Pulmonary Vascular Dysfunctions in Cystic Fibrosis.

Cystic fibrosis (CF) is an inherited disorder caused by a deleterious mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Given that the CFTR protein is a chloride channel expressed on a variety of cells throughout the human body, mutations in this gene impact several organs, particularly the lungs. For this very reason, research regarding CF disease and CFTR function has historically focused on the lung airway epithelium. Nevertheless, it was discovered more than two decades ago that CFTR is also expressed and functional on endothelial cells. Despite the great strides that have been made in understanding the role of CFTR in the airway epithelium, the role of CFTR in the endothelium remains unclear. Considering that the airway epithelium and endothelium work in tandem to allow gas exchange, it becomes very crucial to understand how a defective CFTR protein can impact the pulmonary vasculature and overall lung function. Fortunately, more recent research has been dedicated to elucidating the role of CFTR in the endothelium. As a result, several vascular dysfunctions associated with CF disease have come to light. Here, we summarize the current knowledge on pulmonary vascular dysfunctions in CF and discuss applicable therapies.

Neighborhood Disadvantage, Quality of Life, and Symptom Burden in Children with Mild Sleep-disordered Breathing.

Rationale: Neighborhood disadvantage (ND) has been associated with sleep-disordered breathing (SDB) in children. However, the association between ND and SDB symptom burden and quality of life (QOL) has not yet been studied.Objectives: To evaluate associations between ND with SDB symptom burden and QOL.Methods: Cross-sectional analyses were performed on 453 children, ages 3-12.9 years, with mild SDB (habitual snoring and apnea-hypopnea index < 3/h) enrolled in the PATS (Pediatric Adenotonsillectomy Trial for Snoring) multicenter study. The primary exposure, neighborhood disadvantage, was characterized by the Child Opportunity Index (COI) (range, 0-100), in which lower values (specifically COI ⩽ 40) signify less advantageous neighborhoods. The primary outcomes were QOL assessed by the obstructive sleep apnea (OSA)-18 questionnaire (range, 18-126) and SDB symptom burden assessed by the Pediatric Sleep Questionnaire-Sleep-related Breathing Disorder (PSQ-SRBD) scale (range, 0-1). The primary model was adjusted for age, sex, race, ethnicity, maternal education, recruitment site, and season. In addition, we explored the role of body mass index (BMI) percentile, environmental tobacco smoke (ETS), and asthma in these associations.Results: The sample included 453 children (16% Hispanic, 26% Black or African American, 52% White, and 6% other). COI mean (standard deviation [SD]) was 50.3 (29.4), and 37% (n = 169) of participants lived in disadvantaged neighborhoods. Poor SDB-related QOL (OSA-18 ⩾ 60) and high symptom burden (PSQ-SRBD ⩾ 0.33) were found in 30% (n = 134) and 75% (n = 341) of participants, respectively. In adjusted models, a COI increase by 1 SD (i.e., more advantageous neighborhood) was associated with an improvement in OSA-18 score by 2.5 points (95% confidence interval [CI], -4.34 to -0.62) and in PSQ-SRBD score by 0.03 points (95% CI, -0.05 to -0.01). These associations remained significant after adjusting for BMI percentile, ETS, or asthma; however, associations between COI and SDB-related QOL attenuated by 23% and 10% after adjusting for ETS or asthma, respectively.Conclusions: Neighborhood disadvantage was associated with poorer SDB-related QOL and greater SDB symptoms. Associations were partially attenuated after considering the effects of ETS or asthma. The findings support efforts to reduce ETS and neighborhood-level asthma-related risk factors and identify other neighborhood-level factors that contribute to SDB symptom burden as strategies to address sleep-health disparities.Clinical trial registered with www.clinicaltrials.gov (NCT02562040).

Circadian blood pressure dysregulation in children with obstructive sleep apnea.

