QoL questionnaires and ICF in children with cystic fibrosis
chapters were represented in the linked categories, chapter d4
(mobility) and categories d4551 (climbing) and d920 (recreation
and leisure) being the most cited.
Only 9 of the 47 categories identified were linked to the items
of DISABKIDS® (DISABKIDS self and DISABKIDS proxy ver-
sion), most pertaining to the body function domain (5 = 55.6%)
in both versions, representing 2 of the 8 chapters that make up
this domain, namely (b1) – mental functions, with category b152
(emotional functions) being the most cited; and (b4) – functions
of the cardiovascular, haematological, immunological and
respiratory systems. Category frequency was 22.2% (2) in the
activities and participation and environmental factors domains
in both versions of the questionnaires.
DISCUSSION
Comparing HRQoL measures with the ICF may reveal its
uniqueness, demonstrating the nature and scope of its content.
In most cases, instruments focus on different aspects of ICF
domains, with “body functions” and “activities and participa-
tion” components usually predominating (7, 8). In the 4 versions
presented here (CFQ parents6–13 ; CFQ 6–11 ; DISABKIDS self ; and
DISABKIDS proxy ), the categories primarily represented body
functions, except for the CFQ 6–11 , version, where the activities
and participation component was the most frequent. By contrast,
environmental factors were poorly described, and none of the
categories were related to body structures.
Although chapters b1 to b5 have been linked to the items
identified, the only chapters that contained categories cited in all
4 versions were Mental functions (b1) and Functions of the car-
diovascular, haematological, immunological and respiratory sys-
tems (b4). Category b152 (emotional functions) has often been
linked in the different instruments (20 times), because a number
of feelings/sensations, including “happy”, “worried”, “angry”,
“annoyed”, “sad”, “upset”, and “bothered”, were represented
in this category. The high attachment index of this category to
different feelings has been addressed in other studies (8, 18).
Because of the clinical manifestations present in the scope
of body functions resulting from the multisystemic nature of
CF, the questionnaires can be used to address a large number
of aspects related to this domain (7, 8, 19). Studies that seek to
identify clinical factors associated with HRQoL in individuals
with CF have concluded that variables assessing exacerbation
and pulmonary function have the greatest impact on HRQoL
(4, 20, 21). Multidirectional relationships between ICF domains
demonstrate that body functions are only 1 aspect for practition-
ers to consider. Other domains should also be incorporated (22).
The complex treatment these children are subjected to, includ-
ing ingested and inhaled medication, nebulization, antibiotics,
pancreatic enzymes, nutritional supplements, respiratory physi-
cal therapy techniques, and hospitalization, is time-consuming
and a burdensome daily routine (3, 13). These factors, together
with body function limitations, may interfere with the perfor-
mance of daily activities and community participation.
The CFQ 6–11 was the only instrument where most items were
related to the activities and participation domain, although the
categories linked CFQ parents6–13 to chapters d1 (learning and app-
lying knowledge) and d2 (general tasks and demands), a finding
not observed in the children’s version. The most representative
chapters were d4 (mobility) and d9 (community, social and
civic life), the latter addressed in all the questionnaires, and
d920 (recreation and leisure) the most frequent category. Since
much of the daily management of CF occurs at home, obser-
ving the child’s ability to perform age-appropriate activities,
585
and participate in typical school-based and leisure activities is
of paramount importance, since participation in the activities
of daily living is considered essential to child development, in
addition to contributing to HRQoL, and is the ultimate goal of
rehabilitation programmes (22–24).
Environmental factors play an important role in children’s
participation in home, school and community activities, and
can act as a support or barrier (25). Despite their relevance,
environmental factors were the categories least covered by
the 4 versions. In this component, the most cited category was
e5800 (health services).
Other categories addressed in this component were education
and training services (e5850); acquaintances, peers, colleagues,
neighbours and community members (e325); societal attitudes
(e460); individual attitudes of acquaintances, peers, colleagues,
neighbours and community members (e425); and drugs (e1101).
According to the model presented by the ICF, the functionality
of an individual with a specific health condition depends on
body-related aspects, as well as personal and environmental
factors (9). The weakness of the instruments in failing to suf-
ficiently address these factors limits an extended evaluation, as
proposed by the ICF, and makes it difficult to identify possible
facilitators or barriers that the environment imposes on children
with CF and their impact on QoL, in addition to showing the
need to refine paediatric HRQoL instruments. This recurrent
weakness has been demonstrated in other studies (7, 8).
In the context faced by children with CF, categories such as:
e310 (immediate family); e355 (health professionals); e410
(individual attitudes of immediate family members); and e450
(individual attitudes of health professionals) could have been
addressed. The environment, access to healthcare and drugs, and
support from the spouse, family, friends, teachers, classmates
and colleagues are important aspects that influence the HRQoL
reported by individuals with CF (26).
All items that could not be linked to the ICF belonged to the
CFQ – Revised versions, most of which referred to personal
factors contained in the demographic sections that make up these
instruments. Age, sex, body mass index (BMI), and other factors
seem to have an impact on HRQoL (27–29). Identifying these
factors in the linking process may reveal a number of relevant
personal factors to add to the ICF if classification of personal
factors is desired in the future (18, 30). The items classified as
not covered (nc) were contained only in the CFQ parents6–13 version,
exhibiting comprehensive content, including “my child leads a
normal life” (item 24), and “my child has a feeding problem”
(item 44), or very specific items such as “my child’s phlegm
has been predominant” (item 37).
This study sought to link the items of 2 specific instruments
that evaluate the HRQoL of children with CF. The included
questionnaires display good psychometric properties. DISAB-
KIDS® contains few items, thereby optimizing application and
assessment time; however, more than half of its items are linked
to body function categories. The versions of the CFQ were
linked to a larger number of categories that addressed the 4 ICF
domains. The CFQ 6–11 was the only version in which the activity
and participation domain exhibited the highest frequency, but did
not address some of the chapters related to the functions of the
digestive, metabolic and endocrine systems (b5), learning and
applying knowledge (d1), and general tasks and demands (d2)
that were covered in the parents’ version. Thus, we suggest the
simultaneous use of the CFQ 6–11 and CFQ parents6–13 questionnaires.
In conclusion, the main HRQoL questionnaires specifically
designed for children with CF were CFQ and DISABKIDS®. The
link between the items of the instruments and the ICF domains
made it possible to acquire information on the scope of the human
J Rehabil Med 51, 2019