CYP3A4-mediated drug interactions represent a significant risk in patients treated with long-term inhaled corticosteroids. The presence of clinically significant CF-related liver disease may enhance this risk.

Background Accelerated lung function decline in cystic fibrosis (CF) is associated with mucoid Pseudomonas aeruginosa infection. Recent data suggest that mucoid P. aeruginosa may amenable to elimination from the airway. We aim to determine whether the initiation of an aggressive antibiotic eradication regimen upon initial discovery of mucoid P. aeruginosa in the CF airway could be successful in clearing the organism from the CF lung. Methods We performed a retrospective analysis of patients with CF who demonstrated new growth of mucoid P. aeruginosa in an airway culture between January 2003 and December 2008. The primary endpoint was clearance of mucoid P. aeruginosa, based upon the Leeds criteria, with no further growth of mucoid P. aeruginosa cultures within 12 months of the initial discovery and treatment. Factors associated with successful clearance were also evaluated. Results Forty‐eight of 355 patients with CF had a new diagnosis of mucoid P. aeruginosa during the study period; 15 patients underwent an eradication attempt, while 33 patients received no increase in therapy. We observed clearance of mucoid P. aeruginosa in 73.3% of patients undergoing an eradication attempt, whereas 36.6% of those that did not undergo attempted eradication cleared the organism at 1 year (P < 0.05). Prolonged mucoid P. aeruginosa airway clearance (>24 months) for mucoid P. aeruginosa was seen in 60.0% in subjects undergoing eradication compared to 21.2% (P = 0.02) in control patients. At the study conclusion, lung function was greater in subjects who underwent an eradication attempt than in patients who did not undergo an eradication attempt (FEV1%: 91.7% vs. 75.0%, P = 0.04). Conclusions Clearance of initial mucoid P. aeruginosa from the airways of select patients with CF is possible with current antibiotic regimens, and the attempt may be associated with improved lung function. Pediatr Pulmonol. 2012. 47:1113–1122. © 2012 Wiley Periodicals, Inc.

ObjectiveTo describe the characteristics of sustained improved nutritional outcomes through the use of quality improvement (QI) methodology.DesignRetrospective analysis of a QI intervention in two institutions, implemented as part of larger national collaboratives.SettingPaediatric cystic fibrosis (CF) programmes in academic centres in Alabama and Illinois.ParticipantsAll paediatric patients enrolled in the CF Foundation (CFF) Patient Data Registry were included.InterventionsImproved and sustained nutrition outcomes occurred through implementation of the CFF practice guidelines for CF nutrition management via care delivery processes, nutritional interventions, team engagement and data display.MeasurementMean body mass index (BMI) percentile, percentage of patients less than 50th percentile and percentage less than 10th percentile for all patients aged 2–20 years were tracked through run charts and statistical process control charts. Mann–Whitney U and χ2tests were used to determine significance between each centre and national outcomes.ResultsEach centre achieved rapid improvement in mean BMI percentile in patients, one centre rising from the 40th percentile in 2001 to the 49th percentile in 2003, the other rising from the 37th percentile in 2003 to the 45th percentile in 2004. These centres have also maintained improved nutritional outcomes, so that they were at the 60th and 55th percentiles, respectively, in 2011. Sustained improvement was accomplished through QI methodology, use of data as a driver for improvement and a change in culture.ConclusionsParticipation in collaboratives led to improved nutrition outcomes while a strong culture of QI facilitated sustained improvement.

Summary Objective The clinical benefit of newborn screening (NBS) for cystic fibrosis (CF) has been primarily nutritional, with less overt respiratory impact. Identification of risk factors for infant CF lung disease could facilitate targeted interventions to improve pulmonary outcomes. Methods This retrospective study evaluated socioeconomic information, clinical data, and results from routine infant pulmonary function testing (iPFT) of infants diagnosed with CF through NBS (N = 43) at a single CF center over a 4-year period (2008–2012). A five-item composite clinical score was developed and combined with socioeconomic indicators to facilitate identification of CF infants at increased risk of early-onset respiratory impairment. Results Paternal education was positively associated with lung function (P = 0.02). Clinical score <7 (on a scale of 0–10) predicted diminished pulmonary measure (P < 0.005). Retrospective risk stratification by clinical score and paternal education identified CF infants at low, intermediate, or high risk of pulmonary disease. Forced expiratory volume (FEV0.5%, mean ± SD) averaged 115 ± 19% in the low-risk group, 97 ± 17% in the intermediate-risk group, and 90 ± 8% in the high-risk group (P < 0.005). Results were similar for mid-expiratory flows (FEF25–75%). Multiple regression analysis confirmed the predictive value of this risk stratification model of CF infant pulmonary health. Conclusion We combined socioeconomic and clinical data to risk-stratify CF infants for early-onset lung disease as quantified by iPFT. Our model showed significant differences in infant pulmonary function across risk groups. The developed tool offers an easily available, inexpensive, and non-invasive way to assess risk of respiratory decline in CF infants and identify those meriting targeted therapeutic attention.

Quality improvement (QI) efforts at the University of Alabama at Birmingham/Children's Hospital Cystic Fibrosis Center began in the spring of 2004, with a collaborative sponsored by the Cystic Fibrosis Foundation. As the authors gained experience with QI processes, significant system changes ensued. In this article, we describe how the center created a culture of improvement that has resulted in significant improvements in clinical outcomes in our patient population.