Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards). 1st Edition.

These first Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards) have been developed to inform the healthcare requirements for CoHD services and enable all Australian patients, families and carers impacted by CoHD (paediatric CoHD and adult congenital heart disease [ACHD]) to live their best and healthiest lives. The CoHD Standards are designed to provide the clarity and certainty required for healthcare services to deliver excellent, comprehensive, inclusive, and equitable CoHD care across Australia for patients, families and carers, and offer an iterative roadmap to the future of these services. The CoHD Standards provide a framework for excellent CoHD care, encompassing key requirements and expectations for whole-of-life, holistic and connected healthcare service delivery. The CoHD Standards should be implemented in health services in conjunction with the National Safety and Quality Health Service Standards developed by the Australian Commission on Safety and Quality in Health Care. All healthcare services should comply with the CoHD Standards, as well as working to their organisation's or jurisdiction's agreed clinical governance framework, to guide the implementation of structures and processes that support safe care.

Health-related quality of life and self-reported health status in adolescents with chronic health conditions before transfer of care to adult health care: an international cohort study.

BACKGROUND: Heath-related quality of life (HRQoL) is lower in adolescents with chronic health conditions compared to healthy peers. While there is evidence of some differences according to the underlying condition and gender, differences by measure and country are poorly understood. In this study we focus on the differences in HRQoL in adolescents with various chronic medical conditions in the year before transfer of care to adult health services. We also study the associations of two different HRQoL measurements to each other and to self-reported health. METHODS: We recruited 538 adolescents from New Children`s Hospital, Helsinki, Finland, and the Royal Children`s Hospital, Melbourne, Australia in 2017-2020. We used two validated HRQoL measurement instruments, Pediatric Quality of Life Inventory (PedsQL) and 16D, and a visual analog scale (VAS) for self-reported health status. RESULTS: In total, 512 adolescents (50.4% female, mean age 17.8 [SD 1.2] years), completed the survey measures. Higher HRQoL was reported in males than females in both countries (PedsQL 79.4 vs. 74.1; 16D 0.888 vs. 0.846), and in adolescents from Finland than Australia (80.6 vs. 72.2 and 0.905 vs. 0.825, p < 0.001 for all). Adolescents with diabetes, rheumatological, nephrological conditions and/or organ transplants had higher HRQoL than adolescents with neurological conditions or other disease syndromes (p < 0.001). PedsQL and 16D scores showed a strong correlation to each other (Spearman correlation coefficient r = 0.81). Using the 7-point VAS (1-7), 52% (248 of 479) considered their health status to be good (6-7) and 10% (48 of 479) rated it poor (1-2). Better self-reported health was associated with higher HRQoL. CONCLUSIONS: The HRQoL of transition aged adolescents varies between genders, diagnostic groups, and countries of residence. The association between self-reported health and HRQoL suggests that brief assessment using the VAS could identify adolescents who may benefit from in-depth HRQoL evaluation. TRIAL REGISTRATION: Trial registration name The Bridge and registration number NCT04631965 ( https://clinicaltrials.gov/ct2/show/NCT04631965 ).

Exploring the transition experiences of young adults with cerebral palsy.

BACKGROUND: It is important that young adults with a chronic health condition or developmental disability, such as cerebral palsy, receive adequate healthcare transition preparation and support to optimise the transition period and transfer from paediatric to adult health services. Understanding the healthcare experiences of young adults during and after the transition period will provide valuable insights into what enables a positive healthcare experience for young adults in the adult health setting. METHODS: Eleven young adults with cerebral palsy who had their last appointment at the Royal Children's Hospital between 2016 and 2018 were purposively recruited for this study. Ten participants completed one-on-one telephone interviews, and one participant provided written responses to interview questions. Five participated via parent proxy. Interviews were recorded, transcribed verbatim, and analysed using the Braun and Clarke six-step thematic analysis to create an interpretive description of participants' transition experiences. RESULTS: Three themes were generated: (1) "preparedness of the young adult and parent," which discussed the preparation for adult healthcare, with subthemes (a) expectations of adult care and (b) development of self-management skills during transition; (2) "coordination of transfer process and continuity of care," which illustrated the impact of transfer coordination on continuity of care; and (3) "adjusting to adult services," which highlighted experiences of care in the adult setting, with subthemes (a) differences between paediatric and adult services, (b) availability and accessibility of adult and community services to meet needs, and (c) autonomy and agency. CONCLUSION: Dedicated transition support for young adults and their parents during transition from paediatric to adult healthcare plays an important role in ensuring a supportive and well-coordinated transition and transfer of care. Experience of care in the adult setting is influenced by a combination of both transition experience and the capacity of adult services to cater for young adults' needs.

