Development of the Parental Experience with Care for Children with Serious Illnesses (PRECIOUS) quality of care measure.

BACKGROUND: Parent-reported experience measures are part of pediatric Quality of Care (QoC) assessments. However, existing measures were not developed for use across multiple healthcare settings or throughout the illness trajectory of seriously ill children. Formative work involving in-depth interviews with parents of children with serious illnesses generated 66 draft items describing key QoC processes. Our present aim is to develop a comprehensive parent-reported experience measure of QoC for children with serious illnesses and evaluate its content validity and feasibility. METHODS: For evaluating content validity, we conducted a three-round Delphi expert panel review with 24 multi-disciplinary experts. Next, we pre-tested the items and instructions with 12 parents via cognitive interviews to refine clarity and understandability. Finally, we pilot-tested the full measure with 30 parents using self-administered online surveys to finalize the structure and content. RESULTS: The Delphi expert panel review reached consensus on 68 items. Pre-testing with parents of seriously ill children led to consolidation of some items. Pilot-testing supported feasibility of the measure, resulting in a comprehensive measure comprising 56 process assessment items, categorized under ten subthemes and four themes: (1) Professional qualities of healthcare workers, (2) Supporting parent-caregivers, (3) Collaborative and holistic care, and (4) Efficient healthcare structures and standards. We named this measure the PaRental Experience with care for Children with serIOUS illnesses (PRECIOUS). CONCLUSIONS: PRECIOUS is the first comprehensive measure and has the potential to standardize assessment of QoC for seriously ill children from parental perspectives. PRECIOUS allows for QoC process evaluation across contexts (such as geographic location or care setting), different healthcare workers, and over the illness trajectory for children suffering from a range of serious illnesses.

PRECIOUS demonstrated satisfactory measurement properties for assessing the quality of care for children with serious illnesses.

OBJECTIVES: To determine the measurement properties of PaRental Experience with care for Children with serIOUS illnesses (PRECIOUS), a parent-reported measure of Quality of Care for seriously ill children across care settings and illness trajectories. STUDY DESIGN AND SETTING: Parents self-administered baseline and 2-week follow-up surveys online. Exploratory Factor Analysis was used to determine PRECIOUS's factor structure and select items. Internal consistency was evaluated with Cronbach's α, test-retest reliability with intraclass correlation coefficients, and convergent validity with Spearman's correlations between PRECIOUS scales and subscales of Measure of Processes of Care and Quality of Children's Palliative Care Instrument. RESULTS: Of 152 parents [108 (71%) mothers, 44 (29%) fathers] who completed the baseline survey, 123 (81%) completed follow-up. Exploratory Factor Analysis grouped PRECIOUS into five scales: collaborative and goal-concordant care (12 items), caregiver support and respectful care (15 items), access to financial and medical resources (five items), reducing caregiving stressors (nine items), and hospitalization-specific processes (four items). Root Mean Square Error of Approximation was 0.040 and Comparative Fit Index was 0.980. Cronbach's α ranged from 0.85 to 0.96. Intraclass correlation coefficients ranged from 0.72 to 0.86. Significant correlations with Measure of Processes of Care and Quality of Children's Palliative Care Instrument confirmed convergent validity. The original 56-item tool was reduced to 45 items. CONCLUSION: PRECIOUS demonstrates satisfactory measurement properties for assessing Quality of Care for seriously ill children.

A qualitative exploration of parental perspectives on quality of care for children with serious illnesses.

Introduction: Being responsive to end-users is essential to good care. Limited in-depth exploration of parental perspectives on care received by children over the course of serious illness has hindered the development of process measures to evaluate quality of care. Our objective was to identify the key process indicators prioritized by parents in the care of seriously ill young children and develop a framework to guide assessment of quality of care. Methods: This qualitative study followed Charmaz's Constructivist Grounded Theory. In-depth semi-structured interviews were conducted with parents of young children with serious illness in Singapore. Participants were sampled across various healthcare settings, children's ages, and illness categories. Theoretical sampling and constant comparative analysis were used to generate initial, focused, and theoretical codes, which informed construction of a conceptual framework. Results: 31 parents participated from July 2021 to February 2022. Initial and focused coding generated 64 quality of care indicators describing key care practices, interactions, and procedures. Indicators were categorized under four themes: (1) efficient healthcare structures and standards, (2) professional qualities of healthcare workers, 3. supporting parent-caregivers, and 4. collaborative and holistic care. Theoretical coding led to the development of the "PaRental perspectives on qualIty of care for Children with sErious iLlnESSes (PRICELESS)" framework which summarizes elements contributing to the parental perception of quality of care. Discussion: The identified process indicators will facilitate the development of standardised parent-reported measures for assessing service quality and benchmarking among providers. The framework provides overall guidance for conceiving quality improvement initiatives.

