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).
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.
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.