Facilitators and barriers to empowerment in children and young people with cystic fibrosis: a meta-synthesis of the qualitative literature

Figures & data

Figure 1. PRISMA diagram.

Table 1. Summary of studies.

Author, year, country	Aims	Participants	Data collection	Qualitative analysis	Empowerment indicators described [18]
Ayers et al. [31] (2011) UK	To understand the nature and treatment of needle-related distress in children with CF	14 child-parent dyads. Children were 5 males, 9 females aged 7–17yrs with CF. All participants White European. Frequency of needle procedures ranged from 1-6 times a year, no other indicators of CF severity reported.	Interviews	Thematic analysis	Feeling in control, having influence
Barker et al. [32] (2012) USA	To investigate perspectives on the support adolescents received from friends/family in CF management	24 adolescents with CF; mean age 15.7 years, 50% female,17% Hispanic no other ethnicity data reported, mean FEV1^a % 73.6% ±24.1,	Interviews	Template analysis	Feeling in control, being in charge, autonomy
Christian & D'Auria [33] (1997) USA	To explore adolescents’ conceptualisations of CF & related life events	20 12–18yr olds with CF; 12 female, 8 male; 17 described as white, 3 as Native American; CF severity: 6 mild, 14 moderate	Interviews	Grounded theory	Feeling in control, having influence, agency
Dashiff et al. [34] (2013) USA	To describe the experience of CF-related diabetes (CFRD), and the relationship of adolescent disease self-management with parental support.	10 15–19yr olds (30% female, 70% male), 90% described as Caucasian (non-Hispanic), 10% Black, CF severity not described. Included parent perspective	Mixed methods: Interviews; quantitive questionnaires	Qualitative description	Being in charge, having mastery, agency, autonomy
D'Auria et al. [35] (1997) USA	To understand the middle-childhood experience of CF as a chronic illness	20 children aged 6–12yrs with CF, 12 males 8 females, all Caucasian, CF severity not reported.	Interviews	Grounded theory	Having influence, agency
Denford et al. [36] (2019) UK	To explore motives, enablers and barriers to physical activity among adolescents with CF	12 12–18yr olds with CF (5 female, 7 male); 9 self-reported as active, 3 as inactive; ethnicity & CF severity not reported	Interviews, photo-elicitation	Thematic analysis	Autonomy, mastery
Durst et al. [37] (2001) USA	To understand CF patients’ common themes and emotional responses, post lung-transplant	19 adolescent transplant recipients with CF. Mean age at transplant 15.7 ± 2.7. Time from transplant to interview 1–58 months. 11 female, 8 male. 15 Caucasian, 3 Latino, 1 African American; no other indicators of CF severity reported	Interviews	Ethnographic study design – no further details of data analysis provided	Feeling in control
Keyte et al. [38] (2019) UK	To explore knowledge, attitudes and beliefs towards risky health behaviours in a paediatric CF population.	10 12–18yr olds with CF (5 female, 5 male) mean age 14; median FEV1^a=69.1	Interviews	Thematic analysis	Agency, autonomy
Moola & Faulkner [39] (2014) Canada	To describe attitudes to illness and how illness narratives affect attitudes towards physical activity in children with CF. Part of a broader study (Moola et al. 2011) evaluating a physical activity counselling intervention.	2 adolescents with CF aged 16 & 17yrs (1 female, 1 male); ethnicity not reported; CF severity: moderate FEV1^a=85%	Multiple interviews. Part of a wider intervention study.	Thematic analysis; Case study analysis	Feeling in control, having mastery, being in charge, having influence, agency, autonomy
