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Born in Southampton in 1971, Tim has lived in South London for the last twenty years and is married

to Katie with a son Felix. first!time "oo# writer, Tim is a full!time $ommuni$ations mana%er and has played field ho$#ey all his life. &is eyes and heart have "een opened "y his health stru%%le with $ysti$ fi"rosis whi$h has "een a hu%e fa$tor in his life, "ut not one that defines it.

HOW HAVE I CHEATED DEATH?
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H O R T A N D M E R R Y L I F E W I T H C Y S T I C F I B R O S I S

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For my parents 'ar%aret and (ou%las, "rothers )hris and *e+, wife Katie and son Felix.

T i m Wo t t o n

HOW HAVE I CHEATED DEATH?
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H O R T A N D M E R R Y L I F E W I T H C Y S T I C F I B R O S I S

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)opyri%ht © Tim ,otton The ri%ht of Tim ,otton to "e identified as author of this wor# has "een asserted "y him in a$$ordan$e with se$tion 77 and 7- of the )opyri%ht, (esi%ns and .atents $t 19--. ll ri%hts reserved. /o part of this pu"li$ation may "e reprodu$ed, stored in a retrieval system, or transmitted in any form or "y any means, ele$troni$, me$hani$al, photo$opyin%, re$ordin%, or otherwise, without the prior permission of the pu"lishers. ny person who $ommits any unauthori+ed a$t in relation to this pu"li$ation may "e lia"le to $riminal prose$ution and $ivil $laims for dama%es. )0. $atalo%ue re$ord for this title is availa"le from the British Li"rary. 0SB/ 97- 1-1923 719 4 www.austinma$auley.$om First .u"lished 564117 ustin 'a$auley .u"lishers Ltd. 68 )anada S9uare )anary ,harf London :11 8LB

.rinted and "ound in ;reat Britain

$#nowled%ments
0 want to than# ilsa &erd for meetin% with me for that $offee in Septem"er 6414, $learin% up my tears and pointin% me in the ri%ht dire$tion to share my story. 0 also want to than# ;us ;riffin for his "ravery that day and his note that verified that my de$ision to open up was the ri%ht one. 0 want to extend my appre$iation to the authors <upert Brow and *onathan Tri%ell for their wise $ounsel and to .aul llen and Stuart &arley for their unstintin% %uidan$e and support for my aspirations. 0 have appre$iated all the advi$e from family and friends, the help with the desi%n of my "lo% and the persistent en$oura%ement to $arry on sharin%.

Foreword
0 was tou$hed to "e as#ed "y Tim ,otton to write an introdu$tion to his "oo#. &e writes "eautifully with su$h honesty, $larity and humour. &is "lo%s mar#in% the year leadin% to his 14th "irthday have had a hu%e followin%. For those affe$ted "y $ysti$ fi"rosis 5)F7 he %ives hope, and for those who don=t #now a"out )F his words are inspirational, ma#in% us aware how mu$h we $an a$hieve and en>oy whatever pro"lems arise. 0 first #new a"out )F 31 years a%o when my nie$e <a$hel was dia%nosed with the disease. ,e dis$overed that my "rother *onathan and sister!in!law )aroline were "oth $arriers of the )F %ene. <a$hel was their first $hild and it too# a few months of tests to dis$over why she was not thrivin% and puttin% on wei%ht as she should have "een. 0t was a very worryin% time. ,e were unaware of anyone in the family havin% )F so #new nothin% a"out it, and were sho$#ed to hear that <a$hel had a %eneti$, life!threatenin% disease. Ba$# then, few $hildren with the $ondition survived into their teens. 0 was livin% in the ?nited States whilst <a$hel was %rowin% up, and was unaware of the day!to!day routine that )aroline and *onathan went throu%h to maintain <a$hel=s health. The daily physiotherapy, the mountain of pills, the $onstant na%%in% fear that a $old or flu or some virus that affe$ted the lun%s would ta#e hold and redu$e her a"ility to "reathe. 0 have vivid memories of her lon% stret$h in hospital when she was a"out nine years old 5for someone with )F, %oin% to hospital is hardly ever for a short period of time.7 <a$hel had a terror of needles so the intravenous medi$ation was a ni%htmare for her. 0 am filled with admiration for the way that *onathan and )aroline en$ompassed all that was ne$essary to #eep <a$hel healthy and still mana%ed to maintain a normal family life. <a$hel was followed "y a "rother and another dau%hter, neither of whi$h have )F.

,at$hin% <a$hel %row up, it was o"vious that "ein% a teena%er with )F was a hu%e "urden and responsi"ility. *ust when a youn% person wants to fit in with their peers and "e a$$epted they have to a$#nowled%e their frailty, ta#e masses of medi$ation and spend time doin% physiotherapy to ma#e sure that they are #eepin% their lun%s as fit as possi"le. 'otivatin% teena%ers is not an easy tas#, so ima%ine tryin% to persuade a fifteen year old to %et up early so that they $an do a re%ime of exer$ise, ta#e a $o$#tail of dru%s, have their "rea#fast and then %o off to s$hool. @n$e home in the evenin%, homewor# has to "e done, and there=s no time to wat$h TA as more physiotherapy needs to "e done. nd this is not for a short period of timeB this re%ime is ne$essary every day, for life. 0t=s never!endin% and relentless. This all happens a%ainst a "a$#drop of misunderstandin% a"out )F as it=s not that well #nown to the %eneral pu"li$. nyone with the $ondition $onstantly has to interpret their symptoms to stran%ers C $ou%hin% fits in the pu"li$ domain have to "e explained, often usin% humour to dissolve diffi$ult predi$amentsD EFes sorry a"out the $ou%h, it will "e the death of meGH @nly half of those with )F will ma#e it "eyond thirty, so a 14th "irthday is an enormous milestone. For most of us, it is impossi"le to understand what it must "e li#e when you $an=t ta#e life for %ranted. 0 %ained some level of insi%ht when 0 starred in the film ILo%an=s <un=, whi$h portrayed a twenty third $entury world where no one was allowed to live "eyond the a%e of thirty. @f $ourse, it=s real life that is perpetually put in dou"t for the li#es of my nie$e <a$hel, and Tim, who have "een told that they were unli#ely to rea$h twenty, thirty or even forty years of a%e. <eadin% this "oo# "rin%s us $loser to understandin% how pre$ious ea$h day is. <ather than loo#in% too far into the future for our hopes, it=s $ru$ial to en>oy the here and now "e$ause life $an so easily "e $ut short. Tim ,otton=s "rilliant "oo# follows his personal stru%%le with )FB the day!to!day frustrations and anxieties, alon%side his survival strate%ies, all underpinned "y the a"solute >oy of

