Disease-modifying therapies for sickle cell disease: Decisional needs and supports among adolescents and young adults.

BACKGROUND: Shared decision-making is one promising solution to addressing barriers in use of disease-modifying therapies for adolescents and young adults (AYAs) with sickle cell disease (SCD). A thorough understanding of decisional needs can guide the development of decisional supports and promote shared decision-making. PROCEDURE: Informed by the Ottawa Decision Support Framework (ODSF), we conducted a qualitative analysis to assess decisional needs and supports reported by AYAs with SCD, their caregivers, and healthcare providers. Semi-structured qualitative interviews were conducted with AYAs and their caregivers, and online crowdsourcing was used with SCD providers. Thematic and descriptive content analyses were used to summarize perspectives on decisional needs and supports regarding disease-modifying therapies. RESULTS: Fourteen AYAs (Mage  = 21 years, 57% male, 93% non-Hispanic Black, 79% HbSS), 11 caregivers (80% female, 100% non-Hispanic Black), and 40 healthcare providers (65% female, 65% non-Hispanic White, Myears in practice  = 14.8 years, 75% physicians) participated. Thematic analysis revealed needs related to: decisional conflict, inadequate knowledge, unclear expectations, and inadequate supports and resources. Six forms of support emerged as important for decision-making: establishing an open and trusting patient/family-provider relationship, providing information, accepting ambivalence and unreadiness, supporting implementation of a decision, addressing inadequate health and social services, and promoting adequate social, emotional, and instrumental help. CONCLUSIONS: This is the first study to assess decisional needs and supports for AYAs with SCD considering disease-modifying therapies. Additional research is needed to examine which decision supports are the most impactful to promote effective shared decision-making in this population.

Neurodevelopmental Outcomes for Individuals With Congenital Heart Disease: Updates in Neuroprotection, Risk-Stratification, Evaluation, and Management: A Scientific Statement From the American Heart Association.

Over the past decade, new research has advanced scientific knowledge of neurodevelopmental trajectories, factors that increase neurodevelopmental risk, and neuroprotective strategies for individuals with congenital heart disease. In addition, best practices for evaluation and management of developmental delays and disorders in this high-risk patient population have been formulated based on literature review and expert consensus. This American Heart Association scientific statement serves as an update to the 2012 statement on the evaluation and management of neurodevelopmental outcomes in children with congenital heart disease. It includes revised risk categories for developmental delay or disorder and an updated list of factors that increase neurodevelopmental risk in individuals with congenital heart disease according to current evidence, including genetic predisposition, fetal and perinatal factors, surgical and perioperative factors, socioeconomic disadvantage, and parental psychological distress. It also includes an updated algorithm for referral, evaluation, and management of individuals at high risk. Risk stratification of individuals with congenital heart disease with the updated categories and risk factors will identify a large and growing population of survivors at high risk for developmental delay or disorder and associated impacts across the life span. Critical next steps must include efforts to prevent and mitigate developmental delays and disorders. The goal of this scientific statement is to inform health care professionals caring for patients with congenital heart disease and other key stakeholders about the current state of knowledge of neurodevelopmental outcomes for individuals with congenital heart disease and best practices for neuroprotection, risk stratification, evaluation, and management.

User-Centered Development of HEARTPrep, a Digital Health Psychosocial Intervention for Prenatally Diagnosed Congenital Heart Disease.

User-centered models for the development of digital health interventions are not consistently applied in healthcare settings. This study used a five-phase, user-centered approach to develop HEARTPrep©, a psychosocial intervention delivered via mobile app and telehealth to mothers expecting a baby with congenital heart disease (CHD) to promote maternal, family, and child well-being. Phases of intervention development were: (I) establishing partnerships; (II) creating content; (III) developing prototype and testable intervention; (IV) conducting think-aloud testing; and (V) completing beta testing. Partnerships with parents, clinicians, and design/technology experts were integral throughout the development of HEARTPrep©. Parents of children with CHD also served as participants in Phases II-V, contributing to the creation of content and providing feedback to inform the iterative refinement of HEARTPrep©. These five phases produced a refined digital health intervention with promising feasibility, usability, and acceptability results. This user-centered approach can be used to develop digital health interventions targeting various health outcomes.

