Barriers for Sports and Exercise Participation and Corresponding Barrier Management in Cystic Fibrosis

Background Nowadays physical activity (PA)/exercise is an important component of cystic fibrosis (CF) therapy. The aim of the study was to assess the barriers to PA and the barrier management and to explore the effect of supervision on the barriers and barrier management during an exercise program. Methods In total, 88 people with CF (pwCF) of the ages 6 to 50 years old (mean 24.2 ± 7.9 yrs) participated in the partially supervised 12-month exercise program and filled in a structured and validated questionnaire about barriers to sports and barrier management at baseline. Results Physical barriers were more relevant than psychosocial barriers and no trend could be seen in the situational and preventive counter strategies. When divided in subgroups, the less active pwCF (7500 steps/day), physical barriers, and psychosocial barriers showed no significant differences. However physical barriers showed a tendency to have a higher value in the less active group compared to the more active group (p > 0.05). Stratified by age or FEV1%pred between the subgroups, no differences could be seen regarding barriers and counter strategies. Conclusions Physical barriers seemed to have a higher priority when it comes to not participating in PA/exercise. Supervision over 6 months during an exercise program did not show a beneficial effect on barriers and barrier management. Besides the motivational aspect of sport counselling, the volitional aspect seemed to be more important to incorporate more PA into daily life. Individual barriers and their concrete counter strategies should be discussed with the patient with CF. Sport counselling is needed permanently and should be part of the CF routine care

How personality influences health outcomes and quality of life in adult patients with cystic fibrosis

Abstract Background The present study evaluates personality traits in adult patients with cystic fibrosis (CF) and correlates these results with health-related quality of life (HRQoL) and other clinical parameters indicative of disease severity. Methods Seventy adults completed the Cystic Fibrosis Questionnaire-Revised (CFQ-R 14+), a CF-specific measure of HRQoL, and a self-administered questionnaire about personality traits and disorders. Mean subscale scores and the prevalence of extreme personality traits on the `Persönlichkeits-Stil- und Störungs-Inventar (PSSI)´ were compared to the norming sample. Moreover, a cluster analysis was conducted to identify personality styles among people with cystic fibrosis (pwCF). The relationship between mean PSSI subscale scores and personality clusters with HRQoL and clinical outcomes, e.g., percent predicted forced expiratory volume in one second (ppFEV1), and body mass index (BMI), was studied by regression analysis considering important confounders. Results On several of the subscales of the personality questionnaire, people with cystic fibrosis (pwCF) showed either significantly higher or lower scores than the norm sample. In further analyses, two personality clusters could be identified. PwCF from the cluster with predominantly low scores on the subscales ‘negativistic’, ‘schizoid’, ‘borderline’, ‘depressed’, and ‘paranoid’ showed better HRQoL than pwCF from the other cluster with mainly high normal or elevated scores. The studied health outcomes proved to be independent of the respective personality clusters. Conclusions In pwCF, HRQoL is mainly determined by psychological factors, including personality. Since more recent personality theories assume that personality is modifiable, our findings imply that patients with accentuated personality traits may benefit from psychosocial support

Effects of a Long-Term Monitored Exercise Program on Aerobic Fitness in a Small Group of Children with Cystic Fibrosis

Background The aim of this study was to investigate the effects of a monitored exercise program on aerobic fitness in children with cystic fibrosis (CF). Methods Six children (2f/4m) with ages ranging from 6 to 14 years (11.3 ± 3.3 years.) and a mean ppFEV1 102.5 ± 13.5% pred. participated in the partially monitored 12-month exercise program. VO2peak and Wpeak were used as parameters of aerobic fitness. Incremental Cardio-Pulmonary Exercise Tests (CPETs) were performed before the program began (T1), after 6 months (T3) of monitoring, and after a further 6 months (T4) without monitoring. Habitual physical activity (HPA) was assessed with accelerometry. Results The values of VO2peak and Wpeak improved slightly from T1 to T3 (p > 0.05), without a further increase after monitoring was stopped (T4). However, the VO2peak and Wpeak values were higher after monitoring was stopped compared to at T1. The exercise program with and without monitoring (p > 0.05) had no or only a slight effect on the FEV1 values, steps/day, and the intensity of HPA. Conclusions Monitoring seems to facilitate the achievement of beneficial effects on physical fitness in CF children. For that reason, continuous individual exercise monitoring programs that involve close contact with an exercise therapist should be provided to maintain long-term motivation and participation in physical activities and sport activities during leisure time

