[Inhaled therapies in cystic fibrosis]

Delivery of drugs by the inhaled route is particularly attractive in cystic fibrosis despite heterogeneous and variable deposition in the lungs.

Proposed therapies include drugs aimed at improving the mucus quality (nebulised hypertonic saline or RhDNase and, in the future, mannitol as a dry powder) or treating chronic colonization and early infection with Pseudomonas aeruginosa (nebulised tobramycine and colistine and, soon, aztreonam lysate, ciprofloxacin and liposomal amikacine). Bronchodilators need to be tested. Corticosteroids have no proven benefit. Other drugs (gene therapy, L-arginine, anti-proteases, etc...) are under development or on trial. In practice, mixtures of drugs should be avoided and the chronological order between drugs observed to achieve correct delivery.

Developments in pharmaceutics and device technology allow us to consider new therapeutic approaches in cystic fibrosis.

More and more efficacious but expensive inhaled treatments are now, and will in the next future, be available for cystic fibrosis patients. Teaching of good inhalational technique and proper hygiene concerning the devices is an absolute necessity before any inhaled treatment is prescribed.