Towards sustainable healthcare system performance in the 21st century in high-income countries: A protocol for a systematic review of the grey literature

© © Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. Introduction There is wide recognition that, if healthcare systems continue along current trajectories, they will become harder to sustain. Ageing populations, accelerating rates of chronic disease, increasing costs, inefficiencies, wasteful spending and low-value care pose significant challenges to healthcare system durability. Sustainable healthcare systems are important to patients, society, policy-makers, public and private funders, the healthcare workforce and researchers. To capture current thinking about improving healthcare system sustainability, we present a protocol for the systematic review of grey literature to capture the current state-of-knowledge and to compliment a review of peer-reviewed literature. Methods and analysis The proposed search strategy, based on the Preferred Reporting Items for Systematic Review and Meta-Analyses guidelines, includes Google Advanced Search, snowballing techniques and targeted hand searching of websites of lead organisations such as WHO, Organisation for Economic Cooperation and Development, governments, public policy institutes, universities and non-government organisations. Documents will be selected after reviewing document summaries. Included documents will undergo full-Text review. The following criteria will be used: grey literature document; English language; published January 2013-March 2018; relevant to the healthcare delivery system; the content has international or national scope in high-income countries. Documents will be assessed for quality, credibility and objectivity using validated checklists. Descriptive data elements will be extracted: identified sustainability threats, definitions of sustainability, attributes of sustainable healthcare systems, solutions for improvement and outcome measures of sustainability. Data will be analysed using novel text-mining methods to identify common concept themes and meanings. This will be triangulated with the more traditional analysis and concept theming by the researchers. Ethics and dissemination No primary data will be collected, therefore ethical approval will not be sought. The results will be disseminated in peer-reviewed literature, as conference presentations and as condensed summaries for policy-makers and health system partners. PROSPERO registration number CRD42018103076

The supportive care needs of parents with a child with a rare disease : results of an online survey

Background: Parents caring for a child affected by a rare disease have unmet needs, the origins of which are complex and varied. Our aim was to determine the supportive care needs of parents caring for a child with a rare disease. Methods: An online survey was developed consisting of 45 questions (108 items) and separated into six domains. The survey included questions about perceived level of satisfaction with receiving care, experiences and needs of providing daily care, the impacts of disease on relationships, the emotional and psychological burdens of disease, and parents overall satisfaction with the support received. Results: Three-hundred and one parents from Australia and New Zealand completed the survey; 91 % (n = 275/301) were mothers, with 132 distinct rare diseases being reported. Fifty-four percent (n = 140/259) of parents were dissatisfied with health professionals’ level of knowledge and awareness of disease; 71 % (n = 130/183) of parents felt they received less support compared to other parents. Information regarding present (60 %, n = 146/240) and future services (72 %, n = 174/240) available for their child were considered important. Almost half of parents (45 %, n = 106/236) struggled financially, 38 % (n = 99/236) reduced their working hours and 34 % (n = 79/236) ceased paid employment. Forty-two percent (n = 99/223) of parents had no access to a disease specific support group, and 58 % (n = 134/230) stated that their number of friends had reduced since the birth of their child; 75 % (n = 173/230) had no contact with other parents with a child with a similar disease, and 46 % (n = 106/230) reported feeling socially isolated and desperately lonely. Most frequent emotions expressed by parents in the week prior to completing the survey were anxiety and fear (53 %, n = 119/223), anger and frustration (46 %, n = 103/223) and uncertainty (39 %, n = 88/223). Conclusion: This study is the first to develop an online survey specifically for use with parents to investigate their supportive care needs across a large and diverse group of rare diseases. The findings highlight that parents with a child with a rare disease have common unmet needs regardless of what disease their child has. Such information may allow health providers to improve child outcomes through improving parental supportive care

Rare disease: a national survey of paediatricians' experiences and needs.

