scholarly journals Effects of exercise and airway clearance (positive expiratory pressure) on mucus clearance in cystic fibrosis: a randomised crossover trial

2019 ◽  
Vol 53 (4) ◽  
pp. 1801793 ◽  
Author(s):  
Tiffany J. Dwyer ◽  
Evangelia Daviskas ◽  
Rahizan Zainuldin ◽  
Jordan Verschuer ◽  
Stefan Eberl ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Crossover Trial ◽  
Treadmill Exercise ◽  
Forced Expiratory Volume ◽  
Mean Difference ◽  
Peak Oxygen Consumption ◽  
Mucus Clearance ◽  
Airway Clearance ◽  
Positive Expiratory Pressure

Exercise improves mucus clearance in people without lung disease and those with chronic bronchitis. No study has investigated exercise alone for mucus clearance in cystic fibrosis (CF). The aim of this study was to compare the effects of treadmill exercise to resting breathing and airway clearance with positive expiratory pressure (PEP) therapy on mucus clearance in adults with CF.This 3-day randomised, controlled, crossover trial included 14 adults with mild to severe CF lung disease (forced expiratory volume in 1 s % predicted 31–113%). Interventions were 20 min of resting breathing (control), treadmill exercise at 60% of the participant's peak oxygen consumption or PEP therapy (including huffing and coughing). Mucus clearance was measured using the radioaerosol technique and gamma camera imaging.Treadmill exercise improved whole lung mucus clearance compared to resting breathing (mean difference 3%, 95% CI 2–4); however, exercise alone was less effective than PEP therapy (mean difference −7%, 95% CI −6– −8). When comparing treadmill exercise to PEP therapy, there were no significant differences in mucus clearance from the intermediate and peripheral lung regions, but significantly less clearance from the central lung region (likely reflecting the huffing and coughing that was only in PEP therapy).It is recommended that huffing and coughing are included to maximise mucus clearance with exercise.

scholarly journals Exercise as a substitute for traditional airway clearance in cystic fibrosis: a systematic review

Thorax ◽  
2020 ◽  
pp. thoraxjnl-2020-215836
Author(s):  
Nathan Ward ◽  
Scott Morrow ◽  
Kathy Stiller ◽  
Anne E Holland
Keyword(s):  
Cystic Fibrosis ◽  
Respiratory Function ◽  
Exercise Intervention ◽  
Treadmill Exercise ◽  
Forced Expiratory Volume ◽  
Mean Difference ◽  
Airway Clearance ◽  
Significant Difference ◽  
Related Quality ◽  
Registration Number

BackgroundExercise and traditional airway clearance techniques (ACTs) are both routinely recommended for people with cystic fibrosis (CF), with some people using exercise as a substitute for traditional ACTs. The effectiveness of this is unclear. We systematically reviewed the evidence for using exercise as a substitute for traditional ACTs in people with CF.MethodsA systematic database and literature search were undertaken of studies comparing exercise to rest or traditional ACTs. Primary outcomes were respiratory function, respiratory exacerbations and health-related quality of life. Secondary outcomes included mucociliary clearance (MCC), sputum weight and ease of expectoration. Data are mean difference (95% CI).ResultsA total of 12 studies (15 reports) were included, all of short duration (single session to 2 weeks). In crossover trials, exercise did not improve forced expiratory volume in one second in comparison to rest, but peak expiratory flow was increased during treadmill exercise (mean difference (MD) range 1.00–1.16 L/s) and cycle ergometry (1.19 (0.96 to 1.42) L/s). Treadmill exercise improved MCC (2.6 (1.6 to 3.6)%) and ease of expectoration (MD range 1.3–1.8 cm) compared with rest. No consistent differences in respiratory function were evident when exercise was compared with traditional ACTs (four crossover studies). There was no significant difference in MCC or sputum weight in studies where forced expirations were included in the exercise intervention.ConclusionsExercise improves ease of expectoration and sputum clearance compared with rest. Exercise, incorporating forced expirations, may have similar effects to traditional ACTs over the short term. There are no data comparing exercise to traditional ACTs over the longer term.PROSPERO registration numberCRD42018102780.

scholarly journals A short-term evaluation of a prototype disposable Oscillating Positive Expiratory Pressure (OPEP) device in a cohort of children with cystic fibrosis

