Implementation of the International Consortium of Health Outcomes Measurement CHD standard set in patients undergoing pulmonary valve replacement.

BACKGROUND: Despite the burden of CHD, a high cost and utilization condition, an implementation of long-term outcome measures is lacking. The objective of this study is to pilot the implementation of the International Consortium of Health Outcomes Measurement CHD standard set in patients undergoing pulmonary valve replacement, a procedure performed in mostly well patients with diverse CHD. METHODS: Patients ≥ 8 years old undergoing catheterization-based pulmonary valve replacement were approached via various approaches for patient-reported outcomes, with a follow-up assessment at 3 months post-procedure. Implementation strategy analysis was performed via a hybrid type 2 design. RESULTS: Of the 74 patients undergoing pulmonary valve replacement, 32 completed initial patient-reported outcomes with variable response rates by strategy (email and in-person explanation 100%, email only 54%, and email followed by text/call 64%). Ages ranged 8-67 years (mean 30). Pre-procedurally, 34% had symptomatic arrhythmias, which improved post-procedure. For those in school, 43% missed ≥ 6 days per year, and over half had work absenteeism. Financial concerns were reported in 34%. Patients reported high satisfaction with life (50% [n = 16]) and health-related quality of life (90% [n = 26]). Depression symptoms were reported in 84% (n = 27) and anxiety in 62.5% (n = 18), with tendency towards improvement post-procedurally. CONCLUSION: Pilot implementation of the International Consortium of Health Outcomes Measurement CHD standard set in pulmonary valve replacement patients reveals a significant burden of disease not previously reported. Barriers to the implementation include a sustainable, automated system for patient-reported outcome collection and infrastructure to assess in real time. This provides an example of implementing cardiac outcomes set in clinical practice.

The experience of fathers of children hospitalised with advanced heart disease.

BACKGROUND: There are little reported data on the perspectives of fathers caring for children with chronic conditions. Although survival of children with advanced heart disease has improved, long-term morbidity remains high. This study describes the experience and prognostic awareness of fathers of hospitalised children with advanced heart disease. METHODS: Cross-sectional survey study of parents caring for children hospitalised with advanced heart disease admitted for ≥ 7 days over a one-year period. One parent per patient completed surveys, resulting in 27 father surveys. Data were analysed using descriptive methods. RESULTS: Nearly all (96%) of the fathers reported understanding their child's prognosis "extremely well" or "well," and 59% felt they were "very prepared" for their child's medical problems. However, 58% of fathers wanted to know more about prognosis, and 22% thought their child's team knew something about prognosis that they did not. Forty-one per cent of fathers did not think that their child would have lifelong limitations, and 32% anticipated normal life expectancies. All 13 fathers who had a clinical discussion of what would happen if their child got sicker found this conversation helpful. Nearly half (43%) of the fathers receiving new prognostic information or changes to treatment course found it "somewhat" or "a little" confusing. CONCLUSIONS: Fathers report excellent understanding of their child's illness and a positive experience around expressing their hopes and fears. Despite this, there remain many opportunities to improve communication, prognostic awareness, and participation in informed decision-making of fathers of children hospitalised with advanced heart disease.

The family burden of paediatric heart disease during the chronic phase of illness.

BACKGROUND: CHD is a lifelong condition with a significant burden of disease to patients and families. With increased survival, attention has shifted to longer-term outcomes, with a focus on social determinants of health. Among children with CHD, socioeconomic status is associated with disparities in outcomes. Household material hardship is a concrete measure of poverty and may serve as an intervenable measure of socioeconomic status. METHODS: A longitudinal survey study was conducted at multiple time points (at acute hospitalisation, then 12-24 months later in the chronic phase) to determine the prevalence of household material hardship among parents of children with advanced heart disease and quality of life during long-term follow-up. RESULTS: The analytic cohort was 160 children with a median patient age of 1 year (IQR 1,4) with 54% of patients <2 years. During acute hospitalisation, over one-third of families reported household material hardship (37%), with significantly lower household material hardship in the chronic phase at 16% (N = 9 of 52). For parents reporting household material hardship during acute hospitalisation, 50% had resolution of household material hardship by the chronic phase. Household material hardship-exposed children were significantly more likely to be publicly insured (56% versus 20%, p = 0.03) with lower quality of life than those without household material hardship (64% versus 82%, p = 0.013). CONCLUSION: The burden of heart disease during the chronic phase of illness is high. Household material hardship may serve as a target to ensure equity in the care and outcomes of CHD patients and their families.

