Outcome of assisted ventilation for acute respiratory failure in cystic fibrosis
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.
KeywordsCystic fibrosis Intensive care unit Mechanical ventilation Mortality Respiratory failure Risk factors Outcome
- 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
- 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.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.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.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.TNO Preventie en Gezondheid. TNO Growth Calculator. http://groeiweb.pgdata.nl/calculator.asp Date last updated: 1/1/2004. Date last accessed: 1/6/2005Google Scholar
- 19.Pinhu L, Whitehead T, Evans T, Griffiths M (2003) Ventilator-associated lung injury. Lancet 361:332–340Google Scholar