Predicting lung function decline in cystic fibrosis: the impact of initiating ivacaftor therapy.

BACKGROUND: Modulator therapies that seek to correct the underlying defect in cystic fibrosis (CF) have revolutionized the clinical landscape. Given the heterogeneous nature of lung disease progression in the post-modulator era, there is a need to develop prediction models that are robust to modulator uptake. METHODS: We conducted a retrospective longitudinal cohort study of the CF Foundation Patient Registry (N = 867 patients carrying the G551D mutation who were treated with ivacaftor from 2003 to 2018). The primary outcome was lung function (percent predicted forced expiratory volume in 1 s or FEV1pp). To characterize the association between ivacaftor initiation and lung function, we developed a dynamic prediction model through covariate selection of demographic and clinical characteristics. The ability of the selected model to predict a decline in lung function, clinically known as an FEV1-indicated exacerbation signal (FIES), was evaluated both at the population level and individual level. RESULTS: Based on the final model, the estimated improvement in FEV1pp after ivacaftor initiation was 4.89% predicted (95% confidence interval [CI]: 3.90 to 5.89). The rate of decline was reduced with ivacaftor initiation by 0.14% predicted/year (95% CI: 0.01 to 0.27). More frequent outpatient visits prior to study entry and being male corresponded to a higher overall FEV1pp. Pancreatic insufficiency, older age at study entry, a history of more frequent pulmonary exacerbations, lung infections, CF-related diabetes, and use of Medicaid insurance corresponded to lower FEV1pp. The model had excellent predictive accuracy for FIES events with an area under the receiver operating characteristic curve of 0.83 (95% CI: 0.83 to 0.84) for the independent testing cohort and 0.90 (95% CI: 0.89 to 0.90) for 6-month forecasting with the masked cohort. The root-mean-square errors of the FEV1pp predictions for these cohorts were 7.31% and 6.78% predicted, respectively, with standard deviations of 0.29 and 0.20. The predictive accuracy was robust across different covariate specifications. CONCLUSIONS: The methods and applications of dynamic prediction models developed using data prior to modulator uptake have the potential to inform post-modulator projections of lung function and enhance clinical surveillance in the new era of CF care.

Technology-Enabled Health Care Collaboration in Pediatric Chronic Illness: Pre-Post Interventional Study for Feasibility, Acceptability, and Clinical Impact of an Electronic Health Record-Linked Platform for Patient-Clinician Partnership.

BACKGROUND: Mobile health (mHealth) technology has the potential to support the Chronic Care Model's vision of closed feedback loops and patient-clinician partnerships. OBJECTIVE: This study aims to evaluate the feasibility, acceptability, and short-term impact of an electronic health record-linked mHealth platform (Orchestra) supporting patient and clinician collaboration through real-time, bidirectional data sharing. METHODS: We conducted a 6-month prospective, pre-post, proof-of-concept study of Orchestra among patients and parents in the Cincinnati Children's Hospital inflammatory bowel disease (IBD) and cystic fibrosis (CF) clinics. Participants and clinicians used Orchestra during and between visits to complete and view patient-reported outcome (PRO) measures and previsit plans. Surveys completed at baseline and at 3- and 6-month follow-up visits plus data from the platform were used to assess outcomes including PRO completion rates, weekly platform use, disease self-efficacy, and impact on care. Analyses included descriptive statistics; pre-post comparisons; Pearson correlations; and, if applicable, effect sizes. RESULTS: We enrolled 92 participants (CF: n=52 and IBD: n=40), and 73% (67/92) completed the study. Average PRO completion was 61%, and average weekly platform use was 80%. Participants reported improvement in self-efficacy from baseline to 6 months (7.90 to 8.44; P=.006). At 6 months, most participants reported that the platform was useful (36/40, 90%) and had a positive impact on their care, including improved visit quality (33/40, 83%), visit collaboration (35/40, 88%), and visit preparation (31/40, 78%). PRO completion was positively associated with multiple indicators of care impact at 3 and 6 months. CONCLUSIONS: Use of an mHealth tool to support closed feedback loops through real-time data sharing and patient-clinician collaboration is feasible and shows indications of acceptability and promise as a strategy for improving pediatric chronic illness management.

Improving Detection of Rapid Cystic Fibrosis Disease Progression-Early Translation of a Predictive Algorithm Into a Point-of-Care Tool.

