Objective
The admission of patients with cystic fibrosis (CF) to the intensive care unit (ICU)
is controversial. Our aim was to study the long-term outcome of patients with CF who
were admitted to the ICU and the effect of ventilation modality.
Methods
The medical records of 104 admissions (1996-2006) of 48 patients with CF (age 18±9 years) were reviewed. Seventeen patients were admitted with reversible conditions
(group 1). Thirty-one patients were admitted for acute on chronic respiratory failure
(group 2).
Results
In group 1, 16 of 17 patients survived up to 10 years from ICU admission. Conversely,
in group 2, 23 of 31 patients (74%) died of respiratory failure. In group 2, 17 of
18 patients who were mechanically ventilated died within 90 days from admission, and
7 of 10 patients treated for prolonged periods with bi-level positive airway pressure
are still alive up to 10 years after admission and transplantation.
Conclusion
Patients requiring mechanical ventilation may have a poor prognosis. The outcome of
treatment with bi-level positive airway pressure is good, even in patients who had
many episodes of acute respiratory failure.
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Heart & Lung: The Journal of Cardiopulmonary and Acute CareAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Lung transplantation and survival in children with cystic fibrosis.N Engl J Med. 2007; 357: 2143-2152
- Early pulmonary infection, inflammation, and clinical outcomes in infants with cystic fibrosis.Pediatr Pulmonol. 2001; 32: 356-366
- Assisted ventilation for patients with cystic fibrosis.JAMA. 1978; 239: 1851-1854
- Invasive mechanical ventilation for acute respiratory failure in children with cystic fibrosis: outcome analysis and case-control study.Pediatr Pulmonol. 2002; 34: 297-303
- Pre-transplant mechanical ventilation and outcome in patients with cystic fibrosis.J Heart Lung Transplant. 2003; 22: 433-438
- Long-term non-invasive positive pressure ventilation among cystic fibrosis patients awaiting lung transplantation.Isr Med Assoc J. 2004; 6: 527-530
- Outcomes of intensive care unit care in adults with cystic fibrosis.Am J Respir Crit Care Med. 2001; 163: 335-338
- Improved outcomes of patients with cystic fibrosis admitted to the intensive care unit.J Cyst Fibros. 2004; 3: 8-14
- Improved survival following lung transplantation with long-term use of bilevel positive pressure ventilation in cystic fibrosis.Lung. 2007; 185: 73-79
- Non-invasive mechanical ventilation for cystic fibrosis patients-a potential bridge to transplantation.Eur Respir J. 1991; 4: 524-527
- Lung transplantation in cystic fibrosis: consensus conference statement.Chest. 1998; 113: 217-226
- Pre-transplant mechanical ventilation increases short-term morbidity and mortality in pediatric patients with cystic fibrosis.J Heart Lung Transplant. 2007; 26: 127-131
- One-year outcome after severe pulmonary exacerbation in adults with cystic fibrosis.Am J Respir Crit Care Med. 2005; 171: 158-164
- Lung transplantation and end of life issues in cystic fibrosis.Paediatr Respir Rev. 2000; 1: 114-120
- The pulmonary physician in critical care. Illustrative case: cystic fibrosis.Thorax. 2003; 58: 357-360
- Cystic fibrosis in adults: outcome after admission to intensive care (Abstract).Pediatr Pulmonol. 2000; : 292
- Nocturnal nasal IPPV stabilizes patients with cystic fibrosis and hypercapnic respiratory failure.Chest. 1992; 102: 846-850
- Non-invasive ventilation assists chest physiotherapy in adults with acute exacerbations of cystic fibrosis.Thorax. 2003; 58: 880-884
- Prediction of mortality in patients with cystic fibrosis.N Engl J Med. 1992; 326: 1187-1191
- Determinants of mortality for adults with cystic fibrosis admitted in Intensive Care Unit: a multicenter study.Respir Res. 2006; 26: 7-14
- Lung transplantation: a decade of experience.J Heart Lung Transplant. 2005; 24: 145-151
Article info
Publication history
Published online: August 26, 2009
Footnotes
Conflict of interest statement: There is no conflict of interest relating to the authors of this article.
Funding: None.
Identification
Copyright
© 2010 Elsevier Inc. Published by Elsevier Inc. All rights reserved.