Cronly 2018

RESPIRATORY CARE Paper in Press. Published on September 25, 2018 as DOI: 10.4187/respcare.
06356
Health-Related Quality of Life in Adolescents and Adults With Cystic

Fibrosis: Physical and Mental Health Predictors
Jennifer A Cronly PhD, Alistair J Duff DClinPsych, Kristin A Riekert PhD,
Anthony P Fitzgerald PhD, Ivan J Perry MD PhD, Elaine A Lehane PhD, Aine Horgan PhD,
Barbara A Howe, Muireann Ni Chroinin MD, and Eileen Savage PhD
BACKGROUND: People with cystic fibrosis face substantial physical, psychological, and social
challenges as they move into adolescence and adulthood, which are likely to impact on their
health-related quality of life. This study sought to examine the relative importance of physical and
mental health variables associated with health-related quality of life in this group. METHODS:
Adults and adolescents (N ⴝ 174; >14 y old) from across 11 adult or pediatric cystic fibrosis clinics
in the Republic of Ireland, completed a background questionnaire that contained self-reported
physical health variables, pulmonary function (ie, FEV1%) and body mass index. Questionnaire
packs also contained the Hospital Anxiety and Depression Scale (HADS) and the Cystic Fibrosis
Questionnaire-Revised, which has been specifically designed to assess health-related quality of life
in patients with cystic fibrosis. RESULTS: HADS depression and/or anxiety scores were negatively
associated with 11 of the 12 Cystic Fibrosis Questionnaire-Revised domain scores. FEV1% was
positively associated with 8 domains when controlling for HADS anxiety but only 4 domains when
controlling for HADS depression. HADS anxiety and depression scores demonstrated larger effect
sizes and explained a greater proportion of the variance than pulmonary function in 8 of the
12 Cystic Fibrosis Questionnaire-Revised domain scores. CONCLUSIONS: Mental health vari-
ables, depression and anxiety, were strongly associated with health-related quality of life in subjects
with cystic fibrosis and demonstrated greater effect sizes and explained a higher proportion of the
variance overall than the physical health indicators, FEV1% and body mass index, which high-
lighted the importance of screening for, and treating, depression and anxiety symptoms. Key words:
anxiety; depression; cystic fibrosis; predictors; health-related quality of life (HRQOL); mental health;
physical health. [Respir Care 0;0(0):1–•. © 0 Daedalus Enterprises]
Introduction lenges.1,2 Adolescence can be a particularly challenging

developmental period due to rapid physical, psychologi-
Adolescents and and adults living with cystic fibrosis cal, and social changes, which may be accompanied by
have significant physical, psychological, and social chal-
The study was conducted through University College Cork, Cork, Re-
public of Ireland.
Prof Savage, Drs Cronly, Horgan, Lehane, and Ms Howe are affiliated Funding was obtained from Cystic Fibrosis Ireland and the Health Re-
with the School of Nursing and Midwifery in University College Cork, search Board Ireland (grant file reference MRCG/2011/9).
Cork, Republic of Ireland. Dr Duff is affiliated with the Department of
Clinical Health Psychology in the Leeds Teaching Hospitals, NHS Trust, The authors have disclosed no conflicts of interest.
Leeds, United Kingdom. Prof Riekert is affiliated with the Johns Hopkins
Adherence Research Center in the John Hopkins School of Medicine, Correspondence: Eileen Savage PhD, School of Nursing and Midwifery,
Baltimore, Maryland. Prof Perry and Dr Fitzgerald are affiliated with the Brookfield Health Sciences Complex, University College Cork, Western
School of Public Health in University College Cork, Cork, Republic of Road, Cork, Republic of Ireland. E-mail: [email protected].
Ireland. Dr Ni Chroinin is affiliated with Department of Pediatrics in
Cork University Hospital, Cork, Republic of Ireland. DOI: 10.4187/respcare.06356
RESPIRATORY CARE • ● ● VOL ● NO ● 1

