Outcomes of infants and children with bronchopulmonary dysplasia-associated pulmonary hypertension who required home ventilation.

BACKGROUND: To characterize a cohort of ventilator-dependent infants and children with bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH) and to describe their cardiorespiratory outcomes. METHODS: Subjects with BPD on chronic home ventilation were recruited from outpatient clinics. PH was defined by its presence on ≥1 cardiac catheterization or echocardiogram on or after 36 weeks post-menstrual age. Kaplan-Meier analysis was used to compare the timing of key events. RESULTS: Of the 154 subjects, 93 (60.4%) had PH and of those, 52 (55.9%) required PH-specific medications. The ages at tracheostomy, transition to home ventilator, and hospital discharge were older in those with PH. Most subjects were weaned off oxygen and liberated from the ventilator by 5 years of age, which did not occur later in subjects with PH. The mortality rate after initial discharge was 2.6%. CONCLUSIONS: The majority of infants with BPD-PH receiving chronic invasive ventilation at home survived after initial discharge. Subjects with BPD-PH improved over time as evidenced by weaning off oxygen and PH medications, ventilator liberation, and tracheostomy decannulation. While the presence of PH was not associated with later ventilator liberation or decannulation, the use of PH medications may be a marker of a more protracted disease trajectory. IMPACT STATEMENT: There is limited data on long-term outcomes of children with bronchopulmonary dysplasia (BPD) who receive chronic invasive ventilation at home, and no data on those with the comorbidity of pulmonary hypertension (PH). Almost all subjects with BPD-PH who were on chronic invasive ventilation at home survived after their initial hospital discharge. Subjects with BPD-PH improved over time as evidenced by weaning off oxygen, PH medications, liberation from the ventilator, and tracheostomy decannulation. The presence of PH did not result in later ventilator liberation or decannulation; however, the use of outpatient PH medications was associated with later ventilation liberation and decannulation.

Factors associated with liberation from home mechanical ventilation and tracheostomy decannulation in infants and children with severe bronchopulmonary dysplasia.

OBJECTIVE: To identify factors associated with the timing of ventilator liberation and tracheostomy decannulation among infants with severe bronchopulmonary dysplasia (sBPD) who required chronic outpatient invasive ventilation. STUDY DESIGN: Multicenter retrospective study of 154 infants with sBPD on outpatient ventilators. Factors associated with ventilator liberation and decannulation were identified using Cox regression models and multilevel survival models. RESULTS: Ventilation liberation and decannulation occurred at median ages of 27 and 49 months, respectively. Older age at transition to a portable ventilator and at discharge, higher positive end expiratory pressure, and multiple respiratory readmissions were associated with delayed ventilator liberation. Surgical management of gastroesophageal reflux was associated with later decannulation. CONCLUSIONS: Ventilator liberation timing was impacted by longer initial admissions and higher ventilator pressure support needs, whereas decannulation timing was associated with more aggressive reflux management. Variation in the timing of events was primarily due to individual-level factors, rather than center-level factors.

Respiratory Outcomes for Ventilator-Dependent Children With Bronchopulmonary Dysplasia.

OBJECTIVES: To describe outpatient respiratory outcomes and center-level variability among children with severe bronchopulmonary dysplasia (BPD) who require tracheostomy and long-term mechanical ventilation. METHODS: Retrospective cohort of subjects with severe BPD, born between 2016 and 2021, who received tracheostomy and were discharged on home ventilator support from 12 tertiary care centers participating in the BPD Collaborative Outpatient Registry. Timing of key respiratory events including time to tracheostomy placement, initial hospital discharge, first outpatient clinic visit, liberation from the ventilator, and decannulation were assessed using Kaplan-Meier analysis. Differences between centers for the timing of events were assessed via log-rank tests. RESULTS: There were 155 patients who met inclusion criteria. Median age at the time of the study was 32 months. The median age of tracheostomy placement was 5 months (48 weeks' postmenstrual age). The median ages of hospital discharge and first respiratory clinic visit were 10 months and 11 months of age, respectively. During the study period, 64% of the subjects were liberated from the ventilator at a median age of 27 months and 32% were decannulated at a median age of 49 months. The median ages for all key events differed significantly by center (P ≤ .001 for all events). CONCLUSIONS: There is wide variability in the outpatient respiratory outcomes of ventilator-dependent infants and children with severe BPD. Further studies are needed to identify the factors that contribute to variability in practice among the different BPD outpatient centers, which may include inpatient practices.

