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Frontiers in Sports and Active Living logoLink to Frontiers in Sports and Active Living
. 2026 Mar 27;8:1786911. doi: 10.3389/fspor.2026.1786911

Mediating effects of physical activity enjoyment on physical activity levels in adults with cystic fibrosis

Matthias Welsner 1,*, Jin-Sun Schermaul 1, Jose Guillermo Ortiz 1, Liron Lechtenberg 1, Christian Taube 1, Florian Stehling 2, Wolfgang Gruber 2
PMCID: PMC13066178  PMID: 41970597

Abstract

Background

This study aimed to investigate the mediating effect of physical activity enjoyment (PAE) on various physical activity (PA) levels in adult people with cystic fibrosis (pwCF) concerning disease-specific and non-disease-specific factors.

Methods

A total of 168 adult pwCF (39.3% females; mean age 36.7 ± 11.9 years) completed questionnaires assessing PAE (Physical Activity Enjoyment Scale, PACES) and PA levels (7-day Physical Activity Recall, PAR). Participants’ demographics (age, sex) and clinical characteristics [ppFEV1; percent predicted forced expiratory volume in 1 s, and BMI; body mass index (kg/m2)]) were extracted from medical records. Mediation analysis was used to examine the direct and indirect effects of disease-specific and non-disease-specific factors on PA, considering PAE as a potential mediator.

Results

Correlation analyses indicated a weak but statistically significant association between ppFEV1 and very vigorous physical activity (VVPA) (r = 0.177; p = 0.021) and PAE (r = 0.221; p = 0.004). PAE was linked to time spent in vigorous physical activity (VPA) (r = 0.202; p = 0.009), VVPA (r = 0.238; p = 0.002), and moderate-to-vigorous physical activity (MVPA) (r = 0.152; p = 0.049). Mediation analysis revealed that PAE fully mediated the association between lung function (ppFEV1) and vigorous PA (VPA), very vigorous PA (VVPA), and moderate-to-vigorous PA (MVPA) but not moderate PA (MPA). PpFEV1 and PAE together accounted for 26–49.3% of the variance in PA, suggesting the presence of other influential factors. No mediation effect was observed between PAE and age and BMI on any of the PA levels

Conclusions

Our findings underscore the importance of both physiological and psychological factors in shaping PA among adults with CF. Beyond traditional clinical management, strategies that enhance PAE may be crucial for promoting sustained PA engagement. Future research should examine additional psychological and environmental factors influencing PAE and develop comprehensive approaches to support active lifestyles in this population.

Keywords: adults, cystic fibrosis, mediation analysis, physical activity, physical activity enjoyment

Background

Physical activity (PA) plays a crucial role in the non-pharmacological care of people with cystic fibrosis (pwCF). Lower PA levels are associated with decreased pulmonary function, impaired glycemic control, and reduced bone mineral density (1). Therefore, high levels of PA and fitness are associated with improved health-related quality of life (HrQoL), potentially slowing disease progression, and are therefore identified as prognostic factors (2, 3).

Despite its importance, the majority of pwCF do not engage in the recommended amounts of moderate-intensity PA of 150 min per week, or at least 75 min per week of vigorous-intensity physical activity, or an equivalent combination of both (3, 4). For additional health benefits, adults should increase their moderate-intensity PA to 300 min per week or equivalent (5). Studies on both pwCF and healthy populations have shown that extended periods of reduced moderate-to-vigorous physical activity (MVPA) result in an earlier onset of obesity and adult non-communicable diseases, such as cardiovascular and metabolic diseases (3, 6). Particularly in view of the increasing life expectancy of pwCF due to improved and new therapies, maintaining or increasing MVPA appears to be a preventive measure to avoid lifestyle diseases.

PwCF presents numerous challenges for individuals seeking to maintain an active lifestyle. Beyond the physical limitations imposed by the disease itself, such as impaired lung function, low muscle mass and strength, and physical symptoms (e.g., breathlessness, cough, and fatigue), pwCF often face a range of practical and psychological barriers to PA. In a systematic review by Denford et al. regarding barriers and facilitators to PA among pwCF, the feeling of “fun” and “joy” appears to be an important factor for sustained PA and has been identified as particularly significant in pwCF (7). Additional barriersinclude limited access to specialized equipment and professional guidance, social anxieties, lack of knowledge about safe exercise practices, and the unpredictable nature of symptom flare-ups (7–10). Moreover, medication side effects can further complicate efforts to engage in regular PA.

Most studies examining PA in pwCF are limited to general correlates, such as sex, anthropometric parameters, and age, as well as disease-specific correlates, including lung function and exercise tolerance (3). It is important to consider that PA is a complex, multidimensional behavior influenced by numerous internal (e.g., motivation, self-efficacy, health status, and enjoyment) and external factors (e.g., facility access, climate, and cultural norms) (11). The interaction between internal and external factors creates a dynamic system that affects PA patterns.

In general, enjoyment, as an intrinsic factor, is pivotal in fostering PA. When individuals find pleasure in PA, they are more likely to develop a long-term commitment to an active lifestyle (12). This positive emotional response creates a self-reinforcing cycle in which physical activity enjoyment (PAE) encourages continued participation, leading to improved physical fitness and overall well-being. Individuals who enjoy PA are more likely to seek out similar activities, explore diverse forms of exercise, and develop a positive attitude towards physical challenges. This intrinsic motivation helps overcome barriers to PA, as anticipated enjoyment becomes an incentive for participation. Furthermore, enjoyment of PA can foster social connections, enhance self-efficacy, and contribute to a positive body image, reinforcing the maintenance of an active lifestyle.

This study aimed to investigate the mediating effect of PAE on various PA levels concerning disease-specific and non-disease-specific factors within an adult CF population. We hypothesized that PAE significantly influences both the quantity and intensity of PA in adults with CF.

