Physical fitness and habitual physical activity in children with cystic fibrosis - do they improve with elexacaftor/tezacaftor/ivacaftor therapy?

Objectives: The aim of this ongoing longitudinal study is to examine the effects of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) on aspects of physical fitness (PF, strength endurance, explosive power, coordination under time pressure, coordination for the precision aspect, trunk flexibility, peak workload, habitual physical activity (HPA, steps/day, intensity), and zscores of FEV1 and BMI in children with cystic fibrosis (CF)). All children have been receiving ELX/TEZ/IVA for 19.2 ± 10.4 (11–33) months. Methods: Six children with CF agreed to participate (3f/3 m) mean age 13 ± 4yrs (9–17 yrs), FEV1 z-score − 1.017 ± 1.817 (-3.2–0.8). PFwas assessed using 5 test items at baseline and 4.51 ± 0.98 years later;strength endurance (PU push-ups, SU sit-ups), explosive power (SLJ standing long jump), coordination under time pressure (JSS jumping side to side), coordination for the precision aspect (BB balancing backward on beams), and trunk flexibility (FB, forward bend). Cycle-ergometry (Godfrey-protocol) was used to determine Wpeak. Lung function was measured by spirometry. Results: Significant improvements were seen in test items representing strength endurance (PU, SU) and coordination under time pressure (JSS) (p < .05). Although HPA expressed as steps/day remained the same, thetime of moderate to vigorous intensity of HPA decreases significantly (p < .05). A slight, not significant, improvement of BMI Z-score was observed (p > .05). No or small changes could be observed for the parameter FEV1 z-score, Wpeak, PF, SLJ, BB and FB (p > 0.05). Conclusion: ELX/TEZ/IVA therapy seems to be a facilitator to increase some aspects of PF. The decrease in intensity of HPA is possibly an effect of the COVID-19 Pandemic that has already been described in healthy and asthmatic children. In this ongoing study, we assume that clearer effects will be shown with a greater number of children included. However, ELX/ TEZ/IVA enhanced muscle strength endurance in children with CF.

Effects of face masks on physical performance, physiological response and subjective respiratory effort during a submaximal bicycle ergometer test

Introduction: Evidence on undesirable side effects of face masks worn during the COVID-19 pandemic is controversial. Materials and Methods: The present study, explores whether wearing a medical face mask (MedMask) affects physical working capacity (PWC) at the heart rate of 130 and 150 beats per minute in comparison to no mask, a filtering face piece mask with exhalation valve class 2 (FFP2exhal), and a cotton fibric mask (community mask). Secondary, physiological and subjective responses were analyzed such as a potential moderating role of subjects’ individual physical fitness level and gender on face mask effects. A submaximal bicycle ergometer protocol was applied in an intra-individual cross-over design using either no mask, a MedMask, FFP2exhal, or a community mask on four days in randomized order. PWC130 and PWC150 were measured as well as transcutaneous carbon dioxide partial pressure, oxygen saturation, breathing rate, blood pressure, perceived respiratory effort and perceived physical exhaustion. Results: Using the MedMask did not lead to a reduction in PWC and a systematic or relevant change in physiological response, neither was this the case when the FFP2exhal or community mask were worn. Perceived respiratory effort was up to one point higher on a zero to ten scale when using face masks p<0.05) compared to the no mask condition. No differences occured in general perceived exertion. Conclusion: These results provide reason to believe that physical performance and physiological responses when wearing face masks are similar to not wearing a face mask, although some more respiratory effort is required.