REVIEW ARTICLE
Children’s Experiences of Cystic Fibrosis: A Systematic
Review of Qualitative Studies
AUTHORS: Nathan Jamieson,a Dominic Fitzgerald, PhD,b,c
Davinder Singh-Grewal, PhD,b,c Camilla S. Hanson, B Psych
(Hons),a,d Jonathan C. Craig, PhD,a,d and Allison Tong, PhDa,d
aKids
Research Institute, bRespiratory Medicine, and cPaediatrics
& Child Health, Children’s Hospital at Westmead, Westmead, NSW,
Australia; and dSchool of Public Health, The University of Sydney,
Sydney, NSW, Australia
KEY WORDS
cystic fibrosis, qualitative research, pediatrics, adolescent,
systematic review
ABBREVIATIONS
CF—cystic fibrosis
CINAHL—Cumulative Index to Nursing and Allied Health Literature
Mr Nathan Jamieson carried out the data collection and
analyses, coded the data, and drafted the initial manuscript;
Dr Fitzgerald, Dr Singh-Grewal, Ms Hanson, and Dr Craig
contributed to the initial analyses and reviewed and revised the
manuscript; Dr Tong conceptualized and designed the study and
drafted the initial manuscript; and all authors approved the final
manuscript as submitted.
www.pediatrics.org/cgi/doi/10.1542/peds.2014-0009
doi:10.1542/peds.2014-0009
Accepted for publication Mar 10, 2014
Address correspondence to Allison Tong, PhD, Centre for Kidney
Research, The Children’s Hospital at Westmead, Westmead, NSW
2145. E-mail: [email protected]
PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275).
Copyright © 2014 by the American Academy of Pediatrics
FINANCIAL DISCLOSURE: The authors have indicated they have
no financial relationships relevant to this article to disclose.
FUNDING: No external funding. Dr Tong is supported by the
National Health and Medical Research Council Fellowship
(1037162).
POTENTIAL CONFLICT OF INTEREST: The authors have indicated
they have no potential conflicts of interest to disclose.
abstract
BACKGROUND AND OBJECTIVE: Cystic fibrosis (CF) is a common lifeshortening genetic disease and is associated with poor psychosocial
and quality of life outcomes. The objective of this study was to describe
the experiences and perspectives of children and adolescents with CF
to direct care toward areas that patients regard as important.
METHODS: MEDLINE, Embase, PsycINFO, and Cumulative Index to Nursing and Allied Health Literature were searched from inception to April
2013. We used thematic synthesis to analyze the findings.
RESULTS: Forty-three articles involving 729 participants aged from 4 to 21
years across 10 countries were included. We identified 6 themes: gaining
resilience (accelerated maturity and taking responsibility, acceptance of
prognosis, regaining control, redefining normality, social support),
lifestyle restriction (limited independence, social isolation, falling behind,
physical incapacity), resentment of chronic treatment (disempowerment
in health management, unrelenting and exhausting therapy, inescapable
illness), temporal limitations (taking risks, setting achievable goals,
valuing time), emotional vulnerability (being a burden, heightened
self-consciousness, financial strain, losing ground, overwhelmed by
transition), and transplant expectations and uncertainty (confirmation
of disease severity, consequential timeliness, hope and optimism).
CONCLUSIONS: Adolescents and children with CF report a sense of vulnerability, loss of independence and opportunities, isolation, and disempowerment. This reinforces the importance of the current model
of multidisciplinary patient-centered care that promotes shared
decision-making, control and self-efficacy in treatment management,
educational and vocational opportunities, and physical and social
functioning, which can lead to optimal treatment, health, and quality
of life outcomes. Pediatrics 2014;133:e1683–e1697
PEDIATRICS Volume 133, Number 6, June 2014
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Cystic fibrosis (CF) is a common lifeshortening inherited disease with an
estimated incidence of 1 in 2500 newborns.1–4 Most patients with CF develop
chronic pulmonary disease and bronchiectasis, as well as pancreatic insufficiency and subsequent malnutrition.5–7
Because of advances in screening,
treatment, and infection control, patients
diagnosed with CF within the past decade
are now expected to survive into their
50s.8,9 However, treatment of CF involves
daily adherence to intensive antibiotic
regimens, vitamin and enzyme supplements, consumption of a calorie-rich
diet, inhaler use, and physically demanding chest physiotherapy. Limited
daily functioning, poor adherence to
treatment, low self-esteem, short stature, and impaired psychosocial outcomes have been reported.10–15
What remains less well known is how
young patients cope with the symptoms,
prognostic uncertainty, and treatment
burden of CF. In-depth insights into
people’s beliefs and attitudes can be
gained by qualitative research, and
synthesis of multiple qualitative studies
can provide a broader scope of data
across different health care contexts
and generate new and more comprehensive understandings of social phenomena.16 We aimed to describe the
breadth of experiences and perspectives of children and adolescents
with CF, to inform ways to deliver
patient-centered care for optimal treatment, health, and quality of life outcomes, and to direct care toward areas
that patients regard as important.
MATERIALS AND METHODS
We followed the Enhancing Transparency of Reporting the Synthesis of
Qualitative research framework.16
Selection Criteria
Qualitative studies that explored the
experiences and perspectives of children and adolescents (#21 years of
e1684
age) diagnosed with CF were included.
We excluded observational studies,
randomized controlled trials, genetic and
microbiological studies, non–primary research articles (letters, commentaries,
and reviews), studies that did not elicit
data from children and adolescents with
CF, quantitative surveys, and non-English
articles due to lack of resources for
translation.
Data Sources and Searches
The search strategy is provided in
Supplemental Table 3. We conducted
searches in Medline, Embase, PsycINFO,
and Cumulative Index to Nursing and Allied Health Literature (CINAHL) from inception to April 2013. We also searched
Google Scholar and the reference lists
of relevant studies and reviews. We
screened the abstracts and excluded
those that that did not meet the inclusion
criteria, then assessed the full-text versions of potentially relevant studies.
