Intensive Care Medicine

, Volume 32, Issue 5, pp 754–758 | Cite as

Outcome of assisted ventilation for acute respiratory failure in cystic fibrosis

  • Martijn G. Slieker
  • Josephus P. J. van Gestel
  • Harry G. M. Heijerman
  • Gerdien A. Tramper-Stranders
  • Ferdinand Teding van Berkhout
  • Cornelis K. van der Ent
  • Nicolaas J. G. Jansen
Brief Report



To assess outcome of assisted ventilation in cystic fibrosis (CF) patients with acute respiratory failure (ARF), to identify risk factors associated with poor outcome and to compare long-term outcome of CF children who were mechanically ventilated for ARF with unventilated CF controls.


Retrospective cohort study.


Two large CF centres in the Netherlands.


CF patients who required assisted ventilation for ARF and unventilated CF controls.



Measurements and results

Thirty-one CF patients required assisted ventilation for ARF between January 1990 and March 2005. All five children (under 2 years of age) and seven adults (27%) survived. In the total population, age was a statistically significant risk factor for poor outcome (p = 0.02). In adult CF patients who required invasive mechanical ventilation, acute on chronic respiratory failure was associated with poor outcome. In children who required mechanical ventilation for ARF, lung function and CF related complications 5 years later were not significantly different compared with controls matched for age, gender and genotype.


CF patients younger than 2 years old, who are ventilated because of ARF, have a good prognosis and their long-term outcome seems identical to unventilated CF controls. ARF in adult CF patients still is associated with high mortality, especially among patients with acute on chronic respiratory failure.


Cystic fibrosis Intensive care unit Mechanical ventilation Mortality Respiratory failure Risk factors Outcome 

Supplementary material

134_2006_85_MOESM1_ESM.doc (116 kb)
Electronic Supplementary Material (DOC 118kb)