The clinical course of cystic fibrosis (CF) lung disease is marked by acute drops of lung function, defined clinically as rapid decline. As such, lung function is monitored routinely through pulmonary function testing, producing hundreds of measurements over the lifespan of an individual patient. Point-of-care technologies aimed at improving detection of rapid decline have been limited. Our aim in this early translational study is to develop and translate a predictive algorithm into a prototype prognostic tool for improved detection of rapid decline. The predictive algorithm was developed, validated and checked for 6-month, 1-year, and 2-year forecast accuracies using data on demographic and clinical characteristics from 30 879 patients aged 6 years and older who were followed in the U.S. Cystic Fibrosis Foundation Patient Registry from 2003 to 2015. Predictions of rapid decline based on the algorithm were compared to a detection algorithm currently being used at a CF center with 212 patients who received care between 2012-2017. The algorithm was translated into a prototype web application using RShiny, which resulted from an iterative development and refinement based on clinician feedback. The study showed that the algorithm had excellent predictive accuracy and earlier detection of rapid decline, compared to the current approach, and yielded a prototype platform with the potential to serve as a viable point-of-care tool. Future work includes implementation of this clinical prototype, which will be evaluated prospectively under real-world settings, with the aim of improving the pre-visit planning process for CF point of care. Likely extensions to other point-of-care settings are discussed.

Use and Incidence of Adverse Effects of Proton Pump Inhibitors in Patients with Cystic Fibrosis.

PURPOSE: To evaluate the incidence of adverse effects associated with chronic proton pump inhibitor (PPI) use as well as the dosing, indication, and duration of use of PPIs in the cystic fibrosis (CF) population at a pediatric academic medical center. METHODS: Study design was a retrospective chart review evaluating patients with CF who were prescribed a PPI for at least 6 months (PPI group) or patients with CF who had never been prescribed a PPI (control group) from June 1, 2014, to May 31, 2015. RESULTS: The study enrolled 126 patients in the PPI group and 49 patients in the control group. Forty-four patients (34.9%) had an indication for both gastroesophageal reflux and enzyme enhancement, with an average PPI daily dose of 1 mg/kg/day. Twenty-one patients (16.7%) in the PPI group had an incidence of hypomagnesemia compared with one patient (2%) in the control group (p=0.097). Overall, 75 patients (59.6%) receiving chronic PPI therapy had at least one pulmonary exacerbation compared with 12 patients (24.5%) in the control group (p<0.001). No significant difference was noted in the incidence of hypocalcemia, low bone mineral density, or positive Clostridium difficile toxin between the two groups. CONCLUSION: The PPI group had a higher risk of pulmonary exacerbation compared with the control group. Further studies are needed to assess adverse effects associated with chronic PPI use in patients with CF.

Protocol of a Pilot Study of Technology-Enabled Coproduction in Pediatric Chronic Illness Care.

BACKGROUND: Pediatric chronic illness care models are traditionally organized around acute episodes of care and may not meet the needs of patients and their families. Interventions that extend the patient-clinician interaction beyond the health care visit, allow for asynchronous and bidirectional feedback loops that span visits and daily life, and facilitate seamless sharing of information are needed to support a care delivery system that is more collaborative, continuous, and data-driven. Orchestra is a mobile health technology platform and intervention designed to transform the management of chronic diseases by optimizing patient-clinician coproduction of care. OBJECTIVE: The aim of this study is to assess the feasibility, acceptability, and preliminary impact of the Orchestra technology and intervention in the context of pediatric chronic illness care. METHODS: This study will be conducted in the cystic fibrosis and inflammatory bowel disease clinics at Cincinnati Children's Hospital Medical Center. We will enroll interested patients and their caregivers to work with clinicians to use the Orchestra technology platform and care model over a 6-month period. In parallel, we will use quality improvement methods to improve processes for integrating Orchestra into clinic workflows and patient/family lifestyles. We will use surveys, interviews, technology use data, and measures of clinical outcomes to assess the feasibility, acceptability, and preliminary impact of Orchestra. Outcome measures will include assessments of: (1) enrollment and dropout rates; (2) duration of engagement/sustained use; (3) symptom and patient-reported outcome tracker completion rates; (4) perceived impact on treatment plan, communication with the clinical team, visit preparation, and overall care; (5) changes in disease self-efficacy and engagement in care; and (6) clinical outcomes and health care utilization. RESULTS: Participant recruitment began in mid-2015, with results expected in 2017. CONCLUSIONS: Chronic disease management needs a dramatic transformation to support more collaborative, effective, and patient-centered care. This study is unique in that it is testing not only the impact of technology, but also the necessary processes that facilitate patient and clinician collaboration. This pilot study is designed to examine how technology-enabled coproduction can be implemented in real-life clinical contexts. Once the Orchestra technology and intervention are optimized to ensure feasibility and acceptability, future studies can test the effectiveness of this approach to improve patient outcomes and health care value.