Moola et al. [40] (2012) Canada	To explore perceptions of exercise in young people with CF	14 young people with CF aged 11–17yrs (10 female, 5 male) 13 described as Caucasian, 1 as Black, 1 as East Indian. CF severity not reported.	Interviews	Grounded theory	Having mastery, agency
Nuttall & Nicholes [41](1992) USA	To describe problems arising in adolescents with CF related to maternal concerns, hospital/ home care, and healthcare professionals’ role in promoting care	20 adolescents with CF; 11 female, 9 male; mean age 17.9yrs; 98% Caucasian; CF severity described as 'moderate advanced'	Interviews	Content analysis (no reference provided)	Feeling in control, being in charge, having influence, agency or autonomy
Pizzignacco & de Lima [42] (2006) Brazil	To identify the routines of CYP with CF and situations that can affect these routines	8 7–18yr olds with CF; gender, ethnicity, & CF severity not reported	Ethnographic design: Interviews, field diary notes & patient chart review	Thematic analysis - no further details of data analysis provided	Feeling in control, being in charge, having influence, agency or autonomy
Savage & Callery [43] (2007) Ireland	To explore children and parents’ perspectives on communication between children, parents & professionals about CF diet.	32 children with CF aged 6–14yrs (18 female, 14 male); ethnicity not reported; CF severity: children in sample ranged from below 3rd to above 97th percentile for height & weight. Included parent perspective	Interviews; observations of clinical consultation	Discourse analysis	Feeling in control, being in charge, having influence, agency or autonomy
Sawicki et al. [44] (2015) USA	To explore facilitators and barriers to adherence with chronic CF therapies; perspectives of adolescents and their parents.	18 adolescents with CF aged 16–21yrs (10 female, 8 male). Ethnicity not reported. Mean FEV1^a=91% (range 38–127%), Mean BMI 21.9 (range 18.7–24.6)	Interviews	Thematic analysis? – no further details of data analysis provided	Feeling in control, having mastery, being in charge, having influence, agency, autonomy
Williams et al. [45] (2007a) UK	To explore the nature and variation in roles of family members, and how responsibility is transferred from the parent/family to the child, and what factors aid or hinder this	32 children with CF aged 7–17yrs (18 female, 14 male); ethnicity not reported; CF severity reported as: 16 mild FEV1^a>70%, 9 moderate 51-69%, 8 severe 20-50% Included parent perspective	Interviews	Framework analysis	Feeling in control, having mastery, being in charge, having influence, agency, autonomy
Williams et al. [46] (2007 b) UK	To explore child and parent accounts of the difficulties of adhering to chest physio for CF, and identify strategies used by families to overcome these.	32 children with CF aged 7–17yrs (18 female, 14 male); ethnicity not reported; CF severity: 15 mild FEV1^a>70%, 9 moderate 51–69%, 8 severe 20–50%. Included parent perspective	Interviews	Framework analysis	Feeling in control, having mastery, being in charge, having influence, agency or autonomy
Williams et al. [47] (2009) UK	To describe the idea of normality: forms, meaning and importance.	32 children with CF aged 7–17yrs (18 female, 14 male); ethnicity not reported; CF severity: 15 mild FEV1^a>70%, 9 moderate 51–69%, 8 severe 20–50%	Interviews	Framework analysis	Feeling in control, having mastery, being in charge, having influence, agency or autonomy