STUDY OBJECTIVES: Obstructive sleep apnea (OSA) adversely affects normal blood pressure (BP) and may disrupt circadian BP patterns. We sought to examine 24-hour circadian BP rhythms in children with OSA and healthy controls. METHODS: Children 5-14 years with OSA and healthy controls underwent 24-hour BP monitoring and actigraphy to quantify sleep. Shape invariant statistical models compared circadian BP patterns (e.g. times of BP peaks, time arrived at peak BP velocity [TAPV]) in the OSA and control groups. RESULTS: The analytic sample included 219 children (mild OSA: n = 52; moderate-to-severe OSA (MS-OSA): n = 50; controls: n = 117). In the morning, the MS-OSA group had earlier TAPV for DBP than controls (51 minutes, p < 0.001). TAPV in the evening was earlier for the MS-OSA group than controls (SBP: 95 minutes, p < 0.001; DBP: 28 minutes, p = 0.028). At mid-day, SBP and DBP velocity nadirs were earlier for the MS-OSA group than controls (SBP: 57 minutes, p < 0.001; DBP: 38 minutes, p < 0.01). The MS-OSA group reached most BP values significantly earlier than controls; the largest differences were 118 minutes (SBP) and 43 minutes (DBP) (p < 0.001). SBP and DBP were elevated in the MS-OSA group (hours 18-21 and 7--12, respectively, p < 0.01) compared to controls. The MS-OSA group was prone to "non-dipping" compared to controls (SBP: odds ratio [OR] = 2.16, 95% CI: 1.09, 4.29; DBP: OR = 3.45, 95% CI: 1.21, 10.23). CONCLUSIONS: Children with MS-OSA had changes in circadian BP patterns, namely earlier TAPV and BP peaks and nadirs than controls. Circadian disturbances in BP rhythms may be key to mapping the natural history of BP dysregulation in children with OSA.

Clinical Characteristics of Primary Snoring vs Mild Obstructive Sleep Apnea in Children: Analysis of the Pediatric Adenotonsillectomy for Snoring (PATS) Randomized Clinical Trial.

Importance: It is unknown whether children with primary snoring and children with mild obstructive sleep apnea (OSA) represent populations with substantially different clinical characteristics. Nonetheless, an obstructive apnea-hypopnea index (AHI) of 1 or greater is often used to define OSA and plan for adenotonsillectomy (AT). Objective: To assess whether a combination of clinical characteristics differentiates children with primary snoring from children with mild OSA. Design, Setting, and Participants: Baseline data from the Pediatric Adenotonsillectomy Trial for Snoring (PATS) study, a multicenter, single-blind, randomized clinical trial conducted at 6 academic sleep centers from June 2016 to January 2021, were analyzed. Children aged 3.0 to 12.9 years with polysomnography-diagnosed (AHI <3) mild obstructive sleep-disordered breathing who were considered candidates for AT were included. Data analysis was performed from July 2022 to October 2023. Main Outcomes and Measures: Logistic regression models were fitted to identify which demographic, clinical, and caregiver reports distinguished children with primary snoring (AHI <1; 311 patients [67.8%]) from children with mild OSA (AHI 1-3; 148 patients [32.2%]). Results: A total of 459 children were included. The median (IQR) age was 6.0 (4.0-7.5) years, 230 (50.1%) were female, and 88 (19.2%) had obesity. A total of 121 (26.4%) were Black, 75 (16.4%) were Hispanic, 236 (51.5%) were White, and 26 (5.7%) were other race and ethnicity. Black race (odds ratio [OR], 2.08; 95% CI, 1.32-3.30), obesity (OR, 1.80; 95% CI, 1.12-2.91), and high urinary cotinine levels (>5 µg/L) (OR, 1.88; 95% CI, 1.15-3.06) were associated with greater odds of mild OSA rather than primary snoring. Other demographic characteristics, clinical examination findings, and questionnaire reports did not distinguish between primary snoring and mild OSA. A weighted combination of the statistically significant clinical predictors had limited ability to differentiate children with mild OSA from children with primary snoring. Conclusions and Relevance: In this analysis of baseline data from the PATS randomized clinical trial, primary snoring and mild OSA were difficult to distinguish without polysomnography. Mild OSA vs snoring alone did not identify a clinical group of children who may stand to benefit from AT for obstructive sleep-disordered breathing. Trial Registration: ClinicalTrials.gov Identifier: NCT02562040.

The interaction between neuromuscular forces, aerodynamic forces, and anatomical motion in the upper airway predicts the severity of pediatric OSA.

