RESEARCH ARTICLE Open Access
Ma ked de e io a ion in he quali y o li e o
pa ien s wi h idiopa hic pulmona y ib osis
du ing he las wo yea s o li e
K. Rajala
1,2*
, J. T. Leh o
3
, E. Su inen
4
, H. Kau iainen
5
, M. Myllä niemi
6
and T. Saa o
7
Abs ac
Backg ound: Idiopa hic pulmona y ib osis (IPF) is a ch onic disease wi h a high symp om bu den and poo su i al
ha in luences pa ien s’heal h- ela ed quali y o li e (HRQOL). We aimed o e alua e IPF pa ien s’symp oms and
HRQOL in a well-documen ed clinical coho du ing hei las wo yea s o li e.
Me hods: In Ap il 2015, we sen he Modi ied Medical Resea ch Council Dyspnea Scale (MMRC), he modi ied
Edmon on Symp om Assessmen Scale (ESAS) and a sel - a ing HRQOL ques ionnai e (RAND-36) o 300 IPF pa ien s,
o which 247 (82%) esponded. The ea e , ollow-up ques ionnai es we e sen e e y six mon hs o wo yea s.
Resul s: Nine y- wo pa ien s died by Augus 2017. Among hese pa ien s, HRQOL was ound o be conside ably low
al eady wo yea s be o e dea h. The mos p ominen declines in HRQOL occu ed in physical unc ion, i ali y,
emo ional ole and social unc ioning (p< 0.001). The p opo ion o pa ien s wi h MMRC sco es ≥3 inc eased nea
dea h. B ea hlessness and a igue we e he mos se e e symp oms. Symp om se e i y o he ollowing symp oms
inc eased signi ican ly and eached he highes mean sco es du ing he las six mon hs o li e (nume ic a ing scale/
s anda d de ia ion): b ea hlessness (7.1/2.8), i edness (7.0/2.3), d y mou h (6.0/3.0), cough (5.8/2.9), and pain wi h
mo emen (5.0/3.5).
Conclusions: To ou knowledge his is he i s s udy demons a ing, ha IPF pa ien s expe ience ema kably low
HRQOL al eady wo yea s be o e dea h, especially ega ding physical ole. In addi ion, hey su e om se e e b ea hlessness
and a igue. Fu he mo e, physical, social and emo ional wellbeing de e io a e, and symp om bu den inc eases nea
dea h. Regula symp om and HRQOL measu emen s a e essen ial o assess pallia i e ca e needs in pa ien s wi h IPF.
Keywo ds: Idiopa hic pulmona y ib osis, Pallia i e ca e, Heal h ela ed quali y o li e, Symp oms
Backg ound
Idiopa hic pulmona y ib osis (IPF) is a ch onic disease
wi h high mo bidi y and poo su i al [1–4]. I occu s
mainly in olde adul s, bu he e iology o his p og essi e
disease is s ill unknown [1]. Al hough he disease ajec-
o y o IPF is a iable, o many pa ien s wi h IPF, su i al
is wo se han many common malignancies. This necessi-
a es ea ly in eg a ion o pallia i e ca e o imp o e pa-
ien s’quali y o li e (QOL) and o elie e symp oms in
addi ion o disease-speci ic pha macological ea men
and lung ansplan assessmen [5–9].
Exis ing s udies ha e shown low heal h- ela ed quali y
o li e (HRQOL) in IPF pa ien s. Howe e , only ew o
hem we e p ospec i e longi udinal s udies, and mos
we e ela i ely small in e ms o sample size o we e
concen a ed on pha macological ea men [10–12]. IPF
pa ien s ha e been shown o su e om lowe HRQOL
in eal-li e s udies han in clinical s udies [12,13].
IPF pa ien s su e om many di icul symp oms, o
which b ea hlessness and cough a e he mos common
ones [8,10,14–20]. In addi ion, a subs an ial p opo ion o
pa ien s epo anxie y, dep ession and pain [10,21–26].
