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