Upper airway neuromuscular response to air pressure during inhalation is an important factor in assessing pediatric subjects with obstructive sleep apnea (OSA). The neuromuscular response's strength, timing, and duration all contribute to the potential for airway collapses and the severity of OSA. This study quantifies these factors at the soft palate, tongue, and epiglottis to assess the relationship between neuromuscular control and OSA severity in 20 pediatric subjects with and without trisomy 21, under dexmedetomidine-induced sedation. The interaction between neuromuscular force and airflow pressure force was assessed based on power transferred between the airway wall and airflow calculated from airway wall motion (from cine magnetic resonance images) and air pressure acting on the airway wall (from computational fluid dynamics simulations). Airway wall motion could be asynchronous with pressure forces due to neuromuscular activation, or synchronous with pressure forces, indicating a passive response to airflow. The obstructive apnea-hypopnea index (oAHI) quantified OSA severity. During inhalation, the normalized work done through asynchronous dilation of the airway at the soft palate, tongue, and epiglottis correlated significantly with oAHI (Spearman's ρ = 0.54, 0.50, 0.64; P = 0.03, 0.03, 0.003). Synchronous collapse at the epiglottis correlated significantly with oAHI (ρ = 0.52; P = 0.02). Temporal order of synchronous and asynchronous epiglottis motion during inhalation predicted the severity of OSA (moderate vs. severe) with 100% sensitivity and 70% specificity. Subjects with severe OSA and/or trisomy 21 have insufficient neuromuscular activation during inhalation, leading to collapse and increased neuromuscular activation. Airflow-driven airway wall motion during late inhalation likely is the main determinant of OSA severity.NEW & NOTEWORTHY This is the first study that combines cine MRI and computational fluid dynamics with in vivo synchronous respiratory flow measurement to quantify the interaction between airway neuromuscular forces, aerodynamic forces, and airway anatomy noninvasively in pediatric patients with obstructive sleep apnea (OSA). The results indicate power transfer predicts OSA severity.

Adenotonsillectomy for Snoring and Mild Sleep Apnea in Children: A Randomized Clinical Trial.

Importance: The utility of adenotonsillectomy in children who have habitual snoring without frequent obstructive breathing events (mild sleep-disordered breathing [SDB]) is unknown. Objectives: To evaluate early adenotonsillectomy compared with watchful waiting and supportive care (watchful waiting) on neurodevelopmental, behavioral, health, and polysomnographic outcomes in children with mild SDB. Design, Setting, and Participants: Randomized clinical trial enrolling 459 children aged 3 to 12.9 years with snoring and an obstructive apnea-hypopnea index (AHI) less than 3 enrolled at 7 US academic sleep centers from June 29, 2016, to February 1, 2021, and followed up for 12 months. Intervention: Participants were randomized 1:1 to either early adenotonsillectomy (n = 231) or watchful waiting (n = 228). Main Outcomes and Measures: The 2 primary outcomes were changes from baseline to 12 months for caregiver-reported Behavior Rating Inventory of Executive Function (BRIEF) Global Executive Composite (GEC) T score, a measure of executive function; and a computerized test of attention, the Go/No-go (GNG) test d-prime signal detection score, reflecting the probability of response to target vs nontarget stimuli. Twenty-two secondary outcomes included 12-month changes in neurodevelopmental, behavioral, quality of life, sleep, and health outcomes. Results: Of the 458 participants in the analyzed sample (231 adenotonsillectomy and 237 watchful waiting; mean age, 6.1 years; 230 female [50%]; 123 Black/African American [26.9%]; 75 Hispanic [16.3%]; median AHI, 0.5 [IQR, 0.2-1.1]), 394 children (86%) completed 12-month follow-up visits. There were no statistically significant differences in change from baseline between the 2 groups in executive function (BRIEF GEC T-scores: -3.1 for adenotonsillectomy vs -1.9 for watchful waiting; difference, -0.96 [95% CI, -2.66 to 0.74]) or attention (GNG d-prime scores: 0.2 for adenotonsillectomy vs 0.1 for watchful waiting; difference, 0.05 [95% CI, -0.18 to 0.27]) at 12 months. Behavioral problems, sleepiness, symptoms, and quality of life each improved more with adenotonsillectomy than with watchful waiting. Adenotonsillectomy was associated with a greater 12-month decline in systolic and diastolic blood pressure percentile levels (difference in changes, -9.02 [97% CI, -15.49 to -2.54] and -6.52 [97% CI, -11.59 to -1.45], respectively) and less progression of the AHI to greater than 3 events/h (1.3% of children in the adenotonsillectomy group compared with 13.2% in the watchful waiting group; difference, -11.2% [97% CI, -17.5% to -4.9%]). Six children (2.7%) experienced a serious adverse event associated with adenotonsillectomy. Conclusions: In children with mild SDB, adenotonsillectomy, compared with watchful waiting, did not significantly improve executive function or attention at 12 months. However, children with adenotonsillectomy had improved secondary outcomes, including behavior, symptoms, and quality of life and decreased blood pressure, at 12-month follow-up. Trial Registration: ClinicalTrials.gov Identifier: NCT02562040.