Transition readiness and anxiety among adolescents with a chronic condition and their parents: A cross-sectional international study.

AIMS: To evaluate associations of age, transition readiness and anxiety in adolescents with chronic conditions and to compare perceptions of adolescents and their parents regarding health self-management and transition readiness. DESIGN: Cross-sectional international study, reported following STROBE guidelines. METHODS: Adolescents and young adults (N = 512, mean age 17.7) with a chronic medical condition and their parents (N = 322) from Finland and Australia. Data were collected through surveys (between September 2017 and December 2020). Adolescents reported the duration of their condition. Age at survey was defined by the response date of the questionnaires. Validated questionnaires were used to measure transition readiness (Am I ON TRAC? for Adult Care) and anxiety related to transition of care (State-Trait Anxiety Inventory short form). Perceptions of health self-management and transition readiness were compared in adolescent/parent dyads. Associations were explored using Spearman's correlation. RESULTS: Duration of condition and age at survey correlated weakly with transition readiness knowledge and behaviour. Higher transition readiness knowledge scores correlated with higher behaviour scores. Higher transition readiness behaviour scores were associated with lower levels of anxiety. Adolescents were less anxious than their parents and adolescents and parents mostly agreed about health self-management and transition readiness. CONCLUSION: Transition readiness should be determined by an assessment of knowledge, self-management and psychosocial skills instead of age alone. Further research should address how well transition readiness predicts positive health outcomes after the transfer of care. IMPLICATIONS FOR PATIENT CARE: Transition readiness and self-management skills should be formally assessed because positive feedback may decrease the anxiety of both adolescents and their parents regarding the transfer of care. REPORTING METHOD: We have adhered to the STROBE statement, using STROBE checklist for cross-sectional studies. PATIENT OR PUBLIC INVOLVEMENT STATEMENT: No patient or public involvement. TRIAL AND PROTOCOL REGISTRATION: ClinicalTrials.org NCT04631965.

The International Network on Oesophageal Atresia (INoEA) consensus guidelines on the transition of patients with oesophageal atresia-tracheoesophageal fistula.

Oesophageal atresia-tracheoesophageal fistula (EA-TEF) is a common congenital digestive disease. Patients with EA-TEF face gastrointestinal, surgical, respiratory, otolaryngological, nutritional, psychological and quality of life issues in childhood, adolescence and adulthood. Although consensus guidelines exist for the management of gastrointestinal, nutritional, surgical and respiratory problems in childhood, a systematic approach to the care of these patients in adolescence, during transition to adulthood and in adulthood is currently lacking. The Transition Working Group of the International Network on Oesophageal Atresia (INoEA) was charged with the task of developing uniform evidence-based guidelines for the management of complications through the transition from adolescence into adulthood. Forty-two questions addressing the diagnosis, treatment and prognosis of gastrointestinal, surgical, respiratory, otolaryngological, nutritional, psychological and quality of life complications that patients with EA-TEF face during adolescence and after the transition to adulthood were formulated. A systematic literature search was performed based on which recommendations were made. All recommendations were discussed and finalized during consensus meetings, and the group members voted on each recommendation. Expert opinion was used when no randomized controlled trials were available to support the recommendation. The list of the 42 statements, all based on expert opinion, was voted on and agreed upon.

The Australian evidence-based clinical practice guideline for attention deficit hyperactivity disorder.

