Chronic Respiratory Disease最新文献

{"title":"Real-world disease burden, mortality, and healthcare resource utilization associated with bronchiectasis.","authors":"Sofia Shoaib, Joseph Feliciano, Elliott C Dasenbrook, Jason Maynard, Lakshmi Batchu, Maitreyee Mohanty, Melanie Lauterio, Alexandra J Feld","doi":"10.1177/14799731241310897","DOIUrl":"10.1177/14799731241310897","url":null,"abstract":"<p><strong>Objectives: </strong>To assess real-world survival and healthcare resource utilization (HCRU) in US patients with non-cystic fibrosis bronchiectasis (NCFBE).</p><p><strong>Methods: </strong>This retrospective analysis, using data from the STATinMED RWD Insights database from Jan 2015-Oct 2022, included adults with NCFBE (from Jan 2015-Oct 2021) and non-NCFBE comparators (from Jan 2015-Aug 2020); baseline characteristics were balanced by inverse probability treatment weighting. Outcomes included survival through end of study. HCRU was assessed over 12 months.</p><p><strong>Results: </strong>117,718 patients with NCFBE and 306,678 comparators were included. Patients with NCFBE had a 77% higher risk of death than comparators (hazard ratio [HR] 1.77 [95% CI 1.74-1.80]). Risk of death was higher among patients aged ≥65 years (vs 18-34 years; HR 11.03 [95% CI 10.36-11.74]), among Black patients (vs White; HR 1.53 [95% CI 1.50-1.55]), and among patients with comorbid COPD (HR 1.42 [95% CI 1.40-1.44]). Patients with NCFBE incurred higher all-cause and respiratory-related HCRU than comparators for outpatient office, outpatient hospital, emergency department (ED), inpatient and respiratory-related pulmonologist visits (all <i>p</i> < .0001); HCRU increased with exacerbations.</p><p><strong>Conclusions: </strong>Patients with NCFBE have high mortality burden and incur high HCRU, both of which are further increased with exacerbations. Prevention and delay of exacerbations are key areas for improvement of disease management.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"22 ","pages":"14799731241310897"},"PeriodicalIF":3.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11808752/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143381718","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Educational needs of people with COPD or asthma entering pulmonary rehabilitation and their significant others: A cross-sectional study.","authors":"Anouk Jl Muijsenberg, Sarah Houben-Wilke, Jan Tatousek, Joyca Lacroix, Martijn A Spruit, Daisy Ja Janssen","doi":"10.1177/14799731251316891","DOIUrl":"10.1177/14799731251316891","url":null,"abstract":"<p><p><b>Objectives:</b> This study aimed to identify educational needs of people with COPD or asthma referred for pulmonary rehabilitation and their significant others. <b>Methods:</b> In this cross-sectional study, information needs were assessed by the Lung Information Needs Questionnaire. Learning styles were assessed with a multiple-choice question (visual, aural, read/write and kinaesthetic - multiple choices were possible). Psychological profiles were assessed by an instrument distinguishing four profiles: fighter, analyst, optimist or sensitive. <b>Results:</b> 121 patients (57% female; age: 65 ± 10 years; 81% COPD) and 67 significant others (55% female; age: 61 ± 12 years) were included. Patients and their significant others had a variety of information needs. Patients and their significant others mostly preferred the aural (39% and 49%, respectively) and read/write learning styles (31% and 36%, respectively) as unimodal learners. 49% of patients and 51% of significant others preferred more than one learning style. Most prevalent psychological profiles were optimist in patients (36%) and fighter in significant others (59%). <b>Discussion:</b> The identified variety in educational needs among people with COPD or asthma and their significant others underlines the importance of addressing this diversity of individual needs in education. Learners should have the opportunity to choose among a variety of topics and have access to multiple learning styles. Furthermore, educational interventions should consider to incorporate different communication strategies.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"22 ","pages":"14799731251316891"},"PeriodicalIF":3.