Specific aspects of lung care in adults with cystic fibrosis

Respiratory impairment is present in almost all adult cystic fibrosis patie nts and makes the prognosis. Viscous, infected and abundant secretions, inf lammation and bronchial oedema, bronchoconstriction and respiratory muscle fatigue lead to airway obstruction, bronchiectasies and respiratory failure . The disease is preferentially located in the upper lobes. Exacerbations o f the disease are due to bronchial infections and are often responsible for drops of the respiratory function. Regular spirometric surveillance is fun damental for the prognosis and the assessment of the effects of the treatme nt. Among adult patients chronic colonisation with mucoid and often multire sistant strains of Pseudomonas Aeruginosa are common. It is treated with TV high doses antibiotic courses and nebulized antibiotics between IV courses . Respiratory failure may require long term oxygen and non invasive mechani cal ventilation. Systemic hypervascularisation around the bronchiectectasie s may lead to moderate to severe hemoptysis, which may require embolization . Pneumothorax are associated with poor prognosis and are treated by pleura l drainage and if failure by thoracoscopy.