ABSTRACT
QUESTION: During an acute exacerbation of cystic fibrosis, is non-invasive ventilation beneficial as an adjunct to the airway clearance regimen? DESIGN: Randomised controlled trial with concealed allocation and intention-to-treat analysis. PARTICIPANTS: Forty adults with moderate to severe cystic fibrosis lung disease and who were admitted to hospital for an acute exacerbation. INTERVENTION: Comprehensive inpatient care (control group) compared to the same care with the addition of non-invasive ventilation during airway clearance treatments from Day 2 of admission until discharge (experimental group). OUTCOME MEASURES: Lung function and subjective symptom severity were measured daily. Fatigue was measured at admission and discharge on the Schwartz Fatigue Scale from 7 (no fatigue) to 63 (worst fatigue) points. Quality of life and exercise capacity were also measured at admission and discharge. Length of admission and time to next hospital admission were recorded. RESULTS: Analysed as the primary outcome, the experimental group had a greater rate of improvement in forced expiratory volume in 1 second (FEV1) than the control group, but this was not statistically significant (MD 0.13% predicted per day, 95% CI -0.03 to 0.28). However, the experimental group had a significantly higher FEV1 at discharge than the control group (MD 4.2% predicted, 95% CI 0.1 to 8.3). The experimental group reported significantly lower levels of fatigue on the Schwartz fatigue scale at discharge than the control group (MD 6 points, 95% CI 1 to 11). There was no significant difference between the experimental and control groups in subjective symptom severity, quality of life, exercise capacity, length of hospital admission or time to next hospital admission. CONCLUSION: Among people hospitalised for an acute exacerbation of cystic fibrosis, the use of non-invasive ventilation as an adjunct to the airway clearance regimen significantly improves FEV1 and fatigue. TRIAL REGISTRATION: ANZCTR 12605000437662.
Subject(s)
Cystic Fibrosis/therapy , Noninvasive Ventilation , Adolescent , Adult , Cystic Fibrosis/physiopathology , Female , Humans , Male , Quality of Life , Respiratory Function Tests , Treatment Outcome , Young AdultABSTRACT
BACKGROUND: Although the benefits of recombinant human deoxyribonuclease (dornase alfa) in patients with cystic fibrosis (CF) are established, its optimal timing in relation to physiotherapy is unknown. As its enzymatic effect lasts for 6-11 hr, dornase alfa may be more efficacious if the time interval between inhalation and chest physiotherapy is increased. The aim of this study was to investigate if a longer time interval between dornase alfa nebulization and chest physiotherapy improves clinical outcomes of subjects with CF. METHODS: A single-blind randomized cross-over trial was conducted on subjects with CF from outpatients of four hospitals. Subjects were in stable health and studied over 6 weeks (utilizing 14-day blocks of morning or evening dornase alfa administration with 14 days washout). Usual regimens for physiotherapy and exercise were unaltered. Thus changing the times altered the dwell time of dornase alfa prior to physiotherapy. Long interval was defined as dwell time of >6 hr and short as < or =6 hr. Outcomes were measured at pre and post each regimen. RESULTS: Twenty subjects aged 7-40 years completed the study. At end of long interval regimen, (median interval = 11.1 hr), FEF(25-75%) and CF-specific quality of life significantly improved compared to baseline values and to short interval regimen (median interval = 0.25 hr) outcomes. FVC, FEV(1), sputum weights, and adherence were similar in both regimens. CONCLUSIONS: A longer time interval between dornase alfa and physiotherapy is more efficacious than short interval. Administration timing of dornase alfa based on patient choice to incorporate longer interval time is likely to be the best regimen for patients previously established on dornase alfa nebulization.
