Long-term multicentre randomised controlled study of high frequency chest wall oscillation versus positive expiratory pressure mask in cystic fibrosis.
Study design (if review, criteria of inclusion for studies)
RCT
List of included studies
x
Participants
patients aged >6 years with CF and the G551D mutation.
Interventions
Patients were randomized 1:1 to ivacaftor 150 mg or placebo every 12 h for 48 weeks.
Outcome measures
Primary end point (lung function) was reported previously. Other outcomes included weight and height measurements and CF Questionnaire-Revised (CFQ-R).
Main results
Studies included 213 patients (aged 20 years, n = 108). In patients 20 years, adjusted mean change from baseline to week 48 in body weight was 2.7 versus -0.2 kg (p = 0.0003). Mean BMI change at week 48 was 0.9 versus -0.1 kg/m(2) (p = 0.0003). There was no linear correlation evident between changes in body weight and improvements in lung function or sweat chloride. Significant CFQ-R improvements were seen in perception of eating, body image, and sense of ability to gain weight.
Authors' conclusions
Nutritional status improved following treatment with ivacaftor for 48 weeks.