Priorities for Well Child Care of Families Affected by Parental Opioid Use Disorder.

BACKGROUND AND OBJECTIVES: Parenting women in treatment for opioid use disorder (OUD) report a lack of family centeredness and anticipatory guidance within well child care (WCC), and WCC utilization is low among affected children. We explore priorities for WCC visit content to inform primary care recommendations for this population. METHODS: This study is a qualitative study of parenting women from one urban, academic OUD treatment program and pediatric primary care clinicians from a nearby affiliated pediatric practice. Eligible parent participants had a child ≤2 years old and were English speaking. Semistructured interviews elicited perspectives on WCC, with questions and prompts related to visit content. Inductive thematic analysis was led by 2 investigators using open coding procedures. RESULTS: Among 30 parent participants, the majority were White (83%) and unmarried (90%). Approximately 60% reported their child received pharmacotherapy for neonatal opioid withdrawal syndrome. Of 13 participating clinicians, 9 were attending pediatricians. Five themes emerged from parental and clinician interviews: (1) improving knowledge and confidence related to child development, behavior, and nutrition; (2) mitigating safety concerns; (3) addressing complex health and subspecialty needs through care coordination; (4) acknowledging parental health and wellbeing in the pediatric encounter; and (5) supporting health education and care related to neonatal opioid withdrawal syndrome. Parents and clinicians expressed difficulty comprehensively addressing such issues due to time constraints, social determinants of health, and significant informational needs. CONCLUSIONS: Parenting women in treatment for OUD and pediatric clinicians share multiple priorities for anticipatory guidance within WCC visits and barriers to addressing them comprehensively.

Development of the data registry for the Cardiac Neurodevelopmental Outcome Collaborative.

Children with congenital heart disease (CHD) can face neurodevelopmental, psychological, and behavioural difficulties beginning in infancy and continuing through adulthood. Despite overall improvements in medical care and a growing focus on neurodevelopmental screening and evaluation in recent years, neurodevelopmental disabilities, delays, and deficits remain a concern. The Cardiac Neurodevelopmental Outcome Collaborative was founded in 2016 with the goal of improving neurodevelopmental outcomes for individuals with CHD and pediatric heart disease. This paper describes the establishment of a centralised clinical data registry to standardize data collection across member institutions of the Cardiac Neurodevelopmental Outcome Collaborative. The goal of this registry is to foster collaboration for large, multi-centre research and quality improvement initiatives that will benefit individuals and families with CHD and improve their quality of life. We describe the components of the registry, initial research projects proposed using data from the registry, and lessons learned in the development of the registry.

Experiences of fetal care, social support, and emotional functioning during the COVID-19 pandemic in women carrying a fetus with CHD.

OBJECTIVE: To explore how the COVID-19 pandemic affected fetal care, social support, and emotional functioning for women carrying a fetus with CHD. METHOD: This was a single-centre qualitative study of 31 women who received a prenatal diagnosis of CHD during the pandemic. Patients completed semi-structured interviews about their experiences with fetal care, social support, and perceptions of risk to themselves and their fetus. Consistent themes regarding the impact of the pandemic were identified using an inductive thematic approach. Demographic data were collected via self-report and chart review. RESULTS: Women generally reported consistent access to fetal care throughout the pandemic, with frequent use of telemedicine in addition to in-person care, but negative impacts resulting from restrictions on family support at appointments. Limited access to social support overall and a loss of pregnancy traditions were described. Many women reported feeling isolated and experiencing worries and fears about COVID-19 but also noted feeling supported by their healthcare team. Partner/family support during appointments and connection to peer-to-peer support were identified as recommendations to mitigate negative impacts. CONCLUSION: Women carrying a fetus with CHD during the COVID-19 pandemic experienced unique stressors that may affect mental health. However, many also experienced unexpected supports that may mitigate effects of pandemic-related stressors. Results can inform efforts to promote positive family outcomes during and following the COVID-19 pandemic.

Factors Associated With Attendance for Cardiac Neurodevelopmental Evaluation.