Dyspnea is a majo con ibu o o HRQOL, and de-
c eased HRQOL is associa ed wi h highe mo ali y [27,
28]. In a p ospec i e Aus alian longi udinal egis y s udy,
impai ed HRQOL was ela ed o equen espi a o y
* Co espondence: [email p o ec ed]
1
Depa men o Pallia i e Ca e, Comp ehensi e Cance Cen e ,, Helsinki
Uni e si y Hospi al, Paciuksenka u 21, Po BOX 180, FI-00290 Helsinki, Finland
2
Facul y o Medicine, Uni e si y o Helsinki, Helsinki, Finland
Full lis o au ho in o ma ion is a ailable a he end o he a icle
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Rajala e al. BMC Pulmona y Medicine (2018) 18:172
h ps://doi.o g/10.1186/s12890-018-0738-x
hospi aliza ions and highe mo ali y [27]. Howe e , o
ou knowledge, no p e ious s udies ha e epo ed changes
in HRQOL and symp om bu den in connec ion wi h
o hcoming dea h.
This s udy aimed o in es iga e IPF pa ien s’HRQOL
and symp om bu den du ing he las wo yea s o li e in
a p ospec i e longi udinal ollow-up s udy o ecognise
needs o pallia i e ca e and end-o -li e ca e planning
and o cha ac e ise hei symp om bu den in a unique
ollow-up se ing.
Ma e ials and me hods
S udy popula ion
The FinnishIPF s udy is a na ional p ospec i e clinical
egis y o IPF pa ien s ha was es ablished in 2012. IPF
diagnosis is based on he ATS/ERS 2011/2015 c i e ia [1,
6]. Nea ly all Finnish IPF pa ien s a e ini ially e alua ed a
public uni e si y and cen al hospi als. Pa ien s om hese
specialis cen es wi h in o med consen a e included in
he FinnishIPF egis y, which consis s o app oxima ely
76% o all Finnish IPF pa ien s [2]. Cu en ly, he egis y
con ains da a om o e 700 IPF pa ien s.
All 300 pa ien s egis e ed in he FinnishIPF s udy in
Ap il 2015 we e asked o pa icipa e in his subs udy by
sending an in o med consen o m oge he wi h he
ques ionnai es. Those who did no espond wi hin wo
weeks we e called and eminded. O he 300 egis e ed
pa ien s, 247 (82%) p o ided in o med consen o his
subs udy, answe ed he i s ques ionnai e and we e in-
cluded in his s udy. Subsequen ly, he same ques ion-
nai e was sen o he pa ien s i e imes a six mon hs
in e als un il Augus 2017.
Da a collec ion and ques ionnai es
Disease and sociodemog aphic cha ac e is ics we e col-
lec ed om pa ien eco ds and wi h a sepa a e ques-
ionnai e (Addi ional ile 1). These included he da e o
bi h, sex, age, ma i al s a us, educa ion, li ing condi-
ions, physical ac i i y le el, he need o assis ance in
daily ac i i ies, he da e o IPF diagnosis, smoking s a us,
and como bidi ies. Pa ien s we e asked he equency o
leisu e ime physical exe cise ha causes b ea hlessness
and swea ing o a minimum 30 min du ing he p eced-
ing six mon hs. Dea h ce i ica es we e acqui ed om
he “Na ional Au ho i y o Collec ing and Compiling
S a is ics on Va ious Fields o Socie y and Economy”.
The ques ionnai es ega ding HRQOL and symp oms
we e he RAND 36-I em Heal h Su ey (RAND-36), he
Modi ied Medical Resea ch Council Dyspnea Scale
(MMRC), and he modi ied Edmon on Symp om Assess-
men Scale (ESAS).
RAND-36 [29] is a gene al QOL measu emen ool wi h
exis ing Finnish gene al popula ion e e ence alues [30].
