Impact of type of health care provider on long term asthma control.

OBJECTIVE: Asthma prevalence is high and adherence to asthma guidelines is still less than adequate. The main objective of this study was to determine if there were significant differences in outcome measures if asthma care was provided per guidelines either by physicians (pediatric pulmonologists) or specialty trained advance practice nurses (APNs). METHODS: This was a three-year, prospective cohort study of children referred by their primary care providers to a tertiary care center for better asthma control. Patients were provided asthma care per NAEPP guidelines including asthma education. Results were compared over time and between patients followed by physicians or APNs. Alpha level of significance was ≤0.05. RESULTS: The sample included 471 children, ages 2-17 years (mean = 6.4 ± 2.4 years). Physicians and APN's provided asthma care. Of the 471 children enrolled in the study, 176 (37%) were followed for the full three-year study period. At the initial visit, physician group reported more short courses of oral steroids and more unscheduled visits to PCP for acute asthma care in the past 6 months compared to those followed by APNs (<0.05 for all). Among the total cohort and both subgroups, there were significant improvements in mean Asthma Control Test (ACT), acute care need and mean days/month with asthma symptoms over a three-year period (p < 0.05). There was significantly more improvement in use of oral steroids and urgent care visits in physician group (p < 0.05). CONCLUSION: When asthma guidelines are followed, improvements in asthma control are achieved in children in both the MD and APN groups.

Home Oxygen Therapy for Children. An Official American Thoracic Society Clinical Practice Guideline.

BACKGROUND: Home oxygen therapy is often required in children with chronic respiratory conditions. This document provides an evidence-based clinical practice guideline on the implementation, monitoring, and discontinuation of home oxygen therapy for the pediatric population. METHODS: A multidisciplinary panel identified pertinent questions regarding home oxygen therapy in children, conducted systematic reviews of the relevant literature, and applied the Grading of Recommendations, Assessment, Development, and Evaluation approach to rate the quality of evidence and strength of clinical recommendations. RESULTS: After considering the panel's confidence in the estimated effects, the balance of desirable (benefits) and undesirable (harms and burdens) consequences of treatment, patient values and preferences, cost, and feasibility, recommendations were developed for or against home oxygen therapy specific to pediatric lung and pulmonary vascular diseases. CONCLUSIONS: Although home oxygen therapy is commonly required in the care of children, there is a striking lack of empirical evidence regarding implementation, monitoring, and discontinuation of supplemental oxygen therapy. The panel formulated and provided the rationale for clinical recommendations for home oxygen therapy based on scant empirical evidence, expert opinion, and clinical experience to aid clinicians in the management of these complex pediatric patients and identified important areas for future research.

Timing of Spirometry May Impact Hospital Length of Stay for Cystic Fibrosis Pulmonary Exacerbation.

PURPOSE: The optimal timing of spirometry during hospitalization for acute pulmonary exacerbation (PEx) in patients with cystic fibrosis (CF) is unclear. We retrospectively evaluated whether measuring spirometry earlier during hospitalization was associated with a shorter length of stay (LOS). METHODS: In this retrospective study, we analyzed data from the electronic medical record of CF patients 6 years of age and older admitted to a single center for acute PEx requiring IV antibiotic therapy between 2009 and 2016. After excluding patient encounters with missing data on covariates, random-effects linear regression was used to predict LOS as a function of days to first pulmonary function testing (PFT), which was spirometry for our study. RESULTS: One thousand thirty-five hospitalizations of 242 patients met inclusion criteria, with 801 including complete data on covariates. Mean LOS was 10 ± 7 days, with mean time to first PFT of 4 ± 3 days after admission. In multivariable analysis, each additional day to first PFT was associated with 0.97 days longer LOS (95% CI 0.29, 1.64; p = 0.005). CONCLUSIONS: As CF researchers and clinicians work to improve management of PEx, the timing of spirometry during hospitalization remains an important question. Obtaining objective lung function data earlier during the course of therapy may provide information which can lead to reduced hospital LOS for PEx.