Validation of an outpatient questionnaire for bronchopulmonary dysplasia control.

INTRODUCTION: Despite bronchopulmonary dysplasia (BPD) being a common morbidity of preterm birth, there is no validated objective tool to assess outpatient respiratory symptom control for clinical and research purposes. METHODS: Data were obtained from 1049 preterm infants and children seen in outpatient BPD clinics of 13 US tertiary care centers from 2018 to 2022. A new standardized instrument was modified from an asthma control test questionnaire and administered at the time of clinic visits. External measures of acute care use were also collected. The questionnaire for BPD control was validated in the entire population and selected subgroups using standard methodology for internal reliability, construct validity, and discriminative properties. RESULTS: Based on the scores from BPD control questionnaire, the majority of caregivers (86.2%) felt their child's symptoms were under control, which did not differ by BPD severity (p = 0.30) or a history of pulmonary hypertension (p = 0.42). Across the entire population and selected subgroups, the BPD control questionnaire was internally reliable, suggestive of construct validity (albeit correlation coefficients were -0.2 to -0.4.), and discriminated control well. Control categories (controlled, partially controlled, and uncontrolled) were also predictive of sick visits, emergency department visits, and hospital readmissions. CONCLUSION: Our study provides a tool for assessing respiratory control in children with BPD for clinical care and research studies. Further work is needed to identify modifiable predictors of disease control and link scores from the BPD control questionnaire to other measures of respiratory health such as lung function testing.

Approaches to Interdisciplinary Care for Infants with Severe Bronchopulmonary Dysplasia: A Survey of the Bronchopulmonary Dysplasia Collaborative.

OBJECTIVE: Bronchopulmonary dysplasia (BPD) remains the most common late morbidity for extremely premature infants. Care of infants with BPD requires a longitudinal approach from the neonatal intensive care unit to ambulatory care though interdisciplinary programs. Current approaches for the development of optimal programs vary among centers. STUDY DESIGN: We conducted a survey of 18 academic centers that are members of the BPD Collaborative, a consortium of institutions with an established interdisciplinary BPD program. We aimed to characterize the approach, composition, and current practices of the interdisciplinary teams in inpatient and outpatient domains. RESULTS: Variations exist among centers, including composition of the interdisciplinary team, whether the team is the primary or consult service, timing of the first team assessment of the patient, frequency and nature of rounds during the hospitalization, and the timing of ambulatory visits postdischarge. CONCLUSION: Further studies to assess long-term outcomes are needed to optimize interdisciplinary care of infants with severe BPD. KEY POINTS: · Care of infants with BPD requires a longitudinal approach from the NICU to ambulatory care.. · Benefits of interdisciplinary care for children have been observed in other chronic conditions.. · Current approaches for the development of optimal interdisciplinary BPD programs vary among centers..

Growth and nutrition in children with established bronchopulmonary dysplasia: A review of the literature.