  1. 1.
    Davis PB, di Sant'Agnese PA (1978) Assisted ventilation for patients with cystic fibrosis. JAMA 239:1851–1854PubMedCrossRefGoogle Scholar
  2. 2.
    Slieker MG, Uiterwaal CSPM, Sinaasappel M, Heijerman HGM, van der Laag J, van der Ent CK (2005) Birth prevalence and survival in cystic fibrosis - A national cohort study in the Netherlands. Chest 128:2309–2315PubMedCrossRefGoogle Scholar
  3. 3.
    Kulich M, Rosenfeld M, Goss CH, Wilmott R (2003) Improved survival among young patients with cystic fibrosis. J Pediatr 142:631–636PubMedCrossRefGoogle Scholar
  4. 4.
    Hodson ME, McKenzie S, Harms HK, Koch C, Mastella G, Navarro J, Strandvik B (2003) Dornase alfa in the treatment of cystic fibrosis in Europe: A report from the Epidemiologic Registry of Cystic Fibrosis. Pediatr Pulmonol 36:427–432PubMedCrossRefGoogle Scholar
  5. 5.
    Charman SC, Sharples LD, McNeil KD, Wallwork J (2002) Assessment of survival benefit after lung transplantation by patient diagnosis. J Heart Lung Transplant 21:226–232PubMedCrossRefGoogle Scholar
  6. 6.
    Liou TG, Adler FR, Cahill BC, Fitzsimmons SC, Huang D, Hibbs JR, Marshall BC (2001) Survival effect of lung transplantation among patients with cystic fibrosis. JAMA 286:2683–2689PubMedCrossRefGoogle Scholar
  7. 7.
    Piper AJ, Parker S, Torzillo PJ, Sullivan CE, Bye PT (1992) Nocturnal nasal IPPV stabilizes patients with cystic fibrosis and hypercapnic respiratory failure. Chest 102:846–850PubMedCrossRefGoogle Scholar
  8. 8.
    Amato MB, Barbas CS, Medeiros DM, Magaldi RB, Schettino GP, Lorenzi-Filho G, Kairalla RA, Deheinzelin D, Munoz C, Oliveira R, Takagaki TY, Carvalho CR (1998) Effect of a protective-ventilation strategy on mortality in the acute respiratory distress syndrome. N Engl J Med 338:347–354PubMedCrossRefGoogle Scholar
  9. 9.
    Hodson ME, Madden BP, Steven MH, Tsang VT, Yacoub MH (1991) Non-invasive mechanical ventilation for cystic fibrosis patients—a potential bridge to transplantation. Eur Respir J 4:524–527PubMedGoogle Scholar
  10. 10.
    Vedam H, Moriarty C, Torzillo PJ, McWilliam D, Bye PT (2004) Improved outcomes of patients with cystic fibrosis admitted to the intensive care unit. J Cyst Fibros 3:8–14PubMedCrossRefGoogle Scholar
  11. 11.
    Berlinski A, Fan LL, Kozinetz CA, Oermann CM (2002) Invasive mechanical ventilation for acute respiratory failure in children with cystic fibrosis: outcome analysis and case-control study. Pediatr Pulmonol 34:297–303PubMedCrossRefGoogle Scholar
  12. 12.
    Sood N, Paradowski LJ, Yankaskas JR (2001) Outcomes of intensive care unit care in adults with cystic fibrosis. Am J Respir Crit Care Med 163:335–338PubMedGoogle Scholar
  13. 13.
    Garland JS, Chan YM, Kelly KJ, Rice TB (1989) Outcome of infants with cystic fibrosis requiring mechanical ventilation for respiratory failure. Chest 96:136–138PubMedCrossRefGoogle Scholar
  14. 14.
    Slieker MG, van Gestel JPJ, Tramper-Stranders GA, Heijerman HG, Teding van Berkhout F, van der Ent CK, Jansen NJG (2005) Survival and prognosis after assisted ventilation for acute respiratory failure in cystic fibrosis (abstract). J Cyst Fibros 3(S1):71Google Scholar
  15. 15.
    van der Laag J, Sinaasappel M, Heijerman HG (1998) Diagnostiek en behandeling van cystic fibrosis [Diagnosis and treatment of cystic fibrosis]. Centraal Begeleidingsorgaan voor de Intercollegiale Toetsing, UtrechtGoogle Scholar
  16. 16.
    Fuchs HJ, Borowitz DS, Christiansen DH, Morris EM, Nash ML, Ramsey BW, Rosenstein BJ, Smith AL, Wohl ME (1994) Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group. N Engl J Med 331:637–642PubMedCrossRefGoogle Scholar
  17. 17.
    Serra A, Polese G, Braggion C, Rossi A (2002) Non-invasive proportional assist and pressure support ventilation in patients with cystic fibrosis and chronic respiratory failure. Thorax 57:50–54Google Scholar
  18. 18.
    TNO Preventie en Gezondheid. TNO Growth Calculator. Date last updated: 1/1/2004. Date last accessed: 1/6/2005Google Scholar
  19. 19.
    Pinhu L, Whitehead T, Evans T, Griffiths M (2003) Ventilator-associated lung injury. Lancet 361:332–340Google Scholar

Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • Martijn G. Slieker
    • 1
  • Josephus P. J. van Gestel
    • 2
  • Harry G. M. Heijerman
    • 3
  • Gerdien A. Tramper-Stranders
    • 1
  • Ferdinand Teding van Berkhout
    • 1
  • Cornelis K. van der Ent
    • 1
  • Nicolaas J. G. Jansen
    • 2
  1. 1.Cystic Fibrosis Centre UtrechtUniversity Medical Centre UtrechtUtrechtThe Netherlands
  2. 2.Paediatric Intensive Care Unit, Wilhelmina Children's HospitalUniversity Medical Centre UtrechtUtrechtThe Netherlands
  3. 3.Adult Cystic Fibrosis CentreHaga Teaching HospitalThe HagueThe Netherlands

Personalised recommendations