Defining and Promoting Pediatric Pulmonary Health: Understanding Sleep and Ventilatory Health.

Healthy sleep and optimal ventilatory control begin in early development and are crucial for positive child outcomes. This paper summarizes information presented at the Sleep and Ventilatory Control sessions of the National Heart, Lung, and Blood-sponsored 2021 Defining and Promoting Pediatric Pulmonary Health workshop. These sessions focused on pediatric sleep health, screening for sleep health and sleep disorders in primary care using the electronic health record, infant sleep and ventilatory control, and home sleep testing. Throughout this summary, we discuss key gaps in and barriers to promoting sleep and ventilatory health that were identified during the workshop sessions. We conclude with strategies to address these gaps and barriers and directions for future multidisciplinary research, patient care, and training.

The Cystic Fibrosis Learning Network: A mixed methods evaluation of program goals, attributes, and impact.

Introduction: The Cystic Fibrosis (CF) Foundation sponsored the design, pilot testing, and implementation of the CF Learning Network (CFLN) to explore how the Foundation's Care Center Network (CCN) could become a learning health system. Six years after the design, the Foundation commissioned a formative mixed methods evaluation of the CFLN to assess: CFLN participants' understanding of program goals, attributes, and perceptions of current and future impact. Methods: We performed semi-structured interviews with CFLN participants to identify perceived goals, attributes, and impact of the network. Following thematic analyses, we developed and distributed a survey to CFLN members and a matched sample of CCN programs to understand whether the themes were unique to the CFLN. Results: Interviews with 24 CFLN participants were conducted. Interviewees identified the primary CFLN goal as improving outcomes for people living with CF, with secondary goals of providing training in quality improvement (QI), creating a learning community, engaging all stakeholders in improvement, and spreading best practices to the CCN. Project management, use of data, common QI methods, and the learning community were seen as critical to success. Survey responses were collected from 103 CFLN members and 25 CCN members. The data revealed that CFLN respondents were more likely than CCN respondents to connect with other CF programs, routinely use data for QI, and engage patient and family partners in QI. Conclusions: Our study suggests that the CFLN provides value beyond that achieved by the CCN. Key questions remain about whether spread of the CFLN could improve outcomes for more people living with CF.

Establishing a Cystic Fibrosis Learning Network: Interventions to promote collaboration and data-driven improvement at scale.

Introduction: A learning health network is a type of learning health system in which stakeholders use network organization to improve health and health care. Building on existing resources in the cystic fibrosis (CF) community, the Cystic Fibrosis Learning Network (CFLN) was designed to improve medical outcomes and quality of life through an intentional focus on achieving reliable evidence-based chronic care delivery and creating a system for data-driven collaborative learning. Methods: We describe the development and growth of the CFLN considering six domains of a Network Maturity Grid: system leadership; governance and policy management; quality improvement (QI); engagement and community building; data and analytics; and research. We illustrate the impact of the CFLN experience on chronic care processes and indicators of collaborative infrastructure. Results: The CFLN represents 36 accredited care centers in the CF Foundation Care Center Network caring for over 6300 patients. Of 6779 patient clinical care visits/quarter, 77% are entered into the CF Foundation Patient Registry within 30 days, providing timely means to track outcomes. Collaborative visit planning is occurring in 93% of clinical care visits to share agenda setting with patients and families. Almost all CFLN teams (94%, n = 34) have a patient/family partner (PFP), and 74% of PFPs indicate they are actively participating, taking ownership of, or leading QI initiatives with the interdisciplinary care team. In 2022, 97% of centers reported completing 1-13 improvement cycles per month, and 82% contributed to monthly QI progress reports to share learning. Conclusion: The CFLN is a maturing, collaborative infrastructure. CFLN centers practice at an advanced level of coproduction. The CFLN fosters interdisciplinary and PFP leadership and the performance of consistent data-driven improvement cycles. CFLN centers are positioned to respond to rapid changes in evidence-based care and advance the practice of QI and implementation science on a broader scale.