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Kevin J. O’Sullivan ◽  
Valerie Power ◽  
Barry Linnane ◽  
Deirdre McGrath ◽  
Magdalena Mulligan ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Clinical Investigation ◽  
Mean Difference ◽  
Short Term ◽  
Intervention Participant ◽  
Post Intervention ◽  
Convenience Sample ◽  
Disposable Device ◽  
Positive Expiratory Pressure

Abstract Background Oscillating Positive Expiratory Pressure (OPEP) devices are important adjuncts to airway clearance therapy in patients with cystic fibrosis (CF). Current devices are typically reusable and require daily, or often more frequent, cleaning to prevent risk of infection by acting as reservoirs of potentially pathogenic organisms. In response, a daily disposable OPEP device, the UL-OPEP, was developed to mitigate the risk of contamination and eliminate the burdensome need for cleaning devices. Methods A convenience sample of 36 participants, all current OPEP device users, was recruited from a paediatric CF service. For one month, participants replaced their current OPEP device with a novel daily disposable device. Assessment included pre- and post-intervention lung function by spirometry, as well as Lung Clearance Index. Quality of life was assessed using the Cystic Fibrosis Questionnaire – Revised, while user experience was evaluated with a post-study survey. Results 31 participants completed the study: 18 males; median age 10 years, range 4–16 years. Lung function (mean difference ± SD, %FEV1 = 1.69 ± 11.93; %FVC = 0.58 ± 10.04; FEV1: FVC = 0.01 ± 0.09), LCI (mean difference ± SD, 0.08 ± 1.13), six-minute walk test, and CFQ-R were unchanged post-intervention. Participant-reported experiences of the device were predominantly positive. Conclusions The disposable OPEP device maintained patients’ lung function during short term use (≤ 1 month), and was the subject of positive feedback regarding functionality while reducing the risk of airway contamination associated with ineffective cleaning. Registration The study was approved as a Clinical Investigation by the Irish Health Products Regulatory Authority (CRN-2209025-CI0085).

Improving airway clearance in cystic fibrosis lung disease

2014 ◽  
pp. 169-187
Author(s):  
Judy M. Bradley ◽  
Katherine O’Neill ◽  
Ruth Dentice ◽  
Mark Elkins
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Cystic Fibrosis Lung ◽  
Airway Clearance ◽  
Cystic Fibrosis Lung Disease

scholarly journals Hyperpolarised 129Xe magnetic resonance imaging to monitor treatment response in children with cystic fibrosis

2019 ◽  
Vol 53 (5) ◽  
pp. 1802188 ◽  
Author(s):  
Jonathan H. Rayment ◽  
Marcus J. Couch ◽  
Nancy McDonald ◽  
Nikhil Kanhere ◽  
David Manson ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cystic Fibrosis ◽  
Magnetic Resonance ◽  
Lung Disease ◽  
Treatment Response ◽  
Forced Expiratory Volume ◽  
Response To Therapy ◽  
Breath Hold ◽  
Pulmonary Exacerbation ◽  
Resonance Imaging

Pulmonary magnetic resonance imaging using hyperpolarised 129Xe gas (XeMRI) can quantify ventilation inhomogeneity by measuring the percentage of unventilated lung volume (ventilation defect per cent (VDP)). While previous studies have demonstrated its sensitivity for detecting early cystic fibrosis (CF) lung disease, the utility of XeMRI to monitor response to therapy in CF is unknown. The aim of this study was to assess the ability of XeMRI to capture treatment response in paediatric CF patients undergoing inpatient antibiotic treatment for a pulmonary exacerbation.15 CF patients aged 8–18 years underwent XeMRI, spirometry, plethysmography and multiple-breath nitrogen washout at the beginning and end of inpatient treatment of a pulmonary exacerbation. VDP was calculated from XeMRI images obtained during a static breath hold using semi-automated k-means clustering and linear binning approaches.XeMRI was well tolerated. VDP, lung clearance index and the forced expiratory volume in 1 s all improved with treatment; however, response was not uniform in individual patients. Of all outcome measures, VDP showed the largest relative improvement (−42.1%, 95% CI −52.1–−31.9%, p<0.0001).These data support further investigation of XeMRI as a tool to capture treatment response in CF lung disease.