Comprehensiveness of Reporting
We evaluated the transparency of reporting of each qualitative study using
the Consolidated Criteria for Reporting
Qualitative Health Research. 17 This
framework included criteria specific
to the research team, study methods,
context of the study, analysis, and
interpretations. Two reviewers (N.J. and
C.H.) assessed each study independently, and disagreements were resolved by discussion with A.T.
Data Analysis
We used thematic synthesis as described
by Thomas and Harden.18 For each study,
all participant quotations and text under
the “Results/Findings” or “Conclusion/
Discussion” sections were extracted and
entered verbatim into HyperRESEARCH
(version 3.5.2; ResearchWare, Inc,
Randolph, MA), software for storing, coding, and searching qualitative data. N.J.
conducted line-by-line coding of the text
into concepts inductively derived from the
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data and transferred concepts between
studies by adding coded text into existing
concepts or creating additional codes
for new concepts. Similar codes were
grouped into themes. The preliminary
themes were discussed in a research
team meeting to ensure that the themes
reflected the full range of experiences
reported across all studies. Patterns and
relationships within and across themes
were examined and mind mapped to
develop an analytical thematic schema.
RESULTS
Literature Search
Our search yielded 1862 articles. Of
these, 43 articles involving at least 729
children with CF were included (Fig 1).
The number of participants was not
reported in 7 studies (Table 1).
Comprehensiveness of Reporting
Studies reported between 7 and 20 of the
24 Consolidated Criteria for Reporting
Qualitative Health Research (Supplemental Table 4). Fifteen studies (35%)
described the participant selection
strategy. Twenty-eight studies (65%)
reported researcher triangulation, and
15 (35%) reported on theoretical saturation, defined as a lack of new concepts
found after subsequent data collection.
Member checking (seeking feedback on
the research findings from participants)
was reported in 2 (5%) studies.
Synthesis
We identified 6 main themes: gaining
resilience, lifestyle restriction, resentment of chronic treatment, temporal
limitations, emotional vulnerability, and
transplant expectations and uncertainty.
Selected participant quotations that
conveyed the meaning of the theme were
chosen from the included the studies and
integrated as examples in the results.
Additional illustrative quotations are
presented in Table 2.
Possible conceptual links between themes
are presented in Fig 2. Participants
REVIEW ARTICLE
FIGURE 1
Search process and results. *Minimum 729 participants; 7 articles did not specify the number of pediatric patients with CF in the sample.
with CF experienced emotional vulnerability and at the same time expressed
capacities to gain resilience in coping
with their illness. Heightened selfconsciousness due to manifestations
of CF contributed to participants’sense
of vulnerability. The chronic treatment
burden imposed restrictions on lifestyle and independence, which intensified participants’ resentment of
their illness. Regaining control contributed to the development of resilience but also reinforced risk-taking
attitudes and decisions, particularly in
adolescents who sought to demonstrate independence and ability to
make their own choices. The need to
value time and reprioritize was driven
by an acute awareness and realization
of their disease severity.
Gaining Resilience
Acceptance of Prognosis
Accelerated Maturity and Taking
Responsibility
Some participants described having to
accept the reality of their incurable
condition and shortened life expectancy
to live an “enjoyable life.”20 Although
they could not be optimistic about their
survival, some believed in the need to
maintain a positive attitude and to “be
happy and make the most of it.”21
Participants felt that they were more
mature and compassionate compared
with their well peers. Having faced the
challenges of living with CF, some believed they gained coping skills, resilience, and appreciation of life and were
less concerned about trivial matters.
One adolescent stated, “I feel that
having CF has pushed me into adulthood...in a good way.”19 They emphasized the importance of a positive
attitude in staying motivated to take
their medications. Signs and symptoms such as hemoptysis encouraged
some participants to be more health
conscious.
PEDIATRICS Volume 133, Number 6, June 2014
Regaining Control
Invasive medical procedures caused
some participants to feel a loss of control
of their own body. One participant explained, “If you have a [IV] line, the control is with the other people.”22 However,
strategies such as needle plans and play
therapy helped alleviate their anxieties
as participants felt that they had
reclaimed control. Also, participants
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e1686
JAMIESON et al
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NS
16
NS
14
7
Moola 201121
Moola 201223
Bregnballe 201129
1
20
15
10
6
6
Christian 199937
D’Auria 199725
D’Auria 200026
Dashiff 201386
Hafetz 201028
Miller 200966
NS
17
23
50
20
Christian 199730
Palmer 200219
Patton 198631
Pendleton 200287
Sawyer 199888
NS
1:1
6:11
NS
NS
24
17
NS
NS
NS
9:14
1:0
NS
NS
7:3
3:2
1:2
1:0
2:3
NS
NS
64
3
NS
33
van Staa 201184
North America
Admi 199624
Barker 201227
Berge 200720
Boyle 200185
France
Henry 200382
The Netherlands
Sinnemma 198883
NS
8
NS
2:5
6:1
2:5
19:21
2:1
NS
17:18
7
40
3
5
35
20–26
11–21
5–12
14–53
8–19
8–19
15–19
6–12
17–22
21
12–18
16–25
11–18
16–21
NS
15–22
12–20
NS
11–17
11–17
14–18
8–16
7–18
11–20
16–20
19–20
4–16
11–18
Patients Sex Age Range
(n)
(M:F)
(y)
Russell 199679
Willis 200136
Brazil
Pizzignacco 200680
Canada
Ellerton 199681
Australia
Brumfield 200477
Fereday 200956
Graetz 200078
Study ID
TABLE 1 Characteristics of Included Studies
NS
NS
Ethnography
NS
NS
Qualitative design
Qualitative design
Grounded theory, single
case study approach
Grounded theory
Grounded theory
Grounded theory
Grounded theory
NS
Grounded hermeneutic
NS
Qualitative design
NS
NS
Narrative approach
NS
Qualitative design
NS
Qualitative design
Roy adaptation model
NS
Qualitative design
Interpretive phenomenology
NS
Conceptual Methodological
Framework
Questionnaires and semistructured face-to-face
interviews
Focus groups and individual face-to-face
interviews
Focus groups and individual face-to-face
interviews
Semistructured face-to-face interviews
Semistructured face-to-face interviews
Semistructured face-to-face interviews
Semistructured face-to-face interviews
