Respiratory therapies for amyotrophic lateral sclerosis: A primer

Respiratory complications are a common cause of morbidity and mortality in amyotrophic lateral sclerosis (ALS). Treatment of respiratory insufficiency with noninvasive ventilation (NIV) improves ALS patients' quality of life and survival. Evidence‐based practice guidelines for the management of ALS patients recommend treatment of respiratory insufficiency with NIV as well as consideration of insufflation/exsufflation to improve clearance of airway secretions. Despite these recommendations respiratory therapies remain underused. In this review we provide a practical guide for the clinician to prescribe and manage respiratory therapies for the patient with ALS. Muscle Nerve 46: 313–331, 2012Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/93524/1/23282_ftp.pd

Validation of the Tranquillity Rating Prediction Tool (TRAPT): comparative studies in UK and Hong Kong

YesThe Tranquillity Rating Prediction Tool (TRAPT) has been used to make predictions of the quality of tranquility in outdoor urban areas using two significant factors i.e. the average level of anthropogenic noise and the percentage of natural features in view. The method has a number of applications including producing tranquillity contours that can inform decisions regarding the impact of new anthropogenic noise sources or developments causing visual intrusion. The methodwas intended for use in mainly outdoor areas and yet was developed using responses from UK volunteers to video clips indoors. Because the volunteers for this study were all UK residents it was important to calibrate responses for other ethnic groups who may respond differently depending on cultural background. To address these issues further studies were performed in Hong Kong using the same video recording played back under the same conditions as the study in the UK. The HK study involved recruiting three groups i.e. residents fromHong Kong, Mainland China and a diverse group from 16 different nations. There was good agreement between all these groups with average tranquillity ratings for the different locations differing by less than one scale point in most cases.The studywas supported by the Bradford Centre for Sustainable Environments at the University of Bradford and by the Research Grants Council of the Hong Kong Special Administrative Region, China (RGC/GRF. CUHK 449612

Mechanical cough augmentation techniques in amyotrophic lateral sclerosis/motor neuron disease

© 2016 The Cochrane Collaboration.This is a protocol for a Cochrane Review (Intervention). The objectives are as follows: To assess the effects of mechanical insufflator/exsufflator (MI-E) and the breath-stacking technique for reducing morbidity and mortality and enhancing quality of life in people with amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND)

The clinical effectiveness and cost-effectiveness of treatments for idiopathic pulmonary fibrosis: a systematic review and economic evaluation

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a life-limiting lung disease that generally affects people over 60 years old. The main symptoms are shortness of breath and cough, and as the disease progresses there is a considerable impact on day-to-day life. Few treatments are currently available. OBJECTIVES: To conduct a systematic review of clinical effectiveness and an analysis of cost-effectiveness of treatments for IPF based on an economic model informed by systematic reviews of cost-effectiveness and quality of life. DATA SOURCES: Eleven electronic bibliographic databases, including MEDLINE, EMBASE, Web of Science, and The Cochrane Library and the Centre for Reviews and Dissemination databases, were searched from database inception to July 2013. Reference lists of relevant publications were also checked and experts consulted. METHODS: Two reviewers independently screened references for the systematic reviews, extracted and checked data from the included studies and appraised their risk of bias. An advisory group was consulted about the choice of interventions until consensus was reached about eligibility. A narrative review with meta-analysis was undertaken, and a network meta-analysis (NMA) was performed. A decision-analytic Markov model was developed to estimate cost-effectiveness of pharmacological treatments for IPF. Parameter values were obtained from NMA and systematic reviews. Univariate and probabilistic sensitivity analyses were undertaken. The model perspective is NHS and Personal Social Services, and discount rate is 3.5% for costs and health benefits. RESULTS: Fourteen studies were included in the review of clinical effectiveness, of which one evaluated azathioprine, three N-acetylcysteine (NAC) (alone or in combination), four pirfenidone, one BIBF 1120, one sildenafil, one thalidomide, two pulmonary rehabilitation, and one a disease management programme. Study quality was generally good, with a low risk of bias. The current evidence suggests that some treatments appear to be clinically effective. The model base-case results show increased survival for five pharmacological treatments, compared with best supportive care, at increased cost. General recommendations cannot be made of their cost-effectiveness owing to limitations in the evidence base. LIMITATIONS: Few direct comparisons of treatments were identified. An indirect comparison through a NMA was performed; however, caution is recommended in the interpretation of these results. In relation to the economic model, there is an assumption that pharmacological treatments have a constant effect on the relative rate of per cent predicted forced vital capacity decline. CONCLUSIONS: Few interventions have any statistically significant effect on IPF and a lack of studies on palliative care approaches was identified. Research is required into the effects of symptom control interventions, in particular pulmonary rehabilitation and thalidomide. Other research priorities include a well-conducted randomised controlled trial on inhaled NAC therapy and an updated evidence synthesis once the results of ongoing studies are reported

