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Survival and prognosis after assisted ventilation for acute respiratory failure in cystic fibrosis


The use of mechanical ventilation for acute respiratory failure (ARF) in patients with cystic fibrosis (CF) has long been discouraged because of a poor outcome in this group of patients. However, new insights into ventilatory strategies and a better life expectancy per se in CF patients necessitate regular reevaluation of this attitude. The most recent studies report a mortality in adult CF patients requiring mechanical ventilation that varies widely between 45% and 80%. Results in children are reported as more favourable, but little is known about the long-term pulmonary outcome of children with CF who required mechanical ventilation for ARF already at a young age.


The goals of this study were threefold: to assess outcome of assisted ventilation in CF patients with ARF; to identify risk factors associated with a poor outcome; and to analyse whether children with CF who required mechanical ventilation for ARF at a young age had more pulmonary and CF-related complications later in life compared with unventilated CF controls.


A retrospective cohort study.


The study was performed at the University Medical Centre Utrecht (Utrecht, The Netherlands) and at the Leyenburg Hospital (The Hague, The Netherlands). These centres care for approximately one-half of the Dutch CF population.


Medical charts were reviewed of all CF patients who had been admitted to the intensive care unit for ARF between January 1990 and August 2003. ARF was defined as a respiratory deterioration requiring assisted ventilation, due to an acute illness in a previously stable patient. Assisted ventilation could be either non-invasive or invasive. Patients with chronic respiratory failure with a slow progressive decline in lung function were not included. To identify risk factors for mortality, the following data were recorded: demographic data, body mass index, spirometric data, mode of ventilation, sputum microbiology, history of hemoptysis or pneumothorax, the presence of CF-related diabetes and the presence of CF-related liver disease.

To analyse long-term outcome in children who survived assisted ventilation for ARF, all unventilated age-matched and gender-matched CF controls were identified. Lung function and the presence of CF-related complications 5 years after admission to the ICU were compared.


Thirty-two CF patients were included, five children (aged 2–18 months) and 27 adults (aged 15 years and older). All children and 30% of the adults survived.

In the total population, age was a statistically significant risk factor for poor outcome (P = 0.03). In the adult population, none of the demographic and clinical data could predict outcome significantly. In patients who had an episode of ARF in childhood, clinical course and lung function 5 years after assisted ventilation was not significantly different compared with their controls.


CF patients aged 2 years or younger who are ventilated because of ARF have a good prognosis and assisted ventilation does not seem to affect lung function or development of CF-related complications later in their lives. ARF in adult CF patients still is associated with a high mortality. Risk factors for poor outcome could not be identified.

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van Gestel, S., Slieker, M., Tramper, G. et al. Survival and prognosis after assisted ventilation for acute respiratory failure in cystic fibrosis. Crit Care 9, P222 (2005).

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  • Mechanical Ventilation
  • Cystic Fibrosis
  • Lung Function
  • Cystic Fibrosis Patient
  • Acute Respiratory Failure