Laryngospasm in amyotrophic lateral sclerosis.

INTRODUCTION: Laryngospasm is an involuntary, sustained closure of sphincter musculature that leads to an unpleasant subjective experience of dyspnea and choking. It is an underreported symptom in amyotrophic lateral sclerosis (ALS). In this study we aimed to better characterize the prevalence and clinical characteristics of laryngospasm in ALS patients. METHODS: The medical records of 571 patients with ALS followed between 2008 and 2018 were searched for evidence of laryngospasm. A total of 23 patients with laryngospasm were identified and the data related to patient and laryngospasm characteristics were extracted. RESULTS: Laryngospasm was reported in 4% of ALS patients. Females comprised 57% of patients and their mean age was 63.4 years. Laryngospasm frequently manifested in patients with moderate bulbar dysfunction and seemed independent of respiratory function. Among laryngospasm patients, 26% were cigarette smokers and 13% had a history of gastroesophageal reflux. The most common reported trigger was excessive saliva irritating the vocal cords (35%) followed by eating a meal (17%). There was significant variation in laryngospasm frequency (up to 5 per hour) and duration (seconds to minutes). Most patients could not identify an effective coping mechanism, although 13% reported that drinking water was effective. DISCUSSION: Despite its low prevalence in ALS, laryngospasm should be included in the symptom inquiry. The present findings may improve patient care through increased recognition of the clinical features of laryngospasm in ALS patients, identifying a link between laryngospasm and moderate bulbar dysfunction, and highlighting trigger avoidance as a management strategy. Additional research is required to understand the pathophysiology and optimal treatment.

Healthcare utilisation and costs of home mechanical ventilation.

BACKGROUND: Individuals using home mechanical ventilation (HMV) frequently choose to live at home for quality of life, despite financial burden. Previous studies of healthcare utilisation and costs do not consider public and private expenditures, including caregiver time. OBJECTIVES: To determine public and private healthcare utilisation and costs for HMV users living at home in two Canadian provinces, and examine factors associated with higher costs. METHODS: Longitudinal, prospective observational cost analysis study (April 2012 to August 2015) collecting data on public and private (out-of-pocket, third-party insurance, caregiving) costs every 2 weeks for 6 months using the Ambulatory and Home Care Record. Functional Independence Measure (FIM) was used at baseline and study completion. Regression models examined variables associated with total monthly costs selected a priori using Andersen and Newman's framework for healthcare utilisation, relevant literature, and clinical expertise. Data are reported in 2015 Canadian dollars ($C1=US$0.78=£0.51=€0.71). RESULTS: We enrolled 134 HMV users; 95 with family caregivers. Overall median (IQR) monthly healthcare cost was $5275 ($2291-$10 181) with $2410 (58%) publicly funded; $1609 (39%) family caregiving; and $141 (3%) out-of-pocket (<1% third-party insurance). Median healthcare costs were $8733 ($5868-$15 274) for those invasively ventilated and $3925 ($1212-$7390) for non-invasive ventilation. Variables associated with highest monthly costs were amyotrophic lateral sclerosis (1.88, 95% CI 1.09 to 3.26, P<0.03) and lower FIM quintiles (higher dependency) (up to 6.98, 95% CI 3.88 to 12.55, P<0.0001) adjusting for age, sex, tracheostomy and ventilation duration. CONCLUSIONS: For HMV users, most healthcare costs were publicly supported or associated with family caregiving. Highest costs were incurred by the most dependent users. Understanding healthcare costs for HMV users will inform policy decisions to optimise resource allocation, helping individuals live at home while minimising caregiver burden.

A Randomized Controlled Clinical Trial of Lung Volume Recruitment in Adults with Neuromuscular Disease.

