Hydroxyurea to prevent brain injury in children with sickle cell disease (HU Prevent)-A randomized, placebo-controlled phase II feasibility/pilot study.

Central nervous system (CNS) injury is common in sickle cell disease (SCD) and occurs early in life. Hydroxyurea is safe and efficacious for treatment of SCD, but high-quality evidence from randomized trials to estimate its neuroprotective effect is scant. HU Prevent was a randomized (1:1), double-blind, phase II feasibility/pilot trial of dose-escalated hydroxyurea vs. placebo for the primary prevention of CNS injury in children with HbSS or HbS-ß0-thalassemia subtypes of SCD age 12-48 months with normal neurological examination, MRI of the brain, and cerebral blood flow velocity. We hypothesized that hydroxyurea would reduce by 50% the incidence of CNS injury. Two outcomes were compared: primary-a composite of silent cerebral infarction, elevated cerebral blood flow velocity, transient ischemic attack, or stroke; secondary-a weighted score estimating the risk of suffering the consequences of stroke (the Stroke Consequences Risk Score-SCRS), based on the same outcome events. Six participants were randomized to each group. One participant in the hydroxyurea group had a primary outcome vs. four in the placebo group (incidence rate ratio [90% CI] 0.216 [0.009, 1.66], p = .2914) (~80% reduction in the hydroxyurea group). The mean SCRS score was 0.078 (SD 0.174) in the hydroxyurea group, 0.312 (SD 0.174) in the placebo group, p = .072, below the p-value of .10 often used to justify subsequent phase III investigations. Serious adverse events related to study procedures occurred in 3/41 MRIs performed, all related to sedation. These results suggest that hydroxyurea may have profound neuroprotective effect in children with SCD and support a definitive phase III study to encourage the early use of hydroxyurea in all infants with SCD.

Visualizing Opioid-Use Variation in a Pediatric Perioperative Dashboard.

BACKGROUND: Anesthesiologists integrate numerous variables to determine an opioid dose that manages patient nociception and pain while minimizing adverse effects. Clinical dashboards that enable physicians to compare themselves to their peers can reduce unnecessary variation in patient care and improve outcomes. However, due to the complexity of anesthetic dosing decisions, comparative visualizations of opioid-use patterns are complicated by case-mix differences between providers. OBJECTIVES: This single-institution case study describes the development of a pediatric anesthesia dashboard and demonstrates how advanced computational techniques can facilitate nuanced normalization techniques, enabling meaningful comparisons of complex clinical data. METHODS: We engaged perioperative-care stakeholders at a tertiary care pediatric hospital to determine patient and surgical variables relevant to anesthesia decision-making and to identify end-user requirements for an opioid-use visualization tool. Case data were extracted, aggregated, and standardized. We performed multivariable machine learning to identify and understand key variables. We integrated interview findings and computational algorithms into an interactive dashboard with normalized comparisons, followed by an iterative process of improvement and implementation. RESULTS: The dashboard design process identified two mechanisms-interactive data filtration and machine-learning-based normalization-that enable rigorous monitoring of opioid utilization with meaningful case-mix adjustment. When deployed with real data encompassing 24,332 surgical cases, our dashboard identified both high and low opioid-use outliers with associated clinical outcomes data. CONCLUSION: A tool that gives anesthesiologists timely data on their practice patterns while adjusting for case-mix differences empowers physicians to track changes and variation in opioid administration over time. Such a tool can successfully trigger conversation amongst stakeholders in support of continuous improvement efforts. Clinical analytics dashboards can enable physicians to better understand their practice and provide motivation to change behavior, ultimately addressing unnecessary variation in high impact medication use and minimizing adverse effects.

2022 Society of Critical Care Medicine Clinical Practice Guidelines on Prevention and Management of Pain, Agitation, Neuromuscular Blockade, and Delirium in Critically Ill Pediatric Patients With Consideration of the ICU Environment and Early Mobility.