Early Detection of Rapid Cystic Fibrosis Disease Progression Tailored to Point of Care: A Proof-of-Principle Study.

Slowing cystic fibrosis (CF) lung disease progression is crucial to survival, but point-of-care technologies aimed at early detection-and possibly prevention-of rapid lung function decline are limited. This proof-of-principle study leverages a rich national patient registry and follow-up data on a local CF cohort to build an algorithm and prototype prognostic tool aimed at early detection of rapid lung function decline. The algorithm was developed using a novel longitudinal analysis of lung function (measured as forced expiratory volume in 1 s of % predicted, FEV1). Covariates included clinical and demographic characteristics selected from the registry based on information criterion. Preliminary assessment of algorithm performance suggested excellent predictive accuracy and earlier detection of rapid decline than standard of care being applied at a local center. Graphical displays were presented and evaluated for clinical utility. Predictions from the algorithms and chosen graphical displays were translated into a prototype web application using RShiny and underwent iterative development based on clinician feedback. This paper suggests that the algorithm and its translation could offer a means for earlier detection and treatment of rapid decline, providing clinicians with a viable point-of-care technology to intervene prior to irreversible lung damage.

Constrictive Bronchiolitis in Cystic Fibrosis Adolescents with Refractory Pulmonary Decline.

RATIONALE: Refractory lung function decline in association with recurrent pulmonary exacerbations is a common, yet poorly explained finding in cystic fibrosis (CF). To investigate the histopathologic mechanisms of pulmonary deterioration during adolescence and early adulthood, we reviewed clinically-indicated lung biopsy specimens obtained during a period of persistent decline. OBJECTIVES: To determine if peribronchiolar remodeling is prominent in lung biopsy specimens obtained in adolescents with CF refractory to conventional therapy. METHODS: Six adolescents with CF (mean age, 16.2 y; mean FEV1, 52% predicted at biopsy) with significant pulmonary deterioration over 12-24 months (mean FEV1 decline of 14% predicted/year) despite aggressive intervention underwent computed tomography imaging and ultimately lung biopsy to aid clinical management. In addition to routine clinical evaluation, histopathologic investigation included staining for transforming growth factor-ß (TGF-ß, a genetic modifier of CF lung disease), collagen deposition (a marker of fibrosis), elastin (to evaluate for bronchiectasis), and α-smooth muscle actin (to identify myofibroblasts). MEASUREMENTS AND MAIN RESULTS: All computed tomography scans demonstrated a mix of bronchiectasis and hyperinflation that was variable across lung regions and within patients. Lung biopsy revealed significant peribronchiolar remodeling, particularly in patients with more advanced disease, with near complete obliteration of the peribronchiolar lumen (constrictive bronchiolitis). Myofibroblast differentiation (a TGF-ß-dependent process) was prominent in specimens with significant airway remodeling. CONCLUSIONS: Constrictive bronchiolitis is widely present in the lung tissue of adolescents with CF with advanced disease and may contribute to impaired lung function that is refractory to conventional therapy (antibiotics, antiinflammatories, and mucolytics). TGF-ß-dependent myofibroblast differentiation is prominent in areas of active fibrogenesis and may foster small airway remodeling in CF lung disease.

Adolescents' Spirituality and Cystic Fibrosis Airway Clearance Treatment Adherence: Examining Mediators.

OBJECTIVE: Adolescent cystic fibrosis (CF) treatment adherence is a significant multidimensional issue. Using the Theory of Reasoned Action (TRA), this study examined the role of spiritual factors in adherence. METHODS: Forty-five 11-19-year-olds diagnosed with CF completed questionnaires concerning psychosocial, spiritual, and adherence-related constructs and Daily Phone Diaries to calculate treatment adherence. Exploratory Factor Analysis identified two spiritual factors used in subsequent analyses. The mediating roles of attitude toward the treatment's value (utility), subjective behavioral norms (the product of perceived behavioral norms and one's motivation to comply with them), self-efficacy for completing the treatments and treatment intentions in the relationship between spiritual factors and treatment adherence were tested with path analysis. RESULTS: Lower 'spiritual struggle' and greater 'engaged spirituality' predicted treatment attitude (utility) and subjective behavioral norms, which, together with self-efficacy, predicted treatment intentions. Finally, treatment intentions predicted airway clearance adherence. CONCLUSIONS: Findings were consistent with the TRA. Engaged spirituality supports pro-adherence determinants and behavior. Spiritual struggle's negative associations with outcomes warrant screening and intervention.