RAND-36 is simila o he p e iously IPF- alida ed
sho -Fo m-36 [30–32]. RAND-36 is di ided in o eigh
heal h concep s [29,30]. Concep s a e sco ed on a scale
om 1 o 100, whe e a lowe sco e indica es a wo se
HRQOL du ing he pas ou weeks [29,30]. The concep s
a e as ollows: “gene al heal h”( i e ques ions), “ i ali y”
( ou ques ions ega ding ene gy le el and i edness),
“bodily pain”( wo ques ions), “physical unc ioning”( en
ques ions ega ding he abili y o ake ca e o pe sonal hy-
giene and he abili y o mo e and exe cise), “physical ole”
( ou ques ions ega ding ole limi a ions due o physical
heal h), “men al heal h”( i e ques ions ega ding mood,
dep ession and anxie y), “emo ional ole”( h ee ques ions
ega ding ole limi a ions due o emo ional p oblems),
and “social unc ioning”( wo ques ions) [29,30].
The sel - a ed MMRC measu es he deg ee o disabili y
ha b ea hlessness causes du ing day- o-day ac i i ies on
a scale om 0 o 4, in which 0 indica es no b ea hlessness
excep du ing s enuous exe cise, 1 indica es sho ness o
b ea h when walking up a sligh hill o hu ying on a le el,
2 indica es walking slowe han people o same age on a
le el because o b ea hlessness o needing o s op o o
b ea h when walking a one’s own pace on a le el, 3 indi-
ca es needing o s op o b ea h a e a ew minu es when
walking on a le el o a e walking app oxima ely 100 m,
and 4 indica es ha he pa ien is oo b ea hless o lea e
he house o is b ea hless when d essing o und essing
[33,34].
The ESAS is a nume ic sel - a ing symp om-based scale
ha was o iginally de eloped o assess he symp oms o
cance pa ien s [35,36]. Di e en symp oms a e measu ed
on Nume ic Ra ing Scale (NRS) om 0 (no symp oms) o
10 ( he wo s possible symp oms) [36–38]. In his s udy,
we used a e sion including 12 symp oms (pain a es ,
pain wi h mo emen , i edness, nausea, dep ession, anx-
ie y, insomnia, loss o appe i e, sho ness o b ea h, cough,
cons ipa ion, d y mou h, and o e all wellbeing). The e is a
lack o e idence o ecommend cu -o poin s o he
ESAS. Howe e , an NRS sco e ≥4iscommonlyusedasa
igge o mo e comp ehensi e symp om assessmen in
clinical p ac ice [39].
S a is ics and e hical aspec s
The s udy popula ion cha ac e is ics a e p esen ed as he
means wi h s anda d de ia ions (SD) o as coun s wi h
pe cen ages. Pa ien s’answe s we e g ouped, acco ding
he ime hey answe ed om he aspec o dea h. The
Kaplan-Meie me hod was used o es ima e he cumula-
i e mo ali y a e he diagnosis. We used es ic ed cubic
splines o de ec a possible non-linea dependency. A non-
linea ela ionship be ween he RAND-36 domains, symp-
om se e i y, he MMRC and ime be o e dea h we e
assessed by using 5-kno - es ic ed cubic spline
Rajala e al. BMC Pulmona y Medicine (2018) 18:172 Page 2 o 9
andom-e ec s eg ession models wi h app op ia e dis i-
bu ion and link unc ions. Models included age and gen-
de (only main e ec s) as co a ia es. A es o in e ac ion
be ween independen a iables was pe o med h ough
he MFPIgen command. The leng h o he dis ibu ion
(mon hs be o e dea h) o kno s was loca ed a he 5 h,
27.5, 50 h, 72.5, and 95 h pe cen iles, which co espond o
ime be o e dea h o −22, −15, −9, −5and−1. The loca-
ions o he kno s we e de e mined by he pe cen iles ec-
ommended in Ha ell’s publica ion [40].The no mali y o
he a iables was es ed by using he Shapi o-Wilk es .
The Finnish gene al popula ion alues o he eigh
Rand-36 domains we e weigh ed o ma ch he gende and
age dis ibu ion o he s udy popula ion, s a is ical analysis
be ween ou popula ion and gene al popula ion was no
pe o med [30]. The S a a 15.0 [41] s a is ical package was
used o he analysis.