Executive Functioning, Language, and Behavioral Abilities Related to Obstructive Sleep Apnea in Down Syndrome.

OBJECTIVES: Obstructive sleep apnea (OSA) is highly prevalent among individuals with Down syndrome (DS), and the nonphysiological consequences of OSA require examination to inform treatment planning. This study aimed to investigate the association between OSA and aspects of language, executive functioning, behavioral, social abilities, and sleep problems in youth with DS aged 6 to 17 years. METHODS: Multivariate analysis of covariance was used to compare 3 groups adjusted for age, participants with DS with untreated OSA (n = 28), participants with DS without OSA (n = 38), and participants with DS with treated OSA (n = 34). To be eligible for the study, participants had to have an estimated mental age of 3 years. No children were excluded based on estimated mental age. RESULTS: After adjusting for age, participants with untreated OSA showed a common pattern of lower estimated marginal mean scores than those with treated OSA and those with no OSA in expressive and receptive vocabulary and higher estimated marginal mean scores with executive functions, everyday memory, attention, internalizing and externalizing behavior, social behavior, and sleep problems. However, only the group differences for executive function (emotional regulation) and internalizing behavior were statistically significant. CONCLUSION: Study findings corroborate and extend prior findings related to OSA and clinical outcomes for youth with DS. The study highlights the importance of OSA treatment in youth with DS and provides clinical recommendations for this population. Additional studies are necessary to control the effects of health and demographic variables.

Comparison of Caregiver- and Child-Reported Quality of Life in Children With Sleep-Disordered Breathing.

OBJECTIVE: Caregivers frequently report poor quality of life (QOL) in children with sleep-disordered breathing (SDB). Our objective is to assess the correlation between caregiver- and child-reported QOL in children with mild SDB and identify factors associated with differences between caregiver and child report. STUDY DESIGN: Analysis of baseline data from a multi-institutional randomized trial SETTING: Pediatric Adenotonsillectomy Trial for Snoring, where children with mild SDB (obstructive apnea-hypopnea index <3) were randomized to observation or adenotonsillectomy. METHODS: The Pediatric Quality of Life Inventory (PedsQL) assessed baseline global QOL in participating children 5 to 12 years old and their caregivers. Caregiver and child scores were compared. Multivariable regression assessed whether clinical factors were associated with differences between caregiver and child report. RESULTS: PedsQL scores were available for 309 families (mean child age, 7.0 years). The mean caregiver-reported PedsQL score was higher at 75.2 (indicating better QOL) than the mean child-reported score of 67.9 (P < .001). The agreement between caregiver and child total PedsQL scores was poor, with intraclass correlation coefficients of 0.03 (95% CI, -0.09 to 0.15) for children 5 to 7 years old and 0.21 (95% CI, 0.03-0.38) for children 8 to 12 years old. Higher child age and health literacy were associated with closer agreement between caregiver and child report. CONCLUSION: Caregiver- and child-reported global QOL in children with SDB was weakly correlated, more so for young children. In pediatric SDB, child-perceived QOL may be poorer than that reported by caregivers. Further research is needed to assess whether similar trends exist for disease-specific QOL metrics.

Standardizing electronic health record ventilation data in the pediatric long-term mechanical ventilator-dependent population.

BACKGROUND: Sharing data across institutions is critical to improving care for children who are using long-term mechanical ventilation (LTMV). Mechanical ventilation data are complex and poorly standardized. This lack of data standardization is a major barrier to data sharing. OBJECTIVE: We aimed to describe current ventilator data in the electronic health record (EHR) and propose a framework for standardizing these data using a common data model (CDM) across multiple populations and sites. METHODS: We focused on a cohort of patients with LTMV dependence who were weaned from mechanical ventilation (MV). We extracted and described relevant EHR ventilation data. We identified the minimum necessary components, termed "Clinical Ideas," to describe MV from time of initiation to liberation. We then utilized existing resources and partnered with informatics collaborators to develop a framework for incorporating Clinical Ideas into the PEDSnet CDM based on the Observational Medical Outcomes Partnership (OMOP). RESULTS: We identified 78 children with LTMV dependence who weaned from ventilator support. There were 25 unique device names and 28 unique ventilation mode names used in the cohort. We identified multiple Clinical Ideas necessary to describe ventilator support over time: device, interface, ventilation mode, settings, measurements, and duration of ventilation usage per day. We used Concepts from the SNOMED-CT vocabulary and integrated an existing ventilator mode taxonomy to create a framework for CDM and OMOP integration. CONCLUSION: The proposed framework standardizes mechanical ventilation terminology and may facilitate efficient data exchange in a multisite network. Rapid data sharing is necessary to improve research and clinical care for children with LTMV dependence.