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11898093/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143604231","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical and socioeconomic parameters as predictors for longer hospital stay and mortality in COPD.","authors":"Dekel Shlomi, Michal Benderly, Liraz Olmer, Ofra Kalter-Leibovici","doi":"10.1177/14799731251355445","DOIUrl":"10.1177/14799731251355445","url":null,"abstract":"<p><p>BackgroundIdentifying clinical and socio-economic predictors of adverse health outcomes in chronic obstructive pulmonary disease (COPD) patients is imperative for reducing exacerbation and mortality rates.MethodsThe COPD Community Disease Management (COPD-CDM) clinical trial evaluated the efficacy of a disease management program among 1202 ambulatory COPD patients receiving recommended care. The study did not find disease management to be superior to recommended care only, in preventing COPD hospital admissions or mortality. This post-hoc analysis examined the association of clinical and socioeconomic parameters with the length of in-hospital stay and all-cause mortality by multivariable non-linear mixed and Cox proportional hazards models, adjusted for age, sex, study arm, recruitment and study period, and medical therapy.ResultsA one-point increment in the Modified Medical Research Council dyspnea scale was associated with a higher hazard for all-cause mortality, (Hazards Ratio [HR]:1.50, 95% confidence interval [CI]: 1.22-1.85); longer in-hospital stay for COPD or all causes; Rate Ratio (95% CI): 1.64 (1.36-1.98), and 1.36 ( 1.19-1.55), respectively. A 50 m increment in six-minute walking distance was associated with fewer days in-hospital for both COPD and all causes; Rate Ratio (95% CI); 0.94 (0.89-0.99) and 0.95 (0.92-0.99), respectively. Unemployment and lower educational attainment were associated with a longer in-hospital stay both for COPD and all causes.ConclusionsClinical and socioeconomic parameters were associated with the number of days in hospital for COPD and all-causes, and all-cause mortality in ambulatory COPD patients. Our findings support the importance of a multi-disciplinary pulmonary approach to improve clinical outcomes among COPD patients.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"22 ","pages":"14799731251355445"},"PeriodicalIF":3.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12206993/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144526627","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Eosinopenia in bronchiectasis: A novel biomarker for morbidity and mortality.","authors":"Shimon Izhakian, Ravid Yehezkely, Assaf Frajman, Ori Mekiten, Ori Hadar, Avigail Rockland, Liel Malka, Lev Freidkin, Dror Rosengarten, Mordechai R Kramer","doi":"10.1177/14799731241296847","DOIUrl":"10.1177/14799731241296847","url":null,"abstract":"<p><strong>Background: </strong>The paradigm of bronchiectasis is shifting away from its exclusive characterization as a neutrophilic condition. Patients with bronchiectasis and high eosinophil levels have been found to have a specific phenotype, but the clinical effect of eosinopenia remains unclear.</p><p><strong>Method: </strong>A retrospective, single-center, observational study was conducted at a tertiary medical center. Adult patients under follow-up for bronchiectasis from January 2007 to August 2020 were categorized by blood eosinophil count (BEC) as follows: eosinopenia (<100 cells/µL), normal (100-299 cells/µL), and eosinophilia (≥300 cells/µL). Data on the first hospitalization due to exacerbation and the community exacerbation rate in the first year of follow-up were analyzed. Mortality rates were assessed up to the end of follow-up on September 1, 2023.</p><p><strong>Results: </strong>The cohort included 724 patients (100%), 61% female (<i>n</i> = 442), of mean age 61 ± 16 years. The median follow-up period was 7.5 years (IQR: 5.1-10.8). Eosinopenia was found in 14.7% (<i>n</i> = 107), normal BEC in 56.6% (<i>n</i> = 417), and eosinophilia in 28.7% (<i>n</i> = 200). Patients with eosinopenia had a higher hazard ratio for first hospitalization than the normal-count group (1.71, 95% CI 1.11-2.64, <i>p</i> = .01) and the highest mean exacerbation rate (<i>p</i> = .04). On multivariate analysis, eosinopenia was significantly associated with higher mortality (HR 2.15, 95% CI 1.42-3.24, <i>p</i> < .001) after adjusting for age and sex.</p><p><strong>Conclusion: </strong>Eosinopenia in bronchiectasis emerged as a potential biomarker for adverse outcomes. Further study of its role in disease behavior may provide insights for the development of therapeutic strategies.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"22 ","pages":"14799731241296847"},"PeriodicalIF":3.