We describe a 27-year-old primigravida suffering from cystic fibrosis.
Her chest was colonised with Burkholderia cepacia and she was in resp
iratory failure for which she required constant nasal intermittent pos
itive pressure ventilation. In view other rapid deterioration, Caesare
an section was performed under epidural anaesthesia at 25 weeks gestat
ion. A live 790-g boy was delivered. Post-operatively she made steady
progress for 5 days although still requiring nasal ventilatory support
. Thereafter she developed pneumonia and required tracheal intubation
and ventilation on the eighth day. Her increasing hypoxaemia and pulmo
nary hypertension failed to respond to any therapy including inhaled n
itric oxide and she died on the tenth postoperative day.