Progressive lung disease in patients with cystic fibrosis (CF) is caus
ed by thick secretions, which cause airway obstruction and subsequent
colonization and infection by inhaled pathogenic microorganisms. Recen
tly, recombinant human DNase has been shown to reduce the viscoelastic
ity of sputum in patients with cystic fibrosis and to improve lung fun
ction. Ultrasonically nebulized hypertonic saline (HS) has been demons
trated to enhance mucociliary clearance and sputum expectoration by re
hydrating airway secretions, and may therefore provide a low cost alte
rnative. We studied the changes in pulmonary function and symptoms in
a group of patients with CF who have moderate to severe lung disease.
The patients were evaluated following 2 weeks of treatment with HS in
an open-label study. Subjects were randomly allocated to receive 10 ml
of either 0.9% NaCl (IS) or 6% NaCl (HS). Twice daily, prior to physi
otherapy, treatments were delivered by a portable ultrasonic nebulizer
. To prevent bronchoconstriction, 600 mg of salbutamol was administere
d prior to the nebulized solutions. A symptom score was recorded and s
pirometry was performed on day 0 before therapy was started, on day 14
(the last day of therapy), and on day 28 (14 days after the last trea
tment with either IS or HS). Fifty-two patients (32 males), with a mea
n age of 16.2 (range 7-36) years completed the study. There was no dif
ference in baseline characteristics between the two groups. Following
2 weeks of treatment, there was a significant improvement from baselin
e in FEV(1) of 15.0 +/- 16.0% (mean +/- SD) in patients treated with H
S, compared with a change of 2.8 +/- 13% in those on IS therapy (P = 0
.004). Furthermore, there was a subjective improvement in the effectiv
eness of chest physiotherapy as reported by those using HS (P = 0.02).
The treatment was well tolerated. We conclude that in patients with C
F, ultrasonically nebulized hypertonic saline improves lung function i
n a way similar to that reported for human recombinant DNase when inha
led over a 2 week period. Nebulized saline also enhances the perceptio
n of effectiveness of chest physiotherapy. (C) 1996 Wiley-Liss, Inc.