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.

Cystic Fibrosis: A Review.

Importance: Cystic fibrosis, a genetic disorder defined by variants in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, affects more than 30 000 individuals in the US and approximately 89 000 worldwide. Absent or decreased function of the CFTR protein is associated with multiorgan dysfunction and shortened life expectancy. Observations: CFTR is an anion channel in the apical membrane of epithelial cells. Loss of function leads to obstructed exocrine glands. Of people with cystic fibrosis in the US, approximately 85.5% have the gene variant F508del. Manifestations of cystic fibrosis in patients with the F508del gene variant begin in infancy with steatorrhea, poor weight gain, and respiratory symptoms (coughing, wheezing). As people with cystic fibrosis age, chronic respiratory bacterial infections cause loss of lung function and bronchiectasis. With the availability of universal newborn screening in multiple countries including the US, many people with cystic fibrosis are asymptomatic at diagnosis. With multidisciplinary care teams that included dietitians, respiratory therapists, and social workers, treatment of cystic fibrosis can slow disease progression. Median survival has improved from 36.3 years (95% CI, 35.1-37.9) in 2006 to 53.1 years (95% CI, 51.6-54.7) in 2021. Pulmonary therapies for patients with cystic fibrosis consist of mucolytics (eg, dornase alfa), anti-inflammatories (eg, azithromycin), and antibiotics (such as tobramycin delivered by a nebulizer). Four small molecular therapies, termed CFTR modulators, that facilitate CFTR production and/or function have received regulatory approval. Examples are ivacaftor and elexacaftor-tezacaftor-ivacaftor. For example, in patients with 1 F508del variant, the combination of ivacaftor, tezacaftor, and elexacaftor improved lung function from -0.2% in the placebo group to 13.6% (difference, 13.8%; 95% CI, 12.1%-15.4%) and decreased the annualized estimated rate of pulmonary exacerbations from 0.98 to 0.37 (rate ratio, 0.37; 95% CI, 0.25-0.55). Improved respiratory function and symptoms have lasted up to 144 weeks in postapproval observational studies. An additional 177 variants are eligible for treatment with the elexacaftor-tezacaftor-ivacaftor combination. Conclusion: Cystic fibrosis affects approximately 89 000 people worldwide and is associated with a spectrum of disease related to exocrine dysfunction, including chronic respiratory bacterial infections and reduced life expectancy. First-line pulmonary therapies consist of mucolytics, anti-inflammatories, and antibiotics, and approximately 90% of people with cystic fibrosis who are 2 years or older may benefit from a combination of ivacaftor, tezacaftor, and elexacaftor.

Transforming the nutrition care model for infants with cystic fibrosis: A qualitative study of clinicians' perspectives.

Clinician perspectives may inform health service strategies to meet optimal nutrition needs for infants with cystic fibrosis (CF). We conducted a qualitative study with CF-specialized dietitians (registered dietitians [RDs]) and physicians between July to December 2020 to characterize the current state of infant nutrition care delivery and organize input into a conceptual model to inform CF care program strategies. Among 42 participants, 36 completed survey responses and 6 completed interviews; 93% were RDs. Three global themes emerged in the current care model: nutrition management, family centered connections, and collaborative care delivery. Within nutrition management, clinicians emphasized providing education, setting goals, and maintaining adequate follow-up with families. Under family centered connections, clinicians expressed the need to foster relationships with families and link families to resources for assistance to social stressors such as food insecurity. Collaborative care delivery for clinicians interviewed was defined by sharing expertise from across the interdisciplinary team. Based on the timing of this study, clinicians reported compelling examples for various modes of telehealth and home weight monitoring to facilitate and support these domains of nutrition care, including potential advantages for education, supporting family needs, and communication. We integrate these themes to propose a conceptual model to organize complementary in-person and telehealth activities and enhance quality infant CF nutrition care delivery. Future implementation can refine this model through testing of practical telehealth interventions to optimize nutrition outcomes for infants with CF.

Sustained Reduction in Time to Data Entry in the Cystic Fibrosis Foundation Registry.