Family Outcomes After the Pediatric Intensive Care Unit: A Scoping Review.

BACKGROUND: Intensivists are increasingly attuned to the postdischarge outcomes experienced by families because patient recovery and family outcomes are interdependent after childhood critical illness. In this scoping review of international contemporary literature, we describe the evidence of family effects and functioning postpediatric intensive care unit (PICU) as well as outcome measures used to identify strengths and weaknesses in the literature. METHODS: We reviewed all articles published between 1970 and 2017 in PubMed, Embase, PsycINFO, Cumulative Index of Nursing and Allied Health Literature (CINAHL), or the Cochrane Controlled Trials Registry. Our search used a combination of terms for the concept of "critical care/illness" combined with additional terms for the prespecified domains of social, cognitive, emotional, physical, health-related quality of life (HRQL), and family functioning. RESULTS: We identified 71 articles reporting on the postPICU experience of more than 2400 parents and 3600 families of PICU survivors in 8 countries. These articles used 101 different metrics to assess the various aspects of family outcomes; 34 articles also included open-ended interviews. Overall, most families experienced significant disruption in at least five out of six of our family outcomes subdomains, with themes of decline in mental health, physical health, family cohesion, and family finances identified. Almost all articles represented relatively small, single-center, or disease-specific observational studies. There was a disproportionate representation of families of higher socioeconomic status (SES) and Caucasian race, and there was much more data about mothers compared to fathers. There was also very limited information regarding outcomes for siblings and extended family members after a child's PICU stay. CONCLUSIONS: Significant opportunities remain for research exploring family functioning after PICU discharge. We recommend that future work include more diverse populations with respect to the critically ill child as well as family characteristics, include more intervention studies, and enrich existing knowledge about outcomes for siblings and extended family.

The Surprise Question as a Trigger for Primary Palliative Care Interventions for Children with Advanced Heart Disease.

There is significant uncertainty in describing prognosis and a lack of reliable entry criteria for palliative care studies in children with advanced heart disease (AHD). This study evaluates the utility of the surprise question-"Would you be surprised if this child died within the next year?"-to predict one-year mortality in children with AHD and assess its utility as entry criteria for future trials. This is a prospective cohort study of physicians and nurses caring for children (1 month-19 years) with AHD hospitalized ≥ 7 days. AHD was defined as single ventricle physiology, pulmonary vein stenosis or pulmonary hypertension, or any cardiac diagnosis with signs of advanced disease. Primary physicians were asked the surprise question and medical record review was performed. Forty-nine physicians responded to the surprise question for 152 patients. Physicians responded "No, I would not be surprised if this patient died" for 54 (36%) patients, 20 (37%) of whom died within 1 year, predicting one-year mortality with 77% sensitivity, 73% specificity, 37% positive predictive value, and 94% negative predictive value. Patients who received a "No" response had an increased 1-year risk of death (hazard ratio 7.25, p < 0.001). Physician years of experience, subspecialty, and self-rated competency were not associated with the accuracy of the surprise question. The surprise question offers promise as a bedside screening tool to identify children with AHD at high risk for mortality and help physicians identify patients who may benefit from palliative care and advance care planning discussions.

Thrombotic microangiopathy following heart transplant in pediatric Danon disease.