^aFEV₁ (forced expiratory volume in 1 s) is a measure of CF disease severity. An FEV₁ of 100% means that lung function is as would be expected for a person of the same age and height who doesn’t have CF. FEV₁ ≥85% is considered normal; 70–84% indicates mild lung disease, 41–69% indicates moderate lung disease; ≤40% suggests severe lung disease (12).

Table 2. An overview of the quality assessment using CASP [29] and Thomas et al.'s [30] criteria.

Authors, Year	Clear aims	Appropriate methodology	Appropriate design	Appropriate recruitment	Appropriate data collection	Appropriate reflexivity	Consideration of ethics	Rigorous analysis	Clear findings	Research valuable	1	2	3
Ayers et al., 2011 [31]	Y	Y	Y	Y	Y	?	Y	Y	Y	Y	Y	Y	N
Barker et al., 2012 [32]	Y	Y	Y	?	Y	?	Y	Y	Y	Y	Y	Y	N
Christian & D'Auria 1997 [33]	Y	Y	Y	Y	Y	?	?	Y	Y	Y	Y	Y	N
Dashiff et al., 2013 [34]	Y	Y	Y	Y	Y	?	Y	?	Y	Y	Y	?	N
D'Auria et al., 1997 [35]	Y	Y	Y	Y	Y	?	Y	Y	Y	Y	Y	Y	N
Dentford et al., 2019 [36]	Y	Y	Y	Y	Y	Y	Y	Y	Y	Y	Y	Y	Y
Durst et al., 2001 [37]	Y	Y	?	Y	?	?	?	?	?	Y	Y	?	N
Keyte et al., 2019 [38]	Y	Y	Y	Y	Y	Y	Y	Y	Y	Y	Y	?	N
Moola & Faulkner 2014 [39]	Y	Y	Y	N	Y	?	?	?	?	Y	Y	?	N
Moola et al., 2012 [40]	Y	Y	Y	Y	Y	Y	Y	Y	Y	Y	Y	Y	N
Nuttall & Nicholes 1992 [41]	Y	Y	Y	Y	Y	?	Y	Y	Y	Y	Y	Y	N
Pizzignacco & de Lima 2006 [42]	Y	Y	Y	Y	Y	?	Y	?	Y	Y	Y	Y	N
Savage & Callery 2007 [43]	Y	Y	Y	Y	Y	?	Y	Y	Y	Y	Y	Y	N
Sawicki et al., 2015 [44]	Y	Y	Y	Y	Y	Y	Y	Y	Y	Y	Y	Y	N
Williams et al., 2007 [45]	Y	Y	Y	Y	Y	?	Y	Y	Y	Y	Y	Y	Y
Williams et al., 2007 [46]	Y	Y	Y	Y	Y	?	Y	Y	Y	Y	Y	Y	Y
Williams et al., 2009 [47]	Y	Y	Y	Y	Y	Y	Y	Y	Y	Y	Y	Y	Y

Y: yes; ?: unclear; N: no; 1: used appropriate data collection methods for helping children to express their views [29]; 2: used appropriate methods for ensuring the data analysis was grounded in the views of children [29]; 3: actively involved children in the design and conduct of the study [29].

Table 3. Table of studies contributing to themes.