Defining Pediatric Chronic Critical Illness: A Scoping Review.

OBJECTIVES: Children with chronic critical illness (CCI) are hypothesized to be a high-risk patient population with persistent multiple organ dysfunction and functional morbidities resulting in recurrent or prolonged critical care; however, it is unclear how CCI should be defined. The aim of this scoping review was to evaluate the existing literature for case definitions of pediatric CCI and case definitions of prolonged PICU admission and to explore the methodologies used to derive these definitions. DATA SOURCES: Four electronic databases (Ovid Medline, Embase, CINAHL, and Web of Science) from inception to March 3, 2021. STUDY SELECTION: We included studies that provided a specific case definition for CCI or prolonged PICU admission. Crowdsourcing was used to screen citations independently and in duplicate. A machine-learning algorithm was developed and validated using 6,284 citations assessed in duplicate by trained crowd reviewers. A hybrid of crowdsourcing and machine-learning methods was used to complete the remaining citation screening. DATA EXTRACTION: We extracted details of case definitions, study demographics, participant characteristics, and outcomes assessed. DATA SYNTHESIS: Sixty-seven studies were included. Twelve studies (18%) provided a definition for CCI that included concepts of PICU length of stay (n = 12), medical complexity or chronic conditions (n = 9), recurrent admissions (n = 9), technology dependence (n = 5), and uncertain prognosis (n = 1). Definitions were commonly referenced from another source (n = 6) or opinion-based (n = 5). The remaining 55 studies (82%) provided a definition for prolonged PICU admission, most frequently greater than or equal to 14 (n = 11) or greater than or equal to 28 days (n = 10). Most of these definitions were derived by investigator opinion (n = 24) or statistical method (n = 18). CONCLUSIONS: Pediatric CCI has been variably defined with regard to the concepts of patient complexity and chronicity of critical illness. A consensus definition is needed to advance this emerging and important area of pediatric critical care research.

Enhancing frontline workforce volunteerism through exploration of motivations and impact during the COVID-19 pandemic.

OBJECTIVES: During a pandemic, healthcare workers are requested to volunteer for potentially high-risk frontline duties outside of usual hospital and primary care clinic settings in order to better serve vulnerable communities. Examination of volunteer motivations and impact of such experiences can drive more effective pandemic response efforts with regards to volunteer recruitment and retention. This study aims to explore the motivational factors underlying healthcare worker volunteerism in COVID-19 pandemic response operations in the community, and to describe the impact of such operations on volunteers, in order to highlight important volunteer recruitment strategies and ensure volunteer sustainability. METHODS: A qualitative phenomenological approach is taken in this study through the use of semi-structured individual interviews of healthcare staff, to provide in-depth exploration of personal experiences. Staff were purposefully sampled to ensure diversity of personal and professional backgrounds. RESULTS: A total of 35 staff, consisting of 17 males and 18 females from medical, nursing, allied health, and administrative backgrounds, who participated in COVID-19 community-based operations in migrant worker dormitories and swab isolation facilities were interviewed. The main motivational factors were volunteer functions of values, understanding and enhancement. Participants were positively impacted on personal and professional levels, including self-growth, societal awareness, as well as skills development in leadership and team management. CONCLUSIONS: Healthcare organizations should consider volunteer functions in matching individual motivations to volunteer opportunities. Additionally, personal narratives and role-modelling by senior staff can serve as useful adjunct strategies to volunteer recruitment, while providing pre-pandemic preparedness, reassurance of safety, support, and recognition.

Epidermolysis bullosa with pyloric atresia associated with compound heterozygous ITGB4 pathogenic variants: Minimal skin involvement but severe mucocutaneous disease.

We report a case of junctional epidermolysis bullosa with pyloric atresia (JEB-PA) with minimal skin involvement but severe protein-losing enteropathy and airway involvement. Genetic analysis revealed heterozygous mutations in the ITGB4 gene encoding integrin ß4 protein. Parental testing confirmed inheritance of frameshift variant (c.794dupC) as maternal and splice site variant (c.1608C>T/p.Cys536Cys) as paternal. Immunofluorescence mapping of her skin revealed a subepidermal blister with decreased and frayed integrin ß4 at both the floor and the roof of the blister, while the intestinal mucosa showed complete absence of integrin ß4. We review the literature and discuss the differential expression of integrins in the skin and gastrointestinal tract, as well as the role of chronic inflammation in the pathogenesis of EB.

The Next Pandemic: Supporting COVID-19 Frontline Doctors Through Film Discussion.

