Outcome of patients with cystic fibrosis admitted to the intensive care unit: is invasive mechanical ventilation a risk factor for death in patients waiting lung transplantation?

OBJECTIVE The admission of patients with cystic fibrosis (CF) to the intensive care unit (ICU) is controversial. Our aim was to study the long-term outcome of patients with CF who were admitted to the ICU and the effect of ventilation modality. METHODS The medical records of 104 admissions (1996-2006) of 48 patients with CF (age 18+/-9 years) were reviewed. Seventeen patients were admitted with reversible conditions (group 1). Thirty-one patients were admitted for acute on chronic respiratory failure (group 2). RESULTS In group 1, 16 of 17 patients survived up to 10 years from ICU admission. Conversely, in group 2, 23 of 31 patients (74%) died of respiratory failure. In group 2, 17 of 18 patients who were mechanically ventilated died within 90 days from admission, and 7 of 10 patients treated for prolonged periods with bi-level positive airway pressure are still alive up to 10 years after admission and transplantation. CONCLUSION Patients requiring mechanical ventilation may have a poor prognosis. The outcome of treatment with bi-level positive airway pressure is good, even in patients who had many episodes of acute respiratory failure.