Theme	Subtheme	Contributing studies
Relational support	Practical support	Barker et al. [32]; Dashiff et al. [34]; Keyte et al. [38]; Moola & Faulkner [39]; Moola et al. [40]; Nuttall & Nicholes [41]; Sawicki et al. [44]; Williams et al. [45]
	Emotional support	Ayers et al. [31]; Barker et al. [32]; Christian & D’Auria [33]; Dashiff et al. [34]; Denford et al. [36]; Keyte et al. [38]; Moola & Faulkner [39]; Moola et al. [40]; Nuttall & Nicholes [41]
Information and understanding	Knowledge about CF	Christian & D’Auria [33]; D’Auria et al. [35]; Dashiff et al. [34]; Denford et al. [36]; Keyte et al. [38]; Moola & Faulkner [39]; Moola et al. [40]; Nuttall & Nicholes [41]; Williams et al. [39]
	Learning from experience	Ayers et al. [31]; Dashiff et al. [34]; Durst et al. [37]; Pizzignacco & de Lima [42]; Sawicki et al. [44]; Williams et al. [47]
Feeling heard and respected	Asserting autonomy	Ayers et al. [31]; Barker et al. [32]; Christian & D’Auria [33]; D’Auria et al. [35]; Denford et al. [36]; Durst et al. [37]; Moola et al. [40]; Nuttall & Nicholes [41]; Pizzignacco & de Lima [42]; Williams et al. [46]
	Shared decision making	Dashiff et al. [34]; Nuttall & Nicholes [41]; Savage & Callery [43]; Sawicki et al. [44]; Williams et al. [46]
	Adults making decisions	Barker et al. [32]; Durst et al. [37]; Moola & Faulkner [39]; Moola et al. [40]; Nuttall & Nicholes [41]; Savage & Callery [43]; Sawicki et al. [44]
	Contextual fit	Barker et al. [32]; Moola & Faulkner [39]; Moola et al. [40]; Nuttall & Nicholes [41]; Pizzignacco & de Lima [42]; Williams et al. [45,47]
Mastery and competence	Achievement	Denford et al. [36]; Moola & Faulkner [39]; Moola et al., 2012; Williams et al. [34]
	Routine	Dashiff et al. [34]; Denford et al. [36]; Durst et al. [37]; Moola & Faulkner [39]; Sawicki et al. [44]; Williams et al. [45]
	Normalising & engaging	Christian & D’Auria [33]; D’Auria et al. [35]; Denford et al., [36]; Durst et al. [37]; Moola & Faulkner [39]; Moola et al. [40]; Savage & Callery [43]; Williams et al. [46,47]
	Positive identity	Christian & D’Auria [33]; D’Auria et al. [35]; Denford et al. [36]; Durst et al. [37]; Keyte et al. [38]; Moola & Faulkner [39]; Moola et al. [40]; Williams et al. [47]
	Feelings of hopelessness	Denford et al. [36]; Moola & Faulkner [39]; Moola et al. [40]; Savage & Callery [43]
Prejudice and assumptions	Bullying	Christian & D’Auria [33]; D’Auria et al. [35]; Moola et al. [40]; Pizzignacco & de Lima [42]; Williams et al. [45]
	Differential treatment	Barker et al. [32]; Christian & D’Auria [33]; Denford et al. [36]; Durst et al. [37]; Moola et al. [40]; Pizzignacco & de Lima [42]; Williams et al. [47]
Navigating being different	Effect of age	Christian & D’Auria [33]; D’Auria et al. [35]; Dashiff et al. [34]; Savage & Callery [43]; Sawicki et al. [44]; Williams et al. [46,47]
	Effect of comparison	Christian & D’Auria [33]; Dashiff et al. [34]; Moola & Faulkner [39]; Moola et al. [40]; Williams et al. [47]
	Perceptions and management of threats to ‘normality’	Christian & D’Auria [33]; D’Auria et al. [35]; Denford et al. [36]; Keyte et al. [38]; Moola & Faulkner [39]; Moola et al. [40]; Pizzignacco & de Lima [42]; Sawicki et al. [44]; Williams et al. [47]
	Deciding if and who to tell	Barker et al. [32]; Christian & D’Auria [33]; D’Auria et al [35]; Pizzignacco & de Lima [42]
	Competing priorities	Barker et al. [32]; Denford et al. [36]; Keyte et al. [38]; Moola et al. [40]; Pizzignacco & de Lima [42]; Savage & Callery [43]; Sawicki et al. [44]; Williams et al. [45,46]
	Positive aspects of difference	Christian & D’Auria [33]; Denford et al. [36]; Durst et al. [37]; Williams et al. [47]

Table 4. Representative quotations.

