Factors Associated With Palliative Care Birth Planning at a Pediatric Hospital.

OBJECTIVE: To identify factors associated with the receipt, completion, and goals of palliative care birth plans during the prenatal period. DESIGN: Retrospective observational study of medical record data. SETTING: Midwestern U.S. quaternary pediatric hospital. PARTICIPANTS: Maternal-fetal dyads who received maternal-fetal medicine and palliative care from July 2016 through June 2021 (N = 128). METHODS: Using demographic and clinical predictors, we performed descriptive statistics, group comparisons (chi-square or Fisher exact test and Wilcoxon rank sum test or Student t test), and logistic regression for three outcomes: birth plan offered, birth plan completed, and goals of care (comfort-focused vs. other). RESULTS: Of 128 dyads, 60% (n = 77) received birth plans, 30% (n = 23) completed them, and 31% (n = 40) expressed comfort-focused goals. Participants with comfort-focused goals compared to other goals were more likely to receive birth plans, odds ratio (OR) = 7.20, 95% confidence interval (CI) [1.73, 29.9], p = .01. Participants of non-Black minority races had lower odds of being offered birth plans when compared to White participants, OR = 0.11, 95% CI [0.02, 0.68], p = .02. Odds of being offered (OR = 11.54, 95% CI [2.12, 62.81], p = .005) and completing (OR = 4.37, 95% CI [1.71, 11.17], p < .001) the birth plan increased with each prenatal palliative care visit. Compared to those without, those with neurological (OR = 9.32, 95% CI [2.60, 33.38], p < .001) and genetic (OR = 4.21, 95% CI [1.04, 17.06], p = .04) diagnoses had increased odds of comfort-focused goals. CONCLUSION: Quality improvement efforts should address variation in the frequency at which birth plans are offered. Increasing palliative care follow-up may improve completion of the birth plan.

Safety and efficacy of waterproof casting for early onset scoliosis.

PURPOSE: The efficacy of traditional Mehta casting in the treatment of early onset scoliosis (EOS) is well-established. However, waterproof casting has not been previously described. Inherent advantages of waterproof casting include clearance for bathing/swimming, avoiding cast holidays, and improved family satisfaction. The purpose of this study was to assess the safety and efficacy of waterproof serial casting at controlling curve progression in EOS. METHODS: The current study is an IRB-approved Level IV retrospective consecutive cohort of EOS patients who underwent a serial 75% body weight traction-elongation-flexion Mehta cast protocol with waterproof cast padding. The addition of 3-point apical translation with stockinettes was utilized during casting. Bracing was initiated after correction < 15° or 1 year of serial casting. RESULTS: Seventeen patients at mean age 21.6 months, with pre-cast Cobb angle 52.3° (R: 35°-82°), underwent serial waterproof casting. In-cast correction index was 64%; for post-cast, Cobb angle was 18.6°. At mean 5.6 years follow-up (R: 2.3-8.9 years), 82% successfully avoided surgical intervention, 53% maintained correction < 25°, and 29% are considered "cured". 3/17 (18%) underwent a 2nd round of casting, and a total of 3/17 (18%) ultimately required surgery at 6.2 years post-casting. No major cast-related complications, decubiti, or cast holidays were encountered. CONCLUSION: Serial waterproof casting is safe and efficacious in EOS when compared to published results of traditional Mehta casting. Of 17 patients with mean pre-cast Cobb 52.3°, 82% successfully avoided surgery and 53% maintained mild curves < 25° magnitude at 5.6 years follow-up. No major complications or skin decubiti occurred, and advantages include clearance for bathing and avoidance of need for cast holidays during treatment. LEVEL OF EVIDENCE: Level IV.

Waterproof Hip Spica Casts for Pediatric Femur Fractures.

BACKGROUND: Hip spica casting is the treatment of choice for femur fractures in children ages 6 months to 5 years. Traditional spica (TS) casting utilizes cotton padding that precludes patient bathing. Waterproof (WP) casting has inherent advantages, including clearance for bathing and improved family satisfaction. This study examines the safety and efficacy of WP hip spica casting for the treatment of pediatric femur fractures. METHODS: This is a retrospective, matched cohort study of patients ≤5 years with a femur fracture treated with hip spica casting. Patients with WP casts were matched to patients with TS casts by age, sex, and fracture type. TS casts utilize a Goretex liner and cotton padding, while WP spicas utilize fully WP materials and can be completely submerged in water. RESULTS: Fifty patients were included (25 WP, 25 TS) without differences between cohorts in age, weight, or sex. There were no differences in operative time, length of stay, or length of time in cast. Patient charges were significantly lower in the WP group ($230 vs. $301, P <0.001). At cast-off, coronal/sagittal alignment and shortening were similar, while 9 TS patients had minor skin and/or cast complications that required outpatient repair versus only 1 WP patient ( P <0.001). CONCLUSIONS: In a matched comparison, WP spica casting significantly reduces skin and cast complications traditionally associated with cotton-based spica casting, with significantly lower charges for WP cast materials. Fracture healing rate, alignment, and shortening at cast-off are similar in WP versus TS casts. WP spica casting is safe and efficacious for pediatric femur fractures, with the inherent advantage of clearance for bathing. LEVEL OF EVIDENCE: Level 3.

