BackgroundIn 2001, Cincinnati Children's Hospital embarked on a journey to improve healthcare delivery to patients with cystic fibrosis (CF). Data from the Cystic Fibrosis Foundation National Patient Registry revealed our below-average clinical outcomes, prompting us to initiate improvement interventions.ObjectiveTo improve clinical outcomes for patients with CF through a comprehensive quality-improvement approach directed at increasing patient centredness and improving healthcare delivery.InterventionsIn 2001, we shared our below-average outcomes with patients, families and care providers. We instituted a quality-improvement steering committee with parental and hospital leadership, and our data-management support was restructured to provide real-time clinical data to monitor our progress. In 2002, our weekly chart conference changed to a prospective planning session and individualised daily schedules were created for inpatients. In 2003, an influenza vaccination campaign was initiated and our infection-control practices were redesigned. In 2005, best-practice guidelines were developed for airway-clearance therapy. In 2007, evidence-based clinical algorithms were designed and implemented and key care-team members were added.MeasurementsPrimary outcome measures were median forced expiratory volume in 1 s per cent predicted (age range 6–17 years) and median body mass index percentile (age range 2–20 years).ResultsFrom 2000 to 2010, median forced expiratory volume in 1 s increased from 81.7% to 100.1% predicted and median body mass index increased from the 35th to the 55th centile.DiscussionBy focusing on specific outcomes, empowering families and patients, effectively using data, and standardising care processes, we transformed the culture and delivery of care for our patients with CF and learned valuable lessons potentially translatable to other chronic-care providers.

LAI is a unique formulation of amikacin that enhances drug delivery and retention in CF airways via incorporation into neutral liposomes. Results of a recent Phase II trial suggest that LAI, with the capacity for once-daily dosing and prolonged off-drug periods, may be an attractive choice of inhaled antibiotic to manage PsA lung infections in CF patients. Further data from Phase III studies assessing the efficacy and safety of LAI should better elucidate its potential.

Positive health outcomes are related to adults’ religious congregational participation. For parents of children with chronic disease, structured daily care routines and or strict infection-control precautions may limit participation. For this exploratory study, we examined the relationship between congregational support and religious coping by parents of children with cystic fibrosis (CF) compared to parents for whom child health issues were not significant stressors. CF parents reported higher levels of emotional support from congregation members and use of religious coping. Within-group differences were found for CF parents by denominational affiliation. Congregational support for parents dealing with child chronic disease is important.