Methods

Subject selection and study design

The eligibility criteria for the study were as follows: participants must have a diagnosis of CF, be at least 18 years of age, and possess adequate German language proficiency. Additionally, candidates needed to be free from significant psychiatric or neurocognitive disorders and demonstrate sufficient cognitive capacity to comprehend and complete the questionnaires without substantial difficulty, as determined by the treating physician's clinical assessment.

All participants provided written informed consent before completing the questionnaires. The study followed the ethical principles of the Declaration of Helsinki and was approved by the local ethics committee of the University Hospital Essen (BO-24-11951). Participants’ demographics and clinical characteristics, including CF-specific data, lung function (ppFEV1; percent predicted forced expiratory volume in 1 s), and body mass index (BMI; kg/m2), were extracted from electronic medical records at the time nearest to the completion of the questionnaires.

The German version of the Physical Activity Enjoyment Scale (PACES) questionnaire was used to assess the extent to which individuals enjoy participating in PA (13). This instrument features 16 items, each evaluated on a 5-point Likert scale ranging from 1 (complete disagreement) to 5 (total agreement). The total score ranges from 16 to 80 points, with higher scores indicating greater enjoyment. The PACES questionnaire has demonstrated both reliability and validity in assessing PAE (14).

To evaluate regular PA patterns, we employed the 7-day Physical Activity Recall (PAR) questionnaire, a frequently used self-reporting tool. This instrument collects data on both PA and sedentary behavior from the previous week. The PAR questionnaire assesses the time (min/week) spent on various intensities of PA, including sleep, moderate physical activity (MPA), vigorous physical activity (VPA), and very-vigorous physical activity (VVPA) (15). The duration of moderate-to-vigorous PA (MVPA) was calculated as the sum of MPA and VPA durations. Its validity has been previously tested, even among pwCF, against objective measures, with generally positive results, leading to a consensus that it is a valid instrument for the measurement of PA (16–18).

To identify factors that influence or interact with PAE, participants were categorized into groups based on age (18-30, >30-45, and ≥45 years), ppFEV1 (<40, 40-69, and ≥70), BMI (<25, ≥25), and sex (male/female).

Statistical analysis

Statistical analyses were performed using GraphPad v10.5. (GraphPad Software, Inc., La Jolla, California, USA). Data are presented as mean ± standard deviation (SD). The Shapiro–Wilk test was used to test for normal data distribution. Depending on the data distribution, either the Student's t-test or the Mann–Whitney U test was utilized to compare two groups (BMI, sex). For the comparison of three groups (ppFEV1, age), either an Analysis of Variance (ANOVA) or the Kruskal–Wallis test was conducted. post-hoc analysis was conducted using either the Bonferroni or Dunn tests, as appropriate.

Mediation analysis was performed using jamovi (version 2.6) (19). This approach allowed for the examination of both the direct and indirect effects of disease-specific and non-disease-specific factors on PA, considering PAE as a potential mediator (Figure 1).

Figure 1.

Diagram illustrating a mediation model with three variables: X, M, and Y. X points directly to Y labeled c, and to M labeled a. M points to Y labeled b. X also points to Y labeled c prime.

A conceptual model of a simple mediation model. X: dependent (predictor) variable. M: mediator. Y: independent (outcome) variable. a: effect of X on M. b: effect of M on Y. c′: direct effect of X on Y (controlling for M). ab: indirect effect of X on Y through M. c = ab + c′: total effect of X on Y.

For all analyses, a p-value ≤ 0.05 was considered statistically significant.

Results

Sample description

A total of 208 pwCF participated in this study. After verifying the completeness of the questionnaires, 168 complete datasets (39.3% females) were deemed suitable for analysis (Figure 2). The mean age of the participants was 36.7 ± 11.9 years, with a mean ppFEV1 of 70.2 ± 22.4 and a BMI of 24.1 ± 4.5. Most pwCF were homozygous for the F508del mutation (46.4%). Of the 168 participants, 139 (83%) received CFTR modulator treatment with elexacaftor-tezacaftor-ivacaftor (ETI). The time interval between the clinical assessment (ppFEV1 and BMI) and the completion of the survey was 0.38 ± 1.85 days. A comprehensive overview of the participants’ demographics and clinical characteristics is presented in Table 1.

Figure 2.

Flowchart illustrating participant inclusion for statistical analysis: out of 208 assessed for eligibility and completeness with all questionnaires received, 50 excluded for incomplete PACES or PAR questionnaires, resulting in 168 included in the final analysis.

Participant flow diagram. PAR, 7-day physical activity recall; PACES, physical activity enjoyment scale.

Table 1.

Participitants’ demographics and clinical characteristics.

Characteristics N = 168
Age (years) 36.7 ± 11.9 [34.9 - 38.5]
Female sex, n (%) 66 (39.3)
Genotype, n (%)
F508del homozygous 78 (46.4)
F508del heterozygous 74 (44.1)
Other 16 (9.5)
CFTR modulator therapy, n (%)
ETI 139 (82.7)
Mono-/dual 7 (4.2)
None 22 (13.1)
ppFEV1 70.2 ± 22.4 [66.8 - 73.6]
FEV1 [L] 2.7 ± 1.1 [2.6–2.9]
ppFVC 87.2 ± 18.6 [84.3–90.0]
FVC [L] 4.1 ± 1.2 [3.9–4.3]
Body weight [kg] 71.6 ± 15.3 [69.2–73.9]
BMI [kg/m2] 24.1 ± 4.5 [23.4 - 24.7]
Oxygen supplementation, n (%) 13 (7.7)
Pseudomonas aeruginosa, n (%) 85 (50.6)
Pancreatic insufficiency, n (%) 149 (88.7)
Cystic fibrosis-related diabetes, n (%) 16 (9.5)
PAE (PACES score) 65.4 ± 11.3 [63.7–67.2]
MPA (min/week) 656.3 ± 591.7 [566.2–746.5]
VPA (min/week) 216.6 ± 275.7 [174.6–258.6]
VVPA (min/week) 179.0 ± 248.4 [141.2–216.9]
MVPA (min/week) 827.9 ± 725.4 [762.4–983.4]

Values are expressed as mean ± standard deviation or number of patients (%). Brackets indicate the 95% confidence intervals (CI).