Communication Training for Pediatric Hospitalists and its Impact on Clinical Practice with Families Using Languages Other than English.

OBJECTIVE: Providing equitable health care for children of families speaking Language Other than English (LOE) relies on linguistic services, including interpretation and translation. Inadequate education on effectively utilizing linguistic services can lead to a knowledge gap and subsequent challenges in leveraging these services. This study aims to evaluate the educational training provided to pediatric hospitalists and its association with clinical practice. METHODS: A multicenter cross-sectional survey of pediatric hospitalists was conducted through the Pediatric Research in Inpatient Setting (PRIS) network, a hospital-based independent research network. The survey was distributed to PRIS site leads with one response per institution. Associations between educational training received on proper communication with families speaking LOE and practice behaviors were analyzed using chi-square or Fisher's exact tests. RESULTS: We received responses from 72 out of 112 PRIS hospital site-leads. Among the respondents, 56% did not receive training on communication during their Graduate Medical Education years (residency or fellowship); 47% did not receive training at their current workplace. Only 6% of those receiving workplace training reported annual updates or refresher courses. Furthermore, 26% of respondents rated the training as "good," while 35% considered it "fair." Respondents who received training during Graduate Medical Education years and at their workplace reported that they asserted/advocated more on behalf of patients speaking LOE than those who did not (P < .01). CONCLUSION: Pediatric hospitalists lack adequate training to communicate effectively with families speaking LOE. Increased training is associated with increased advocacy. Further work should explore increasing communication training and its effect on patient outcomes.

Linguistic Services for Hospitalized Children With Non-English Language Preference: A PRIS Network Survey.

BACKGROUND AND OBJECTIVES: Linguistic services, including verbal interpretation and written translation, are critical to providing equitable health care for families with non-English language preference (NELP). Despite evidence of provider disuse and misuse of linguistic services and resultant adverse outcomes, few studies have assessed the practices of pediatric hospitalists related to the use of linguistic services. Our objectives were to evaluate the current practices of communication and linguistic services used by pediatric hospitalists for hospitalized children with NELP and the barriers encountered in their use. METHODS: We conducted a multicenter cross-sectional survey of pediatric hospitalist site leaders through the Pediatric Research in Inpatient Setting network, an independent, hospital-based research network. The survey was created through an iterative process and underwent a face validation process with hospitalists and a survey methodology expert. RESULTS: We received responses from 72 out of 112 hospitalists (64%). Interpreter services were available widely; translation services were available in 49% of institutions. Difficulty accessing the services timely was reported as the most common barrier. Among respondents, 64% "strongly agree" or "somewhat agree" that they visit and give updates more frequently to English-speaking families than to NELP. Hospitalists reported using interpreter services "always" during 65% of admissions, 57% of discharges, and 40% on rounds. CONCLUSIONS: Families with NELP do not receive appropriate linguistic care when hospitalized. Providers update English-speaking families more frequently than non-English speaking families. Future directions include optimizing workflow to reduce the time constraints on hospitalists and increasing the timeliness and quality of interpreters and translators.

Current state and practice variation in the use of Meningitis/Encephalitis (ME) FilmArray panel in children.

BACKGROUND: The Meningitis/Encephalitis FilmArray® Panel (ME panel) was approved by the U.S. Food and Drug Administration in 2015 and provides rapid results when assessing patients with suspected meningitis or encephalitis. These patients are evaluated by various subspecialties including pediatric hospital medicine (PHM), pediatric emergency medicine (PEM), pediatric infectious diseases, and pediatric intensive care unit (PICU) physicians. The objective of this study was to evaluate the current use of the ME panel and describe the provider and subspecialty practice variation. METHODS: We conducted an online cross-sectional survey via the American Academy of Pediatrics Section of Hospital Medicine (AAP-SOHM) ListServe, Brown University PEM ListServe, and PICU Virtual pediatric system (VPS) Listserve. RESULTS: A total of 335 participants out of an estimated 6998 ListServe subscribers responded to the survey. 68% reported currently using the ME panel at their institutions. Among test users, most reported not having institutional guidelines on test indications (75%) or interpretation (76%). 58% of providers self-reported lack of knowledge of the test's performance characteristics. Providers from institutions that have established guidelines reported higher knowledge compared to those that did not (51% vs. 38%; p = 0.01). More PHM providers reported awareness of ME panel performance characteristics compared to PEM physicians (48% vs. 27%; p = 0.004); confidence in test interpretation was similar between both groups (72 vs. 69%; p = 0.80). CONCLUSION: Despite the widespread use of the ME panel, few providers report having institutional guidelines on test indications or interpretation. There is an opportunity to provide knowledge and guidance about the ME panel among various pediatric subspecialties.