BACKGROUND: Bronchopulmonary dysplasia (BPD) remains the most common late morbidity of preterm birth. Clinical care and research have largely focused on the pathogenesis and prevention of BPD. Preterm infants who develop BPD have significant medical needs that persist throughout their hospital course and continue after discharge, including those associated with growth and nutrition. The objective of this study was to review the available literature on nutrition and growth in infants with established BPD and to identify the knowledge and research gaps to provide direction for future studies. METHODS: We conducted a literature search in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines using Ovid MEDLINE, CINAHL, and Embase. Titles, abstracts, and full texts were independently reviewed by the authors and selected based on predetermined inclusion/exclusion criteria. Results were summarized qualitatively. RESULTS: Excluding duplicates, 2315 articles were identified. Thirty articles were selected for inclusion. We identified the following key components of nutrition support and clinical care: energy expenditure, growth, and metabolism; body composition; enteral nutrition; supplements; parenteral nutrition; and respiratory outcomes. CONCLUSIONS: Despite a large body of literature describing the role of growth and nutrition in the prevention of BPD, research is lacking with respect to interventions and management in the population with established BPD. Thus, organized approaches for clinical interventions and trials with respect to growth and nutrition in infants and young children with established BPD are needed. These studies should include multiple centers because of the small numbers of patients with BPD at each site.

Adjusting ventilator settings to avoid air trapping in extremely premature infants reduces the need for tracheostomy and length of stay.

Despite the improving understanding of how lung mechanics and tidal volume requirements evolve during the evolution of bronchopulmonary dysplasia (BPD), clinical management continues to be heterogeneous and inconsistent at many institutions. Recent reports have examined the use of high tidal-volume low respiratory rate strategies in these patients once disease has been well established to help facilitate their eventual extubation and improve their long-term neurodevelopmental outcomes. In this retrospective observational research study, we describe how intentional adjustment of ventilator settings based on patient lung mechanics by an interdisciplinary BPD team improved the care of the at-risk population of infants, reduced the need for tracheostomies, as well as length of stay over a period of over 3 years. The team aimed to establish consistency in the management of these children using a high tidal volume, low-rate approach, and titrating PEEP to address the autoPEEP and bronchomalacia that is frequently observed in this patient population.

Zinc status and growth in infants and young children with cystic fibrosis.

BACKGROUND: Zinc deficiency is associated with poor growth in children without cystic fibrosis (CF), but its impact on growth in children with CF is unknown. OBJECTIVE: To determine the prevalence of low serum Zn (sZn) and its relationship with growth in the first 3 years of life in children with CF. METHODS: We utilized data from infants with CF who were enrolled in a longitudinal study of nutrition and lung health and had sZn measured as part of clinical care. Cross-sectional correlations between sZn levels and growth z scores were assessed by Pearson's correlation coefficient. To identify factors associated with sZn status and its association to longitudinal growth patterns, multiple regression analysis with repeated measures were performed using generalized estimating equations. RESULTS: A total of 106 sZn measurements from 53 infants were identified. Seventeen infants (32%) had intermittent Zn insufficiency, defined as at least one sZn <70 mcg/dl in their first 3 years of life. There were no significant cross-sectional associations between sZn and growth z scores. However, analysis of longitudinal growth patterns revealed that weight- and length-for-age z scores in children with intermittent Zn insufficiency were lower during early infancy and their weight-for-length z scores at age 3 years were also lower compared to those who were always Zn sufficient. CONCLUSION: Low sZn occurs in one-third of children with CF in the first 3 years of life. Cross-sectional and longitudinal analyses revealed discrepant associations between sZn and growth. Therefore, prospective studies are needed to understand the role of Zn in growth in CF.

Nutrition and growth in infants with established bronchopulmonary dysplasia.

Bronchopulmonary dysplasia (BPD) remains the most common late morbidity of preterm birth. Ongoing clinical care and research have largely focused on the pathogenesis and prevention of BPD in preterm infants. However, preterm infants who develop BPD have significant medical needs that persist throughout their neonatal intensive care unit course and continue post-discharge, including those associated with growth and nutrition. The objective of this manuscript was to provide a review on nutrition and growth in infants with established BPD after discharge from the hospital and to identify the knowledge and research gaps to provide direction for future studies.

Outcomes of repeat sweat testing in cystic fibrosis newborn screen positive infants.