INTRODUCTION: Timely data entry into patient registries is foundational to learning health systems such as the Cystic Fibrosis Learning Network. The US Cystic Fibrosis Foundation Patient Registry (CFFPR) is an established registry that collects encounter data for clinical and research activities. Coordinators manually enter approximately 1,500 encounters annually at our institution, but there is limited evidence for interventions facilitating timely data entry. Our institution aimed to reduce the number of days between a clinical encounter and data entry into the CFFPR from an average of 43 days (range 0 to 183 days) to less than 30 days in a 3-month interval. METHODS: Data coordinators tested interventions to address barriers in four themes: accountability, work burden, communication, and visibility using plan-do-study-act cycles. We used statistical process control charts to assess progress on average time of entry. Coordinators provided feedback about acceptability and satisfaction for process changes. RESULTS: Initial interventions standardized process and reduced average time to data entry from 42.6 to 22.5 days in 3 months, but this process was not stable in the subsequent 6 months. Subsequent changes to increase metric visibility and improve team communication increased stability and decreased the average time to data entry to 23.0 days. Coordinators reported high satisfaction with process changes and have sustained improved time for over 2 years. CONCLUSIONS: This quality improvement project reduced and maintained data entry time by addressing significant barriers without additional personnel. Increased access to near real-time data in CFFPR accelerates learning for clinical care, quality improvement, and research.

Impact of guideline-recommended dietitian assessments on weight gain in infants with cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF)-specialized nutrition care strives to meet normal infant growth, but the relationship of dietitian assessments to weight outcomes is unknown. We characterize nutrition management for inadequate weight gain and assess association of dietitian assessments and center-level weight-for-age Z-scores (WAZ). METHODS: We used encounter data from 226 infants across 28 US CF Centers from the Baby Observational Nutritional study between January 2012 through December 2017. We identified dietitian assessments and consensus guideline-recommended responses to inadequate weight gain: calorie increases, pancreatic enzyme replacement therapy (PERT) increases, or shortened time to next visit. We compared center assessments by funnel plot and summarize median WAZ by center. RESULTS: Of 2,527 visits, 808 (32%) visits had identified inadequate weight gain, distributed in 216 infants. Assessments occurred in 1953 visits (77%), but varied widely between centers (range 17% - 98%). For inadequate weight gain, most and least common responses were calorie increase (64%) and PERT increase (21%). Funnel plot analysis identified 4 high-performers for frequent dietitian assessments (range 92% - 98%) and 4 under-performers (range 17% - 56%). High-performers treated inadequate weight gain more often with adequate calories (24/30, 80% v. 12/23, 52%) and closer follow up (104/164, 63% v. 60/120, 49%) compared to under-performers. Three of 4 high-performing sites met center nutrition goals for positive median WAZ at 2 years old unlike 3 under-performers (WAZHigh 0.33 v. WAZLow -0.15), despite similar patient characteristics. CONCLUSION: We characterized multicenter variation in dietitian assessments, identifying opportunities to improve care delivery to target early nutrition outcomes.

Financial impacts of the COVID-19 pandemic on cystic fibrosis care: lessons for the future.

BACKGROUND: Chronic care delivery models faced unprecedented financial pressures, with a reduction of in-person visits and adoption of telehealth during the COVID-19 pandemic. We sought to understand the reported financial impact of pandemic-related changes to the cystic fibrosis (CF) care model. METHODS: The U.S. CF Foundation State of Care surveys fielded in Summer 2020 (SoC1) and Spring 2021 (SoC2) included questions for CF programs on the impact of pandemic-related restrictions on overall finances, staffing, licensure, and reimbursement of telehealth services. Descriptive analyses were conducted based on program type. RESULTS: Among the 286 respondents (128 pediatric, 118 adult, 40 affiliate), the majority (62%) reported a detrimental financial impact to their CF care program in SoC1, though fewer (42%) reported detrimental impacts in SoC2. The most common reported impacts in SoC1 were redeployment of clinical staff (68%), furloughs (52%), hiring freezes (51%), decreases in salaries (34%), or layoffs (10%). Reports of lower reimbursement for telehealth increased from 30% to 40% from SoC1 to SoC2. Projecting towards the future, only a minority (17%) of program directors in SoC2 felt that financial support would remain below pre-pandemic levels. CONCLUSIONS: The COVID-19 pandemic resulted in financial strain on the CF care model, including challenges with reimbursement for telehealth services and reductions in staffing due to institutional changes. Planning for the future of CF care model needs to address these short-term impacts, particularly to ensure a lack of interruption in high-quality multi-disciplinary care.