Lumacaftor alone and combined with ivacaftor: preclinical and clinical trial experience of F508del CFTR correction.

Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator protein (CFTR), leading to significant morbidity and mortality. CFTR is a chloride and bicarbonate channel at the epithelial cell membrane. The most common CFTR mutation is F508del, resulting in minimal CFTR at the plasma membrane. Current disease management is supportive, whereas an ultimate goal is to develop therapies to restore CFTR activity. We summarize experience with lumacaftor, a small molecule that increases F508del-CFTR levels at the plasma membrane. Lumacaftor in combination with ivacaftor, a modulator of CFTR gating defects, improves clinical outcome measures in patients homozygous for the F508del mutation. Lumacaftor represents a significant advancement in the treatment of biochemical abnormalities in CF. Further development of CFTR modulators will improve upon current therapies, although it remains unclear whether this approach will provide therapies for all CFTR mutations.

Decision Making for Children with Obstructive Sleep Apnea without Tonsillar Hypertrophy.

OBJECTIVE: Evidence-based medicine is the gold standard practice model for patient management. Our aim was to determine whether decisions made by pediatric subspecialists regarding management of obstructive sleep apnea in children without tonsillar hypertrophy adhered to this model or were based on clinical experiences. STUDY DESIGN: Single-institution prospective study. SETTING: Multidisciplinary upper airway center in an academic pediatric hospital. SUBJECTS AND METHODS: Twelve pediatric subspecialists representing 8 specialties participating in upper airway clinics and management conferences. Real-time decisions made in treatment conferences and upper airway clinics were collected. Physicians were queried regarding the basis of their decisions, and these decisions were then classified into 10 categories. RESULTS: Over 13 days (10 case conferences, 3 half-day clinics), 324 decisions were made for 58 patients (mean age = 8.9 ± 7.4 years, mean body mass index percentile = 75 ± 29); 34% (n = 108) of decisions were evidence based; 59% (n = 193) were nonevidence based; and 7% (n = 23) were based on parental preference. Providers were able to cite specific studies for <20% of these decisions. There was no significant increase in the proportion of evidence-based decisions made over time. CONCLUSIONS: We deemed 34% of decisions regarding the management of obstructive sleep apnea in children without tonsillar hypertrophy to be evidence based and found that sharing the basis for decisions did not improve the percentage of evidence-based decisions over time. These findings reflect significant evidence gaps and highlight the need for a systematic literature evaluation to identify best practice in managing this population. We recommend that these evidence gaps be further characterized and incorporated into an agenda for future research.

Concentration of fractional excretion of nitric oxide (FENO): A potential airway biomarker of restored CFTR function.

BACKGROUND: Lower airway biomarkers of restored cystic fibrosis transmembrane conductance regulator (CFTR) function are limited. We hypothesized that fractional excretion of nitric oxide (FENO), typically low in CF patients, would demonstrate reproducibility during CFTR-independent therapies, and increase during CFTR-specific intervention (ivacaftor) in patients with CFTR gating mutations. METHODS: Repeated FENO and spirometry measurements in children with CF (Cohort 1; n=29) were performed during hospital admission for acute pulmonary exacerbations and routine outpatient care. FENO measurements before and after one month of ivacaftor treatment (150 mg every 12h) were completed in CF patients with CFTR gating mutations (Cohort 2; n=5). RESULTS: Cohort 1: Mean forced expiratory volume in 1s (FEV1 % predicted) at enrollment was 72.3% (range 25%-102%). Mean FENO measurements varied minimally over the two inpatient and two outpatient measurements (9.8-10.9 ppb). There were no clear changes related to treatment of pulmonary exacerbations, gender, genotype or microbiology, and weak correlation with inhaled corticosteroid use (P<0.05). Between the two inpatient measurements, FEV1 % predicted increased by 7.3% (P<0.03) and FENO did not change. In Cohort 2, mean FENO increased from 6.6 ppb (SD=2.19) to 11.8 ppb (SD=4.97) during ivacaftor treatment. Mean sweat chloride dropped by 58 mM and mean FEV1 % predicted increased by 10.2%. CONCLUSIONS: Repeated FENO measurements were stable in CF patients, whereas FENO increased in all patients with CFTR gating mutations treated with ivacaftor. Acute changes in FENO may serve as a biomarker of restored CFTR function in the CF lower airway during CFTR modulator treatment.