Copyright (C) 2018 Daedalus Enterprises ePub ahead of print papers have been peer-reviewed, accepted for publication, copy edited
and proofread. However, this version may differ from the final published version in the online and print editions of RESPIRATORY CARE
RESPIRATORY CARE Paper in Press. Published on September 25, 2018 as DOI: 10.4187/respcare.06356
PREDICTORS OF HRQOL IN CYSTIC FIBROSIS
declining health, increasing treatment burden, and greater

awareness of the potential negative consequences of the QUICK LOOK
illness.3-5 As adolescents move into young adulthood, they Current knowledge
also face increasing social and psychological challenges,
Living with cystic fibrosis poses significant chal-
such as moving out of the family home, becoming more
lenges, and people with cystic fibrosis are at risk of
independent, forming intimate relationships, and entering
depression and anxiety symptoms. It is important to
third-level education or the employment sector.6,7 In ad-
examine the variables associated with health-related
dition, individuals with cystic fibrosis may face challenges
quality of life in this population so that it can be
associated with sexuality, fertility, and parenthood.5,7,8 Rec-
strengthened and maintained. Physical health indica-
ognition of these challenges has led to an increasing focus tors, FEV1%, and recent pulmonary exacerbations,
on the importance of psychological and social functioning, have been linked to health-related quality of life in
and health-related quality of life (HRQOL) in both ado- this population.
lescents and adults with cystic fibrosis.
As a multidimensional concept, HRQOL refers to the What this paper contributes to our knowledge
impact of disease on holistic well-being as well as on Anxiety and depression symptoms are significantly as-
physical, social, and psychological functioning.9,10 The pa- sociated with health-related quality of life in people
tient’s own perspective is valued, including the subjective with cystic fibrosis. Analysis of our data indicated that
experience of emotional well-being, the impact of symp- anxiety and depression symptoms impacted health-re-
toms on his or her life ability to participate in daily activ- lated quality of life in its broadest sense, and affected
ities, social engagements, education, and employment, and physical, emotional, and social functioning. Mental
relationships with others and the environment.11-13 Recogni- health indicators demonstrated larger effect sizes and
tion of the importance of HRQOL in people with cystic fi- explained more of the variance than physical health
brosis has led to a growing body of research and the devel- indicators across a wide range of health-related quality-
opment of a specific HRQOL instrument, the Cystic Fibrosis of-life domains.
Questionnaire-Revised.14 In reflecting its multidimensional
nature, this instrument measures HRQOL across 12 domains,
including physical functioning, emotional functioning, social
iety symptoms,16 it is important to investigate the impact
functioning, role, vitality, body image, eating, treatment bur-
these have on HRQOL.
den, health perceptions, weight, respiratory symptoms, and
A small number of studies investigated the associations
digestive symptoms.
between depression and anxiety, and HRQOL in people
A systematic review of 23 studies examined the rela- with cystic fibrosis, which indicated that these symptoms
tionship between HRQOL in people with cystic fibrosis are negatively associated with HRQOL in this popula-
and demographic and physical health variables, including tion.2,17-19 Few studies used multivariate analysis to quan-
age, sex, socioeconomic status, employment status, pul- tify the effects of depression and anxiety on HRQOL. One
monary function (ie, FEV1%), pulmonary exacerbations, study, of 129 adults in a single United Kingdom cystic
and body mass index (BMI).15 FEV1% and pulmonary fibrosis center, showed depression and anxiety symptoms
exacerbations emerged as the strongest predictors of to be significant negative predictors of HRQOL, and ex-
HRQOL, with studies consistently finding 9 of the 12 as- plained the greater degree of variance than the physical
sessed domains positively associated with FEV1% and 8 of health indictors, including FEV1%, BMI, or recent hospi-
the 12 domains negatively associated with pulmonary ex- talizations.2 HRQOL is a multidimensional concept how-
acerbations. BMI was significantly correlated with just ever, and this study did not examine the various domains
2 Cystic Fibrosis Questionnaire-Revised domains: body of HRQOL including physical, social, or emotional func-
image and weight. Sex also emerged as significant, with tioning.
males reporting higher scores in physical functioning and Another study found that, when controlling for FEV1%,
females reporting higher scores in body image. Age was anxiety was a significant predictor of 6 HRQOL domains
negatively correlated with just 1 domain, treatment bur- (vitality, emotional functioning, social functioning, treat-
den. Although indicators of physical health emerged as ment burden, health perceptions, and respiratory symp-
strong predictors of HRQOL, this systematic review did toms) and that depression was a significant predictor of
not examine the role of mental health variables. Few stud- 3 domains (emotional functioning, eating, and body im-
ies have examined the relationship between mental health age).19 However, this study did not examine the variance
indicators, such as depression and anxiety symptoms, and accounted for by physical and mental health variables.
HRQOL in people with cystic fibrosis. Given that individ- There is a dearth of studies that used multivariate methods
uals with cystic fibrosis are at risk of depression and anx- to examine the relative contribution of both physical and
2 RESPIRATORY CARE • ● ● VOL ● NO ●

