Impact of Pulmonary Rehabilitation Services in Patients with Different Lung Diseases

Background: the effect of pulmonary rehabilitation (PR) services, beyond research contexts, on patients with lung diseases other than COPD requires further study. Objectives: to (i) assess the impact of a publicly funded PR on patients’ exercise capacity, self-efficacy, and health-related quality of life (HRQoL), and (ii) explore whether the effects vary across lung diseases. Methods: this retrospective pre–post study analyzed data from the Winnipeg Regional Health Authority PR program between 2016 and 2019. Results: 682 patients completed the full PR program. Pooled analyses found significant improvements in the patients’ exercise capacity (six-minute walk test (6MWT) (13.6%), fatigue (10.3%), and dyspnea (6.4%)), Self-Efficacy for Managing Chronic Disease 6-Item Scale (SEMCD6) (11.6%), and HRQoL (Clinical COPD Questionnaire (CCQ) (18.5%) and St George’s Respiratory Questionnaire (SGRQ) (10.9%)). The analyses conducted on sub-groups of patients with chronic obstructive pulmonary disease (COPD), asthma, bronchiectasis, interstitial lung diseases (ILDs), other restrictive lung diseases (e.g., obesity, pleural effusion, etc.), lung cancer, and pulmonary hypertension (PH) indicated that, except for patients with PH, all the patients improved in the 6MWT. Fatigue decreased in patients with COPD, ILDs, and other restrictive lung diseases. Dyspnea decreased in patients with COPD, asthma, and lung cancer. SEMCD6 scores increased in COPD, ILDs and PH patients. CCQ scores decreased in all lung diseases, except lung cancer and PH. SGRQ scores only decreased in patients with COPD. Conclusion: PR services had a significant impact on patients with different lung diseases. Therefore, publicly funded PR should be available as a critical component in the management of patients with these diseases.

Study Protocol of the Exercise Study: Unraveling Limitations for Physical Activity in Children With Chronic Diseases Order to Target Them With Tailored Interventions—A Randomized Cross Over Trial

Introduction: Physical activity is associated with many physiological and psychological health benefits across the lifespan. Children with a chronic disease often have lower levels of daily physical activity, and a decreased exercise capacity compared to healthy peers. In order to learn more about limitations for physical activity, we investigate children with four different chronic diseases: children with a Fontan circulation, children with Broncho Pulmonary Dysplasia (BPD), Pompe disease and inflammatory bowel disease (IBD). Each of these diseases is likely to interfere with physical activity in a different way. Knowing the specific limitations for physical activity would make it possible to target these, and increase physical activity by a personalized intervention. The aim of this study is to first investigate limitations for physical activity in children with various chronic diseases. Secondly, to measure the effects of a tailored exercise intervention, possibly including a personalized dietary advice and/or psychological counseling, on exercise capacity, endurance, quality of life, fatigue, fear for exercise, safety, muscle strength, physical activity levels, energy balance, and body composition.Methods and Analysis: This randomized crossover trial will aim to include 72 children, aged 6–18 years, with one of the following diagnosis: a Fontan circulation, BPD, Pompe disease and IBD. Eligible patients will participate in the 12-week tailored exercise intervention and are either randomized to start with a control period or start with the intervention. The tailored 12-week exercise interventions, possibly including a personalized dietary advice and/or psychological counseling, will be designed based on the found limitations for physical activity in each disease group during baseline measurements by the Rotterdam Exercise Team. Effects of the tailored training interventions will be measured on the following endpoints: exercise capacity (measured by cardiopulmonary exercise test), endurance, physical activity levels, muscle strength, quality of life, fatigue, fear for exercise, disease activity, cardiac function (in children with a Fontan circulation), energy balance, and body composition.Ethics and Dissemination: Conducted according to the Declaration of Helsinki and Good Clinical Practice. Medical-ethical approval was obtained.Trial Registration Number: NL8181, https://www.trialregister.nl/trial/8181.

