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Background: Detecting airway inflammation non-invasively in infants with cystic fibrosis (CF) is difficult. We hypothesized that markers of inflammation in CF [IL-1β, IL-6, IL-8, IL-10, IL-17A, neutrophil elastase (NE) and tumor necrosis factor (TNF-α)] could be measured in infants with CF from nasal fluid and would be elevated during viral infections or clinician-defined pulmonary exacerbations (PEx)., Methods: We collected nasal fluid, nasal swabs, and hair samples from 34 infants with CF during monthly clinic visits, sick visits, and hospitalizations. Nasal fluid was isolated and analyzed for cytokines. Respiratory viral detection on nasal swabs was performed using the Luminex NxTAG® Respiratory Pathogen Panel. Hair samples were analyzed for nicotine concentration by reverse-phase high-performance liquid chromatography. We compared nasal cytokine concentrations between the presence and absence of detected respiratory viruses, PEx, and smoke exposure., Results: A total of 246 samples were analyzed. Compared to measurements in the absence of respiratory viruses, mean concentrations of IL-6, IL-8, TNF-α, and NE were significantly increased while IL-17A was significantly decreased in infants positive for respiratory viruses. IL-17A was significantly decreased and NE increased in those with a PEx. IL-8 and NE were significantly increased in infants with enteric pathogen positivity on airway cultures, but not P. aeruginosa or S. aureus. Compared to those with no smoke exposure, there were significantly higher levels of IL-6, IL-10, and NE in infants with detectable levels of nicotine., Conclusions: Noninvasive collection of nasal fluid may identify inflammation in infants with CF during changing clinical or environmental exposures., Competing Interests: Declaration of Competing Interest The authors have no declarations of interest., (Copyright © 2023 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)

Background: Mental health screening in accordance with consensus guidelines became routine clinical practice in our cystic fibrosis (CF) Center in 2015. We hypothesized improvement in anxiety and depression symptoms over time and associations between elevated screening scores and disease severity. We aimed to observe the impact of the COVID-19 pandemic and modulator use on mental health symptoms., Methods: Retrospective chart reviews were conducted for people 12 years and older with at least one Generalized Anxiety Disorder-7 (GAD-7) or Patient Health Questionnaire-9 (PHQ-9) screening for six years. Descriptive statistics were used to summarize demographic variables and logistic regression and linear mixed models were used to evaluate the relationship between screening scores and clinical variables., Results: Analyses included 150 participants (ages 12-22 years). The percentage of minimal to no symptom scores increased over time for anxiety and depression. Increased mental health visits and CFRD were associated with higher PHQ-9 and GAD-7 scores. Higher FEV1pp was associated with lower GAD-7 and PHQ-9 scores. More effective modulator use was associated with lower PHQ-9 scores. Mean PHQ-9 and GAD-7 scores were not significantly different when comparing pre-pandemic and pandemic scores., Conclusion: Disruptions in screening during the pandemic were minimal and symptom scores remained stable. Individuals with higher mental health screening scores were more likely to have CFRD and utilization of mental health services. Consistent mental health monitoring and support is needed so individuals with CF can endure anticipated and unanticipated stressors including changes in physical health, healthcare, and societal stressors such as COVID-19 pandemic., Competing Interests: Declaration of Competing Interest The authors declare no conflict of interest., (Copyright © 2023. Published by Elsevier B.V.)

Background: The extent to which environmental exposures and community characteristics of the built environment collectively predict rapid lung function decline, during adolescence and early adulthood in cystic fibrosis (CF), has not been examined., Objective: To identify built environment characteristics predictive of rapid CF lung function decline., Methods: We performed a retrospective, single-center, longitudinal cohort study (n = 173 individuals with CF aged 6-20 years, 2012-2017). We used a stochastic model to predict lung function, measured as forced expiratory volume in 1 s (FEV 1 ) of % predicted. Traditional demographic/clinical characteristics were evaluated as predictors. Built environmental predictors included exposure to elemental carbon attributable to traffic sources (ECAT), neighborhood material deprivation (poverty, education, housing, and healthcare access), greenspace near the home, and residential drivetime to the CF center., Measurements and Main Results: The final model, which included ECAT, material deprivation index, and greenspace, alongside traditional demographic/clinical predictors, significantly improved fit and prediction, compared with only demographic/clinical predictors (Likelihood Ratio Test statistic: 26.78, p < 0.0001; the difference in Akaike Information Criterion: 15). An increase of 0.1 μg/m 3 of ECAT was associated with 0.104% predicted/yr (95% confidence interval: 0.024, 0.183) more rapid decline. Although not statistically significant, material deprivation was similarly associated (0.1-unit increase corresponded to additional decline of 0.103% predicted/year [-0.113, 0.319]). High-risk regional areas of rapid decline and age-related heterogeneity were identified from prediction mapping., Conclusion: Traffic-related air pollution exposure is an important predictor of rapid pulmonary decline that, coupled with community-level material deprivation and routinely collected demographic/clinical characteristics, enhance CF prognostication and enable personalized environmental health interventions., (© 2023 The Authors. Pediatric Pulmonology published by Wiley Periodicals LLC.)