This case describes an uncommon acute complication of diffuse thrombotic angiopathy and associated aHUS/TTP in an 11-year-old girl with Danon disease who underwent orthotopic heart transplant. Shortly after transplant, despite an uncomplicated operative course, the patient developed severe kidney injury and progressive altered mental status, culminating in cerebral edema, brain herniation, and death. She had received a single dose of tacrolimus (FK506) and a single dose of antithymocyte globulin. Sources of progressive somnolence, including oversedation from impaired renal clearance of opiates, and severe myopathy as has been previously described in Danon disease, were ruled out, and the patient continued to decline. Initial brain CT scan early after transplant showed no signs of cerebral edema, but repeat CT indicated severe cerebral edema. Based on autopsy, diffuse thrombotic angiopathy, with signs of hemolytic anemia with schistocytes, was likely responsible for her deterioration in the broader condition of aHUS/TTP.

An Assessment of Clinical and System Drivers of Family Satisfaction in the PICU.

OBJECTIVE: Investigate clinical and system drivers of family satisfaction in the PICU. DESIGN: Mixed methods qualitative and quantitative (observational) study. Qualitative interviews with families were performed as a pilot to inform modality of survey distribution based on family preferences. A validated pediatric satisfaction survey deployed to family members for 7 months with a corresponding chart review and administrative data collection. SETTING: PICU in a tertiary children's hospital. PATIENTS: Two hundred six families of patients admitted to the PICU more than 48 hours. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Families preferred surveys distributed electronically on a tablet in the PICU setting. The Pediatric Family Satisfaction-ICU survey was used to assess comfort with medical decision-making and communication with the care team. Capture rate of all eligible patients was 69.5% and response rate was 90.8%. Overall, 64.7% of respondents were highly satisfied, whereas over one third were not highly satisfied; families of Hispanic ethnicity (odds ratio of lower satisfaction of families with Hispanic ethnicity: 2.09; 95% CI, 1.01-4.33; p = 0.047) and high social stressors (odds ratio of higher satisfaction among high stressed subgroup: 0.49; 95% CI, 0.24-0.99; p = 0.047) reported statistically significant lower satisfaction. Additional free-text responses were identified in 21% of respondents, with the majority of comments indicating wishes for improvements clustered around communication with the medical team or sleeping environment of families and patients. CONCLUSIONS: High capture rates of family satisfaction in the PICU can be obtained with a PICU-specific survey, limiting barriers to completion by including family preferences, and distributing in the PICU setting. Less than two-third of PICU families are highly satisfied; patients of Hispanic ethnicity and those with high social stressors predict low satisfaction, whereas illness severity, age, and PICU length of stay did not have statistical significance. Local improvement teams can use this approach to drive enhanced satisfaction.

Acute Presentation and Management of the Encephalopathic Child With an Undiagnosed Inborn Error of Metabolism.

BACKGROUND: Inborn errors of metabolism (IEM) commonly present in infancy and, less commonly, later in life. CASE REPORT: This case describes an IEM, specifically, ornithine transcarbamylase deficiency, in a previously healthy 7-year-old boy who presented to an emergency department with vomiting for approximately 24 h prior to admission. The child became progressively encephalopathic while in the emergency department, but an ammonia level was not obtained until several hours after admission. Irreversible brain damage with cerebral edema was already present at time of diagnosis, leading to death. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: This case emphasizes that acute hyperammonemia can rapidly cause irreversible neurological damage and, in the case of a newly encephalopathic pediatric patient, ammonia levels should be evaluated early to facilitate proper diagnostic tests and treatment.

The increasing onset of type 1 diabetes in children.

OBJECTIVE: To identify trends in the recent onset of type 1 diabetes (T1D) in Colorado youth seen at the Barbara Davis Center (BDC) and compare these changes over time. STUDY DESIGN: A retrospective chart review was performed of patients ages 0-20 years at diagnosis of T1D and type 2 diabetes who were seen at the BDC, were living within Colorado at diagnosis, and were seen within 1 month of diagnosis between 1996 and 2010. The review included age of onset, sex, month and season of onset, islet autoantibodies, diabetes type, hemoglobin A1c level, and body mass index. RESULTS: Newly diagnosed youth with diabetes (n = 2841) were seen at the BDC between 1996 and 2010. Of these, 2686 (94.4%) had T1D. The number of newly diagnosed youth increased over the 15 years by 5.71% per year when adjusted for population (P < .0001). When analyzed in 5-year periods, the average number of new onset T1D cases, age-adjusted to the population, increased by 9.46% per year from 1996-2000 to 2001-2005. The increase was only 4.86% per year from 2001-2005 to 2006-2010. Islet autoimmune markers appeared to correlate with changes in T1D new onset cases. CONCLUSION: T1D in youth increased significantly from the late 1990s-2005 and has increased at a lesser rate more recently. Data suggests that even though T1D has increased in all age groups, the greatest increase was in the 5-9 year age category.