defyin% the odds and livin% life to the full. For anyone affe$ted "y )F or fa$in% any form of life $hallen%e this is a $ompellin% and heartenin% story. For everyone it offers hope and inspiration. 0 $ele"rate with Tim his unli#ely "ut triumphant 14th "irthday and wish him all the "est for the next forty yearsG Jenn A!"tter C #ti$ Fi%ro#i# &' ()tron

(re*)$e
At %irt+ in M)r$+ ,-.,/ I w)# di)!no#ed wit+ t+e 0i*e1 t+re)tenin! i00ne## $ #ti$ *i%ro#i# 2CF3 )nd not e45e$ted to 0i6e m"$+ 5)#t m teen#7 )F is a serious %eneti$ disorder with redu$ed life expe$tan$y. 0t is $aused "y mutations in the $ysti$ fi"rosis trans!mem"rane $ondu$tan$e re%ulator 5)FT<7 %ene, whi$h re%ulates the produ$tion of mu$us, sweat and di%estive en+ymes. For a person to have )F, "oth his or her parents have to "e $arriers of this mutated )FT< %ene. The name $ysti$ fi"rosis refers to the $hara$teristi$ s$arrin% 5fi"rosis7 and $yst formation within the pan$reas, first re$o%ni+ed in the 1934s. 0t is one of the ?K=s most $ommon life threatenin% inherited diseases, affe$tin% over 9,444 people. 0n the ?nited States, approximately 34,444 people have )F. round 1,444 new $ases of )F are dia%nosed ea$h year. /ot su$h a lon% time a%o, the dia%nosis of )F meant an early death. ;ermani$ fol#lore has a sayin%D E $hild who tastes li#e salt when #issed on the forehead will soon die.H Before the 1984s, the pro%nosis on )F was so ne%ative that do$tors hardly "elieved that a $hild with this $ondition $ould live "eyond seven years of a%e. The life expe$tan$y of )F patients has "een in$reasin% over the past 14 years. The $ondition affe$ts the internal or%ans, espe$ially the lun%s and di%estive system, "y $lo%%in% them with thi$#, sti$#y mu$us. This ma#es it hard to "reathe and di%est food. (iffi$ulty "reathin% is the most serious symptom and results from fre9uent lun% infe$tions that are treated with, thou%h not $ured "y, anti"ioti$s and other medi$ations. multitude of other symptoms, in$ludin% sinus infe$tions, poor %rowth, diarrhoea, and infertility result from the effe$ts of )F on other parts of the "ody.

:a$h wee# in the ?K, five "a"ies are "orn with the $onditionB however, ea$h wee#, three youn% lives are also lost to it. Aast improvements in treatment have in$reased the life expe$tan$y of patients, "ut $urrently there=s no $ure for )F. s my lon%evity in this world was perpetually in dou"t, it had a dramati$ effe$t on what 0 did, how 0 felt and my priorities in life. This "oo# depi$ts the $ountdown to my landmar# 14 th year in diary format with the $lo$# ti$#in% from the day 0 turned 39 to the day 0 rea$h 14, as 0 unpi$# how 0=ve $heated death for so lon% and lay out my formula for survival. This is the first "oo# written "y anyone with )F who has rea$hed this si%nifi$ant a%e milestone. ,hen people loo# at me, they see a married man with a %or%eous wife and son, wor#in% full!time in London and playin% re%ular sports. ,hat they don=t see is my $hroni$ illness, whi$h $lo%s up my lun%s and di%estive system with a thi$#, sti$#y mu$us. 0t ma#es it hard to "reathe, exer$ise and di%est food. ll my life C every day C has "een a "attle to defy the odds around my life expe$tan$y, $urrently set at 16 years C thou%h for a lon% time it was fixed at 34. 0 #new that life was li#ely to "e short, and havin% my own property, wife and $hild seemed unattaina"le. 'y very existen$e has always seemed held in an hour%lass with the %rains of sand runnin% out fast. :very hour of every day is important as 0 never #now when my hour%lass will run out of time. 0 underta#e heavy and demoralisin% daily medi$ation whi$h "rin%s re%ular dar#, sad and frau%ht moments. 0 $annot afford to let up or $ut any $orners medi$ally, as any "i% lapse $ould "e my undoin%. 0t is relentless C li#e havin% a heavy $hest $old every day of your life C and unfor%ivin%, #illin% many sufferers early. Throu%hout my life, 0=ve had the misfortune to see many of my $ontemporary fellow sufferers pass away in adulthood, teens or early twenties. To $ompound my life, )F is not that well #nown, understood, and $annot "e easily seen or ever properly