Body composition and functional correlates of CF youth experiencing pulmonary exacerbation and recovery.

BACKGROUND: Youth with cystic fibrosis (CF) and pulmonary exacerbation (PEx) often experience weight loss, then rapid weight gain. Little is known about body composition and its relationship to functional outcomes during this critical period. METHODS: Twenty CF youth experiencing PEx were assessed on the day following admission and 7-17 days later at discharge for body mass index (BMI), fat mass index (FMI), lean mass index (LMI), skeletal muscle mass index (SMMI), and functional measures: percent predicted forced expiratory volume in 1 second (FEV1) (ppFEV1), maximal inspiratory and expiratory pressures (MIPs and MEPs), and handgrip strength (HGS). Changes from admission to discharge and correlations among body composition indices and functional measures at both times are reported. RESULTS: Upon admission, participant BMI percentile and ppFEV1 varied from 2 to 97 and 29 to 113, respectively. Thirteen had an LMI below the 25th percentile and nine had a percent body fat above the 75th percentile. BMI and FMI increased significantly (p = 0.03, 0.003) during hospitalization. LMI and SMMI did not change. FEV1 and MIPS increased (p = 0.0003, 0.007), independent of weight gain, during treatment. HGS did not improve. CONCLUSIONS: Many youth with CF, independent of BMI, frequently carried a small muscle mass and disproportionate fat at the time of PEx. During hospital treatment, weight gain largely represented fat deposition; muscle mass and strength did not improve. A need for trials of interventions designed to augment muscle mass and function, and limit fat mass accretion, at the time of PEx is suggested by these observations.

Computational assessment of upper airway muscular activity in obstructive sleep apnea - In vitro validation.

Neuromuscular control of the upper airway contributes to obstructive sleep apnea (OSA). An accurate, non-invasive method to assess neuromuscular function is needed to improve surgical treatment outcomes. Currently, surgical approaches for OSA are based on airway anatomy and are often not curative. When the airway surface moves, the power transferred between air in the airway lumen and the structures of the upper airway may be a measure of airway neuromuscular activity. The aim of this study was to validate power transfer as a measure of externally applied forces, representing neuromuscular activity, through cine computed tomography (CT) imaging and computational fluid dynamics (CFD) analysis in a 3D-printed airway model. A hollow elastic airway model was manufactured. An insufflation/exsufflation device generated airflow within the model lumen. The model was contained in an airtight chamber that could be positively or negatively pressurized to represent muscular forces. These forces were systematically applied to dilate and collapse the model. Cine CT imaging captured airway wall movement during respiratory cycles with and without externally applied forces. Power transfer was calculated from the product of wall movement and internal aerodynamic pressure forces using CFD simulations. Cross-correlation peaks between power transfer and changes in externally applied pressure during exhalation and inhalation were -0.79 and 0.95, respectively. Power transfer calculated via cine CT imaging and CFD was an accurate surrogate measure of externally applied forces representing airway muscular activity. In the future, power transfer may be used in clinical practice to phenotype patients with OSA and select personalized therapies.

Neurobehavioral morbidity of pediatric mild sleep-disordered breathing and obstructive sleep apnea.