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11700409/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142920763","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pulmonary rehabilitation healthcare professionals understanding and experiences of the protected characteristics of service users: A qualitative analysis.","authors":"Holly Drover, Sally J Singh, Mark W Orme, Enya Daynes","doi":"10.1177/14799731241307253","DOIUrl":"10.1177/14799731241307253","url":null,"abstract":"<p><strong>Background: </strong>Health inequalities can affect access and uptake to pulmonary rehabilitation (PR). An individual's protected characteristics (age, disability, gender reassignment, marriage and civil partnership, pregnancy and maternity, race, religion or belief, sex and sexual orientation) may contribute to health inequalities. Healthcare professionals (HCPs) experiences of the inclusivity and representativeness of PR services and knowledge of protected characteristics are unknown, however are vital for the identification and resolution of health inequalities. This qualitative study explored HCPs understanding of protected characteristics and their perception of the inclusivity, representativeness and equitable benefit of their PR services.</p><p><strong>Methods: </strong>Semi-structured qualitative interviews were conducted in person or via videoconferencing with HCPs involved in PR from two healthcare providers. Interviews were analysed using reflexive thematic analysis.</p><p><strong>Results: </strong>12 interviews were conducted with physiotherapists (<i>n</i> = 6), occupational therapists (<i>n</i> = 2), nurses (<i>n</i> = 2) and exercise physiologists (<i>n</i> = 2). Participants had a median (IRQ) age of 43 (13) and 75% (<i>n</i> = 9) were female. Four themes were generated. 1: 'I don't really know as much as I should' [about protected characteristics]; 2: It's uncomfortable collecting protected characteristics…; 3: 'I don't think [service users] are as representative as they could be'; 4: A conventional rehabilitation programme does not meet the needs of all.</p><p><strong>Conclusions: </strong>This study highlighted several challenges in HCPs understanding of protected characteristics and the representativeness of PR that must be addressed to ensure equity. Strategies, to understand barriers in accessing PR that limit representativeness should be explored.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"22 ","pages":"14799731241307253"},"PeriodicalIF":3.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11733882/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142982900","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Remote assessment of exercise capacity in adults with chronic respiratory disease: Safety, reliability and acceptability.","authors":"Narelle S Cox, Simone Dal Corso, Angela T Burge, Janet Bondarenko, Jaycie Perryman, Anne E Holland","doi":"10.1177/14799731251318033","DOIUrl":"10.1177/14799731251318033","url":null,"abstract":"<p><strong>Objectives: </strong>To assess the safety, reliability and acceptability of the modified incremental step test (MIST) supervised remotely via videoconferencing in adults with chronic respiratory disease.</p><p><strong>Methods: </strong>Adults with chronic respiratory disease undertaking pulmonary rehabilitation were invited to undertake the MIST under two testing conditions: in-person supervision and remote supervision via video-conferencing. Test order was randomised.</p><p><strong>Results: </strong>38 participants (<i>n</i> = 18 Female; mean (SD) age 68 (10) years; 56% chronic obstructive pulmonary disease) undertook two MIST evaluations. There was excellent agreement between tests for total step count (ICC_2,1 0.93, 95%CI 0.86 to 0.96), despite higher counts with in-person supervision (MD 12 steps, 95%CI 1 to 24). There was very good agreement, and no difference between tests, for nadir oxygen saturation (ICC_2,1 0.797, 95%CI 0.643 to 0.889) and peak heart rate (ICC_2,1 0.782, 95%CI 0.620 to 0.880). Participant satisfaction with telehealth was high, and confidence was not different between testing conditions. There were no adverse events and remote testing was acceptable to participants.</p><p><strong>Discussion: </strong>In this single centre cohort study MIST supervised remotely via video-conferencing was safe, reliable and acceptable to people with chronic respiratory disease.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"22 ","pages":"14799731251318033"},"PeriodicalIF":3.