Evaluating barriers to and promoters of telehealth during the COVID-19 pandemic at U.S. cystic fibrosis programs.

BACKGROUND: Cystic fibrosis (CF) care programs in the United States rapidly adopted telehealth during the COVID-19 pandemic. Understanding factors that promote or impede telehealth will inform planning for future telehealth-enabled care models. METHODS: Adult, pediatric, and affiliate CF care programs in the United States (n = 287) were surveyed twice eight months apart in 2020-2021 about telehealth use. Programs were asked to describe barriers to and promoters of telehealth. RESULTS: Ninety-seven percent of programs provided telehealth services. In the first CF Care Program State of Care Survey (SoC1), programs estimated that 57% of patients exclusively received in-person care, 36% of patients received telehealth by phone/computer with video, and 8% of patients received telephone-only care. In the second CF Care Program State of Care Survey (SoC2), programs estimated that 80% of visits were in-person and 15% were via audio and video telehealth. Pediatric programs (21%) were less likely than adult (37%) or affiliate (41%) programs to recommend telehealth (p = 0.007). All programs ranked lack of internet access as the highest barrier to patient engagement with telehealth. Promoters of telehealth were increased accessibility and avoidance of infection transmission. Top ranked changes to improve telehealth were expanded provision of remote monitoring devices and technology access. Similar proportions of program types anticipated institutional telehealth expansion. CONCLUSION: During the COVID-19 pandemic, CF programs in the United States identified factors to improve future care delivery via telehealth. Targeting specific barriers and promoters will improve the use and quality of telehealth throughout the care center network.

Telehealth use in cystic fibrosis during COVID-19: Association with race, ethnicity, and socioeconomic factors.

BACKGROUND: Due to the COVID-19 pandemic, there was an uptake of telehealth in cystic fibrosis care. Previous studies show disparities in telehealth use based on socioeconomic status (SES). We aimed to: (1) understand telehealth use and perceptions and (2) identify the facilitators and barriers to telehealth use among people with CF and their families (PwCF) from diverse racial/ethnic and socioeconomic backgrounds. METHODS: We conducted an analysis of the 2020 Cystic Fibrosis State of Care surveys completed by PwCF (PFSoC), CF Care Programs (SoC1) and the CF Foundation Patient Registry (CFFPR). RESULTS: A total of 424 PwCF and 286 programs responded to the PFSoC and SoC1. Among PwCF, 90% self-identified as White, 6% as Hispanic/Latino, and 2% as Black. Racial/ethnic minorities were less likely to have had a telehealth visit (p=.015). This difference was pronounced among the Hispanic/Latino population (p<.01). Telehealth use did not differ by health insurance and was similarly offered independent of financial status. Compared to PwCF who denied financial constraints, those who reported financial difficulties found telehealth more difficult to use (p=.018) and were less likely to think that their concerns (p=.010) or issues that mattered most to them (p=.020) were addressed during telehealth. Programs perceived lack of technology, language barriers, and home conditions as barriers to telehealth in vulnerable populations. CONCLUSION: PFSoC and SoC1 identified differences in telehealth use and care perceptions by ethnicity, race, and socioeconomic characteristics. Further studies are needed to understand how telehealth can change access to CF care in diverse subpopulations.

Remote monitoring in telehealth care delivery across the U.S. cystic fibrosis care network.

