Inhaled interventions in cystic fibrosis: mucoactive and antibiotic therapies.

Treatment of cystic fibrosis lung disease has developed from an understanding of the abnormal airway surface liquid resulting from a lack of function of the cystic fibrosis transmembrane regulator protein. Mucus plugging resulting in infection and inflammation leads to airway wall destruction and bronchiectasis. Inhaled therapies have formed the backbone of treatments. In combination with antibiotics delivered direct to the airway, mucoactive drugs, including mucolytics and hyperosmolar agents, are utilised to improve mucociliary clearance to reduce infection and inflammation. Until recently, airway therapies were delivered as nebulisers, but we have now entered the era of dry powder inhalers for treatment of cystic fibrosis lung disease. The theory and practice of these therapies are discussed in this review.

Inhaled mannitol for the treatment of cystic fibrosis.

Inhaled dry powder mannitol is well established for use in bronchial provocation testing. Inhaled mannitol also increases mucociliary clearance, and therefore could have a role in treating chronic suppurative lung disease. There have been a number of studies in cystic fibrosis and bronchiectasis. An international Phase III trial has just been published that suggests the use of regular inhaled mannitol increases lung function and reduces exacerbation frequency in cystic fibrosis. Inhaled mannitol exerts its effects in a number of ways, most importantly like hypertonic saline, setting up an osmotic gradient so water flows into the airway lumen, increasing mucus hydration and mucociliary clearance. Its formulation as a dry powder makes it quick and convenient to take.