Glycogen storage disease type 1a in the Ohio Amish.

Glycogen storage disease type 1a (GSD1a) is an inborn error of glucose metabolism characterized by fasting hypoglycemia, hepatomegaly, and growth failure. Late complications include nephropathy and hepatic adenomas. We conducted a retrospective observational study on a cohort of Amish patients with GSD1a. A total of 15 patients cared for at a single center, with a median age of 9.9 years (range 0.25-24 years) were included. All patients shared the same founder variant in GCPC c.1039 C > T. The phenotype of this cohort demonstrated good metabolic control with median cohort triglyceride level slightly above normal, no need for continuous overnight feeds, and a higher quality of life compared to a previous GSD cohort. The most frequent complications were oral aversion, gross motor delay, and renal hyperfiltration. We discuss our unique care delivery at a single center that cares for Amish patients with inherited disorders.

Bolus gastric feeds improve nutrition delivery to mechanically ventilated pediatric medical patients: Results of the COntinuous vs BOlus multicenter trial.

BACKGROUND: Comparison of bolus gastric feeding (BGF) vs continuous gastric feeding (CGF) with respect to timing and delivery of energy and protein in mechanically ventilated (MV) pediatric patients has not been investigated. We hypothesized that bolus delivery would shorten time to goal nutrition and increase the percentage of goal feeds delivered. METHODS: Multicenter, prospective, randomized comparative effectiveness trial conducted in seven pediatric intensive care units (PICUs). Eligibility criteria included patients aged 1 month to 12 years who were intubated within 24 h of PICU admission, with expected duration of ventilation at least 48 h, and who were eligible to begin enteral nutrition within 48 h. Exclusion criteria included patients with acute or chronic gastrointestinal pathology or acute surgery. RESULTS: We enrolled 158 MV children between October 2015 and April 2018; 147 patients were included in the analysis (BGF = 72, CGF = 75). Children in the BGF group were slightly older than those in the CGF; otherwise, the two groups had similar demographic characteristics. There was no difference in the percentage of patients in each group who achieved goal feeds. Time to goal feeds was shorter in the BGF group (hazard ratio 1.5 [CI 1.02-2.33]; P = 0.0387). Median percentage of target kilocalories (median kcal 0.78 vs 0.59; P ≤ 0.0001) and median percentage of protein delivered (median protein 0.77 vs 0.59; P ≤ 0.0001) was higher for BGF patients. There was no difference in serial oxygen saturation index between groups. CONCLUSION: Our study demonstrated shorter time to achieve goal nutrition via BGF compared with CGF in MV pediatric patients. This resulted in increased delivery of target energy and nutrition. Further study is needed in other PICU populations.

Vaccination patterns of the northeast Ohio Amish revisited.

OBJECTIVES: The Holmes County Amish have low vaccination rates, an increasingly diverse population, and have an increased incidence of certain inherited diseases. The objectives were to evaluate; the rate and influences of vaccine hesitancy compared to a decade ago, vaccination patterns between Amish affiliations, vaccine practices of Amish special needs children, and the Amish's acceptance of a COVID-19 vaccine. STUDY DESIGN: In April of 2020, a survey assessing vaccination patterns and beliefs were mailed to 1000 Amish families, including ultra-conservative Amish sects and special needs families. RESULTS: The response rate was 39%. Among 391 respondents, 59% did not vaccinate their children, compared to only 14% that refused all vaccinations reported by Wenger et al in the same community only a decade ago. The ultra-conservative Amish rejected vaccines more often. Amish special needs children were more likely to receive vaccines than healthy Amish children. 75% responded they would reject a COVID-19 vaccine. Fear of adverse effects was the most common reason to reject vaccines. Families that accepted vaccines were more likely to cite a healthcare worker as the primary influence to vaccinate. Wives were more likely to cite their spouse as the primary influence to vaccinate. Families that rejected vaccines were more likely to state their bishop was the most influential person on vaccination. CONCLUSION: The Holmes County Amish have decreasing vaccine acceptance. Efforts to improve vaccination will require a targeted focus on the primary influences and beliefs of sub-populations within the Amish. Physician advocacy, peer mentorship, father-directed education, and close partnership with Church leadership will be needed to limit vaccine-preventable disease. The Amish may be at risk for low uptake of a COVID-19 vaccine.

Vaccine Hesitancy and Low Immunization Rates in Children with Down Syndrome.