BACKGROUND: Cystic fibrosis (CF) programs and people with CF (PwCF) employed various monitoring methods for virtual care during the COVID-19 pandemic. This paper characterizes experiences with remote monitoring across the U.S. CF community. METHODS: The CF Foundation (CFF) sponsored distribution of home spirometers (April 2020 to May 2021), surveys to PwCF and CF programs (July to September 2020), and a second program survey (April to May 2021). We used mixed methods to explore access, use, and perspectives regarding the use of remote monitoring in future care. RESULTS: By October 2020, 13,345 spirometers had been distributed, and 19,271 spirometers by May 2021. Programs (n=286) estimated proportions of PwCF with home devices increased over seven months: spirometers (30% to 70%), scales (50% to 70%), oximeters (5% to 10%) with higher estimates in adult programs for spirometers and oximeters. PwCF (n=378) had access to scales (89%), followed by oximeters (48%) and spirometers (47%), often using scales and oximeters weekly, and spirometers monthly. Over both surveys, some programs had no method to collect respiratory specimens for cultures associated with telehealth visits (47%, n=132; 41%, n=118). Most programs (81%) had a process for phlebotomy associated with a telehealth visit, primarily through off-site labs. Both PwCF and programs felt future care should advance remote monitoring and recommended improvements for access, training, and data collection systems. CONCLUSIONS: PwCF and programs experienced unprecedented access to remote monitoring and raised its importance for future care. Improvements to current systems may leverage these shared experiences to augment future care models.

Giant pulmonary artery aneurysms / Aneurismas gigantes de arteria pulmonar

The accompanying images demonstrate giant pulmonary artery aneurysms in a patient with idiopathic pulmonary arterial hypertension (Image 1). In addition to the main pulmonary artery, both the left and right pulmonary arteries are aneurysmal and are compressing the lung parenchyma (Image 2).

Las imágenes adjuntas muestran aneurismas gigantes de la arteria pulmonar en un paciente con hipertensión arterial pulmonar idiopática (Imagen 1). Además de la arteria pulmonar principal, tanto la arteria pulmonar izquierda como la derecha son aneurismáticas y están comprimiendo el parénquima pulmonar (Imagen 2).

Integration of mental health screening and treatment into cystic fibrosis clinics: Evaluation of initial implementation in 84 programs across the United States.

BACKGROUND: A large-scale epidemiological study of 6088 individuals with cystic fibrosis (CF) and 4102 caregivers in nine countries documented elevated symptoms of depression and anxiety, leading to international guidelines for annual screening and follow-up. To facilitate national implementation, 84 CF programs funded a mental health coordinators (MHC). Implementation was evaluated after 1 year using the consolidated framework for implementation research (CFIR) to identify facilitators and barriers. METHODS: A 45-item internet survey was developed to assess relevant CFIR implementation steps. Surveys were completed in 2016. It assessed five domains tailored to study aims: (a) Intervention characteristics, (b) outer setting, (c) inner setting, (d) characteristics of individuals, and (e) process of implementation. RESULTS: Response rate was 88%, with pediatric and adult programs equally represented. A majority of MHCs were social workers (54.1%) and psychologists (41.9%); 41% had joined the team in the past year. Facilitators across the five domains included universal uptake of screening tools, greater awareness and detection of psychological symptoms, reduced stigma, and positive feedback from patients and families. Barriers included limited staff time, space, and logistics. DISCUSSION: This is the largest systematic effort to integrate mental health screening and treatment into the care of individuals with a serious, chronic illness and their caregivers. MHCs implementing screening, interpretation and follow-up reported positive results, and significant barriers. This national implementation effort demonstrated that depression and anxiety can be efficiently evaluated and treated in a complex, chronic disease. Future efforts include recommending the addition of screening scores to national CF Registries and examining their effects on health outcomes.

Implementation strategies for depression and anxiety screening in a pediatric cystic fibrosis center: A quality improvement project.