The e hics commi ee o Helsinki Uni e si y Cen al
Hospi al app o ed his s udy (381/13/03/01/2014). The
Finnish Na ional Ins i u e o Heal h and Wel a e (Dn o
THL/1161/5.05.01/2012) app o ed he sc eening o hos-
pi al egis ies o pa ien s wi h IPF. All pa icipa ing pa-
ien s p o ided w i en in o med consen o pa icipa e
o his speci ic s udy.
Resul s
O he 247 pa ien s included in he s udy 92 (37%) died
by Augus 2017 and we e included in ou ollow-up co-
ho (Fig. 1).
The cumula i e mo ali y o he pa ien coho (n=92)
is p esen ed in Fig. 2. The median o e all su i al was 4.4
yea s (IQR 3.1–5.7). Pa ien cha ac e is ics a e shown in
Table 1. A majo i y o he pa ien s had como bidi ies, o
which ca dio ascula diseases we e he mos common
ones. None o he pa ien s had lung cance when en e ing
he s udy, bu wo pa ien s we e diagnosed wi h lung
cance du ing he ollow-up. Lung unc ion measu emen s
wi hin he las six mon hs o li e we e a ailable in only 28
(30%) o he pa ien s (mean FVC 2.2 l (SD 0.6), 57%).
An i ib o ic medica ion was used by 33 (35%) o he
pa ien s.
The p opo ion o he pa ien s who could no pe o m
con inuous mode a e in ensi y physical exe cise o a
leas 30 min du ing he p e ious six mon hs inc eased
om 34% 18–24 mon hs be o e dea h o 62% du ing he
las six mon hs o li e. Six mon hs be o e dea h, 67% o
he pa ien s epo ed needing assis ance wi h hei daily
ac i i ies, while 18–24 mon hs be o e dea h his p opo -
ion was 56%.
Heal h- ela ed quali y o li e
Figu e 3shows he changes in he di e en dimensions
o HRQOL measu ed wi h RAND-36 du ing wo yea s
Fig. 1 Flowcha o pa ien ec ui men and esponse a e
Fig. 2 Cumula i e mo ali y a e he diagnosis o IPF. Time-poin o
he diagnosis is ma ked wi h 0 and 95% con idence in e als wi h
he g ey a ea. Kaplan-Meie me hod was used o es ima e he
cumula i e mo ali y
Rajala e al. BMC Pulmona y Medicine (2018) 18:172 Page 3 o 9
be o e dea h. The decline in HRQOL was highly signi i-
can in all dimensions excep in physical ole, which
showed e y low sco es by 24 mon hs be o e dea h.
Symp oms
The in ensi y change o he symp oms measu ed by he
ESAS du ing he las wo yea s o li e is p esen ed in
Fig. 4. The in ensi y o all symp oms excep pain a es
and insomnia inc eased signi ican ly nea dea h. Du ing
he las six mon hs o li e, he mean NRS sco es we e as
ollows: 7.1 (SD 2.8) o b ea hlessness, 7.0 (SD 2.3) o
i edness, 6.0 (SD 2.5) o wellbeing, 6.0 (SD 3.0) o d y
mou h, 5.8 (SD 2.9) o cough, 5.0 (SD 3.5) o pain wi h
mo emen , 3.9 (SD 3.1) o insomnia, 3.9 (SD 2.9) o
anxie y, 3.8 (SD 2.9) o dep ession, 3.6 (SD 3.1) o con-
s ipa ion, 3.4 (SD 3.3) o loss o appe i e, 3.1 (SD 2.8)
o pain a es and 1.8 (SD 2.5) o nausea.
The s eep change in he p opo ion o pa ien s wi h
MMRC sco es ≥3 (needing o s op walking a e app oxi-
ma ely 100 m o a ew minu es because o b ea hlessness)
du ing he las wo yea s o li e is shown in Fig. 5.
