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Background: Oscillating positive expiratory pressure (OPEP) devices play a key role in airway clearance, particularly in patients with cystic fibrosis. These devices, however, have the potential to become reservoirs for pathogenic organisms and require daily, or even more frequent, cleaning. This places a large burden on patients and their carers., Aims: The objective of this work was to develop a disposable OPEP device, with comparable mechanical performance to commercial devices, that negates the need for cleaning after use thus reducing microbiological risks., Methods: 3D printing was used to iterate and develop a prototype disposable device (The University of Limerick OPEP, abbreviated to the UL-OPEP) that was compared with a selection of commercially available devices for mean pressure and oscillation amplitude (cmH 2 O), as well as oscillation frequency (Hz). All devices were tested using a healthy volunteer at a target expiratory flow of ~ 20 L/min. The target therapeutic range was 10-20 cmH 2 O at a flow rate of 10-20 L/min as is reported widely in the literature., Results: The prototype disposable device achieved a mean pressure of 14.82 cmH 2 O at a mean flow rate of 18.82 L/min, and generated an oscillation frequency of 26 Hz with an amplitude of 1.28 cmH 2 O. These characteristics compare favourably with existing, more complex, reusable OPEP devices., Conclusions: The UL-OPEP device is a small, disposable OPEP device, that generates pressure and oscillation amplitudes for clinically effective airway clearance. The device negates the need for cleaning and disinfecting, removing the risk of devices acting as a potential reservoir for pathogenic organisms while maintaining mucus-clearing benefits., (© 2022. The Author(s), under exclusive licence to Royal Academy of Medicine in Ireland.)

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).

Background: Oscillating positive expiratory pressure devices aid removal of excess secretions and reduce gas trapping in patients with hypersecretory pulmonary diseases, for example, cystic fibrosis. Oscillating positive expiratory pressure works when the patient exhales actively against a fixed resistor, which generates mean intrapulmonary pressures of 10-20 cm H 2 O with rapid fluctuations of at least 1 cm H 2 O from the mean. In this study, we evaluated the performance of oscillating positive expiratory pressure therapy by pediatric subjects with cystic fibrosis to determine adherence to target therapeutic pressures., Methods: Twenty-one pediatric subjects were recruited. Each had a history of using an oscillating positive expiratory pressure device twice daily and had received standardized training and instructions from the same specialist physiotherapist. Performance was evaluated by using a flow and pressure sensor placed in-line between the participant's mouth and the device. The participants performed expirations as per their normal routine., Results: None of the participants achieved target therapeutic pressure ranges during expiration. The mean ± SD pressure generated was 16.2 ± 6.8 cm H 2 O, whereas mean ± SD flow was 31.3 ± 8.9 L/min. The mean ± SD expiration length was 2.5 ± 1.4 s., Conclusions: Despite standardized instruction, the results demonstrated considerable variation among the participants and overall poor technique during use. Outcomes of this study indicated that airway clearance effects of oscillating positive expiratory pressure were compromised due to poor technique., Competing Interests: The authors have disclosed no conflicts of interest., (Copyright © 2019 by Daedalus Enterprises.)