Objective: To describe the experiences of Australian paediatricians while caring for children with rare diseases, and their educational and resource needs. Design: A brief online survey was developed and deployed to a representative sample of 679 paediatricians from the Australian Paediatric Surveillance Unit database. Results: Of the 679 paediatricians, 242 (36%) completed the survey. The respondents were representative of all states and territories of Australia, urban and rural regions, and hospital and private practice. Almost all respondents (93%) had seen children with one or more of >350 different rare diseases during their career; 74% had seen a new patient with rare disease in the last 6 months. The most common problems encountered while caring for patients were: diagnostic delays (65%), lack of available treatments (40%), clinical guidelines (36%) and uncertainty where to refer for peer support (35%). Few paediatricians said that rare diseases were adequately covered during university (40%) or the Fellowship of the Royal Australasian College of Physicians (50%) training, and 28% felt unprepared to care for patients with rare diseases. Paediatricians wanted lists of specialist referral services (82%) and online educational modules about rare diseases (78%) that could be accessed via one online portal that consolidated multiple resources. Smartphone applications on rare diseases were favoured by paediatricians aged <50 years and by female paediatricians. Conclusions: An online educational portal should be developed and maintained for accuracy and currency of information to support dissemination of rare disease guidelines, referral pathways and coordination services relevant to Australian paediatricians and other health professionals who care for children with rare diseases

Patients with dementia and their carers in general practice

In 2004, 6.5% of Australians over 65 years of age were estimated to have dementia and the prevalence of dementia is rising asa result of ouraging population. There is evidence to show that carer wellbeing is important for the wellbeing of the patient. Increasing burden of care may lead to depression, anxiety, and more frequent physical illness in the carer, and earlier institutionalisation of patient

Significant reductions in tertiary hospital encounters and less travel for families after implementation of Paediatric Care Coordination in Australia 11 Medical and Health Sciences 1117 Public Health and Health Services

Background: Over a third of Australian children have long-term health conditions, often involving multiple organ systems and resulting in complex health care needs. Our healthcare system struggles to meet their needs because of sectoral fragmentation and episodic models of care. Children with medical complexity (CMC) currently rely on tertiary paediatric hospitals for most of their healthcare, but this is not sustainable. We evaluated the impacts of Care Coordination on tertiary hospital service use and family outcomes. Methods: A pre- and post-implementation cohort evaluation of the Care Coordination service at a tertiary paediatric hospital network, was undertaken. From July 2015 CMC enrolled in the service had access to a Care Coordinator, shared-care plans, linkage with local general practitioners (GPs), and access to a 24-h Hotline from August 2016. CMC were those with ≥4 emergency department (ED) presentations, hospital stays of ≥14 days, or ≥ 10 outpatient appointments in 12 months. Medically fragile infants at risk of frequent future hospital utilisation, and children with medical problems complicated by difficult family psychosocial circumstances were also included. Care Coordinators collected outcomes for each enrolled child. Administrative data on hospital encounters 6 months pre- and post-enrolment were analysed for children aged > 6 months. Results: An estimated 557 hospital encounters, were prevented in the 6 months after enrolment, for 534 children aged > 6 months. ED presentations decreased by 40% (Chi2 = 37.95; P < 0.0001) and day-only admissions by 42% (Chi2 = 7.54; P < 0.01). Overnight admissions decreased by 9% but this was not significant. An estimated Au4.9 million was saved over 2 years due to prevented hospital encounters. Shared-care plans were developed for 83.5%. Of 84 children who had no regular GP, 58 (69%) were linked with one. Fifty-five (10%) of families were linked to the 24-h Hotline to enable remote access to support and advice. Over 50,000 km of family travel and 370 school absences was prevented. Conclusions: The Care Coordination service has clear benefits for the tertiary paediatric hospital network and for families. Ongoing evaluation is essential for continuous improvement and to support adjustments to the model according to the local context

Australian children living with rare diseases: experiences of diagnosis and perceived consequences of diagnostic delays