Discussion
In his s udy, we demons a e a apidly inc easing impai -
men in HRQOL and escala ing symp om bu den in IPF
pa ien s app oaching dea h. Low HRQOL oge he wi h
se e e b ea hlessness and a igue we e de ec ed as ea ly as
wo yea s be o e dea h. In addi ion, se e al dimensions o
HRQOL declined u he and he se e i y o many symp-
oms o he han dyspnea inc eased du ing he las wo
yea s o li e.
In he p esen s udy, HRQOL was conside ably im-
pai ed wo yea s p io o dea h in IPF pa ien s. Physical
ole, i.e., ole limi a ions due o physical heal h, was excep-
ionally low, bu physical unc ioning, i ali y and gene al
heal h appea ed o be below he gene al popula ion le el
as well. Simila o ou indings, an Aus alian egis y
s udy o 516 IPF pa ien s epo ed HRQOL impai men s
in all domains, wi h he lowes sco e in ac i i y, i.e., ac i -
i ies ha cause o a e limi ed by b ea hlessness [27]. The
impo ance o dec eased HRQOL was u he highligh ed
in a ecen s udy by Fu ukawa e al. ha demons a ed
ha low HRQOL was ac ually an independen p ognos ic
ac o [28].
Al hough p e ious s udies ha e demons a ed low
HRQOL in IPF pa ien s [10–12], none o hem ocused
on he HRQOL om he aspec o app oaching dea h in
a ollow-up se ing. The uniqueness o his s udy s ems
om he con inuing ollow-up un il dea h, and he sub-
sequen inding o a salien decline in HRQOL du ing
he las wo yea s o li e ha was in ensi ied nea dea h.
De e io a ion was iden i ied in all domains excep phys-
ical ole, which was al eady ema kably low wo yea s
be o e dea h. The mos in eg al impai men was in phys-
ical, social and emo ional unc ioning and i ali y. In he
Aus alian egis y s udy, app oxima ely one- hi d (38%)
o he IPF pa ien s expe ienced clinically impo an di -
e ences in he decline o HRQOL du ing 12 mon hs
[27]. Howe e , in ha s udy, no HRQOL da a was a ail-
able om he pe iod p eceding dea h [27].
In lung cance , unc ional conce ns ela ing o physical
mo emen o unc ioning p edomina e pa ien s’symp-
om bu den h oughou he disease cou se and ha e a
nega i e impac on HRQOL [42]. In addi ion, he se e -
i y o symp oms escala es, and he numbe o se e e
symp oms inc eases du ing he las h ee mon hs o li e
[42–44]. A s eep decline in HRQOL a he end-o -li e is
Table 1 Pa ien cha ac e is ics
To al numbe o pa ien s 92
Age, mean ( ange) 75 (57–92)
Males n (%) 67 (73)
Du a ion o IPF in yea s, mean (SD) 3.6 (2.3)
Educa ion in yea s, mean (SD) 10 (3)
Li ing alone, n (%) 31 (34)
Wo king, n (%) 4 (4)
Smoking s a us, n (%)
a
Smoke 6 (7)
Ex-smoke 50 (54)
Ne e -smoke 36 (39)
FVC (li es), mean (SD)
b
2.9 (0.8)
FVC (% o p edic ed), mean (SD)
b
78 (16)
Di usion capaci y, mean(SD)
c
54 (13)
Co-mo bidi ies
e
, n (%)
Hype ension 41 (45)
Co ona y hea disease 35 (38)
Diabe es 24 (26)
Hea ailu e 23 (25)
COPD 20 (22)
Cance 16 (17)
As hma 8 (9)
No co-mo bidi ies 13 (14)
Numbe o co-mo bidi ies, median ( ange) 2 (0–7)
Place o dea h, n (%)
b
Hospi al
d
62 (67)
Home 19 (22)
Nu sing home 4 (5)
Hospice 3 (3)
a
smoking s a us, o ced olume i al capaci y (FVC) and di usion capaci y a e eco ded
a he ime o diagnosis and o he ac o s a he ime o he i s ques ionnai e
b
Da a missing om 4 pa ien s;
c
da a missing om 12 pa ien s;
d
10 in in ensi e ca e
uni ;
e
pa ien - epo ed co-mo bidi ies
Rajala e al. BMC Pulmona y Medicine (2018) 18:172 Page 4 o 9
ypical in pa ien s dying o cance compa ed wi h o he
e minally ill pa ien s [45,46]. In COPD pa ien s,
HRQOL g adually declines o e ime wi hou a s eepe
decline a he end-o -li e [47]. Ou da a imply ha pa-
ien s wi h IPF expe ience g adual impai men s in
HRQOL compa able o COPD pa ien s bu su e om a
p onounced, apid de e io a ion in HRQOL du ing he
las yea o li e, mo e closely esembling he disease a-
jec o y o cance .