Semistructured face-to-face interviews
Semistructured face-to-face interviews
Semistructured retrospective face-to-face
interviews
Serial face-to-face interviews
Serial unstructured face-to-face interviews
Semistructured face-to-face interviews
Focus groups
Face-to-face interviews and questionnaires
Structured interviews with some open ended
questions
Semistructured interviews
Unstructured face-to-face interviews
Semistructured face-to-face interviews and
questionnaires
Semistructured face-to-face interviews
Semistructured face-to-face interviews
Focus groups and structured individual face-toface interviews
Semistructured face-to-face interviews
Unstructured face-to-face interviews
Focus groups
Structured face-to-face interviews and
questionnaires
Semistructured face-to-face interviews
Semistructured face-to-face interviews
Data Collection (Qualitative)
Content analysis
NS
Grounded theory analysis
Constant comparative method
Inductive approach
Thematic analysis
Correlation analysis
Constant comparative method
Constant comparative method
Content analysis
Constant comparative method
Constant comparative method
Template analysis
Grounded hermeneutic analysis
NS
Thematic analysis
NS
Thematic analysis
Thematic analysis
Thematic analysis
Iterative, interpretive, and
inductive analysis
Content analysis
Thematic analysis
Adaptive categorization
Thematic analysis
Thematic analysis
Thematic analysis
NS
Analysis
Parent–child collaborative decision
making
Transition to adulthood
Stress and coping responses
Spirituality as a coping aid
Reproductive health
Disclosure of health-related information
Growing up with CF
Influence of peer relationships on
adjustment
CF-related diabetes
Awaiting lung transplantation
Growing up with CF
Support from family and friends
Gender differences in self-perception
Transition to adult health care
services
Growing up with CF
Transition to adult health care services
Independence
Quality of life
Transition to adult health care services
Physical therapy
Parental support
Social support
Daily routine
Transition to adult health services
Gender differences
Transition to adult health care services
Physical activity
Support from family and friends
Research Topic and Scope
3:7
NS
13:19
NS
NS
5:9
NS
13:14
3:5
1:0
NS
0:5
49:52
7:9
7:9
10
NS
32
NS
NS
14
11
27
8
3
10
5
101
32
32
7–17
17–21
10–17
6–14
8–18
19–22
12–16
9–21
NS
7–17
10–16
17–47
13–17
11–19
16–43
Patients Sex Age Range
(n)
(M:F)
(y)
NS
NS
NS
Ethnography
Mixed methods (phenomenology,
descriptive exploration)
Framework model
NS
NS
Phenomenology
NS
NS
Grounded theory
Qualitative design
NS
NS
Conceptual Methodological
Framework
Constant comparative method
Thematic analysis
Thematic analysis
Thematic analysis
NS
Constant comparative method
Content analysis
Thematic analysis
Thematic analysis
Content analysis
Constant comparative method
Structured face-to-face interviews
Semistructured face-to-face interviews
Semistructured face-to-face interviews
Serial semistructured face-to-face interviews
and focus groups
Semistructured face-to-face interviews
Semistructured face-to-face interviews
Semistructured face-to-face interviews
Face-to-face interviews
Semistructured telephone interviews
Open-ended questionnaire
Semistructured face-to-face interviews and
clinical observation
Semistructured face-to-face interviews
NS
Thematic analysis
Thematic analysis
Thematic analysis
Analysis
Serial semistructured telephone interviews
Focus groups
Structured, open-ended interviews and
questionnaires
Data Collection (Qualitative)
Physiotherapy
Evaluation of a community befriending
program
Infection risk
Segregation to prevent cross-infection
Diet management
Psychosocial impact
Adjustment difficulties to treatment
regimes
Awaiting lung transplantation
Personal definitions of pulmonary
exacerbation
Transition to adult health care services
Needle-related distress
Communication with doctors
Physical therapy
Health care
Transition to adult health care services
Research Topic and Scope
NS = not stated or not applicable.
Grounded theory means theories that are grounded in the empirical data and built up inductively through a process of careful analysis and comparisons. Phenomenology entails studying people’s understandings and interpretations of their experiences
in their own terms and emphasizing them as explanations for their actions. Mixed methods include both quantitative and qualitative methods. Narrative methodology is based on the belief that what people think and mean is transparent and readily
accessible. Ethnography is the attempt to discover and describe individual social and cultural groups. Roy adaptation model considers the process of individual adjustment in response to groups. Hermeneutics is the examination of the way people develop
interpretations of their lives in relation to life experiences.
Williams 200797
Reynolds 201395
Russo 200632
Savage 200596
Macdonald 201094
MacDonald 200638
Bywater 198133
Foster 200193
Al-Yateem 201234
Ayers 201122
Beresford 200392
United Kingdom
Abbott 200991
Swisher 200889
Tivorsak 200490
Zack 200335
Study ID
TABLE 1 Continued
REVIEW ARTICLE
PEDIATRICS Volume 133, Number 6, June 2014
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believed that the ability to manage their
own health and to participate in physical
and recreational activities meant that CF
had not defeated or limited them. They
derived a sense of normality, “mastery,”
and “competence” from taking control
over their health, with 1 girl describing
physical activity as her “sanctuary.”23
Redefining Normality
Over time, some believed that they became less concerned about the differences in their physical appearance and
capabilities compared with their healthy
peers. One participant described that he
had “nothing to hide and nothing to
advertise.”24 By meeting other young
people with CF, some learned to respect
and accept their own physical capacities rather than compare themselves
with their well peers. Younger children
reported a loss of having a normal
identity at the time of diagnosis: “I just
thought she must be insane...because I
always thought I was just like everybody
else.”25
Social Support
Emotional and practical support from
carers, friends, and family was described
as a resource that enabled participants
to cope with their day-to-day disease
management. Having a shared experience and interacting with other patients
with CF promoted social connectedness
and sharing of coping strategies and
information. Children and adolescents,
particularly girls, reported that they
depended on their parents to encourage
adherence to medications and therapy.
