Outcome of adult patients with cystic fibrosis

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Rev Mal Respir 2002 Sep;19(4):425-30 Related Articles, Links

[Outcome of adult patients with cystic fibrosis admitted to intensive

care with respiratory failure: the role of non-invasive ventilation]

Cadiergue V, Philit F, Langevin B, Durieu I, Bertocchi M, Guerin C,

D.

Service de Reanimation Medicale et d'Assistance Respiratoire, Hopital de

la Croix Rousse, Lyon, France.

Recourse to mechanical ventilation may prove necessary in adult patients

with cystic fibrosis who have reached the stage of severe respiratory

insufficiency. We report the experience of an intensive care service

using non-invasive ventilation (NIV) as the first step in the management

of acute respiratory failure in these patients. The records of 16

patients with cystic fibrosis presenting with acute respiratory failure

and treated with NIV were analysed retrospectively. The characteristics

of the group were: mean age 26.9 +/- 9.5 years; mean FEV1 21.5 +/- 10.4%

predicted; mean body mass index 16.8 +/- 2.1; mean Pa CO(2) on admission

66 +/- 15 mm Hg. The mean duration of NIV in the ICU was 10 +/- 7 days.

Eight patients (50%) died after having been intubated on account of

failure of NIV. The eight survivors were discharged home with long-term

NIV (mean duration 235 +/- 158 days). Six of them have received a lung

transplant. The mode of onset of respiratory failure was an important

prognostic factor: a rapid onset (<7 days) was invariably associated with

death, on the other hand a gradual deterioration (> 7 days) was noted in

the eight patients able to leave the ICU. In conclusion NIV may be

regarded as the treatment of choice in patients with cystic fibrosis

admitted to ICU with respiratory failure. In the case of persistent

hypercapnia after the acute episode long-term NIV may keep them stable

while awaiting lung transplantation.

PMID: 12417858 [PubMed - indexed for MEDLINE]

Becki

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