BMI, body mass index; ppFEV1, percent predicted forced expiratory volume in one second; ppFVC, percent predicted Forced Vital Capacity; CFTR, cystic fibrosis transmembrane conductance regulator, MPA, moderate physical activity; VPA, vigorous physical activity; VVPA, very vigorous physical activity; MVPA, moderate-to-vigorous physical activity; PAE, physical activity enjoyment; ETI, elexacaftor, tezacaftor, ivacaftor.

Group differences physical activity enjoyment (PAE, PACES score)

For a complete overview of the group differences for PAE, see Figure 3.

Figure 3.

Figure showing four panels (A–D), each with four grouped bar graphs. Graphs compare outcomes by ppFEV1, BMI, age, and sex. Panel A presents physical activity enjoyment (PACES score), with a significant sex difference. Panel B shows MPA (minutes/week) with a significant sex difference. Panel C displays VPA (minutes/week), with significant differences for ppFEV1 and sex. Panel D presents MVPA (minutes/week), showing no significant differences across variables. Error bars and statistical markers, including asterisks and \"ns\" for not significant, are included.

Group differences for PAE (A) and different PA levels (B-D). Distribution of patient numbers (n) across the groups. Stratification includes: ppFEV1 < 40 (n = 22), 40–69 (n = 54), ≥70 (n = 92); BMI <25 (n = 116), ≥25 (n = 52); age 18–30 (n = 58), >30–45 (n = 76), >45 (n = 34); and sex (male, n = 102; female, n = 66). BMI, body mass index; ppFEV1, percent predicted forced expiratory volume in one second; MPA, moderate physical activity; VPA, vigorous physical activity; VVPA, very vigorous physical activity; MVPA, moderate-to-vigorous physical activity; PAE, physical activity enjoyment. * p < 0.05, ** p < 0.01, *** p < 0.001, NS, non-significant.

Female pwCF exhibited lower PAE levels than their male counterparts (p = 0.033). Those pwCF with preserved lung function (ppFEV1 ≥ 70) demonstrated a higher PACES score than those with a mildly reduced ppFEV1 of 40-69 (p = 0.041, but not those with ppFEV1 < 40 (p = 0.151). No significant group differences were observed in age (p = 0.569) and BMI (p = 0.349).

Group differences PA levels

Figure 3 presents a comparative analysis of the groups concerning the different PA levels. Male pwCF demonstrated a significantly greater duration of VVPA than their female counterparts (p < 0.001). Additionally, no significant differences in VVPA were identified concerning ppFEV1, BMI, and age (all p > 0.05). Subgroup analyses revealed that pwCF with ppFEV1 ≥ 70 engaged in more VVPA than those with ppFEV1 ranging from 40 to 69 (p = 0.039) but not those with ppFEV1 < 40 (p = 0.074). No significant differences were observed among the groups for MPA, VPA, or MVPA (all p > 0.05).

Correlation analysis

As shown in Table 2, the correlation analyses indicated a weak but statistically significant association between ppFEV1 and both VVPA (r = 0.177; p = 0.021) and PAE (r = 0.221; p = 0.004). In contrast, no significant correlation was observed between ppFEV and MPA (r = 0.056; p = 0.469), VPA (r = 0.086; p = 0.267), or MVPA (r = 0.079; p = 0.311). PAE was linked to the time spent in VPA (r = 0.202; p = 0.009), VVPA (r = 0.238; p = 0.002), and MVPA (r = 0.152; p = 0.049), but not with MPA (r = 0.092; p = 0.236). Additionally, there was no correlation found between BMI or age and PA levels (all p > 0.05).

Table 2.

Correlation matrix.

Variable Correlation coefficient p-value Age (years) BMI (kg/m2) ppFEV1 MPA (min/week) VPA (min/week) VVPA (min/week) MVPA (min/week)
MPA (min/week) Pearson's r -0.008 0.001 0.056 —
p-value 0.915 0.988 0.469 —
VPA (min/week) Pearson's r -0.085 -0.075 0.086 0.307*** —
p-value 0.275 0.336 0.267 <.001 —
VVPA (min/week) Pearson's r -0.056 -0.021 0.177* 0.040 0.261*** —
p-value 0.472 0.791 0.021 0.608 <.001 —
MVPA (min/week) Pearson's r -0.039 -0.027 0.079 0.932*** 0.630*** 0.132 —
p-value 0.616 0.724 0.311 <.001 <.001 0.089 —
PAE (PACES score) Pearson's r -0.003 -0.012 0.221** 0.092 0.202** 0.238** 0.152*
p-value 0.964 0.874 0.004 0.236 0.009 0.002 0.049

BMI, body mass index; ppFEV1, percent predicted forced expiratory volume in one second; MPA, moderate physical activity; VPA, vigorous physical activity; VVPA, very vigorous physical activity; MVPA, moderate-to-vigorous physical activity, PAE, physical activity enjoyment.

*p < 0.05, **p < 0.01, ***p < 0.001, NS, non-significant.

Mediation analysis

Mediation analysis supported the hypothesis that PAE mediates the relationship between ppFEV1 and PA (Table 3). PpFEV1 demonstrated a positive association with PAE at different PA levels (MPA: a = 0.111, p = 0.010; VPA: a = 0.111, p = 0.009, VVPA: a = 0.111, p = 0.008 and MVPA: a = 0.111, p = 0.009), and PAE was positively correlated with time spent in different PA levels: VPA (b = 4.639, p = 0.002), VVPA (b = 4.594, p < 0.001, and MVPA (b = 9.063, p = 0.025), but not to MPA (b = 4.369, p = 0.174). A bootstrap interval with 5000 repetitions for the indirect effect (ab) was entirely above zero for VPA [ab = 0.523, 95%CI (0.100, 1.13)], VVPA [ab = 0.512, 95%CI (0.112, 1.04)], and MVPA [ab = 1.01, 95%CI (0.0384, 2.49)], indicating that PAE fully mediates the association between ppFEV1 and VPA, VVPA, and MVPA, but not with MPA [ab = 0.487; 95%CI (−0.152, 1.56)]. No mediation effect was observed between PAE and age and BMI on any of the PA levels (Supplement 1 and 2).