Rationale: Clinical care guidelines advise that lung volume recruitment (LVR) be performed routinely by people with neuromuscular disease (NMD) to maintain lung and chest wall flexibility and slow lung function decline. However, the evidence base is limited, and no randomized controlled trials of regular LVR in adults have been published. Objectives: To evaluate the effect of regular LVR on respiratory function and quality of life in adults with NMD. Methods: A randomized controlled trial with assessor blinding was conducted between September 2015 and May 2019. People (>14 years old) with NMD and vital capacity <80% predicted were eligible, stratified by disease subgroup (amyotrophic lateral sclerosis/motor neuron disease or other NMDs), and randomized to 3 months of twice-daily LVR or breathing exercises. The primary outcome was change in maximum insufflation capacity (MIC) from baseline to 3 months, analyzed using a linear mixed model approach. Results: Seventy-six participants (47% woman; median age, 57 [31-68] years; mean baseline vital capacity, 40 ± 18% predicted) were randomized (LVR, n = 37). Seventy-three participants completed the study. There was a statistically significant difference in MIC between groups (linear model interaction effect P = 0.002, observed mean difference, 0.19 [0.00-0.39] L). MIC increased by 0.13 (0.01-0.25) L in the LVR group, predominantly within the first month. No interaction or treatment effects were observed in secondary outcomes of lung volumes, respiratory system compliance, and quality of life. No adverse events were reported. Conclusions: Regular LVR increased MIC in a sample of LVR-naive participants with NMD. We found no direct evidence that regular LVR modifies respiratory mechanics or slows the rate of lung volume decline. The implications of increasing MIC are unclear, and the change in MIC may represent practice. Prospective long-term clinical cohorts with comprehensive follow-up, objective LVR use, and clinically meaningful outcome data are needed. Clinical trial registered with anzctr.org.au (ACTRN12615000565549).

Understanding the experiences of lung volume recruitment among boys with Duchenne muscular dystrophy: A multicenter qualitative study.

BACKGROUND: Despite recommendations for regular lung volume recruitment (LVR) use in clinical practice guidelines for children with neuromuscular disease, adherence to LVR is poor. We aimed to describe the experience of LVR by boys with Duchenne muscular dystrophy (DMD), their families, and healthcare providers (HCPs), as well as to identify the barriers and facilitators to LVR use. METHODS: This multicenter, qualitative study evaluated boys with DMD (n = 11) who used twice-daily LVR as part of a randomized controlled trial, as well as their parents (n = 11), and HCPs involved in the clinical use of LVR (n = 9). Semistructured interviews were conducted to identify participants' understanding of LVR therapy and their beliefs, barriers and facilitators to its use. Thematic analysis was conducted using an inductive approach. A subanalysis compared adherent and nonadherent children. RESULTS: Seven themes were identified related to participants' beliefs and experiences with LVR: emotional impact, adaptation to LVR, perceived benefits of LVR, routine, family engagement, clinical resources, and equipment-related factors. Strategies to improve adherence were also identified, including education, reinforcement and demonstration of LVR benefit, as well as clinician support. There were no thematic differences between adherent and nonadherent children. DISCUSSION: Despite the benefits of LVR and positive experiences with it by many families, there remain barriers to adherence to treatment. HCPs need to balance the need for early introduction to give families time to adapt to LVR while ensuring that the benefit of LVR outweighs the burden. Clinician support is important for family engagement.

Formal ventilation patient education for ALS predicts real-life choices.

Our objective was to evaluate a single-session, hands-on education programme on mechanical ventilation for ALS patients and caregivers in terms of knowledge, change in affect and to determine whether ventilator decisions made after the education sessions predict those made later in life. Questionnaires were administered to 26 patients and 26 caregivers on four separate occasions. The questionnaires assessed knowledge of ventilatory support, feedback on the nature of the education programme, as well as self-reported emotional well-being. All patients were followed until their death or until initiation of invasive ventilation. Both groups demonstrated significant improvements in knowledge as a result of the education session which was retained after one month. There was no change in patient or caregiver reports' self-reported emotional well-being. The choices of ventilatory support expressed at one month (T4) accurately predicted the real-life clinical choices made by 76% of patients. Any difference resulted from choosing palliative care. Hands-on patient and caregiver education results in improved knowledge, assists in decision-making with respect to ventilatory support, and is not associated with a worsening of affect. It also provides for an accurate prediction of real-life choices and avoids undesired life support interventions and critical care admissions.

