Table 1 Illustrative quotes exploring PA perceptions among children and young people with CF

Physical activity participation

Perceived barrier

Perceived enabler

Limited PA facilities available locally

Community activities facilitated by private clubs (Thai boxing, football, dance, table tennis, gymnastics) and/or local authorities.

“A few more different clubs that do different sports that are around, because there isn’t many.” (P4, pg7, lines 306–307).

The swimming centre up the road, and out there is a big Astro Turf…so I’ll usually go there, and the other places are sports clubs and stuff.” (P2, pg7, lines 301–302).

Limited time available to introduce and explore new activities

Curricular physical education (PE) and additional non-structured activities such as walking to and from school and play during recess.

“Maybe if I’d seen a game or something on the telly, or one of my mates was going to somewhere and said that “it’s good”. “You should come and try it”. I might have a go, but I probably wouldn’t, because I don’t really have time…” (P7, pg10, lines 431–440).

“I don’t do any out of school, but in school, apart from PE I do football at breaks and dinners.” (P3, pg3, lines 120–121).

Autonomy promoted by independent travel

“I can do it [travel] on my own. It’s like I’m not dependent on everyone else to do it for me.” (P7, pg9, lines 361–362).

“It’s either a lift or, because it’s across the [name of local] field across the road, so I can just walk over to that…” (P6, pg8, lines 326–327).

Predisposing factors

Am I able?

Participants attributed poor PA-related performance to CF related symptoms, such as breathlessness, fatigue and pain.

CF was not perceived as a barrier to PA per se.

“I can do it [PA], but not as good as other people…I get tired quicker than them, or out of breath more quicker. I can do it to a certain point, but then I have to stop.” (P3, pg13, lines 573–579).

“…there’s nothing really…wrong with me I can still do it…I’m not stuck like at home or in hospital. I’m out, like able to do anything, really.” (P1, pg12, lines 515–525).

For some this results in frustration, anger and boredom;

“I know just because I’ve got CF doesn’t mean I can’t do it [PA].” (P3, pg23, lines 1020–1021).

“Well, it’s a bit annoying, because they’re all doing it, and I’m sat at the side, and it winds me up that I should be able to do it, but I just can’t.” (P3, pg13, lines 584–585).

“… I have to do twice as much as my mate, to do what my mates do, so then when I can do what my mates are doing I just feel better, because I know that it doesn’t show that it’s affecting me, and I can keep up with my mates and just do all the exercise and everything.” (P6, pg20, lines 846–849).

CF- related illness can render participants incapable of engaging in PA.

Perceptions of current well-being

“…Like when I’m ill I feel like I can’t do anything. I’m sitting on the couch and watching TV, and I’m not doing much.” (P9, pg23, lines 1004–1005).

“…’cos I am generally quite well, I can do it.” (P7, pg16, line 702).

“I tend to have quite a high lung function, and I don’t really get ill a lot…” (P7, pg17, line 707).

Is it worth it?

Some participants reported disliking the experience of pain, fatigue and breathlessness associated with PA;

All participants report enjoying PA. Enjoyment also appears to be inextricably linked to physical benefits gained through PA

“(I dislike) The way you get tired and out of breath sometimes.” (P3, pg23, line 1025).

“I like it [PA] because it helps my chest and stuff.” (P2, pg19, line 864)

“That now and then it gives you the pains the next day. Like you’re dragging your legs up the stairs the next day.” (P9, pg35, lines 1517–1518).

Participants also recognise health benefits associated with PA, both in the short and long term.

“…it [PA] keeps you active and your lungs clear, and instead of just sitting in hospital or something.” (P1, pg14, lines 631–632).

Engaging in PA becomes a normaliser;

“It’s just like you’re doing it because you can, and you want to. You kind of feel the same as everyone else for an hour and a half…” (p7, pg19, lines 803–804).

Parental support can generate a negative affect;

Family support and encouragement

“I did a mile on the treadmill the other day, and Dad was like, “No, you’re going to do another one… (I feel like) I’m going to slap him. Push him off his bike. You do another mile!” (P7, pg12, lines 493–503).

...my Mum always like, not makes me go, but if like I’m just too tired, I don’t want to, she’d be like, “Oh no, come on, let’s get out or something.” (P1, pg14, lines 641–643).

Peer support

“…my friends who knock on for me, they are dead nice because they always ask if I’m ok if I’m out of breath and stuff when I’m out playing footy and stuff.” (P2, pg21, lines 942–944).

“Like one of us wins a race or wins a game or something, I can go, “Oh yes, well, I’ve got CF”, and then it’s like pulling a CF card…I just find it funny, because they’re like, “Aaaaaaah! She’s done it again”…we have a laugh about it….” (P7, pg13–14, lines 565–574).

Significant coaches (conventional and novel, including PE teachers) influence

“Well, a mixture of everyone. There isn’t really anyone that influences me any more than someone else… Family, people in the CF team, my PE teachers.” (P4, pg11, lines 498–503).

The CF specialist physiotherapist was identified by participants (P2, 3 and 9) as the CF clinician who most encourages them to be physically active. Participants also perceive health-related information and advice to be trustworthy and reliable;

“I think that it’s good advice, and that I should take it.” (P1, pg11, line 482).

Family facilitating activity (e.g. driving to sports clubs or engaging in family activities)

“My Mum or Dad would usually take me.” (P4, pg7, line 279).