Inspiratory Muscle Training in Nemaline Myopathy.

BACKGROUND: Respiratory muscle weakness is a common feature in nemaline myopathy. Inspiratory muscle training (IMT) is an intervention that aims to improve inspiratory muscle strength. OBJECTIVE: The aim of this controlled before-and-after pilot study was to investigate if IMT improves respiratory muscle strength in patients with nemaline myopathy. METHODS: Nine patients (7 females; 2 males, age 36.6±20.5 years) with respiratory muscle weakness and different clinical phenotypes and genotypes were included. Patients performed eight weeks of sham IMT followed by eight weeks of active threshold IMT. The patients trained twice a day five days a week for 15 minutes at home. The intensity was constant during the training after a gradual increase to 30% of maximal inspiratory pressure (MIP). RESULTS: Active IMT significantly improved MIP from 43±15.9 to 47±16.6 cmH2O (p = 0.019). The effect size was 1.22. There was no significant effect of sham IMT. Sniff nasal inspiratory pressure, maximal expiratory pressure, spirometry, and diaphragm thickness and thickening showed no significant improvements. CONCLUSIONS: This pilot study shows that threshold IMT is feasible in patients with nemaline myopathy and improves inspiratory muscle strength. Our findings provide valuable preliminary data for the design of a larger, more comprehensive trial.

Pulmonary rehabilitation in Africa: where are we? a multimethod study.

Pulmonary rehabilitation (PR) is an integral part of the management of patients with chronic respiratory diseases. However, there is limited information available on the effectiveness and practice of PR in Africa. This study was conducted to examine the prevalence, structure, and organization of PR in Africa, as well as its substance and claimed efficacy. We conducted a multimethod study involving systematic review of PR studies (obtained from PubMed, Google Scholar, and Cochrane databases) and a web-based survey of African healthcare professionals engaged in PR (using a standardized questionnaire). The review included papers on at least one component of PR in Africa and excluded those on PR from other continents or assessing pulmonary disorders in general without PR, cardio-rehabilitation, or physiotherapy practice in general in Africa. The Cochrane risk of bias and the Newcastle Ottawa scale instruments were used to assess the quality of included studies. We narratively synthesised data across the studies to produce a holistic picture. Of the 14 studies included for qualitative synthesis, seven were randomized controlled trials on the effectiveness of PR treatments with a total number of 333 participants. Of the 39 surveys mailed to health professionals working in Africa, only 14 (35.8%) were returned. We found aerobic exercise and breathing exercises were the most used technique and that quality of life, exercise capacity, and lung function improved significantly after PR treatments. There were differences in the duration, frequency, and length of the programs across the continent. Half of the respondents indicated that their institutions had one or more PR programs for inpatient, outpatient, maintenance, and/or home-based programs. Additionally, aerobic activities, upper and lower extremity strength training were the most frequently used exercise modalities in PR programs, followed by breathing exercises. Pulmonary rehabilitation is understudied in Africa, but it has been linked to improved lung function, exercise capacity, and quality of life. There is a need to invest in techniques tailored to the continent to enhance the implementation of pulmonary rehabilitation in Africa.

Measurement validity of an electronic training device to assess breathing characteristics during inspiratory muscle training in patients with weaning difficulties.

Inspiratory muscle training (IMT) improves respiratory muscle function and might enhance weaning outcomes in patients with weaning difficulties. An electronic inspiratory loading device provides valid, automatically processed information on breathing characteristics during IMT sessions. Adherence to and quality of IMT, as reflected by work of breathing and power generated by inspiratory muscles, are related to improvements in inspiratory muscle function in patients with chronic obstructive pulmonary disease. The aim of this study was to investigate the validity of an electronic training device to assess and provide real-time feedback on breathing characteristics during inspiratory muscle training (IMT) in patient with weaning difficulties. Patients with weaning difficulties performed daily IMT sessions against a tapered flow-resistive load of approximately 30 to 50% of the patient's maximal inspiratory pressure. Airflow and airway pressure measurements were simultaneously collected with the training device (POWERbreatheKH2, POWERbreathe International Ltd, UK) and a portable spirometer (reference device, Pocket-Spiro USB/BT100, M.E.C, Belgium). Breath by breath analysis of 1002 breaths of 27 training sessions (n = 13) against a mean load of 46±16% of the patient's maximal inspiratory pressure were performed. Good to excellent agreement (Intraclass correlation coefficients: 0.73-0.97) was observed for all breathing characteristics. When individual differences were plotted against mean values of breaths recorded by both devices, small average biases were observed for all breathing characteristics. To conclude, the training device provides valid assessments of breathing characteristics to quantify inspiratory muscle effort (e.g. work of breathing and peak power) during IMT in patients with weaning difficulties. Availability of valid real-time data of breathing responses provided to both the physical therapist and the patient, can be clinically usefull to optimize the training stimulus. By adapting the external load based on the visual feedback of the training device, respiratory muscle work and power generation during IMT can be maximized during the training.

