Tolerability of Hypertonic Saline in Infants With Cystic Fibrosis

The objective of the study is to determine whether or not inhalation of hypertonic saline will be tolerated by infants with cystic fibrosis and the effect of inhalation on their lung function.

Cystic fibrosis lung disease is characterized by mucous retention which favors secondary bacterial infection and inflammation, which leads to lung damage and ultimately respiratory failure. Classically, therapeutic interventions are aimed to improve mucociliary clearance, to reduce both bacterial load and lower airway inflammation. Hypertonic saline (HS) has been used for the induction of sputum production in all age groups to obtain secretions from the lower respiratory for diagnostic purposes. Hypertonic saline is also used in older children with CF, who do not produce sputum spontaneously, to obtain representative samples for microbiology. There is evidence from studies in patients with cystic fibrosis that HS can improve mucociliary clearance. The improvement was more impressive in areas that were well ventilated, making it likely that HS will work better in patients with relatively preserved pulmonary function. Newer evidence also suggests that the osmotic effect on the airway surface that was expected to be short lived, may actually persist for longer time periods (up to 8 hours). All these data indicate that HS may be a useful agent in the treatment of CF patients. As the effect on mucociliary clearance was found to be better in areas with adequate ventilation, it is logical to assume that treatment with HS may be most efficacious when initiated early in the disease process. So far, no data on the tolerability of inhalation of HS are available for infants with cystic fibrosis. However, evidence from infants with AIDS as well as recent studies in infants with bronchiolitis suggest that hypertonic saline can be safely administered by inhalation in infants. Nevertheless, proof of tolerability in CF infants is a prerequisite for longer term studies of HS in this age group. In older children, tolerability has been tested by measuring pulmonary function both before and after inhalation of HS saline. Similar data are not yet available for infants.

5 ml of 7% saline will be administered via mask with Pari LC Plus nebuliser and a Pari Ultra Ned compressor.

The immediate effect of HS on lung function in infants with CF, as measured by differences in expiratory flows before and after inhalation of hypertonic saline

The usefulness of inhalation of hypertonic saline as a technique to obtain secretions from the lower respiratory tract for microbiological diagnosis

Inclusion Criteria: Confirmed diagnosis of cystic fibrosis Age 2 months to 2 years Routinely scheduled infant pulmonary function test. Clinical stability (no respirator tract infection for 4 weeks prior to study Exclusion Criteria: Acute respiratory symptoms Wheezing at the time of study Supplemental oxygen therapy Oxygen saturation < 95 % on room air