Real-life impact of highly effective CFTR modulator therapy in children with cystic fibrosis

Introduction: Recently, cystic fibrosis transmembrane regulator modulator therapy with elexacaftor/tezacaftor/ivacaftor has become available for children with cystic fibrosis (CF) carrying at least one F508del mutation.Objective: To assess the intermediate term effects of elexacaftor/tezacaftor/ivacaftor in children with cystic fibrosis in a real-world setting.Methods: We performed a retrospective analysis of records of children with cystic fibrosis, who started elexacaftor/tezacaftor/ivacaftor between 8/2020 and 10/2022. Pulmonary function tests, nutritional status, sweat chloride and laboratory data were assessed before, 3 and 6 months after the start of elexacaftor/tezacaftor/ivacaftor respectively.Results: Elexacaftor/tezacaftor/ivacaftor was started in 22 children 6–11 years and in 24 children 12–17 years. Twenty-seven (59%) patients were homozygous for F508del (F/F) and 23 (50%) patients were transitioned from ivacaftor/lumacaftor (IVA/LUM) or tezacaftor/ivacaftor (TEZ/IVA) to elexacaftor/tezacaftor/ivacaftor. Overall, mean sweat chloride concentration decreased by 59.3 mmol/L (95% confidence interval: −65.0 to −53.7 mmol/L, p < 0.0001) under elexacaftor/tezacaftor/ivacaftor. Sweat chloride concentration also decreased significantly after transition from IVA/LUM or TEZ/IVA to elexacaftor/tezacaftor/ivacaftor (−47.8 mmol/l; 95% confidence interval: −57.6 to −37.8 mmol/l, n = 14, p < 0.0001). Sweat chloride reduction was more marked in children with the F/F than in those with the F/MF genotype (69.4 vs 45.9 mmol/L, p < 0.0001). At 3 months follow-up, body-mass-index-z-score increased by 0.31 (95% CI, 0.2–0.42, p < 0.0001) with no further increase at 6 months. BMI-for-age-z-score was more markedly improved in the older group. Overall pulmonary function (percent predicted FEV1) at 3 months follow-up increased by 11.4% (95% CI: 8.0–14.9, p < 0.0001) with no further significant change after 6 months. No significant differences were noted between the age groups. Children with the F/MF genotype had a greater benefit regarding nutritional status and pulmonary function tests than those with the F/F genotype. Adverse events led to elexacaftor/tezacaftor/ivacaftor dose reduction in three cases and a temporary interruption of therapy in four cases.Conclusion: In a real-world setting, elexacaftor/tezacaftor/ivacaftor therapy had beneficial clinical effects and a good safety profile in eligible children with cystic fibrosis comparable to previously published data from controlled clinical trials. The positive impact on pulmonary function tests and nutritional status seen after 3 months of elexacaftor/tezacaftor/ivacaftor therapy was sustained at 6 months follow-up

Trainability of Health-Related and Motor Performance Fitness in Adults with Cystic Fibrosis within a 12-Month Partially Supervised Exercise Program

