207 Non-invasive ventilation in cystic fibrosis

Cystic fibrosis (CF) is characterized by chronic airway and systemic inflammation complicated by intermittent episodes of acute exacerbations that contribute to significant morbidity. Objectives: To characterize and identify possible risk factors related to exacerbations in CF patients attending the National Reference Center in Uruguay. Methods: We analyzed data from all patients at the database of our center for the period 2011–2012. One patient who died and two patients, who underwent double lung transplantation, were excluded. Exacerbations were defined as acute/ sub-acute worsening of respiratory symptoms severe enough to warrant intravenous (IV) antibiotics and hospitalization. Results: 54 patients were included, 57.4% male, mean age 26±9, BMI 22±4. At least one of the most common mutation delta F508, was present in 35%, pancreatic insufficiency in 44%, diabetes in 9% and hemoptysis in 62%. Up to 70% were colonized with: Pseudomonas aeruginosa (PA) 52%, St. aureus 26.3%, Alcaligenes 13%, Stenotrophomonas 2.6%. Mean FVC 90.1±23%, mean FEV1 80.6±27.8; 18.5% FEV1 <60%. Patients received treatment with: inhaled tobramycin 44%, pulmozyme 51.8%, Hypertonic Saline solution 61%. 35% of patients had at least one exacerbation or hospitalization/year. Using a linear regression model no correlation was found between exacerbations and: sex, age, age at diagnosis, colonization with PA, or hemoptysis. Exacerbations were strongly associated with poor lung function (R: 0.51, p = 0.0001). Conclusions: Data from a well characterized CF population suggest that acknowledging trends in lung function may contribute to identify patients at risk of exacerbation and increased morbidity.