RATIONALE: A guideline that both evaluates current practice and provides recommendations to address sedation, pain, and delirium management with regard for neuromuscular blockade and withdrawal is not currently available. OBJECTIVE: To develop comprehensive clinical practice guidelines for critically ill infants and children, with specific attention to seven domains of care including pain, sedation/agitation, iatrogenic withdrawal, neuromuscular blockade, delirium, PICU environment, and early mobility. DESIGN: The Society of Critical Care Medicine Pediatric Pain, Agitation, Neuromuscular Blockade, and Delirium in critically ill pediatric patients with consideration of the PICU Environment and Early Mobility Guideline Taskforce was comprised of 29 national experts who collaborated from 2009 to 2021 via teleconference and/or e-mail at least monthly for planning, literature review, and guideline development, revision, and approval. The full taskforce gathered annually in-person during the Society of Critical Care Medicine Congress for progress reports and further strategizing with the final face-to-face meeting occurring in February 2020. Throughout this process, the Society of Critical Care Medicine standard operating procedures Manual for Guidelines development was adhered to. METHODS: Taskforce content experts separated into subgroups addressing pain/analgesia, sedation, tolerance/iatrogenic withdrawal, neuromuscular blockade, delirium, PICU environment (family presence and sleep hygiene), and early mobility. Subgroups created descriptive and actionable Population, Intervention, Comparison, and Outcome questions. An experienced medical information specialist developed search strategies to identify relevant literature between January 1990 and January 2020. Subgroups reviewed literature, determined quality of evidence, and formulated recommendations classified as "strong" with "we recommend" or "conditional" with "we suggest." Good practice statements were used when indirect evidence supported benefit with no or minimal risk. Evidence gaps were noted. Initial recommendations were reviewed by each subgroup and revised as deemed necessary prior to being disseminated for voting by the full taskforce. Individuals who had an overt or potential conflict of interest abstained from relevant votes. Expert opinion alone was not used in substitution for a lack of evidence. RESULTS: The Pediatric Pain, Agitation, Neuromuscular Blockade, and Delirium in critically ill pediatric patients with consideration of the PICU Environment and Early Mobility taskforce issued 44 recommendations (14 strong and 30 conditional) and five good practice statements. CONCLUSIONS: The current guidelines represent a comprehensive list of practical clinical recommendations for the assessment, prevention, and management of key aspects for the comprehensive critical care of infants and children. Main areas of focus included 1) need for the routine monitoring of pain, agitation, withdrawal, and delirium using validated tools, 2) enhanced use of protocolized sedation and analgesia, and 3) recognition of the importance of nonpharmacologic interventions for enhancing patient comfort and comprehensive care provision.

Pre-operative fasting times for clear liquids at a tertiary children's hospital; what can be improved?

BACKGROUND: The goal of preoperative fasting is to prevent pulmonary aspiration during general anesthesia. Fasting times are often prolonged leading to patient discomfort and risk for adverse events. This retrospective quality improvement survey evaluated effective nil-per-os (NPO) times and causes for prolonged NPO times with the aim to suggest improvement strategies by a newly founded fasting task force. METHODS: Data from all electronic anesthesia records from 2019 at our institution were reviewed for fasting times. Our NPO instructions follow American Society of Anesthesiology guidelines and are calculated based on the patient's arrival time (90 min before operating room [OR] time). Primary outcome was the effective NPO time for clear liquids, secondary outcomes were incidence of delays and the parental compliance with the NPO instructions. Data are presented as median (interquartile range). RESULTS: 9,625 cases were included in the analysis. NPO time was documented in 72.1% with a median effective NPO time of 7:13 h (7:36). OR in room times were documented in 72.8%, 2,075 (29.5%; median time 0:10 h [0:21]) were earlier and 4,939 (70.5%; median time 0:29 h [0:54]) were later than scheduled. Parental NPO compliance showed a median deviation for clear liquid intake of 0:55 h (8:30). CONCLUSIONS: This study revealed that effective NPO times were longer than current ASA guidelines. Contributing causes include case delays and parental non-compliance to NPO instructions. Thus, task force recommendations include change NPO instruction calculations to scheduled OR time versus arrival time, and encourage parents to give their child clear liquids at the instructed time.

