Non invasive mechanical ventilation in cystic fibrosis: physiological effects and monitoring.

Chronic respiratory insufficiency in cystic fibrosis (CF) is characterised by an increase in respiratory load, which leads to alveolar hypoventilation with the preservation of respiratory muscle strength. Non-invasive mechanical ventilation (NIMV) is able to improve alveolar ventilation and thus gas exchange and allow the the respiratory muscles to rest in patients with cystic fibrosis with variable severity of lung disease. These effects state the superiority of NIMV compared to oxygen therapy in CF. Invasive, highly specialized monitoring is recommended for the evaluation of ventilatory modes and the performances of new ventilators. After a successful evaluation, the monitoring of patients starting NIMV can be performed with simple, non invasive parameters such as gas exchange, tidal volume, respiratory rate, minute ventilation and comfort. In the long term however, studies are warranted to assess the benefit of NIMV in terms of lung function, quality of sleep and most importantly, quality of life.