Wellbeing in Children and Adolescents with Fontan Physiology.

OBJECTIVE: To assess health-related quality of life (HRQOL) and global quality of life (QOL) in children and adolescents with Fontan physiology and identify key predictors influencing these outcomes. STUDY DESIGN: Cross-sectional analysis of 73 children and adolescents enrolled in the Australia and New Zealand Fontan Registry aged 6-17 years, at least 12 months post-Fontan operation. Assessments included the Pediatric Quality of Life Inventory (PedsQL) for HRQOL and a developmentally-tailored visual analogue scale (0-10) for global QOL, along with validated sociodemographic, clinical, psychological, relational, and parental measures. Clinical data were provided by the Australia and New Zealand Fontan Registry. RESULTS: Participants (mean age: 11.5 ± 2.6 years, 62% male) reported lower overall HRQOL (P < .001), and lower scores across all HRQOL domains (all P < .0001), compared with normative data. Median global QOL score was 7.0 (IQR 5.8-8.0), with most participants (79%) rating their global QOL ≥6. Anxiety and depressive symptoms requiring clinical assessment were reported by 21% and 26% of participants, respectively. Age, sex, and perceived seriousness of congenital heart disease explained 15% of the variation in HRQOL scores, while depressive symptoms and treatment-related anxiety explained an additional 37% (final model: 52% of variance explained). For global QOL, sociodemographic and clinical factors explained 13% of the variance in scores, while depressive symptoms explained a further 25% (final model: 38% of variance explained). Parental factors were not associated with child QOL outcomes. CONCLUSIONS: Children and adolescents with Fontan physiology experience lower HRQOL than community-based norms, despite reporting fair overall QOL. Psychological factors predominantly influenced QOL outcomes, indicating strategies to bolster psychological health could improve QOL in this population.

Development of a new social prescribing intervention for families of children with cerebral palsy.

AIM: To co-design a social prescribing intervention (the EPIC-CP programme: Equitable Pathways and Integrated Care in Cerebral Palsy) with children with cerebral palsy (CP), their families, and clinicians to address unmet social needs. METHOD: The study was conducted (August 2021 to March 2023) at the paediatric rehabilitation departments of the three tertiary paediatric hospitals in New South Wales, Australia. Eligible participants attended or worked at one of the departments, including children with CP, parents/caregivers, and clinicians. Mixed-methods co-design was used in intervention co-production and prototyping. The project was overseen by research advisors with lived experience of CP. RESULTS: More than 200 participants contributed to the co-design research. Families experienced a substantial burden of unmet social needs. Co-designed interventions involved systematic identification of unmet social needs with (1) targeted community resources and (2) engagement with a 'community linker' who supported children/young people and their families to access health, education, and social services that matched their identified needs and preferences. Research participants co-developed the programme logic model and prototype. This was piloted in research action cycles and iteratively refined until consensus was achieved. INTERPRETATION: We co-designed a social prescribing programme responsive to the needs of its end-users and purposefully developed to be embedded in the Australian health setting. A pilot randomized controlled trial will further evaluate this intervention.

Quality of Life and Well-Being in Adults With Fontan Physiology: Findings From the Australian and New Zealand Fontan Registry Quality of Life Study.

BACKGROUND: To characterize global and health-related quality of life (QOL) among adults with Fontan physiology enrolled in the Australian and New Zealand Fontan Registry (ANZFR), and identify sociodemographic, clinical, psychological, and relational factors associated with outcomes. METHODS AND RESULTS: Using a cross-sectional survey design, 66 adults with Fontan physiology (58% women; mean age, 29.6±7.7 years; range, 18-50 years) completed validated self-report measures. Health-related QOL was assessed using the Pediatric Quality of Life Inventory, and global QOL was assessed using a visual analog scale (0-10). Participants reported lower total health-related QOL (P<0.001), as well as lower physical (P<0.001) and social (P=0.002) functioning compared with normative data. Median global QOL was 7.0 (interquartile range: 5.0-8.0) and most participants (71%) rated their QOL ≥6. For health-related QOL, age, sex, university education, and length of hospital stay in the past 12 months explained 27% of the variance in scores, while general psychological stress, medical traumatic stress, communication problems, and access to emotional support explained a further 44% of variance (final model: 71% of variance explained). For global QOL, sociodemographic and clinical factors explained 20% of the variance in scores, while psychological stress and sense of coherence explained a further 24% (final model: 44% of variance explained). CONCLUSIONS: Adults with Fontan physiology reported lower overall health-related QOL compared with community-based norms. Variance in QOL outcomes were predominantly attributable to psychological and relational factors. Tailored screening and assessment to identify Fontan patients at greatest risk of lower QOL, and a proactive approach to supportive care, are needed.

Integrated psychological care in pediatric hospital settings for children with complex chronic illness and their families: a systematic review.

