Improving performance in the detection and management of cystic fibrosis-related diabetes in the Mountain West Cystic Fibrosis Consortium.

OBJECTIVE: Cystic fibrosis (CF)-related diabetes (CFRD) is associated with increased morbidity and mortality. Improved detection and management may improve outcomes; however, actual practice falls short of published guidelines. We studied efforts to improve CFRD screening and management in the Mountain West CF Consortium (MWCFC). RESEARCH DESIGN AND METHODS: This is a prospective observational cohort study evaluating quality improvement by accredited CF centers in Arizona, Colorado, New Mexico, and Utah performed between 2002 and 2008. After Institutional Review Board (IRB) approval, centers evaluated adherence with CF Foundation guidelines for CFRD. Each center developed and implemented quality improvement plans to improve both screening and management. Centers were reassessed 1 year later. RESULTS: Initially, each CF center had low adherence with screening recommendations (26.5% of eligible patients) that did not improve during the study. However, patients with confirmed CFRD markedly increased (141 (12% of MWCFC patients) to 224 (17%), p<0.001), and with improved adherence to management guidelines, patients with CFRD had increased weight (56.8-58.9 kg, p<0.001), body mass index (21.1-21.4, p=0.003), and weight-for-age z-score (-1.42 to -0.84, p<0.001). Quality improvement methods were specific to the practice settings of each center but shared the common goal of adhering to CFRD care guidelines. 1 year after implementation, no center significantly differed from any other in level of adherence to guidelines. CONCLUSIONS: Improving adherence with CFRD care guidelines requires substantial effort and may be incompletely successful, particularly for CFRD screening, but the effort may significantly improve patient monitoring and clinically relevant outcomes such as weight.

Trajectories of adherence to airway clearance therapy for patients with cystic fibrosis.

OBJECTIVE: Although cross-sectional studies have demonstrated poor adherence to airway clearance therapy (ACT) for patients with cystic fibrosis (CF), no studies have identified longitudinal patterns of adherence. The objective was to characterize and identify predictors of ACT adherence trajectories for individuals with CF. METHODS: Secondary data analyses were conducted for a randomized clinical trial examining differences in three ACTs. Participants (n = 153; M = 14.3 years, 55% male, 86% Caucasian, baseline FEV(1)% predicted: M = 86.7)/primary caregivers completed Daily Phone Diaries, an empirically supported adherence measure, every 4 months. RESULTS: Group-based trajectory modeling revealed the best-fitting solution was a three-group model: low-adherence (14%), medium-adherence (49%), and high-adherence (37%) groups. ACT type was the only significant predictor of adherence trajectories. DISCUSSION: Three trajectories of adherence to ACT for patients with CF were found. With the identification of trajectories, adherence interventions can be targeted for the subgroup at highest risk in order to prevent poor health outcomes.

Lessons learned from a randomized trial of airway secretion clearance techniques in cystic fibrosis.

RATIONALE: Airway secretion clearance therapies are a cornerstone of cystic fibrosis care, however longitudinal comparative studies are rare. Our objectives were to compare three therapies [postural drainage and percussion: (postural drainage), flutter device (FD), and high frequency chest wall oscillation: (vest)], by studying (1) change in pulmonary function; (2) time to need for intravenous (IV) antibiotics, (3) use of pulmonary therapies, (4) adherence to treatment, (5) treatment satisfaction, and (6) quality of life. METHODS: Participants were randomly assigned to one of three therapies twice daily. Clinical outcomes were assessed quarterly over 3 years. RESULTS: Enrollment goals were not met, and withdrawal rates were high, especially in postural drainage (51%) and FD (26%), compared to vest (9%), resulting in early termination. FEV(1) decline, time to need IV antibiotics, and other pulmonary therapies were not different. The annual FEF(25-75%) predicted rate of decline was greater in those using vest (P = 0.02). Adherence was not significantly different (P = 0.09). Overall treatment satisfaction was higher in vest and FD than in postural drainage (P < 0.05). Health-related quality of life was not different. The rate of FEV(1) decline was 1.23% predicted/year. CONCLUSIONS: The study was ended early due to dropout and smaller than expected decline in FEV(1). Patients were more satisfied with vest and FD. The longitudinal decline in FEF(25-75%) was faster in vest; we found no other difference in lung function decline, taken together this warrants further study. The slow decline in FEV(1) illustrates the difficulty with FEV(1) decline as a clinical trial outcome.