Lung volume recruitment slows pulmonary function decline in Duchenne muscular dystrophy.

OBJECTIVE: To evaluate the long-term effect on measures of forced vital capacity (FVC) before and after the introduction of regular lung volume recruitment (LVR) maneuvers (breath-stacking) in individuals with Duchenne muscular dystrophy (DMD). DESIGN: Retrospective cohort study of pulmonary function data, including FVC, cough peak flow (CPF), maximum inspiratory pressure (MIP), and maximum expiratory pressure (MEP). Data were collected for 33 months prior to and 45 months after LVR introduction. SETTING: Ambulatory care in a tertiary level regional rehabilitation center in Canada. PARTICIPANTS: All individuals (N=22) with DMD (mean age ± SD, 19.6±2.4y), who were prescribed LVR and reported adherence with therapy. INTERVENTIONS: Introduction of regular LVR (breath-stacking); 3 to 5 maximal lung inflations (maximum insufflation capacity [MIC]) using a hand-held resuscitation bag and mouthpiece, twice daily. MAIN OUTCOME MEASURES: Measures included the rate of decline of FVC in percent-predicted, before and after the introduction of regular LVR. Changes in maximum pressures (MIP, MEP), MIC, and cough peak flows were also measured. RESULTS: At LVR initiation, FVC was 21.8±16.9 percent-predicted, and cough peak flows were <270L/min (144.8±106.9L/min). Annual decline of FVC was 4.7 percent-predicted a year before LVR and 0.5 percent-predicted a year after LVR initiation. The difference, 4.2 percent-predicted a year (95% confidence interval, 3.5-4.9; P<.000), represents an 89% improvement in the annual rate of FVC decline. CONCLUSIONS: The rate of FVC decline in DMD patients improves dramatically with initiation of regular LVR.

The experience of family caregivers of ventilator-assisted individuals who participated in a pilot web-based peer support program: A qualitative study.

Introduction: Family caregivers play an important role supporting the day-to-day needs of ventilator-assisted individuals (VAIs) living at home. Peer-to-peer communication can help support these caregivers and help them sustain caregiving in the community. Online peer-support has been suggested as a way to help meet caregivers' support needs. Methods: A qualitative descriptive approach was used to elicit the perspectives of support received from caregivers who participated in a pilot web-based peer support program from October to December 2018. Data were collected through the transcripts of weekly online peer-to-peer group chats. Data were analyzed using an integration of thematic and framework analysis. Results: In total, eight caregivers and five peer mentors participated in the pilot. All five mentors and four of the caregivers participated in the weekly chats. We identified three themes, a) The experience of caregivers is characterized by unique challenges related to the complexity of VAI care including technology; b) Mentors and caregiver participants reciprocally share support; c) Despite hardships, there are things that make caregiving easier and joyful. Discussion: Our results add to the growing body of evidence pointing to the importance of online communities for supporting vulnerable caregivers. The reciprocal element of peer support, where trained mentors and untrained participants both benefit from support, can help sustain peer-support interventions. Despite the challenges of providing care to a VAI, there are facilitators that may help ease the caregiving experience and caregivers can benefit from ongoing support that is tailored to their needs along the caregiving trajectory.

Cardiorespiratory management of Duchenne muscular dystrophy: emerging therapies, neuromuscular genetics, and new clinical challenges.

The life-limiting complications of Duchenne muscular dystrophy (DMD) include loss of lung function and progressive cardiomyopathy; when patients are treated with assisted ventilation, cardiac function becomes the main determinant of survival. Therapy for DMD is changing rapidly, with the emergence of new genetic and molecular therapeutic options, the proliferation of which has fostered the perception that DMD is a potentially curable disease. However, data for respiratory and cardiac outcomes are scarce and available evidence is not uniformly positive. Patients who share a dystrophin (DMD) genotype can have highly divergent cardiorespiratory phenotypes; genetic modifiers of DMD gene expression are a probable cause of respiratory and cardiac phenotypic variability and discordance. In this Personal View, we provide an overview of new and emerging DMD therapies, highlighting the limitations of current research and considering strategies to incorporate cardiorespiratory assessments into clinical trials. We explore how genetic modifiers could be used to predict cardiorespiratory natural history and how manipulation of such modifiers might represent a promising therapeutic strategy. Finally, we examine the changing role of respiratory physicians, cardiologists, and intensive care clinicians on the frontline of a challenging new clinical landscape.