Improving Knowledge of Active Safety and QI Projects Amongst Practitioners in a Pediatric ICU.

Introduction: The success of quality improvement (QI) projects depends on many factors, with communication and knowledge of project-specific practice change being fundamental. This project aimed to improve the knowledge of active safety and QI projects. Methods: Two interventions were trialed to improve knowledge: paired email and meeting announcements followed by a daily huddle to review ongoing projects. Knowledge, measured as the ability to recall a project and its practice change, was the primary outcome. The frequency and duration of the Huddle were process and balancing measures, respectively. Results: Seven days after a meeting/email announcement, 3 of 13 (23%) faculty and fellows recalled the announced practice change. Investigators then tested the effects of the Huddle by assessing practitioners' knowledge of safety and QI project-related practice changes on the first and last day of a service week. The average percentage of items recalled increased from the beginning to end of a service week by 33% [46% to 79%, 95% confidence interval (CI) 12-53] for faculty and 27% (51% to 77%, 95% CI 13-40) for fellows. The Huddle occurred in four of seven (interquartile range 2-5) days/wk with a mean duration of 4.5 (SD 2) minutes. Follow-up assessment 2 years after Huddle implementation demonstrate sustained increase in item recall [faculty +36% (95% CI +13% to 40%); fellows +35% (95% CI +23% to 47%)]. Conclusions: A daily huddle to discuss safety and QI project-related practice change is an effective and time-efficient communication method to increase knowledge of active projects.

The relationship between dental anxiety and other kinds of anxiety: a naturalistic, cross-sectional and comparative study.

BACKGROUND: Dental anxiety is of public health importance because it leads to postponed dental treatment, which comes with health complications. The present study investigated whether there is a correlation between the degree of dental anxiety and other kinds of anxiety and whether there are prognostic factors for the different kinds of anxiety. METHOD: In the sample (N = 156) from a dental practice in a large German city, 62% of patients received a check-examination and 38% received dental surgery. The target variables were recorded with validated questionnaires: dental anxiety (IDAF-4c+), subclinical anxiety (SubA), anxiety of negative evaluation (SANB-5), current general anxiety (STAI state), loneliness (LS-S) and self-efficacy (GSW-6). The applied statistics were: t-tests for 31 variables, correlation matrix and multivariate and bivariate regression analyses. RESULTS: The dental surgery patients displayed more dental anxiety and more dental interventions than the check-examination group. The main result was a positive correlation of all kinds of anxiety with each other, a positive correlation of loneliness and neuroticism with all forms of anxiety and a negative correlation between all forms of anxiety and self-efficacy. Especially dental anxiety is positively associated with other kinds of anxiety. In multivariate regression models only neuroticism is associated with dental anxiety, but feelings of loneliness are positively associated with with the other kinds of anxiety assessed in this study. The higher the self-efficacy, the lower the level of general anxiety. CONCLUSIONS: In dentistry, anxiety from negative experiences with buccal interventions should be distinguished from anxiety caused by personality traits. Self-efficacy tends to protect against anxiety, while loneliness and neuroticism are direct or indirect risk factors for anxiety in this urban dentistry sample. Dental anxiety seems to be independent from biographical strains but not from neuroticism. In practice, more attention must be paid to anxiety control, self-management and efforts to improve the confidence of patients with emotional lability, less self-confidence and propensity to shame.