Table 3.

Results of a simple mediation analysis with a.) MPA, b.) VPA, c.) VVPA and d.) MVPA as dependent variable (X), ppFEV1 as independent variable (Y) and PAE as mediator variable (M).

a.) Mediation analysis for ppFEV1 (X), MPA (Y) and PAE (M)
Mediation Estimates
95% Confidence Interval
Effect Label Estimate SE Lower Upper Z p % Mediation
Indirect a × b 0.487 0.445 −0.152 1.56 1.095 0.274 32.7
Direct c 1.001 1.926 −2.773 4.80 0.519 0.603 67.3
Total c + a × b 1.488 1.849 −2.015 5.19 0.805 0.421 100.0
Path Estimates
95% Confidence Interval
Label Estimate SE Lower Upper Z p
ppFEV1 → PAE a 0.111 0.0433 0.0254 0.195 2.575 0.010
PAE → MPA (min/week) b 4.369 3.2111 −1.7040 10.958 1.361 0.174
ppFEV1 → MPA (min/week) c 1.001 1.9265 −2.7728 4.797 0.519 0.603
b.) Mediation analysis for ppFEV1 (X), VPA (Y) and PAE (M)
Mediation Estimates
95% Confidence Interval
Effect Label Estimate SE Lower Upper Z p % Mediation
Indirect a × b 0.523 0.268 0.100 1.13 1.955 0.051 49.3
Direct c 0.537 0.947 −1.175 2.52 0.567 0.571 50.7
Total c + a × b 1.060 0.899 −0.615 2.93 1.179 0.238 100.0
Path Estimates
95% Confidence Interval
Label Estimate SE Lower Upper Z p
ppFEV1 → PAE a 0.111 0.0426 0.0283 0.196 2.617 0.009
PAE → VPA (min/week) b 4.693 1.5030 1.7283 7.660 3.123 0.002
ppFEV1 → VPA (min/week) c 0.537 0.9473 −1.1748 2.522 0.567 0.571
c.) Mediation analysis for ppFEV1 (X), VVPA (Y) and PAE (M)
Mediation Estimates
95% Confidence Interval
Effect Label Estimate SE Lower Upper Z p % Mediation
Indirect a × b 0.512 0.237 0.11225 1.04 2.16 0.030 26.0
Direct c 1.457 1.004 −0.49982 3.47 1.45 0.146 74.0
Total c + a × b 1.970 0.992 −0.00243 3.98 1.99 0.047 100.0
Path Estimates
95% Confidence Interval
Label Estimate SE Lower Upper Z p
ppFEV1 → PAE a 0.111 0.0421 0.0301 0.193 2.65 0.008
PAE → VVPA (min/week) b 4.594 1.1617 2.3031 6.906 3.96 <.001
ppFEV1 → VVPA (min/week) c 1.457 1.0037 −0.4998 3.469 1.45 0.146
d.) Mediation analysis for ppFEV1 (X), MVPA (Y) and PAE (M)
Mediation Estimates
95% Confidence Interval
Effect Label Estimate SE Lower Upper Z p % Mediation
Indirect a × b 1.01 0.640 0.0384 2.49 1.578 0.115 39.7
Direct c 1.54 2.368 −2.9694 6.28 0.649 0.516 60.3
Total c + a × b 2.55 2.252 −1.6915 7.06 1.131 0.258 100.0
Path Estimates
95% Confidence Interval
Label Estimate SE Lower Upper Z p
ppFEV1 → PAE a 0.111 0.0427 0.0271 0.197 2.610 0.009
PAE → MVPA (min/week) b 9.063 4.0395 1.0253 16.957 2.244 0.025
ppFEV1 → MVPA (min/week) c 1.538 2.3683 −2.9694 6.278 0.649 0.516

ppFEV1, percent predicted forced expiratory volume in one second; MPA, moderate physical activity; VPA, vigorous physical activity; VVPA, very vigorous physical activity; MVPA, moderate-to-vigorous physical activity, PAE, physical activity enjoyment.

Discussion

Our data indicate that among adult pwCF, the extent and intensity of PA are significantly influenced by pulmonary function (ppFEV1) and PAE. Notably, mediation analysis revealed that PAE fully mediated the effect of ppFEV1 on PA, especially at higher PA levels, underscoring the central role of affective factors in PA engagement. Conversely, age and nutritional status (BMI) played only minor roles and did not significantly affect PA levels in our cohort.

A detailed comparison of our findings with the literature reveals both convergences and notable divergences regarding the determinants of PA in adult pwCF. Consistent with established research, our data corroborate the positive association between pulmonary function (ppFEV1) and engagement in higher-intensity physical activities (20, 21). This alignment suggests that preserved lung function remains a physiological prerequisite for vigorous exertion, likely due to the reduced ventilatory limitation and enhanced exercise capacity characteristic of higher ppFEV1 values (2). However, our study extends these observations by elucidating the psychological mechanisms underpinning this relationship. While previous studies have largely treated ppFEV1 as a direct correlate of PA behavior (3), our mediation analysis demonstrates that this association is not merely physiological but is substantially channeled through PAE. This finding suggests that the pathway from better lung function to higher activity levels is, to a significant extent, affectively mediated; individuals with superior lung function may derive greater enjoyment from strenuous activities, thereby reinforcing their participation. This affective mediation aligns with self-determination theory (SDT), which posits that intrinsic motivation - driven by enjoyment and perceived competence - is critical for sustained PA, especially in chronic illness populations (22, 23).