BACKGROUND AND OBJECTIVES: Neurodevelopmental evaluation of toddlers with complex congenital heart disease is recommended but reported frequency is low. Data on barriers to attending neurodevelopmental follow-up are limited. This study aims to estimate the attendance rate for a toddler neurodevelopmental evaluation in a contemporary multicenter cohort and to assess patient and center level factors associated with attending this evaluation. METHODS: This is a retrospective cohort study of children born between September 2017 and September 2018 who underwent cardiopulmonary bypass in their first year of life at a center contributing data to the Cardiac Neurodevelopmental Outcome Collaborative and Pediatric Cardiac Critical Care Consortium clinical registries. The primary outcome was attendance for a neurodevelopmental evaluation between 11 and 30 months of age. Sociodemographic and medical characteristics and center factors specific to neurodevelopmental program design were considered as predictors for attendance. RESULTS: Among 2385 patients eligible from 16 cardiac centers, the attendance rate was 29.0% (692 of 2385), with a range of 7.8% to 54.3% across individual centers. In multivariable logistic regression models, hospital-initiated (versus family-initiated) scheduling for neurodevelopmental evaluation had the largest odds ratio in predicting attendance (odds ratio = 4.24, 95% confidence interval, 2.74-6.55). Other predictors of attendance included antenatal diagnosis, absence of Trisomy 21, higher Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category, longer postoperative length of stay, private insurance, and residing a shorter distance from the hospital. CONCLUSIONS: Attendance rates reflect some improvement but remain low. Changes to program infrastructure and design and minimizing barriers affecting access to care are essential components for improving neurodevelopmental care and outcomes for children with congenital heart disease.

Group Well Child Care for Mothers with Opioid Use Disorder: Framework for Implementation.

OBJECTIVE: For parents with opioid use disorder (OUD) and their children, group well child care (WCC) is an under-studied intervention that may reduce stigma, increase quality of care, and improve clinical outcomes. We explored barriers and facilitators to this intervention using an implementation science framework. METHODS: A qualitative study was conducted from October 2020-March 2021 as part of the planning phase of a cluster-randomized trial of group WCC. Parent participants were recruited from one urban, university-affiliated OUD treatment center to participate in semi-structured telephone interviews. Eligible parents had a child under two years old and were English speaking. Clinician participants were recruited from a nearby pediatric primary care practice. Inductive thematic analysis of interview responses was led by two investigators using open coding procedures. RESULTS: Thirty-one parents and thirteen pediatric clinicians participated in the interviews. Most parents (68%) reported that they would be likely or very likely to bring their child to the OUD treatment center for WCC. Six themes emerged describing perceived implementation barriers, including intervention difficulty, complexity, and potential negative outcomes such as loss of privacy. Six themes emerged as implementation facilitators: (1) focus on parental OUD and recovery, (2) peer support, (3) accessibility and coordination of care, (4) clinician skill and expertise in parental OUD, (5) increased time for patient care, and (6) continuity of care. CONCLUSIONS FOR PRACTICE: Parents and clinicians expressed multiple perceived benefits of this intervention. Identified barriers and facilitators will inform implementation and evaluation of group WCC within one OUD treatment program.

A Psychosocial Care Model for Families Affected by Congenital Heart Disease.

OBJECTIVES: To develop a model of family-based psychosocial care for congenital heart disease (CHD). DESIGN: Qualitative study using crowdsourced data collected from parents of young children with CHD who received care across 42 hospitals. SETTING: Yammer, a social networking platform used to facilitate online crowdsourcing and qualitative data collection. SUBJECTS: Geographically diverse sample of 100 parents (72 mothers and 28 fathers) of young children with CHD. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Parents joined a private group on Yammer and responded to 37 open-ended study questions over a 6-month period. Qualitative data were coded and analyzed using an iterative process. Three broad themes corresponding to pillars of family-based psychosocial care were identified: pillar 1) parent partnership in family-integrated medical care, pillar 2) supportive interactions focused on parent and family wellbeing, and pillar 3) integrated psychosocial care and peer support for parents and families. Each pillar was supported by subthemes corresponding to specific intervention strategies. Most parents described the need for intervention strategies across multiple pillars, with almost half reporting needs across all three pillars of psychosocial care. Parents' preferences for psychosocial support changed over time with changes to their child's medical status and across care settings (e.g., hospital, outpatient clinic). CONCLUSIONS: Results support a model of family-based psychosocial care that is multidimensional and flexible to meet the needs of families affected by CHD. All members of the healthcare team play an important role in providing psychosocial support. Future research incorporating components of implementation science is needed to promote uptake of these findings, with the goal of optimizing family-based psychosocial support in the hospital setting and beyond.