Early neuromuscular electrical stimulation reduces the loss of muscle mass in critically ill patients - A within subject randomized controlled trial.

PURPOSE: To investigate the effect of Neuromuscular Electrical Stimulation (NMES) on muscle thickness, strength and morphological and molecular markers of the quadriceps. MATERIALS AND METHODS: Adult critically ill patients with an expected prolonged stay received unilateral quadriceps NMES sessions for 7 consecutive days. Before and after the intervention period, quadriceps thickness was measured with ultrasound. After the intervention period, strength was assessed in cooperative patients and muscle biopsies were taken. Multivariable regression was performed to identify factors affecting muscle thickness loss. RESULTS: Muscle thickness decreased less in the stimulated leg (-6 ± 16% versus -12 ± 15%, p = 0.014, n = 47). Strength was comparable. Opioid administration, minimal muscle contraction and more muscle thickness loss in the non-stimulated muscle were independently associated with better muscle thickness preservation. Stimulated muscles showed a shift towards larger myofibers and higher MyHC-I gene expression. NMES did not affect gene expression of other myofibrillary proteins, MuRF-1 or atrogin-1. Signs of myofiber necrosis and inflammation were comparable for both muscles. CONCLUSIONS: NMES attenuated the loss of muscle mass, but not of strength, in critically ill patients. Preservation of muscle mass was more likely in patients receiving opioids, patients with a minimal muscle contraction during NMES and patients more prone to lose muscle mass. TRIAL REGISTRATION: clinicaltrials.govNCT02133300.

Differences in Respiratory Muscle Responses to Hyperpnea or Loaded Breathing in COPD.

INTRODUCTION: We aimed to compare acute mechanical and metabolic responses of the diaphragm and rib cage inspiratory muscles during two different types of respiratory loading in patients with chronic obstructive pulmonary disease. METHODS: In 16 patients (age, 65 ± 13 yr; 56% male; forced expiratory volume in the first second, 60 ± 6%pred; maximum inspiratory pressure, 82 ± 5%pred), assessments of respiratory muscle EMG, esophageal pressure (Pes) and gastric pressures, breathing pattern, and noninvasive assessments of systemic (V˙O2, cardiac output, oxygen delivery and extraction) and respiratory muscle hemodynamic and oxygenation responses (blood flow index, oxygen delivery index, deoxyhemoglobin concentration, and tissues oxygen saturation [StiO2]), were performed during hyperpnea and loaded breathing. RESULTS: During hyperpnea, breathing frequency, minute ventilation, esophageal and diaphragm pressure-time product per minute, cardiac output, and V˙O2 were higher than during loaded breathing (P < 0.05). Average inspiratory Pes and transdiaphragmatic pressure per breath, scalene (SCA), sternocleidomastoid, and intercostal muscle activation were higher during loading breathing compared with hyperpnea (P < 0.05). Higher transdiaphragmatic pressure during loaded breathing compared with hyperpnea was mostly due to higher inspiratory Pes (P < 0.05). Diaphragm activation, inspiratory and expiratory gastric pressures, and rectus abdominis muscle activation did not differ between the two conditions (P > 0.05). SCA-blood flow index and oxygen delivery index were lower, and SCA-deoxyhemoglobin concentration was higher during loaded breathing compared with hyperpnea. Furthermore, SCA and intercostal muscle StiO2 were lower during loaded breathing compared with hyperpnea (P < 0.05). CONCLUSION: Greater inspiratory muscle effort during loaded breathing evoked larger rib cage and neck muscle activation compared with hyperpnea. In addition, lower SCA and intercostal muscle StiO2 during loaded breathing compared with hyperpnea indicates a mismatch between inspiratory muscle oxygen delivery and utilization induced by the former condition.

Can inspiratory muscle training improve weaning outcomes in difficult to wean patients? A protocol for a randomised controlled trial (IMweanT study).

