Table Of ContentIssue 2: Winter 2013/14
Marking the Trust’s 50th
Tackling adherence to
transform lives
Trials or tribulations?
The magazine of the Cystic Fibrosis Trust
We won’t celebrate being 50 until everyone can
The Cystic Fibrosis Trust is 50 years old. But we’re not about to throw a party.
Although we’ve achieved a lot in our frst 50 years, we haven’t yet achieved our ultimate goal. We
haven’t yet beaten cystic fbrosis – completely beaten it – once and for all.
So instead of partying, we’re working harder than ever. Working for the day when everyone with
cystic fbrosis can receive the treatments that will transform their daily experience. When
everyone can live a long and healthy life in spite of their condition. When everyone can
celebrate their 50th birthday – and look forward with confdence to celebrating many, many more.
That’s our goal. When we reach it – and with your support we will reach it – the Cystic Fibrosis
Trust will no longer be needed. And that’s the day we’ll fnally be ready to throw a party.
Blow up your No Party balloon at cysticfbrosis.org.uk/no-party
Regulars
12
Getting active 15
Day in the life 19
Out in the
News community 23
16
Features
9 23
Two-point
perspective 24
Just ask 26
News 6–11
20
Marking the
Trust’s 50th 12
Tackling adherence,
to transform lives 16
Trials and
tribulations 20
10 24
4 The magazine of the Cystic Fibrosis Trust cysticfbrosis.org.uk
Welcome
Welcome to the frst issue of
i magazine in our 50th year
(and also my frst as editor!).
The chances are you have been
involved with the Trust and its
work at some point. So thank
you, on behalf of the Trust, for all
your support and encouragement
during our frst 50 years.
sA you iw l rea d on ap ges ,41–21 our t05 h anniev rsary
is not a time to ceel brate , not hw en many ep oelp iw th
the conid tion sti l od nt’ il ev to see their t04 h birthad y.
nI stea ,d ew are reod ubil ng our efforts to create a
future hw ere ew haev beaten cystic fbrosis an d
eev ryone iw th the conid tion can eepx ct a ol ng an d
heatl hy il fe. That future starts here an d by oj ining our
t05 h anniev rsary camap ign , you can be ap rt of this
aw tershe d moment.
nI this issue you iw l asl o see ho w technool gy is
changing the aw y young ep oelp engage iw th their
treatments ap( ges ,)81–61 an d rea d about the ecx iting
state of cystic fbrosis research toad y , an d the
imop rtance of cil nica l triasl .
Together ew can make 41 0 2 the year the game change.d
Henry Fogarty
idE tor
lA communications shou dl be sent to
Editor, i magazine, Cystic Fibrosis Trust,
11 London Road, Bromley, Kent BR1 1BY
Interim cross-
hosip ta l staff shou dl haev hygiene
50 ways to say thank
infection guidelines measures ealpx ine d an d be ketp
informe d of their infection status ;
you for 50 years
to combat superbug an d surev ial nce of infection
Fifty ‘thank you’ awards are being shou dl be ep rforme d at regual r
launched by the Trust this year to A working group brought interav sl , iw th arp orp iate
mark 50 years of hard work and together by the Cystic Fibrosis measures up t in alp ce if a ep rson
dedication from people from all areas Trust and including healthcare tests op sitiev for M. abscessus.