The effect of emerging molecular and genetic therapies on cardiopulmonary disease in Duchenne muscular dystrophy.

Gene therapy is an attractive approach being intensively studied to prevent muscle deterioration in patients with Duchenne muscular dystrophy. While clinical trials are only in early stages, initial reports are promising for its effects on ambulation. Cardiopulmonary failure, however, is the most common cause of mortality in Duchenne muscular dystrophy (DMD) patients, and little is known regarding the prospects for gene therapy on alleviating DMD-associated cardiomyopathy and respiratory failure. Here we review current knowledge regarding effects of gene therapy on DMD cardiomyopathy and discuss respiratory endpoints that should be considered as outcome measures in future clinical trials.

Objective measurement of lung volume recruitment therapy: laboratory and clinical validation.

Lung volume recruitment manoeuvres are often prescribed to maintain respiratory health in neuromuscular disease. Unfortunately, no current system accurately records delivered dose. This study determined the performance characteristics of a novel, objective, manual lung volume recruitment bag counter ('the counter') with bench and healthy volunteer testing, as well as in individuals with neuromuscular disease. We undertook (1) bench test determination of activation threshold, (2) bench and healthy volunteer fidelity testing during simulated patient interface leak and different pressure compressions and (3) comparisons with self-report in individuals with neuromuscular disease. The data are reported as summary statistics, compression counts, percentage of recorded versus delivered compressions and concordance (Cohen's kappa (K) and absolute agreement). RESULTS: Minimum counter activation pressure under conditions of zero leak was 1.9±0.4 cm H2O. No difference was observed between the number of repetitions delivered and recorded during high airway pressure condition. Interface leak approximating 25% resulted in underestimation of repetition counts, and once the leak was at 50% or beyond, the counter recorded no activity. Faster sampling frequency collected data with more fidelity. Counter data agreed with diary self-report during community trials (16 participants, 960 participant days, 77% agreement, Cohen's Κ=0.66 and p<0.001). Disagreement typically favoured more diary reported (18%) than counter (5%) sessions. CONCLUSIONS: The performance characteristics of a new lung volume recruitment counter have been established in both laboratory and community settings. Objective usage and dosage data should accelerate new knowledge development and better translation of lung volume recruitment therapy into policy and practice.

The Oral Secretion Scale and Prognostic Factors for Survival in Subjects With Amyotrophic Lateral Sclerosis.

BACKGROUND: Noninvasive ventilation (NIV) can be tolerated in patients with amyotrophic lateral sclerosis (ALS), unless bulbar impairment becomes severe. Excessive oral secretions may result in NIV intolerance and insufficient ventilation. OBJECTIVE: To assess the reliability of the Oral Secretion Scale (OSS) for predicting the tolerance of NIV, when to initiate hospice or transition to tracheostomy, and prognostic factors for survival of users of NIV. METHODS: A validated OSS was developed to measure oral secretions in correlation with the ability to swallow saliva and clear the upper airway: OSS score of 4 = normal (automatic swallow); OSS score of 3 = infrequent secretions (automatic swallow decreased); OSS score of 2 = occasional drooling and/or pooling (conscious swallow required); OSS score of 1 = severe, frequent drooling and/or pooling (conscious swallow difficult); OSS score of 0 = most severe, constant drooling/pooling (conscious swallow impossible). A total of 137 subjects were followed up prospectively during ongoing patient visits from NIV initiation until death or tracheostomy. Survival was calculated by using Kaplan-Meier analysis. OSS scores when NIV became intolerable were determined. Uni- and/or multivariate Cox-regression analyses showed prognostic factors that affect survival. RESULTS: The median months of survival from NIV initiation were the following: 11 (95% CI 7.3-14.0), 5 (95% CI 3.1-6.1), and 1 (95% CI 1.1-1.5) stratified by OSS scores of 4, 3-2, and 1, respectively; and 21 (95% CI 8.6-33.2), 8 (95% CI 3.4-11.5), 6 (95% CI 4.2-8.2), and 2 (95% CI 1.5-2.7) stratified by 24, 17-23, 4-16, and <4 h/d of NIV use, respectively. Survival was significantly (P < .001) longer with an OSS score of 4 than an OSS score of 1 at NIV initiation; and significantly (P < .001) longer when NIV used 24 h/d than <24 h/d. In the subjects unable to tolerate NIV, ≥80% had OSS score of 1 or 0. Univariate and multivariate analyses hazard ratio 4.91, 95% CI 2.98-8.09, P < .001, and hazard ratio 4.60, 95% CI 2.66-7.96, P < .001), respectively, showed hours per day of NIV use was a significant factor associated with survival. CONCLUSIONS: The subjects with an OSS score of 4 tolerated NIV and survived significantly longer than subjects with an OSS score of <4. An OSS score of 1 signaled NIV intolerance and the need for hospice or transition to planned tracheostomy. Use of OSS can help guide NIV management decisions.

