313 An international study of survival of children with cystic fibrosis after the first episode of invasive mechanical ventilation
Journal of Cystic Fibrosis, 2015
The outcome of cystic fibrosis (CF) patients who require invasive mechanical ventilation (IMV) is... more The outcome of cystic fibrosis (CF) patients who require invasive mechanical ventilation (IMV) is generally considered to be poor, although limited data are available. Objectives To determine a current estimate of survival in the UK, Ireland, Australia and New Zealand amongst children with CF requiring IMV, in a setting where both CF services and intensive care are well resourced. Methods Analysis of data from two clinical databases collected on Paediatric Intensive Care Unit (PICU) admissions in the UK and Ireland (PICANet database) and Australia and New Zealand (ANZPIC database). The primary outcome was survival at discharge from PICU of patients after their first episode of IMV. Results There were 139 CF admissions to intensive care in the PICANet study population. The survival to discharge for these patients was 121/139 (87.1%; 95% Confidence Interval: 80.5 to 91.7%). There were 84 CF admissions to intensive care in the ANZPIC study population. The survival to discharge for these patients was 79/84 (94.0%; 95% CI 86.8 to 97.4%). Survival was better for younger age groups (log-rank test p Conclusion The survival at discharge from paediatric intensive care for non-transplant admissions that required mechanical ventilation in patients with CF is better than previously reported. IMV should not be withheld solely due to a diagnosis of CF.
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