One participant believed that the loss of
accountability to their parents after
moving out of home contributed to the
onset of depression.
Lifestyle Restriction
Falling Behind
The participants felt socially “out of the
loop”26 and out of place because of
frequent absences from school and
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TABLE 2 Illustrative Quotations
Theme
Gaining resilience
Accelerated maturity
and
taking responsibility
Source Text
“That [hemoptysis] was more of a reminder, like look
you have this thing and you’ve got to keep at it
because if you don’t this stuff is going to happen....
It just sort of reinforced, just keep going. Don’t
start slipping now.”24
“But it’s up to me to take care of myself.... I’m the one
living with it every day.”19
“I feel that having CF has pushed me into adulthood,
but not in a bad way, more in a good way.”19
Acceptance of prognosis “It (CF) is a part of who I am....I was thinking about life
in general and how I knew that my disease was
going to kill me off younger. I probably will not be
able to see my grand children grow up, kind of
thing....It really got to me that whole year and then I
eventually, I just accepted the fact that everyone
dies. It does not matter when you go, but you go. I
just kind of got a positive attitude, that I might as
well be happy and make the most of it.”21
Regaining control
One child used the example of being terrified of
medical needles but not frightened when she had
her lip pierced. “I think it’s because I have the
control. It’s my body and something I wanted to do.
If you have a [IV] line, the control is with the other
people.”22
“Sometimes it’s easier to not do your treatments and
say to yourself I’m going to die anyways....What’s
the point, you’re prolonging the inevitable...but, I
don’t think that way....You gotta take your health in
your own hands.”20
“The most important thing to me is my health and
getting better. That sort of thing, that’s probably
the most important thing, just being able to
control it.”36
Redefining normality
“Someone was reading the paper, where it was like,
30 000 other Americans [with CF], and I was
thinking, ‘Oh, I wonder where all those people are?’...
All these years I thought I was just the one person
[with CF].”30
“Us cystic fibrosis people have the same feelings as
everybody else, but other people don’t think that....
We’re really not differenter than anybody else
because we just have a little problem just like
everybody else does.”25
Social support
“I think my depression mostly started when I started
college....I always lived at home before and my
mom helped me do my treatments and when I
moved to college...it was all on me....That was my
biggest down time.”20
“Socialising with them made a lot of difference for
me because they’re going through the same thing
as I am every day...going out into the big wide
world and going to school.”68
Lifestyle restriction
Falling behind
“CF makes it harder in terms of I’m not at school as
much as some of the others, so you’re kind of out
of the loop when you come back after 3 wk. Who’s
seeing who, you know. That can change radically in
3 wk.”26
“The first year I missed a lot and really got behind.
School was really hard; I got no help until eighth
e1688
Contributing Studies
Ref. Nos.
19–21, 24, 26, 36,
37, 80, 84
extracurricular activities, mainly due to
pulmonary exacerbations and lung
deterioration. Some felt they lacked
capacity and support to perform academically. For example, 1 adolescent
described having to leave school being
unable to achieve second grade literacy.
Limited Independence
20, 21
19, 20, 22, 25, 35, 36,
79, 89, 97
24, 25, 30, 80
19, 20, 22, 23, 25, 28, 30,
32, 35, 38, 77, 78, 80,
92, 94
Most participants felt their lives were
marked by a loss of freedom and opportunities because of their poor health
and time-consuming treatments. Some
described their parents as interfering,
domineering, and overprotective. They
felt exasperated and patronized by
constant reminders from parents about
taking medications; however, some felt
that these reminders were essential “or
else [they] won’t do it.”27 Others felt
that they should be given more responsibility as they got older,28 and 1
participant suggested they should be
given a trial period to prove they could
independently manage their illness.29
To avoid having additional restrictions
imposed, some refrained from asking
for advice from their doctors about
high-risk activities such as drinking
alcohol, which led to high-risk behaviors including defiance29 and hiding
problems with adherence: “I’ll not
do [physical therapy] just to spite
them.”27 Being highly dependent on
care, some older participants stated
that their career and education options
were limited to those that were located
in close proximity to their home or CF
clinic.
Physical Incapacity
26, 31, 37
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Short stature, weakness, fatigue, and
susceptibility to infections contributed
to participants’ frustration, social isolation, and feelings of being different.
They had “to try twice as hard...to do as
good as the other kids”30 and found
that physical exertion took a toll on
their health and energy. Children felt
upset about being too tired to play.
Adolescents, particularly boys, were
REVIEW ARTICLE
TABLE 2 Continued
Theme
Limited independence
Physical incapacity
Social isolation
Resentment of chronic
treatment
Inescapable illness
Source Text
Contributing Studies
Ref. Nos.
grade so I dropped out. The next year they told me I
could graduate. Well, I got mad and my mom
agreed. I couldn’t read at a second grade level, do
math, or write. So I went to a special school and
learned more there than ever before. School has
always been a pain in the neck. I never did like it.”31
Alternatively, some participants revealed that
19, 21, 27–29, 32, 36,
a reason for not asking their doctor questions (eg,
37, 80, 92, 97
drinking alcohol, taking part in risky activities such
as extreme fairground rides) was because they
might not want to hear about restrictions to their
lifestyle.78
“I don’t think I could have gone to a school far away
because I had to be close to the CF Center and my
parents in case I got sick.”19
Older children with more severe symptoms who
were independently performing their own
physiotherapy techniques also felt it was too much
hassle to stay away from home because of the
amount of medication and equipment they needed
and because they felt emotionally uncomfortable
(embarrassed) about doing their physiotherapy
away from home.83
“It just started to get harder like to do sports and 19, 23, 26, 30, 32, 34,
baseball and cheerleading. I’d start getting more
36, 38, 89, 91, 96
short of breath and tired and coughing.”26
“We play a game called ‘Capture’ in our
neighborhood....I can’t play that too much in a row,
or I really don’t feel good the next day.”
