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

Abstract

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.

Digital technologies in bronchiectasis physiotherapy services: a survey of patients and physiotherapists in a UK centre

“…Digital technologies to monitor and improve ACT quality in children with Cystic Fibrosis has been described, highlighting the importance of prescription conformant ACT to ensure clinical benefit. Exploration of technology application in this way in other disease areas may be important to better understand how and when ACTs will be useful (17). Accelerated by the COVID-19 pandemic, respiratory digital monitoring is increasing and there is a need to promote sustainable implementation in order to achieve success within routine clinical care.…”