mental health variables in predicting HRQOL across do- recent clinic appointment. The height and weight variables
mains, which possibly led to confounding bias in reported were used to calculate BMI. The HADS is a depression
findings. It is important to disentangle the effects of phys- and anxiety screening tool suitable for use in community
ical and mental health variables so that HRQOL can be and hospital populations22 and has been validated for use
optimized in patients with cystic fibrosis. The aim of in adolescents ages ⬎12 y.23 It contains 14 items: 7 de-
this study was to examine the variables associated with pression and 7 anxiety. Both depression and anxiety scales
HRQOL in adolescents and adults with cystic fibrosis score ranges are 0 –21. Established thresholds indicate el-
with a focus on the relative importance of physical health evated depression or anxiety symptoms for a score of ⬎7 on
indicators, FEV1% and BMI, and of the mental health either of the respective subscales. A score of ⬎10 indi-
variables, depression and anxiety symptoms. cates clinically important symptoms of depression or anx-
iety. Previous research has indicated that the HADS has
Methods excellent internal consistency, test-retest reliability, dis-
criminant validity, and factor structure.23,24
Study Design The Cystic Fibrosis Questionnaire-Revised is a valid and
reliable measure of HRQOL in people with cystic fibrosis.14
This study was part of a large, national, cross-sectional The version developed for patients ages ⱖ14 y has 50 items
study on the prevalence of depression and anxiety in ad- that are categorized under 9 HRQOL domains and 3 symp-
olescents and adults with cystic fibrosis and their caregiv- tom domains. With regard to the multidimensional nature of
ers. This paper reported only on the data from adolescents HRQOL, the domains include the following: physical func-
and adults with cystic fibrosis and ages ⱖ14 y. Ethical ap- tioning, role, vitality, emotional functioning, social function-
proval to conduct the study was granted by the relevant Clin- ing, body image, eating, treatment burden, health percep-
ical Research Ethics Committee (Ref ECM4[ii]04/12/12). tions, weight, respiratory symptoms, and digestive symptoms.
Scaled scores for each of the domains range from 0 to 100;
Participants higher scores indicate higher HRQOL. The Cystic Fibrosis
Questionnaire-Revised has strong psychometric properties
The sample of 174 adults and adolescents ages ⱖ14 y with good internal validity, adequate reliability, and clinical
with cystic fibrosis were recruited from 11 adult or pedi- sensitivity.14,15,25
atric cystic fibrosis centers across 9 hospitals in the Re-
public of Ireland. Inclusion criteria were a cystic fibrosis Procedure
diagnosis by sweat test and age ⱖ14 y old. Exclusion
criterion was having received an organ transplantation. Adult patients were recruited during a routine clinic
According to data published by the Cystic Fibrosis Reg- visit or online. However, adolescents were only recruited
istry of Ireland in 2014 (the year of data collection), there at clinic visits. Of the 174 participants, 94 were recruited
were 595 adults and 342 adolescents (12–18 y old), with from clinic settings and 80 were recruited online. In the
cystic fibrosis registered as living in the Republic of Ire- clinics, the adults gave informed consent, and parental
land.20 It was calculated that a sample size of 382 adults informed consent was required before the research team
and 248 adolescents would be needed to estimate the prev- approached the adolescents. The participants had the op-
alence rates of depression and anxiety to within 3% with tion of completing the questionnaire in the clinic or later at
95% confidence. However, due to challenges in recruit- home. Those who opted for the latter (n ⫽ 28) were pro-
ment, this sample size could not be attained.21 The sample vided with a stamped addressed envelope for return of the
size of 141 adults and 33 adolescents (14 –18 y old) in- questionnaire to the research team. The overall response
cluded in the HRQOL analysis represented 23.7% of the rate for recruitment in adult clinics was 51%, and 39% in
adult and 9.6% of the adolescent cystic fibrosis population pediatric clinics.
in Ireland, respectively. Challenges associated with access and data collection in
clinics resulted in slow recruitment, and, consequently, the
Measures decision was made to also recruit online.21 An e-mail link
to the questionnaire was sent by the charity Cystic Fibrosis
Questionnaire packs contained the following instru- Ireland to its community network of 345 adults with cystic
ments: a background questionnaire, the Hospital Anxiety fibrosis. The online version of the questionnaire was de-
and Depression Scale (HADS), and the Cystic Fibrosis veloped and administered by using the cloud-based soft-
Questionnaire- Revised for adolescents and adults ⱖ14 y. ware SurveyMonkey (at SurveyMonkey.com, San Mateo,
The background questionnaire contained items on demo- California). Along with the link to the questionnaire, an
graphic and physical health variables, including self-re- online information sheet and a consent form were also
ported height, weight, and FEV1% obtained from the most included. A total of 99 online questionnaires were returned,