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11780657/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143064135","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Active cycle of breathing technique versus oscillating positive expiratory pressure therapy: Effect on lung function in children with primary ciliary dyskinesia; A feasibility study.","authors":"Bishara Fashho, Nisreen Rumman, Jane Lucas, Hadeel Halaweh","doi":"10.1177/14799731251314872","DOIUrl":"10.1177/14799731251314872","url":null,"abstract":"<p><p><b>Background:</b> Primary Ciliary Dyskinesia (PCD) is a rare genetic disorder requiring airway clearance techniques for mucus removal. We aimed to evaluate the feasibility and the effect of the active cycle of breathing technique (ACBT) versus oscillating positive expiratory pressure therapy (OPEP) in improving lung function and functional exercise capacity among children with PCD in Palestine. <b>Methods:</b> 32 PCD children (6-18 years) were included in a 12-week home-based feasibility study. They were assigned randomly into two groups: ACBT and OPEP. Data collection included spirometry measurements, and the six-minute walk test (6MWT). <b>Results:</b> After 12 weeks of regular airway clearance techniques (ACT), the FEV₁, MEF_25-75%, and the 6MWT demonstrated statistically significant differences (<i>p</i> = .02, <i>p</i> = .04, and <i>p</i> = .05 respectively) between the two groups, in favor of the OPEP group with the effect size of Cohen's d (0.86, 0.76, and 0.71) respectively. However, there was no significant difference (<i>p</i> > .05) between the two groups in FVC and FEV₁/FVC. Additionally, only in the OPEP group, significant differences were recorded between pre and post-tests for FEV1 and 6MWT (<i>p</i> < .05). <b>Conclusion:</b> The randomized study design comparing ACBT and OPEP was feasible and acceptable to patients. OPEP demonstrates potential for managing respiratory health; however, treatments should be individualized to address each patient's specific needs. Further research with larger cohorts is needed to assess the effectiveness of both methods.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"22 ","pages":"14799731251314872"},"PeriodicalIF":3.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11729442/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142977674","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"What are the important components of physical activity for people with fibrotic interstitial lung disease?","authors":"Mariana Hoffman, Christie R Mellerick, Angela T Burge, Anne E Holland","doi":"10.1177/14799731251358584","DOIUrl":"10.1177/14799731251358584","url":null,"abstract":"<p><p>IntroductionFibrotic interstitial lung disease (fILD) is characterized by scarring of the lungs, decline in lung function and compromised exercise capacity. People with fILD participate in less daily physical activity compared to healthy controls, however, the relative importance of physical activity components (volume, intensity, duration) is unknown. This study aimed to examine the relationship between the components of physical activity and established disease severity and impact measures in fILD.MethodsParticipant data were from baseline assessment of a randomized controlled trial recruiting people with fILD and exertional desaturation during exercise testing on room air. Physical activity components including volume (steps per day), intensity (light, moderate and vigorous) and duration (time spent in different physical activity intensities) were objectively assessed for 7 days using two physical activity monitors. Associations of these components with lung function (FVC, FEV₁, TLCO), functional exercise capacity (6-min walk distance, 6MWD) and dyspnea (Dyspnea-12 questionnaire) were investigated.Results106 participants with mild to severe fILD were included. Physical activity volume was not associated with 6MWD (r = 0.027, <i>p</i> = 0.78) and weakly associated with lung function (FVC r = 0.33, <i>p</i> = 0.001; FEV₁ r = 0.34, <i>p</i> = 0.001). Physical activity intensity and duration were weakly associated with 6MWD (light r = 0.22 <i>p</i> = 0.02; moderate r = 0.42, <i>p</i> < 0.001; vigorous r = 0.33, <i>p</i> = 0.01). Time spent in vigorous physical activity was weakly associated with lung function (FVC r = 0.19, <i>p</i> = 0.05; FEV₁ r = 0.18, <i>p</i> = 0.006). Dyspnea scores were not associated with any physical activity variables. Relationships remained consistent after adjusting for age, gender and disease severity, with the exception of vigorous physical activity which was no longer associated with lung function or 6MWD.ConclusionIn people with fILD, the physical activity components of volume, intensity and duration were associated with different measures of disease severity and impact. This highlights the importance of considering specific physical activity components when evaluating and promoting physical activity in this group.