Increased Mortality in Adult Cystic Fibrosis Patients with Medicaid Insurance Awaiting Lung Transplantation.

PURPOSE: Cystic fibrosis (CF) is a progressive disease resulting in end-stage lung disease. Lung transplantation (LTx) is an important consideration in these patients. Studies have suggested greater post-LTx mortality among CF patients with public insurance. We evaluated the influence of insurance status on survival among CF patients during their time on the LTx waitlist. METHODS: Adult LTx candidates diagnosed with CF and listed since the implementation of the lung allocation score, from May 2005 until September 2013, were identified in the United Network for Organ Sharing database. Waitlist mortality was compared across projected primary payment type (private insurance or self-pay; Medicaid; Medicare) using Kaplan-Meier functions and Fine-Gray competing-risks survival analysis, accounting for the competing risk of transplantation. RESULTS: 1770 LTx candidates with CF were included in univariate survival analyses, with Medicaid increasing waitlist mortality hazard relative to private insurance (HR 2.28; 95 % CI 1.62, 3.21; p < 0.001) and relative to Medicare (HR 2.23; 95 % CI 1.43, 3.48; p < 0.001). A multivariable competing-risks model confirmed greater waitlist mortality among Medicaid patients relative to private insurance (HR 2.57; 95 % CI 1.56, 4.23; p < 0.001) or patients with Medicare (HR 4.02; 95 % CI 1.98, 8.17; p < 0.001) after adjusting for potential confounders. No differences in waitlist survival were found between Medicare and private insurance. CONCLUSIONS: CF patients with Medicaid insurance have higher risk of death while awaiting LTx when compared to patients with Medicare or private insurance. The impact of insurance status on survival in this population begins before LTx and compounds the disparities previously observed in post-transplant outcomes.

Clinical management of pediatric patients with cystic fibrosis and autism spectrum disorder.

BACKGROUND: Cystic Fibrosis (CF) and autism spectrum disorder (ASD) are life-long conditions with intense treatment burdens for patients and families. Patients with a concurrent diagnosis (CF-ASD) experience unique obstacles to CF care. This study describes the experiences of our multidisciplinary CF team in caring for patients with CF-ASD and provides insight into provider and parental perspectives on clinical management. METHODS: This is a three-part qualitative study involving (1) retrospective chart review of patients with CF-ASD, (2) surveys with multidisciplinary care team members, and (3) semistructured interviews with caregivers of patients with CF-ASD. Challenges in clinical management of this specific cohort were compiled using data from chart review and care team surveys. Strategies to address these concerns were identified and rated by individual families based on relevance and practicality. RESULTS: Within our CF center, 12 patients have an official diagnosis of ASD. Median age of patients with CF-ASD was 8.5 years (range 3-20 years), 67% were male, and 83% were on highly effective modulator therapy. Clinical barriers included sensory processing issues, environmental overstimulation, intolerance to procedures and to disrupted routines. Potentially impactful strategies include patient-specific coping plans, guided behavioral interventions, parental advocacy, and improved communication between the family and multidisciplinary team. CONCLUSION: Children with CF-ASD face extraordinary challenges beyond the experience of neurotypical children with CF. Increased awareness of this complex dual diagnosis will help providers be sensitive to the unique needs of these patients, help build consistent and trustworthy relationships with their families and deliver effective clinical care despite limitations.

Long-term pulmonary sequelae in adolescents post-SARS-CoV-2 infection.

