she was younger it was awful, as she criedand cried and I used to get really upset. She knows Ihave a problem with my lungs but she doesn't knowthe extent of it.

She’s too young and I answer her questions as theyarise. If I have a coughing fit, she gets worried andrubs my back and then runs to get me a drink. She isvery caring and at her age she loves helping every-one. Long may it last!

I would advise anyone thinking of having a baby todiscuss it long and hard with your CF doctor and CFnurses and if they agree it's the right thing for you,make sure you have lots and lots of family help,because when the baby is born you will need it moreand more with the passing of time.

I wouldn't be without Alice for anything. She’s mydream come true and the love and happiness shebrings to my life is inexplicable. The most importantjob I will ever have is bringing up my daughter to be aconfident, happy and secure child.

And I hope I will succeed.

Sinead Sheehan

I have chased Alice in the park, rolled downhills, done handstands, danced till my headspun...but every time I look at the preciouslittle miracle that I brought into the world awave of inexplicable love surges throughme....

ADVANTAGES OF A CF GENE

15

By Maria Daly

Advantages you laugh… advantages! This may sur-prise you but there are hidden advantages of havingthe CF gene. In the past if genetic testing was avail-able, the discovery that you were a CF carrier wouldhave brought you great happiness. As will berevealed, being a CF carrier was of great benefitthroughout history. As you may know CF is the mostcommon recessive disease in Caucasians worldwideand Ireland has the highest incidence in the world.Have you ever wondered why 1 in 19 people inIreland are carriers of the disease but it has a muchlower frequency of just 1 in 25 in Caucasians world-wide? Or why it has an even lower frequencyamongst other non-Caucasian races? This articledetails some of the research explaining the reasonwhy it was so useful to be a CF carrier a few cen-turies ago and why the CF gene is so common today.

Pressures of life are commonplace in a world wherethere is constant competition for limited resources. Anadvantage with regard to health and most importantlythe ability to procreate in comparison to other individ-uals of your species becomes obvious when popula-tions are studied over time spans of thousands, orhundreds of thousands of years. This is sometimesknown as survival of the fittest. Populations need toadapt better to their environment in order to survive,whether it be in the sea or land or sky. An example ofthis is the streamlined body of fish and dolphins whichis the best type of body to have when swimming inthe sea. Obviously fish and dolphins that swam fasterdue to their body shape had more offspring as theywere able to escape predators. Through time theentire population gradually became streamlined andswam faster as these were the individuals which sur-vived. In genetics, this is called positive selection asthe streamlined body was an advantage.

Since the CF gene causes cystic fibrosis it seemsobvious that it's not an advantage to have it and thefact that it is present in so many of the Irish popula-tion also seems to go against the laws of genetics. Infact, taking positive selection into account it wouldsound more reasonable if the gene declined in thepopulation through time and it was very rare today.However, this logical reasoning ignores the pressuresof life which have affected the prevalence of the CFgene. When we take a deeper look at the CF genewe realise that it has in fact undergone at least onepositive selection event in the past which has causedit to be so common today. It is clear that having twocopies of the CF gene would not confer advantage asthis causes cystic fibrosis. Therefore, in the past carri-

ers of CF that have one copy of the gene must havehad some advantage over non-carriers who did nothave any copies. This is called heterozygote advan-tage.

In the 1980s, it was originally proposed that the highfrequency was due to a fertility advantage in CF carri-ers but this idea was proved incorrect. In 1989, theadvantage was thought to be caused by CF carriershaving increased resistance to intestine-targeting bac-teria that cause diarrhoea. There is now substantialevidence that the high frequency of CF was causedby positive selection. The advantage of being a CFcarrier has been suggested to be caused byincreased resistance to many different diseases, suchas, tuberculosis (TB), influenza, malaria, bubonicplaque, syphilis or cholera. However, the diseaseswith the most substantial experimental evidence sup-porting this possibility are typhoid fever and TB.