Table 1. Demographic and Physical and Mental Health Variables this, the diagnosis was based on clinical presentation and
diagnostic sweat testing.
Variable Results
Age, mean ⫾ SD y (N ⫽ 174) 27.4 ⫾ 9.4

Sex, n (%) Physical and Mental Health Indicators
Male 82 (47.7)
Female 90 (52.3)
The mean ⫾ SD value for FEV1% was 69.0% ⫾ 24.8%
Physical health indicators, mean ⫾ SD
of predicted (n ⫽ 122). Of the sample, 15.6% (n ⫽ 19) had
FEV1% (n ⫽ 122) 69.0 ⫾ 24.8
BMI, kg/m2 (n ⫽ 119) 21.5 ⫾ 2.7
an FEV1% value of ⬍40%, which was indicative of ad-
Mental health indicators, mean ⫾ SD score vanced pulmonary disease.28 The mean ⫾ SD value for
HADS anxiety (n ⫽ 173) 5.6 ⫾ 4.0 BMI was 21.5 ⫾ 2.7 kg/m2 (n ⫽ 119), and 15.1% (n ⫽ 18)
HADS depression (n ⫽ 173) 2.7 ⫾ 3.2 had a BMI of ⬍19 kg/m2, which is associated with in-
creased risk of mortality in patients with cystic fibrosis.30
BMI ⫽ body mass index
HADS ⫽ Hospital Anxiety and Depression Scale The mean ⫾ SD scores for HADS anxiety and depression
were 5.6 ⫾ 4.0) and 2.7 ⫾ 3.2, respectively. Descriptive
statistics for physical and mental health variables are pre-
sented in Table 1. In total, 26.0% of the participants (n ⫽ 45)
although 19 were incomplete and not included for analy- had elevated anxiety symptom scores (⬎7) and 12.7%
ses. The 80 online responses represented 23% of the avail- (n ⫽ 22) had scores in the clinically important range (⬎10).
able population. For depression, 8.1% (n ⫽ 14) had elevated symptoms and
3.5% (n ⫽ 6) had symptoms in the clinically important
Statistical Analysis range.
Data were analyzed by using IBM SPSS 22.0 for Win-
dows (IBM, Armonk, New York). Descriptive statistics Predictors of HRQOL
were performed to describe participant demographics. To
determine the predictors of Cystic Fibrosis Questionnaire-
HADS anxiety was a significant negative predictor of
Revised domain scores, multiple linear regressions were
10 of the 12 Cystic Fibrosis Questionnaire-Revised do-
conducted. For each multiple regression model, the Cystic
main scores. In the regression models, in which anxiety
Fibrosis Questionnaire-Revised domain score was entered
was included as a predictor, FEV1% was a significant
as the dependent variable, and age, sex, FEV1% BMI, and
positive predictor for 8 of the 12 domains. Age was not a
either HADS anxiety or depression were entered as inde-
significant predictor for any of the Cystic Fibrosis Ques-