The Current State of Combined Pediatric Anesthesiology-Critical Care Practice: A Survey of Dual-Trained Practitioners in the United States.

BACKGROUND: Combined practice in pediatric anesthesiology (PA) and pediatric critical care medicine (PCCM) was historically common but has declined markedly with time. The reasons for this temporal shift are unclear, but existing evidence suggests that length of training is a barrier to contemporary trainees. Among current practitioners, restriction in dual-specialty practice also occurs, for reasons that are unknown at present. We sought to describe the demographics of this population, investigate their perceptions about the field, and consider factors that lead to attrition. METHODS: We conducted a cross-sectional, observational study of physicians in the United States with a combined practice in PA and PCCM. The survey was distributed electronically and anonymously to the distribution list of the Pediatric Anesthesia Leadership Council (PALC) of the Society for Pediatric Anesthesia (SPA), directing the recipients to forward the link to their faculty meeting our inclusion criteria. Attending-level respondents (n = 62) completed an anonymous, 40-question multidomain survey. RESULTS: Forty-seven men and 15 women, with a median age of 51, completed the survey. Major leadership positions are held by 44%, and 55% are externally funded investigators. A minority (26%) have given up one or both specialties, citing time constraints and politics as the dominant reasons. Duration of training was cited as the major barrier to entry by 77%. Increasing age and faculty rank and lack of a comparably trained institutional colleague were associated with attrition from dual-specialty practice. The majority (88%) reported that they would do it all again. CONCLUSIONS: The current cohort of pediatric anesthesiologist-intensivists in the United States is a small but accomplished group of physicians. Efforts to train, recruit, and retain such providers must address systematic barriers to completion of the requisite training and continued practice.

Development and Usability Testing of the Society for Pediatric Anesthesia Pedi Crisis Mobile Application.

When life-threatening, critical events occur in the operating room, the fast-paced, high-distraction atmosphere often leaves little time to think or deliberate about management options. Success depends on applying a team approach to quickly implement well-rehearsed, systematic, evidence-based assessment and treatment protocols. Mobile devices offer resources for readily accessible, easily updatable information that can be invaluable during perioperative critical events. We developed a mobile device version of the Society for Pediatric Anesthesia 26 Pediatric Crisis paper checklists-the Pedi Crisis 2.0 application-as a resource to support clinician responses to pediatric perioperative life-threatening critical events. Human factors expertise and principles were applied to maximize usability, such as by clustering information into themes that clinicians utilize when accessing cognitive aids during critical events. The electronic environment allowed us to feature optional diagnostic support, optimized navigation, weight-based dosing, critical institution-specific phone numbers pertinent to emergency response, and accessibility for those who want larger font sizes. The design and functionality of the application were optimized for clinician use in real time during actual critical events, and it can also be used for self-study or review. Beta usability testing of the application was conducted with a convenience sample of clinicians at 9 institutions in 2 countries and showed that participants were able to find information quickly and as expected. In addition, clinicians rated the application as slightly above "excellent" overall on an established measure, the Systems Usability Scale, which is a 10-item, widely used and validated Likert scale created to assess usability for a variety of situations. The application can be downloaded, at no cost, for iOS devices from the Apple App Store and for Android devices from the Google Play Store. The processes and principles used in its development are readily applicable to the development of future mobile and electronic applications for the field of anesthesiology.

Pediatric-Oncology Simulation Training for Resident Education.