Analyzing clinical and system drivers of satisfaction in the intensive care unit as a component of high quality care.

BACKGROUND: Quality improvement in the intensive care unit has transitioned from focusing on mortality to improving care and reducing morbidity. OBJECTIVE: This study prospectively investigated clinical and system drivers of family satisfaction in a large quaternary hospital ICU. METHODS: A validated tool was distributed to family members and a registry chart analysis was conducted. The aims were to assess associations with high or low family satisfaction to evaluate unit-level satisfaction. Candidate predictors were selected from univariate logistic regressions and finalized in a multivariate model by a stepwise selection approach. RESULTS: Overall, 75% (n = 188) of respondents (n = 250) indicated high satisfaction. Respondents with higher satisfaction had a Plan of the Day posted (OR = 3.3, 95% CI: 1.63, 6.89, p = 0.001), and did not live with the patient (OR =0.5, 95% CI: 0.25, 0.96, p = 0.044). CONCLUSION: This study indicates that communication and transparency of plans contributes to family satisfaction with ICU care.

Parent Preferences for Transparency of Their Child's Hospitalization Costs.

Importance: Health care in the US is often expensive for families; however, there is little transparency in the cost of medical services. The extent to which parents want cost transparency in their children's care is not well characterized. Objective: To explore the preferences and experiences of parents of hospitalized children regarding the discussion and consideration of health care costs in the inpatient care of their children. Design, Setting, and Participants: This cross-sectional multicenter survey study included 6 geographically diverse university-affiliated US children's hospitals from November 3, 2017, to November 8, 2018. Participants included a convenience sample of English- and Spanish-speaking parents of hospitalized children nearing hospital discharge. Data were analyzed from January 1, 2020, to June 25, 2021. Main Outcomes and Measures: Parents' preferences and experiences regarding transparency of their child's health care costs. Multivariable linear regression examined associations between clinical and sociodemographic variables with parents' preferences for knowing, discussing, and considering costs in the clinical setting. Factors included family financial difficulties, child's level of chronic disease, insurance payer, deductible, family poverty level, race, ethnicity, parental educational level, and study site. Results: Of 644 invited participants, 526 (82%) were enrolled (290 [55%] male), of whom 362 (69%) were White individuals, 400 (76%) were non-Hispanic/Latino individuals, and 274 (52%) had children with private insurance. Overall, 397 families (75%) wanted to discuss their child's medical costs, but only 36 (7%) reported having a cost conversation. If cost discussions were to occur, 294 families (56%) would prefer to speak to a financial counselor. Ninety-eight families (19%) worried discussing costs would hurt the quality of their child's care. Families with a medical financial burden unrelated to their hospitalized child had higher mean agreement that their child's physician should consider the family's costs in medical decision-making than families without a medical financial burden (effect size, 0.55 [95% CI, 0.18-0.92]). No variables were consistently associated with cost transparency preferences. Conclusions and Relevance: Most parents want to discuss their child's costs during an acute hospitalization. Discussions of health care costs may be an important, relatively unexplored component of family-centered care. However, these discussions rarely occur, indicating a tremendous opportunity to engage and support families in this issue.

Development of an international standard set of clinical and patient-reported outcomes for children and adults with congenital heart disease: a report from the International Consortium for Health Outcomes Measurement Congenital Heart Disease Working Group.

AIMS: Congenital heart disease (CHD) is the most common congenital malformation. Despite the worldwide burden to patient wellbeing and health system resource utilization, tracking of long-term outcomes is lacking, limiting the delivery and measurement of high-value care. To begin transitioning to value-based healthcare in CHD, the International Consortium for Health Outcomes Measurement aligned an international collaborative of CHD experts, patient representatives, and other stakeholders to construct a standard set of outcomes and risk-adjustment variables that are meaningful to patients. METHODS AND RESULTS: The primary aim was to identify a minimum standard set of outcomes to be used by health systems worldwide. The methodological process included four key steps: (i) develop a working group representative of all CHD stakeholders; (ii) conduct extensive literature reviews to identify scope, outcomes of interest, tools used to measure outcomes, and case-mix adjustment variables; (iii) create the outcome set using a series of multi-round Delphi processes; and (iv) disseminate set worldwide. The Working Group established a 15-item outcome set, incorporating physical, mental, social, and overall health outcomes accompanied by tools for measurement and case-mix adjustment variables. Patients with any CHD diagnoses of all ages are included. Following an open review process, over 80% of patients and providers surveyed agreed with the set in its final form. CONCLUSION: This is the first international development of a stakeholder-informed standard set of outcomes for CHD. It can serve as a first step for a lifespan outcomes measurement approach to guide benchmarking and improvement among health systems.