BACKGROUND: Guidelines to integrate mental health screening (MHS) into Cystic fibrosis (CF) care has increased adoption across the United States and European CF Centers, however, there has been limited discussion on specific strategies for implementation. OBJECTIVE: Share key strategies that led to successful MHS implementation in one pediatric CF center and report implementation and screening outcomes. METHODS: Parent partners, mental health experts from the Department of Psychiatry, and the CF clinic team codesigned interventions to support three major stakeholders: (a) patients and families (b) clinic team, and (c) hospital system. The mental health coordinator approached patients (12 and older) to introduce MHS and administered screening and reviewed results using an electronic tablet and digital measurement-feedback system. We used strategies that promoted visibility of screening progress and sharing of data with hospital administration. Descriptive statistics were used to assess prevalence of clinically significant symptoms of anxiety and depression and symptom severity within our sample. RESULTS: Over the first 2 years of the project, we exceeded our goal of screening 80% of eligible patients per year (80%-95% screened) and are on a similar trajectory within the first 7 months of year 3. We identified high prevalence of clinically significant symptoms of depression (16%) and anxiety (14%) similar to those found in other chronic illness populations. These data helped us advocate hospital leadership to support the development of new mental health services to address identified needs at our center. CONCLUSION: Leveraging coproduction to address stakeholder needs led to successful implementation of a sustainable MHS process.

The Trach Safe Initiative: A Quality Improvement Initiative to Reduce Mortality among Pediatric Tracheostomy Patients.

OBJECTIVE: To describe the Trach Safe Initiative and assess its impact on unanticipated tracheostomy-related mortality in outpatient tracheostomy-dependent children (TDC). METHODS: An interdisciplinary team including parents and providers designed the initiative with quality improvement methods. Three practice changes were prioritized: (1) surveillance airway endoscopy prior to hospital discharge from tracheostomy placement, (2) education for community-based nurses on TDC-focused emergency airway management, and (3) routine assessment of airway events for TDC in clinic. The primary outcome was annual unanticipated mortality after hospital discharge from tracheostomy placement before and after the initiative. RESULTS: In the 5 years before and after the initiative, 131 children and 155 children underwent tracheostomy placement, respectively. At the end of the study period, the institution sustained Trach Safe practices: (1) surveillance bronchoscopies increased from 104 to 429 bronchoscopies, (2) the course trained 209 community-based nurses, and (3) the survey was used in 488 home ventilator clinic visits to identify near-miss airway events. Prior to the initiative, 9 deaths were unanticipated. After Trach Safe implementation, 1 death was unanticipated. Control chart analysis demonstrates significant special-cause variation in reduced unanticipated mortality. DISCUSSION: We describe a system shift in reduced unanticipated mortality for TDC through 3 major practice changes of the Trach Safe Initiative. IMPLICATION FOR PRACTICE: Death in a child with a tracheostomy tube at home may represent modifiable tracheostomy-related airway events. Using Trach Safe practices, we address multiple facets to improve safety of TDC out of the hospital.

Surveillance endoscopy after tracheostomy placement in children: Findings and interventions.

OBJECTIVES/HYPOTHESIS: The Seattle Children's Hospital implemented the Trach Safe Initiative to improve airway safety in tracheostomy-dependent children (TDC). A key tenet of this initiative is surveillance endoscopy. The objectives of this study were to describe the prevalence of abnormal airway changes in TDC, identify risk factors for these changes, and describe the frequency of airway interventions. STUDY DESIGN: Retrospective case series. METHODS: This is a review of children 0 to 21 years old who underwent tracheostomy and surveillance endoscopy from February 1, 2014 to January 1, 2019. Descriptive statistics were used to report the prevalence of abnormal airway changes and interventions following tracheostomy. Pearson χ2 tests and logistic regression were used to identify risk factors for the development of abnormal changes. RESULTS: There were 127 children identified. The median time from tracheostomy to initial surveillance endoscopy was 1.6 months (interquartile range = 1.3-2.4 months). At initial endoscopy, 86.6% of patients had at least one abnormal airway finding. The most common findings were subglottic edema/stenosis (57.3%), glottic edema (37.3%), and suprastomal granulation tissue (31.8%). Prematurity and a history of failed extubations were significantly associated with abnormal findings on endoscopy (odds ratio [OR] = 7.2, P = .01 and OR = 4.1, P = .03, respectively). Of those with abnormal findings, 32.7% underwent an intervention to improve airway patency and safety. The most common interventions performed were suprastomal granuloma excision (44.4%), steroid injection (22.2%), and balloon dilation of the glottis or subglottis (19.4%). CONCLUSIONS: The prevalence of early abnormal airway changes in TDC is high, particularly in young children with a history of prematurity and failed extubation. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:1327-1332, 2020.