Ou esul s co obo a e ea lie indings on dyspnea
and cough as he mos se e e symp oms in IPF pa ien s
ega dless o he disease phase [10,21,27,48]. The in-
ensi y o dyspnea inc eased du ing he ollow-up, being
one o he mos se e e symp oms be o e dea h. In a
p e ious IPF egis y s udy, dyspnea yielded he s on-
ges associa ion wi h impai ed HRQOL accoun ing o
71% o he a ia ion in HRQOL [27]. In addi ion,
exe ion dyspnoea measu ed by he MMRC has been
shown o co ela e o HRQOL and symp om bu den
[21,49].
In addi ion o dyspnea, o he ac i i y-limi ing symp oms
such as a igue and pain in mo emen we e among he
mos se e e symp oms in ou s udy. These ac i i y-limi -
ing symp oms can lead o physical inac i i y and unc-
ional impai men , igge ing a icious ci cle wi h wo se
HRQOL. The in ensi y o dep ession and anxie y we e,
howe e , ela i ely mild wo yea s be o e dea h, al hough
hese symp oms inc eased he ea e . In a ecen s udy,
dep ession was an independen p edic o o HRQOL im-
pai men , al hough i only accoun ed o 3.5% o he a i-
a ion, whe eas dyspnea accoun ed o 71% [27]. Ou
esul s sugges ha he elie o ac i i y-limi ing symp oms
oge he wi h psychosocial suppo may imp o e HRQOL
in ad anced IPF.
Fig. 3 Rela ionships be ween Heal h- ela ed quali y o li e domains (RAND-36) wo yea s be o e dea h. The cu es we e de i ed om a 5-kno
es ic ed cubic splines eg ession models. The models we e adjus ed o age and gende . The g ey a ea ep esen s 95% con idence in e als.
Dashed lines ma k Finnish gene al popula ion le els
Rajala e al. BMC Pulmona y Medicine (2018) 18:172 Page 5 o 9

As discussed abo e, pa ien s wi h ad anced IPF, COPD
and lung cance su e om a hea y symp om bu den
and de e io a ing HRQOL. This calls o comp ehensi e
symp om managemen and in eg a ed pallia i e ca e
concomi an wi h disease-modi ying he apies [21,27,
47,50–52]. Ea ly in eg a ed pallia i e ca e o pa ien s
wi h lung cance has shown subs an ial bene i s, such as
lowe dep ession sco es, highe HRQOL, be e commu-
nica ion o end-o -li e ca e p e e ences, less agg essi e
ca e a he end-o -li e, and longe o e all su i al [51,
53]. Simila ly, a andomised ial demons a ed be e
con ol o dyspnea and a su i al bene i wi h in eg a ed
pallia i e ca e in pa ien s wi h COPD and in e s i ial
lung disease [54]. In addi ion o cance pa ien s, ea ly in-
eg a ed pallia i e ca e may educe end-o -li e acu e ca e
u ilisa ion, and allow pa ien s wi h IPF o die in hei
p e e ed loca ions [55–58]. In eg a ed pallia i e ca e in
IPF pa ien s seems o lowe espi a o y- ela ed eme -
gency oom isi s and hospi alisa ions and may allow
mo e pa ien s o die a home [55]. In his s udy, 67% o
pa ien s died in hospi al and 11% in in ensi e ca e,
which is in line wi h ea lie indings, implying he
necessi y o imp o emen s in ad anced ca e planning
and pallia i e ca e o pa ien s wi h IPF [55,59]. Ou e-
sul s p o ide insigh in o he mos impo an needs o
end-s age IPF pa ien s and suppo he use o
ea ly-in eg a ed pallia i e ca e, which should include
symp om con ol beyond ea men o dyspnea and psy-
chosocial suppo .
