Background: Hispanic people with cystic fibrosis (CF) have decreased life expectancy and earlier acquisition of Pseudomonas aeruginosa compared to non-Hispanic white individuals with CF. Racial and ethnic differences in the airway microbiome of CF may contribute to known health disparity, but have not been studied. The objective was to describe differences in the upper airway microbial community in Hispanic and non-Hispanic white children with CF. Methods: This prospective, observational cohort study of fifty-nine Hispanic and non-Hispanic white children with CF, ages 2-10 years old, was performed at Texas Children’s Hospital (TCH) from February 2019 to January 2020. Oropharyngeal swabs were collected from the cohort during clinic visit. Swab samples underwent sequencing (16S V4 rRNA), diversity analysis, and taxonomic profiling. Key demographic and clinical data were collected from the electronic medical record and the Cystic Fibrosis Foundation Patient Registry (CFFPR). Statistical analysis compared sequencing, demographic, and clinical data. Results: We found no significant difference in Shannon diversity or relative abundance of bacterial phyla between Hispanic and non-Hispanic children with CF. However, a low abundant taxa- “uncultured bacterium” belonging to the order Saccharimonadales was significantly higher in Hispanic children (mean relative abundance=0.13%) compared to the non-Hispanic children (0.03%). Hispanic children had increased incidence of Pseudomonas aeruginosa (p=0.045) compared to non-Hispanic children. Conclusion: We did not find a significant difference in the airway microbial diversity between Hispanic and non-Hispanic white children with CF. However, we found a greater relative abundance of Saccharimonadales and higher incidence of Pseudomonas aeruginosa in Hispanic children with CF.

Background Children with chronic lung disease (CLD) of prematurity who require invasive home mechanical ventilation (iHMV) are medically vulnerable and experience high caregiving and healthcare costs. Predictors for duration of iHMV remain unclear, which can make prognostication and decision-making challenging. Methods A retrospective cohort study of children with CLD of prematurity requiring invasive iHMV was conducted from an independent children’s hospital records (2005-2021). The primary outcome was iHMV duration, defined as time from initial discharge home on iHMV until cessation of positive pressure ventilation (day and night). Two new variables were included: corrected tracheostomy age (CTA) (chronological age at discharge minus age at tracheotomy) and level of ventilator support at discharge (minute ventilation per kg per day). Univariable Cox regression was performed with variables of interest compared to iHMV duration. Significant nonlinear factors (P<0.05) were included in the multivariable analysis. Results One-hundred-and-nineteen patients used iHMV primarily for CLD of prematurity. Patient median index hospitalization lasted 12 months (IQR 8.0,14.4). Once home, half of patients were weaned off iHMV by 36.0 months and 90% by 52.2 months. Being Hispanic/Lantix ethnicity (HR 0.14 (95% CI 0.04, 0.53), p<0.01) and having a higher CTA were associated with increased iHMV duration (HR 0.66 (CI 0.43, 0.98), p<0.05). Conclusions Disparity in iHMV duration exists among patients using iHMV after prematurity. Prospective multisite studies that further investigate new analytic variables, such as CTA and level of ventilator support, and address standardization of iHMV care are needed to create more equitable iHMV management strategies.

Introduction: The 2017-2018 National Survey of Children’s Health estimates that 30 million (42%) US children have experienced at least one adverse childhood experience (ACE), including abuse, neglect, and household dysfunction. ACEs negatively impact long-term health, and there has been no study of ACEs in cystic fibrosis (CF). We assessed willingness to disclose ACEs experienced by children with CF by surveying their parents and adults with CF. Methods: We anonymously surveyed parents of children with CF and adults with CF at the Northwestern University/ Lurie Children’s CF Center to determine their willingness to disclose ACEs. Results: The survey was completed by 46/157 (29%) parents and 36/105 (34%) adults with CF. Few parents (22%) and adults (17%) were willing to discuss most or all specific ACEs, more were willing to disclose the number of ACEs experienced in a category (57% parents, 47% adults), and the majority were willing to participate in anonymous research about ACEs (76% parents, 67% adults). Most parents (63%) and adults (50%) would prefer to have ACEs screened separately from their CF appointment, and most parents (63%) and adults (56%) wanted to learn more about ACEs from a member of their care team. Conclusions: Participants preferred to disclose the number of categorical ACEs rather than specific ACEs and most were open to participating in anonymous ACEs research. More research is needed before widespread adoption of ACE screening in CF.