Highlights from the 2018 North American cystic fibrosis conference.

The 32nd annual North American Cystic Fibrosis Conference was held in Denver, CO on Oct. 18 to 20, 2018. This review highlights presentations in several topic areas, including the pathophysiology and basic science of cystic fibrosis lung disease, clinical trials, clinical care, and quality improvement. Citations from the conference are by first author and abstract or symposium number, as designated in the previously published supplement.

Highlights from the 2017 North American Cystic Fibrosis Conference.

The 31st annual North American Cystic Fibrosis Conference (NACFC) was held in Indianapolis, IN on November 2-4, 2017. Abstracts of presentations from the conference were published in a supplement to Pediatric Pulmonology [2017; Pediatr Pulmonol Suppl. 52: S1-S776]. The current review summarizes several major topic areas addressed at the conference: the pathophysiology and basic science of cystic fibrosis (CF) lung disease, clinical trials, clinical management issues, and quality improvement (QI). In this review, we describe emerging concepts in several areas of CF research and care.

Highlights from the 2016 North American Cystic Fibrosis Conference.

The 30th annual North American Cystic Fibrosis Conference (NACFC) was held in Orlando, FL, on October 27-29, 2016. Abstracts were published in a supplement to Pediatric Pulmonology. This review summarizes several major topic areas addressed at the conference: the pathophysiology of cystic fibrosis (CF) lung disease, clinical trials, clinical management issues, and quality improvement. We sought to provide an overview of emerging concepts in several areas of CF research and care, rather than a comprehensive review of the conference. Citations from the conference are by first author and abstract number or symposium number, as designated in the supplement.

Characterization of Inpatient Cystic Fibrosis Pulmonary Exacerbations.

BACKGROUND AND OBJECTIVES: Pulmonary exacerbations lead to significant morbidity and mortality in patients with cystic fibrosis (CF). National consensus guidelines exist, but few studies report current practice in the treatment and monitoring of pulmonary exacerbations. The goal of this study was to characterize consistency and variability in the inpatient management of CF-related pulmonary exacerbations. We focused on the use of guideline-recommended maintenance therapies, antibiotic selection and treatment regimens, use of systemic corticosteroids, and frequency of lung function testing. We hypothesized that significant variability in these treatment practices exists nationally. METHODS: This trial was a retrospective cross-sectional study. It included patients with CF aged ≤18 years hospitalized for pulmonary exacerbations between July 1, 2010, and June 30, 2015, at hospitals within the US Pediatric Health Information System database that are also Cystic Fibrosis Foundation-accredited care centers. One exacerbation per patient was randomly selected over the 5-year study period. RESULTS: From 38 hospitals, 4827 individual pulmonary exacerbations were examined. Median length of stay was 10.0 days (interquartile range, 6-14.0 days). Significant variation was seen among centers in the use of hypertonic saline (11%-100%), azithromycin (5%-83%), and systemic corticosteroids (3%-61%) and in the frequency of lung function testing. Four different admission antibiotic regimens were used >10% of the time, and the most commonly used admission antibiotic regimen comprised 2 intravenous antibiotics with no additional oral or inhaled antibiotics (29%). CONCLUSIONS: Significant variation exists in the treatment and monitoring of pulmonary exacerbations across Pediatric Health Information System-participating, Cystic Fibrosis Foundation-accredited care centers. Results from this study can inform future research working toward standardized inpatient pulmonary exacerbation management to improve CF care for children and adolescents.