The ela i ely small s udy popula ion limi s ou s udy,
as did no ha ing sys ema ic ollow-up da a on lung
unc ion, which is a leas pa ially due o he poo con-
di ions o many o ou pa ien s. Ou s udy is o ou
knowledge i s o p esen ollow-up da a o HRQOL
and symp oms o o e wo yea s be o e dea h. The
s eng h is i s eal-li e longi udinal design, wi h a unique
coho o IPF pa ien s app oaching dea h wi h an ou -
s anding esponse a e, pa icula ly conside ing he ac
ha some o he pa ien s we e p obably oo weak o e-
spond du ing hei inal days o weeks o li e. To ou
knowledge, his is he i s s udy desc ibing comp ehen-
si e pa ien - epo ed da a on he HRQOL and symp om
bu den o IPF pa ien s om he pe spec i e o ap-
p oaching dea h.
Fig. 4 Rela ionships o symp om se e i y measu ed by ESAS wo yea s be o e dea h. The cu es we e de i ed om a 5-kno es ic ed cubic
splines eg ession models. The models we e adjus ed o age and gende . G ey a ea ep esen s 95% con idence in e als. ESAS, Edmon on
symp om assessmen scale
Rajala e al. BMC Pulmona y Medicine (2018) 18:172 Page 6 o 9
Conclusion
Pa ien s wi h IPF su e om excep ionally low HRQOL
oge he wi h se e e b ea hlessness and a igue al eady
wo yea s be o e dea h. In addi ion, physical and
emo ional wellbeing u he de e io a es nea dea h con-
cu en ly wi h escala ing o e all symp om bu den. In
clinical p ac ice, s uc u ed measu emen s o HRQOL
and symp oms a e necessa y o guide high-quali y
ea ly-in eg a ed pallia i e ca e and end-o -li e planning
in IPF pa ien s.
Addi ional ile
Addi ional ile 1: Disease and sociodemog aphic cha ac e is ics. The
ques ionnai e used o collec disease and sociodemog aphic cha ac e is ics.
(DOCX 35 kb)
Abb e ia ions
COPD: Ch onic obs uc i e pulmona y disease; EOL: End-o -li e;
ESAS: Modi ied Edmon on Symp om Assessmen Scale; FVC: Fo ced i al
capaci y; HRQOL: Heal h- ela ed quali y o li e; IPF: Idiopa hic pulmona y
ib osis; IQR: In e qua ile ange; MMRC: Modi ied Medical Resea ch Council
Dyspnea Scale; NRS: Nume ic a ing scale; RAND-36: RAND 36-I em Heal h
Su ey; SD: S anda d de ia ion; SGRQ: S Geo ges Respi a o y Ques ionnai e
Acknowledgemen s
We a e g a e ul o he pa ien s ha consen ed in pa icipa ing his s udy.
The au ho s exp ess g a i ude o he pa icipan s o he FinnishIPF conso ium:
Kaa eenaho R, Saa elainen S, Kankaan an a H, Böök A, Salomaa ER, Kaunis o J,
Hodgson U and Pu oki i M. The au ho s a e also immensely g a e ul o he
nume ous pulmona y physicians, who ha e con ibu ed o he s udy by
including pa ien s and asking o in o med consen s: Vaden J, Pekonen M,
Tapanainen H, Lajunen H, Saa inen A, Suu onen U, Lammi L, Leh onen K,
Männis ö J, Salmi I, To kko M, To kko P, E kkilä M, Ande sen H, Jaakkola J, Rinne H,
Alho M-L, Pie iläinen M, Toljamo T, Palomäki M, Nylund E, Ahonen E, Impola P,
Sa ia o S, Pusa L, Vilkman S, Ek oos H, Vuo i P Hedman J, Lah i M and Mu su A.
