Outcome of patients with cystic fibrosis admitted to the intensive care unit: is invasive mechanical ventilation a risk factor for death in patients waiting lung transplantation?
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.


CONCLUSIONS
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.
Efrati
Ori
O
Pediatric Pulmonology Unit, The Edmond and Lili Safra Children's Hospital, Sheba Medical Center Ramat-Gan, Tel Hashomer 52621, Israel. ori.efrati@sheba.health.gov.il
Bylin
Irena
I
Segal
Eran
E
Vilozni
Daphna
D
Modan-Moses
Dalit
D
Vardi
Amir
A
Szeinberg
Amir
A
Paret
Gideon
G
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000401:mortality* / Q000503:physiopathology; D005260:Female; D006801:Humans; D007223:Infant; D007362:Intensive Care Units; D016040:Lung Transplantation; D008297:Male; D011379:Prognosis; D012121:Respiration, Artificial / Q000009:adverse effects*; D012129:Respiratory Function Tests; D012131:Respiratory Insufficiency / Q000401:mortality* / Q000503:physiopathology; D012307:Risk Factors; D015996:Survival Rate; D016896:Treatment Outcome