Improved outcomes of patients with cystic fibrosis admitted to the intensive care unit

J Cyst Fibros. 2004 Mar;3(1):8-14. doi: 10.1016/j.jcf.2003.12.003.


Background: Invasive mechanical ventilation (IMV) has been previously associated with a poor outcome for patients with cystic fibrosis (CF), but with improved survival and the availability of lung transplantation intensive care unit (ICU) admission is being increasingly considered. This study aimed to review the outcomes of adult CF patients admitted to ICU, and to identify factors that may have influenced outcomes.

Methods: A retrospective audit was conducted of CF patients admitted to ICU. Anthropometric data, spirometry, nutritional status, sputum microbiology, arterial blood gas tensions, and mode of ventilation used were recorded. Immediate and 1-year survival and lung transplantation utilisation were recorded.

Results: Twenty patients were admitted to ICU and nine (45%) survived to hospital discharge. Five of the nine survivors had potentially reversible conditions. Four patients admitted with respiratory exacerbations alone who survived, were maintained on non-invasive ventilation (NIV). The relative risk of deaths for patients with a BMI < 18 and a FEV1 < 24% predicted were 3.25 (1.27-3.25), and 3.68 (1.11-16.33), respectively.

Conclusions: The outcome of patients with CF admitted to ICU has improved, with 45% of the patients in this study surviving to hospital discharge. Five of these survivors underwent successful lung transplantation. Long-term use of NIV post discharge may have contributed to this favourable outcome. A BMI < 18 and FEV1 < 24% predicted were associated with a significantly higher relative risk of death.

MeSH terms

  • Adolescent
  • Adult
  • Cause of Death*
  • Critical Care / methods
  • Cystic Fibrosis / diagnosis
  • Cystic Fibrosis / mortality*
  • Cystic Fibrosis / therapy*
  • Female
  • Hospital Mortality / trends*
  • Hospitalization
  • Humans
  • Intensive Care Units* / statistics & numerical data*
  • Male
  • Medical Audit*
  • New South Wales / epidemiology
  • Retrospective Studies
  • Risk Assessment
  • Severity of Illness Index
  • Survival Analysis
  • Treatment Outcome