pendent variables. Standardized coefficients were used as
tionnaire-Revised domains in these models. Sex was a
a measure of relative effect size, and squared part corre-
lational coefficients were used to calculate the proportion significant predictor of body image and weight because
of the variance explained by each predictor in the model. being male was associated with lower scores in each of
Depression and anxiety were not entered into the same these domains. The values for these regression models, the
model due to collinearity (r ⫽ 0.62, P ⫽ .001). effect sizes, and the proportion of the variance accounted
for by each predictor are illustrated in Table 2.
Results HADS depression was a significant negative predictor
of 11 of the 12 Cystic Fibrosis Questionnaire-Revised do-
Sample Demographics mains. In the regression models in which depression was
included as a predictor, FEV1% was a significant positive
Participants (N ⫽ 174) ranged in age from 14 to 64 y predictor of 4 of the 12 domains. BMI was a significant
(mean ⫾ SD, 27.4 ⫾ 9.4 y), of whom, 33 (19.2%) were predictor of 2 domains in these regression models. Age
adolescents ages 14 –18 y. Just over half of the sample was not a significant predictor of any of the domain scores,
(52.3% [n ⫽ 90]) were female, and 47.7% (n ⫽ 82) were but sex was a significant predictor of weight, body image,
male. All identified their nationality as Irish (Table 1). and physical functioning because being male was associ-
The mean ⫾ SD age of cystic fibrosis diagnosis was ated with lower Cystic Fibrosis Questionnaire-Revised
3.9 ⫾ 8.6 y. Consensus statements in 200426 and 200527 scores in weight and body image but higher scores in
recommended neonatal screening programs for cystic fi- physical functioning. The values for these regression mod-
brosis in every country. However, neonatal screening for els, along with effect sizes and the proportion of the vari-
cystic fibrosis consistent with best practice guidelines28 ance, accounted for by each predictor are presented in
was not introduced into Ireland until 2011,29 and, before Table 3.

Table 2. Multiple Linear Regressions With CFQ-R Domain Scores as Dependent Variables and With Age, Sex, FEV1%, BMI, and HADS Anxiety
as Independent Variables
Regression
Standardized Part
Dependent Variable, Independent Model
Coefficient
CFQ-R Domains Variable
Correlation Adjusted R2
Coefficient2
␤ P % P
Physical functioning 52.3 ⬍.001