Virtually Delivered Psychosocial Intervention for Prenatally Diagnosed Congenital Heart Disease: Feasibility and Acceptability of HEARTPrep.

Prenatal diagnosis of congenital heart disease (CHD) often leads to anxiety, depression, and traumatic stress in expectant mothers, with long-term implications for the child and family. However, psychosocial intervention is rarely incorporated into prenatal care. HEARTPrep is a virtually delivered psychosocial intervention aimed at reducing distress and social isolation and increasing parenting self-efficacy and hope for mothers expecting a baby with CHD to promote long-term child/family well-being. This study evaluated the feasibility and acceptability of HEARTPrep. Participants were mothers receiving cardiology care for a fetal CHD diagnosis. Partners could participate with the mother. HEARTPrep was delivered through a mobile app and telehealth. Feasibility was assessed through enrollment/retention rates. Acceptability was assessed through 20 Likert-scale and five open-ended questions. Of 39 recruited mothers, 35 (90%) enrolled. Half of partners (48%) also participated. Twenty-seven of 35 enrolled mothers (77%) completed HEARTPrep. On a scale from 0 (Not at All) to 4 (Very), mean item acceptability scores ranged from 3.5 to 3.9. Mothers reported HEARTPrep helped them feel less distressed (mean: 3.74), less alone (3.84), more prepared (3.89), and more hopeful (3.84). Opportunities to process emotions, develop coping skills, learn with their partner, navigate relationships, understand they are not alone, connect with peer support, access resources, and prepare for stressors were described as helpful. HEARTPrep is feasible and acceptable for mothers expecting a baby with CHD. Future research will evaluate its efficacy in preventing/reducing maternal mental health problems and improving postnatal clinical outcomes.

The Child Healthcare at MATER Pediatric Study (CHAMPS): a 2-arm cluster randomized control trial of group well child care for mothers in treatment for opioid use disorder and their children.

BACKGROUND: Studies suggest that group-based well child care-a shared medical appointment where families come together as a group to receive pediatric primary care-increases patient-reported satisfaction and adherence to recommended care. Evidence supporting the use of group well child care for mothers with opioid use disorder, however, is lacking. The overall objective of the Child Healthcare at MATER Pediatric Study (CHAMPS) trial is to evaluate a group model of well child care for mothers with opioid use disorder and their children. METHODS: CHAMPS is a single-site 2-arm cluster randomized controlled trial. A total of 108 mother-child dyads will be enrolled into the study. Twenty-six clusters of approximately 4 mother-infant dyads each will be randomized 1:1 to one of two study arms (intervention or control). Clustering will be based on child's month of birth. In the intervention arm, group well child care will be provided on-site at a maternal substance use disorder treatment program. Mother-child dyads in the control arm will receive individual well child care from one nearby pediatric primary care clinic. Dyads in both study arms will be followed prospectively for 18 months, and data will be compared between the two study arms. Primary outcomes include well child care quality and utilization, child health knowledge, and parenting quality. DISCUSSION: The CHAMPS trial will provide evidence to determine if a group well child care offered on-site at an opioid treatment program for pregnant and parenting women is beneficial over individual well child care for families impacted by maternal opioid use disorder. TRIAL REGISTRATION: ClinicalTrials.gov identifier: NCT05488379. Registered on Aug. 04, 2022.

Prevalence and associated factors of post-traumatic stress disorder in parents whose infants have single ventricle heart disease.