Background: Treatment regimens for cystic fibrosis (CF) continue to evolve and grow in complexity. Treatment regimen burden, and associated sequelae, are incompletely understood., Objective: Quality improvement (QI) methods were used to investigate treatment burden of CF care, family and care team partnerships, and potential interventions to reduce burden., Methods: Patients 6-24 years with CF and caregivers of patients 6-13 years were surveyed. Portions of validated tools and existing surveys measured burden and family-care team partnership. An automated report calculated treatment complexity. Plan-do-study-act cycles tested survey administration during CF visits and run charts tracked progress. Interventions to reduce burden were tracked, and bidirectional assessments explored partnerships among patients, families and clinicians., Results: Over 6 months, 110 patients and 62 caregivers completed assessments. Caregivers reported lower burden/higher quality of life (74.0, range 22.2-100) than patients (66.5, range 16.7-100). The mean treatment complexity score was 17.2 (range 6-34). Treatment complexity and burden increased with patient age (p < .05 and p < .01 respectively). Lower lung function correlated with higher patient-reported burden (p < .01) and higher treatment complexity (p < .0001). As burden increased, providers more often performed select interventions (discussed combining treatments, simplified regimens, or involved other team members (p < .05 for each)). Families reported high partnership (mean scores 4.7-4.8, 5 = high), and providers reported high utilization of partnership tools (tool used in 77% of encounters)., Conclusion: We assessed, quantified, and responded to treatment burden and complexity in real-time during outpatient CF visits. Systematic and individualized assessments of treatment complexity and burden may enhance treatment adherence while preserving quality of life., (© 2021 Wiley Periodicals LLC.)

Background: Pulmonary function tests (PFTs) are performed routinely to evaluate lung function in patients with cystic fibrosis (CF). Staff at the Cincinnati Children's Hospital Medical Center CF Center observed stress in patients before PFTs. An interdisciplinary quality improvement (QI) team was assembled to address this clinical issue., Methods: The Plan-Do-Study-Act method of QI was used to investigate feasibility of assessing stress and offering brief interventions to reduce stress before PFTs. Interventions included listening to music, covering the PFT screen, or doing breathing meditation before PFTs. Patients rated stress levels on a 1 to 5 Likert scale before and after testing., Results: Of 75 patient encounters, interventions were trialed in 20. Fifteen patients who tried an intervention reported wanting to use the intervention again (five encounters had missing data); patients reported that the intervention benefited performance on PFTs in eight encounters (40%). The average pre-PFT stress rating for encounters that trialed an intervention was 2.1 and post-PFT rating was 2.0. Average stress pre-PFT and post-PFT ratings were 1.7 and 1.6 respectively, for encounters that did not trial an intervention. Median length of PFT encounter was 15  minutes regardless of whether intervention was trialed., Conclusion: Some patients with CF utilized interventions, while many opted out. This QI effort identified feasible outpatient clinic interventions that did not negatively impact clinic flow. Finding ways to reduce stress associated with PFTs could have a meaningful impact on patient performance and emotional well-being for a subset of patients., (© 2020 Wiley Periodicals, Inc.)

Background: Research has shown that broad cognitive functioning in individuals with CF is intact. Specific executive functioning (EF) deficits have been identified, however, and adults with CF report more symptoms of ADHD than the general population. EF skills are critical to the management of a complex disease like CF although studies have not adequately examined EF mechanisms in CF. This manuscript (a) described EF in a small sample of children with CF, (b) summarized relations found between EF and psychosocial variables, and (c) presented a conceptual model by which to understand EF's impact on adherence in CF., Methods: Data for this preliminary study were collected from 19 children with CF and their caregivers (ages, 6-18). Caregivers completed questionnaires assessing their child's physical and mental health, their own functioning, and overall family functioning. EF was measured using a parent-report rating scale. Patient health data were collected from the electronic medical record., Results: This sample did not demonstrate elevated levels of EF impairment. Worse EF was related to poor family communication/cohesion, as well as higher treatment burden, worse lung function, poorer adherence, and older age. From these findings, a preliminary model was developed describing EF in the context of CF and adherence., Conclusions: Findings from this preliminary study suggest that the CF regimen and associated symptoms may overload otherwise adequate EF skills. Reducing disease burden and preventing burnout should be a focus of treatment. A better understanding of EF in CF and the impact on adherence would allow for better clinical management and effective design of interventions., (© 2020 Wiley Periodicals, Inc.)