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Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was shown to be safe and efficacious in people with cystic fibrosis (CF) heterozygous for F508del and a minimal function mutation (F/MF) or homozygous for F508del (F/F) in two pivotal Phase 3 trials, significantly improving percentage predicted forced expiratory volume in 1 second, Cystic Fibrosis Questionnaire-Revised, Respiratory Domain (CFQ-R RD) scores, and sweat chloride concentration. Here, we analyzed the 11 non-respiratory domains (non-RDs) of the CFQ-R, which assess general health-related quality of life (i.e., Physical Functioning, Role Functioning, Vitality, Health Perceptions, Emotional Functioning, and Social Functioning) and quality of life impacted by CF (i.e., Body Image, Eating Problems, Treatment Burden, Weight, and Digestive Symptoms), for participants in these two Phase 3 trials. ELX/TEZ/IVA treatment led to higher scores in all CFQ-R non-RDs, with improvements in most domains compared with control treatments. These findings demonstrate that ELX/TEZ/IVA improves a range of CF-specific symptoms and general functioning and well-being.

Background: Evidence-based guidelines are needed for effective delivery of home oxygen therapy to appropriate patients with chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD). Methods: The multidisciplinary panel created six research questions using a modified Delphi approach. A systematic review of the literature was completed, and the Grading of Recommendations Assessment, Development and Evaluation approach was used to formulate clinical recommendations. Recommendations: The panel found varying quality and availability of evidence and made the following judgments: 1) strong recommendations for long-term oxygen use in patients with COPD (moderate-quality evidence) or ILD (low-quality evidence) with severe chronic resting hypoxemia, 2) a conditional recommendation against long-term oxygen use in patients with COPD with moderate chronic resting hypoxemia, 3) conditional recommendations for ambulatory oxygen use in patients with COPD (moderate-quality evidence) or ILD (low-quality evidence) with severe exertional hypoxemia, 4) a conditional recommendation for ambulatory liquid-oxygen use in patients who are mobile outside the home and require >3 L/min of continuous-flow oxygen during exertion (very-low-quality evidence), and 5) a recommendation that patients and their caregivers receive education on oxygen equipment and safety (best-practice statement). Conclusions: These guidelines provide the basis for evidence-based use of home oxygen therapy in adults with COPD or ILD but also highlight the need for additional research to guide clinical practice.

Rationale: Cough and phlegm are common symptoms of chronic obstructive pulmonary disease (COPD) and may significantly affect quality of life. This study assessed the burden of cough and phlegm on clinical outcomes and quality of life among people with a self-reported physician diagnosis of COPD. Methods: Patient-reported data from the COPD Foundation’s Patient-Powered Research Network (COPD PPRN) were utilized. Cough and phlegm severity and frequency were assessed by responses to questions on the COPD Assessment Test (CAT) and categorized into none/low, moderate and severe. Quality of life domains were evaluated using the Patient-Reported Outcome Measurement Information System (PROMIS-29). Associations between cough and phlegm levels and PROMIS-29 domains were examined using multivariate analysis of variance (MANOVA). Results: The 5286 participants were average age 64.4 years (SD=11.4), 87.9% white, 60.4% female, 51.2% married, and 42.2% with caregivers. Approximately three-fourths of the participants had moderate or severe cough or phlegm levels. Respondents with moderate and high cough or phlegm had significantly worse dyspnea (p