Healthcare utilization and costs of pediatric home mechanical ventilation in Canada.

BACKGROUND: Children using home mechanical ventilation (HMV) live at home with better quality of life, despite financial burden for their family. Previous studies of healthcare utilization and costs have not considered public and private expenditures, including family caregiver time. Our objective was to examine public and private healthcare utilization and costs for children using HMV, and variables associated with highest costs. METHODS: Longitudinal, prospective, observational cost analysis study (2012-2014) collecting data on public and private (out-of-pocket, third-party insurance, and caregiving) costs every 2 weeks for 6 months using the Ambulatory Home Care Record. Functional Independence Measure (FIM), WeeFIM, and Caregiving Impact Scale (CIS) were measured at baseline and study completion. Regression modeling examined a priori selected variables associated with monthly costs using Andersen and Newman's framework for healthcare utilization, relevant literature, and clinical expertise. Data are reported in 2015 Canadian dollars ($1CAD = $0.78USD). RESULTS: Forty two children and their caregivers were enrolled. Overall median (interquartile range) monthly healthcare cost was $12 131 ($8159-$15 958) comprising $9929 (89%) family caregiving hours, $996 (9%) publicly funded, and $252 (2%) out-of-pocket (<1% third-party insurance) costs. With higher FIM score (lower dependency), median costs were reduced by 4.5% (95% confidence interval: 8.3%-0.5%), adjusted for age, sex, tracheostomy, and daily ventilation duration. Note: since the three cost categories did not sum to the total statistically derived median cost, the percentage of each category used the sum of median public + caregiver lost time + private out-of-pocket + third-party insurance as the denominator. CONCLUSIONS: For HMV children, most healthcare costs were due to family caregiving costs. More dependent children incur highest costs. The financial burden to family caregivers is substantial and needs to considered in future policy decisions related to pediatric HMV.

Development of a Web-Based Peer Support Program for Family Caregivers of Ventilator-Assisted Individuals Living in the Community: Protocol for a Pilot Randomized Controlled Trial.