of the cystic fbrosis community. professionals and experts on
microbiology and cross-infection rD nA rd e w oJ nes , Consutl ant
Categories for consied ration incul ed has released new advice to eR sip ratory hP ysician at nU iev rsity
heatl h rp ofessionasl , researchers , help protect people with cystic oH sip ta l oS uth aM nchester ,
funrd aisers , ov ul nteers an d fbrosis from catching infection hw o chairs the grou ,p sai :d
ambassaod rs hw o haev heepl d raise with M. abscessus. nI“ fection contro l continues to be
aaw reness of cystic fbrosis issues a maoj r issue for cystic fbrosis
an d the Trust. There iw l asl o be The grou p aw s set u p in the aw ke care. Fool iw ng a consutl ation ,
recognition for sep cia l achieev ments of a reop rt in the aL ncet in ,3102 ne w interim nationa l guied il nes
– those hw o haev ed id cate d their il fe that reev ael d eiv ed nce from a haev been ed ev ol ep d to rp otect
to heipl ng ep oelp iw th cystic fbrosis , grou p of acaed mic hp ysicians ap tients from the risk of
or hw o haev achieev d something at aP owp rth oH sip ta l that M. crossi- nfection to transmissibel
remarkabel . abscessus cou dl transfer betew en bacteria l infections such
ep oelp iw th cystic fbrosis in sip te as M. abscessus.”
evE“ rything the Trust has achieev d of eix sting crossi- nfection op il cies.
has been as a resutl of tael nte ,d eP oelp iw th cystic fbrosis hw o
see�l ss ep oelp throughout the KU The grous’p ne w recommenad tions haev concerns about this issue
an d beyon ,d hw o are iw il ng to go incul ed : a l centres shou dl haev shou dl sep ak to their cil nician.
aboev an d beyon d to suop rt the a ol ca l crossi- nfection op il cy
Trust an d make a id fference ”, sai d its sep cifc to M. abscessus that You can fnd more on
Chief exE cutiev dE ewO n. T“ his year ard esses issues of monitoring cross-infection and the interim
ew aw nte d to say thank you to those an d cel anil ness ; ep oelp iw th cystic guidelines at cysticfbrosis.org.
hw o haev giev n an d continue to giev fbrosis , their carers an d uk/crossinfection.
their suop rt.”
The Trust is iniv ting the hw oel cystic
fbrosis community to get inov evl d by
nominating the ep rson or ep oelp hw o
they fee l ed serev recognition. A ap ne l
of uj gd es featuring members of the
Trust an d the iw ed r community iw l
ed cied hw o iw ns the aaw rsd , hw ich
iw l be haned d out at av rious Trust
eev nts ud ring the year.
Visit cysticfbrosis.org.uk/50awards
for more information on the
nomination process and criteria.
1,000 Cranes:
By Oliver Knott, the video is based on a Japanese belief that if you fold 1,000 cranes in one
year then a wish will be granted. http://bit.ly/Cranes1000
6 The magazine of the Cystic Fibrosis Trust cysticfbrosis.org.uk
News
Campaigning hard for equal
access to vital medicines
In the past few months the Trust has been
lobbying decision makers in Scotland
and Wales to ensure that vital medicines
for people with rare conditions such
as cystic fbrosis are appraised fairly
when assessing their value.
nI cO tober the cS ottish goev rnment
gaev cause for hoep hw en it aske d
the cS ottish eM id cines Consortium to
fn d an d aylp id fferent arp oaches to
eav ul ating rd ugs for sma l op up al tions ,
Improving the The Trust al unche d its consutl ation
in ored r to increase access. The Trust is
after ied ntifying issues that are
rate of successful cail ng for any ne w system to take into
rp eev nting ep oelp iw th cystic fbrosis
account uq ail ty of il fe benefts an d aol w
transplantation from getting a ul ng transalp nt ,
for a al ck of aav ial bel research ad ta.
guied d by ap tients hw o came
The Trust is shortly to release a foraw r d to share their eepx rience. A Task an d Finish rG ou p has been set
report based on the fndings of u p to reiv e w the arp aisa l system , an d
the open consultation launched eJ ssica oJ nes , oP il cy ivdA ser at CF sep ciail st rD oG rod n aM crG egor
in 2013 to address the severe the Trust , sai :d eW“ aw nte d the has been iniv te d to oj in. The aim is to
shortfall in successful lung consutl ation to funad mentayl imelp ment the fnid ngs of the reiv e w
transplants being carried out for eolpx re the issues that are by srp ing.