Background Asthma is a chronic respiratory disease that affects millions worldwide. Medication management is the current mainstay of treatment; however, there is evidence to suggest additional benefit with lifestyle changes, particularly with increased physical activity. Objective To discover and evaluate the effects of physical activity on asthma outcomes. Methods Systematic search of PubMed, Excerpta Medica database, Cumulative Index to Nursing and Allied Health Literature, Cochrane Library, Rehabilitation and Sports Medicine Source, Scopus, and Web of Science identified 11,155 results. Thirty-five articles met our inclusion criteria spanning 20 studies. Data extraction was conducted by 6 independent reviewers, and final results were evaluated by a seventh reviewer and the senior author. Results Wide variation among selected studies, including the heterogeneity of interventions and outcome variables, did not support a meta-analysis. Mixed results of the effects of physical activity on asthma outcomes were found. Most studies suggest that physical activity improves asthma control, quality of life, lung function parameters, and inflammatory serologies, whereas 3 found no improvements in any of these outcomes. No studies reported worsening asthma outcomes. Conclusions This review highlights the emerging and promising role of physical activity as a nonpharmacologic treatment for asthma. Additional high-quality randomized controlled trials are needed to overcome the problems of measurement heterogeneity and the dilution of outcome effect size measurement related to physical activity interventions for asthma.

BACKGROUND: The majority of prescriptions for supplemental oxygen are written when patients are discharged to home from the hospital and the evaluation of these patients is inconsistent. Respiratory Therapists receive training in the evaluation and management of patients needing oxygen. The primary goal of the study was to estimate the frequency with which respiratory therapists (RTs) evaluate the need for home oxygen in patients hospitalized for COPD exacerbations before discharge. METHODS: An online questionnaire was distributed to RTs in the United States by the American Association for Respiratory Care. RTs were asked to indicate how frequently they evaluate the need for home oxygen on an ordinal scale: Never, Rarely/occasionally, Sometimes, Most of the time, Almost every time, or Every time. Consistent evaluation for home oxygen was defined as performing an evaluation for home oxygen therapy Almost every time or Every time (ie, > 75% of the time). Bivariate and multivariable analyses were assessed using the Fisher exact test and logistic regression models. RESULTS: Of 611 respondents, 490 were eligible for analysis. Fifty-eight percent of RTs reported consistently evaluating patients for home oxygen at rest, whereas 43% reported doing so during activity and 14% during sleep. Consistent evaluation for home oxygen requirements at rest was significantly associated with more years of practice (P = .03; highest among RTs with ≥ 30 y of practice at 40%), region of practice (P = .001; highest in the Midwest at 44%), and greater familiarity with criteria for home oxygen (P < .001; highest among RTs who selected Very familiar with guidelines from the Centers for Medicare and Medicaid Services at 58%). Practice in the Midwest and greater familiarity with criteria for home oxygen was associated with consistent evaluation for home oxygen during activity. Practice in the Midwest (vs Northeast; adjusted odds ratio 2.56, P < .001) and being very familiar with home oxygen criteria (vs not at all familiar; adjusted odds ratio 5.66, P < .001) were independently associated with a higher odds of evaluating for home oxygen at rest and with activity. Only 25% of RTs were involved in making decisions about home oxygen equipment. CONCLUSIONS: RTs do not consistently evaluate patients hospitalized for COPD exacerbations for home oxygen prior to discharge, and only a minority of RTs are involved in selecting home oxygen equipment.

Background: Low physical activity in patients with chronic obstructive pulmonary disease (COPD) is associated with increased morbidity and mortality. To inform the design of a home-based physical activity promotion program for patients with COPD recently discharged from a minority-serving hospital, we conducted a cohort study to evaluate objectively measured daily physical activity and patient-reported outcomes. Methods: This was a 12-week prospective cohort study of patients with a physician diagnosis of COPD recently hospitalized (≤ 12 weeks) for respiratory symptoms. Daily physical activity was recorded using wrist-based and “clip-on” pedometers, and analyzed as mean daily step counts averaged over 7 days. Results: Twenty-two patients were enrolled a median (interquartile range, [IQR]) of 14 (7 to 29) days after hospital discharge. The median daily step count (IQR) in the first week after enrollment (week 1) was 3710 (1565 to 5129) steps. The median within-person change in daily step count (IQR) from week 1 to week 12 was 314 (-30 to 858) steps (p=0.28). Within-person correlation of week-to-week daily step counts was high (r ≥ 0.75). Time from hospital discharge to enrollment was not correlated with mean daily step counts on week 1 (r= -0.13) and only weakly correlated with change in mean daily step counts from week 1 to week 12 (r=0.37). Conclusions: Daily physical activity was variable in this cohort of recently hospitalized patients with COPD, but with little within-person change over a 12-week period. These observations highlight the need for flexible physical activity promotion programs addressing the needs of a heterogeneous patient population.

