QoL questionnaires and ICF in children with cystic fibrosis
the biopsychosocial impacts of CF and ICF have yet to
be established. As such, the objectives of this study were
to identify the main HRQoL questionnaires specifically
designed for children with CF and to examine how their
content is represented in the ICF categories.
METHODS
The study was conducted in 2 stages. The first stage involved a
review of the literature to identify and select specific quality of
life (QoL) questionnaires for children with CF, and the second
consisted of linking the items of the questionnaires to the ICF
according to the methodology proposed by Cieza et al. (11).
The first step was performed on the PubMed, SciELO and
LILACS electronic databases in June 2017 using the descriptors
Quality of Life Questionnaires AND Cystic Fibrosis AND Child.
The search found 176 studies in English, Portuguese and Spanish
between 1980 and 2017, sorted by title and abstract. In the studies
that evaluated the QoL of children with CF, we sought to identify
the most commonly used instruments in the literature. Two ques-
tionnaires were selected: the Cystic Fibrosis Questionnaire (CFQ)
and DISABKIDS® – Cystic Fibrosis Module (DISABKIDS®
– CFM). The former, which has been translated and validated in
different languages (4, 12–14), is widely used in the literature
and evaluates individuals from childhood to adulthood through
4 versions: CFQ 6–11 , CFQ 12–13 , CFQ 14+ and CFQ parents6–13 . Based
on the criteria of the present study, the CFQ 6–11 and CFQ parents6–13
versions, which contain 35 and 44 questions, respectively, were
included in addition to the demographic sections.
The DISABKIDS® – CFM is 1 of 7 modules developed by
the DISABKIDS group for specific health conditions. This short
10-item questionnaire is divided into 2 scales (impact and treat-
ment). The former (4 items) describes the feelings of tiredness
and exhaustion, and the latter (6 items) the emotional impact of
the treatment. There is a version for children and adolescents
from 8 to 18 years (DISABKIDS self ) and another, with parallel
items, for parents or caregivers (DISABKIDS proxy ).
In the second stage of the study, the methodology used to
link the QoL instruments to the ICF (9) was based on the rules
proposed by Cieza et al., in 2002 (15). This methodology has
been updated twice (11, 16) and is widely disseminated in the
583
literature. The link between the items of the QoL instruments
and the ICF was determined by 2 independent physical therapists
with theoretical knowledge and previous practical ICF expe-
rience in health services. In case of disagreement, a third senior
physical therapist with broad ICF experience was consulted in
order to reach a consensus.
Absolute and relative frequencies were used in descriptive
statistical analysis. The degree of agreement between the 2
professionals who established the link between the items and
the ICF was calculated using the kappa coefficient, and the clas-
sification suggested by Landis & Koch (17), in which values
less than zero are poor, between 0 and 0.2 slight, between 0.21
and 0.4 fair, between 0.41 and 0.6 moderate, between 0.61 and
0.8 substantial and between 0.81 and 1.0 almost perfect. In all
analyses, a 95% confidence interval (95% CI) and the Statistical
Package for the Social Sciences (SPSS), version 20.0 were used.
RESULTS
A total of 130 concepts were identified from the 112 items and 47
different ICF categories were linked (QFC 6–11 k=0.646; CFQ pa-
k=0.670; DISABKIDS self and DISABKIDS proxy k=0.622).
rents6–13
Twenty-one (44.7%) concepts were related to the body
function domain, 20 (42.6%) to the activity and participation
domain, and 6 (12.8%) to environmental factors. Thirteen items
(10%) could not be linked to the ICF because they represent
personal factors or are not covered by the ICF. All of these un-
related items were contained in the CFQ – Revised (CFQ parents6–13
and CFQ 6–11 ) (Fig. 1).
The absolute and relative frequencies of the categories iden-
tified in the questionnaires are presented in Table I, and divided
by ICF domains, chapters, and categories.
Half (15) of CFQ parents6–13 categories were related to body fun-
ction (b), 40% (12) to activities and participation (d) and 10% (3)
to environmental factors (e). Four of the 8 body function domain
chapters were linked to these items, the most cited was (b1) –
mental functions, and the most detailed categories were the 4-level
categories b1522 – range of emotion and b1801 – body image.
However, most of the linked categories in CFQ 6–11 were
related to the activities and participation domain (14 = 51.3%),
body function domain (9 = 33.3%) and environmental factors
(4 = 14.3%). Six of the 9 activities and participation domain
Fig. 1. Distribution of the content of the questionnaires in relation to the domains of the ICF. (b) body function; (d) Activity and participation; (e)
environmental factors; Pf: personal factors; nc: not covered; CFQ: Cystic Fibrosis Questionnaire
J Rehabil Med 51, 2019