7. Disease
        Genetic
            Cystic fibrosis
Treatment [Cystic fibrosis]

Treatment
[Cystic fibrosis]

General principle

To prevent irreversible changes by:

Treatment

 

Physiotherapy

More effective when combined with physical exercise.

An effective cough is the most important method of clearing secretion.

Also include:

 

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

If not effective after 2-3 weeks, then

If still not effective after 2-4 weeks, then

Moderate pulmonary disease

If exacerbation:

Severe pulmonary disease

(Almost always with pseudomonas aeruginosa)

IF severe exacerbation:

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