Just over 100 years ago, John Scott Haldane published a seminal report about the therapeutic potential of supplemental oxygen to treat hypoxemia. In the 1980s, a pair of clinical trials confirmed the benefit of long-term oxygen therapy in improving survival in patients with COPD associated with severe resting hypoxemia. This review provides a summary of evidence supporting long-term and short-term oxygen therapy, as well as the various types of oxygen equipment commonly used in homes to deliver supplemental oxygen. Because the majority of orders for home oxygen occur at hospital discharge following acute illness, a typical conversation between a patient and their pulmonologist following a COPD exacerbation is presented. The SHERLOCK Consortium, a multi-stakeholder group established following the publication of the COPD National Action Plan in 2017 is also detailed. Interim results of the SHERLOCK Consortium, which suggest a chain of care involving 9 steps to ensure that patients are successfully initiated on home oxygen therapy during transitions from hospital to home, are presented. Recommendations to support evidence-based policies in this high-risk population are provided.

Previous work indicates the potential for community health workers and peer coaches serving as patient navigators to improve processes of care and health outcomes during care transitions, but have not been sufficiently tested to determine if such programs improve measures of patient experience in minority serving institutions. The objectives of the Patient Navigator to Reduce Readmissions (PArTNER) study was to: 1) conduct a pragmatic clinical effectiveness trial comparing a multi-faceted, stakeholder-supported Navigator intervention (in-person CHW visits in the hospital and after hospital discharge, plus telephone-based peer coaching) versus usual care on the experience of hospital-to-home care transitions in patients hospitalized with heart failure, pneumonia, chronic obstructive pulmonary disease, myocardial infarction, or sickle cell disease; 2) examine the effectiveness of the Navigator intervention in patient subgroups; and 3) understand the barriers and facilitators of successfully implementing the Navigator intervention across patient populations. The co-primary outcomes are the 30-day changes in: 1) Patient Reported Outcomes Measurement Information System (PROMIS) emotional distress-anxiety, and 2) PROMIS informational support. Secondary outcomes at 30 and 60 days include other PROMIS health measures and hospital readmissions. Innovative features of the PArTNER study include early and continuous engagement of patients, their caregivers, clinicians, health system administrators, and other stakeholders to inform the design and implementation of the Navigator intervention. In this report, we describe the design of the PArTNER study. Keywords: Pragmatic clinical trial, Hospital-to-home transition, Hospital readmissions, Community health worker, Peer coaching

Rationale: Patients hospitalized with chronic obstructive pulmonary disease (COPD) who require supplemental oxygen (O2) are at increased risk of hospital readmissions. There is a paucity of information regarding quality of evaluation and documentation regarding the need for supplemental O2 in this population. Objective: To determine the extent to which evaluation and documentation regarding the need for supplemental O2 occurs prior to hospital discharge in patients with COPD. Methods: We conducted a two-center retrospective cohort study of hospitalized adults with a physician diagnosis of COPD. We reviewed electronic health records to ascertain whether patients underwent evaluation beyond rest oximetry documenting hypoxemia and if there was adequate documentation of supplemental O2 requirements prior to discharge. Results: Of 526 patients hospitalized with a primary or secondary discharge diagnosis of COPD, 335 patients (mean age 69 years, 78% with diagnosis of COPD exacerbation) met eligibility criteria. Overall, 1 in 5 (22%, 73/335) hospitalized patients with COPD had an evaluation beyond rest oximetry for supplemental O2 requirements during admission. Adequate documentation of supplemental O2 requirements occurred in even fewer patients (16%, 54/335). Both evaluation (26% versus 5%, p=0.002) and documentation (19% versus 4%, p=0.001) of supplemental O2 requirements were more common in patients hospitalized for a COPD exacerbation compared to those hospitalized with COPD but without an exacerbation. Conclusions: Evaluation and documentation of supplemental O2 requirements beyond rest oximetry occur infrequently in patients hospitalized with COPD.