Age ⫺0.08 .29 ⬍0.01
Sex 0.12 .09 0.01
FEV1% 0.49 .001 0.19
BMI 0.12 .14 0.01
HADS anxiety ⫺0.34 .001 0.10
Role 21.0 ⬍.001
Age 0.12 .24 0.01
Sex ⫺0.09 .36 ⬍0.01
FEV1% 0.32 .002 0.08
BMI 0.06 .52 ⬍0.01
HADS anxiety ⫺0.31 .002 0.09
Vitality 23.4 ⬍.001
Age ⫺0.08 .42 ⬍0.01
Sex 0.06 .50 ⬍0.01
FEV1% 0.21 .037 0.04
BMI ⫺0.03 .73 ⬍0.01
HADS anxiety ⫺0.39 .001 0.14
Emotional functioning 54.8 ⬍.001
Age ⫺0.01 .86 ⬍0.01
Sex 0.01 .91 ⬍0.01
FEV1% 0.18 .02 0.03
BMI 0.05 .55 ⬍0.01
HADS anxiety ⫺0.68 .001 0.43
Social functioning 11.9 ⬍.001
Age ⫺0.03 .81 ⬍0.01
Sex 0.01 .94 ⬍0.01
FEV1% 0.28 .011 0.06
BMI ⫺0.01 .93 ⬍0.01
HADS anxiety ⫺0.22 .03 0.05
Body image 31.6 ⬍.001
Age ⫺0.08 .39 ⬍0.01
Sex ⫺0.26 .003 0.07
FEV1% 0.15 .12 0.02
BMI 0.14 .13 0.02
HADS anxiety ⫺0.46 .001 0.20
Eating 20.5 ⬍.001
Age 0.04 .72 ⬍0.01
Sex 0.03 .73 ⬍0.01
FEV1% 0.13 .20 0.04
BMI 0.22 .03 0.05
HADS anxiety ⫺0.36 .001 0.12
Treatment burden 13.9 ⬍.001
Age 0.03 .80 ⬍0.01
Sex 0.01 .91 ⬍0.01
FEV1% 0.36 .001 0.11
BMI 0.05 .64 ⬍0.01
(continued)

Table 2. Continued
Regression
Standardized Part
Dependent Variable, Independent Model
Coefficient
CFQ-R Domains Variable
Coefficient2
␤ P % P
Health perceptions 54.6 ⬍.001

Age ⫺0.10 .21 0.01
Sex ⫺0.06 .37 ⬍0.01
FEV1% 0.51 .001 0.21
BMI 0.06 .40 ⬍0.01
HADS anxiety ⫺0.39 .001 0.14
Weight 19.7 ⬍.001
Age ⫺0.10 .31 0.01
Sex ⫺0.26 .006 0.07
FEV1% 0.12 .24 0.01
BMI 0.39 .001 0.13
Respiratory symptoms 32.0 ⬍.001
Age 0.01 .90 ⬍0.01
Sex 0.05 .56 ⬍0.01
FEV1% 0.47 .001 0.18
BMI 0.09 .35 0.01
Digestive symptoms 20.4 ⬍.001
Age ⬍0.01 .98 ⬍0.01
Sex 0.06 .53 ⬍0.01
FEV1% 0.11 .08 0.01
BMI 0.04 .70 ⬍0.01
HADS anxiety ⫺0.44 .001 0.18
N ⫽ 121.
CFQ-R ⫽ Cystic Fibrosis Questionnaire-Revised
HADS ⫽ Hospital Anxiety and Depression Scale
Discussion pression symptoms is important to maintaining overall well-

being, and physical, social, and emotional functioning in
In examining the associations between HRQOL and so- patients with cystic fibrosis.
ciodemographic, physical, and mental health variables, this Depression and anxiety have distinct clinical features
study highlighted the role of mental health in HRQOL in but frequently co-exist31 and were correlated in this sam-
people with cystic fibrosis. Previous research identified ple. This statistical correlation made it difficult to disen-
associations between physical health variables, such as tangle the distinctive contribution of each in predicting
FEV1% and HRQOL,15 but the results of this study indi- HRQOL. Nevertheless, the separate regression models in-
cate that mental health variables, depression and anxiety, dicated that both anxiety and depression symptoms were
are more strongly associated with HRQOL than physical associated with HRQOL across a wide range of domains.
health indicators. Depression and anxiety symptoms are Depression symptoms account for high proportions of the
associated with all but 1 Cystic Fibrosis Questionnaire- variance in social functioning, vitality, and role, which
Revised domain, with larger effect sizes and explaining indicated that these symptoms had a strong impact on an
more of the variance in 8 of the 12 HRQOL domain scores. individual’s ability or desire to interact with peers and
Analysis of our data underlined the impact that depression engage in daily activities, education, or work. Alterna-
and anxiety symptoms have on HRQOL in its broadest tively, anxiety symptoms accounted for a high degree of
sense, which affect an individual’s participation in social the variance in the domains of emotional functioning and
activities, education or employment, their sense of vitality, body image, which indicated that anxiety may disrupt one’s
physical and emotional health, and how they experience sense of confidence and well-being. Analysis of these re-
eating and digestion. Therefore, managing anxiety and de- sults also indicated that co-morbidity of depression and

