Physiotherapy for cystic fibrosis in Australia and New Zealand

Key points

• The Thoracic Society of Australia and New Zealand (TSANZ) guideline makes 30 graded recommendations on physiotherapy for people with cystic fibrosis (CF) across the lifespan.
• Airway clearance techniques should be performed throughout life; no single technique has proved superior, so the choice should be individualised.
• Head-down postural drainage should not be used routinely in infants or in patients with known or suspected gastro-oesophageal reflux; modified postural drainage is recommended for infants and young children.
• Exercise is recommended at all ages, with prescriptions tailored to the individual; strength training and weight-bearing activity support muscle and bone health.
• Screening for urinary incontinence, non-invasive ventilation (NIV) in selected patients and rehabilitation before and after lung transplantation are part of care.

Overview

This clinical practice guideline aims to optimise physiotherapy management of CF in Australia and New Zealand. It is based on a systematic literature search up to June 2014 (MEDLINE, CINAHL, EMBASE, PEDro), with recommendations graded under the National Health and Medical Research Council (NHMRC) framework (grade shown in brackets). It covers airway clearance, inhalation therapy, exercise, musculoskeletal care, continence, the newly diagnosed patient, NIV and lung transplantation.

Airway clearance

The active cycle of breathing technique (ACBT) (B), positive expiratory pressure (PEP) therapy at 10–20 cmH2O, oscillating PEP and autogenic drainage (B) are all effective and can be performed independently. Several systematic reviews found no single technique superior. Head-down tilt may provoke reflux, dyspnoea and desaturation; in infants, modified (no tilt) postural drainage was linked to fewer radiological changes and better lung function at six years of age. Exercise can reduce the mechanical impedance of sputum (B), give short-term improvements in lung function (A) and ease expectoration (B).

Inhalation therapy

Nebulised drugs should be taken via a mouthpiece where possible (C), with inhalation technique adapted to the device (C). Bronchodilators should be delivered by metered dose inhaler unless nebulisation is clinically needed (C), and inhaled corticosteroids by metered dose inhaler with spacer (B), followed by rinsing the mouth. Hypertonic saline may be inhaled before or during airway clearance (B); the timing of dornase alfa can follow pragmatic reasons or individual preference.

Exercise, musculoskeletal health and continence

An exercise test should be considered for assessment and exercise prescription (C), and a six-minute walk test is part of the initial lung transplant assessment (C). Training should take place on at least three (preferably five or more) days per week, 30 minutes per session, raising heart rate to 75% of maximum and combining aerobic and resistance work; supplemental oxygen should be considered for severe exercise-induced desaturation (C). Spinal pain affects 43–94% of people with CF; a musculoskeletal assessment is advised at annual review from about age eight (C). Urinary incontinence is reported in 22–74% of girls and women with CF; men and women should be screened (C), women with stress incontinence taught pelvic floor exercises (C), and airway clearance done in postures that keep a neutral lumbar spine (C).

New diagnosis, NIV and transplantation

Newly diagnosed infants may receive percussion for 3–5 minutes in each of 5 modified postural drainage positions (B) plus daily age-appropriate play (C); children and adults should do regular exercise (B), with other airway clearance added as required (C). NIV should be considered for acute respiratory failure in patients listed for transplantation (C), may be trialled for symptomatic nocturnal ventilatory failure (B) and can support airway clearance and exercise in severe disease (B), always with heated humidification (C). Exercise training is advised on the transplant waiting list (C) and supervised rehabilitation after transplantation (B). Respiratory equipment should never be shared between patients.

Frequently asked questions

Which airway clearance technique is best in cystic fibrosis?

The guideline notes that no single technique has proved superior; ACBT, PEP, oscillating PEP and autogenic drainage are all effective, so the choice should be individualised.

How often should people with CF exercise?

Training should take place on at least three, preferably five or more, days a week for 30 minutes per session, tailored to the individual.

Why screen for urinary incontinence?

Incontinence is more common in people with CF than in healthy peers and is linked to anxiety, depression and poorer quality of life; physiotherapy treatment can improve pelvic floor strength and reduce leakage.

Source

Button BM, Wilson C, Dentice R, Cox NS, Middleton A, Tannenbaum E, et al. Physiotherapy for cystic fibrosis in Australia and New Zealand: A clinical practice guideline. Respirology 2016;21(4):656-667. doi:10.1111/resp.12764. Thoracic Society of Australia and New Zealand clinical practice guideline (April 2016). Summary prepared by Medpresso from the guideline version published by the Thoracic Society of Australia and New Zealand; it is not a substitute for the full text or for medical advice.