Assisted ventilation for patients with cystic fibrosis
P. B. Davis and P. A. di Sant'Agnese
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.
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