CF Management

• Airway Clearance:

  • Daily airway clearance regimen for life
  • Start as soon as diagnosed even if asymptomatic
  • Teach airway clearance techniques to parents
  • No head-down tilt for infants/young children
  • Once child is approx 7 years of age should be able to do own airway clearance with supervision
  • Adherence may be a problem with adolescent patients
• Airway Clearance methods:

  • percussion
  • gravity assisted positioning
• Timing of physiotherapy and inhaled medication:

  1. DNase – why pre-physiotherapy? - it breaks down sputum, making it more runny
  2. Inhaled bronchodilator – why pre-physiotherapy? - makes airways more dilated which allows sputum to come out more easily
  3. Hypertonic saline/mannitol – why pre-physiotherapy? - draws water into airways, which facilitates airway clearance
  4. Physiotherapy treatment
  5. Inhaled steroid – why post-physiotherapy? - can reach further into lungs after airway clearance physio
  6. Inhaled antibiotic – why post-physiotherapy? - can reach further into lungs after airway clearance physio
• Exercise:

  • Encourage regular exercise as well as airway clearance
  • No group exercise sessions (infection control)