BACKGROUND: Infants with a positive cystic fibrosis (CF) newborn screen, only one identified CFTR mutation (NBS+/1 mut), and an initial intermediate sweat chloride (30-59 mmol/L) should have repeat sweat chloride testing (SCT). However, the outcome of repeat SCT and the relationship between initial sweat Cl and subsequent CF diagnosis have not been reported. OBJECTIVE: The objective of this study was to analyze the outcomes of repeat SCT and subsequent CF diagnosis in NBS+/1 mut infants based on their initial sweat chloride concentration. METHODS: We retrospectively identified all infants born in Indiana from 2007 to 2017 with NBS+/1 mut and initial SCT in the intermediate range. For each infant, we recorded the initial and repeat SCT results and/or a final CF diagnosis. RESULTS: From 2007 through 2017 there were 2822 NBS+/1 mut infants of which 2613 (82%) had at least one SCT result. No infants with an initial SCT of 30-39 mmol/L were subsequently diagnosed with CF. Of the 31 infants with an initial SCT of 40-49 mmol/L, only 1 was subsequently diagnosed with CF. In contrast, 61% of those with SCTs of 50-59 mmol/L were later diagnosed with CF. CONCLUSION: These results suggest that infants with a positive NBS for CF and one CFTR mutationwhose initial sweat chloride concentration is 50-59 mmol/L need to be monitored more closely forCF with strong consideration for earlier repeat SCTs and immediate genotyping.

Factors Associated with Neurodevelopmental Impairment in Bronchopulmonary Dysplasia.

OBJECTIVE: To identify factors associated with neurodevelopmental impairment (NDI) in patients with bronchopulmonary dysplasia (BPD). STUDY DESIGN: We identified 151 patients with moderate to severe BPD from 2010 to 2014 with complete Bayley Scales of Infant Development (BSID) scores at 24 months corrected age. We defined NDI as any diagnosis of cerebral palsy or ≥1 BSID composite scores of <80. RESULTS: The mean corrected age at BSID was 23 ± 1 months; 18% had a cognitive score of <80, 37% had a communication score of <80, and 26% had a motor score of <80. Cerebral palsy was diagnosed in 22 patients (15%); 84 (56%) patients did not have NDI. Patients with NDI had lower birth weight, but there was no difference in gestational age at birth, severe intraventricular hemorrhage (IVH), necrotizing enterocolitis, or patent ductus arteriosus ligation compared with patients with no NDI. Ventilator days were greater in patients with NDI than in patients without NDI. More patients with NDI received furosemide and systemic corticosteroids and the hospital length of stay was longer than in patients with no NDI. Logistic regression modeling demonstrated that for every additional 100 g of birth weight the odds of NDI decreased by 35% and for every additional hospital day the odds of NDI increased by 1.3%. CONCLUSIONS: In our cohort of patients with moderate to severe BPD, the majority had no NDI, and low birth weight and length of hospital stay were associated with increased risk of developing NDI. This finding suggests that there are potentially modifiable factors associated with better neurodevelopmental outcomes in patients with BPD that deserve further study.

Disability and physical and communication-related barriers to health care related services among Florida residents: A brief report.

BACKGROUND: Research has not fully characterized barriers to health care faced by persons with disabilities (PWD) which constitutes a critical gap given the increased risk of chronic illness faced by PWD. OBJECTIVE: To understand the current barriers to seeking health care-related services for PWD in Florida. METHODS: The study was based on a random-digit-dial telephone interview survey of respondents aged 18 and over (n = 1429). Multivariable logistic regression assessed the relationship between disability and physical and communication barriers. RESULTS: One thousand four hundred and twenty-nine Florida residents participated in the survey. Thirty-three percent of respondents (n = 471) reported having a disability. PWD were significantly older (mean age 68 vs. 61) and had lower levels of income and education than persons without disabilities (PWOD) (p < 0.05). In adjusted analyses, PWD had significantly higher odds of encountering a physical environment barrier (Odds Ratio [OR] = 16.6 95% CI: 7.9, 34.9), a clinical experience barrier (OR = 13.9 95% CI: 6.9, 27.9) a communication and knowledge barrier (OR = 6.7 95% CI: 4.0, 11.3) and a barrier coordinating care (OR = 5.7 95% CI: 3.4, 9.6) compared to persons without disabilities (PWOD). CONCLUSIONS: PWD disproportionately face health care access difficulties that can impede the receipt of high quality care within and between provider visits. Efforts to reduce physical barriers and improve communication between providers and PWD may improve functional status and quality of life for these patients.