ima%ined "y most people. 0t=s li#e a form of Ilo$#ed!in syndrome= as all the dama%e is on the inside and not very o"vious to the na#ed eye. &istori$ally, 0 tended to suppress all my emotions a"out )F and "e very %uarded a"out dis$losin% it to stran%ers. 0 never wanted to "e viewed as the Iill person= and "e defined "y my $ondition and 0 $ertainly never wanted anyone to feel sorry for me or "e pitied. But after so many years of se$re$y and prote$tion, there was a hu%e amount of unresolved sadness and emotion under the surfa$e. s 0 defied the odds to approa$h this milestone, 0 had an epiphany that survivin% )F has "een the "i%%est a$hievement in my life, "ut it was hidden away and 0 wasn=t tal#in% a"out it or usin% it for my own or others= "enefit. The addition of dia"etes to my medi$al re%ime reinfor$ed the feelin% that my survival was somethin% to "e $ele"rated and shared. /ow 0 wanted to spea# up... 0 de$ided to ta#e the lid off of my own .andora=s Box and #eep a diary in the year leadin% to my 14th to properly refle$t, for the first time on my life C past, present and future C my trials and tri"ulations, happy times and what it has ta#en for me to survive this $hroni$ illness for 14 years. 0 $hose not to loo# "a$# in an%er "ut to loo# forward with positivity. s 0 stirred my $ons$ien$e, 0 "e%an a >ourney of dis$overy that awa#ened my soul to the overridin% desire to share my hard!fou%ht "ut enri$hin% life!lessons of hope as a form of $atharti$ pro$ess for myself and also to help others. t the same time, 0 wanted to in$rease the awareness and understandin% of )F and share my "i%%er pi$ture of life perspe$tive, perseveran$e and optimism with a wider audien$e. @ne prime driver was to inspire people that even at your lowest e"" with the odds sta$#ed a%ainst you, that there is usually a way to over$ome. There=s a sayin% that Iwhat $an=t "e $ured, needs to "e endured=. By endurin% all my life, 0 realised 0 had some extraordinary stories to share and some uni9ue life insi%hts. 0 wanted to hi%hli%ht the approa$h 0 have honed over the years that #eeps me alive and #i$#in%, where )F does not define me

"ut is >ust somethin% 0 have to I%et on with= to lead my "usy life. :a$h $hapter is a diary extra$t whi$h $overs the full spe$trum of my daily life with flash"a$#s to my past and survival se$rets C how 0 have $heated deathJ Tim Wotton

,7 Li6in! t+e Dre)m
Early March 2010
0=ve "een havin% the same re$urrin% dream for nearly 14 years. $tually, it feels more li#e a ni%htmare than a dream. 0t=s pretty damn fri%htenin% and 0 wouldn=t wish it on my worst enemy. 0n this ever!repeatin% dream, 0=m held prisoner in my own version of ;roundho% (ay where it appears 0 have su$h a fero$ious illness that 0 have to "e ta#in% a multitude of medi$ation from the instant 0 wa#e to the moment 0 put my head down at ni%ht. 0 am always $ou%hin% and it feels li#e havin% the effe$ts of a heavy $hest $old every sin%le day, so it must "e some form of lun% $ondition that never %oes away. 0n my dream, as soon as 0 wa#e up, 0 rea$h into my "edside drawer, whi$h is li#e a mini $hemist=s shop, and 0 pi$# up the first of many dru%s. The dru%s don=t seem to "e re$reational. 0 really seem to need them. ,here a smo#er=s initial instin$t on wa#in% is to %ra" their pa$#et of $i%arettes, mine is to start ta#in% a $olourful mix of anti"ioti$sB all different shapes and si+es. Before 0 even ma#e myself a $up of tea in the mornin% 0=ve ta#en 18 ta"lets. Then 0 have to use a whirrin% ne"uli+er devise $ontainin% a medi$ine. This produ$es a fine mist throu%h a mouthpie$e whi$h 0 "reathe in for 14 minutes. Followin% this a$tivity 0 lie "a$# down on my "ed. /ot for a rest "ut so that 0 $an pat my $hest with my hands to "rin% up mu$us whi$h seems to "e waitin% to "e $ou%hed up C it=s always lur#in% thereB >ust waitin% to $ome up. 0t=s pretty dis%ustin% really to "e produ$in% su$h phle%m so early ea$h mornin% "ut in my dream it seems perfe$tly normal to me. )uriously, 0 %et the feelin% that 0=ve never smo#ed one $i%arette in my life so this lun% related $ondition

has not "een "rou%ht on "y my own a$tions C it=s >ust there, is part of me and my (/ . @$$asionally, 0 $ou%h so hard that 0=m si$# on myself, mainly >ust li9uid "ut nonetheless pretty awful and a %rim start to my day. 0ma%ine itK you wa#e up and within 64 minutes you=re si$# on yourself C not an inspirin% thou%ht really and an utterly depressin% openin% to anyone=s day. Both up and down my house there are drawers and areas full of dru%s and medi$al e9uipment C some are stashed away and some are on show for ease of use. Li#e an al$oholi$ with se$ret stashes of vod#a dotted around the house, 0 have medi$ation hidin% pla$es spread everywhere. 'ay"e 0 live in a show home for a pharma$euti$al $ompany and 0=m their medi$ation %uinea pi%J 0=m still not finished with this $ra+y $lini$al s$hedule as next up 0=m pri$#in% my fin%er with a sharp lan$elet in order to $he$# my "lood su%ar levels via this little %ad%et. This %ives me a s$ore that 0 need to ta#e time to re%ister and >ot down in a note"oo#. t last, 0 $an have some "rea#fast "ut that is a$$ompanied "y even more ta"lets and an insulin in>e$tion into my stoma$h, whi$h tends to "e a "it painful if 0 %et the an%le of the needle wron%. Sometimes when 0 withdraw the needle, 0 "leed from the point of in>e$tion whi$h if not wiped up invaria"ly ends up on my $lothin%. The drama never $eases with this $onditionK @n the worst mornin%s, 0=ve "een "oth si$# and "led on myself and 0=ve not even %ot showered, $han%ed or even left my house yet. 'ana%in% this illness is non!stop and 0 seem to need the most ama+in% patien$e not to %et wound up or annoyed "y the whole thin%. 0 seem to do all this so systemati$ally as if it was a $hore li#e "rushin% one=s teeth. This dream $arries on throu%hout my day where there=s a similarly ri%orous routine at lun$htime and in the evenin%. t wor#, 0 usually $on$eal my medi$ation C "lood test, pills and in>e$tion C from those around me as 0 don=t want to draw too mu$h attention to the whole pro$ess.