OBJECTIVE: The objective of this article was to provide an overview of the development and recommendations from the Australian evidence-based clinical practice guideline for attention deficit hyperactivity disorder (ADHD). The guideline aims to promote accurate and timely identification and diagnosis, and optimal and consistent treatment of ADHD. METHODS: Development integrated the best available evidence with multidisciplinary clinical expertise and the preferences of those with lived experience, underpinned by the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) framework. The 23 guideline development group members included psychiatrists, paediatricians, general practitioners, psychologists, speech pathologists, occupational therapists, educators, Indigenous psychologists, and people with a lived experience; with two independent chairs and a methodologist. Where appropriate, evidence reviews from the National Institute for Health and Care Excellence (NICE) 2018 'Attention Deficit Hyperactivity Disorder: Diagnosis and Management' guideline were updated. Fifty prioritised clinical questions were addressed in 14 systematic reviews (new and updated from NICE 2018) and 28 narrative reviews. RESULTS: The 113 clinical recommendations apply to young children (5 years and under), children, adolescents and adults. They provide guidance for clinicians on identification, screening, diagnosis, multimodal treatment and support, including pharmacological and non-pharmacological interventions. The guideline and supporting information are available online: https://adhdguideline.aadpa.com.au/. CONCLUSIONS: The guideline was approved by the National Health and Medical Research Council (NHMRC) of Australia and relevant medical and allied health professional associations. It is anticipated that successful implementation and uptake of the guideline by organisations, health care providers and other professionals will increase delivery of evidence-based treatment and improve health outcomes for the more than 800,000 Australians with ADHD.

Disease control and psychiatric comorbidity among adolescents with chronic medical conditions: a single-centre retrospective study.

BACKGROUND: To investigate disease control, psychiatric comorbidity, substance use and their possible associations in adolescents with chronic medical conditions before transfer to adult healthcare. METHODS: We collected clinical data from the year preceding transfer of care and psychiatric data from the records of the paediatric hospital in Helsinki, Finland (population base 1.7 million). Participants were grouped into three disease and/or adherence control categories (good, some evidence of concern, poor) based on clinical data from the medical records of the year preceding the transfer of care. Participants completed the Adolescent's Substance Use Measurement Questionnaire before transfer of care and were divided into four risk subgroups accordingly. RESULTS: In total, 253 adolescents (mean age 17.3 years, SD 1.2) from six paediatric subspecialties participated in this study. Disease control and/or adherence were rated as good in 28% (n=70), moderate in 42% (n=105) and poor in 30% (n=76) in the year before participants transferred to adult health services. A quarter of participants had at least one psychiatric diagnosis during adolescence. Adolescents with concomitant psychiatric diagnoses more often had poor disease control of their chronic medical condition than adolescents with only a medical condition (44% vs 26%; n=25 of 59 vs 51 of 194, respectively). More than half of adolescents (56%) were abstinent or used substances infrequently; 10% (n=26) reported hazardous substance use. CONCLUSIONS: Psychiatric comorbidity in adolescents with chronic medical conditions is common. Its negative association with disease control and possible substance use should be considered in the transition process to adult health services.

Carer perspectives of a transition to adult care model for adolescents with an intellectual disability and/or autism spectrum disorder with mental health comorbidities.

BACKGROUND: Transition to adult care for adolescents with an intellectual disability and/or autism spectrum disorder with coexisting mental health disorders, often termed 'dual disability', is complex. It requires a family-centred approach, with collaboration among health, disability and social services and early planning. AIM: To describe carer perspectives of transition to adult care and the outcomes of a transition support intervention, Fearless, Tearless Transition, for adolescents with dual disabilities piloted at a tertiary children's hospital. METHODS: Carers of adolescents with a dual disability were invited to complete a survey at the commencement of their participation in the Fearless, Tearless Transition model, and again at the conclusion of the project. Within this intervention, carers and adolescents were encouraged to attend dedicated transition clinics and participate in a shared care general practitioner (GP) and paediatrician process. RESULTS: One hundred and fifty-one carers of adolescents with dual disabilities were included in Fearless, Tearless Transition. Of this cohort, 138 adolescents and their carers received support in a dedicated transition clinic with 99 carers completing the initial survey at the commencement of the model. Eighty-two per cent of carers reported moderate to high levels of anxiety about transitioning from paediatric to adult care with 39% feeling 'unprepared' about transition. Eighty-one per cent reported having inadequate access to respite care with 47% reporting a lack of access to services in the community and 56% expressing dissatisfaction with their GPs. One hundred and two families participated in the shared care process with 80 GPs and 33 paediatricians. Twenty-two carers completed the second survey reporting a modest but significant improvement in preparedness for transition to adult care. CONCLUSION: This study highlights the potential to improve transition outcomes for adolescents with dual disabilities and their carers through early, centralized transition planning, consistent methods of assessing adolescent and carer needs and shared care.