RATIONALE: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes long-term pulmonary sequelae in adults, but little is known about pulmonary outcomes in pediatrics. OBJECTIVE(S): The aim of this study was to describe long-term subjective and objective pulmonary abnormalities after SARS-CoV-2 infection in pediatric populations. METHODS: Single-center, retrospective cohort of patients seen in post-coronavirus disease 2019 (COVID-19) pulmonary clinic in 2021. Subjects evaluated had persistent pulmonary symptoms 4 weeks or more after initial infection. Clinical testing included a 6-min walk test (6MWT), chest X-ray, pre- and postbronchodilator spirometry, plethysmography, and diffusion capacity. Patients were followed 2-to-3-months after the initial visit with repeat testing. The primary outcome was the presence of abnormal pulmonary function testing. Secondary measures included variables associated with pulmonary outcomes. RESULTS: Eighty-two adolescents were seen at a median of 3.5 months postinfection, with approximately 80% reporting two or more symptoms at clinic presentation (cough, chest pain, dyspnea at rest, and exertional dyspnea). At follow-up (~6.5 months) exertional dyspnea persisted for most (67%). Spirometry was normal in 77% of patients, but 31% had a positive bronchodilator response. No abnormalities were noted on plethysmography or diffusion capacity. Clinical phenotypes identified included inhaled corticosteroid responsiveness, paradoxical vocal fold motion disorder, deconditioning, and dysautonomia. Multivariable modeling demonstrated that obesity, anxiety, and resting dyspnea were associated with reduced 6MWT, while female sex and resting dyspnea were associated with higher Borg Dyspnea and Fatigues scores. CONCLUSIONS: This is the largest study to date of pediatric patients with long-term pulmonary sequelae post-COVID-19. Identified clinical phenotypes and risk factors warrant further study and treatment.

A natural language processing pipeline to synthesize patient-generated notes toward improving remote care and chronic disease management: a cystic fibrosis case study.

OBJECTIVES: Patient-generated health data (PGHD) are important for tracking and monitoring out of clinic health events and supporting shared clinical decisions. Unstructured text as PGHD (eg, medical diary notes and transcriptions) may encapsulate rich information through narratives which can be critical to better understand a patient's condition. We propose a natural language processing (NLP) supported data synthesis pipeline for unstructured PGHD, focusing on children with special healthcare needs (CSHCN), and demonstrate it with a case study on cystic fibrosis (CF). MATERIALS AND METHODS: The proposed unstructured data synthesis and information extraction pipeline extract a broad range of health information by combining rule-based approaches with pretrained deep-learning models. Particularly, we build upon the scispaCy biomedical model suite, leveraging its named entity recognition capabilities to identify and link clinically relevant entities to established ontologies such as Systematized Nomenclature of Medicine (SNOMED) and RXNORM. We then use scispaCy's syntax (grammar) parsing tools to retrieve phrases associated with the entities in medication, dose, therapies, symptoms, bowel movements, and nutrition ontological categories. The pipeline is illustrated and tested with simulated CF patient notes. RESULTS: The proposed hybrid deep-learning rule-based approach can operate over a variety of natural language note types and allow customization for a given patient or cohort. Viable information was successfully extracted from simulated CF notes. This hybrid pipeline is robust to misspellings and varied word representations and can be tailored to accommodate the needs of a specific patient, cohort, or clinician. DISCUSSION: The NLP pipeline can extract predefined or ontology-based entities from free-text PGHD, aiming to facilitate remote care and improve chronic disease management. Our implementation makes use of open source models, allowing for this solution to be easily replicated and integrated in different health systems. Outside of the clinic, the use of the NLP pipeline may increase the amount of clinical data recorded by families of CSHCN and ease the process to identify health events from the notes. Similarly, care coordinators, nurses and clinicians would be able to track adherence with medications, identify symptoms, and effectively intervene to improve clinical care. Furthermore, visualization tools can be applied to digest the structured data produced by the pipeline in support of the decision-making process for a patient, caregiver, or provider. CONCLUSION: Our study demonstrated that an NLP pipeline can be used to create an automated analysis and reporting mechanism for unstructured PGHD. Further studies are suggested with real-world data to assess pipeline performance and further implications.