Financial Difficulties in Families of Hospitalized Children.

BACKGROUND: High costs of hospitalization may contribute to financial difficulties for some families. OBJECTIVE: To examine the prevalence of financial distress and medical financial burden in families of hospitalized children and identify factors that can predict financial difficulties. DESIGN, SETTING, AND PARTICIPANTS: Cross-sectional survey of parents of hospitalized children at six children's hospitals between October 2017 and November 2018. MAIN OUTCOMES AND MEASURES: The outcomes were high financial distress and medical financial burden. Multivariable logistic regression identified predictors of each outcome. The primary predictor variable was level of chronic disease (complex chronic disease, C-CD; noncomplex chronic disease, NC-CD; no chronic disease, no-CD). RESULTS: Of 644 invited participants, 526 (82%) were enrolled, with 125 (24%) experiencing high financial distress, and 160 (30%) reporting medical financial burden. Of those, 86 (54%) indicated their medical financial burden was caused by costs associated with their hospitalized child. Neither C-CD nor NC-CD were associated with high financial distress. Child-related medical financial burden was associated with both C-CD and NC-CD (adjusted odds ratio [AOR], 4.98; 95% CI, 2.41-10.29; and AOR, 2.57; 95% CI, 1.11-5.93), compared to no-CD. Although household poverty level was associated with both measures, financial difficulties occurred in all family income brackets. CONCLUSION: Financial difficulties are common in families of hospitalized children. Low-income families and those who have children with chronic conditions are at particular risk; however, financial difficulties affect all subsets of the pediatric population. Hospitalization may be a prime opportunity to identify and engage families at risk for financial distress and medical financial burden.

Rhabdomyolysis in adolescent athletes: review of cases.

Rhabdomyolysis is a syndrome characterized by muscle pain, weakness and myoglobinuria and ranges in severity from asymptomatic to life threatening with acute kidney failure. While a common condition in adult populations, it is understudied in pediatrics and the majority of adolescent cases are likely exercise-induced, caused by strenuous exercise in athletes. Recently, in our pediatric sports medicine practice, we have seen numerous cases of late adolescent high school athletes who present with severe muscle pain and were found to have elevated creatine kinase levels. The cases review potential contributing factors including characteristics of the workout, use of supplements, caffeine, medication, and metabolic or genetic predisposition. Treatment for exercised-induced rhabdomyolysis rarely requires more than rehydration. Return to play should be progressive, individualized, and include acclimatization and monitoring of hydration status, though guidelines require further review.

Why parents use the emergency department during evening hours for nonemergent pediatric care.

BACKGROUND: Emergency departments (EDs) are commonly used by pediatric patients for nonemergent reasons. There is little information regarding how parents perceive their use of the ED and primary care availability during evening hours. METHODS: We conducted a survey of parental perspectives of ED use during evening hours. Participants were parents of pediatric patients (age 0-18 years) at a large quaternary medical center's ED presenting between 17:00 and 22:00 hours from January 15, 2013 to March 12, 2013. RESULTS: Most patients had a primary care pediatrician (98/102, 96.1%); 80% of their pediatricians (78/98) did not have evening hour availability. Overall, 46.7% of parents would have preferred to go to their primary care pediatrician. CONCLUSIONS: Many parents who expect outpatient treatment prefer to take their child to a primary care pediatrician during evening hours, but present to the ED because of lack of primary care access.