In contrast to the strong link between ppFEV1 and vigorous PA, the absence of a significant association between ppFEV1 and MPA in our cohort warrants discussion. This divergence from some prior reports, which have indicated correlations across all intensity levels, may reflect the unique behavioral patterns of the modern CF population, particularly in the context of effective CFTR-modulator therapies (24, 25). It is plausible that while reduced lung function imposes a ceiling on vigorous exertion, it does not necessarily preclude engagement in moderate-intensity activities. Consequently, the variance in MPA may be driven more by environmental or motivational factors rather than physiological capacity, a hypothesis supported by the lack of mediation by PAE in this specific intensity domain.

Furthermore, the identification of PAE as a full mediator for vigorous activities aligns with, yet theoretically advances, the findings of Denford et al. (7), who identified ‘fun’ and ‘joy’ as qualitative facilitators. Our study quantifies this concept, demonstrating that PAE is not merely a peripheral benefit but a central, independent determinant of PA behavior. This is particularly relevant when contrasted with studies in healthy populations, where the relationship between physiological capacity and PA is often more direct (26). In the context of chronic disease, where physical limitations are omnipresent, the affective response to exercise appears to gain prominence as a regulatory factor for engagement (27). The lack of influence from age and BMI observed in our study further distinguishes our cohort from general population data, where these demographic factors typically show stronger inverse correlations with PA levels (28). This suggests that in pwCF, the disease-specific physiological and psychological variables may overshadow the general age-related decline in activity often seen in healthy adults.

However, PAE and ppFEV1 together accounted for only 26–49.3% of the variance in PA intensity and extent, suggesting that other factors are also influential. Both our results and previous studies indicate that other psychological variables, such as self-efficacy, motivation, and symptoms of anxiety or depression, can substantially affect PA engagement in pwCF (10, 29, 30).

The introduction of highly effective CFTR modulator therapies (HEMT) has significantly altered the trajectory of the disease, yielding a multitude of benefits for individuals afflicted with this genetic disorder. Notably, these therapies have led to clinical stabilization, as evidenced by improvements in pulmonary function, nutritional status, and reduction in exacerbation rates (31). Furthermore, the enhanced quality of life (HrQoL) associated with these treatments has become a hallmark of CF care (32). The high proportion of pwCF receiving HEMT in our cohort (83%) likely played a significant role in the observed PA levels. While medication use was not included as a separate mediator in our analysis due to its strong correlation with lung function, we acknowledge that CFTR modulators act as a key facilitator by improving clinical stabilization and physical health. This pharmacological benefit may create the physiological prerequisite that allows patients to engage in and enjoy physical activity, thereby interacting with the psychological factors examined in this study.

However, as the disease's management has become increasingly effective, a shift in focus has occurred, with comorbidities such as overweight and obesity gaining prominence. The favorable conditions created by CF modulator therapies, which enable individuals to maintain or increase their PA, have inadvertently contributed to an increased risk of obesity (33, 34). Consequently, it is essential to address this emerging concern and develop targeted interventions to mitigate the risk of lifestyle-related diseases (35). As the physical limitations associated with CF recede, other factors influencing PA emerge. Self-efficacy, motivation, enjoyment, and environmental factors are increasingly recognized as crucial determinants of PA levels in individuals with CF (36). Therefore, a holistic approach to PA promotion becomes more important in future.

Our data underscore a distinct need for such enjoyment-focused strategies. With a mean PACES score of 65.4 ± 11.3, enjoyment levels in our cohort were only moderate, suggesting that current standard care does not fully exploit the potential of intrinsic motivation. Furthermore, given that our mediation analysis established PAE as a full mediator for vigorous PA, targeting these psychological barriers is not optional but essential. Interventions that leverage social engagement, supervision, and virtual elements are therefore directly supported by our finding that enhancing the affective response to exercise is a key mechanism to increase activity levels, independent of clinical status. To adapt strategies for enhancing PAE in pwCF, several key approaches warrant consideration. Leveraging social engagement and group dynamics is essential, as social interaction significantly enhances enjoyment by fostering accountability, camaraderie, and a sense of belonging (37).

Guidance and organized assistance can significantly contribute to improving PAE (38). Supervised exercise programs have been particularly successful in enhancing outcomes for pwCF, as they offer a secure and supportive setting in which pwCF can participate in regular PA under the supervision of trained healthcare professionals (39, 40). These programs provide numerous benefits, including better lung function, increased strength and endurance, fewer respiratory symptoms, improved mental health, such as reduced anxiety and depression, and better adherence to exercise, which is essential for sustaining these advantages (41). One major benefit of supervision is the promotion of positive social interaction. When individuals engage in PA with the support and encouragement of a supervisor, they are more likely to feel a sense of community and belonging. Another significant advantage of supervision is personalized instruction. When individuals receive customized guidance and feedback from a supervisor, they tend to feel more confident and capable in their PA. This can result in increased PAE and a lower risk of injury, as individuals can learn and refine their techniques in a safe, supportive environment. In addition to the benefits of positive social interaction and personalized instruction, supervision can help reduce anxiety related to trying new activities or pushing physical limits.

A key strength of this study lies in the application of mediation analysis, which offers distinct advantages over simple correlation approaches. While standard correlations can confirm that lung function is associated with PA, they do not elucidate the mechanisms driving this relationship. By employing mediation analysis, we were able to decompose the effect of ppFEV1 on PA into direct and indirect components. This allowed us to demonstrate that the influence of lung function on vigorous activity is largely transmitted through the psychological pathway of enjoyment, providing deeper insight into the behavioral drivers in adults with CF than associative measures alone could offer. Virtual group workouts, such as online cycling classes or fitness challenges, have gained popularity, offering flexibility while maintaining social connections (42). Digital tools and gamification elements such as rewards and progress tracking can make PA more engaging (43, 44). Virtual reality (VR) workouts, such as immersive cycling or dance games, have also shown promise in enhancing enjoyment by making exercise feel like a game (45). Monotony is a common barrier to enjoyment, and introducing variety, such as alternating between cardio, strength training, and flexibility exercises, can sustain interest (46). Emphasizing the immediate non-physical benefits of PA, such as stress relief, mental clarity, and emotional well-being, can enhance intrinsic motivation (47).