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"22 ","pages":"14799731251358584"},"PeriodicalIF":2.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12326094/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144788400","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Patient and healthcare professional perspectives on telehealth in the care of interstitial lung disease.","authors":"Sheetal Deshpande, Gabriella Tikellis, Hayley Barnes, Karen Symons, Anne E Holland, Ian Glaspole, Yet Hong Khor","doi":"10.1177/14799731251366945","DOIUrl":"10.1177/14799731251366945","url":null,"abstract":"<p><p>BackgroundWhile telehealth has been used for specialist care during the COVID-19 pandemic, its benefits and barriers to use for delivering care in interstitial lung disease (ILD) remain unclear. We aimed to explore perspectives of patients and healthcare professionals (HCPs) towards telehealth use in ILD.MethodThis qualitative study using semi-structured interviews was undertaken in 18 patients with ILD from a single quaternary ILD centre and 18 HCPs of four different disciplines from various ILD centres around Australia. Interviews were transcribed verbatim and coded by two investigators independently using thematic analysis. Themes were developed by consensus.ResultsPatients and HCPs had experienced telehealth via video and telephone, predominantly for teleconsultation and/or telerehabilitation. For patients, benefits included provision of ongoing access to healthcare and significant reduction in travel. Healthcare professionals reported teleconsultations played an important role in patient follow-up rather than at initial contact. Concerns of suboptimal rapport and communication and a lack of physical examination were raised by both groups. Individual patient suitability, improvement in technological platform and support, and improved workflow for clinicians were important factors for utilisation of telehealth. Patients were open to the use of home monitoring devices, however, HCPs felt further research was required prior to clinical implementation.ConclusionThis study highlights positive and negative experiences of telehealth for patients and HCPs in the management of ILD . It also identifies areas for improvement to create a tailored telehealth model for ILD care.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"22 ","pages":"14799731251366945"},"PeriodicalIF":2.3,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12361724/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144871664","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The experience of adults with cystic fibrosis using long-term domiciliary non-invasive ventilation.","authors":"Jocelyn Choyce, Alistair Hewison, Amelia Swift, Joanna L Whitehouse","doi":"10.1177/14799731241249476","DOIUrl":"10.1177/14799731241249476","url":null,"abstract":"<p><p><b>Background:</b> The use of non-invasive ventilation (NIV) in patients with advanced cystic fibrosis (CF) has increased in recent years. Research evidence supports its clinical benefits, but less is known about the patients' experience of its long-term use in a domiciliary setting.<b>Objective:</b> To investigate patients' lived experience of using long-term domiciliary NIV.<b>Methods:</b> Semi-structured, qualitative interviews were conducted with adults with CF using long-term domiciliary NIV for respiratory failure. The data collected were subject to thematic analysis.<b>Results:</b> Nine adults (6 female), 5 of whom were awaiting lung transplantation, with a mean age of 39 years and mean FEV₁ per cent predicted of 28%, were recruited. Data analysis revealed 2 themes: gratitude, and determination despite challenges. Patients identified some troubling side effects from NIV but were grateful for its symptomatic relief and were determined to continue using it to improve their quality of life.<b>Conclusions:</b> Participants reported experiences of NIV to be generally positive in terms of symptom relief and quality of life. These findings provide an initial insight into patients' experience of NIV and have the potential to help guide and improve care.</p>","PeriodicalId":10217,"journal":{"name":"Chronic Respiratory Disease","volume":"22 ","pages":"14799731241249476"},"PeriodicalIF":3.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11775957/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143058065","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}