“I don’t do the activities I used to do because I get too
out of breath....I would love to be able to run again,
I get too out of breath and I used to love to run....
Now I get in a coughing spell and I can’t breathe.”76
When friends asked one participant about his “big 19, 26, 27, 30–32, 95
fingers” (clubbing), he told them, “It was because
my Dad had really big fingers. I remember getting
a really big sinking feeling in my stomach like, ‘My
gosh somebody noticed something different/
weird about me.’ I felt like I was different. I
resented....I didn’t like my fingers, you know. I
didn’t want them to be that way. I wanted them to
be like everybody else’s fingers.”26
“I have to be real careful when I tell my boyfriend I
have CF. In all times past, once they’ve found out,
they’ve split.”31
The treatments that became part of their routine
20, 21, 23–25, 30, 33,
served as a constant reminder that they had CF, and
80, 93, 97
only in this sense did they feel different from others.24
“When I was a kid, there was a round board and I
would lie on it while my mom whacked my chest.
Like a beating with a cupped hand—I hated it. Ever
since I was little and she did that, and I knew that it
was PT, I have despised it. It takes away from my
time, and that is something that I do not like. When
you have to sit down and do your mask for 30
minutes, and the PEP mask for 20, it drives the nail
home that you do have a lung disease and
sometimes I just want to forget about it...as I am
doing it, ‘wow, I have to do this, to prevent myself
PEDIATRICS Volume 133, Number 6, June 2014
concerned about being unable to achieve their ideal masculine physique.
Social Isolation
Participants felt ostracized when others avoided them because of their
coughing or differences in their physical appearance, such as finger clubbing. Frequent absences from school
also made it harder to establish
friendships, because participants
“never got to know people as well.”26
Children described being bullied or
abandoned by others who thought CF
was contagious: “All the kids saying
‘eww’...then they would say ‘Oh shut up
AIDS girl.’”30 Some felt that others accused them of using CF as an excuse to
avoid school. CF was also believed to
“jeopardize” romantic relationships,
with 1 participant reporting that her
past boyfriend “split”31 when he found
out she had CF. Having to avoid “highrisk” environments and being isolated
from other patients in wards, participants felt socially excluded and
expressed “boredom.”32
Resentment of Chronic Treatment
Inescapable Illness
Participants wanted “escape” or a
“break” from their illness. However, the
relentless need to take medications and
undergo physical therapy served as
constant reminders of their incurable
illness. One participant stated that “it
drives the nail home that you do have
lung disease,”23 and another was “unbearably depressed”33 about how the
treatment reminded her of her illness.
Some saw treatment as another indication of being different or weaker
than their well peers.
Disempowerment in Health
Management
Some participants felt that doctors used
only “high level talk”31 and complex
medical terminology. When they perceived
their doctors were communicating solely
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TABLE 2 Continued
Theme
Disempowerment in
health management
Unrelenting and
exhausting therapy
Source Text
Contributing Studies
Ref. Nos.
from dying.’ It takes away from my time for sports,
friends, and family.”23
“Yeah, they always talk superficial—always on this 20, 23, 27, 29, 31, 34,
high level talk in these medical terms. I wish they’d
35, 38, 80, 92
say it in English. I always want to say ‘OK, OK, I’ll do
it’—but why do I always feel so cruddy? I’m really
concerned about my appearance, I’m so skinny,
but they won’t tell me anything.”31
“I hate when it (physical therapy—PT) gets in the way 19–22, 24, 26–29, 36,
of all of these things. At camp, it really gets in the
80, 96, 97
way when you wake up and the bell goes and you
have to go to breakfast. But I have to do PEP
(breathing mask), and that holds me back. Or, it is
night time and they are all going to go out
somewhere. And maybe I have to go back to the
cabin and get my PEP which is really far away. It is
just annoying...that was the worst and I hated it so
much....I know that I need to do physical activity,
but it is just sometimes hard when things
interfere, like medicine or PEP.”23
“I cough a lot and take a lot of drugs, the others
[children] only take them when they are ill, I take
them all the time.”72
Temporal limitations
Valuing time
“I wish I had done more things when I was younger. I 19–21, 23, 24, 36,
was healthier. And I wish there were nobody had
37, 80, 97
told me not to do this or that. It was like I would be
able to do it then. I’ll never let it [CF] stop me from
doing anything. That’s how I deal with it now.”24
“Usually, I try to take that attitude, because what is
the point. I am not going to keep drilling on the fact
that I have CF because it is not really going to help
with anything and it is not going to make it go
away. I just rather you know, just love life, you
know? Make the most of life now.”21
“You read about the life expectancy of what, 30 or 40?
That’s kind of strange, but you can’t be afraid to
live.”19
“Maybe you realize that...how special life is...how
short it is and how important it is to be happy.”36
Setting achievable goals “I was looking at more part-time work due to my CF 19, 21, 23, 24, 36
and having CF—that I’d need more time to do
physio and to keep my health, keep it 100%
because I would have to put in so much at work
and not wanting them to know that I had CF. So I
was reluctant to get full-time work and when I did
there were doubts in my mind.”36
Taking risks
Risk-taking behaviors: Male respondents reported 20, 24, 28, 37, 95, 97
risk-taking behaviors such as smoking tobacco and
drinking alcohol. They acknowledged that tobacco
was “bad for their CF” but believed that alcohol
was less harmful.20
“You blow it [treatments] off a lot....Short-term
nothing happens if you skip something...but then it
catches up with you after a while...and then you get
revitalized to do them again.”20
“I wasn’t supposed to [smoke]...because I have CF....
That’s what I thought for most of my life...then
somebody offered me a cigarette....I was like, all
right, I’ll try it, one’s not going to hurt...but then,
one, two, three, and I’m hooked.”20
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to their parents, participants felt ignored
and devalued. They wanted clinicians to
listen to their needs rather than “just
clinically going through [the illness and
treatment],”34 and they believed doctors
should communicate directly with them.
