Objectives: To assess outcome of assisted ventilation in cystic fibrosis (CF) patients with acute respiratory failure (ARF), to identify risk factors associated with poor outcome and to compare long-term outcome of CF children who were mechanically ventilated for ARF with unventilated CF controls.
Design: Retrospective cohort study.
Setting: Two large CF centres in the Netherlands.
Patients: CF patients who required assisted ventilation for ARF and unventilated CF controls.
Measurements and results: Thirty-one CF patients required assisted ventilation for ARF between January 1990 and March 2005. All five children (under 2 years of age) and seven adults (27%) survived. In the total population, age was a statistically significant risk factor for poor outcome (p=0.02). In adult CF patients who required invasive mechanical ventilation, acute on chronic respiratory failure was associated with poor outcome. In children who required mechanical ventilation for ARF, lung function and CF related complications 5 years later were not significantly different compared with controls matched for age, gender and genotype.
Conclusions: CF patients younger than 2 years old, who are ventilated because of ARF, have a good prognosis and their long-term outcome seems identical to unventilated CF controls. ARF in adult CF patients still is associated with high mortality, especially among patients with acute on chronic respiratory failure.