INTRODUCTION: Respiratory muscle dysfunction has been associated with failure to wean from mechanical ventilation. It has therefore been hypothesised that these patients might benefit from inspiratory muscle training (IMT). Evidence, however, is thus far limited to data from small, single-centre studies with heterogeneity in inclusion criteria, training modalities and outcomes. The aim of this study is to evaluate the effects of a novel IMT method on weaning outcomes in selected patients with weaning difficulties. METHODS: This study is designed as a double-blind, parallel-group, randomised controlled superiority trial with 1:1 allocation ratio. Patients with weaning difficulties will be randomly allocated into either an IMT group (intervention) or a sham-IMT group (control). Ninetypatients (45 in each group) will be needed to detect a 28% difference in the proportion of weaning success between groups (estimated difference in primary outcome based on previous studies) with a risk for type I error (α) of 5% and statistical power (1-ß) of 80%. Patients will perform four sets of 6-10 breaths daily against an external load using a tapered flow resistive loading device (POWERbreathe KH2, HaB International, UK). Training intensity in the intervention group will be adjusted to the highest tolerable load. The control group will train against a low resistance that will not be modified during the training period. Training will becontinued until patients are successfully weaned or for a maximum duration of 28 days. Pulmonary and respiratory muscle function, weaning duration, duration of mechanical ventilation, ventilator-free days and length of stay in the intensive care unit will be evaluated as secondary outcomes. Χ2 tests and analysis of covariance with adjustments for baseline values of respective outcomesas covariates will be used to compare results after the intervention period between groups. ETHICS AND DISSEMINATION: Ethics approval was obtained from the local ethical committee (Ethische Commissie Onderzoek UZ/KU Leuven protocol ID: S60516). Results from this randomised controlled trial will be presented at scientific meetings as abstracts for poster or oral presentations and published in peerreviewed journals. TRIAL STATUS: Enrolment into the study have started in August 2017. Data collection and data analysis are expected to be completed in September 2021. TRIAL REGISTRATION NUMBER: NCT03240263.

Randomised controlled trial of adjunctive inspiratory muscle training for patients with COPD.

BACKGROUND: This study aimed to investigate whether adjunctive inspiratory muscle training (IMT) can enhance the well-established benefits of pulmonary rehabilitation (PR) in patients with COPD. METHODS: 219 patients with COPD (FEV1: 42%±16% predicted) with inspiratory muscle weakness (PImax: 51±15 cm H2O) were randomised into an intervention group (IMT+PR; n=110) or a control group (Sham-IMT+PR; n=109) in this double-blind, multicentre randomised controlled trial between February 2012 and October 2016 (ClinicalTrials.gov NCT01397396). Improvement in 6 min walking distance (6MWD) was a priori defined as the primary outcome. Prespecified secondary outcomes included respiratory muscle function and endurance cycling time. FINDINGS: No significant differences between the intervention group (n=89) and the control group (n=85) in improvements in 6MWD were observed (0.3 m, 95% CI -13 to 14, p=0.967). Patients who completed assessments in the intervention group achieved larger gains in inspiratory muscle strength (effect size: 1.07, p<0.001) and endurance (effect size: 0.79, p<0.001) than patients in the control group. 75 s additional improvement in endurance cycling time (95% CI 1 to 149, p=0.048) and significant reductions in Borg dyspnoea score at isotime during the cycling test (95% CI -1.5 to -0.01, p=0.049) were observed in the intervention group. INTERPRETATION: Improvements in respiratory muscle function after adjunctive IMT did not translate into additional improvements in 6MWD (primary outcome). Additional gains in endurance time and reductions in symptoms of dyspnoea were observed during an endurance cycling test (secondary outcome) TRIAL REGISTRATION NUMBER: NCT01397396; Results.

Inspiratory muscle training reduces diaphragm activation and dyspnea during exercise in COPD.