Background. Regular physical activity plays an important role in the treatment of patients with cystic fibrosis (CF). This study is aimed at investigating the effects of a 12-month partially supervised exercise program on attributes of health-related and motor performance fitness, lung function (ppFEV1), BMI, and habitual physical activity (HPA, steps/day) in adults with CF. Methods. Attributes of health-related and motor performance fitness were examined at the beginning (T0), after 6 (T1), and 12 months (T2) on the basis of five test items: forward bend (FB), bent knee hip extension (HE), plank leg raise (PLR), standing long jump (SLJ), and standing on one leg (OLS). Additionally, we recorded HPA by accelerometry, peak exercise performance (Wpeak) by an incremental cycle test, ppFEV1, and BMI. During the first six months, there was close supervision by an experienced sport therapist. Results. 26 CF patients (8 female, mean age 26.5±7.9 years; ppFEV1 53.7±21.0) completed the exercise program. Significant improvements were recorded from T0 to T1 (FB: p≤0.05; PLR, OLS: p≤0.01) and from T0 to T2 (FB, PLR: p≤0.01 and HE, OLS: p≤0.05). Wpeak, ppFEV1, BMI, and HPA showed no significant improvement between the single test points and over the entire study period (all p>0.05). Conclusion. Our results show trainability of adults with CF in aspects of health-related and motor performance fitness during a partially supervised exercise program. Close supervision positively influences the results. Using a simple test setup seems to be a promising tool for evaluating the effects of exercise programs in CF and could serve as an additional outcome parameter in future clinical trials. Trial registration: ClinicalTrials.gov (retrospectively registered May 8, 2018)

Proof of principle study: diagnostic accuracy of a novel algorithm for the estimation of sleep stages and disease severity in patients with sleep-disordered breathing based on actigraphy and respiratory inductance plethysmography

Purpose!#!In this proof of principle study, we evaluated the diagnostic accuracy of the novel Nox BodySleep!##!Methods!#!Patients received PSG according to AASM. Sleep stages were manually scored using the AASM criteria and the recording was evaluated by the novel algorithm. The results were analyzed by descriptive statistics methods (IBM SPSS Statistics 25.0).!##!Results!#!We found a strong Pearson correlation (r=0.91) with a bias of 0.2/h for AHI estimation as well as a good correlation (r=0.81) and an overestimation of 14 min for total sleep time (TST). Sleep efficiency (SE) was also valued with a good Pearson correlation (r=0.73) and an overestimation of 2.1%. Wake epochs were estimated with a sensitivity of 0.65 and a specificity of 0.59 while REM and non-REM (NREM) phases were evaluated a sensitivity of 0.72 and 0.74, respectively. Specificity was 0.74 for NREM and 0.68 for REM. Additionally, a Cohen's kappa of 0.62 was found for this 3-class classification problem.!##!Conclusion!#!The algorithm shows a moderate diagnostic accuracy for the estimation of sleep. In addition, the algorithm determines the AHI with good agreement with the manual scoring and it shows good diagnostic accuracy in estimating wake-sleep transition. The presented algorithm seems to be an appropriate tool to increase the diagnostic accuracy of portable monitoring. The validated diagnostic algorithm promises a more appropriate and cost-effective method if integrated in out-of-center (OOC) testing of patients with suspicion for SDB

Association between habitual physical activity (HPA) and sleep quality in patients with cystic fibrosis

Purpose!#!Sleep disturbances and poor sleep quality are known to be present in patients with CF. Regular physical activity plays an important role in the treatment of CF patients due to its positive influence on progression of disease and quality of life. The aim of this work is to create a home-based sleep and activity profile and to investigate the influence of habitual physical activity (HPA) on sleep quality in children, adolescents, and adults with CF.!##!Methods!#!A total of 109 CF patients (64 male, mean age 22.7 ± 12.0 years; mean ppFEV1 63.0 ± 26.7) were equipped with an actigraph for a home-based collection of data on sleep and activity over 4 weeks.!##!Results!#!Age, FEV1, and BMI affect sleep and activity in CF patients. Especially younger age and higher FEV1 show a great influence on certain aspects of sleep (SE, TST, TIB, WASO, # of awakenings) and activity and its different intensities. General HPA does not affect sleep, but there is a strong correlation between times spent in vigorous to very vigorous intensities and better sleep quality.!##!Conclusion!#!Besides younger age and higher FEV1, daily activity in higher intensities influences sleeping behavior of CF patients in a positive way. Patients with poor sleep quality and sleep disturbances possibly benefit from an intensification of physical activity in the home environment. TRAIL REGISTRATION: number: 14-6117-BO (University Duisburg-Essen) and NCT03518697 (clinical trials)

Obstructive sleep apnea and nocturnal hypoxemia in adult patients with cystic fibrosis

