J Pediatr. 1989 Mar;114(3):368-77.

Multicenter randomized double-blind parallel controlled trial.

All participants with FEF 25-75 < 25% predicted, and whose awake arterialized capillary PO2 < 65 mm Hg on 2 occasions. 10 patients dropped out voluntarily (4 in oxygen group). O2 group (10 completed study): dropouts 2 died during follow-up; 2 survived. Air group (8 completed study, all alive at follow-up): dropouts 4 died at follow-up; 2 survived. 28 people with CF older than 12 years of age. Oxygen group mean age 23 +/- 6.7 years, air group mean age 22.1 +/- 4.9 years. 9 males and 5 females in each group.

O2 therapy or room air gas at night only, titrated to increase awake PaO2 > 70 mm Hg.

Mortality, lung function, anthropometrics, RAG, psychosocial questionnaire.

Over the follow-up period there were four deaths in each group, and oxygen therapy had no significant effect on the frequency of hospitalizations. Progression of disease was ascertained from nutritional status, pulmonary function, blood gas values, exercise ability, and right ventricular ejection fraction response to exercise (as measured by equilibrium-gated radionuclide angiocardiography), and psychologic status was measured by standardized tests of mood, self-esteem, and cognitive function; group comparisons for the first year revealed no significant differences; however, school or work attendance was maintained in the oxygen group but deteriorated in the air group. Clinical signs of cor pulmonale were documented during follow-up in 10 patients in toto, and all lived at least 9 months from the onset of these signs.

The lack of association between the onset of these signs and imminent death, or the usefulness of measurements of the maximal oxygen uptake during progressive exercise and the right ventricular ejection fraction response to exercise as prognostic indicators, suggest that death may not be the result of cor pulmonale. We conclude that nocturnal oxygen treatment in patients with cystic fibrosis did not appear to affect mortality rates, the frequency of hospitalizations, or the progression of disease; oxygen use should be instituted only after the development of symptoms related to hypoxemia.