people with cystic fbrosis. creating these rp obel ms an d ew
dE ewO n ew cl ome d this moev by the
haev brought together eepx rts in
hW iel transalp ntation serev s sma l cS ottish eH atl h iM nister : T“ he Cystic
cystic fbrosis an d transalp ntation
numbers , it is crucia l for those that Fibrosis Trust has been ol bbying the
to ed sign meaningfu l aw ys of
nee d it. There are nearyl 08 ep oelp goev rnment to ensure that concerns
ard essing these chael nges. The
iw th cystic fbrosis aw iting for a regarid ng the eix sting arrangements for
rea l ow rk is uj st beginning an d
ul ng transalp nt in the ,K U an d this is arp aising ne w meid cines ew re hear.d “
ew hoep our reop rt iw l make a
il keyl to increase as suriv av l rates for
signifcant op sitiev imap ct.” T“ he aK yl ed co issue al i d bare the nee d
those iw th the conid tion imrp oev .
for change , an d ew are encourage d
The aw it can be agonisingyl ol ng The reop rt iw l l be reel ase d in al te that the goev rnment has resop ned d to
an d one in three ep oelp iw th cystic February , fool ew d by a camap ign to the concerns raise d by us an d other
fbrosis on the ul ng transalp nt il st make sure its recommenad tions are meid ca l charities an d ap tient grousp .”
id es before they can receiev one. taken on boar d by op il cy makers.
nI aW el s the Trust urge d suop rters to
rw ite to their ol ca l sA sembyl eM mber to
ep tition the iM nister for eH atl h & oS cia l
New partnership to test rD aJ net elA n , iD rector of eR search eS riv ces , aM rk rD akefor d ,M A rp essing
at the Trust , highil ghte d ho w this
treatment for lung function for the recognition of the hoil stic benefts
ap rtnershi p iul strates the Trusts’
of meid cations for rarer conid tions in the
The Cystic Fibrosis Trust has emhp asis on coal boration iw th eW sl h sA sembyl s’ reiv e.w
ap rtnere d iw th oN av iB otics tL d inud stry , as ed taiel d in the research
an d eH atl h cS iences cS otal n d to strategy al unche d in :3 1 0 2 oW“ rking
ed ev ol p yL noev ®x as a treatment coal boratiev yl iw th acaed mics an d
for ep rsistent ul ng infection in inud stry increases our caap city to
cystic fbrosis. suop rt transformationa l research
rp oej cts. This ow rk forms ap rt of
The rd ug is arl eayd a recognise d our fev y- ear strategy hw ich aims to
treatment for a non CFr- eal te d see the Trust engage col seyl iw th
conid tion. ap rtners in research an d inud stry.”
7 The magazine of the Cystic Fibrosis Trust cysticfbrosis.org.uk
News
News in brief
George’s ‘grand’
.2102 eH is asl o eD up ty Chairman
‘No Party’ campaign
cycling tour of SHN olB o d an d Transalp nt ,
marks Trust’s 50th year hw ose staff seel cte d the Trust as
Where others relax on holiday, their Charity aP rtner for the year ,
Trust Chairman George Jenkins an d Chairman of oP rt of oD ev r
set himself the target of riding an d oD ev r aH rbour oB ar.d eG orge
1,000 miles over the summer to aw s aaw red d the EBO for seriv ces
raise money for the Trust and to heatl hcare in the 102 uQ eens'
awareness of cystic fbrosis. iB rthad y oH nours.
olA ng the aw y he raise d an fA ter his al st ried , eG orge sai :d I"
increid bel !05,4£ am absoul teyl ap ssionate about
The Cystic Fibrosis Trust is asking the Cystic Fibrosis Trust an d the
all of its supporters to mark its 50th eG orge comelp te d his chael nge ow rk it od es to giev ep oelp hoep
anniversary, not by celebrating, hw en he crosse d the fnishing il ne no w an d for the future. I ol st my
of the rP ued ntia l iR ed oL nod n- son adA m to cystic fbrosis an d
but by joining with it to fnally beat
uS rrey 01 on 4 uA gust. eG orge I am ed termine d to od a l I can
cystic fbrosis.
took ap rt in this m-01 iel cycel for the cause so thank you to
The Trusts’ C‘ ystic Fibrosis is oN aP rty’ chael nge aol ngsied 23 other eev ryone hw o sop nsore d me.
camap ign , hw ich al unche d in aJ nuary Trust suop rters , incul id ng some Those uS rrey hisl ew re not easy"!
