Effects of a supervised, outpatient exercise and physiotherapy programme in children with cystic fibrosis

Donald Urquhart1, Zoe Sell, Elaine Dhouieb

  • 1Department of Paediatric Respiratory Medicine, Royal Hospital for Sick Children, Edinburgh, UK. don.urquhart@luht.scot.nhs.uk

Insights

A year-long supervised exercise and physiotherapy program for children with cystic fibrosis (CF) significantly reduced intravenous antibiotic use and improved exercise capacity. The program also enhanced quality of life, showing a trend toward reduced lung function decline in CF patients.

Area of Science:

  • Pediatric Pulmonology
  • Rehabilitation Medicine
  • Clinical Exercise Physiology

Background:

  • Children with cystic fibrosis (CF) often experience reduced exercise capacity and frequent exacerbations requiring intravenous (IV) antibiotics.
  • Previous studies suggest outpatient exercise and physiotherapy may benefit CF patients.
  • The long-term effects of supervised programs on key CF outcomes require further investigation.

Purpose of the Study:

  • To evaluate the impact of a year-long supervised outpatient exercise and physiotherapy program on children with CF.
  • To assess changes in exercise capacity, lung function, quality of life, and IV antibiotic requirements.

Main Methods:

  • 12 children with CF (age ≥10 years) with ≥4 IV antibiotic courses in 2009 participated.
  • Participants engaged in fortnightly supervised exercise and physiotherapy sessions throughout 2010.
  • They also performed home exercise 3x/week and additional physiotherapy when unwell.

Main Results:

  • A significant reduction in IV antibiotic days from 60 to 50 days (P=0.02) was observed.
  • Exercise capacity, measured by Modified Shuttle Test (MST) distance and level attained, significantly improved (P=0.04).
  • Quality of life scores (CFQ-UK) significantly improved across physical, emotional, treatment, and respiratory domains (P≤0.002).

Conclusions:

  • Supervised outpatient exercise and physiotherapy programs improve quality of life and exercise tolerance in children with CF.
  • These programs are associated with reduced IV antibiotic use and a trend toward slower lung function decline.
  • The cost savings from reduced IV antibiotic use have implications for healthcare service planning.

Related Concept Videos

Chest Physiotherapy01:24

Chest Physiotherapy

Chest Physiotherapy (CPT) is a therapeutic technique used in respiratory care to improve ventilation, clear bronchial secretions, and enhance the efficiency of respiratory muscles. This therapy includes three primary procedures: postural drainage, percussion, and vibration. It can be performed on spontaneously breathing patients and those who are intubated and mechanically ventilated.
Purpose
CPT is primarily used for patients with excessive bronchial secretions who have difficulty clearing...
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic sinusitis...
Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...