Primary Care Experiences of Emergency Department Patients With Limited Health Literacy.

It is unclear why patients with limited health literacy have fewer visits with a personal doctor and more emergency department (ED) visits than patients with adequate health literacy. We identified significant differences in perceived access to a personal doctor and high-quality provider interactions among adults with limited compared to adequate health literacy presenting for emergency treatment. Practice and provider strategies to ensure that patients have timely access to care and high-quality provider interactions may address some of the reasons patients with limited health literacy use more emergency department-based and less preventive care than those with adequate health literacy.

Decision support for chronic pain care: how do primary care physicians decide when to prescribe opioids? a qualitative study.

BACKGROUND: Primary care physicians struggle to treat chronic noncancer pain while limiting opioid misuse, abuse, and diversion. The objective of this study was to understand how primary care physicians perceive their decisions to prescribe opioids in the context of chronic noncancer pain management. This question is important because interventions, such as decision support tools, must be designed based on a detailed understanding of how clinicians use information to make care decisions. METHODS: We conducted in-depth qualitative interviews with family medicine and general internal medicine physicians until reaching saturation in emergent themes. We used a funneling approach to ask a series of questions about physicians' general decision making challenges and use of information when considering chronic opioids. We then used an iterative, open-coding approach to identify and characterize themes in the data. RESULTS: We interviewed fifteen physicians with diverse clinical experiences, demographics, and practice affiliations. Physicians said that general decision making challenges in providing pain management included weighing risks and benefits of opioid therapies and time and resource constraints. Also, some physicians described their active avoidance of chronic pain treatment due to concerns about opioid risks. In their decision making, physicians described the importance of objective and consistent information, the importance of identifying "red flags" related to risks of opioids, the importance of information about physical function as an outcome, and the importance of information that engenders trust in patients. CONCLUSIONS: This study identified and described primary care physicians' struggles to deliver high quality care as they seek and make decisions based on an array of incomplete, conflicting, and often untrusted patient information. Decision support systems, education, and other interventions that address these challenges may alleviate primary care physicians' struggles and improve outcomes for patients with chronic pain and other challenging conditions.

Visitability surveillance, prevalence, and correlates in Florida.

BACKGROUND: A primary means of social connection is visiting friends and families in their homes. Visitability is designing houses in a way that enables people to visit others' homes regardless of physical limitations or use of mobility assistive devices. OBJECTIVE: The goals of this study were to develop a set of questions about visitability that could be used for surveillance and to assess the prevalence and correlates of visitability features in Florida. METHODS: We added five questions to the 2011 Florida Behavioral Risk Factor Surveillance System (n = 12,399 respondents) and used complementary log-log regression models to estimate the prevalence ratio of each visitability feature. RESULTS: The prevalence of visitability features in Florida homes was high for respondents with and without disabilities, though there was variation by visitability feature. A level entrance to the home and wide doorways were present in most respondents' homes (84.9% and 86.2%, respectively), while a main floor bathroom (59.6%) and a zero-step entrance (45.4%) were reported less commonly. People with a disability were less likely to report that their own home had doorways wide enough to accommodate a wheelchair compared to people without a disability (PR = 0.87, 95% CI: 0.80-0.95). Visitability features were less common in households with lower income and also in trailers or mobile homes than in detached single-family homes. CONCLUSIONS: The survey questions used in this study could be implemented in other states to measure and track visitability and monitor progress toward the Healthy People 2020 goal. Building or retro-fitting homes to include visitability features could increase the participation and inclusion of people with disabilities in community life.