, 4 1 0 2 is asking eev ryone iw thin the hw o ha d traev el d od nw from
cystic fbrosis community to bol w idE nburgh to take ap rt. Be inspired by George and
u p a baol on onil ne , an d share their take on a fundraising challenge
eepx riences of cystic fbrosis an d hw at eG orge aw s eel cte d as Chairman in our 50th year – visit
they are bringing to the oN‘ aP rty.’ of the Cystic Fibrosis Trust in uJ yl cysticfbrosis.org.uk/events.
oH“ w cou dl ew ceel brate our t05 h
anniev rsary hw en ew kno w many ep oelp
iw th cystic fbrosis are sti l not il iv ng
to ceel brate their t04 h birthad y ”? sai d
aI in cM nA rd e , w the Trusts’ iD rector of
aM rketing. nI“ stea ,d ew aw nt our t05 h
year to be the moment hw en the cystic
fbrosis community comes together an d
seizes the moment to beat cystic fbrosis
for goo.d ”
fA“ ter bol iw ng u p a baol on , suop rters
can choose hw ether they aw nt to he pl
imrp oev the il ev s of ep oelp iw th cystic
fbrosis by aid ng their ov ice to one of
our camap igns , or organising their onw
oN aP rty eev nt , be it a uq iz night , coffee
morning – or eev n a oN aP rty ap rty ”!
aed d aI in.
eR a d more about the Trusts’ t05 h
anniev rsary , see ap ges .41– 2 1
To blow up your balloon and read
the stories and experiences of
other ‘No Party’ goers, visit eG orge eJ nkins ,EBO Chairman of the Cystic Fibrosis Trust ,
cysticfbrosis.org.uk/no-party. cycel d 0,1 miel s ud ring the summer of 3102 to raise money.
8 The magazine of the Cystic Fibrosis Trust cysticfbrosis.org.uk
News
Registry Report reveals
majority of people with
cystic fbrosis now in
Peer reviews put patients in
work or study
position of power
The UK CF Registry Data Report for In the last few months the Trust has
2012, released in October 2013, reveals undertaken a number of peer reviews
that cystic fbrosis is increasingly of adult and paediatric cystic fbrosis
moving from a childhood killer to an services around the UK.
adult condition.
oJ intyl organise d by the Trust , the
The ne w statistics sho w that the meid an rB itish aP eid atric eR sip ratory oS ciety
suriv av l for ep oelp iw th cystic fbrosis in an d the rB itish Thoracic oS ciety ,
the KU has increase d to .34 5 years. ep er reiv esw ol ok at the cil nica ,l
sp ychosocia l an d business actiiv ties
The reop rt asl o reev asl that cystic real ting to the ed il ev ry of care.
fbrosis is el ss of a barrier to norma l il fe ,
iw th .07 %4 of ep oelp iw th the conid tion To make the rp ocess more accessibel
abel to ow rk or stuyd , an d a signifcant to ap tients , the Trust no w up bil shes
number consied ring themseevl s retire.d a oneap- ge summary of the reiv e w
aol ngsied the fu l reop rt.
The KU CF eR gistry is one of the most
comrp ehensiev ad ta sets on cystic Read the peer reviews and summaries
fbrosis in the ow r ,dl containing ed taisl online at cysticfbrosis.org.uk/peer.
for oev r %9 of the KU CF op up al tion.
