Assisted ventilation for patients with cystic fibrosis.

Forty-six patients with cystic fibrosis (CF), 1 month to 32 years of 
age, had 51 episodes of assisted ventilation. Thirty-five (69%) died after 
1 to 395 days of mechanical ventilation and six others (12%) 
died in the hospital 1 to 42 days after assisted ventilation 
therapy was discontinued. Only three patients survived longer than one year 
after discharge. These results are much poorer than those reported for 
patients with chronic obstructive pulmonary disease of other causes probably because 
of the different natural history and response to therapy in CF. 
Patients with CF whose conditions progressively deteriorated despite optimal therapy and 
no longer respond to antibiotic and physical therapy are not good 
candidates for mechanical ventilation. The occasional patient with good baseline status 
in whom acute respiratory failure develops should be considered for assisted 
ventilation. 