INTRODUCTION: We sought to evaluate pediatric oncology simulations intended to improve pediatric residents' skills and comfort in caring for children with cancer. METHOD: In a non-randomized trial, controls (the first three rotations) received a standard set of lectures, and the intervention arm received these lectures plus five simulation-training scenarios-fever/neutropenia, a new leukemia diagnosis, end-of-life care discussion, tumor lysis syndrome, and a mediastinal mass. All residents were tested after the rotation on the first three scenarios; management skills were evaluated independently by two raters. Before and after training, all residents completed an emotional-appraisal questionnaire evaluating each scenario as a perceived challenge or threat. Analysis of variance (ANOVA) measured differences by study arm in skills-checklist assessments and appraisals; repeated-measures ANOVA measured changes in emotional-appraisal scores. RESULTS: Forty-two residents (9 control, 33 intervention) participated. Inter-rater agreement for skills-checklist scores using average-measures intraclass correlation was high (0.847), and overall mean scores were significantly higher for the intervention than control group across both raters (P = 0.005). For all residents, perceived challenge increased in the end-of-life simulation, and perceived threat decreased in all three test scenarios. The intervention group, regardless of training year, evaluated the teaching scenarios favorably and felt that challenging oncology situations were addressed, skills were enhanced, and the simulations should be offered to other residents. CONCLUSIONS: It was feasible to introduce residents to difficult pediatric oncology scenarios using simulation. The intervention group performed more skills than controls when tested, and perceive threat declined in all residents after their pediatric oncology rotation.

Massive Fetal Transfusion During an Emergency Ex Utero Intrapartum Treatment for a Congenital Infantile Fibrosarcoma: A Case Report.

Although originally described to assist airway management of fetal congenital malformations with life-threatening airway obstruction, the indications for an ex utero intrapartum treatment (EXIT) have expanded to include surgical resection of lesions that are potentially incompatible with life in the absence of uteroplacental circulatory support. We describe the case of an infantile fibrosarcoma (IFS) that presented with fetal hydrops and was successfully managed with an emergency EXIT that necessitated the initiation of a massive fetal blood transfusion both with and without the support of uteroplacental circulation.

Clinical Practice Guideline: Maintenance Intravenous Fluids in Children.

Maintenance intravenous fluids (IVFs) are used to provide critical supportive care for children who are acutely ill. IVFs are required if sufficient fluids cannot be provided by using enteral administration for reasons such as gastrointestinal illness, respiratory compromise, neurologic impairment, a perioperative state, or being moribund from an acute or chronic illness. Despite the common use of maintenance IVFs, there is high variability in fluid prescribing practices and a lack of guidelines for fluid composition administration and electrolyte monitoring. The administration of hypotonic IVFs has been the standard in pediatrics. Concerns have been raised that this approach results in a high incidence of hyponatremia and that isotonic IVFs could prevent the development of hyponatremia. Our goal in this guideline is to provide an evidence-based approach for choosing the tonicity of maintenance IVFs in most patients from 28 days to 18 years of age who require maintenance IVFs. This guideline applies to children in surgical (postoperative) and medical acute-care settings, including critical care and the general inpatient ward. Patients with neurosurgical disorders, congenital or acquired cardiac disease, hepatic disease, cancer, renal dysfunction, diabetes insipidus, voluminous watery diarrhea, or severe burns; neonates who are younger than 28 days old or in the NICU; and adolescents older than 18 years old are excluded. We specifically address the tonicity of maintenance IVFs in children.The Key Action Statement of the subcommittee is as follows:1A: The American Academy of Pediatrics recommends that patients 28 days to 18 years of age requiring maintenance IVFs should receive isotonic solutions with appropriate potassium chloride and dextrose because they significantly decrease the risk of developing hyponatremia (evidence quality: A; recommendation strength: strong).

The Simulation-Based Assessment of Pediatric Rapid Response Teams.

OBJECTIVE: To create scenarios of simulated decompensating pediatric patients to train pediatric rapid response teams (RRTs) and to determine whether the scenario scores provide a valid assessment of RRT performance with the hypothesis that RRTs led by intensivists-in-training would be better prepared to manage the scenarios than teams led by nurse practitioners. STUDY DESIGN: A set of 10 simulated scenarios was designed for the training and assessment of pediatric RRTs. Pediatric RRTs, comprising a pediatric intensive care unit (PICU) registered nurse and respiratory therapist, led by a PICU intensivist-in-training or a pediatric nurse practitioner, managed 7 simulated acutely decompensating patients. Two raters evaluated the scenario performances and psychometric analyses of the scenarios were performed. RESULTS: The teams readily managed scenarios such as supraventricular tachycardia and opioid overdose but had difficulty with more complicated scenarios such as aortic coarctation or head injury. The management of any particular scenario was reasonably predictive of overall team performance. The teams led by the PICU intensivists-in-training outperformed the teams led by the pediatric nurse practitioners. CONCLUSIONS: Simulation provides a method for RRTs to develop decision-making skills in managing decompensating pediatric patients. The multiple scenario assessment provided a moderately reliable team score. The greater scores achieved by PICU intensivist-in-training-led teams provides some evidence to support the validity of the assessment.