Mental health history and social barriers impacting caregivers of infants with cystic fibrosis.

OBJECTIVES: Caregivers of infants with cystic fibrosis (CF) carry a heavy treatment burden for their child along with the inherent difficulties of raising an infant. This study investigated the impact of self-reported caregiver mental health diagnoses and social barriers during the 1st year of life on clinical outcomes. METHODS: A retrospective chart review was conducted for infants seen in a large tertiary hospital CF clinic over a 5-year period. Baseline characteristics were collected, and documentation from physician and social work notes were reviewed. Demographics and clinical characteristics were compared by the presence or absence of self-reported mental health diagnoses, social barriers, and "emotional concern." RESULTS: Analyses were conducted on 71 patients. Thirty-five percent of caregivers disclosed mental health diagnoses, 52% identified social barriers to care, and 55% reported feeling upset or fatigued. Having a caregiver with a self-reported mental health diagnosis was associated with tobacco smoke exposure (p < .001) and increased odds of hospitalizations (odds ratio [OR], 3.01; 95% confidence interval [CI], 1.49-6.06), emergency department/urgent care visits (OR, 3.17; 95% CI, 1.32-7.64), and longer lengths of stay (OR, 1.93; 95% CI, 1.69-2.20). Caregivers who expressed emotional concern had infants with significantly lower weight-for-length percentiles (p = .012). DISCUSSION: Caregiver mental health and social barriers to care are important determinants to address as they may impact clinical outcomes in infants with CF. Identifying barriers and struggles early increases the likelihood that clinical teams can intervene and provide support. Further research into mental health and socioeconomic barriers faced by caregivers of infants with CF is crucial.

Education and Employment After Lung Transplantation in Adolescents and Young Adults.

Pediatric transplant recipients experience barriers to social functioning, including participation in school and work, but our understanding of barriers to these activities is limited by insufficient data collection and standardization. Existing studies rely on cross-sectional surveys of transplant survivors that are subject to survivorship and nonresponse bias, or analyses of large registry data that lack detail on educational progress and work participation. We report our experience using the electronic medical record to retrospectively review work and educational attainment in this population, and identify specific barriers that were encountered and how they were addressed by the patient and care team. We reviewed current literature on post-transplant survey participation and compared questionnaires to our current documentation practice for tracking education and employment progress in the transplant recipient population. Based on this review, we discuss the possibility, barriers, and implications of conducting a standardized assessment to track social participation outcomes of transplantation.

Long-term work participation among cystic fibrosis patients undergoing lung transplantation.

BACKGROUND: Patients with cystic fibrosis (CF) experience obstacles to employment, regardless of whether they have undergone lung transplantation (LTx). We investigated socioeconomic and clinical factors predicting long-term employment outcomes in CF patients receiving LTx. METHODS: Data from the United Network for Organ Sharing registry were used to identify CF patients 18-59years-old who received LTx between 2000 and 2010 and survived greater than 5years. Long-term employment status was determined by center-reported follow-up data on patients working for income, collected at the 5th transplant anniversary. After multiple imputation to complete missing data on covariates, multivariable logistic regression was used to identify associations between characteristics at or after LTx and long-term work participation. RESULTS: There were 745 patients who met inclusion criteria and contributed employment data within 365days of their 5th LTx anniversary. In this cohort, 48% (358/745) were working for income 5years after LTx. Younger age, male gender, better pulmonary function attained post-transplant, pre-transplant work participation, and private health insurance (compared to government Medicaid or Medicare insurance) at the time of transplant predicted greater odds of post-transplant employment. CONCLUSIONS: Lack of work experience and reliance on government health insurance at the time of transplant predict lower long-term work participation among LTx recipients with CF. By contrast, long-term employment outcomes were not negatively affected by comorbidities at or after transplantation in this cohort. Despite resolving some physiological obstacles to employment in patients with CF, LTx may introduce new socioeconomic barriers to employment.