This study has several limitations. First, reliance on self-reported measures introduces potential recall and social desirability biases; more objective measures, such as accelerometry, would strengthen future work. Second, recruitment from a single center may limit generalizability; thus, multicenter or international samples are recommended for future studies. Third, although we included several relevant covariates, unmeasured factors such as specific psychological traits, the extent of social support, and environmental considerations may have impacted our findings. Because PAE and ppFEV1 explained only a modest proportion of the variance in PA, it is evident that additional, as yet unexamined, factors contribute to PA levels in this group.

Conclusions

In conclusion, both physiological (ppFEV1) and psychological (PAE) factors play significant roles in shaping PA among adult pwCF. Beyond traditional clinical management, strategies that enhance PAE may be crucial for promoting sustained engagement. The limited explanatory power of ppFEV1 and PAE underscores the necessity of investigating additional psychological and environmental factors that influence PAE in pwCF. Future research should further examine these factors and develop comprehensive approaches to support active lifestyles in this unique population.

Acknowledgments

The authors would like to thank Martin Bullmann, Stefanie Flakowski, Conny Linde, and Kiara Dütsch for collecting and distributing the questionnaires and for encouraging pwCF to participate in the study. The authors would also like to thank the pwCF who participated in the study and completed the questionnaires.

Funding Statement

The author(s) declared that financial support was not received for this work and/or its publication.

Footnotes

Edited by: Owen William Tomlinson, University of Exeter, United Kingdom

Reviewed by: Cigdem Emirza Cilbir, Health Institutes of Türkiye, Türkiye

Emma Powell, Newman University, United Kingdom

Data availability statement

The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.

Ethics statement

The studies involving humans were approved by Ethics Committee of the University Hospital Essen (BO-24-11951). The studies were conducted in accordance with the local legislation and institutional requirements. The participants provided their written informed consent to participate in this study.

Author contributions

MW: Formal analysis, Writing – original draft, Project administration, Validation, Data curation, Conceptualization. JS: Data curation, Writing – review & editing. JO: Writing – review & editing, Data curation. LL: Data curation, Writing – review & editing. CT: Writing – review & editing, Project administration. FS: Writing – original draft, Project administration, Conceptualization. WG: Writing – original draft, Formal analysis, Conceptualization, Project administration.

Conflict of interest

The author(s) declared that this work was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Generative AI statement

The author(s) declared that generative AI was used in the creation of this manuscript.

During the preparation of this manuscript, the authors used ChatGPT (version 5.1) for proofreading and wording. The authors have reviewed and edited the output and take full responsibility for the content of this publication.

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Publisher's note

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Supplementary material

The Supplementary Material for this article can be found online at: https://www.frontiersin.org/articles/10.3389/fspor.2026.1786911/full#supplementary-material

Table1.docx (25.4KB, docx)
Table2.docx (25.7KB, docx)