ECMO for Pediatric Lung Transplantation.

Extracorporeal membrane oxygenation (ECMO) has been reported as an alternative to cardiopulmonary bypass during lung transplantation. The reports in the literature have been limited to adult practice and associated with decreased pulmonary and renal complications, lower mortality, and lower in-hospital mortality. We present four pediatric lung transplantations performed on ECMO and discuss relevant perfusion management.

Simulation and the diagnostic process: a pilot study of trauma and rapid response teams.

BACKGROUND: Simulation is frequently used to recreate many of the crises encountered in patient care settings. Teams learn to manage these crises in an environment that maximizes their learning experiences and eliminates the potential for patient harm. By designing simulation scenarios that include conditions associated with diagnostic errors, teams can experience how their decisions can lead to errors. The purpose of this study was to assess how trauma teams (TrT) and pediatric rapid response teams (RRT) managed scenarios that included a diagnostic error. METHODS: We developed four scenarios that would require TrT and pediatric RRT to manage an error in diagnosis. The two trauma scenarios (spinal cord injury and tracheobronchial tear) were designed to not respond to the heuristic management approach frequently used in trauma settings. The two pediatric scenarios (foreign body aspiration and coarctation of the aorta) had an incorrect diagnosis on admission. Two raters independently scored the scenarios using a rating system based on how teams managed the diagnostic process (search, establish and confirm a new diagnosis and initiate therapy based on the new diagnosis). RESULTS: Twenty-one TrT and 17 pediatric rapid response managed 51 scenarios. All of the teams questioned the initial diagnosis. The teams were able to establish and confirm a new diagnosis in 49% of the scenarios (25 of 51). Only 23 (45%) teams changed their management of the patient based on the new diagnosis. CONCLUSIONS: Simulation can be used to recreate conditions that engage teams in the diagnostic process. In contrast to most instruction about diagnostic error, teams learn through realistic experiences and receive timely feedback about their decision-making skills. Based on the findings in this pilot study, the majority of teams would benefit from an education intervention designed to improve their diagnostic skills.

Emergency Information Forms for Children With Medical Complexity: A Simulation Study.

BACKGROUND: Emergency information forms (EIFs) have been proposed to provide critical information for optimal care of children with medical complexity (CMC) during emergencies; however, their impact has not been studied. The objective of this study was to measure the impact and utility of EIFs in simulated scenarios of CMC during medical emergencies. METHODS: Twenty-four providers (12 junior, 12 experienced) performed 4 simulations of CMC, where access to an EIF was block randomized by group. Scenario-specific critical action checklists and consequential pathways were developed by content experts in simulation and pediatric subspecialists. Scenarios ended when all critical actions were completed or after 10 minutes, whichever came first. Two reviewers independently evaluated the video-recorded performances and calculated scenario-specific critical action scores. Performance in scenarios with and without an EIF was compared with Pearson's χ(2) and Mann-Whitney U tests. Interrater reliability was assessed with intraclass correlation. Each provider rated the utility of EIFs via exit questionnaires. RESULTS: The median critical action score in scenarios with EIFs was 84.2% (95% confidence interval [CI], 71.7%-94.1%) versus 12.5% (95% CI, 10.5%-35.3%) in scenarios without an EIF (P < .001); time to completion of scenarios was shorter (6.9 minutes [interquartile range 5.8-10 minutes] vs 10 minutes), and complication rates were lower (30% [95% CI, 17.4%-46.3%] vs 100% [95% CI, 92.2%-100%]) with EIFs, independent of provider experience. Interrater reliability was excellent (intraclass correlation = 0.979). All providers strongly agreed that EIFs can improve clinical outcomes for CMC. CONCLUSIONS: Using simulated scenarios of CMC, providers' performance was superior with an EIF. Clinicians evaluated the utility of EIFs very highly.