Themes	Subthemes	Exemplary quotations
Relational support	Practical support	“Well thumps, and huffs and puffs, and stuff like that. Mum tells me what breaths to do and I do them” (Williams et al. [45])
	Emotional support	“I think it's really helpful if you could talk to somebody else that had CF… I think that is one of the most helpful things that has helped me, those CF camps that we can't, we can't have them anymore … I learned a lot from them.” (Christian & D’Auria [33])
Information and understanding	Knowledge about CF	“I didn't take my pills at school because that was when I first started, 'cause I was like "Why do you take your pills?" And I didn't really know what to say to them because I was younger ….” (Christian & D’Auria [33]) “I don’t know very much. I know it’s more or less similar to Down’s Syndrome. It’s from the same chromosome, I think it is, but I really don’t know for sure” (Pizzignacco & de Lima [42])
	Learning from experience	‘I had a bad experience and I suppose it stuck’ (Ayers et al. [31]) One 18-year-old said that after he had gotten sick and was hospitalized following a summer of “slacking off” on his treatments, he got a “wake up call” and “started to take [adherence to treatment] more seriously.” (Sawicki et al. [44])
Feeling heard and respected	Asserting autonomy	“I play football because it’s fun…I definitely wouldn’t have been so keen on the idea if I was being told I had to play football” (Denford et al. [36])
	Shared decision making	Physiotherapist: “so what’s your analysis of the whole thing [child], if you were to pick one technique, what would you prefer?” Child: “The breathing [humming], that’s better” (Savage & Callery [43])
	Adults making decisions	“It’s boring you just have to sit there and listen to all this information about me. I don’t like it. They just talk to my Mum and Dad” (Savage & Callery [43])
	Contextual fit	“cause sometimes she’ll say it and it’ll really get to me and I’ll be like, ‘Don’t tell me what to do’ or ‘I’ll do whatever I want’, you know, ‘I can take care of myself’. So it’s not that she’s saying anything differently, it’s just the way I’m perceiving it that day” (Barker et al. [32])
Mastery and competence	Achievement	“…you know it’s [exercise] going to be hard…you know it’s going to be painful…But that sense of achievement at the end…And that’s where having CF gives me an edge… I’m used to pushing through” (Denford et al. [36])
	Routine	“There was a big emphasis during my upbringing…the way parents dealt with physio and exercise from day one…Like if I miss one session it wouldn’t kill me, but it’s like a religious practice… [Exercise and physiotherapy] is just what you do” (Denford et al. [36])
	Normalising & engaging	“Football was the one thing I did that had absolutely nothing to do with having cystic fibrosis. I mean obviously it did in terms of the implication of cardio on my lungs, but that wasn’t what I thought about. When I was playing football, I was just like everyone else and could forget that I was the kid with that illness…It’s about being normal, and healthy and having fun, and not just being the kid with CF for a while” (Denford et al. [36])
	Positive identity	Their surgical scars represented `strength', `survival', and `a signature point in my life' (Durst et al. [37]) “…like Josh Llewellyn-Jones… very fit inspirational guys…they’re an inspiration for ‘what can CF patients do’ (Denford et al. [36])
	Feelings of hopelessness	“I did suffer from depression for a while…I just didn’t care about anything to do with CF…I just wanted to be left alone…I didn’t believe that I could do anything…I just thought it was unfair” (Denford et al. [36]) “It is ‘like whatever, I have to do it for the rest of my life anyways.’ After a while, it gets very irritating. It is like ‘I have to do this for the rest of my life? Screw it! Who cares! I am not going to do it anymore.” (Moola & Faulkner [39])
Prejudice and assumptions	Bullying	“All the kids, you know, saying "ewwr"…. On the bus” (Christian & D’Auria [33]}
	Differential treatment	“I do not want to be treated differently . . . so what, I have CF. One of my cousins, he went easy on me in sports and I got really mad.” (Moola et al. [40]) “…my mom never wants me to leave the house…but I did not go through transplant to live my life in a bubble” (Durst et al. [37])
Navigating being different	Effect of age	“They [parents] really had to learn to let me go, I think, and that started…when I was younger when they handed over my medications to me and gave me the responsibility of taking them.” (Sawicki et al. [44])
	Effect of comparison	“Sometimes, when I am running, I get more tired easily and faster than other people. Sometimes that kind of makes me not want to always do sports . . .” (Moola et al. [40]) “I did a marathon a few years ago, to raise awareness of CF… my CF isn’t that bad, I have a relatively mild mutation. So that is one of the other things that motivates me. Because I can do it, and I think that [there are] so many people with CF who would like to do more activity, but can’t” (Denford et al. [36])
	Deciding if and who to tell	“I'm not going to tell my coach I have it. So he won't treat me any different than the other kids.” (Christian & D’Auria [33])
	Perceptions & management of threats to ‘normality’	“it’s quite hard when I’ve got friends staying or something, it’s like I have to go through to the spare room do my breathing stuff , whereas if I didn’t have CF I wouldn’t have to, I just hate having to do all the stuff I have to do.” (Williams et al. [46])
	Competing priorities	“I know that I should do it (activity)—it should be higher on the priority list. The more important things for me are like work and school” (Moola et al. [40]) “…my mom never wants me to leave the house…but I did not go through transplant to live my life in a bubble” (Durst et al. [37])
	Positive aspects of difference	“Having CF gives me an edge… I’m used to pushing through. Where maybe some of the others are a bit quicker to stop. But then, for them, the stakes aren’t quite as high” (Denford et al. [36])

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