Obstructive lung disease in children with idiopathic scoliosis.

OBJECTIVE: To measure the prevalence of obstructive lung disease (OLD) among patients undergoing preoperative pulmonary assessment for idiopathic scoliosis. STUDY DESIGN: This was a retrospective, descriptive review from clinical data in a tertiary care pediatric hospital in the US. Patients (n = 176) with idiopathic scoliosis with Cobb angles of ≥ 40 degrees who performed acceptable and repeatable preoperative pulmonary function testing were included. The primary outcome measure was the forced expiratory volume in the first second (FEV1)/forced vital capacity (FVC) ratio. RESULTS: The prevalence of OLD (low FEV1/FVC ratio) was 39% (68/176 patients). In multivariate modeling, radiographic measures were poor predictors of pulmonary function outcomes of FVC (r(2) 0.06), FEV1 (r(2) 0.05), FEV1/FVC ratio (r(2) 0.08), and total lung capacity (r(2) 0.06). CONCLUSIONS: OLD is common in patients with idiopathic scoliosis. We recommend preoperative pulmonary function testing for patients with idiopathic scoliosis under consideration for spinal fusion surgery.

The impact of transforming healthcare delivery on cystic fibrosis outcomes: a decade of quality improvement at Cincinnati Children's Hospital.

BACKGROUND: In 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. OBJECTIVE: To improve clinical outcomes for patients with CF through a comprehensive quality-improvement approach directed at increasing patient centredness and improving healthcare delivery. INTERVENTIONS: In 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. MEASUREMENTS: Primary 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). RESULTS: From 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. DISCUSSION: By 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.

Obstructive lung disease is common in children with syndromic and congenital scoliosis: a preliminary study.

BACKGROUND: It is well known that restrictive lung disease (RLD) is associated with scoliosis. This study identifies that obstructive lung disease (OLD) is associated with syndromic scoliosis and congenital scoliosis. METHODS: We searched a local database for patients with scoliosis who underwent a pulmonary function testing (PFT) from 2004 to 2009. All patients with congenital scoliosis or syndromic thoracolumbar scoliosis with a Cobb angle of ≥40 degrees and acceptable and repeatable PFT testing were included in the study. OLD was defined as an forced expiratory volume in the first second/forced vital capacity ratio below 95% confidence interval. Bronchoscopy videos and computed tomography scans or magnetic resonance images were reviewed to identify anatomic causes of lower airway disease. RESULTS: A total of 18 patients met the criteria for inclusion. The median age at diagnosis was 11.3 years. The median primary Cobb angle was 60 degrees. The prevalence of OLD was 33% and RLD was 57%. The 6 children with OLD underwent preoperative bronchoscopy and chest computed tomography or magnetic resonance imaging to identify anatomic causes of lower airway obstruction. The 4 children with OLD and right-sided major thoracic curves had compression of the right mainstem bronchus between the spine (posterior) and the right pulmonary artery (anterior). The 2 children with OLD and left-sided major thoracic curves had compression of the left mainstem bronchus between the spine (posterior) and the descending aorta (anterior) or the left atrium (anterior). CONCLUSIONS: In our study, the prevalence of OLD in children with congenital scoliosis or syndromic scoliosis was 33%, which was elevated when compared with the population prevalence of 2% to 5%. Mainstem airway compression from spine rotation was discovered to be the potential mechanism of disease. LEVEL OF EVIDENCE: Level IV, prognostic study investigating the effect of a patient characteristic on the outcome of disease.

A semiparametric approach to estimate rapid lung function decline in cystic fibrosis.

PURPOSE: Detecting the onset of rapid lung function decline is important to reduce mortality rates in cystic fibrosis (CF) and other lung diseases. The most common approach is conventional linear mixed modeling-estimating a population-level slope of lung function decline and using random effects to address serial correlation-but this ignores nonlinear features of disease progression and distinct sources of variability. The purpose of this article was to estimate patient-specific timing and degree of rapid decline while appropriately characterizing natural progression and variation in CF. METHODS: We propose longitudinal semiparametric mixed modeling and contrast it with the conventional approach, which restricts lung function (measured as forced expiratory volume in 1 second as a percentage of predicted, FEV1%) to linear decline. Each approach is applied to clinical encounter data from the United States CF Foundation Patient Registry. RESULTS: Timing and degree of rapid FEV1% decline vary across patients and as a function of key covariates. Patients experience maximal FEV1% loss by early adulthood more severe than indicated by conventional slope analysis. CONCLUSIONS: Semiparametric mixed modeling provides a means to estimate patient-specific changes in CF disease progression and may be used to inform prognostic decisions in chronic care settings and clinical studies.