STUDY OBJECTIVES: Obstructive sleep apnea is associated with neurobehavioral dysfunction, but the relationship between disease severity as measured by the apnea-hypopnea index and neurobehavioral morbidity is unclear. The objective of our study is to compare the neurobehavioral morbidity of mild sleep-disordered breathing versus obstructive sleep apnea. METHODS: Children 3-12 years old recruited for mild sleep-disordered breathing (snoring with obstructive apnea-hypopnea index < 3) into the Pediatric Adenotonsillectomy Trial for Snoring were compared to children 5-9 years old recruited for obstructive sleep apnea (obstructive apnea-hypopnea 2-30) into the Childhood Adenotonsillectomy Trial. Baseline demographic, polysomnographic, and neurobehavioral outcomes were compared using univariable and multivariable analysis. RESULTS: The sample included 453 participants with obstructive sleep apnea (median obstructive apnea-hypopnea index 5.7) and 459 participants with mild sleep-disordered breathing (median obstructive apnea-hypopnea index 0.5). By polysomnography, participants with obstructive sleep apnea had poorer sleep efficiency and more arousals. Children with mild sleep-disordered breathing had more abnormal executive function scores (adjusted odds ratio 1.96, 95% CI 1.30-2.94) compared to children with obstructive sleep apnea. There were also elevated Conners scores for inattention (adjusted odds ratio 3.16, CI 1.98-5.02) and hyperactivity (adjusted odds ratio 2.82, CI 1.83-4.34) in children recruited for mild sleep-disordered breathing. CONCLUSIONS: Abnormal executive function, inattention, and hyperactivity were more common in symptomatic children recruited into a trial for mild sleep-disordered breathing compared to children recruited into a trial for obstructive sleep apnea. Young, snoring children with only minimally elevated apnea-hypopnea levels may still be at risk for deficits in executive function and attention. TRIAL REGISTRATION: Pediatric Adenotonsillectomy for Snoring (PATS), NCT02562040; Childhood Adenotonsillectomy Trial (CHAT), NCT00560859.

Improving Home Ventilator Alarm Use Among Children Requiring Chronic Mechanical Ventilation.

BACKGROUND AND OBJECTIVES: Children requiring long-term mechanical ventilation are at high risk of mortality. Setting ventilator alarms may improve safety, but best practices for setting ventilator alarms have not been established. Our objective was to increase the mean proportion of critical ventilator alarms set for those children requiring chronic mechanical ventilation followed in our pulmonary clinic from 63% to >90%. METHODS: Using the Institute for Healthcare Improvement Model for Improvement, we developed, tested, and implemented a series of interventions using Plan-Do-Study-Act cycles. We followed our progress using statistical process control methods. Our primary interventions were: (1) standardization of the clinic workflow, (2) development of an algorithm to guide physicians in selecting and setting ventilator alarms, (3) updating that algorithm based on review of failures and inpatient testing, and (4) enhancing staff engagement to change the culture surrounding ventilator alarms. RESULTS: We collected baseline data from May 1 to July 13, 2017 on 130 consecutive patients seen in the pulmonary medicine clinic. We found that 63% of critical ventilator alarms were set. Observation of the process, standardization of workflow, and adaptation of an alarm algorithm led to an increase to 85.7% of critical alarms set. Through revising our algorithm to include an apnea alarm, and maximizing provider engagement, more than 95% of critical ventilator alarms were set, exceeding our goal. We sustained this improvement through January 2021. CONCLUSIONS: Our stepwise approach, including process standardization, staff engagement, and integration of an alarm algorithm, improved the use of ventilator alarms in chronically ventilated pediatric patients.

Cystic fibrosis learning network telehealth innovation lab during the COVID-19 pandemic: a success QI story for interdisciplinary care and agenda setting.

INTRODUCTION: The Cystic Fibrosis Foundation chronic care guidelines recommend monitoring clinical status of a patient with cystic fibrosis (CF) through quarterly interdisciplinary visits. At the beginning of the COVID-19 pandemic, the Cystic Fibrosis Learning Network (CFLN) designed and initiated a telehealth (TH) innovation lab (TH ILab) to support transition from the classic CF care model of quarterly in-person office visits to a care model that included TH. AIM: The specific aims of the TH ILab were to increase the percentage of virtual visits with interdisciplinary care (IDC) from 60% to 85% and increase the percentage of virtual visits in which patients and families participated in shared agenda setting (AS) from 52% to 85% by 31 December 2020. METHODS: The model for improvement methodology was used to determine the ILab aims, theory, interventions and measures. In the testing phase of the ILab, data related to process and outcome measures as well as learnings from plan-do-study-act cycles were collected, analysed and shared weekly with the TH ILab teams. Participating centres created processes for IDC and AS for TH visits and developed and shared quality improvement tools specific to their local context with other centres during the ILab weekly meetings and via a secure CFLN-maintained platform. RESULTS: Both specific aims were achieved ahead of the expected target date. By August 2020, 85% of the TH ILab visits provided IDC and 92% of patients were seen for CF care by teams from the TH ILab that participated in AS. CONCLUSION: Shared learning through a collaborative, data-driven process in the CFLN TH ILab rapidly led to standardised TH IDC and AS, which achieved reliable and sustainable processes which could be reproduced by other networks.