0n the evenin%, the pattin% on my $hest ta#es lon%er and 0 have even more se$retions to $ou%h up. lso, over the years, there have "een many people 5male and female7 who have helped me with this evenin% physiotherapy C for the last de$ade, it=s "een my wife whi$h feels fun$tional and not at all romanti$. nnoyin%ly, this tas# often means that the food we are $oo#in% that ni%ht is over!$oo#ed or "urnt and we eat later than we would li#e. Before 0 #now it, 0=m "a$# at my "edside poppin% pills from my $hemist=s drawer "efore 0 turn the li%ht out. 'ost days are $ompletely exhaustin% as a result of the $ontinuous $ou%hin%. 0 have a sense that 0 want to "e more spontaneous and "rea# free from this serial dru% ta#in% "ut my $ons$ien$e won=t let me for%et any of my treatments and the re%ime is adhered to reli%iouslyB li#e $lo$#!wor# every day. 0=m on some #ind of $ra+y medi$al merry!%o!round and there=s no way to $lim" off. T+en I w)8e "5 *rom m dre)m9 The realisation dawns on me that this is not a$tually a dream, "ut it is in fa$t my harsh reality livin% with and $opin% with $ysti$ fi"rosis 5)F7 and )F!related type 1 dia"etes. 0t=s always "een thus. 0 am 9uite literally Ilivin% my dream=. 'y ;roundho% (ay starts and ends with pills and 0 $an only survive "y "ein% utterly $ommitted to this punishin% daily routine. 0n essen$e, my existen$e on this planet is %overned "y the ne$essity of a $o$#tail of around 14 ta"lets ea$h day, intravenous treatments for three wee#s at a time 5the )F version of $hemotherapy7, ne"ulisers, lon% physiotherapy sessions, asthma inhalers and re%ular visits to hospitals and the pharma$ist. #no$#!on effe$t of a life!time of medi$ation is that nearly half of my dru%s re%ime is needed to $ounter the side effe$ts of the anti"ioti$s and 64 years of steroids has led to the development of my dia"etes and osteoporosis.

For the first time in my life 0 feel the desire to ta#e sto$#. 0t %ot me thin#in% a"out my overall inta#e of dru%s durin% my life, so 0 %ot a pen and paper and started to wor# out the )F num"ers. s a rou%h $al$ulation over >ust the last 68 years, 0 have swallowed :;< ta"lets every wee#, whi$h e9uates to ,=/>?< a year and @?=/<<< for the whole period. So it=s $lear 0=m very %ood at ta#in% pills and 0=ve %ot my te$hni9ue all lo$#ed down. 0ndeed, 0 mi%ht well "e a human rattle. 0 wonder what the world re$ord is for the amount of ta"lets ta#en in one mouthful. 0t=s pro"a"ly held "y one of us )F %uys. @K, 0 #now it=s not somethin% that 0 $an add to my )A, "ut if there=s an :n%lish team for pill poppin%, my name would "e in that s9uad. 0 do have to "ite my ton%ue not to lau%h when people tell me that they suffered ta#in% a few ta"lets in a day or when they stru%%le to remem"er their one ta"let a day of pres$ri"ed medi$ation. The most illuminatin% are those that $hoose not to use anti"ioti$s as they want their "ody to fi%ht the infe$tion. ,hat luxury it is that they $an "e so do%mati$. (o 0 thin# a"out anythin% when 0=m ta#in% my medi$ationJ /ot really, 0 >ust do it. 0=ve had to put aside any ethi$al "arriers, thou%h 0 will 9uestion and $hallen%e my do$tor and 0 won=t add to my re%ime without %ood reason. lso durin% this time, 0 have had ,</<<< ne"uliser sessions, ,;/:<< physiotherapy sessions, over >< 0A treatments, ?<< visits to my $hemist and :>< appointments at Frimley .ar# hospital. )<0)K:FG ,hen 0 loo# at my )F in plain num"ers, it=s sho$#in%ly dauntin% and surreal. &ave 0 really ta#en all those ta"letsJ &ave 0 had that many physiotherapies, 0As and trips to medi$al $entresJ ,as that all meJ 0 expe$t for someone who doesn=t visit a do$tor or ta#e a pill that often, those rare o$$asions are 9uite memora"le and $hallen%in%K and worth %ripin% a"out. s 0=ve spent so many hours of my life $o$ooned in this )F hell, only the really sho$#in% moments stand out. 0 thin# "y

