Table 1.

Characteristics of included studies

Reference	Location	Participants	Data collection	Data analysis	Summary of findings
Fereday et al 38	Australia	Twenty-five participants (aged 4–16 years). Fourteen had a diagnosis of type 1 diabetes, six asthma and five cystic fibrosis.	A combination of focus groups, interviews, drawing maps, taking photos, and traffic light posters	Interpretive phenomenological analysis	Children and young people described their active participation in a wide variety of physical activities including organised sports and play but made very little mention of any negative influence or impact due to their disease. Their parents' stories described the diligent background planning and management undertaken to enable their child to participate in a wide range of physical activities.
Happ et al 42	USA	Eleven child-parent pairs. Five girls, six boys (aged 10–16 years). All had a diagnosis of CF. Six children were from the experimental group, and five from the attention-control group. Parent interview participants were nine mothers and four fathers, aged 29–51 years. All participants were Caucasian.	Individual child and parent interviews, conducted at 2 months into the exercise programme and again at 6 months	Thematic analysis	Five major thematic categories describing child and parent perceptions and experience of the bicycle exercise programme were identified in the transcripts: (A) Motivators. (B) Barriers. (C) Effort/work. (D) Exercise routine. (E) Sustaining exercise. Research participation, parent-family participation, health benefits, and the child’s personality traits were primary motivators. Competing activities, priorities and responsibilities were the major barriers to implementing the exercise programme as prescribed. Motivation waned and the novelty wore off for several (approximately half) parent-child dyads, who planned to decrease or stop the exercise programme after the study ended.
Moola 39	Canada	Two children. One male, one female. Participants were randomly selected from an ongoing trial.	Semistructured interviews and field notes	Case study analysis	The findings beg researchers to consider: (A) How children with life-limiting diseases borrow multiple illness narrative types. (B) The role of development in influencing the kinds of stories that children can tell. (C) The impact of illness narratives on physical activity. By rendering the tales of two youths with CF in this study, we respond to Aurthur Frank’s call; taking a multiple narrative turn, we listen to stories of a different kind of suffering.
Moola 41	Canada	Fourteen participants. Ten male, five female (aged 11–17 years). All had a diagnosis of CF. Although the majority of the sample was Caucasian, one participant self-identified as Black and the other as East Indian.	Semistructured interviews	Grounded theory	The participants demonstrated positive or negative perceptions towards physical activity and different experiences—such as parental support and illness narratives—influenced youths’ perceptions. In addition, the participants experienced physical activity within the context of reduced time. Recommendations for developing physical activity interventions, including the particular need to ensure that such interventions are not perceived as wasteful of time, are provided.
Moola et al 40	Canada	Twenty-nine parents who provided care to a child with CF or CHD between the ages of 10 years and 18 years, participated (16 parents from the CF clinic and 13 parents from the CHD centre). Parents were from a range of urban and rural locations across Ontario and Quebec and access to physical activity opportunities varied.	Semistructured interviews	Thematic analysis	Parents discussed the numerous benefits and barriers associated with physical activity for both child and self. Role modelling was a critical social process to overcoming barriers. Parents’ experiences were situated within the broader family context characterised by a prevailing sense of stress and complexity.
Shelley et al 44 2018	UK	Nine participants, five female, four male (aged 8–16 years). All participants had a confirmed diagnosis of CF.	Semistructured interviews	Interpretive phenomenological analysis	Findings suggest that experiences of PA in children and young people with CF are largely comparable to their non-CF peers, with individuals engaging in a variety of activities. CF was not perceived as a barrier per se, although participants acknowledged that they could be limited by their symptoms. Maintenance of health emerged as a key facilitator. In some cases PA offered patients the opportunity to ‘normalise’ their condition. Participants reported enjoying wearing the monitoring devices and had good compliance. Wrist-worn devices and devices providing feedback were preferred. HCPs recognised the potential benefits of the devices in clinical practice. Recommendations based on these findings are that interventions to promote PA in children and young people with CF should be individualised and involve families to promote PA as part of an active lifestyle. Patients should receive support alongside the PA data obtained from monitoring devices.
Swisher 43	USA	Ten participants (aged 13–17 years). All participants had a diagnosis of CF.	Semistructured telephone interviews	Verbatim and transcripts were coded using the line-by-line coding process, thus allowing the researcher to deconstruct the data into discrete pieces of information that could be compared and grouped into categories. In order for a code to be assigned to a response, the code had to be identified by both principal investigators and the graduate student.	All participants articulated understanding the importance of participating in physical activity for health benefits. Factors that served as facilitators to participation in physical activity included improving general or lung-specific health, as well as mental health. Barriers included general discomfort, increased lung symptoms and disinterest.

CF, cystic fibrosis; CHD, Congenital heart disease; HCP, Health care professional; PA, Physical activity.