INTRODUCTION: Post-traumatic stress disorder occurs in parents of infants with CHD, contributing to psychological distress with detrimental effects on family functioning and well-being. We sought to determine the prevalence and factors associated with post-traumatic stress disorder symptoms in parents whose infants underwent staged palliation for single ventricle heart disease. MATERIALS AND METHODS: A large longitudinal multi-centre cohort study evaluated 215 mothers and fathers for symptoms of post-traumatic stress disorder at three timepoints, including post-Norwood, post-Stage II, and a final study timepoint when the child reached approximately 16 months of age, using the self-report questionnaire Impact of Event Scale - Revised. RESULTS: The prevalence of probable post-traumatic stress disorder post-Norwood surgery was 50% of mothers and 39% of fathers, decreasing to 27% of mothers and 24% of fathers by final follow-up. Intrusive symptoms such as flashbacks and nightmares and hyperarousal symptoms such as poor concentration, irritability, and sudden physical symptoms of racing heart and difficulty breathing were particularly elevated in parents. Higher levels of anxiety, reduced coping, and decreased satisfaction with parenting were significantly associated with symptoms of post-traumatic stress disorder in parents. Demographic and clinical variables such as parent education, pre-natal diagnosis, medical complications, and length of hospital stay(s) were not significantly associated with symptoms of post-traumatic stress disorder. DISCUSSION: Parents whose infants underwent staged palliation for single ventricle heart disease often reported symptoms of post-traumatic stress disorder. Symptoms persisted over time and routine screening might help identify parents at-risk and prompt referral to appropriate supports.

Barriers and facilitators to discussing parent mental health within child health care: Perspectives of parents raising a child with congenital heart disease.

This study aimed to identify barriers and facilitators to discussing parent mental health within child health care for parents of children with congenital heart disease (CHD). Seventy-nine parents of young children with CHD who received care across 40 hospitals in the United States responded to questions about barriers and facilitators to discussing their mental health with their child's health care providers. Responses were analyzed using qualitative research methods. Parents described multiple barriers: (1) belief that parent mental health support was outside the care team's scope of practice, (2) perceived expectation to "stay strong," (3) fear of negative judgment or repercussion, (4) individual preferences for communication/support, (5) desire to maintain care resources on their child, (6) perceived need to compartmentalize emotions, and (7) negative reactions to past emotional disclosure. Parents also described several facilitators: (1) confidence in the care team's ability to provide support, (2) intentional efforts by the care team to provide support, (3) naturally extroverted tendencies, and (4) developing personal connections with health care providers. It is important that health care providers normalize the impact of child illness on the family and create an environment in which parents feel comfortable discussing mental health challenges.

The Relationship of Family Factors to Psychosocial Outcomes in Children with Hypoplastic Left Heart Syndrome at 6 Years of Age.

OBJECTIVE: The objective of this study was to describe the relationships between family factors and outcomes for children with hypoplastic left heart syndrome (HLHS). STUDY DESIGN: This cross-sectional study was ancillary to the Pediatric Heart Network Single Ventricle Reconstruction Extension Study to examine family factors including parental mental health, quality of life (QOL), family resources, function and management, and their relationships to child psychosocial outcomes (adaptive behavior, internalizing and externalizing behaviors and health-related quality of life [HRQOL]) at 6 years of age. RESULTS: Participants were parents (115 mothers, 71 fathers) of children with HLHS. Parents reported anxiety, QOL and family resources that were worse than the general population; 33% reported family dysfunction. There were no meaningful differences between reports from mothers and fathers. Parental perception of better child health was associated with better family management of the condition (P < .05). Several family management factors explained a moderate amount of variance in adaptive behavior (ΔR2 = 0.08-0.14), adaptive skills (ΔR2 = 0.19-0.21), and HRQOL scores (ΔR2 = 0.04-0.18); little variance was explained in internalizing problems (ΔR2 = 0.02-0.03) (all P < .05) above and beyond demographic and clinical variables. CONCLUSIONS: HLHS has a significant impact on both children and families. Relationships between child and family characteristics may impose risk or protection. Improved understanding of these associations should guide counseling and tailored interventions.

Opportunities to Increase Well-Child Care Engagement for Families Affected by Maternal Opioid Use Disorder: Perceptions of Mothers and Clinicians.