This paper describes an innovative just-in-time health humanities programme to educate and provide support to COVID-19 frontline doctors-in-training. The programme incorporates small-group screening of the Netflix documentary, The Next Pandemic from the Explained series, followed by a one-hour facilitated discussion to explore themes surrounding the current pandemic and its impact on frontline doctors in a tertiary paediatric hospital in Singapore. Themes derived from the film included preparedness, blame, and the impact on healthcare workers and public, which were further discussed to include concerns regarding current local readiness levels given global connectivity, the need for international cooperation, and the effects of blame such as racism and prejudice. The association with culture; the current impact on healthcare workers, physician-patient relationships, and the public including the role of social media, the government and associated public reactions were also explored. These rich discussions demonstrate the pivotal role health humanities has in times of uncertainty such as an emerging infectious disease outbreak by providing timely pandemic education and supporting reflective learning.

Epidemiology of Pediatric Tracheostomy and Risk Factors for Poor Outcomes: An 11-Year Single-Center Experience.

OBJECTIVE: Children with long-term tracheostomies are at higher risk of complications. This study aims to describe the epidemiology, outcomes, and factors associated with successful decannulation in children undergoing tracheostomy. STUDY DESIGN: Case series with chart review. SETTING: Tertiary hospital. SUBJECTS AND METHODS: A retrospective analysis was conducted on pediatric tracheostomies performed from 2006 to 2016. Demographics, preexisting comorbidities, indications for tracheostomy, and pretracheostomy ventilatory requirements were collected. A multivariate regression model with covariates of age, failure to thrive (FTT), and comorbidities was used to identify factors associated with successful decannulation. Secondary outcomes were ventilation and oxygen requirements at hospital discharge, hospital and intensive care unit length of stay, and complications. RESULTS: In total, 105 patients received a tracheostomy at a median age of 8.0 months (interquartile range, 2.0-45.0). The most common indication was anatomic airway obstruction (55 of 105, 52.5%). Forty-four (41.9%) patients had preexisting FTT. In-hospital mortality was 14 of 105 (13.3%). None were directly related to tracheostomy. At discharge, 40 of 91 (44.0%) and 12 of 91 (13.2%) required home mechanical ventilation and supplemental oxygen, respectively. Forty-one (39%) patients underwent successful decannulation at a median 408 days (interquartile range, 170-1153) posttracheostomy. On adjusted analysis, unsuccessful decannulation was more common in patients with FTT and neurologic comorbidities. Postoperative complications were more common in younger patients and those with a longer time to decannulation. CONCLUSION: Neurologic comorbidities and FTT were risk factors for unsuccessful decannulation after pediatric tracheostomy. Nutritional interventions may have a role in improving long-term outcomes following pediatric tracheostomies and should be investigated in future studies.

Ketamine-based procedural sedation and analgesia for botulinum toxin A injections in children with cerebral palsy.

INTRODUCTION: Adequate procedural sedation and analgesia (PSA) is essential to reduce pain and distress for children undergoing intramuscular botulinum toxin (BoNT-A) injections. This study describes our institution's experience with ketamine-based PSA in terms of safety and efficacy in children with cerebral palsy receiving BoNT-A injections. MATERIAL AND METHODS: This is an analysis of ketamine-based PSA for children undergoing BoNT-A injections between January 2000 and October 2014. All patients received PSA according to our institution's sedation protocol. From 2000 to 2012, intravenous ketamine and midazolam PSA was administered. From 2013 onwards, intravenous ketamine was used as a sole agent for PSA. RESULTS: A total of 152 BoNT-A procedures were performed successfully on 87 children. The median age of the children was 5 years 5 months with 9 children younger than 36 months. Ten procedures (6.6%) were associated with acute transient self-limiting side effects: Four developed rashes, three had nausea and vomiting, one child had limb tremors and another child complained of mild headache. One child reported nightmares on the evening of the procedure during the two-week post-procedure review. No child experienced serious adverse events. CONCLUSION: Administration of ketamine-based PSA for intramuscular BoNT-A procedures in children can be both safe and efficacious.

Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome may have a hypothalamus-periaqueductal gray localization.

BACKGROUND: Anatomical localization of the rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome has proved elusive. Most patients had neuroimaging after cardiorespiratory collapse, revealing a range of ischemic lesions. PATIENT DESCRIPTION: A 15-year-old obese boy with an acute febrile encephalopathy had hypoventilation, autonomic dysfunction, visual hallucinations, hyperekplexia, and disordered body temperature, and saltwater regulation. These features describe the ROHHAD syndrome. Cerebrospinal fluid analysis showed pleocytosis, elevated neopterins, and oligoclonal bands, and serology for systemic and antineuronal antibodies was negative. He improved after receiving intravenous steroids, immunoglobulins, and long-term mycophenolate. Screening for neural crest tumors was negative. CONCLUSION: Magnetic resonance imaging of the brain early in his illness showed focal inflammation in the periaqueductal gray matter and hypothalamus. This unique localization explains almost all symptoms of this rare autoimmune encephalitis.