Funding
The Academy o Finland, Sig id Jusélius Founda ion, Founda ion o he Finnish
An i-Tube culosis Associa ion, Go e nmen al subsidy o heal h sciences
esea ch ha e suppo ed Lung Fac o esea ch g oup. Kaisa Rajala has ecei ed
g an s men ioned on con lic s o in e es .
A ailabili y o da a and ma e ials
Ou da ase se is no publicly a ailable due o he ela i ely small Finnish IPF
popula ion we could no gua an ee indi iduals anonymi y.
Au ho s’con ibu ions
S udy design: KR, JL, ES, TS, MM. Da a Collec ion: KR, ES, MM. Da a analysis:
KR, JL, ES, TS, MM, HK. W i en epo : KR, JL, ES, TS, MM, HK. All au ho s ead
and app o ed he inal manusc ip . KR akes esponsibili y o he whole wo k.
E hics app o al and consen o pa icipa e
The Finnish Na ional Ins i u e o Heal h and Wel a e (Dn o THL/1161/5.05.01/
2012) app o ed he sc eening o hospi al egis ies o pa ien s wi h IPF. Helsinki
Uni e si y Cen al Hospi al e hics commi ee app o ed his s udy (381/13/03/
01/2014). All pa icipa ing pa ien s ga e hei w i en in o med consen o
pa icipa e o his speci ic s udy.
Consen o publica ion
No applicable.
Compe ing in e es s
Kaisa Rajala has ecei ed g an s om Founda ion o he Finnish An i-
Tube culosis Associa ion, Helsinki Uni e si y Hospi al Comp ehensi e Cance
Fig. 5 Change in he p opo ion o pa ien s wi h MMRC sco e ≥3
du ing he las wo yea s o li e. The cu es we e de i ed om a 5-
kno es ic ed cubic splines logis ic eg ession models. The models
we e adjus ed o age and gende . The g ey a ea ep esen s 95%
con idence in e als
Rajala e al. BMC Pulmona y Medicine (2018) 18:172 Page 7 o 9
Cen e and Väinö and Laina Ki i ounda ion. O he au ho s ha e no con lic s
o in e es a ec ing his wo k.
Publishe ’sNo e
Sp inge Na u e emains neu al wi h ega d o ju isdic ional claims in published
maps and ins i u ional a ilia ions.
Au ho de ails
1
Depa men o Pallia i e Ca e, Comp ehensi e Cance Cen e ,, Helsinki
Uni e si y Hospi al, Paciuksenka u 21, Po BOX 180, FI-00290 Helsinki, Finland.
2
Facul y o Medicine, Uni e si y o Helsinki, Helsinki, Finland.
3
Depa men o
Oncology, Pallia i e Ca e Uni , Tampe e Uni e si y Hospi al and Facul y o
Medicine and Li e Sciences, Uni e si y o Tampe e, Tampe e, Finland.
4
Facul y
o Medicine, Uni e si y o Helsinki, Helsinki, Finland.
5
P ima y Heal h Ca e
Uni , Kuopio Uni e si y Hospi al, Finland and Folkhälsan Resea ch Cen e ,
Helsinki, Finland.
6
Uni e si y o Helsinki and Helsinki Uni e si y Hospi al, Hea
and Lung Cen e , Depa men o Pulmona y Medicine, Helsinki, Finland.
7
Helsinki Uni e si y Hospi al, Comp ehensi e Cance Cen e , Depa men o
Pallia i e Ca e and Facul y o Medicine, Uni e si y o Helsinki, Helsinki,
Finland.
Recei ed: 10 July 2018 Accep ed: 8 No embe 2018
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