Treatment
[Cystic fibrosis]
General principle
To prevent irreversible changes by:
- Clearing mucopurulent secretions
- Preventing and treating infections
Treatment
- Physiotherapy
- Physical activity
- Nutrition - improve calory intake
- Antibiotics
- Aerosol and other inhalation agents
- Consider oxygen therapy in those with hypoxemia at rest
- Lung transplant in selected cases
Physiotherapy
More effective when combined with physical exercise.
An effective cough is the most important method of clearing secretion.
Also include:
- postural drainage
- chest percussion
- positive expiratory pressure
- forced expiration techniques
Aerosols and other inhalation agents
Inhalation of sterile normal/hypertonic saline aerosol
-> help clearing secretion.
May trigger asthma attack in some (esp hypertonic saline)
Short-acting beta-2 agonist should be considered.
Dornase alfa (an enzyme that reduces the viscosity of purulent lung secretions) - available under PBS for selected patients.
Antibiotics
Mild pulmonary disease
- First line: Cotrimoxazle AND/OR cefaclor
If not effective after 2-3 weeks, then
- Second line: Amoxycillin/clavulanate
If still not effective after 2-4 weeks, then
- Third line: Ciprofloxacin OR [inhaled gentamicin OR tobramycin]
Moderate pulmonary disease
- First line: Amoxycillin/clavulanate
OR
- Second line: Amoxycillin PLUS di/flucloxacillin
If exacerbation:
- Ciprofloxacin AND/OR [inhaled gentamicin OR tobramycin]
Severe pulmonary disease
(Almost always with pseudomonas aeruginosa)
- Amoxycillin/clavulanate
OR
- Amoxycillin PLUS di/flucloxacillin
OR
- Ciprofloxacin OR chloramphenicol
IF severe exacerbation:
- ADD: inhaled gentamicin or tobramycin