BACKGROUND: Across Europe, Canada, Australia, and the United States, the prevalence of home mechanical ventilation (HMV) prevalence is 6.6-12.9 per 100,000. At-home ventilator-assisted individuals (VAIs) are often vulnerable and highly comorbid, requiring complex care. In Canada, most VAI care is provided by family, leading to poor health-related quality of life and increased caregiver burden. No supportive interventions or peer support programs are tailored to VAI caregivers. Owing to the financial, geographic, and time limitations, Web-based support delivery may especially meet VAI family caregiver needs. We have developed a peer mentor training and Web-based peer support program for VAI caregivers including information-sharing, peer-to-peer communication, and peer mentorship. OBJECTIVE: Study Stage 1 aims to (1) evaluate the face and content validity of the peer mentor training program and (2) investigate participant satisfaction. Study Stage 2 aims to evaluate (1) the feasibility of participant recruitment and Web-based program delivery; (2) acceptability, usability, and satisfactoriness; (3) experiences of caregivers and peer mentors with the Web-based peer support program; and (4) effect of the Web-based peer support program on caregiver health outcomes. METHODS: Study Stage 1: We will train 7 caregivers to act as peer mentors for the Web-based peer support program trial; they will complete questionnaires rating the utility of individual training sessions and the training program overall. Study Stage 2: We will recruit 30 caregiver peers for a pilot randomized controlled trial of the 12-week Web-based peer support program using a waitlist control; the program includes private chat, a public discussion forum, and weekly moderated chats. Caregiver peers will be randomized to the intervention or waitlist control group using a 1:1 ratio using Randomize.net. Both groups will complete pre- and postintervention health outcome questionnaires (ie, caregiving impact, mastery, coping, personal gain, positive affect, and depression). Caregiver peers in the intervention arm will only complete a program evaluation and will be invited to participate in an interview to provide insight into their experience. Peer mentors will be invited to participate in a Web-based focus group to provide insight into their experience as mentors. We will judge the feasibility per the number of recruitment and program delivery goals met, use analysis of covariance to compare health outcomes between intervention and control groups, and analyze qualitative data thematically. RESULTS: Peer mentor training was completed with 5 caregivers in July 2018. To date, 2 caregivers have beta-tested the website, and the Web-based peer support program trial will commence in September 2018. Results are expected by early 2019. CONCLUSIONS: This study will result in the production and initial evaluation of a rigorously developed, evidence- and stakeholder-informed Web-based peer training and peer support program for caregivers of VAIs residing at home. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): PRR1-10.2196/11827.

Lung volume recruitment acutely increases respiratory system compliance in individuals with severe respiratory muscle weakness.

The aim of the present study was to determine whether lung volume recruitment (LVR) acutely increases respiratory system compliance (Crs) in individuals with severe respiratory muscle weakness (RMW). Individuals with RMW resulting from neuromuscular disease or quadriplegia (n=12) and healthy controls (n=12) underwent pulmonary function testing and the measurement of Crs at baseline, immediately after, 1 h after and 2 h after a single standardised session of LVR. The LVR session involved 10 consecutive supramaximal lung inflations with a manual resuscitation bag to the highest tolerable mouth pressure or a maximum of 50 cmH2O. Each LVR inflation was followed by brief breath-hold and a maximal expiration to residual volume. At baseline, individuals with RMW had lower Crs than controls (37±5 cmH2O versus 109±10 mL·cmH2O-1, p<0.001). Immediately after LVR, Crs increased by 39.5±9.8% to 50±7 mL·cmH2O-1 in individuals with RMW (p<0.05), while no significant change occurred in controls (p=0.23). At 1 h and 2 h post-treatment, there were no within-group differences in Crs compared to baseline (all p>0.05). LVR had no significant effect on measures of pulmonary function at any time point in either group (all p>0.05). During inflations, mean arterial pressure decreased significantly relative to baseline by 10.4±2.8 mmHg and 17.3±3.0 mmHg in individuals with RMW and controls, respectively (both p<0.05). LVR acutely increases Crs in individuals with RMW. However, the high airway pressures during inflations cause reductions in mean arterial pressure that should be considered when applying this technique.

Daytime Mouthpiece for Continuous Noninvasive Ventilation in Individuals With Amyotrophic Lateral Sclerosis.