OBJECTIVE: To synthesize and critically evaluate evidence on the effectiveness of integrated psychological care models for children with complex chronic illness within pediatric hospital settings and provide recommendations for successful implementation. DESIGN: Six electronic databases (Medline, Cochrane, Embase, PsycINFO, Scopus, CINAHL) were systematically searched for English language studies including families of children aged 0-17 years with complex chronic illness. Eligible studies reported on psychology or neuropsychology screening, assessment, intervention, or services provided within a pediatric hospital setting. RESULTS: Fifteen studies were identified for review; nine assessed a psychological service, five examined psychosocial screening, and one examined a neuropsychology service. Three studies demonstrated the effectiveness of integrated psychological services in improving child or parent physical, psychological, or behavioral health outcomes. Uptake of psychosocial screening was high (84-96%), but only 25-37% of children or families identified as 'at-risk' engaged with on-site psychology services. Integrated psychological services offering consultations at the same time and location as the child's medical visit reported the highest rates of uptake (77-100%). CONCLUSIONS: The available evidence supports co-location of child medical and psychological services. A more consistent and comprehensive approach to the assessment of patient- and caregiver-reported outcomes and implementation effectiveness is recommended.

The Australian and New Zealand Fontan Registry Quality of Life Study: Protocol for a population-based assessment of quality of life among people with a Fontan circulation, their parents, and siblings.

INTRODUCTION: Advances in the care of patients with single-ventricle congenital heart disease have led to a new generation of individuals living with a Fontan circulation. For people with Fontan physiology, physical, psychological and neurodevelopmental challenges are common. The objective of this study is to describe and develop a deeper understanding of the factors that contribute to quality of life (QOL) among children, adolescents and adults living with a Fontan circulation across Australia and New Zealand, their parents and siblings. METHODS AND ANALYSIS: This article presents the protocol for the Australian and New Zealand Fontan Registry (ANZFR) QOL Study, a cross-sectional, population-based study designed to examine QOL among people of all ages with a Fontan circulation, their parents and siblings. Study eligibility criteria includes (1) individuals with a Fontan circulation aged ≥6 years, at least 12 months post-Fontan procedure and enrolled in the ANZFR; (2) parents of individuals enrolled in the ANZFR; and (3) siblings aged ≥6 years of an individual enrolled in the ANZFR. A novel, online research platform is used to distribute personalised assessments tailored to participant age and developmental stage. A suite of validated psychometric self-report and parent-proxy report instruments capture potential correlates and predictors of QOL, including symptoms of psychological distress, personality attributes, coping and cognitive appraisals, family functioning, healthcare experiences and costs, access to emotional support and socioeconomic factors. Clinical characteristics are captured via self-report and parent-proxy report, as well as the ANZFR. Descriptive analyses and multilevel models will be used to examine QOL across groups and to investigate potential explanatory variables. ETHICS AND DISSEMINATION: Approval has been obtained from all relevant Human Research Ethics Committees (HRECs), including the Sydney Children's Hospitals Network and the Royal Children's Hospital Melbourne HRECs. Study findings will be published in peer-reviewed journals and presented at national and international meetings and seminars.

Diagnosis and management of childhood obesity: a survey of general practitioners in South West Sydney.

AIM: To describe general practitioners' (GPs) diagnosis and management of overweight and obesity in children, their attitudes regarding obesity and their awareness of National Health and Medical Research Council (NHMRC) clinical practice guidelines. METHOD: A cross-sectional written survey of members of the Liverpool Division of General Practice (located in South West Sydney, Australia). RESULTS: Of 137 questionnaires sent, 85 (62%) were returned. Although the majority prescribed the correct interventions, there was variability in complications screening, ranging from 75% screening for psychosocial problems to 30% for fatty liver. Less than a third (28%) of GPs used NHMRC guidelines in their practice and only 9% used body mass index charts to correctly diagnose childhood obesity. GPs felt that childhood obesity was a significant issue and identified parental denial and lack of community support as barriers to treatment. CONCLUSION: Although NHMRC guideline adherence was far from universal, the GPs in our survey are motivated and aware of the importance of managing childhood obesity. If the primary care approach is to work, then GPs need support and education in an environment where there is greater community awareness of the impact of childhood obesity.

The first eighteen months of a paediatric ambulatory and community service.

Hospital admission is distressing and traumatic for children as they are separated from their families and home. Internationally, and in Australia, alternative models of health care are being developed to meet the needs of children and their families. We describe the first eighteen months of the establishment of a paediatric ambulatory and community service in a district health service in New South Wales. Key outcomes include: increased referral to the service from acute hospital and primary care services; parental satisfaction and saved hospital bed-days. Lessons learnt in the setting up of this service include the need for proactive engagement of consumers and stakeholders; clear definition of roles and responsibilities; and measurable and reasonable performance indicators.