Table 3. Multiple Linear Regressions With CFQ-R Domain Scores as Dependent Variables and Age, Sex, FEV1%, BMI, and HADS Depression
as Independent Variables
Regression
Standardized Part
Dependent Variable Independent Model
Coefficient
CFQ-R Domains Variables
Coefficient2
␤ P % P
Physical functioning 52.7 ⬍.001

Age ⫺0.06 .44 ⬍0.01
Sex 0.15 .040 0.02
FEV1% 0.42 .001 0.13
BMI 0.13 .10 0.01
HADS depression ⫺0.37 .001 0.11
Role 35.7 ⬍.001
Age 0.16 .09 0.02
Sex ⫺0.07 .81 ⬍0.01
FEV1% 0.18 .069 0.02
BMI 0.08 .36 0.01
Vitality 34.0 ⬍.001
Age ⫺0.04 .65 ⬍0.01
Sex 0.09 .30 0.01
FEV1% 0.08 .41 0.01
BMI ⫺.02 .87 ⬍0.01
Emotional functioning 51.1 ⬍.001
Age 0.03 .72 ⬍0.01
Sex 0.06 .41 ⬍0.01
FEV1% 0.06 .52 ⬍0.01
BMI 0.07 .35 ⬍0.01
Social functioning 27.8 ⬍.001
Age 0.01 .88 ⬍0.01
Sex 0.02 .82 ⬍0.01
FEV1% 0.13 .21 0.01
BMI 0.01 .95 ⬍0.01
Body image 28.3 ⬍.001
Age ⫺0.05 .58 ⬍0.01
Sex ⫺0.23 .01 0.05
FEV1% 0.07 .48 ⬍0.01
BMI 0.16 .10 0.02
Eating 29.5 ⬍.001
Age 0.07 .45 ⬍0.01
Sex 0.06 .52 ⬍0.01
FEV1% 0.01 .90 ⬍0.01
BMI 0.24 .01 0.05
Treatment burden 17.3 ⬍.001
Age 0.05 .66 ⬍0.01
Sex 0.02 .83 ⬍0.01
FEV1% 0.29 .01 0.06
BMI 0.06 .56 ⬍0.01
(continued)

Table 3. Continued
Regression
Standardized Part
Dependent Variable Independent Model
Coefficient
CFQ-R Domains Variables
Coefficient2
␤ P % P
Health perceptions 60.0 ⬍.001