default, 0 "lo$# out the ma>ority of my sufferin% as a way of prote$tion from the daily %rind of it all. 0 thin# if 0 dwelled upon every moment where my illness has to ta#e $entre sta%e in my life, 0 would $ome undone very 9ui$#ly. ll this merely maintains my $urrent state of health as there is no $ure yet for )F. 0 $annot afford to let up on it or $ut any $orners medi$ally, as any "i% lapse $ould "e the end for me. This $ondition is $ompletely unfor%ivin% and relentless C li#e an in$omin% tide "atterin% a%ainst a sand$astle. Trapped "y treatment >ust to stay alive, 0=m 9uite literally trapped in my own ;roundho% (ay. 0=ve not had a day off from this illness in nearly 14 years C fittin% in my life, wor#, family, sport around this dauntin% and in$essant routine. By sti$#in% to this endless re%ime, 0=m >ust a"out stayin% afloat C $onstantly treadin% water C 0 daren=t stop swimmin% for a se$ond in $ase 0 %o under the surfa$e, never to return. ,hile thin#in% a"out the relentless nature of my illness, 0 had the most sho$#in% thou%ht "ased on the )F version of the film ITwo ,ee#s= /oti$e= C what would happen to me if 0 stopped my treatments for >ust two wee#sJ &ow ill would 0 "e$omeJ :ven when 0 miss the odd treatment the effe$t on my health is pretty noti$ea"le. 0=m pretty sure that after two wee#s of livin% without due dili%en$e, 0=d "e in hospital and really stru%%lin%. ,ith su$h a $ondition to $ontend with, in every way, 0 firmly "elieve that 0=m only as healthy as my last treatment. 0t is a%ainst this daily "a$#drop that 0 survive ea$h day, "attlin% a%ainst the odds that this horri"le illness heaps on me. .eople a$ross the world aspire and strive to Ilive the dream=. ?nfortunately 0=m livin% mineG 0 $an only hope that one day when my mornin% alarm %oes off, that somethin% would have $han%ed and the ni%htmare ;roundho% (ay spell will have "een "ro#enB and 0 $an live a normal life untou$hed "y medi$ation and $lini$al re%ime. ?ntil that day arrives, on hearin% that alarm, 0=ll #eep rea$hin% into my "edside dru%s drawerK

:7 Co"ntdown Be!in#
Wednesday 17 March 2010
0t=s my 39th "irthday today. 0 too# the day off wor# to have an ex$ursion with Katie in London. 0t was one of those lovely and rare days out to%ether now that we are parents. ,e en>oyed a pleasant wal# whilst holdin% hands in St. *ames .ar#, remem"erin% a%ain what "ein% a $ouple is all a"out. This was followed "y wat$hin% the peli$ans flirt with tourists while eatin% our pi$ni$ on a par# "en$h. The hi%hli%ht of the day was the matinee performan$e of ILes 'isera"les= at a Shafts"ury venue theatre C my first time and 0 loved it, li#e so many "efore me. fter $lappin% to a standstill at the finale, Katie and 0 meandered out of the theatre into a "ustlin% .i$$adilly )ir$us with the $urious deli%ht of an evenin% stret$hin% ahead with no need to rush. n aperitif at the @pera Bar in )ovent ;arden pre$eded a deli$ious Thai meal. ,hat a lovely day C a perfe$t day. 0 also made a phone $all to my twin "rother, *e+, with whom 0 share my spe$ial day. @"viously, this is the person 0=ve #nown all my life sin$e we first met 39 years a%o a$ross a $rowded wom"G 'y twin loo#s nothin% li#e me C he is tall, dar# and handsome and loo#s more li#e my elder "rother )hris. So what happened to meJ 0=m short and "londeG 0f 0 wasn=t a twin there mi%ht "e some serious 9uestions to "e as#edG 0 do "ear a resem"lan$e to my mum=s father so 0 #now 0=m safe in that respe$tK 'ost people view their "irthdays as spe$ial and en>oy the attention that they "rin% from family and friends. 0=m no different in this respe$t. 'y "irthdays mean a %reat deal to me as ea$h one si%nifies a %lorious a$hievement. :a$h one $ele"rates the simple fa$t that 0=m still here.

ll my life C every day C has "een a "attle to stay alive and #eep defyin% the odds around my life expe$tan$y 5$urrently set at late thirties for )F sufferers, althou%h for a lon% time it was fixed at thirty7. &ow does that feel to have one=s very existen$e always in dou"tJ )onsider one=s life expe$tan$y "ein% held in an hour%lass. /ow ima%ine that the %rains of sand in your %lass appear to "e runnin% out very 9ui$#lyK &ow would that feelJ ,hat would you do differently or prioritise "efore your sand ran outJ 0n my twenties 0 saw only a hopeless end. n early death seemed inevita"le. 0 made the most of today "e$ause tomorrow mi%ht never have arrived. This always reminded me of the 1972 film ILo%an=s <un= whi$h depi$ts a future so$iety in whi$h the population is maintained in e9uili"rium "y re9uirin% the death of everyone rea$hin% a parti$ular a%e. ,hen the palm flower $rystal em"edded in the palm of everyone=s ri%ht hand $han%es $olour from red to "la$# people have rea$hed the I Lastday’ and are exe$uted. 0n my real!life version of the film, how lon% $an 0 #eep runnin% away from the )F %rim reaperJ For most of my life thirty seemed una$hieva"le and forty felt an impossi"le a%e to rea$h. This $on$ern and fixation over my life expe$tan$y was exa$er"ated after the sad deaths of $ertain )F friends leadin% up to my 34th "irthday. ,ith ea$h departure, 0 9uestioned why 0 was still alive when they were not. How had I cheated death? How many more birthdays would I have? Would I ever live lon enou h to !eel over the hill rather than si" !eet under it? ,hen 0 finally made it to thirty, it felt li#e 0=d rea$hed the &oly ;rail. 0t was $ertainly a tippin% point in my life. 0 wanted to $arry with me the spirit of my friends who had died, and ma#e my lon%er life $ount for somethin%. ,hen so mu$h of a )F life is not at all normal, livin% "eyond thirty was extremely