OBJECTIVE: Previous research suggests gaps in well-child care (WCC) adherence, quality, and effectiveness for children impacted by parental opioid use disorder (OUD). The objective of this study was to gather in-depth information regarding maternal and clinician-reported factors that enhance ("facilitators") or hinder ("barriers") WCC engagement as well as mothers' experiences during WCC visits. METHODS: Thirty mothers who were in treatment for OUD and 13 clinicians working at a pediatric primary care clinic participated in this qualitative study. All participants completed one data collection telephone session which involved a brief questionnaire followed by a semi-structured interview. Thematic analyses of the interview transcripts were conducted using an inductive approach. RESULTS: Three broad themes were identified as facilitators of WCC by mothers and clinicians, including: 1) continuity in care, 2) addressing material needs, and 3) clinician OUD training and knowledge. Themes identified as barriers to WCC included: 1) stigma toward mothers with OUD, 2) gaps in basic parenting knowledge, 3) competing specialized health care needs, and 4) insufficient time to address all concerns. CONCLUSION: WCC programs or clinical pathways designed for families affected by maternal OUD should consider these barriers and facilitators of WCC engagement and affect experiences of WCC for mothers and clinicians.

Pediatric and Congenital Cardiovascular Disease Research Challenges and Opportunities: JACC Review Topic of the Week.

The National Heart, Lung, and Blood Institute convened a workshop in August 2021 to identify opportunities in pediatric and congenital cardiovascular research that would improve outcomes for individuals with congenital heart disease across the lifespan. A subsidiary goal was to provide feedback on and visions for the Pediatric Heart Network. This paper summarizes several key research opportunities identified in the areas of: data quality, access, and sharing; aligning cardiovascular research with patient priorities (eg, neurodevelopmental and psychological impacts); integrating research within clinical care and supporting implementation into practice; leveraging creative study designs; and proactively enriching diversity of investigators, participants, and perspectives throughout the research process.

Meeting Parents' Needs for Education and Preparation following Congenital Heart Disease Diagnosis: Recommendations from a Crowdsourced Study.

OBJECTIVE: This article characterizes the educational needs of parents following fetal or neonatal congenital heart disease (CHD) diagnosis and generates recommendations for meeting these needs. STUDY DESIGN: Online crowdsourcing methods were used to collect qualitative data from 95 parents of children with CHD regarding their needs for education and preparation following fetal or neonatal diagnosis. Data were analyzed using qualitative methods and themes were organized around the substructure of met and unmet needs. RESULTS: Two themes represented consistently met needs, whereas 10 themes represented needs that were either inconsistently met or consistently unmet. Parents reported needing more information about social, emotional, and financial supports, preparation for long-term care, and guidance toward reputable online resources. Parents also provided recommendations for meeting these needs. CONCLUSION: Parents' needs for education and preparation following CHD diagnosis are much broader in scope than what they currently receive. Addressing these may support parental coping and active participation in medical decision-making. KEY POINTS: · CHD counseling after diagnosis may provide opportunities to promote parents' mental health.. · Guidelines recommend that this counseling should include emotional and decision-making support, however, it is unclear what parents actually receive.. · This study found that parents' needs for education and preparation following CHD diagnosis are much broader in scope than what they currently receive..

Partnering With Stakeholders to Inform the Co-Design of a Psychosocial Intervention for Prenatally Diagnosed Congenital Heart Disease.

Input from diverse stakeholders is critical to the process of designing healthcare interventions. This study applied a novel mixed-methods, stakeholder-engaged approach to co-design a psychosocial intervention for mothers expecting a baby with congenital heart disease (CHD) and their partners to promote family wellbeing. The research team included parents and clinicians from 8 health systems. Participants were 41 diverse parents of children with prenatally diagnosed CHD across the 8 health systems. Qualitative data were collected through online crowdsourcing and quantitative data were collected through electronic surveys to inform intervention co-design. Phases of intervention co-design were: (I) Engage stakeholders in selection of intervention goals/outcomes; (II) Engage stakeholders in selection of intervention elements; (III) Obtain stakeholder input to increase intervention uptake/utility; (IV) Obtain stakeholder input on aspects of intervention design; and (V) Obtain stakeholder input on selection of outcome measures. Parent participants anticipated the resulting intervention, HEARTPrep, would be acceptable, useful, and feasible for parents expecting a baby with CHD. This model of intervention co-design could be used for the development of healthcare interventions across chronic diseases.