Among patients with chronic obstructive pulmonary disease (COPD), those with the lowest maximal inspiratory pressures experience greater breathing discomfort (dyspnea) during exercise. In such individuals, inspiratory muscle training (IMT) may be associated with improvement of dyspnea, but the mechanisms for this are poorly understood. Therefore, we aimed to identify physiological mechanisms of improvement in dyspnea and exercise endurance following inspiratory muscle training (IMT) in patients with COPD and low maximal inspiratory pressure (Pimax). The effects of 8 wk of controlled IMT on respiratory muscle function, dyspnea, respiratory mechanics, and diaphragm electromyography (EMGdi) during constant work rate cycle exercise were evaluated in patients with activity-related dyspnea (baseline dyspnea index <9). Subjects were randomized to either IMT or a sham training control group ( n = 10 each). Twenty subjects (FEV1 = 47 ± 19% predicted; Pimax = -59 ± 14 cmH2O; cycle ergometer peak work rate = 47 ± 21% predicted) completed the study; groups had comparable baseline lung function, respiratory muscle strength, activity-related dyspnea, and exercise capacity. IMT, compared with control, was associated with greater increases in inspiratory muscle strength and endurance, with attendant improvements in exertional dyspnea and exercise endurance time (all P < 0.05). After IMT, EMGdi expressed relative to its maximum (EMGdi/EMGdimax) decreased ( P < 0.05) with no significant change in ventilation, tidal inspiratory pressures, breathing pattern, or operating lung volumes during exercise. In conclusion, IMT improved inspiratory muscle strength and endurance in mechanically compromised patients with COPD and low Pimax. The attendant reduction in EMGdi/EMGdimax helped explain the decrease in perceived respiratory discomfort despite sustained high ventilation and intrinsic mechanical loading over a longer exercise duration. NEW & NOTEWORTHY In patients with COPD and low maximal inspiratory pressures, inspiratory muscle training (IMT) may be associated with improvement of dyspnea, but the mechanisms for this are poorly understood. This study showed that 8 wk of home-based, partially supervised IMT improved respiratory muscle strength and endurance, dyspnea, and exercise endurance. Dyspnea relief occurred in conjunction with a reduced activation of the diaphragm relative to maximum in the absence of significant changes in ventilation, breathing pattern, and operating lung volumes.

Postoperative inspiratory muscle training in addition to breathing exercises and early mobilization improves oxygenation in high-risk patients after lung cancer surgery: a randomized controlled trial.

OBJECTIVES: The aim was to investigate whether 2 weeks of inspiratory muscle training (IMT) could preserve respiratory muscle strength in high-risk patients referred for pulmonary resection on the suspicion of or confirmed lung cancer. Secondarily, we investigated the effect of the intervention on the incidence of postoperative pulmonary complications. METHODS: The study was a single-centre, parallel-group, randomized trial with assessor blinding and intention-to-treat analysis. The intervention group (IG, n = 34) underwent 2 weeks of postoperative IMT twice daily with 2 × 30 breaths on a target intensity of 30% of maximal inspiratory pressure, in addition to standard postoperative physiotherapy. Standard physiotherapy in the control group (CG, n = 34) consisted of breathing exercises, coughing techniques and early mobilization. We measured respiratory muscle strength (maximal inspiratory/expiratory pressure, MIP/MEP), functional performance (6-min walk test), spirometry and peripheral oxygen saturation (SpO2), assessed the day before surgery and again 3-5 days and 2 weeks postoperatively. Postoperative pulmonary complications were evaluated 2 weeks after surgery. RESULTS: The mean age was 70 ± 8 years and 57.5% were males. Thoracotomy was performed in 48.5% (n = 33) of cases. No effect of the intervention was found regarding MIP, MEP, lung volumes or functional performance at any time point. The overall incidence of pneumonia was 13% (n = 9), with no significant difference between groups [IG 6% (n = 2), CG 21% (n = 7), P = 0.14]. An improved SpO2 was found in the IG on the third and fourth postoperative days (Day 3: IG 93.8 ± 3.4 vs CG 91.9 ± 4.1%, P = 0.058; Day 4: IG 93.5 ± 3.5 vs CG 91 ± 3.9%, P = 0.02). We found no association between surgical procedure (thoracotomy versus thoracoscopy) and respiratory muscle strength, which was recovered in both groups 2 weeks after surgery. CONCLUSIONS: Two weeks of additional postoperative IMT, compared with standard physiotherapy alone, did not preserve respiratory muscle strength but improved oxygenation in high-risk patients after lung cancer surgery. Respiratory muscle strength recovered in both groups 2 weeks after surgery. CLINICAL TRIALSGOV ID: NCT01793155.

Efficacy of a Novel Method for Inspiratory Muscle Training in People With Chronic Obstructive Pulmonary Disease.