fulfillin%. 0t=s somethin% that my healthy peers too# for %ranted "ut for me it was "rea#in% the )F "oundaries. There are not too many people with )F that 0 #now in the ?K who are over forty and it always felt inspirational when anyone rea$hed this si%nifi$ant milestone. (ue to my life!lon% "attle for survival, 0 am hu%ely sentimental and 0 parti$ularly want to do somethin% memora"le on my "irthday. 0 want to ma#e ea$h one $ount for me and those around me. 0 see ea$h "irthday as a time of $ele"ration for my family, friends, medi$al staff and $omplimentary therapists who have supported me and have "een su$h a valua"le part of my prolon%ed existen$e. s 0 loo# ahead to my 14 th "irthday, it feels natural to also loo# "a$# and refle$t on my previous "irthdays and the >ourney 0=ve "een on. @ne milestone that feels li#e a watershed was my 17 th "irthday when 0 was feelin% unwell and under%oin% a heavy intravenous treatment. This meant that 0 $ouldn=t %o out to party with *e+ and our mutual "est friends. :very fi"re of my "ody was s$reamin% with an%er and frustration a"out the apparent unfairness of stayin% in while my friends partied. 0 vowed never to miss out on future "irthdays. 'y "irthdays $ould never "e >ust another day. :a$h one was %oin% to "e a $ele"ration of my life C a life worth livin% and well!lived. <ather fortuitously, my "irthday, 17 'ar$h, $oin$ides with the 0rish national holiday St. .atri$#=s (ay C a day to "e >ovial and to let one=s hair down. For many of my "irthday evenin%s, 0 have tried to out drin# the 0rishK "ut it should "e noted that 0=ve always failed misera"ly. s well as or%anisin% a$tivities durin% the day, *e+ and 0 have always "een in$lined to or%anise a party for the ma>ority of our "irthdays with extra "i% venues and wider invites for the #ey milestone a%es of ei%hteen, twenty one, twenty five, thirty and thirty five. There=s a sayin% that Iif you "oo# them, they will $ome= and my friends have fed off my infe$tious desire to

$ele"rate life to the full and have as mu$h fun as possi"le. ,hen 0 invite them, people have tended to flo$#. 0 am fortunate to have very faithful and relia"le friends. 0=d li#e to thin# that an a"idin% memory for most of my friends will "e of me ta#in% $entre sta%e with a pint in one hand and a rum and $o#e in the other lau%hin% and en>oyin% myselfB literally s9uee+in% every drop out of lifeG There have "een o$$asions when 0=ve %one too far with my Ipartyin%=. @n my 61th "irthday, 0 was so drun# that 0 was physi$ally $arried shoulder hi%h "y my flatmates out of a pu" in .utney to a $a$ophony of hysteri$al $heers from the 0rish thron%. This was definitely an example of triumph of the spirit C unfortunately it was a pint of different spirits that did for me that ni%htG To ma#e matters worse, 0 threw up in the taxi on the way home and was in a horri"le state the next day when 0 played ho$#ey in 'aidenhead. 0 was still "ein% si$# durin% the pre! mat$h team tal# whi$h $onsisted of the sound advi$eD E(on=t pass the "all to TimGH &avin% somehow %ot throu%h the %ame, "a$# in the opposition $lu"house $anteen, 0 sat on my own meti$ulously eatin% a "owl of soup while the rest of the players $heered on :n%land in a Five /ations ru%"y %ame next door. nother time, followin% a drin#!fuelled "irthday "ash on rea$hin% twenty nine, 0 %ot extremely emotional and melan$holi$ outside a .utney ni%ht$lu" with Sasha, a ho$#ey friend. .erhaps this $ould "e attri"uted to the underlyin% sadness 0 felt that the Iuna$hieva"le= a%e of thirty was loomin% whi$h $ould hasten my potential demise. But as 0 approa$h the forty year milestone, in 'ar$h 6411, somethin% has $han%ed in meK 0 have flipped my mindset from a fear of dyin% to a desire to live for mu$h lon%er as there is so mu$h 0 still want to see and do. This was immeasura"ly ma%nified when 0 "e$ame a hus"and to Katie in 6443 and a father to Felix in 6447. 0 now view the future in a different way C wel$omin% ea$h year. 0 mentally pro>e$t and expe$t a lon%er life with my own family to live it with. 0 firmly "elieve that the "est "irthdays of all are those that haven=t happened yet.

0 see rea$hin% forty as a $ele"ration of my life to date and what it has ta#en for me to surviveB "ut 0 also view it as a laun$h pad for the rest of my life C with my hour%lass half full rather than half empty. 0 would $ertainly want to $ele"rate my 14th milestone with the mother of all parties. 0f %ettin% to forty was the e9uivalent of $lim"in% 'ount :verest than 0=m on the as$ent with the summit in si%ht C almost mysti$al throu%h the $louds. But will 0 ma#e it to the topJ For ea$h of us, transitionin% to a new de$ade is a reason for >u"ilation. For me, it is a$hievin% the impossi"le. The $ountdown to my 14th offi$ially "e%ins and 0=ve %ot to ma#e it, espe$ially now that 0=ve started this >ournalG

@7 Si4t Fi6e Ro#e#
#riday 1$ March 2010
0t=s un$anny in life how one situation leads dire$tly to another. t a $lient meetin% at wor# today, 0 had to deal with the $lassi$ )F s$enario and its #no$#!on dilemmas. Someone made a comment about my cough… 0t was a fairly inno$uous remar# from this wor# $lient who didn=t #now me that well. ,e were in a meetin% room to%ether dis$ussin% a parti$ular pro>e$t plan when 0 felt the need to $lear my throat. w#wardly, it sounded pretty loud and then tri%%ered a sustained $ou%hin% stint. %&ou h any harder and you’ll et a old watch'( was the $li$hL $omment from the $lient. 0n my experien$e, this is 9uite a $ommon phrase when someone hears a ha$#in% $ou%h. &e wasn=t "ein% mali$ious and may have "een surprised "y the tena$ity of my $ou%hin%. By $ra$#in% a >o#e he was pro"a"ly loo#in% to ma#e li%ht of the situation. ,hat do 0 say in returnJ (o 0 i%nore it or ta$#le it there and thenJ ,hat=s the li#elihood he would have ever heard of my illness anywayJ 'y eternal dilemmaK This %ot me thin#in%D ,hat=s with the )F $ou%h and why is the $ondition so diffi$ult to explain to stran%ersJ For most people with )F the need to $ou%h 5to help $lear se$retions7 is the most $onsistent irritation. This does vary for every sufferer as there is not a one!si+e!fits!all version of )F. 0n fa$t there are over 1444 variations of the $ondition, some a lot more severe than others. The pro"lem is that 0 $an=t always predi$t when a throat ti$#le will turn into a full!"lown $ou%hin% atta$#.