In Ireland a huge proportion of people died fromtyphoid during the Potato Famine. This was becausein 1847 soup kitchens were set up in towns whichcaused a huge influx of people into them for aid. Thiscaused epidemics of typhoid as well as cholera anddysentery which claimed more lives than starvation atthe time. It was shown by a study on the incidence oftyphoid fever in humans that there was a lower fre-quency of typhoid fever in people who were CF carri-ers. Typhoid has been a prevalent disease throughoutEuropean and Irish history and it killed about 15% ofthose who became infected. This means that CF car-riers would survive where non-carriers died. Thiswould increase the amount of people in the popula-tion that were CF carriers compared to non-carriers. Itwas obviously a large advantage to have one copy ofthe CF gene during this time.

The science behind this protective value of the CFgene lies in the ability of the cystic fibrosis protein toregulate both chloride ion and sodium ion pathwaysand the CF mutation causes these not to functionproperly. As you may know sodium chloride (NaCl) issalt so this is the reason that the sweat of PWCF issalty! It was found in a 1998 study that bacteriawhich cause typhoid fever use the CF protein forentry into the body through the intestine. CF carriersonly have one CF protein that functions properly butsince they have one regular protein they do not haveCF. Scientists investigated whether this meant asmaller amount of bacteria which caused typhoidfever entered the intestine of CF carriers.

THE ADVANTAGES OF A CF GENETHE ADVANTAGES OF A CF GENE

DELTA F508 POPULATION ADVANTAGE

If this was the case CF carriers would have a betterchance of survival. They showed that mice whichwere DeltaF508 CF carriers were more resistant totyphoid fever than non-carrier mice so the advantagesof the CF gene have been shown to be true of miceand men! However, since the Potato Famine occurredso recently in our past it is unlikely to be a sufficientexplanation for the high incidence of CF in Irelandand we must look elsewhere to find a more extensiveexplanation.

New research published this year shows that TB isthe disease that is most likely to have been responsi-ble for the high incidence of CF, ruling out bothtyphoid fever and cholera. A pandemic of TB calledthe "white plague" occurred in Europe in the early17th century and this accounted for the death of 20%of all Europeans. Not only did TB claim so many livesduring that time, it also continued to claim lives upuntil the early 20th century in Ireland. The scientistsshow that it is evident that TB spans both the neces-sary geographical area where a high CF incidenceoccurs and length of history to enable it to have sucha high incidence. This is not the case with eithercholera or typhoid fever.

This explains the high frequency of CF in Caucasiansof European origin but it does not explain why CF hasan even higher frequency in Ireland than in the rest ofEurope. Ireland is an island and has therefore beenfairly isolated from other populations. As we all knowthroughout Irish history a number of groups of peoplehave come to conquer Ireland such as the Vikings,the Normans and the Celts. These came in smallgroups and it seems likely that these people mayhave coincidentally carried a higher frequency of theCF gene with them than existed in their original popu-lation. This is called the founder effect and could bepartially responsible for the higher incidence of CF inIreland. The discovery that a mutant CF-causing genecalled G551D, which is responsible for 7% of cases ofCF in Ireland, originated from the Celts when theylanded in Ireland between the 4th and 6th centuriessupports this idea.

There are a huge number of cystic fibrosis mutations.Only a small number of these mutations are found inthe Irish population with the most common DeltaF508having a frequency of 77%. A lower number of typesof mutations are seen in Ireland than other parts ofEurope. This is due to Ireland's status as an islandwhich results in the population mixing less unlike pop-ulations on the European mainland which could traveland mix with relative ease.

All of this research shows us that the high frequencyof CF has been influenced by both the founder effectand the disease history of our island. Even the factthat Ireland is an island affects the occurrence of CF.

