Quantity and quality of airway clearance in children and young people with cystic fibrosis

Raywood, Emma; Shannon, Harriet; Filipow, Nicole; Tanriver, Gizem; Stanojevic, Sanja; Kapoor, Kunal; Douglas, Helen; O’Connor, Rachel; Murray, Nicky; Black, Bridget; Main, Eleanor

doi:10.1016/j.jcf.2022.09.008

Cited by 8 publications

(6 citation statements)

References 15 publications

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“…[ 16 ]). The devices incorporated positive expiratory pressure (PEP) (Astra PEP and Pari PEP) and oscillating PEP (OPEP) (Acapella and Aerobika) mechanisms [ 16 , 17 ]. Data were synced to a secure Azure cloud for access by the research team.…”

Section: Methodsmentioning

confidence: 99%

“…Data were synced to a secure Azure cloud for access by the research team. Raw data were pre-processed through the removal of blank, duplicate and non-physiological values, and non-linear baseline drift was corrected [ 17 ]; ACT data synced outside the study window or on the day of study recruitment were excluded.…”

Section: Methodsmentioning

confidence: 99%

“…Pre-processed ACT data were summarised into treatments, detailing the number of breaths, mean mid-expiratory breath pressure (cmH 2 O) (termed “pressure” throughout) and mean breath length (s) (termed “length” throughout). Breath length is related to lung size during growth and development, so it was further standardised against an age-specific threshold [ 17 ]. Per treatment, the difference in mean expired breath length from the individualised threshold was calculated as a percentage.…”

Section: Methodsmentioning

confidence: 99%

“…Project Fizzyo, a longitudinal observational cohort study in the UK, used remote monitoring with electronic pressure sensors attached to four different commercially available ACT devices to record real-time, breath-by-breath pressure data during usual treatments undertaken at home over 16 months [ 16 ]. An analysis of baseline data in the first 2 months of the study, comprising 742 084 breaths from 9081 ACT treatments, demonstrated that the number of treatments undertaken and the number of treatments that met recommendations varied within and between CYPwCF over time [ 17 ].…”

Section: Introductionmentioning

confidence: 99%

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Real-world effectiveness of airway clearance techniques in children with cystic fibrosis

Filipow,

Stanojevic,

Raywood

et al. 2023

Eur Respir J

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BackgroundCystic Fibrosis (CF) is commonly characterised by thick respiratory mucous. From diagnosis, people with CF are prescribed daily physiotherapy, including airway clearance treatments (ACTs). ACTs consume a large proportion of treatment time, yet the efficacy and effectiveness of ACTs are poorly understood. This study aimed to evaluate associations between quality and quantity of ACTs and lung function in children and young people with CF.MethodsProject Fizzyo, a longitudinal observational cohort study, used remote-monitoring electronic pressure sensors with 4 different commercial ACT devices to record real-time, breath-by-breath pressure data during usual ACTs undertaken at home over 16 months in 145 children. ACTs were categorised either as conformant or not with current ACT recommendations based on breath pressure and length measurements, or as missed treatments if not recorded. Daily, weekly and monthly associations between ACT category and lung function were investigated using linear mixed effects regression models adjusting for clinical confounders.ResultsAfter exclusions, 45 224 ACTs (135 individuals) and 21 069 days without treatments (141 individuals) were analysed. Average age of participants was 10.2 years (sd=2.9). Conformant ACTs (21%) had significantly higher FEV1(mean effect size 0.23, 95%CI [0.19, 0.27]) than non-conformant (79%) or missed treatments. There was no benefit from non-conformant or missed treatments, and no significant difference in FEV1between them (mean effect size 0.02 (95%CI, [-0.01, 0.05]).ConclusionACTs are beneficial when done as recommended, but most people use techniques that do not improve lung function. Work is needed to monitor and improve ACT quality and to increase the proportion of people doing effective airway clearance at home.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Real-world effectiveness of airway clearance techniques in children with cystic fibrosis

Filipow,

Stanojevic,

Raywood

et al. 2023

Eur Respir J

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“…CF-related lung disease is the major cause of morbidity and mortality 1 ; the build-up of mucus in the airways causes chronic and recurrent inflammation, leading to epithelial damage, tissue remodelling, and progressive deterioration of lung function, ultimately resulting in respiratory failure. 2 Respiratory mucus is loosened and removed by daily respiratory physiotherapy (RP), which is perceived as the most burdensome routine in disease management (2 h on average every day), 3 consisting of airway clearance therapy (ACT), exercise prescription and education, and inhalation therapy. 4 …”

Section: Introductionmentioning

confidence: 99%

Perceived burden of respiratory physiotherapy in people with cystic fibrosis taking elexacaftor–tezacaftor–ivacaftor combination: a 1-year observational study

Blardone,

Gambazza,

Mariani

et al. 2024

Ther Adv Respir Dis

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Background: To limit the progression of disease, people with cystic fibrosis (pwCF) perform daily respiratory physiotherapy, which is perceived as the most burdensome routine in managing their condition. The elexacaftor–tezacaftor–ivacaftor (ETI) combination has changed respiratory management. Objective: To investigate how the perceived treatment burden changed in 1 year of treatment with ETI. Design: Prospective observational study. Methods: Ad hoc questionnaires for the pwCF and for the caregivers of pwCF < 18 years were administered before the initiation of ETI therapy and then at 6–12 months. The Cystic Fibrosis Questionnaire-Revised (CFQ-R) and the Sinonasal Outcome Test (SNOT-22) were administered to explore disease-related symptoms and social limitations. The International Physical Activity Questionnaire was used to determine levels of physical activity. Mixed-effect models were fitted to explore whether the time engaged in respiratory physiotherapy changed during 1 year. Results: The study included 47/184 pwCF aged 21.4 (5.7) years, who completed 1 year of ETI therapy. At 6 months, time on aerosol therapy was decreased by 2.5 (95% CI −32.9 to 27.8) min/day, time on airway clearance therapies (ACTs) was decreased by 8.8 (95% CI −25.9 to 8.3) min/day, and time for cleaning and disinfecting respiratory equipment was decreased by 10.6 (95% CI −26.5 to 5.3) min/day. At 1 year, gains in time saved were nearly 15 min/day on average. At 1 year, 5/47 (10.6%) pwCF reported that they had discontinued positive expiratory pressure mask. Conclusion: PwCF on ETI may note less time engaged in their daily respiratory physiotherapy routine. Nonetheless, aerosol therapy, ACTs and maintaining respiratory equipment were still perceived as time-consuming daily activities.

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Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis

Main

Rand

2023

Cochrane Database of Systematic Reviews

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Quantity and quality of airway clearance in children and young people with cystic fibrosis

Cited by 8 publications

References 15 publications

Real-world effectiveness of airway clearance techniques in children with cystic fibrosis

Real-world effectiveness of airway clearance techniques in children with cystic fibrosis

Perceived burden of respiratory physiotherapy in people with cystic fibrosis taking elexacaftor–tezacaftor–ivacaftor combination: a 1-year observational study

Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis

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