Respiratory Measures in Amyotrophic Lateral Sclerosis

Objective: Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disease that causes skeletal muscle weakness, including muscles involved with respiration. Death often results from respiratory failure within 3€“5 years. Monitoring respiratory status is therefore critical to ALS management, as respiratory/pulmonary function tests (PFTs) are used to make decisions including when to initiate noninvasive ventilation. Understanding the different respiratory and PFTs as they relate to disease progression and survival may help determine which tests are most suitable. Methods: This review describes the tests used to assess respiratory muscle and pulmonary function in patients with ALS and the correlations between different respiratory measures and clinical outcomes measures. Results: The most commonly used measurement, forced vital capacity (VC), has been shown to correlate with clinical milestones including survival, but also requires good motor coordination and facial strength to form a tight seal around a mouthpiece. Other tests such as slow VC, sniff inspiratory pressure, or transdiaphragmatic pressure with magnetic stimulation are also associated with distinct advantages and disadvantages. Conclusions: Therefore, how and when to use different tests remains unclear. Understanding how each test relates to disease progression and survival may help determine which is best suited for specific clinical decisions

MiToS and King\u27s staging as clinical outcome measures in ALS: A retrospective analysis of the FORTITUDE-ALS trial

OBJECTIVE: To evaluate the Milano-Torino staging (MiToS) and King\u27s staging systems as potential outcome measures for clinical trials in amyotrophic lateral sclerosis (ALS) by assessing these outcomes in FORTITUDE-ALS. METHODS: This was a RESULTS: The full analysis set consisted of 456 patients randomized 3:1 ( CONCLUSION: This exploratory analysis showed the feasibility of MiToS and King\u27s staging as potential outcome measures in ALS. Additional studies of these staging systems are needed to further explore their utility in ALS clinical trials

Influence of Additional Post-Bronchoscopy Visit on Patient Satisfaction after Flexible Bronchoscopy

Background/Aims: Many patients undergoing a flexible bronchoscopy (FB) experience anxiety and discomfort during the procedure. We assessed whether an additional patient visit after a FB would improve patient satisfaction. Methods: The study patients were randomly assigned to a control and post-visit groups. The physicians who were scheduled to perform the FB visited the study patients. The control group had one visit before the FB and the postvisit group had a before and after FB visit. The post-visit group received additional information and support during the second visit. Twenty-four hours after the FB, the participants completed questionnaires about discomfort and satisfaction with the procedure. Results: The control and post-visit groups included 151 and 153 patients, respectively. The post-visit group reported having more information after the FB than the control group. The additional post-bronchoscopy visit improved the general patient tolerability of the procedure. The willingness to return for another FB was not affected by the post-bronchoscopy patient visit. Conclusions: The post-bronchoscopy visit improved patient satisfaction and general tolerability to the procedure. Subjective patient tolerability with the FB may be improved through a post-bronchoscopy visit by providing more information and emotional support to patients.Y