BACKGROUND: Most inspiratory muscle training (IMT) interventions in patients with chronic obstructive pulmonary disease (COPD) have been implemented as fully supervised daily training for 30 minutes with controlled training loads using mechanical threshold loading (MTL) devices. Recently, an electronic tapered flow resistive loading (TFRL) device was introduced that has a different loading profile and stores training data during IMT sessions. OBJECTIVE: The aim of this study was to compare the efficacy of a brief, largely unsupervised IMT protocol conducted using either traditional MTL or TFRL on inspiratory muscle function in patients with COPD. DESIGN: Twenty patients with inspiratory muscle weakness who were clinically stable and participating in a pulmonary rehabilitation program were randomly allocated to perform 8 weeks of either MTL IMT or TFRL IMT. METHODS: Participants performed 2 daily home-based IMT sessions of 30 breaths (3-5 minutes per session) at the highest tolerable intensity, supported by twice-weekly supervised sessions. Adherence, progression of training intensity, increases in maximal inspiratory mouth pressure (Pimax), and endurance capacity of inspiratory muscles (Tlim) were evaluated. RESULTS: More than 90% of IMT sessions were completed in both groups. The TFRL group tolerated higher loads during the final 3 weeks of the IMT program, with similar effort scores on the 10-Item Borg Category Ratio (CR-10) Scale, and achieved larger improvements in Pimax and Tlim than the MTL group. LIMITATIONS: A limitation of the study was the absence of a study arm involving a sham IMT intervention. CONCLUSIONS: The short and largely home-based IMT protocol significantly improved inspiratory muscle function in both groups and is an alternative to traditional IMT protocols in this population. Participants in the TFRL group tolerated higher training loads and achieved larger improvements in inspiratory muscle function than those in the MTL group.

Feasibility of neuromuscular electrical stimulation in critically ill patients.

OBJECTIVE: Critically ill patients often develop intensive care unit-acquired weakness. Reduction in muscle mass and muscle strength occurs early after admission to the intensive care unit (ICU). Although early active muscle training could attenuate this intensive care unit-acquired weakness, in the early phase of critical illness, a large proportion of patients are unable to participate in any active mobilization. Neuromuscular electrical stimulation (NMES) could be an alternative strategy for muscle training. The aim of this study was to investigate the safety and feasibility of NMES in critically ill patients. DESIGN: This is an observational study. SETTING: The setting is in the medical and surgical ICUs of a tertiary referral university hospital. PATIENTS: Fifty patients with a prognosticated prolonged stay of at least 6 days were included on day 3 to 5 of their ICU stay. Patients with preexisting neuromuscular disorders and patients with musculoskeletal conditions limiting quadriceps contraction were excluded. INTERVENTION: Twenty-five minutes of simultaneous bilateral NMES of the quadriceps femoris muscle. This intervention was performed 5 days per week (Monday-Friday). Effective muscle stimulation was defined as a palpable and visible contraction (partial or full muscle bulk). MEASUREMENTS: The following parameters, potentially affecting contraction upon NMES, were assessed: functional status before admission to the ICU (Barthel index), type and severity of illness (Acute Physiology And Chronic Health Evaluation II score and sepsis), treatments possibly influencing the muscle contraction (corticosteroids, vasopressors, inotropes, aminoglycosides, and neuromuscular blocking agents), level of consciousness (Glasgow Coma Scale, score on 5 standardized questions evaluating awakening, and sedation agitation scale), characteristics of stimulation (intensity of the NMES, number of sessions per patient, and edema), and neuromuscular electrophysiologic characteristics. Changes in heart rate, blood pressure, oxygen saturation, respiratory rate, and skin reactions were registered to assess the safety of the technique. RESULTS: In 50% of the patients, an adequate quadriceps contraction was obtained in at least 75% of the NMES sessions. Univariate analysis showed that lower limb edema (P<.001), sepsis (P=.008), admission to the medical ICU (P=.041), and treatment with vasopressors (P=.011) were associated with impaired quadriceps contraction. A backward multivariate analysis identified presence of sepsis, lower limb edema, and use of vasopressors as independent predictors of impaired quadriceps contraction (R2=59.5%). Patients responded better to NMES in the beginning of their ICU stay in comparison with after 1 week of ICU stay. There was no change in any of the safety end points with NMES. CONCLUSIONS: Critically ill patients having sepsis, edema, or receiving vasopressors were less likely to respond to NMES with an adequate quadriceps contraction. Neuromuscular electrical stimulation is a safe intervention to be administered in the ICU.