Effects of neuromuscular electrical stimulation on exercise capacity, muscle strength and quality of life in COPD patients: A Systematic Review with Meta-Analysis

Objective To determine the effects of neuromuscular electrical stimulation on disabilities and activity limitation of individuals affected by chronic obstructive pulmonary disease. Data sources MEDLINE, PEDro database, Cochrane Controlled Trials Register, and SciELO, were searched from inception until October 2021. Review methods Inclusion criteria were patients with COPD, randomized controlled trials comparing neuromuscular electrical stimulation alone or combined conventional pulmonary rehabilitation and neuromuscular electrical stimulation versus control or sham or pulmonary rehabilitation in disabilities and activity limitation in COPD. There were no mandatory language or publication date restrictions. Two reviewers selected studies independently. Weighted mean differences and 95% confidence intervals were calculated. Results 32 studies met the study criteria, including 1.269 participants. Neuromuscular electrical stimulation improved exercise capacity (MD 1.10, 95% CI: 0.33, 1.86, N = 147), and muscle strength (0.53, 95% CI: 0.20, 0.87, N = 147) compared to sham group. Combined neuromuscular electrical stimulation and conventional rehabilitation improved exercise capacity (MD 34.28 meters, 95% CI: 6.84, 61.73, N = 262) compared to conventional rehabilitation alone. No adverse events were reported. Conclusions Neuromuscular electrical stimulation resulted in small improvement in disabilities and activity limitation (below the MCID) in COPD. Thus, the inclusion of neuromuscular electrical stimulation in rehabilitation programs must consider the cost Because of inadequate methodological conduction and reporting of methods, some studies were of low quality.

Clinical impact of subcutaneous treprostinil in trisomy 21 patient with pulmonary arterial hypertension associated with CHD

Subcutaneous treprostinil is commonly used to improve idiopathic pulmonary arterial hypertension in children. However, its effectiveness has not been reported in trisomy 21. We report the case of 9-year-old boy in trisomy 21 with CHD-pulmonary artery hypertension after surgical correction of CHD. Haemodynamics and exercise capacity dramatically improved with a transition from oral selexipag to subcutaneous treprostinil.

The association of lung function and pulmonary vasculature volume with cardiorespiratory fitness in the community

IntroductionCardiorespiratory fitness is not limited by pulmonary mechanical reasons in the majority of adults. However, the degree to which lung function contributes to exercise response patterns among ostensibly healthy individuals remains unclear.MethodsWe examined 2314 Framingham Heart Study participants who underwent cardiopulmonary exercise testing (CPET) and pulmonary function testing. We investigated the association of FEV1, FVC, FEV1/FVC and DLCO with the primary outcome of peak VO2, along with other CPET parameters using multivariable linear regression. Finally, we investigated the association of total and peripheral pulmonary blood vessel volume with peak VO2.ResultsWe found lower FEV1, FVC and DLCO were associated with lower peak VO2. For example, a one-liter lower FEV1 and FVC were associated with 7.1% (95% CI: 5.1%, 9.1%) and 6.0% (95% CI: 4.3%, 7.7%) lower peak VO2, respectively. By contrast, FEV1/FVC ratio was not associated with peak VO2. Lower lung function was associated with lower oxygen uptake efficiency slope oxygen pulse slope, VO2 at AT, VE at AT and breathing reserve. In addition, lower total and peripheral pulmonary blood vessel volume were associated with a lower peak VO2.ConclusionIn a large, community-based cohort of adults, we found lower FEV1, FVC and DLCO were associated with lower exercise capacity, as well as oxygen uptake efficiency slope and ventilatory efficiency. In addition, lower total and peripheral pulmonary blood vessel volume were associated with lower peak VO2. These findings underscore the importance of lung function and blood vessel volume as contributors to overall exercise capacity.

Stimulating the sir2-pgc-1α axis rescues exercise capacity and mitochondrial respiration in Drosophila tafazzin mutants.

Cardiolipin (CL) is a phospholipid required for proper mitochondrial function. Tafazzin remodels CL to create highly unsaturated fatty acid chains. However, when tafazzin is mutated, CL remodeling is impeded, leading to mitochondrial dysfunction and the disease Barth syndrome. Patients with Barth syndrome often have severe exercise intolerance, which negatively impacts their overall quality of life. Boosting NAD+ levels can improve symptoms of other mitochondrial diseases, but its effect in the context of Barth syndrome has not been examined. We demonstrate for the first time that nicotinamide riboside (NR) can rescue exercise tolerance and mitochondrial respiration in a Drosophila tafazzin mutant and that the beneficial effects are dependent on sir2 and pgc-1α . Overexpressing pgc-1α increased the total abundance of cardiolipin in mutants. In addition, muscles and neurons were identified as key targets for future therapies because sir2 or pgc-1α overexpression in either of these tissues is sufficient to restore the exercise capacity of Drosophila tafazzin mutants.