References

  • 1.Puppo H, Torres-Castro R, Vasconcello-Castillo L, Acosta-Dighero R, Sepúlveda-Cáceres N, Quiroga-Marabolí P, et al. Physical activity in children and adolescents with cystic fibrosis: a systematic review and meta-analysis. Pediatr Pulmonol. (2020) 55:2863–76. 10.1002/ppul.25038 [DOI] [PubMed] [Google Scholar]
  • 2.Hebestreit H, Hulzebos EHJ, Schneiderman JE, Karila C, Boas SR, Kriemler S, et al. Cardiopulmonary exercise testing provides additional prognostic information in cystic fibrosis. Am J Respir Crit Care Med. (2019) 199(8):987–95. 10.1164/rccm.201806-1110OC [DOI] [PubMed] [Google Scholar]
  • 3.Kinaupenne M, de Craemer M, Schaballie H, Vandekerckhove K, van Biervliet S, Demeyer H. Physical activity and its correlates in people with cystic fibrosis: a systematic review. Eur Respir Rev. (2022) 31(165):220010. 10.1183/16000617.0010-2022 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 4.Kinaupenne M, van Biervliet S, van Hoorenbeeck K, Schaballie H, Vandekerckhove K, Demeyer H, et al. Lower physical activity levels in youth with cystic fibrosis compared to healthy controls: a multicentre comparative study. Respir Med. (2024) 232:107749. 10.1016/j.rmed.2024.107749 [DOI] [PubMed] [Google Scholar]
  • 5.Bull FC, Al-Ansari SS, Biddle S, Borodulin K, Buman MP, Cardon G, et al. World Health Organization 2020 guidelines on physical activity and sedentary behaviour. Br J Sports Med. (2020) 54(24):1451–62. 10.1136/bjsports-2020-102955 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 6.Scully KJ, Jay LT, Freedman S, Sawicki GS, Uluer A, Finkelstein JS, et al. The relationship between body composition, dietary intake, physical activity, and pulmonary status in adolescents and adults with cystic fibrosis. Nutrients. (2022) 14(2):310. 10.3390/nu14020310 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 7.Denford S, van Beurden S, 'Halloran O, Williams P, A C. Barriers and facilitators to physical activity among children, adolescents, and young adults with cystic fibrosis: a systematic review and thematic synthesis of qualitative research. BMJ Open. (2020) 10(2):e035261. 10.1136/bmjopen-2019-035261 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 8.Ladune R, Filleul V, Falzon C, Hayotte M, Mély L, Vallier J-M, et al. Perceptions of barriers to and facilitators of physical activity in adults with cystic fibrosis. Physiother Theory Pract. (2021):1–11. 10.1080/09593985.2021.2005201 [DOI] [PubMed] [Google Scholar]
  • 9.Dillenhoefer S, Stehling F, Welsner M, Schlegtendal A, Sutharsan S, Olivier M, et al. Barriers for sports and exercise participation and corresponding barrier management in cystic fibrosis. Int J Environ Res Public Health. (2022) 19(20):13150. 10.3390/ijerph192013150 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 10.Hurley N, Moyna NM, Kehoe B, McCaffrey N, Redmond K, Hardcastle SJ. Factors influencing physical activity in adults with cystic fibrosis. BMC Pulm Med. (2021) 21(1):113. 10.1186/s12890-021-01482-x [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 11.Caspersen CJ, Powell KE, Christenson GM. Physical activity, exercise, and physical fitness: definitions and distinctions for health-related research. Public Health Rep. (1985) 100(2):126–31. [PMC free article] [PubMed] [Google Scholar]
  • 12.Leisterer S, Gramlich L. Having a positive relationship to physical activity: basic psychological need satisfaction and age as predictors for students’ enjoyment in physical education. Sports (Basel). (2021) 9(7). 10.3390/sports9070090 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 13.Jekauc D, Nigg C, Nigg CR, Reichert M, Krell-Roesch J, Oriwol D, et al. Measurement properties of the German version of the physical activity enjoyment scale for adults. PLoS One. (2020) 15(11):e0242069. 10.1371/journal.pone.0242069 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 14.Jekauc D, Voelkle M, Wagner MO, Mewes N, Woll A. Reliability, validity, and measurement invariance of the German version of the physical activity enjoyment scale. J Pediatr Psychol. (2013) 38(1):104–15. 10.1093/jpepsy/jss088 [DOI] [PubMed] [Google Scholar]
  • 15.Sallis JF, Haskell WL, Wood PD, Fortmann SP, Rogers T, Blair SN, et al. Physical activity assessment methodology in the five-city project. Am J Epidemiol. (1985) 121(1):91–106. 10.1093/oxfordjournals.aje.a113987 [DOI] [PubMed] [Google Scholar]
  • 16.Ruf KC, Fehn S, Bachmann M, Moeller A, Roth K, Kriemler S, et al. Validation of activity questionnaires in patients with cystic fibrosis by accelerometry and cycle ergometry. BMC Med Res Methodol. (2012) 12:43. 10.1186/1471-2288-12-43 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 17.Hayden-Wade HA, Coleman KJ, Sallis JF, Armstrong C. Validation of the telephone and in-person interview versions of the 7-day PAR. Med Sci Sports Exerc. (2003) 35(5):801–9. 10.1249/01.MSS.0000064941.43869.4E [DOI] [PubMed] [Google Scholar]
  • 18.Sloane R, Snyder DC, Demark-Wahnefried W, Lobach D, Kraus WE. Comparing the 7-day physical activity recall with a triaxial accelerometer for measuring time in exercise. Med Sci Sports Exerc. (2009) 41(6):1334–40. 10.1249/MSS.0b013e3181984fa8 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 19.The jamovi project (2025). 2.6 [Computer Software]. Available from: Available online at: https://www.jamovi.org/about.html (cited July 25, 2025).
  • 20.Cherobin IA, Dalcin P, Ziegler B. Association between lung function, physical activity level and postural evaluation variables in adult patients with cystic fibrosis. Clin Respir J. (2018) 12(4):1510–7. 10.1111/crj.12698 [DOI] [PubMed] [Google Scholar]
  • 21.Curran M, Tierney AC, Button B, Collins L, Kennedy L, McDonnell C, et al. Physical activity and sedentary behavior in adults with cystic fibrosis: association with aerobic capacity, lung function, sleep, well-being, and quality of life. Respir Care. (2022) 67(3):339–46. 10.4187/respcare.09270 [DOI] [PubMed] [Google Scholar]
  • 22.Teixeira PJ, Carraca EV, Markland D, Silva MN, Ryan RM. Exercise, physical activity, and self-determination theory: a systematic review. Int J Behav Nutr Phys Act. (2012) 9:78. 10.1186/1479-5868-9-78 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 23.Xu Z, Shamsulariffin S, Azhar Y, Xi M. Does self-determination theory associate with physical activity? A systematic review of systematic review. Int J Psychol. (2025) 60(3):e70044. [DOI] [PubMed] [Google Scholar]
  • 24.Savi D, Di Paolo M, Simmonds N, Onorati P, Internullo M, Quattrucci S, et al. Relationship between daily physical activity and aerobic fitness in adults with cystic fibrosis. BMC Pulm Med. (2015) 15:59. 10.1186/s12890-015-0036-9 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 25.Hebestreit H, Kieser S, Rüdiger S, Schenk T, Junge S, Hebestreit A, et al. Physical activity is independently related to aerobic capacity in cystic fibrosis. Eur Respir J. (2006) 28(4):734–9. 10.1183/09031936.06.00128605 [DOI] [PubMed] [Google Scholar]
