Background: Cystic fibrosis (CF) is a monogenetic autosomal recessive multi-organ disease affecting approximately 50,000 patients worldwide. Overall median survival is continually increasing but pulmonary disease remains the most common cause of death. Guidelines have been published in relation to the outpatient maintenance of lung health for CF patients and treatment of acute lung exacerbations but little information exists about the management of the critically ill CF patient. Invasive mechanical ventilation in CF patients with acute respiratory failure is associated with poor outcome and high mortality.
Methods: Retrospective analysis of adult patients with CF who required endotracheal intubation and invasive mechanical ventilation in the Medical Intensive Care Unit (MICU).
Results: Between the years 2003 - 2009, 14 adult patients with CF required endotracheal intubation and invasive mechanical ventilation in the Medical Intensive Care Unit (MICU) of the Penn State Milton S. Hershey Medical Center, Hershey, Pennsylvania, USA. Eleven patients died in the MICU because of progressive respiratory failure and inability to liberate from mechanical ventilation. Seven individuals consistently manifested arterial partial pressures of carbon dioxide (PaCO(2)) greater than 20.00 kPa despite high levels of conventional modes of mechanical ventilation.
Conclusion: Intubated CF patients with respiratory failure have a high mortality rate. Based on our experience, multiple factors contribute to severe hypercapnia and the effectiveness of conventional modes of mechanical ventilation in many of these patients is limited.
Keywords: Cystic fibrosis; Mechanical ventilation; Critical care; Hypercapnia; Respiratory failure.