Abstract Background Obstructive sleep apnea (OSA), nocturnal hypoxemia and excessive daytime sleepiness (EDS) are common comorbidities in people with cystic fibrosis (pwCF). Most of the data showing this originates from children and adolescents. The aim of this study was to collect data on sleep parameters, EDS and pulmonary function from a large cohort of adult pwCF. Methods Full overnight polysomnography (PSG) was performed. EDS was determined using the Epworth Sleepiness Scale (ESS). Demographic and clinical data (body mass index [BMI], pulmonary function, capillary blood gases) were collected. Results A total of 52 adult pwCF were included (mean age 30.7 ± 8.0 years, mean percent predicted forced expiratory volume in 1 s [ppFEV1] of 52.1 ± 14.8). Overall AHI was in the normal range (4.5 ± 4.0/h); 21/52 pwCF (40%) had an apnea-hypopnea index > 5/h. Nocturnal hypoxemia was found in 25% of participants and this was associated with ppFEV1 (p = 0.014), awake oxygen saturation (SpO2; p = 0.021) and awake partial pressure of oxygen (pO2; p = 0.003); there were no significant differences in age, lung function and BMI were found for pwCF with versus without OSA (all p > 0.05). Eight pwCF (15%) had an ESS score > 10 (indicating EDS). OSA was best predicted by awake pO2 (area under the curve [AUC] 0.66, p = 0.048), while nocturnal hypoxemia was best predicted by ppFEV1 (AUC 0.74, p = 0.009), awake pO2 (AUC 0.76, p = 0.006) and awake SpO2 (AUC 0.71; p = 0.025). Conclusion OSA, nocturnal hypoxemia and EDS were common in adult pwCF, but no strong predictors were identified. Therefore, we suggest regular PSG and ESS scoring in adult pwCF, regardless of disease severity

Additional file 1 of Cough suppression and HRQoL in adult people with cystic fibrosis: an unexplored correlation

Additional file 1: Testing of the assumptions regarding the respective statistical procedure

Longitudinal changes in habitual physical activity in adult people with cystic fibrosis in the presence or absence of treatment with elexacaftor/tezacaftor/ivacaftor

BackgroundHabitual physical activity (PA) and exercise training are accepted as important aspects of care for people with cystic fibrosis (pwCF) to improve health-related measures of physical fitness, which in turn have a positive impact on quality of life and prognosis. In the last decade, effective CFTR modulator therapies have become a promising treatment for pwCF by targeting the underlying cause of CF. This highly effective therapy improves clinical outcomes and quality of life in people with specific CFTR mutations. Little is known about the longitudinal pattern of PA or the impact of the highly effective modulator therapy with Elexacaftor/Tezacaftor/Ivacaftor (ETI) on PA in adult pwCF. This study assessed the course of device-based PA measurement in adult pwCF and evaluated the effects of ETI on habitual physical activity in those who were eligible for ETI.MethodsData from adult pwCF (aged ≥18 years) were analysed at baseline and follow-up, using identical assessments at both time points. Outcome parameters were PA in steps/day and the intensity of PA. The group that received ETI was treated for an average of 33 weeks and not for the entire duration of the period. The data were collected between 2021 and 2022, following the removal of absolute pandemic restrictions/lockdowns.ResultsFollow-up duration was 5.6 years in pwCF with ETI (ETI group, n = 21) and 6.5 years in pwCF without ETI (non-ETI group, n = 6). From baseline to follow-up, pwCF treated with ETI had a significant increase in steps/day (+25%, p = 0.019) and a non-significant increase in moderate-to-vigorous intensity time (+5.6%, p = 0.352). Conversely, individuals in the non-ETI group showed a non-significant decrease in both steps/day −3.2%, p = 0.893) and moderate-to-vigorous intensity time (−25%, p = 0.207). The ETI group showed a significant decrease in percent predicted forced expiratory volume in 1 s (ppFEV1) and FEV1 z-score before the start of ETI treatment, both of which improved significantly after therapy initiation. Body weight and body mass index also improved significantly with ETI use.ConclusionsThese data suggest that ETI treatment has a positive effect on habitual physical activity behavior in the adult pwCF studied