Enabling successful transition-Evaluation of a transition to adult care program for pediatric liver transplant recipients.

BACKGROUND: This study aimed to evaluate the transition to adult care program instituted for liver transplant recipients (LTRs) at a large tertiary pediatric hospital in Melbourne, Australia. Evaluation included the change in a Global Assessment Measure (GAM) before and after the transition program, satisfaction with the program, and measures of transition success including rejection rates and attendance at appointments post-transfer. We hypothesized that the introduction of our structured transition program would improve disease understanding, health system understanding, and self-care. We also hypothesized that those who had undergone the transition program would have lower failure to attend rates and lower rates of rejection than historical controls. METHODS: A LTR transition program was instituted at our service from 2013 to 2015. The program involved initial assessment of competencies with a Global Assessment Measure (GAM), followed by the introduction of a personalized goal setting program addressing issues identified in dedicated transition clinics. Assessment of competencies was compared between the commencement of the program and immediately prior to transfer. Patient satisfaction with the transition process was assessed at an interview 6-12 months after transfer to the adult service. Rejection rates and failure to attend rates were compared between the intervention group and a group of LTRs who did not receive the intervention. RESULTS: Twenty-eight LTRs participated in the study; 20 received the transition intervention and 8 served as controls. Within the intervention group, all domains of transition competency and reported anxiety regarding transferring had significantly improved at the conclusion of the intervention and all reported satisfaction with the transition program with most (81%) reporting readiness to transfer. There were no significant differences in rejection rates or failure to attend rates between those who did and did not receive the transition intervention. CONCLUSION: A longitudinal holistic transition program has the potential to positively impact the competencies and readiness of LTRs to successful transition and transfer to adult care.

Bridge study protocol: an international, observational cohort study on the transition of healthcare for adolescents with chronic conditions.

INTRODUCTION: More than 10% of adolescents live with a chronic disease or disability that requires regular medical follow-up as they mature into adulthood. During the first 2 years after adolescents with chronic conditions are transferred to adult hospitals, non-adherence rates approach 70% and emergency visits and hospitalisation rates significantly increase. The purpose of the Bridge study is to prospectively examine associations of transition readiness and care experiences with transition success: young patients' health, health-related quality of life (HRQoL) and adherence to medical appointments as well as costs of care. In addition, we will track patients' growing independence and educational and employment pathways during the transition process. METHODS AND ANALYSIS: Bridge is an international, prospective, observational cohort study. Study participants are adolescents with a chronic health condition or disability and their parents/guardians who attended the New Children's Hospital in Helsinki, Finland, or the Royal Children's Hospital (RCH) in Melbourne, Australia. Baseline assessment took place approximately 6 months prior to the transfer of care and follow-up data will be collected 1 year and 2 years after the transfer of care. Data will be collected from patients' hospital records and from questionnaires completed by the patient and their parent/guardian at each time point. The primary outcomes of this study are adherence to medical appointments, clinical health status and HRQoL and costs of care. Secondary outcome measures are educational and employment outcomes. ETHICS AND DISSEMINATION: The Ethics Committee for Women's and Children's Health and Psychiatry at the Helsinki University Hospital (HUS/1547/2017) and the RCH Human Research Ethics Committee (38035) have approved the Bridge study protocol. Results will be published in international peer-reviewed journals and summaries will be provided to the funders of the study as well as patients and their parents/guardians. TRIAL REGISTRATION NUMBER: NCT04631965.