0ma%ine the start up $ou%hs as tremors and the "i% $ou%hin% fit that inevita"ly follows as the a$tual earth9ua#e with a similar <i$hter s$ale of severity. @n$e the main eruption has o$$urred, that=s not the end of it as there $ould "e hours of mini after!sho$#s. ,hen 0=m $ou%hin%, 0=m sure the %eneral pu"li$ in my vi$inity, whether it "e on pu"li$ transport, in the offi$e or on a sports pit$h, thin# that 0=m annoyin% and possi"ly $allous to "e out with su$h an awful infe$tion C surely 0 should "e at home tu$#ed up in "ed when 0 sound so awfulJ Some mi%ht even despise me that 0 $ould "e infe$tious and spreadin% my %erms near them. 'ore ironi$ally, there $ould "e some people who even thin# that 0 have $aused this myself and the noise they hear is a smo#er=s $ou%h C whi$h $ouldn=t "e further from the truth. Fou are more li#ely to $at$h a ve%an mun$hin% on a "a$on sandwi$h than me smo#in% a $i%aretteG Sometimes after some sustained $ou%hin%, my larynx $an "e ti%ht and painful whi$h $auses ea$h su"se9uent $ou%h to $ome out in a hi%h!pit$hed tone. .eople $an often $onfuse this noise with a snee+e so with ea$h $ou%h 0 %et EBless youGH from the nearest person to me. 0=m sure their #indness runs out after my umpteenth Isnee+e= as they stop sayin% anythin% after a while and pro"a"ly sit there wonderin% why on earth 0 don=t sort it outG The sin%le a$t of $ou%hin% for someone with )F $an at times "e a reflex su"$ons$ious a$t. 0 will use a sin%le $ou%h to sense $he$# how my $hest and lun%s feel at any %iven moment C li#e revvin% the en%ine of a $ar. 0f it=s a dry $ou%h, then 0 $an relaxB if it sounds $hesty then 0 $an prepare myself for the need to %o to the toilet to $lear my se$retions. Similar to how new"orn "a"ies have their own distin$tive $ry that only their mums $an hear from afar, )F sufferers tend to have their own si%nature $ou%h. 0ndeed, it is the predominant si%ht and sound of my illness. 0f 0 was "ein% mis$hievous, 0 would pla$e a lar%e "et that there are no "ur%lars in this world with )FG

0 $an "e heard "y my wife, son, family and $lose friends "efore they even see me. %I )new where you were' I could hear your cou h*( 0t=s not all "ad and it $an "e handy. 'y wife Katie listens out for my $ou%h in pu"li$ pla$es to tra$# me down if 0=ve %one ,@L. 0n a supermar#et, she #nows whi$h aisle 0=m on when 0=ve %one off the "eaten tra$# C usually findin% me in the sweets and "is$uits se$tion where 0 >oin other dia"eti$s who pil%rima%e to reminis$e of a "y%one era. ,hen ta#in% an exam at ?niversity, a %ood friend re$ounted afterwards that he %ot so frustrated with his ina"ility to answer the 9uestions that to stave off "oredom, he $ounted my $ou%hs on his notepad. 0 was %lad to "e of help to the poor $hapG There are $ertain pu"li$ pla$es where it really is a pain for a $ou%hin% fit to start. Aenues where silen$e is a pre!re9uisite are the worst. )inemas, theatres and li"raries are some of the pla$es where a I)F earth9ua#e= has $aused me and those around me mu$h annoyan$e. $ou%hin% session $an feel li#e holdin% one=s "reath when swimmin% in an underwater tunnel C no one $an help me %et throu%h it C only 0 $an %et to the other end, whi$h in my $ase is to %et "eyond the extended ha$# and "reathe normally a%ain. 0 $an "e prone to $ou%hin% so hard at times that my head feels li#e the inside of a $hur$h "ell when it=s $lan%in%. Thou%h lu$#ily my head is still in pla$e at the end of itG 0t $an=t "e ni$e for others to listen to this infernal noise "ut when 0=m in mid ha$#, to "e honest, 0 $ouldn=t $are too mu$h if 0=m upsettin% anyone else. They only have to hear my $ou%h for a few se$onds. 0=m now approa$hin% 14 years of livin% with it. 0 #now whi$h one 0=d rather deal withK Stran%ely enou%h, 0=ve never as#ed anyone to find out their feelin%s on it, "ut this pu"li$ $ou%hin% must "e worse for )F females as it=s not exa$tly feminine and sexy. 0 tend to $ou%h or $lear my throat durin% the $ourse of most days and this is inflamed "y exer$ise "ut parti$ularly "y $old and damp weather. Some days are mana%ea"le and 0 don=t