BACKGROUND: Noninvasive ventilation (NIV) is commonly used to provide ventilatory support for individuals with amyotrophic lateral sclerosis (ALS). Once 24-h ventilation is required, the decision between invasive tracheostomy ventilation and palliation is often faced. This study describes the use and outcomes of daytime mouthpiece ventilation added to nighttime mask ventilation for continuous NIV in subjects with ALS as an effective alternative. METHODS: This was a retrospective study of 39 subjects with ALS using daytime mouthpiece ventilation over a 17-y period. RESULTS: Thirty-one subjects were successful with mouthpiece ventilation, 2 were excluded, 2 stopped because of lack of motivation, and 4 with bulbar subscores of the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (b-ALSFRS-R) between 0 and 3 physically failed to use it consistently. No subject in the successful group had a b-ALSFRS-R score of <6. Thirty of the successful subjects were able to generate a maximum insufflation capacity - vital capacity difference with lung volume recruitment. The median (range) survival to tracheostomy or death from initiation of nocturnal NIV and mouthpiece ventilation were 648 (176-2,188) and 286 (41-1,769) d, respectively. Peak cough flow with lung-volume recruitment >180 L/min at initiation of mouthpiece ventilation was associated with a longer survival (637 ± 468 vs 240 ± 158 d (P = .01). CONCLUSIONS: Mouthpiece ventilation provides effective ventilation and prolonged survival for individuals with ALS requiring full-time ventilatory support and maintaining adequate bulbar function.

Cough Augmentation Techniques in the Critically Ill: A Canadian National Survey.

BACKGROUND: Critically ill mechanically ventilated patients experience impaired airway clearance due to ineffective cough and impaired secretion mobilization. Cough augmentation techniques, including mechanical insufflation-exsufflation (MI-E), manually assisted cough, and lung volume recruitment, improve cough efficiency. Our objective was to describe use, indications, contraindications, interfaces, settings, complications, and barriers to use across Canada. METHODS: An e-mail survey was sent to nominated local survey champions in eligible Canadian units (ICUs, weaning centers, and intermediate care units) with 4 telephone/e-mail reminders. RESULTS: The survey response rate was 157 of 238 (66%); 78 of 157 units (50%) used cough augmentation, with 50 (64%) using MI-E, 53 (68%) using manually assisted cough, and 62 (79%) using lung volume recruitment. Secretion clearance was the most common indication (MI-E, 92%; manually assisted cough, 88%; lung volume recruitment, 76%), although the most common units (44%) used it <50% of the time. Use during weaning from invasive (MI-E, 21%; manually assisted cough, 39%; lung volume recruitment, 3%) and noninvasive ventilation (MI-E, 21%; manually assisted cough, 33%; lung volume recruitment, 21%) was infrequent. The most common diagnoses were neuromuscular disease (97%) and spinal cord injury (83%). Pneumothorax was the most frequently identified absolute contraindication for MI-E (93%) and lung volume recruitment (83%); rib fracture was most frequently identified for manually assisted cough (69%). MI-E mean inspiratory pressure was 31 cm H2O, and expiratory pressure was -32 cm H2O. Mucus plugging requiring tracheostomy inner change was the most frequent complication for MI-E (23%), chest pain for manually assisted cough (36%), and hypotension for lung volume recruitment (17%). The most commonly cited barriers were lack of expertise (70%), knowledge (65%), and resources (52%). CONCLUSIONS: We found moderate adoption of cough augmentation techniques, particularly for secretion management. Lack of expertise and knowledge are potentially modifiable barriers addressed with educational interventions.

Loss of Dignity in Severe Chronic Obstructive Pulmonary Disease.

CONTEXT: The maintenance of dignity is an important concept in palliative care, and the loss of dignity is a significant concern among patients with advanced cancer. OBJECTIVES: The goals of this study were to examine whether loss of dignity is also a concern for patients receiving interdisciplinary rehabilitation for Stage III or IV chronic obstructive pulmonary disease. We examined the prevalence and correlates of loss of dignity and determined whether it improves with treatment. METHODS: Inpatients underwent a structured interview inquiry around their sense of dignity and completed measures of pulmonary, physical, and psychological function at admission (n = 195) and discharge (n = 162). RESULTS: Loss of dignity was identified as a prominent ongoing concern for 13% of patients. It was correlated with measures of depression and anxiety sensitivity, but not with pulmonary capacity or functional performance. A robust improvement in loss of dignity was demonstrated, with 88% of those who reported a significant problem at admission no longer reporting one at discharge. CONCLUSION: The prevalence of a problematic loss of dignity among patients with severe chronic obstructive pulmonary disease is at least as high as among those receiving palliative cancer care. Loss of dignity may represent a concern among people with medical illnesses more broadly, and not just in the context of "death with dignity" at the end of life. Furthermore, interdisciplinary care may help to restore a sense of dignity to those individuals who are able to participate in rehabilitation.