Trust Chief exE cutiev dE ewO n Coalition of charities to focus
ew cl ome d the fnid ngs but focuse d on on Who Benefts?
the future , saying : T“ he 2102 eR gistry
eR op rt rp oev s that cystic fbrosis is nI eS tp ember the Cystic Fibrosis Trust
a conid tion that has benefte d from oj ine d a coail tion of more than 05
imrp oev ments in treatments , stanad rsd organisations set u p to giev a ov ice to
of care an d access to ne w meid cines. the miil ons of ep oelp hw o haev been
heepl d by benefts.
sA“ ew enter our t05 h anniev rsary year in
,41 0 2 ew iw l be ow rking eev n hared r , in The nationiw ed camap ign aims to change
conuj nction iw th scientists an d cil nicians , the aw y ew think about ep oelp hw o
to imrp oev suriv av l rates an d uq ail ty of il fe.” receiev benefts , using rea l stories to
sho w the reail ty of hw o neesd he , p l hw y
For the frst time a seap rate summary they nee d it an d the id fference it makes.
od cument aw s up bil she d aol ngsied the
fu l scientifc reop rt. The Trust wants to hear from people
with stories about how benefts
Read and download both reports have supported them.
at cysticfbrosis.org.uk/registry. Contact Louise Banks,
Senior Communications Manager,
(
Addressing ‘nonsense’
mutations
A project led by Professor Smythe,
University of Sheffeld, is testing a new
Re-vamped clinical
compound that will help to counteract
conference looks to a group of mutations present in 10% of
people with cystic fbrosis.
new era
September 2013 saw the Cystic Cal ss I or n‘ onsense ’ mutations , are
Fibrosis Trust’s new-look, two-day ed tecte d by a uq ail ty contro l system
clinical conference open at the that stosp cesl attemtp ing to rp oud ce
Manchester Conference Centre, under the CFT R rp otein that is fautl y in ep oelp
the banner of ‘Moving forward together iw th cystic fbrosis.
in a new era’.
nA ora l rd ug cael d tA aul ren can arl eayd
aD y 1 aw s oep n to anyone inov evl d in comep nsate for nonsense mutations
the rp oiv sion of cil nica l care at sep ciail st in iv tro but its cil nica l effect has not
CF centres an d cil nics across the .KU been as great as aw s eepx cte ,d an d it
This mutl iid sciilp nary eev nt feature d is hoep d the comop un d that rP ofessor
takl s an d ow rkshosp on a iw ed range of mS ythe an d his team are inev stigating
subej cts. iw l enhance the effect of tA aul ren.
tA an eev ning recetp ion at the en d of Find out more at
the frst ad y , rB itish high uj mep r aD tl on cysticfbrosis.org.uk/smythe.
rG ant rp oiv ed d an insip rationa l takl as
guest sep aker.
Campaign success for access to
The secon d ad y aw s an iniv teo- nyl psychology services in Scotland
Centre iD rectors eM eting , aime d
at faciil tating communication an d The Cystic Fibrosis Trust can report
id scussion. tI asl o serev d as a al unching some success in its efforts to achieve
ap d for the 2102 KU CF eR gistry eR op rt. equal access in Scotland to specialist
psychology, after discovering a
epO ning the conference , Chief desperate lack in some regions.
exE cutiev dE ewO n sop ke of the iv ta l
real tionshi p betew en the Trust an d the At the end of 2013 the West of Scotland
cil nica l community , something hw ich has Adult Cystic Fibrosis Unit based in
been h“ ariw-d re d into hw at ew[ ] od since Gartnavel General Hospital gained a
our founad tion , 05 years ago netx year.” specialist clinical psychologist post.
dE asl o highil ghte d the nee d to The campaign began after the Trust’s
better engage iw th the cystic fbrosis psychosocial survey identifed a
community , a ed” fning ap rt ” of the number of areas where a specialist
Trusts’ mission going foraw rsd . psychologist is badly needed but
unavailable due to lack of funding.
Read the full text of Ed’s speech at Supporters were invited to share stories
http://cftrust.blogspot.co.uk. about when they have either benefted
from, or suffered through a lack of,
access to psychological support.
01 The magazine of the Cystic Fibrosis Trust cysticfbrosis.org.uk
News
News in brief