$ou%h too mu$h. But other days, often frustratin%ly for no o"vious reason, my lun%s are a"solute sho$#ers and play up li#e nau%hty $hildren. 0n some ways, )F $ou%hin% days are li#e Forrest ;ump=s prover"ial "ox of $ho$olates C %+ou never )now what you’re onna et ne"t,( This $ou%hin% malar#ey is not >ust a day a$tivity. 0 have had the odd ni%ht where 0 wa#e up $ou%hin% so hard that 0=m s$ared that 0=ll $ho#e to death and 0 #eep myself awa#e petrified "eyond "elief that if 0 fall asleep, 0 won=t "e alive in the mornin%. 0n those very dar# moments, it=s not lost on me that the words I$ou%hin%= and I$offin= sound the same. ,hen 0 wa#e up in the mornin%, there is a palpa"le sense of relief and euphoria that 0=m still alive. But after a "ad ni%ht or any health set "a$#, 0 feel small, insi%nifi$ant and my self! esteem is low. 0 want to "e invisi"le. So "a$# to my dilemma of how to respond to my wor# $lient=s remar#D %&ou h any harder and you’ll et a old watch*( (o you #now that if 0 had a pound for ea$h time 0 have heard this, whether out and a"out or playin% sport, 0 would have enou%h money to "uyK that %old wat$hG By default, 0=m usually pretty %uarded a"out dis$losin% my $ondition to stran%ers and normally only mention my )F on a need to #now "asis in professional situations. 'y mind is s$ram"led "y what 0 would li#e to tell them to firmly put them in the pi$ture versus %lossin% over it and #eepin% it at an elementary level. fter so many years handlin% this s$enario, 0 have %ot a fairly %ood sense for what the person in front of me $an a$tually $omprehend or sometimes more importantly what they want to ta#e on "oard. 0n this respe$t, some people exude warmth and others >ust don=t C 0 refer to the $old ones as the %I’m alri ht -ac)( people. :ither way, 0 tend to %ravitate to whatever level they=re at in relation to what 0 $hoose to reveal a"out my illness. So$ially, 0 have always felt at odds with introdu$in% myself and my illness in the same "reath, so 0 tend to wait for people to %et to #now me firstB and when 0 feel it is appropriate 0 %ive them the low down without ma#in% too mu$h fuss over

it. *ust o$$asionally, people 0 tell have %ot a $urrent or past $onne$tion to )F and are reasona"ly #nowled%ea"le. Sometimes they #now a )F person from s$hool or ?niversity C more often than not, they re%retfully inform me that their friend is no lon%er with us. The worst s$enario followin% my )F explanation to a stran%er is when 0 am met "y silen$e C may"e they are stunned that 0 have it, don=t #now enou%h to $omment or $ouldn=t $are less and want to i%nore it C as 0 feel in$lined to fill the silent %ap and end up prattlin% on and sayin% too mu$h. /o, in this $ase, silen$e is not %olden. 0n life one is prone to storin% up a $ertain response if anyone says somethin% inflammatory to you on a $ertain su">e$t B?T the situation never arises so your witty retort lays dormant. ,ell, if someone ever said to meD %+ou’re cou hin badly'( 0 would "e tempted to reply in >estD %I’m sur.rised to hear you say that I’m cou hin badly' as I have been .ractisin all day,( So today, 0 ended up sayin% to this $lientD %E"cuse my cou hin / I’ve ot a .retty severe lun condition* 0ot sure i! you have heard o! it* It’s called cystic !ibrosis*( nd thus the se$ond dilemma is $reated. )ysti$ whatJ (espite its severity and prevalen$e, the words I$ysti$= and Ifi"rosis= do not mean mu$h outside of the medi$al fraternity, the sufferers themselves, their family and $lose friends. 0n fairness, the two words don=t really resonate and are hard to say C one youn% patient $ouldn=t pronoun$e it and settled for $allin% her illness Isixty five roses=, whi$h sounds near enou%h. 0n my experien$e, )F is li#e a form of Ilo$#ed!in syndrome= as all the dama%e is on the inside and not very o"vious to the na#ed eye C all the ruin is happenin% on the inside and is not visi"le. 'y heavy daily medi$ation re%ime mana%es a $ondition that is not well understood, $annot "e easily seen or ever properly ima%ined "y most people. The life of a )F person $an

"e des$ri"ed, B?T it $annot "e easily ima%ined for the ma>ority of people. ll this for the "lan# loo# 0 re$eive when revealin% my $ondition to most stran%ers C that loo# where they nod their heads "ut are thin#in% %+ou lost me at cystic but I better )ee. loo)in li)e I understand and care*( EBy the time 0 %et to sayin% Ifi"rosis=, most people=s eyes have already %la+ed over. That loo# pun$tuates the entire life of someone with )F. 0 find it sadly ironi$ that 0 follow my daily re%ime with military pre$ision >ust to stay alive and pra$ti$ally no!one has a $lueG 0f 0=m honest, 0=m not surprised that most people have little or no idea a"out the $ondition "ut after 14 years it does %et frustratin%. .eople don=t realise how serious the $ondition is and that it=s a #iller and ta#es people in their youth. @ften they re$o%nise the name and possi"ly #now that it=s related to lun%s or needs physiotherapy, "ut not mu$h "eyond that. To the untrained eye, 0 don=t loo# si$#. 0n fa$t 0 $an appear so well on the outside that even $lose friends $an for%et 0 have one of the most $ommon life!threatenin% inherited diseases. There=s an odd di$hotomy here as 0 want to loo# well "ut also want people to #now that 0 am ill underneath my s#in and what it ta#es for me to survive. 0t would "e %reat for my $ondition C the one that 0 fi%ht so hard to survive every day of my life C to "e more universally understood and re$o%ni+ed. 0t=s tou%h to #eep %ivin% your all to a $ause that re$eives a "lan# loo# from the ma>ority of fol#. ?ltimately 0 wanted to edu$ate people a"out what it=s li#e to live with this disease and that it=s no flu#e 0=m still around. 0=ve had to fi%ht "loody hard to stay alive. 'ay"e in the future, after readin% this, you=ll remem"er Isixty five roses= and #now a little "it moreK