Some participants appreciated nurses
who “treat you like you are part of their
family.”35
Unrelenting and Exhausting Therapy
Treatment of CF, particularly medications,
physical therapy, and lung clearance, was
described as intensive, invasive, and
physically strenuous. Children were
frightened of the pain of injections, and
they experienced fatigue due to prescribed physical exercise. Despite the
physical demands of treatment, some
adolescents believed that persevering
with therapy was necessary to prevent
health deterioration and lung infections.
One participant said, “I’m just fighting to
get rid of something for a certain amount
of time.... It’s a never-ending battle.”26
Temporal Limitations
Valuing Time
Being acutely aware of their “shadowy
future”21 and shortened life expectancy,
some participants chose to “make the
most out of life now”21 rather than waste
time worrying about the future. Some
were concerned that they had wasted
their time so far (“I just sometimes feel I
haven’t done much with my life”36) and
despaired at how their physical therapy
“robbed”23 them of their precious time.
Participants chose to spend as much
time as possible with family and friends.
Setting Achievable Goals
Some participants accounted for the
health and lifestyle limitations caused by
CF when they set career and relationships goals, and they needed to consider
air quality and associated workplace
risks, employment benefits and health
insurance, and the availability of flexible
hours or part-time work. They also
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TABLE 2 Continued
Theme
Source Text
Contributing Studies
Ref. Nos.
These risk-taking behaviors demonstrated the
conflict between the adolescent’s cognitive ability
to make rational decisions and his developmental
needs for independence and control.30
Where infection risk was not apparently considered,
social factors were often cited as a reason for the
decision. “At the end of the day you cannot sort of
shut yourself away....I think it’s really important to
think about infection, but I think some people
could take it too far.”95
Transplant expectations
and uncertainty
Confirmed severity
of disease
Consequential
timeliness
Hope and optimism
Emotional vulnerability
Being a burden
Financial strain
“Being listed [for transplant] signified a point in the 37, 38
chronic illness trajectory at which there was no
longer any advantage in delaying the transplant
procedure.”37
“The introduction of discussions regarding referral
for transplant displaces this status quo of ‘normal
life’ . . . while considering that transplant may be
something that will be required in the future, are
not usually prepared for the conversation when it
is raised, despite being given cues from health
professionals indicating a deteriorating picture
over time.”38
“I was shocked that he had even thought, how dare
they say I was as bad, that I have only got so long to
live or something like that.”38
“I was angry, I thought it was too soon.”38
“Everyone on the team admits don’t do it until the
37, 38
precise time. It is a very strange balance, you
know. You have to find the exact point at which to
get you on the list, because if you go too early, you
are sacrificing maybe a year of good health.”38
“If I was a video recorder, I’d be on pause at the
moment. I’m not really living, I’m just getting by.”38
“Increased health, that is the obvious benefit. It is
37, 38, 79, 80
hard work after the transplant. The surgery is no
fun, it is painful. But the rewards—the possible
benefits, you know, I think, far outweigh that.”37
“It was an exciting feeling, it was like, God, I’m going
on the transplant list, I’m going to be normal like
everyone else.”38
“I always got sick when we went on vacations and it... 20, 26, 94
ruined it for the rest of the family....My brother
would get really mad at me because we had to cut
it short or because it wasn’t as fun as it would
have been.”20
“My CF has always been an issue between them
[parents] because the last five years I’ve been in
and out of the hospital and...they’ve had fights over
the phone a lot about who should be taking care of
me and how stressful it is....It’s been awful.”20
“The insurance is the biggest concern. It’s really the 19
only thing with my CF that I worry about. I have
learned how to deal with the insurance people....
It’s like you have to really fight for yourself....It’s
probably the hardest thing I’ve had to deal with.”19
“Paying for the insurance and copays is hard....What
is also hard is affording the groceries....With CF
and diabetes you need to eat a lot of healthy food
PEDIATRICS Volume 133, Number 6, June 2014
developed alternative strategies if they
could not pursue their initial vocational
goals. In particular, boys expressed optimism and refused to allow their condition to define their career goals.
Conversely, some adolescent girls felt
insecure about their future career
pathway and doubted their opportunities for full-time employment.
Taking Risks
Some adolescents wanted to take risks
because they felt they had nothing to lose
in their limited time. They described
taking drugs, drinking alcohol, smoking,
and “blow[ing] off treatment,”20 choices
that allowed them to show CF had not
restricted their lifestyle. Some acknowledged that certain environments would
expose them to infection risk but believed that complete avoidance “could
take it too far” and lead to “missing
out.”32 For some, the consequences of
poor health after smoking or refusing
treatment and therapy “caught up” with
them, and in turn they became motivated
to continue with their treatments.
Emotional Vulnerability
Being a Burden
Some participants deemed themselves
highly dependent on family members for
day-to-day medical treatment. For this
reason, they felt guilty for taking parental
attention away from their siblings and for
depriving their families of vacation time
because of their ill health. One participant
believed that her parents’ arguments
about her CF care caused their marital
problems.
Financial Strain
In a study conducted in the United
States, adolescents were anxious about
the financial burden of their disease,
including insurance, medications, and
costs of nutrient-dense foods. A major
concern was negotiating with insurance companies to cover the costs of
equipment and medications.
