Physiotherapeutic management strategies for the treatment of cystic fibrosis in adults

Abstract

Physiotherapy has long been considered a cornerstone of condition management for people with cystic fibrosis (CF). The presentation of CF has changed over time with an increased life expectancy and increased expectations of people with CF to have a complete lifestyle. In turn, the scope of strategies used in physiotherapy for CF have also changed dramatically over the years, moving away from routine postural drainage and manual techniques toward an individualized regimen including the choice of many different forms of airway clearance, such as both independent and assisted, exercise, treatments to promote continence and good posture, inhalation therapy, oxygen, and noninvasive ventilation. This article describes the techniques and overall strategies used by physiotherapists in helping people with CF to manage the symptoms and progression of their condition.

Physiotherapy in cystic fibrosis: optimising techniques to improve outcomes

“…3 Optimising inhalation techniques is essential to ensure optimal deposition and drug delivery with each dose. The success of inhalation therapy is highly dependent on indication, the administration device and the inhalation technique.…”

“…New technologies have been developed to produce more homogenous particle sizes and to increase the speed of nebulisation. 3 These include vibrating mesh technology (VMT) systems and adaptive aerosol devices (AAD). These devices are silent, battery powered, portable and have faster delivery times.…”

“…Traditionally, clinicians advise nebulisation 30 minutes prior to airway clearance therapies (ACTs) and that at least one hour should be allowed between the nebulisation of dornase alfa and a nebulised antibiotic to avoid denaturation. 3 This recommendation is based on evidence that dornase alfa makes sputum more pourable within 30 minutes. 15 However alternative timings have been suggested.…”

“…2 The challenge faced by clinicians is to balance CF treatment needed to maintain optimal health status (thus allowing people with CF to achieve personal and professional milestones) with the burden that treatment imposes. 3 It is therefore essential that physiotherapy treatments are optimised to ensure clinical efficacy and optimal outcomes. In essence, the aim of paediatric CF management is to transition 'healthy' children to adult care with normal lung function and nutritional status.…”

Airways Clearance Techniques in Cystic Fibrosis: Physiology, Devices and the Future

“…A number of adjunctive techniques and devices have been used to assist those who are unable, for whatever reason, to clear pulmonary secretions effectively and have been extensively reviewed (Cystic Fibrosis Trust, 2003;Yankaskas et al 2004;Kendrick, 2007;van der Schans, 2007;Bott et al, 2009;Flume et al, 2009;Daniels, 2010).…”