Age ⫺0.07 .37 ⬍0.01
Sex ⫺0.04 .59 ⬍0.01
FEV1% 0.41 .001 0.12
BMI 0.08 .26 0.01
Weight 20.1 ⬍.001
Age ⫺0.10 .35 0.01
Sex ⫺0.26 .007 0.06
FEV1% 0.09 .39 0.01
BMI 0.39 .001 0.13
Respiratory symptoms 31.5 ⬍.001
Age 0.03 .79 ⬍0.01
Sex 0.07 .43 ⬍0.01
FEV1% 0.43 .001 0.13
BMI 0.10 .30 0.01
Digestive symptoms 25.6 ⬍.001
Age 0.04 .70 ⬍0.01
Sex 0.09 .32 0.01
FEV1% ⫺0.003 .98 ⬍0.01
BMI 0.06 .55 ⬍0.01
n ⫽ 121.
CFQ-R ⫽ Cystic Fibrosis Questionnaire-Revised
HADS ⫽ Hospital Anxiety and Depression Scale
anxiety symptoms is likely to have a more negative impact the variance accounted for by age and sex were very small
on HRQOL than each symptom alone. in our study, which indicate that physical and mental health
The sociodemographic variables of age and sex were indicators play a more important role in predicting HRQOL.
not strongly associated with HRQOL in this study. Previ- Physical health was significantly associated with
ous research indicates that males have higher HRQOL HRQOL in our study because FEV1% was associated with
overall than females,32 but multivariate analysis in this a wide range of domains, including physical function and
study revealed that, when physical and mental health in- vitality but also social functioning and role. These results
dicators are taken into account, being male is positively indicated that poorer lung function may hinder an individ-
associated with just 1 domain, physical functioning. Being ual’s ability to interact with peers and participate in
female is associated with higher scores in the domains of daily activities. However, when the effects of depres-
body image and weight. Previous work indicated that a sion are controlled, FEV1% explains little of the vari-
lower BMI may have a more negative impact on HRQOL ance in the domains of role, vitality, or social and emo-
in males with cystic fibrosis because they may wish to be tional functioning. Therefore, this study provided
more muscular and robust compared with females with evidence that, although poor or declining physical health
cystic fibrosis, for whom a lower BMI is considered de- can have a negative impact on the multifaceted aspects
sirable.32 Females with cystic fibrosis were reported to be of HRQOL, depression symptoms mediate this effect
more content with their body shape and weight than healthy and play a greater role in predicting the variance across
controls,33 but lower BMI is associated with lower FEV1% most domains.
in both males and females and, therefore, is a cause of When interpreting the results of our study, some limi-
concern.34 Nevertheless, the effect sizes and proportions of tations must be taken into consideration. First, the study

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RESPIRATORY CARE Paper in Press. Published on September 25, 2018 as DOI: 10.4187/respcare.

Health-Related Quality of Life in Adolescents and Adults With Cystic

Introduction lenges.1,2 Adolescence can be a particularly challenging

RESPIRATORY CARE • ● ● VOL ● NO ● 1

PREDICTORS OF HRQOL IN CYSTIC FIBROSIS

declining health, increasing treatment burden, and greater

2 RESPIRATORY CARE • ● ● VOL ● NO ●

PREDICTORS OF HRQOL IN CYSTIC FIBROSIS

RESPIRATORY CARE • ● ● VOL ● NO ● 3

PREDICTORS OF HRQOL IN CYSTIC FIBROSIS

Age, mean ⫾ SD y (N ⫽ 174) 27.4 ⫾ 9.4

4 RESPIRATORY CARE • ● ● VOL ● NO ●

PREDICTORS OF HRQOL IN CYSTIC FIBROSIS

Physical functioning 52.3 ⬍.001

RESPIRATORY CARE • ● ● VOL ● NO ● 5

PREDICTORS OF HRQOL IN CYSTIC FIBROSIS

Health perceptions 54.6 ⬍.001

Discussion pression symptoms is important to maintaining overall well-

6 RESPIRATORY CARE • ● ● VOL ● NO ●

PREDICTORS OF HRQOL IN CYSTIC FIBROSIS

Physical functioning 52.7 ⬍.001

RESPIRATORY CARE • ● ● VOL ● NO ● 7

PREDICTORS OF HRQOL IN CYSTIC FIBROSIS

Health perceptions 60.0 ⬍.001

8 RESPIRATORY CARE • ● ● VOL ● NO ●

PREDICTORS OF HRQOL IN CYSTIC FIBROSIS

adopted 2 recruitment processes for adults, in clinics and REFERENCES

RESPIRATORY CARE • ● ● VOL ● NO ● 9

PREDICTORS OF HRQOL IN CYSTIC FIBROSIS

10 RESPIRATORY CARE • ● ● VOL ● NO ●

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