We report our experience of using noninvasive ventilation (NIV) in 15 adult cystic fibrosis (CF) patients with chronic respiratory failure, admitted in intensive care unit (ICU) during episodes of acute infectious exacerbation. The mean age of the patients was 28.73 ± 5.66 years, 8 males and 7 females. Prior to ICU admission, all patients had had FEV1<51%. Oxygen saturation on room air was 83,5%±4.5 with PCO2 raging from 4.2 to 6.59 kPa. At admission all were initially treated with NIV to avoid adverse effects of tracheal intubation, none of the patient required intubation. We used NIV-BiPAP mode with 40% of oxygen, aiming to maintain stable hemodynamic in all patients. Settings were adjusted to arterial blood gas values. Once the SatO2 was stable 90% or over and PCO2 ranged from 4 to 5.29kPa the NIV was stopped in all but one patient. Length of stay in ICU was 13,13±5,35 days. All patients were discharged alive from the ICU. In the follow up a year after discharge we recorded 1 death, 4 patients included in lung-transplant waiting list, 3 patients in a process for lung transplant evaluation and the rest of the patients hadn’t required re-admissions in ICU. Our results confirm the published data that NIV can serve as a bridge towards lung transplantation for CF patients with end-stage lung disease and not merely prolonging the suffering. NIV improved hypoxia and fairly corrected hypercapnia in our patients. For these patients, there is a possibility of prolonging life if they are successfully treated for their acute episode of respiratory failure until transplantation.
 

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