More than 1.5 million adults in the United States use supplemental oxygen for a variety of respiratory disorders to improve their quality of life and prolong survival. This document describes recommendations from a multidisciplinary workshop convened at the ATS International Conference in 2017 with the goal of optimizing home oxygen therapy for adults. Ideal supplemental oxygen therapy is patient-specific, provided by a qualified clinician, includes an individualized prescription and therapeutic education program, and offers oxygen systems that are safe, promote mobility, and treat hypoxemia. Recently, patients and clinicians report a growing number of problems with home oxygen in the United States. Oxygen users experience significant functional, mechanical, and financial problems and a lack of education related to their oxygen equipment-problems that impact their quality of life. Health care providers report a lack of readily accessible resources needed to prescribe oxygen systems correctly and efficiently. Patients with certain lung diseases are affected more than others because of physically unmanageable or inadequate portable systems. Analysis is needed to quantify the unintended impact that the Centers for Medicare and Medicaid Services Competitive Bidding Program has had on patients receiving supplemental oxygen from durable medical equipment providers. Studies using effectiveness and implementation research designs are needed to develop and evaluate new models for patient education, identify effective ways for stakeholders to interface, determine the economic benefit of having respiratory therapists perform in-home education and follow-up testing, and collaborate with technology companies to improve portable oxygen devices. Generation of additional evidence of the benefit of supplemental oxygen across the spectrum of advanced lung diseases and the development of clinical practice guidelines should both be prioritized.

Commercially available pedometers have been used as tools to measure endpoints in studies evaluating physical activity promotion programs. However, their accuracy in patients recovering from COPD exacerbations is unknown. The objectives of this study were to 1) assess the relative accuracy of different commercially available pedometers in healthy volunteers and 2) evaluate the accuracy of the top-performing commercially available pedometer in patients recovering from COPD exacerbations following hospital discharge.Twelve healthy volunteers wore 2 pedometers, 2 smartphones with pedometer apps and an accelerometer for 15 minutes of indoor activity. The top-performing device in healthy volunteers was evaluated in 4 patients recovering from COPD exacerbations following hospital discharge during 6 minutes of walking performed at home. Bland-Altman plots were employed to evaluate accuracy of each device compared with direct observation (the reference standard).In healthy volunteers, the mean percent error compared to direct observation of the various devices ranged from -49% to +1%. The mean percent error [95% confidence interval (CI)] of the top-performing device in healthy volunteers, the Fitbit ZipThe accuracy of commercially available pedometers in healthy volunteers is highly variable. The top-performing pedometer in our study, the Fitbit Zip

Limited socioeconomic resources contribute to high readmission rates at minority serving institutions (MSIs). A better understanding of patient-level factors and need for patient navigators could inform approaches to enhance care transitions tailored to these vulnerable patient populations. We sought to understand the perspectives of patients and their caregivers about hospital to home transitions from an MSI, as well as their attitudes about patient navigators to facilitate care transitions. We conducted qualitative research using focus groups (FGs)-five disease-specific patient FGs and two caregiver FGs, including 23 patients and 10 caregivers. Findings support the need for additional services to address: (1) gaps in the hospital discharge; (2) socioeconomic resources; (3) access to post-discharge care; (4) patient's health care seeking behaviors; (5) patient anxiety; (6) self-management education; and (7) social supports for patients and caregivers. While caregivers uniformly expressed interest in patient navigators, support for navigators among patients was more variable.