Inhaled tobramycin effectively reduces FEV1 decline in cystic fibrosis. An instrumental variables analysis.

RATIONALE: The efficacy of inhaled tobramycin on chronic Pseudomonas aeruginosa infections in patients with cystic fibrosis (CF) has been established in clinical trials. However, little is known about its clinical effectiveness on lung function outside randomized controlled trial settings; conventional analysis of existing registry data has heretofore been confounded by treatment selection bias. OBJECTIVE: To determine effectiveness of inhaled tobramycin on FEV1 decline in patients with chronic P. aeruginosa infections using observational data from the Cystic Fibrosis Foundation Patient Registry. METHODS: Patient-level tobramycin use was measured at first chronic P. aeruginosa infection (n = 13,686 patients; age, 6-21 yr). Decline in FEV1 2 years after infection was estimated for patients treated with tobramycin and compared with untreated patients. Multiple linear regressions with confounder adjustment and propensity scores were used to estimate mean FEV1 decline for each group. Because care is organized by centers, we used center-specific prescription rates as an instrument to reduce treatment-by-condition bias. MEASUREMENTS AND MAIN RESULTS: Using center-level prescribing rates, instrumental variables analysis showed less FEV1 decline for patients who received tobramycin when first eligible compared with those who did not receive tobramycin (difference, 2.55% predicted; 95% confidence interval, 0.16-4.94; P = 0.0366). CONCLUSIONS: Inhaled tobramycin is effective in reducing lung function decline among patients 6 to 21 years of age with CF. Because CF care is organized by center, using center-specific prescription rates as an instrumental variable is a feasible approach to using the Cystic Fibrosis Foundation Patient Registry to determine treatment effectiveness. More generally, this approach can correct for treatment-by-condition bias arising from observational studies.

Is adolescents' religious coping with cystic fibrosis associated with the rate of decline in pulmonary function?-A preliminary study.

Religious coping is associated with health outcomes in adolescents with chronic disease. Identifying potentially modifiable spiritual factors is important for improving health outcomes. The purpose of this study was to determine if associations exist between rate of change in pulmonary function and subsequent religious coping by adolescents with cystic fibrosis (CF). Retrospective cohort design employing the Brief R-COPE and calculated decline in lung function over a three-year period were utilized. Data were obtained for 28 adolescents; median age 13.5 years. Use of pleading or negative religious coping was associated with a worse clinical trajectory. Pleading may be ineffective as disease progression is modifiable through adherence to evidence-based treatments. Given established relationships of religious coping with general coping, the effects of declining pulmonary function may be broader. Changes in pulmonary function suggest opportunities for chaplains to explore options to cognitively reframe negative religious coping.

Prevention of tracheostomy-related pressure ulcers in children.

BACKGROUND AND OBJECTIVES: Pressure ulcers are commonly acquired in pediatric institutions, and they are a key indicator of the standard and effectiveness of care. We recognized a high rate of tracheostomy-related pressure ulcers (TRPUs) in our ventilator unit and instituted a quality improvement program to develop and test potential interventions for TRPU prevention, condensed them into a clinical bundle, and then implemented the bundle into our standard practice. METHODS: The intervention model used a rapid-cycle, Plan-Do-Study-Act (PDSA), framework for improvement research. All tracheostomy-dependent patients admitted to our 18-bed ventilator unit from July 2008 through December 2010 were included. TRPU stage and description, number of days each TRPU persisted, and bundle compliance were recorded in real time. All TRPUs were staged by a wound-care expert within 24 hours. The interventions incorporated into the TRPU-prevention bundle included frequent skin and device assessments, moisture-reducing device interface, and pressure-free device interface. RESULTS: There was a significant decrease in the rate of patients who developed a TRPU from 8.1% during the preintervention period, to 2.6% during bundle development, to 0.3% after bundle implementation. There was a marked difference between standard and extended tracheostomy tubes in TRPU occurrence (3.4% vs 0%, P = .007) and days affected by a TRPU (5.2% vs 0.1%, P < .0001). CONCLUSIONS: Education and ongoing assessment of skin integrity and the use of devices that minimize pressure at the tracheostomy-skin interface effectively reduce TRPU even among a population of children at high risk. These interventions can be integrated into daily workflow and result in sustained effect.