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Overwhelmed by Transition
TABLE 2 Continued
Theme
Heightened selfconsciousness
Losing ground
Overwhelmed by
transition
Source Text
and the healthy foods seem to be the most
expensive.”19
Younger children (6–9 y) did not understand that they 19, 20, 23–31, 36,
were born with CF and thought peers avoided
66, 80, 93, 97
them because they were afraid of “catching” CF.25
Several described developing a private cough and
a social cough to minimize their differences and
avoid negative social consequences.30
“There was one person in my school who, because
we all had a packed lunch, he’d always say ‘Oh,
druggy!’...He was older than me, and he’d say it,
‘Druggy. Druggy.’ He’d shout it out really loud.”79
“When I cough, it sounds like I’m choking. The kids
are scared to be around me. I don’t have a lot of
friends.”31
“Two years ago I was capable of downhill skiing,
20, 21, 26, 36, 37, 92
walking to class without oxygen—running
around. Now, I am a lot sicker. I am in the hospital
a lot more, which is taking away from both my
social life and my academic performance.”37
“I also know that I am not going to live as long as
everybody else, so that is hard. I feel like it is out of
my control, I feel helpless, how I used to be able to
do it (physical activity), and now, I can’t. It is kind of
depressing. It makes me think that it is
a progressive disease, and it makes me think that
it is getting worse....It makes me worried.”21
For example, one participant noted the lack of detail: 29, 34, 35, 77, 79,
“Ah, very little, almost nothing. I mean all they do is
80, 84, 85
to tell you that you are transferring.”34
But most young adults and especially parents said it
had been more stressful and difficult than
anticipated. Those who had had frequent contact
with pediatric health care providers found it hard
to establish trust and familiarity with the new
staff, as reflected by metaphors like “being lost,”
“falling into a deep hole,” “feeling abandoned,”
and even “waking up in a horror movie.” However,
this was seen as temporary; transition was
perceived as a rite of passage: “You have to get
used to it, that’s all.”
Heightened Self-Consciousness
Many participants expressed embarrassment about their small physical stature,
coughing, and taking medications in
public. Feelings of being different were
exacerbated by symptoms that prohibited them from engaging in social
andphysicalactivities. Someparticipants
developed a “private cough and a social
cough”30 or avoided taking medications
in an effort to appear normal. Being
defined as “patients or handicapped”24
and being treated differently by their
peers, teachers, and coaches caused
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Contributing Studies
Ref. Nos.
frustration, and participants felt demeaned. In an effort to appear normal,
many participants chose not to tell their
friends and teachers about their CF.
Losing Ground
Some participants felt powerless as the
illness progressed: “I feel helpless, how
I used to be able to do [activities], and
now, I can’t.”21 Participants were anxious about potentially developing other
illnesses that they perceived were associated with CF, including diabetes
and depression.
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Many participants felt overwhelmed
and unprepared for the transition from
pediatric to adult care. Some felt that
there had been “no real discussion”34
about transition until it had occurred.
They had difficulty coping with the unfamiliar setting and lack of rapport
with new health care workers. One
participant was concerned about perceived greater exposure to infection in
the adult setting. Conversely, some
found the transition process beneficial
and enjoyed experiencing what they
felt was age-appropriate care.
Transplant Expectations and
Uncertainty
Confirmation of Disease Severity
Being listed for lung transplantation
was alarming for some participants
because it signaled that their health had
deteriorated to a critical stage. Some
believed they were listed too soon because they did not perceive themselves
to be seriously ill, and they felt unprepared and shocked.
Consequential Timeliness
The importance of timing of being
waitlisted or receiving a lung transplant
at the right time was described by some
as being a “very strange balance” having
to “find the exact point at which to get
you on the list, because if you go too
early, you are sacrificing maybe a year
of good health.”37 The time from being
placed on the transplant list to being
transplanted was marked by uncertainty, anxiety, disappointing false alarms, and the feeling that life was like
a “video recorder...on pause. I’m not
really living, I’m just getting by.”38 Some
felt that more regular contact with the
transplant center would reassure them
that they had not been forgotten.
Hope and Optimism
Some participants held hope that
a transplant would provide them with
REVIEW ARTICLE
FIGURE 2
Thematic schema.
better health, greater life expectancy,
and the chance to be “normal like everyone else.” Transplant was perceived
to offer a chance of good health, without “[lungs with] scars and years of
infection and damage.”37 Others were
optimistic about the potential for future medical advances to find a cure
and improve their health.
DISCUSSION
Children and adolescents with CF have
a shortened life expectancy, significant
comorbidities, and treatment burdens
that impair their identity, daily functioning, and life goals. Our study
highlights the personally significant experiences of children with CF, including
their emotional vulnerabilities and their
strategies and capacities to develop
resilience. Challenges of living with CF
include accepting their prognosis, adhering to a demanding treatment regimen, losing independence, transitioning
to adult care, limiting social participation, and missing education and career
opportunities, which reinforced their
sense of abnormality, resentment, and
disempowerment. Social support from
family, friends, and other patients with
CF is valued, and internal coping strategies encompass redefining normality,
taking responsibility, setting achievable
or realistic goals, and holding hope
for lung transplantation and medical
advances.
The feeling of being different from others
appeared to be more acute in younger
children. Some adolescents believed
that CF caused them to mature beyond
theirchronological age, whichmotivated
them to accept their illness and adhere
to their prescribed treatment,but others
chose to engage in high-risk behaviors to
demonstrate that they were not limited
by CF. Those with milder diseases were
still able to retain a sense of control
through participation in physical, school,
and social activities. However, patients
with more severe CF contended with
feelings of powerlessness.
The themes identified in our review may
help explain why some children and
adolescents with CF report lower quality
of life than their well peers.40,41 Of note,
girls with CF have reported more emotional symptoms and greater pain, spend
more time in the hospital than boys with
a similar level of illness,41–43 and have
a worse prognosis than boys.44–48 Our
findings suggest that boys may emphasize taking responsibility for their
treatments and maintaining a positive
attitude, whereas some girls expressed
the value of sharing responsibility for
their health management with, for example, their parent or doctor. Some boys
reported body image concerns and were
PEDIATRICS Volume 133, Number 6, June 2014
motivated to exercise to achieve a more
muscular physique. Studies have also
found that a decrease in lung function
predicts a poor health-related quality of
life over time.49 This is supported by our
findings that many of the psychosocial
issues in CF relate to issues of fatigue
and physical incapacity.
