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 2014;133;e1683; 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: http://pediatrics.aappublications.org/content/133/6/e1683.full.html

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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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

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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

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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

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

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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.

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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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

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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

Source Text

Gaining resilience Accelerated maturity and taking responsibility

“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

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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

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

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TABLE 2 Continued Theme

Source Text

Limited independence

Physical incapacity

Social isolation

Resentment of chronic treatment Inescapable illness

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

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

Source Text

Disempowerment in health management

Unrelenting and exhausting therapy

Temporal limitations Valuing time

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

“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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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

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

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 selfconsciousness

Losing ground

Overwhelmed by transition

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 e1692

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.

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

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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

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

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PEDIATRICS Volume 133, Number 6, June 2014

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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 2014;133;e1683; originally published online May 19, 2014; DOI: 10.1542/peds.2014-0009 Updated Information & Services

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