A case-referent study: light at night and breast cancer risk in Georgia.

BACKGROUND: Literature has identified detrimental health effects from the indiscriminate use of artificial nighttime light. We examined the co-distribution of light at night (LAN) and breast cancer (BC) incidence in Georgia, with the goal to contribute to the accumulating evidence that exposure to LAN increases risk of BC. METHODS: Using Georgia Comprehensive Cancer Registry data (2000-2007), we conducted a case-referent study among 34,053 BC cases and 14,458 lung cancer referents. Individuals with lung cancer were used as referents to control for other cancer risk factors that may be associated with elevated LAN, such as air pollution, and since this cancer type was not previously associated with LAN or circadian rhythm disruption. DMSP-OLS Nighttime Light Time Series satellite images (1992-2007) were used to estimate LAN levels; low (0-20 watts per sterradian cm(2)), medium (21-41 watts per sterradian cm(2)), high (>41 watts per sterradian cm(2)). LAN levels were extracted for each year of exposure prior to case/referent diagnosis in ArcGIS. RESULTS: Odds ratios (OR) and 95% confidence intervals (CI) were estimated using logistic regression models controlling for individual-level year of diagnosis, race, age at diagnosis, tumor grade, stage; and population-level determinants including metropolitan statistical area (MSA) status, births per 1,000 women aged 15-50, percentage of female smokers, MSA population mobility, and percentage of population over 16 in the labor force. We found that overall BC incidence was associated with high LAN exposure (OR = 1.12, 95% CI [1.04, 1.20]). When stratified by race, LAN exposure was associated with increased BC risk among whites (OR = 1.13, 95% CI [1.05, 1.22]), but not among blacks (OR = 1.02, 95% CI [0.82, 1.28]). CONCLUSIONS: Our results suggest positive associations between LAN and BC incidence, especially among whites. The consistency of our findings with previous studies suggests that there could be fundamental biological links between exposure to artificial LAN and increased BC incidence, although additional research using exposure metrics at the individual level is required to confirm or refute these findings.

Prostate cancer incidence and tumor severity in Georgia: descriptive epidemiology, racial disparity, and geographic trends.

PURPOSE: Limited research has been conducted to describe the geographical clustering and distribution of prostate cancer (PrCA) incidence in Georgia (GA). This study describes and compares the temporal and geographic trends of PrCA incidence in GA with a specific focus on racial disparities. METHODS: GA Comprehensive Cancer Registry PrCA incidence data were obtained for 1998-2008. Directly standardized age-adjusted PrCA incidence rates per 100,000 were analyzed by race, stage, grade, and county. County-level hotspots of PrCA incidence were analyzed with the Getis-Ord Gi* statistic in a geographic information system; a census tract-level cluster analysis was performed with a Discrete Poisson model and implemented in SaTScan(®) software. RESULTS: Significant (p < 0.05) hotspots of PrCA incidence were observed in nine southwestern counties and six centrally located counties among men of both races. Six significant (p < 0.1) clusters of PrCA incidence rates were detected for men of both races in north and northwest central Georgia. When stratified by race, clusters among white and black men were similar, although centroids were slightly shifted. Most notably, a large (122 km radius) cluster in northwest central Georgia was detected only in whites, and two smaller clusters (0-32 km radii) were detected in Southwest Georgia only in black men. Clusters of high-grade and late-stage tumors were identified primarily in the northern portion of the state among men of both races. CONCLUSIONS: This study revealed a pattern of higher incidence and more advanced disease in northern and northwest central Georgia, highlighting geographic patterns that need more research and investigation of possible environmental determinants.