Patients are increasingly viewed as key stakeholders who can contribute in meaningful ways to clinical research and are emphasized in research funded by the Patient-Centered Outcomes Research Institute (PCORI). We are not aware of other peer-reviewed publications that report methods and outcomes of patient engagement to refine study design for a PCORI-sponsored clinical effectiveness trial. The aim of this report was to describe the process and outcomes of involving patients in the design of a clinical trial to promote adherence to supplemental oxygen therapy among patients with chronic obstructive pulmonary disease. In-person focus groups and individual discussions via telephone and email were used to elicit feedback to refine the intervention and clarify outcomes of highest importance to patients. A total of 25 patients and five caregivers provided feedback. Their feedback has informed decisions regarding the length of intervention sessions (20 min and in some cases longer was acceptable), the importance of including caregivers, and discussion topics (e.g., social discomfort about using oxygen in public, identifying personally relevant reasons to use oxygen, pulmonary rehabilitation). Multiple outcomes were rated as highly important to patients (physical function, fatigue, sleep, anxiety, depression, and ability to participate in social roles and activities), and the outcome that was ranked as most important varied by individual. Therefore, multiple patient-reported outcomes will be used as endpoints for the clinical trial. Patient involvement led to refinements of the intervention and clinical trial endpoints to better address the expressed needs and concerns of patients and caregivers.

We have developed GaitTrack, a phone application to detect health status while the smartphone is carried normally. GaitTrack software monitors walking patterns, using only accelerometers embedded in phones to record spatiotemporal motion, without the need for sensors external to the phone. Our software transforms smartphones into health monitors, using eight parameters of phone motion transformed into body motion by the gait model. GaitTrack is designed to detect health status while the smartphone is carried during normal activities, namely, free-living walking. The current method for assessing free-living walking is medical accelerometers, so we present evidence that mobile phones running our software are more accurate. We then show our gait model is more accurate than medical pedometers for counting steps of patients with chronic disease. Our gait model was evaluated in a pilot study involving 30 patients with chronic lung disease. The six-minute walk test (6 MWT) is a major assessment for chronic heart and lung disease, including congestive heart failure and especially chronic obstructive pulmonary disease (COPD), affecting millions of persons. The 6 MWT consists of walking back and forth along a measured distance for 6 minutes. The gait model using linear regression performed with 94.13% accuracy in measuring walk distance, compared with the established standard of direct observation. We also evaluated a different statistical model using the same gait parameters to predict health status through lung function. This gait model has high accuracy when applied to demographic cohorts, for example, 89.22% accuracy testing the cohort of 12 female patients with ages 50-64 years.

About 1 in 5 patients hospitalized for exacerbations of chronic obstructive pulmonary disease (COPD) in the United States are readmitted within 30 days. The U.S. Centers for Medicare and Medicaid Services has recently expanded its Hospital Readmissions Reduction Program to financially penalize hospitals with higher than expected all-cause 30-day readmission rates following a hospitalization for COPD exacerbation. In October 2013, the COPD Foundation convened a multi-stakeholder National COPD Readmissions Summit to summarize our understanding of how to reduce hospital readmissions in patients hospitalized for COPD exacerbations. Over 225 individuals participated in the Summit, including patients, clinicians, health service researchers, policy makers and representatives of academic health care centers, industry, and payers. Summit participants recommend that programs to reduce hospital readmissions: 1) Include specific recommendations about how to promote COPD self-management skills training for patients and their caregivers; 2) Adequately address co-existing disorders common to COPD in care plans during and after hospitalizations; 3) Include an evaluation of adverse events when implementing strategies to reduce hospital readmissions; and 4) Develop a strategy (e.g., a learning collaboratory) to connect groups who are engaged in developing, testing, and implementing programs to reduce hospital readmissions for COPD and other conditions.