Evaluation of a decision aid for making choices about intubation and mechanical ventilation in chronic obstructive pulmonary disease.

To assist patients with chronic obstructive pulmonary disease (COPD) in advance planning for life-threatening exacerbations, we developed a structured decision aid that describes the process, risks, and outcomes of intubation and mechanical ventilation (MV). Thirty-three patients with severe COPD took part in a before-after evaluation study. At baseline, only two participants (6%) reported that they had already made an advance decision about MV. After reviewing the decision aid, 31 participants (94%) reported that they had made a choice, which in 23 cases (74% of those deciding) was to forego MV. These choices were associated with more accurate expectations of MV outcome, and reduced decisional conflict. Qualitatively, participants who would accept MV emphasized their wish to prolong life, whereas those who would forego MV were more influenced by the burdens of treatment and the perception of a poor long-term outcome. However, there was evidence that 24% of participants did not completely comprehend the decision aid and 27% found the experience to be stressful. These findings indicate that a decision aid for MV helps patients plan for life-threatening exacerbations, and may be a useful adjunct to counseling for some patients with severe COPD.

A Breathlessness Catastrophizing Scale for chronic obstructive pulmonary disease.

OBJECTIVE: Catastrophizing about breathlessness may be related to disability in patients with chronic obstructive pulmonary disease (COPD), but assessment options are limited. This study reports the initial validation of a 13-item Breathlessness Catastrophizing Scale (BCS). METHOD: Pulmonary rehabilitation inpatients completed spirometric, functional performance and questionnaire assessments at admission (N=242) and discharge (n=186). RESULTS: The BCS comprised a unifactorial scale that demonstrated excellent internal consistency (Cronbach's alpha=.96) and correlated with measures of anxiety sensitivity, depression, and self-efficacy, but not with performance on walk and stair-climbing tests. BCS scores improved robustly with rehabilitation, approaching a medium effect size (d=.43), and demonstrated a modest association with enhanced performance in a stair-climbing test of exercise tolerance. CONCLUSION: The BCS is a reliable measure of catastrophizing in severe COPD that has good convergent validity and sensitivity to change. Its association with functional performance requires further investigation. However, it appears that a high level of catastrophizing about breathlessness is not a barrier to functional improvement with inpatient pulmonary rehabilitation.

Home mechanical ventilation in Canada: a national survey.

BACKGROUND: No comprehensive Canadian national data describe the prevalence of and service provision for ventilator-assisted individuals living at home, data critical to health-care system planning for appropriate resourcing. Our objective was to generate national data profiling service providers, users, types of services, criteria for initiation and monitoring, ventilator servicing arrangements, education, and barriers to home transition. METHODS: Eligible providers delivering services to ventilator-assisted individuals (adult and pediatric) living at home were identified by our national provider inventory and referrals from other providers. The survey was administered via a web link from August 2012 to April 2013. RESULTS: The survey response rate was 152/171 (89%). We identified 4,334 ventilator-assisted individuals: an estimated prevalence of 12.9/100,000 population, with 73% receiving noninvasive ventilation (NIV) and 18% receiving intermittent mandatory ventilation (9% not reported). Services were delivered by 39 institutional providers and 113 community providers. We identified variation in initiation criteria for NIV, with polysomnography demonstrating nocturnal hypoventilation (57%), daytime hypercapnia (38%), and nocturnal hypercapnia (32%) as the most common criteria. Various models of ventilator servicing were reported. Most providers (64%) stated that caregiver competency was a prerequisite for home discharge; however, repeated competency assessment and retraining were offered by only 45%. Important barriers to home transition were: insufficient funding for paid caregivers, equipment, and supplies; a shortage of paid caregivers; and negotiating public funding arrangements. CONCLUSIONS: Ventilatory support in the community appears well-established, with most individuals managed with NIV. Although caregiver competency is a prerequisite to discharge, ongoing assessment and retraining were infrequent. Funding and caregiver availability were important barriers to home transition.