Children and adolescents living with
other chronic and life-limiting illnesses
including cancer, juvenile idiopathic arthritis, and chronic kidney disease also
experience a loss of normality and of
control over their bodies because of
regular invasive procedures, social
isolation, lifestyle restraints, and heightened self-consciousness about the differences in their physical appearance
compared with their well peers.50–55
Being able to participate in physical activity is viewed as important in both the
CF and asthma populations.56
What may be unique in children and
adolescents with CF is how their susceptibility to infections (including
cross-infection necessitating segregation32,57,58) limits their lifestyle, reduces
their capacity to participate in physical
activity, and perpetuates anxiety, isolation, and uncertainty. Also, some felt
embarrassed and worried that they
would be abandoned by peers who
thought their illness was spread by
coughing.
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In our review, we undertook a comprehensive search and a rigorous independent assessment of the transparency of
study reporting. Researcher triangulation
was achieved by involving several researchers in the process of analyzing the
data, which ensured that the coding
framework captured the breadth of data
reported in the primary studies. Software
was used to code the data, allowing an
auditable development of the findings.
However, our study has some limitations.
Wewereunabletodescribeagedifferences
because the majority of the data (quotations) from the included studies were not
tagged to a specific age or age group.
Most studies were conducted in developed
countries, and non–English language
articles were excluded, making the
transferability of our findings uncertain.
treatment and feel in control of their
treatment.65,66
There is now a need to better meet the
longer-term needs of patients with CF
who will live longer, especially their psychosocial needs, as described in our review. The Home, Education, Activities, Drug
and Alcohol Use, Sexuality and Suicide
Youth is a validated tool that can be used
to assess psychosocial status and healthrisk behaviors in adolescents.67 Such
tools may be used to identify patients
who will benefit from psychosocial services,68,69 which could be adapted for the
CF population and thereby support clinicians in promoting psychosocial coping
among children with CF.
facilitated online support networks for
pediatric patients with CF may be particularly useful, but they must be closely
monitored to ensure exchange of accurate information. There is some reported
success in the literature in the field of
peer-to-peer health care, where patients
and their families use the Internet to find
and share practical health care tips.
We suggest that more qualitative research is needed to assess the attitudes
and experiences of children and adolescents regarding lung transplantation.
Also, there is a paucity of qualitative data
on perceptions and concerns about
sexuality and infertility that could be
explored further. Comprehensive and
validated CF-specific quality of life
instruments, such as the Cystic Fibrosis
Questionnaire–Revised, are available but
may be adapted to capture domains relating to decision-making in health care,
empowerment, financial and employment concerns, and future outlook.
CF care sets a benchmark in terms of
providing comprehensive multidisciplinary care in pediatrics.59–61 Nonadherence
to treatment remains a significant problem, particularly in the pediatric population.62–64 Our study suggests that this
may result from self-consciousness and
striving to appear normal, engaging in
risk-taking behaviors to gain a sense of
control and independence, needing to
“escape” the illness and exhaustion from
the demands of treatment, and a sense of
disempowerment. Motivating factors for
treatment adherence included determination to maintain health in their hope
for transplantation and the desire to take
responsibility and demonstrate maturity.
Transitioning from pediatric to adult
healthcareservicesremainachallenge,70–73
with some adolescents reporting difficulties in coping with pressures of their
new health care responsibilities.71 Our
findings suggest the need to individualize the transition approach, focusing
on the individual’s readiness and expectations for transition, which can be facilitated by creating a care plan with the
patient and their family.74 A collaborative
process through introduction of the
adult-style clinic in the pediatric setting
has been implemented in CF clinics in
the United Kingdom and Australia. Successful collaboration can be promoted
through the appointment of a transition
coordinator within the CF team to liaise
between the pediatric and adult teams
and provide education to families.
Topromoteindependence, empowerment,
and confidence in self-management,
which may lead to improved adherence,
we suggest shared decision-making processes where patients are actively involved in decisions about their own
Also, our findings highlight the benefits of
peer support from other patients with CF,
butface-to-faceinteractionisnotpossible
if patients are physically isolated to
prevent cross-infection in clinical settings.75,76 Therefore, social media and
Children and adolescents living with CF
experience vulnerability, resentment, and
disempowerment. Health care decisionmaking can be challenging given the
high treatment burden and uncertainties
about their prognosis, and it is arguably
more difficult in the context of lung
transplantation because the marked the
severity of their disease and timing of
waitlisting have consequences for expected survival and life goals. We suggest that
multidisciplinary patient-centered care
should encompass strategies that promote shared decision-making, control and
self-efficacy in treatment management,
educational and vocational opportunities,
and physical and social functioning, which
may lead to optimal treatment, health, and
quality of life outcomes.
of Genetic Services Committee, ACMG.
American College of Medical Genetics.
Laboratory standards and guidelines
for population-based cystic fibrosis
carrier screening. Genet Med. 2001;3(2):
149–154
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Children's Experiences of Cystic Fibrosis: A Systematic Review of Qualitative
Studies
Nathan Jamieson, Dominic Fitzgerald, Davinder Singh-Grewal, Camilla S. Hanson,
Jonathan C. Craig and Allison Tong
Pediatrics; originally published online May 19, 2014;
DOI: 10.1542/peds.2014-0009
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PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly
publication, it has been published continuously since 1948. PEDIATRICS is owned, published,
and trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk
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Children's Experiences of Cystic Fibrosis: A Systematic Review of Qualitative
Studies
Nathan Jamieson, Dominic Fitzgerald, Davinder Singh-Grewal, Camilla S. Hanson,
Jonathan C. Craig and Allison Tong
Pediatrics; originally published online May 19, 2014;
DOI: 10.1542/peds.2014-0009
The online version of this article, along with updated information and services, is
located on the World Wide Web at:
/content/early/2014/05/14/peds.2014-0009
PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly
publication, it has been published continuously since 1948. PEDIATRICS is owned,
published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point
Boulevard, Elk Grove Village, Illinois, 60007. Copyright © 2014 by the American Academy
of Pediatrics. All rights reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.
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