[Mechanical ventilation by nasal mask in children with cystic fibrosis. Initial results of a non-invasive method].

Background: Chronic respiratory failure (CRF) with hypoxia and hypercapnia is the last ineluctable phase in cystic fibrosis (CF). Nasal positive pressure ventilation (NPPV), a non-invasive method, may be given to CF children with CRF, especially to patients accepted for transplantation (T). This method improves ventilatory function by resting the chronically exhausted respiratory muscles, facilitates bronchial drainage by physiotherapy, prevents the exacerbations of the illness and prepares patients for T.
Methods and Patients: NPPV was used in 6 CF patients (mean age 13 years 6 months). One of them was transplanted 15 days later, two of them were accepted for T. All had hypoxia. Five of them had hypercapnia.
Results: NPPV was given to four patients for 3 to 14 months. The preliminary results were positive. One patient gained weight, two had more fluid sputum. One patient showed an increase in functional respiratory tests (FRT: PaO2, vital capacity, FEV-1) while these tests were stabilized in the others.
Conclusions: NPPV in an effective non-invasive method for use with CF children. It is indicated for CF patients accepted for T and also earlier, for CF patients with CRF in order to prevent acute exacerbations and functional respiratory deterioration.