scholarly journals Upper Airway Findings and Markers of Lung Disease Progression in Patients with Cystic Fibrosis

2020 ◽  
Vol 24 (04) ◽  
pp. e434-e437
Author(s):  
Luciane Mazzini Steffen ◽  
Luise Sgarabotto Pezzin ◽  
Natassia Sulis ◽  
Nedio Steffen ◽  
Leonardo Araujo Pinto
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Disease Progression ◽  
Nasal Polyps ◽  
Nasal Swab ◽  
Statistical Significance ◽  
Upper Airway ◽  
Forced Expiratory Volume ◽  
Upper Respiratory Tract ◽  
Upper Airways

Abstract Introduction Cystic fibrosis (CF) is a genetic disease that limits the quality of life mainly due to respiratory symptoms. The relationship between findings of the upper airways and CF lung disease is not yet completely understood. Objective The aim of the present study is to describe the most frequent nasal findings and pathogens in patients with CF and investigate the association between the findings of the upper respiratory tract and markers of lung disease progression. Methods Retrospective study in patients with CF from the Pediatric Pulmonology Department who underwent otorhinolaryngological evaluation between 2015 and 2017. Nasal endoscopy and nasal swab collection were part of the evaluation. The severity markers used were: percentage of predicted forced expiratory volume in the first second (FEV1%), body mass index (BMI) and the Shwachman-Kulczycki (SK) clinical score. Results A total of 48 patients with CF were included. The mean of the predicted percentage of FEV1% was 83.36 ± 30.04. The average 14 and SK score 89.11 ± 10.50. The bacteriology of the nasal swab was positive in 27 (54.1%) patients. Staphylococcus aureus was positive in 18 patients, Pseudomonas aeruginosa in 5, Pseudomonas cepacea in 3 and Stenotrophomonas maltophila in 1 patient. Nasal polyps were found in nine participants. Nasal polyps were found in nine participants and were associated with lower SK score. Conclusion The pathogens found in the upper airway were, in order: S. aureus, P. aeruginosa, P. cepacea e S. maltophila. The presence of polyps in the nasal cavity showed statistical significance and appears to have association with the prognostic factor measured by the SK score.

scholarly journals WS21.4 Resistive inspiratory manoeuvre as airway clearance technique in patients with cystic fibrosis: a randomised crossover trial

2016 ◽  
Vol 15 ◽  
pp. S33-S34 ◽  
Author(s):  
B. Herrero Cortina ◽  
M. San Miguel Pagola ◽  
A. Fernandez Cadenas ◽  
A.T. Ríos Cortés ◽  
T. Del Corral ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Crossover Trial ◽  
Airway Clearance ◽  
Clearance Technique

scholarly journals The Role of Serine Proteases and Antiproteases in the Cystic Fibrosis Lung

2015 ◽  
Vol 2015 ◽  
pp. 1-10 ◽  
Author(s):  
Matthew S. Twigg ◽  
Simon Brockbank ◽  
Philip Lowry ◽  
S. Peter FitzGerald ◽  
Clifford Taggart ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Immune Responses ◽  
Lung Disease ◽  
Healthy Individual ◽  
Chronic Infection ◽  
Serine Proteases ◽  
High Concentration ◽  
Mucus Clearance ◽  
Potential Therapeutics

Cystic fibrosis (CF) lung disease is an inherited condition with an incidence rate of approximately 1 in 2500 new born babies. CF is characterized as chronic infection of the lung which leads to inflammation of the airway. Sputum from CF patients contains elevated levels of neutrophils and subsequently elevated levels of neutrophil serine proteases. In a healthy individual these proteases aid in the phagocytic process by degrading microbial peptides and are kept in homeostatic balance by cognate antiproteases. Due to the heavy neutrophil burden associated with CF the high concentration of neutrophil derived proteases overwhelms cognate antiproteases. The general effects of this protease/antiprotease imbalance are impaired mucus clearance, increased and self-perpetuating inflammation, and impaired immune responses and tissue. To restore this balance antiproteases have been suggested as potential therapeutics or therapeutic targets. As such a number of both endogenous and synthetic antiproteases have been trialed with mixed success as therapeutics for CF lung disease.

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