LONG-TERM NASAL INTERMITTENT POSITIVE PRESSURE VENTILATION IN PATIENTS WITH CYSTIC-FIBROSIS AND HYPERCAPNIC RESPIRATORY-FAILURE (1991-1996)

In patients with cystic fibrosis (CF), nasal intermittent positive pre ssure ventilation (NIPPV) is currently used as a short-term bridge to transplantation but its precise role has yet to be determined. Patient s were offered a therapeutic trial of NIPPV when candidates for lung t ransplantation, with respiratory failure unresponsive to medical treat ment. Twelve patients, six male of mean age of 26 +/- 1.4 years, had a trial of NIPPV. At recruitment the mean percentage predicted forced e xpired volume in one second (FEV1) was 15.1% +/- 1.2%, arterial carbon dioxide (PaCO2) 8.7 +/- 0.6 kPa, arterial oxygen (PaO2) with variable FiO(2) 7.4 +/- 0.6 kPa and arterial bicarbonate (HCO3-) 40.1 +/- 1.6 mmol l(-1). Ten cases tolerated NIPPV for 1-15 months, mean 5.1 +/- 1. 4 months, with subjective improvement in headache and quality of sleep . At 3 months, there was significant improvement in forced vital capac ity, PaCO2 and arterial HCO3- and there was a reduction in the number of hospital inpatient days (P<O.05). Subsequently three cases had lung transplantation, four died on the active list and three are awaiting organs. Two patients failed to tolerate NIPPV owing to abdominal bloat ing and increasing hypercapnia. In conclusion, NIPPV, if tolerated, wa s a useful adjunct in the treatment of CF patients with hypercapnic re spiratory failure awaiting transplantation. Further prospective studie s are required to determine the optimum time to commence NIPPV and to clarify its precise role.