BACKGROUND: Children and families living with rare disease often experience significant health, psychosocial, economic burdens and diagnostic delays. Experiences appear to be constant, regardless of the specific rare disease diagnosis. Systematically collected Australian data to support policy response on rare diseases are scarce. We address this gap by providing survey results about 462 children aged <19 years living with approximately 200 different rare diseases. RESULTS: Of 462 children, 96% were born in Australia, 55% were male, median age was 8.9 years (0-18.2). Four-hundred-and-twenty-eight (93%) had received a definitive diagnosis but 29 (7%) remained undiagnosed. Before receiving the correct diagnosis 38% consulted ≥ 6 different doctors. Among those with a diagnosis, 37% believed the diagnosis was delayed and 27% initially received a wrong diagnosis. Consequences of delayed diagnosis include anxiety, loss of reproductive confidence because of an ill-defined genetic risk, frustration and stress (54%), disease progression (37%), delays in treatment (25%) and inappropriate treatments (10%). Perceived reasons for diagnostic delays included lack of knowledge about the disease among health professionals (69.2%), lack of symptom awareness by the family (21.2%) and difficulties accessing tests (17.9%). Children with inborn errors of metabolism were less likely to have a delayed diagnosis compared with other disease groups (Chi-Sq = 17.1; P < 0.0001), most likely due to well-established and accessible biochemical screening processes. Diagnosis was given in person in 74% of cases, telephone in 18.5% and via a letter in 3.5%. Some families (16%) were dissatisfied with the way the diagnosis was delivered, citing lack of empathy and lack of information from health professionals. Psychological support at diagnosis was provided to 47.5%, but 86.2% believed that it should always be provided. Although 74.9% of parents believed that the diagnosis could have an impact on future family planning, only 44.8% received genetic counselling. CONCLUSION: Parents of children living with rare chronic and complex diseases have called for better education, resourcing of health professionals to prevent avoidable diagnostic delays, and to facilitate access to early interventions and treatments. Access to psychological support and genetic counselling should be available to all parents receiving a life-changing diagnosis for their child

Childhood interstitial lung disease due to surfactant protein C deficiency: Frequent use and costs of hospital services for a single case in Australia

Background: Rare chronic diseases of childhood are often complex and associated with multiple health issues. Such conditions present significant demands on health services, but the degree of these demands is seldom reported. This study details the utilisation of hospital services and associated costs in a single case of surfactant protein C deficiency, an example of childhood interstitial lung disease. Methods. Hospital records and case notes for a single patient were reviewed. Costs associated with inpatient services were extracted from a paediatric hospital database. Actual costs were compared to cost estimates based on both disease/procedure-related cost averages for inpatient hospital episodes and a recently implemented Australian hospital funding algorithm (activity-based funding). Results: To age 8 years and 10 months the child was a hospital inpatient for 443 days over 32 admissions. A total of 298 days were spent in paediatric intensive care. Investigations included 58 chest x-rays, 9 bronchoscopies, 10 lung function tests and 11 sleep studies. Comprehensive disease management failed to prevent respiratory decline and a lung transplant was required. Costs of inpatient care at three tertiary hospitals totalled $966,531 (Australian dollars). Disease- and procedure-related cost averages underestimated costs of paediatric inpatient services for this patient by 68%. An activity-based funding algorithm that is currently being adopted in Australia estimated the cost of hospital health service provision with more accuracy. Conclusions: Health service usage and inpatient costs for this case of rare chronic childhood respiratory disease were substantial. This case study demonstrates that disease- and procedure-related cost averages are insufficient to estimate costs associated with rare chronic diseases that require complex management. This indicates that the health service use for similar episodes of hospital care is greater for children with rare diseases than other children. The impacts of rare chronic childhood diseases should be considered when planning resources for paediatric health services. © 2014 Hime et al.; licensee BioMed Central Ltd