Approximately 20% of patients hospitalized for COPD exacerbations in the United States will be readmitted within 30 days. The Centers for Medicare and Medicaid Services has recently proposed to revise the Hospital Readmissions Reduction Program to financially penalize hospitals with high all-cause 30-day rehospitalization rates after a hospitalization for COPD exacerbation on or after October 1, 2014.To report the results of a systematic review of randomized clinical trials evaluating interventions to reduce the rehospitalizations after COPD exacerbations.Multiple electronic databases were systematically searched to identify relevant studies published between January 1966 and June 2013. Titles, abstracts, and, subsequently, full-text articles were assessed for eligibility. Each study was appraised using predefined criteria.Among 913 titles and abstracts screened, 5 studies (1,393 participants) met eligibility criteria. All studies had a primary outcome of rehospitalization at 6 or 12 months. No study examined 30-day rehospitalization as the primary outcome. Each study tested a different set of interventions. Two studies (one conducted in Canada and one conducted in Spain and Belgium) showed a decrease in all-cause rehospitalization over 12 months in the intervention group versus comparator group (mean number of hospitalizations per patient, 1.0 vs. 1.8; P = 0.01; percent hospitalized, 45 vs. 67%; P = 0.028; respectively). The only study conducted in the United States found a greater than twofold higher risk of mortality in the intervention group (17 vs. 7%, P = 0.003) but no significant difference in rehospitalizations. It was unclear which set of interventions was effective or harmful.The evidence base is inadequate to recommend specific interventions to reduce rehospitalizations in this population and does not justify penalizing hospitals for high 30-day rehospitalization rates after COPD exacerbations.

About 20% of patients hospitalized for chronic obstructive pulmonary disease (COPD) exacerbations are readmitted within 30 days. High 30-day risk-standardized readmission rates after COPD exacerbations will likely place hospitals at risk for financial penalties from the Centers for Medicare and Medicaid Services starting in fiscal year 2015. Factors contributing to hospital readmissions include healthcare quality, access to care, coordination of care between hospital and ambulatory settings, and factors linked to socioeconomic resources (e.g., social support, stable housing, transportation, and food). These concerns are exacerbated at minority-serving institutions, which provide a disproportionate share of care to patients with low socioeconomic resources. Solutions tailored to the needs of minority-serving institutions are urgently needed. We recommend research that will provide the evidence base for strategies to reduce readmissions at minority-serving institutions. Promising innovative approaches include using a nontraditional healthcare workforce, such as community health workers and peer-coaches, and telemedicine. These strategies have been successfully used in other conditions and need to be studied in patients with COPD.

Background Objective measurement of airflow obstruction by spirometry is an essential part of the diagnosis of asthma or COPD. During exacerbations, the feasibility and utility of spirometry to confirm the diagnosis of asthma or chronic obstructive pulmonary disease (COPD) are unclear. Addressing these gaps in knowledge may help define the need for confirmatory testing in clinical care and quality improvement efforts. This study was designed to determine the feasibility of spirometry and to determine its utility to confirm the diagnosis in patients hospitalized with a physician diagnosis of asthma or COPD exacerbation. Methods Multi-center study of four academic healthcare institutions. Spirometry was performed in 113 adults admitted to general medicine wards with a physician diagnosis of asthma or COPD exacerbation. Two board-certified pulmonologists evaluated the spirometry tracings to determine the proportion of patients able to produce adequate quality spirometry data. Findings were interpreted to evaluate the utility of spirometry to confirm the presence of obstructive lung disease, according to the 2005 European Respiratory Society/American Thoracic Society recommendations. Results There was an almost perfect agreement for acceptability (κ = 0.92) and reproducibility (κ =0.93) of spirometry tracings. Three-quarters (73%) of the tests were interpreted by both pulmonologists as being of adequate quality. Of these adequate quality tests, 22% did not present objective evidence of obstructive lung disease. Obese patients (BMI ≥30 kg/m2) were more likely to produce spirometry tracings with no evidence of obstructive lung disease, compared to non-obese patients (33% vs. 8%, p = 0.007). Conclusions Adequate quality spirometry can be obtained in most hospitalized adults with a physician diagnosis of asthma or COPD exacerbation. Confirmatory spirometry could be a useful tool to help reduce overdiagnosis of obstructive lung disease, especially among obese patients.