OBJECTIVES: To determine the prevalence of vaccine hesitancy and refusal among parents of children with Down syndrome and to determine how well the Parent Attitudes about Childhood Vaccines Survey (PACV) is associated with vaccine receipt among children with Down syndrome. STUDY DESIGN: We mailed the PACV to parents of children with Down syndrome who attend the Down Syndrome Clinic at Akron Children's Hospital and examined associations between PACV scores and immunization status at 19 months of age. RESULTS: Of 120 surveys sent, 63 parents completed the PACV (52% response rate) of which 60 were linked to vaccination records. Of these 60 respondents, 55 children were ≥19 months old. PACV scores were significantly correlated with days of underimmunization at 19 months of age. All parents who refused all vaccines had PACV scores of ≥50. Only 58% of children were up to date for the combined 7 vaccine series at 19 months of age. CONCLUSIONS: The PACV may be a valuable tool to identify vaccine hesitancy among parents of young children with Down syndrome. Special emphasis is needed to increase adherence with on-time vaccine recommendations for children with Down syndrome to optimize their health and to potentially avoid hospitalizations.

Abnormal Newborn Screening Follow-up for Severe Combined Immunodeficiency in an Amish Cohort with Cartilage-Hair Hypoplasia.

Cartilage-hair hypoplasia (CHH) is an autosomal recessive, short limb skeletal dysplasia with a variable immunologic phenotype. The spectrum of immune function ranges from clinically normal to severe combined immunodeficiency (SCID). Multiple studies have shown that abnormal immune parameters may not predict severe outcomes. Newborn screening (NBS) using T cell receptor excision circle (TREC) assay can now effectively identify infants with severe T cell deficiency who are at risk for SCID. NBS has allowed for cost-effective identification of patients with SCID and improved outcomes with hematopoietic stem cell transplant (HSCT). Ohio reports two abnormal TREC results: decreased and absent TREC. This study evaluated the laboratory and clinical differences in eight Amish patients with CHH with an abnormal TREC result on the NBS. There were four patients with absent TREC and four patients with decreased TREC. The absent TREC patients had lower CD3, CD4, naïve CD4, CD8 cells, and phytohemagglutinin (PHA)-induced lymphocyte proliferation. Three patients with absent TREC were diagnosed with SCID and two underwent successful HSCT. Patients with absent TREC experienced more CHH-related morbidity including anemia requiring transfusion, Hirschsprung's disease, and failure to thrive. No patients with decreased TREC required HSCT. Our study indicates that CHH patients with absent TREC tend to have more severe immunological and clinical phenotype than patients with decreased TREC. Confirmation of these trends in a larger group would guide providers and parents in a timely referral for HSCT, or cost-effective surveillance monitoring of children with a life-threatening illness.

Evaluating the Effect of Flow and Interface Type on Pressures Delivered With Bubble CPAP in a Simulated Model.

BACKGROUND: Bubble CPAP, used for spontaneously breathing infants to avoid intubation or postextubation support, can be delivered with different interface types. This study compared the effect that interfaces had on CPAP delivery. We hypothesized that there would be no difference between set and measured levels between interface types. METHODS: A validated preterm infant nasal airway model was attached to the ASL 5000 breathing simulator. The simulator was programmed to deliver active breathing of a surfactant-deficient premature infant with breathing frequency at 70 breaths/min inspiratory time of 0.30 s, resistance of 150 cm H2O/L/s, compliance of 0.5 mL/cm H2O, tidal volume of 5 mL, and esophageal pressure of -10 cm H2O. Nasal CPAP prongs, size 4030, newborn and infant RAM cannulas were connected to a nasal airway model and a bubble CPAP system. CPAP levels were set at 4, 5, 6, 7, 8, and 9 cm H2O with flows of 6, 8, and 10 L/min each. Measurements were recorded after 1 min of stabilization. The analysis was performed using SAS 9.4. The Kolmogorov-Smirnov test assessed normality of the data. The Friedman test was used to compare non-normally distributed repeated measures. The Wilcoxon signed-rank test was used to conduct post hoc analysis. All tests were 2-sided, and P values of <.05 were considered as indicating significant differences unless otherwise indicated. RESULTS: At lower set CPAP levels, 4-6 cm H2O, measured CPAP dropped precipitously with the nasal prongs with the highest flow setting. At higher CPAP levels, 7-9 cm H2O measured CPAP concomitantly increased as the flow setting increased. Statistically significant differences in set and measured CPAP occurred for all devices across all CPAP levels, with the measured CPAP less than set for all conditions, P < .001. CONCLUSIONS: Set flow had a profound effect on measured CPAP. The concomitant drop in measured pressure with high and low flows could be attributed to increased resistance to spontaneous breathing or insufficient flow to meet inspiratory demand. Clinicians should be aware of the effect that the interface and flow have on CPAP delivery.