Given the clinical importance of aerobic fitness in people with CF, incorporating S is recommended to provide a safe and valid measure of V̇O in children, adolescents and adults who span the spectrum of CF disease severity.

Background: The development of cystic fibrosis (CF)-related diabetes (CFRD) in paediatric groups is associated with a reduced aerobic fitness. However, this has yet to be investigated in adults with more severe lung disease. Methods: Cardiopulmonary exercise and glycaemic control tests were retrospectively analysed in 46 adults with CF (age: 26.9 y [range: 16.3-66.5 y]; forced expiratory volume in 1s: 65.3% [range: 26.8-105.7%]; 26 males), diagnosed with CFRD (n = 19), impaired glucose tolerance (IGT; n = 8) or normal glucose tolerance (NGT; n = 19). Results: Maximal oxygen uptake (V̇O2max) was reduced in adults with IGT and CFRD compared to their age-and gender-matched counterparts with NGT (p < 0.05); however, there was no difference when lung function was included as a covariate (all p > 0.05). V̇O2max was greater in adults who experienced post-reactive hypoglycaemia vs. NGT without hypoglycaemia (p < 0.05). The frequency of ventilatory limitation (84%, 63% and 37%, respectively; p < 0.05) but not ventilation-perfusion mismatch (42%, 38% and 16%, respectively; p > 0.05), was greater with CFRD and IGT vs. NGT. There was also no difference in arterial oxygen saturation changes between groups (p > 0.05). Gender and body mass index were significant predictors of V̇O2max (adjusted R 2 = 0.37, p < 0.01), but glycaemic control did not explain additional variance (p > 0.05). Conclusions: Adults with CF-related dysglycaemia had a reduced V̇O2max compared to age-and gender-matched counterparts, due to a greater degree of CF lung disease in these populations.

Methods: Three adolescents (14.0 ± 1.4 years) with CF (FEV 1 % predicted: 62.5 ± 17.1; F508del/F508del genotype) completed an exhaustive maximal cardiopulmonary exercise test on a cycle ergometer to determine peak oxygen uptake ( VO 2peak ) and measure changes in gas exchange and ventilation during exercise at 6 weeks. We also analyzed wrist-worn device-based physical activity (PA) data in two of the three cases. Validated acceleration thresholds were used to quantify time spent in each PA intensity category.Results: Clinically meaningful improvements in VO 2peak were observed in all three cases (+17.6%, +52.4%, and +32.9%, respectively), with improvements greatest in those with more severe lung disease and lower fitness at baseline. Although lung function increased in all cases, inconsistent changes in markers of ventilatory and peripheral muscle efficiency likely suggest different mechanisms of improvement in this case group of adolescents with CF. Device-based analysis of PA was variable, with one case increasing and one case decreasing. Conclusion:In this case series, we have observed, for the first time, improvements in exercise capacity following 6 weeks of treatment with Elexacaftor/Tezacaftor/ Ivacaftor. Improvements were greatest in the presence of more severe CF lung disease and lower aerobic fitness at baseline. The mechanism(s) responsible for these changes warrant further investigation in larger trials.

Identification and recognition of experiences of rural pregnant women with substance use disorder is needed to inform public policy and medical training. This paper reviews and qualitatively synthesizes literature exploring the experiences of this population. Embase, PubMed, and Web of Science were used to identify literature through August 2022 using the search terms, such as pregnancy, substance use or abuse, stigma, and rural. Cited and citing research were also considered. Exclusion criteria included articles that failed to consider rural pregnant women’s perspectives on stigma experienced, included potential confounds, occurred outside of the United States or Canada, and were published before January 2000. Nine articles met the inclusion criteria. Data were synthesized by the listed authors and assessed for common themes. A review of the articles revealed three common themes: stigma occurs in community settings, stigma occurs in healthcare settings, and comprehensive care is important to ensure appropriate support to this population. Stigma as a barrier seems to improve when women have strong social support and access to comprehensive care networks. Addressing this stigma through programs, such as peer social guidance and comprehensive health centers, may provide appropriate support to pregnant, rural women with SUD to further navigate their health needs.

This study investigated whether Elexacaftor-Tezacaftor-Ivacaftor (Kaftrio ), a cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulator, could improve exercise capacity in adolescents with CF. After six weeks treatment, Kaftrio improved both maximal and submaximal indices of aerobic fitness. Improvements were independent of changes in ventilatory function during exercise and physical activity. Interestingly, pulmonary oxygen uptake per unit of power output was higher in two, out of three, cases who presented with more severe CF lung disease. These findings suggest improved O extraction and/or consumption in the exercising muscle and demonstrate, for the first time, that short-term treatment with Kaftrio might improve aerobic fitness in people with CF, especially in those with more severe lung disease and deconditioning.

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