Resistance to diseases by CF carriers is the mainreason that CF is the most common recessive dis-ease in Caucasians worldwide. As shown by newresearch TB may be more responsible for the CF inci-dence than typhoid but it is likely that both of thesediseases have been involved. However, Ireland hasan even higher incidence due to the founder effectand the fact that it is isolated from mainland Europe.

As you can see the cystic fibrosis mutation has amore complicated and exciting history than you mayhave thought. It has witnessed plagues, famines andinvasions. It has spread through populations andgiven CF carriers an added bonus of resistance tosome diseases. You may often curse the CF mutationbut some of your ancestors would not have lived if itdid not exist. It seems that every cloud does havesome sort of silver lining.

New research (other references are available uponrequest):Poolman EM and Galvani AP (2007) Evaluating can-didate agents of selective pressure for cystic fibrosis.Journal of the Royal Society, Interface / The RoyalSociety 4: 91-98

16

MMyy DDrreeaamm

Lend me a dream to cling to I’ve none of my own any more,

They’re lying shattered at my feet in piecesDead, forgotten, untold

Pour me a dream to float inTo lose myself forever

For my life is a fragile bubbleFree, elusive, secure

Cascading waters, flying sprayGlistening pebbles, deep green pools

Shadow in the lock overcome with aweAvaricious revelant but owner

never by law

Jean Byrne (RIP)

TRIBUTE TO JEAN

17

Jean Therese ByrneRIP

Jean worked tirelessly for the CFassociation of Ireland on a voluntarybasis. She represented PWCF inIreland at the National ExecutiveCommittee of the CF Associationsince 2002 having been secretary ofthe former CF Adult Group for severalyears. Jean attended European CFConferences and brought you updatesin Future Force magazine and mostrecently acted as your representativeon the St Vincent’s Patient LiasonGroup. Jean had already compiledhalf of this edition of Future Forcebefore her untimely death. Jean was atruly amazing person, I could write forever and still never manage to captureher brilliance- a brilliance that any of

you fortunate enough to meet Jean inperson will have felt. One very lovelyquote best summarises Jean, if that ispossible: “Jean had qualities that areabsent in so many of us. She was fairand just and seemed to have an innersteel that fortified her with an unend-ing spirit and resolve” (L. Foley, CFRegistry of Ireland).

Jean, you touched us all. You walkedunafraid. We miss you deeply. FutureForce will be continued just as youwished for your readers. Thank youfor all that you did for Future Force,for CFAI and for PWCF in Ireland.Rest in Peace. As you used to say:‘Dyslexics of the world untie’

There she is. The girl in the bluedress- can you see her at theback? Almost hiding in thewings, always shy of centrestage, too modest to seek thespotlight yet proud as punchamong her peers- the YoungScientist’s past and present atthe 40th Anniversary celebra-tions. Jean Byrne and her col-league Eizabeth Dowlingremembering their greatachievement back in 1992 whenthey won the European YoungScientist of the Year. This wasJean Byrne, jeanjeanie to manyof you on the CFAI communityforum, Jeaniebop to me, aka‘eco spice’ in Lourdes.

Jean sadly died 04March2007aged just 31 years. The YoungScientist success was a featherin the cap of many great achive-ments for Jean. She graduatedfrom Trinity college Dublin withHonours BA(Mod) inEnvironmental Science in 1998and completed her MSc inEnvironmental ResourceManagement in UCD in 2000.Jean worked as a remote sens-ing specialist - analysing sateliteimagery of the world for ERAMapTec Ltd. Jean focussed her‘spare’ time on you, the FutureForce readers. She designed,wrote, compiled and edited yourfavourite magazine.

You lit up my lifeJean. I love you

always and I’ll missyou forever

xxx Rory

“Jean was always full of lifeand an inspiration to be

with”.Martin Critchely, ERA

Maptec Limited

“Jean was always a step aheadof the game, so in tune withdevelopments in CF outsideIreland and had no problembeing a ‘thorn in peoples sides’so that things might improvehere”. Patricia Duffy PWCF.

JJeeaanniieebboopp