Background: Burkholderia contaminans is an emerging pathogen in the cystic fibrosis (CF) setting. Included in the Burkholderia cepacia complex (Bcc), B. contaminans is a Gram negative, motile, obligate aerobe previously classified as a pseudomonad. Previous reports have described B. contaminans isolation from patients in Portugal, Switzerland, Spain, Argentina and the USA. This, however, is the first report relating to B. contaminans affecting Irish patients with CF, initially detected in a paediatric setting., Case Presentation: Burkholderia contaminans was identified in the routine analysis of sputum from a fourteen year old boy, at his annual review and subsequently from the sputum from his 19 year old brother. RecA gene sequencing and pulsed field gel electrophoresis (PFGE) were unable to distinguish between the isolates, which demonstrated with susceptibility to ciprofloxacin, cotrimoxazole, meropenem, pipercillin/tazobactam and ceftazidime. Both isolates were resistant to aztreonam, with reduced susceptibility to tobramycin. Following treatment with intravenous meropenem and ceftazidime, oral ciprofloxacin and nebulised tobramycin for 6 weeks, sputum specimens from both patients were negative for B. contaminans. No other member of the local CF cohort proved positive., Conclusions: Bcc bacteria are associated with poor prognosis in CF and decreased life expectancy, specifically leading to a more rapid decline in lung function and, in some cases, to a fatal necrotizing pneumonia known as the "cepacia syndrome". Some species exhibit innate resistance to multiple antimicrobial agents and their transmission rate can be high in susceptible patients. In that context, we describe the first incidence of CF-related B. contaminans in Ireland and its successful eradication from two patients, one paediatric, using an aggressive antimicrobial regimen.

Background: Pseudomonas aeruginosa is a pathogen associated with cystic fibrosis that has potential to decrease lung function and cause respiratory failure. Paranasal sinuses are increasingly recognised as potential reservoirs for intermittent colonisation by P. aeruginosa. This case documents investigation and outcome of P. aeruginosa recurrence in a male paediatric patient over an eight year period., Case Presentation: A 12 year old Irish male paediatric cystic fibrosis patient experienced intermittent culturing of P. aeruginosa from the oropharyngeal region, indicating chronic infection of the sinuses despite absence of symptoms, retaining good lung function, and normal bronchoscopy and bronchoalveolar lavage. However, P. aeruginosa was isolated from a sinus wash-out and was identified as a unique strain of P. aeruginosa that was also cultured from cough swabs. Despite treatment, successful eradication from the paranasal sinuses was not achieved., Conclusions: Few reports have addressed the paranasal sinuses as a reservoir for lung infection in cystic fibrosis patients despite increased recognition of the need to investigate this niche. In this case, attempts at eradication of P. aeruginosa present in paranasal sinuses including oral and nebulised antimicrobials proved unsuccessful. However, detection of P. aeruginosa in the paranasal sinuses instigated antimicrobial treatment which may have contributed to prevention of migration to the lower airways. Our outcome provides additional insight and may indicate utility of nasal lavage or nasal endoscopy in paediatric cystic fibrosis patients' annual review clinic visits.

Rationale: There is limited evidence supporting the routine use of palivizumab in paediatric cystic fibrosis (CF) patients to reduce respiratory syncytial virus (RSV) infection and related hospitalisation. Despite this, anti-RSV prophylaxis is increasingly common. This is the first report from Ireland regarding palivizumab outcomes for children with CF, under 2 years old, despite the greatest prevalence of CF globally. Methods: An audit was performed at a tertiary hospital in Ireland's mid-West to document all children with CF, <24 months old, who received palivizumab over a five year period and comparision made with all eligible children for the prior five year period who had not received the product (also CF patients). Palivizumab was administered to both cohorts in their first year of life. Hospitalisation rates were compared using Fisher's exact test. Incidence of RSV and Pseudomonas aeruginosa infection was recorded. Results: A total of 19 patients who received palivizumab were included in the study; comparision was made with a retrospective control group of 30 patients. Prophylactic palivizumab did not prevent hospitalisation for 10/19 patients, 3 of whom were affected by RSV. This was significantly greater than in the control group, where no hospitalisations were recorded (p < 0.0001). P. aeruginosa was isolated in one case from the study cohort, while no P. aeruginosa was detected in the control group. Conclusions: This study, the first of its kind from Ireland where CF prevalence is highest, does not provide unequivocal support for prophylactic use of palivizumab in CF patients under 2 years. Despite being derived from a small sample size, based on these data and complementary clinical observation, we have discontinued such prophylaxis. However, should reported incidence of RSV-related hospitalisation increase, there is scientific plausibility for appropriately powered, randomised, controlled trials of palivizumab. [ABSTRACT FROM AUTHOR]