Development of the Fearless, Tearless Transition model of care for adolescents with an intellectual disability and/or autism spectrum disorder with mental health comorbidities.

AIM: First, to understand the barriers to achieving effective transition and the supports required from the perspective of parents and carers, adolescents with intellectual disability and/or autism spectrum disorder and co-existing mental health disorders (often termed 'dual disability'), and those who provide services to this group. Second, to develop an informed model of shared care to improve the transition of adolescents with dual disabilities. METHOD: Carers and a young adult with a dual disability were surveyed about their experience of transition care. Other key stakeholders including paediatricians, general practitioners, and policy makers were also interviewed. These data informed the model of care. RESULTS: Paediatricians and general practitioners reported difficulties establishing working relationships to foster smooth transitions, and carers reported lacking a regular general practitioner with adequate expertise to care for people with dual disabilities. A process of shared care between paediatricians and general practitioners was developed and initiated by a dedicated transition manager, who assisted with care coordination and service linkages. Standardized clinical assessment tools were also introduced to determine patient and carer support needs. INTERPRETATION: This study highlights the potential to improve transition outcomes for adolescents with dual disabilities and their carers through early transition planning, consistent methods of assessing patient and carer needs, and shared care. WHAT THIS PAPER ADDS: Adolescents with co-occurring disabilities require a collaborative health and disability service interface. Fearless, Tearless Transition is a new approach to transitioning adolescents with dual disabilities from paediatric to adult care. Carers of adolescents with dual disabilities require support navigating and negotiating services. Engaging general practitioners and paediatricians in shared care early during the transition process is essential.

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement.

The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival. KEYPOINTS.

Traversing the liminal: what can Fontan adults' transition experiences and perspectives teach us about optimizing healthcare?

BACKGROUND: Successfully transitioning to adult care is important for congenital heart disease (CHD) patients, particularly those at the worst end of the spectrum with a Fontan circulation, as their ongoing health engagement affects their health outcomes. Yet, there is a lack of literature exploring patient perspectives about their transition experiences, and what helps or hinders successful transition to adult care. METHODS: Young adults with a Fontan circulation (n = 18) from the Australian and New Zealand Fontan Registry participated in in-depth phone interviews. Thematic analyses were used to analyze the qualitative interview data. RESULTS: We identified six key themes which stood out from patient experiences, including differences between pediatric and adult congenital cardiac care, the need for preparation through formal transition programs, and the important role of support systems. CONCLUSION: To become active managers of their healthcare and remain engaged with the healthcare system, young people would benefit from coordinated efforts between pediatric and adult care to formally prepare them for transition to adult care. This should include involvement from their support network and ongoing education about their health condition. The study highlights the importance of studying patient perspectives through qualitative research to better inform health service provision in the CHD space.

Adolescent and parent perspectives prior to involvement in a Fontan transition program.

Background Successful transition to adult healthcare is particularly important for congenital heart disease (CHD) patients who have undergone palliative surgery, as they risk adverse events if not followed closely. This study examines young people at the worst end of the CHD spectrum who are born with a single ventricle (pumping heart chamber), and who undergo a series of operations that culminate in the Fontan surgical procedure. Purpose To explore adolescents with a Fontan circulation, and their parents', readiness for transition to adult care and pre-implementation of a transition program. Methods Seventeen adolescents (15-18 years) and 15 of their parents completed questionnaires at the start of their first transition clinic. Results Adolescents reported poor knowledge about their Fontan circulation, and 41% had a poor understanding of the purpose of their medications/treatments. Over half of the adolescents had poor knowledge around medical help-seeking (when, who, how). Most reported feeling comfortable with discussing their medical issues with their cardiologist, but considerably less so about sensitive adolescent issues, in particular, emotional wellbeing. Parents reported high levels of anxiety around transition to adult care services. Conclusion Findings pre-program indicate poor adolescent health knowledge, a lack of focus in health services on emotional wellbeing and high parental anxiety. These findings highlight the need for dedicated programs that focus on early preparation, parental involvement and acknowledgment, transition as a process, strong integration and prioritisation in the health system with a youth-friendly and holistic focus, in particular, around emotional wellbeing.