Simulation-Based Assessment of ECMO Clinical Specialists.

OBJECTIVE: The aims of the study were (1) to create multiple scenarios that simulate a range of urgent and emergent extracorporeal membrane oxygenation (ECMO) events and (2) to determine whether these scenarios can provide reliable and valid measures of a specialist's advanced skill in managing ECMO emergencies. DESIGN: Multiscenario simulation-based performance assessment was performed. SETTING: The study was conducted in the Saigh Pediatric Simulation Center at St. Louis Children's Hospital. SUBJECTS: ECMO clinical specialists participated in the study. MEASUREMENTS AND MAIN RESULTS: Twenty-five ECMO specialists completed 8 scenarios presenting acute events in simulated ECMO patients. Participants were evaluated by 2 separate reviewers for completion of key actions and for global performance. The scores were highest for the hemodilution scenario, whereas the air entrainment scenario had the lowest scores. Psychometric analysis demonstrated that ECMO specialists with more than 1 year of experience outperformed the specialists with less than 1 year of experience. Participants endorsed these sessions as important and representative of events that might be encountered in practice. CONCLUSIONS: The scenarios could serve as a component of an ECMO education curriculum and be used to assess clinical specialists' readiness to manage ECMO emergencies.

The important role of simulation in sedation.

PURPOSE OF REVIEW: This article reviews the development of simulation-based training strategies to educate sedation providers. RECENT FINDINGS: Medical simulation has been utilized to train and evaluate providers in numerous domains related to sedation. Sedation providers come to the patient with a wide range of clinical training and experience and simulation can serve as a platform for achieving a baseline skill level and for periodic retraining. Although widely accepted by participants in simulation training, data demonstrating simulation's efficacy in improving sedation-related clinical outcomes are lacking. SUMMARY: Simulation provides an opportunity for sedation providers to develop deliberative practice, to consider rare or challenging clinical conditions, and to benefit from directed feedback, in a manner that does not put patients in harm's way.

Decision making in trauma settings: simulation to improve diagnostic skills.

INTRODUCTION: In the setting of acute injury, a wrong, missed, or delayed diagnosis can impact survival. Clinicians rely on pattern recognition and heuristics to rapidly assess injuries, but an overreliance on these approaches can result in a diagnostic error. Simulation has been advocated as a method for practitioners to learn how to recognize the limitations of heuristics and develop better diagnostic skills. The objective of this study was to determine whether simulation could be used to provide teams the experiences in managing scenarios that require the use of heuristic as well as analytic diagnostic skills to effectively recognize and treat potentially life-threatening injuries. METHODS: Ten scenarios were developed to assess the ability of trauma teams to provide initial care to a severely injured patient. Seven standard scenarios simulated severe injuries that once diagnosed could be effectively treated using standard Advanced Trauma Life Support algorithms. Because diagnostic error occurs more commonly in complex clinical settings, 3 complex scenarios required teams to use more advanced diagnostic skills to uncover a coexisting condition and treat the patient. Teams composed of 3 to 5 practitioners were evaluated in the performance of 7 (of 10) randomly selected scenarios (5 standard, 2 complex). Expert rates scored teams using standardized checklists and global scores. RESULTS: Eighty-three surgery, emergency medicine, and anesthesia residents constituted 21 teams. Expert raters were able to reliably score the scenarios. Teams accomplished fewer checklist actions and received lower global scores on the 3 analytic scenarios (73.8% [12.3%] and 5.9 [1.6], respectively) compared with the 7 heuristic scenarios (83.2% [11.7%] and 6.6 [1.3], respectively; P < 0.05 for both). Teams led by more junior residents received higher global scores on the analytic scenarios (6.4 [1.3]) than the more senior team leaders (5.3 [1.7]). CONCLUSIONS: This preliminary study indicates that teams led by more senior residents received higher scores when managing heuristic scenarios but were less effective when managing the scenarios that require a more analytic approach. Simulation can be used to provide teams with decision-making experiences in trauma settings and could be used to improve diagnostic skills as well as study the decision-making process.