Airway clearance and exercise for people with cystic fibrosis: Balancing longevity with life

Abstract

Airway clearance has been an integral part of cystic fibrosis (CF) care for almost as long as CF has been identified as a condition. From diagnosis as a neonate through to end-of-life care, airway clearance is an everyday aspect of life, adding a considerable treatment burden to the lives of people with CF. There are many different techniques used for airway clearance which have evolved over time with an aim to improve effectiveness, support adherence and, more recently, to consider the impact of burden. A popular thought is whether airway clearance could be replaced by exercise. With new precision therapies in the form of CFTR modulators available, the CF landscape is rapidly changing, raising the question of whether certain treatments are needed at all. Depending on factors such as CFTR mutation, age, and pre-existing lung damage before starting a CFTR modulator, individuals with CF may need different levels of intensity and type of maintenance treatment. Precision medicine is likely to lead to the need for increased precision and individualized management around other maintenance therapies such as airway clearance.

Exercise as an Airway Clearance Technique in people with Cystic Fibrosis (ExACT-CF): rationale and study protocol for a randomised pilot trial

“…Airway clearance is a long-established treatment for pwCF, dating back almost to when CF was identified 6 , yet tACT (chest physiotherapy) is consistently reported to be the most burdensome treatment for pwCF, with self-reported adherence lower than other treatments such as medication 9 . CFTR modulators are changing the landscape of CF care and ways to reduce the burden of treatment for pwCF is a priority 6 .…”

“…Airway clearance is a long-established treatment for pwCF, dating back almost to when CF was identified 6 , yet tACT (chest physiotherapy) is consistently reported to be the most burdensome treatment for pwCF, with self-reported adherence lower than other treatments such as medication 9 . CFTR modulators are changing the landscape of CF care and ways to reduce the burden of treatment for pwCF is a priority 6 . Aerobic capacity is thought to be one of the best predictors of survival for people with CF [46][47][48] and exercise training is already part of the physiotherapy management of pwCF 2 , however it is currently recommended in addition to airway clearance, rather than this purpose alone 5 .…”

“…There are many accepted chest physiotherapy tACTs to manage the respiratory health of pwCF, including active cycle of breathing techniques (ACBT), autogenic drainage (AD), positive expiratory pressure (PEP) and oscillating PEP devices, postural drainage, and percussion techniques, with the choice depending on preference, age, airway pathophysiology and adverse events. tACTs have evolved over time 6 , driven largely by increased life expectancy and a need for independence. However, pwCF consider tACTs burdensome 7 ; those who are younger and with less advanced lung disease perceive little benefit 8 and self-reported adherence is as low as 30% 9 .…”

Review of respiratory physiotherapy methods in patients with cystic fibrosis

“…Drenaż ułożeniowy to technika wykorzystująca ułożenie ciała w odniesieniu do siły grawitacji w celu przesunięcia wydzieliny do centralnych oskrzeli. Dodatkowe zastosowanie technik manualnych, takich jak wstrząsanie, wibracja oraz oklepywanie, ma na celu przesunięcie wydzieliny wraz z wydechem [14]. W grupie niemowląt, małych dzieci, a także dzieci niewspółpracujących stosowany jest drenaż ułożeniowy i zmodyfikowany z oklepywaniem, wstrząsaniem, uciskaniem lub wspomaganiem wydechów.…”

Editorial: Insights in pediatric pulmonology 2021

“…Airways clearance techniques (ACT) are certainly a key point treatment in CF affected children. Its usefulness in the long-term period is undisputed ( 14 , 15 ). Vandervoort and colleagues questioned if a single ACT session using positive expiratory pressure (PEP) mask has a short-term positive effect on forced expiratory volume in 1 s (FEV 1 ) and lung clearance index (LCI) in children affected by CF and primary ciliary dyskinesia (PCD).…”