  • 26.Laudani L, Vannozzi G, Sawacha Z, della Croce U, Cereatti A, Macaluso A. Association between physical activity levels and physiological factors underlying mobility in young, middle-aged and older individuals living in a city district. PLoS One. (2013) 8(9):e74227. 10.1371/journal.pone.0074227 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 27.Conroy DE, Berry TR. Automatic affective evaluations of physical activity. Exerc Sport Sci Rev. (2017) 45(4):230–7. 10.1249/JES.0000000000000120 [DOI] [PubMed] [Google Scholar]
  • 28.Engst S, Fangrat K, Lane H, Lombardo M. Lifestyle, age, and heart disease evidence from European datasets. Healthcare (Basel). (2025) 13(10):1123. 10.3390/healthcare13101123 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 29.Burnett DM, Barry AN, Mermis JD. Physical activity level and perception of exercise in cystic fibrosis. Respir Care. (2020) 65(4):500–6. 10.4187/respcare.07193 [DOI] [PubMed] [Google Scholar]
  • 30.Gruber W, Stehling F, Schermaul JS, Ortiz JG, Lechtenberg L, Taube C, et al. Predictors of physical activity enjoyment in adults with cystic fibrosis: the role of quality of life and motivation-a single-center study. Healthcare (Basel). (2025) 13(17). 10.3390/healthcare13172194 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 31.Sutharsan S, Dillenhoefer S, Welsner M, Stehling F, Brinkmann F, Burkhart M, et al. Impact of elexacaftor/tezacaftor/ivacaftor on lung function, nutritional status, pulmonary exacerbation frequency and sweat chloride in people with cystic fibrosis: real-world evidence from the German CF registry. Lancet Reg Health Eur. (2023) 32:100690. 10.1016/j.lanepe.2023.100690 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 32.Fajac I, Daines C, Durieu I, Goralski JL, Heijerman H, Knoop C, et al. Non-respiratory health-related quality of life in people with cystic fibrosis receiving elexacaftor/tezacaftor/ivacaftor. J Cyst Fibros. (2022). [DOI] [PubMed] [Google Scholar]
  • 33.Szentpetery S, Fernandez GS, Schechter MS, Jain R, Flume PA, Fink AK. Obesity in cystic fibrosis: prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry. J Cyst Fibros. (2022) 21(5):777–83. 10.1016/j.jcf.2022.03.010 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 34.Salvatore D, Padoan R, Amato A, Salvatore M, Campagna G, On Behalf Of The Italian Cf Registry Working G. Nutritional trends in cystic fibrosis: insights from the Italian cystic fibrosis patient registry. J Clin Med. (2024) 13(13):3652. 10.3390/jcm13133652 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 35.Ratti GA, Smith H, Mirfakhraee S, Reisch J, Cohen L, Jain R, et al. Development of metabolic syndrome in people with cystic fibrosis one year after exposure to elexacaftor-tezacaftor-ivacaftor. J Cyst Fibros. (2025) 24(1):47–52. 10.1016/j.jcf.2024.09.022 [DOI] [PubMed] [Google Scholar]
  • 36.Berthold A, Barr E, Kasi A, Lichten L, Hunt WR. Perception and participation in sport and exercise in cystic fibrosis: the impact of CFTR modulators. Respir Med. (2024) 235:107840. 10.1016/j.rmed.2024.107840 [DOI] [PubMed] [Google Scholar]
  • 37.Eime RM, Young JA, Harvey JT, Charity MJ, Payne WR. A systematic review of the psychological and social benefits of participation in sport for adults: informing development of a conceptual model of health through sport. Int J Behav Nutr Phys Act. (2013) 10:135. 10.1186/1479-5868-10-135 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 38.Curran M, Tierney AC, Button B, Collins L, Kennedy L, McDonnell C, et al. The effectiveness of exercise interventions to increase physical activity in cystic fibrosis: a systematic review. J Cyst Fibros. (2022) 21(2):272–81. 10.1016/j.jcf.2021.10.008 [DOI] [PubMed] [Google Scholar]
  • 39.Hebestreit H, Kriemler S, Schindler C, Stein L, Karila C, Urquhart DS, et al. Effects of a partially supervised conditioning program in cystic fibrosis: an international multicenter randomized controlled trial (ACTIVATE-CF). Am J Respir Crit Care Med. (2022) 205(3):330–9. 10.1164/rccm.202106-1419OC [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 40.Welsner M, Gruber W, Mellies U, Olivier M, Sutharsan S, Taube C, et al. Trainability of health-related and motor performance fitness in adults with cystic fibrosis within a 12-month partially supervised exercise program. Pulm Med. (2021) 2021:5581812. 10.1155/2021/5581812 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 41.Sieniawska J, Kamizela A, Madoń M, Proszowska P, Sieniawska D. The benefits of physical activity for patients with cystic fibrosis: a literature review. Quality in Sport. (2024) 18:53457. 10.12775/QS.2024.18.53457 [DOI] [Google Scholar]
  • 42.Liu R, Menhas R, Dai J, Saqib ZA, Peng X. Fitness apps, live streaming workout classes, and virtual reality fitness for physical activity during the COVID-19 lockdown: an empirical study. Front Public Health. (2022) 10:852311. 10.3389/fpubh.2022.852311 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 43.Mazeas A, Forestier C, Harel G, Duclos M, Chalabaev A. The impact of a gamified intervention on daily steps in real-life conditions: retrospective analysis of 4800 individuals. J Med Internet Res. (2024) 26:e47116. 10.2196/47116 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 44.Xu L, Shi H, Shen M, Ni Y, Zhang X, Pang Y, et al. The effects of mHealth-based gamification interventions on participation in physical activity: systematic review. JMIR Mhealth Uhealth. (2022) 10(2):e27794. 10.2196/27794 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 45.Hajder D, Bjelica B, Bubanj S, Aksovic N, Markovic M, Arsenijevic R, et al. A systematic review and meta-analysis of virtual and traditional physical activity programs: effects on physical, health, and cognitive outcomes. Healthcare (Basel). (2025) 13(7):711. 10.3390/healthcare13070711 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 46.Juvancic-Heltzel JA, Glickman EL, Barkley JE. The effect of variety on physical activity: a cross-sectional study. J Strength Cond Res. (2013) 27(1):244–51. 10.1519/JSC.0b013e3182518010 [DOI] [PubMed] [Google Scholar]
  • 47.Martin-Rodriguez A, Gostian-Ropotin LA, Beltran-Velasco AI, Belando-Pedreno N, Simon JA, Lopez-Mora C, et al. Sporting mind: the interplay of physical activity and psychological health. Sports (Basel). (2024) 12(1):37. 10.3390/sports12010037 [DOI] [PMC free article] [PubMed] [Google Scholar]

Associated Data

This section collects any data citations, data availability statements, or supplementary materials included in this article.

Supplementary Materials

Table1.docx (25.4KB, docx)
Table2.docx (25.7KB, docx)

Data Availability Statement

The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.


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