Background

1. Are palliative and end of life care plans being utilised to support children and young people with congenital heart disease and their families? A review of the literatureKerry Gaskin, Senior Lecturer, Children and Young People’s Nursing,Centre for Children and Families Applied Research, Coventry Universitykerry.gaskin@coventry.ac.uk Background Palliative and end of life care for children and young people is a small but growing speciality, however little has been published about the long term needs of children with complex congenital heart disease (CHD). Approximately 5,000 babies are born with CHD every year in the UK and about half of these presentations are major and life threatening, requiring surgery and life-long follow-up1. There is no epidemiological data available that specifically denotes the number of children and young people (CYP) in the UK with CHD that are living with life-limiting complex health care needs, requiring palliative and end of life care. However these numbers are probably a small percentage of the 23,500 CYP in the UK who have been diagnosed with health conditions for which there is no cure2. Furthermore, the success of paediatric cardiology, interventions and surgical techniques has resulted in a considerable increase in the population of CYP with congenital heart disease over the last 40 to 50 years. The 18 year survival rate for complex defects was 10% for those born before1960 but is 50% for those born in the 1980s3. The British Cardiac Society Working Party4 estimated that by 2010 there would be over 185,000 adults in the UK living with congenital heart disease, with over 17,000 having complex conditions. The number of cases of CHD has consequently risen dramatically, with an estimated 50% increase in complex conditions over 10 years1. It has therefore been recognised that seamless transition between children’s and adults’ services must include palliative and end of life care, but are services available to meet this growing demand? A literature search undertaken in 2011 regarding palliative care (PC) in CYP with CHD only identified papers referring specifically to the surgical palliation and not the holistic palliative treatment of these chronic conditions. The question that arises therefore is whether health care professionals are utilising appropriate palliative and end of life care plans to support CYP with CHD and their families through their chronic and sometimes complex health care journeys and transition to adult services. • Aim • The aim of this literature review was to identify • Whether Children and Young People (CYP) with Congenital Heart Disease (CHD) and their families being adequately prepared and supported during palliative/end of life care • Whether palliative and end of life care plans being integrated into the care of CYP with CHD and their families • Method • Search Strategy • An electronic search of CINAHL, (Pubmed) Medline, PsycINFO; the British Nursing Index (BNI) and Cochrane database • Primary Search May 2011, limited to Full Text, 2000-2011 and written in English • Secondary Search May 2013, limited to Full Text, 2011-2013 and written in English • A systematic approach 5 was utilised to review evidence from different paradigms • Search terms • Palliative care, end of life care, children, paediatric, pediatric, support, family support, care planning, preparation, heart, congenital heart disease, cardiac, heart failure Findings Table 3 Characteristics of the Studies • Main Themes Arising 6-18 • Need for individualised and holistic models of care • Need for independence and active involvement • Need for physical, psychological and social impact/support • Need to foster communication • Need to improve coordination of care and service delivery • Need for bereavement support [anticipatory] • Themes specific to CYP with CHD6 • Early deaths (majority in first year of life) - Mainly due to discontinuation of treatment • Environment (most died in intensive care) • Distance from home (quarter were a long distance away) • Variation in care documentation (no clarification of end of life experience) Figure 1 Systematic Approach to Search and Appraisal Primary Search May 2011 n=57 articles identified from searches n= 6 articles manual trawling of reference lists Total = 63 Critical Appraisal Stage 1 assessment of relevance Papers eliminated according to inclusion/exclusion criteria (n=46 ) Total = 10 Including CYP with CHD Conclusion Only one paper specifically considered end of life care for children with advanced heart disease6, however this was a retrospective review of medical records and therefore did not elucidate the end of life experiences of the CYP or their families. Other papers reviewed only included a small number of CYP with CHD or cardiovascular problems in their sample and mainly focused on parents needs. The synergy of current palliative and end of life care services with cardiac services for CYP with CHD therefore needs further exploration in order to ensure that professionals are giving high quality evidence based care that meets the specialist needs of this group of CYP and their families. Whilst palliative and end of life care in the UK is tending to shift toward incorporating the ACT integrated care pathways 19-21 little evidence exists to demonstrate service user and service provider perspectives on the efficacy of such an approach in enhancing the care of CYP and their families. Current research also focuses heavily on the needs of parents during end of life care; studies now need to be undertaken to identify the needs of CYP and their siblings to ensure that their needs and wishes are considered too. Search broadened to include support of children with other malignant/non-malignant diseases (n=7) Total = 17 Critical appraisal stage 2 – data extraction Papers rejected following more detailed appraisal (n=9) Total = 8 Secondary Search (May 2013) n=13 Critical Appraisal Stage 1 assessment of relevance Papers rejected according to inclusion/exclusion criteria (n=8) Total = 5 including CYP with CHD Primary and secondary search Total = 13 papers Paper specifically focusing on 'Patterns of Care at End of Life in Children with Advanced Heart Disease' 6 n= 1 References Cardio and Vascular Coalition (2008) Cardio and Vascular Coalition (CVC) Briefing Paper. Congenital Heart Disease. http://www.bhf.org.uk/plugins/PublicationsSearchResults/DownloadFile.aspx?docid=3618796d-064e-4fd1-b638-8df577b31c75&version=-1&title=Congenital+Heart+Disease+Briefing&resource=Z155 (2008, accessed June 2011) Congenital Heart Disease-United Kingdom (2011) ‘Types of Congenital Heart Disease and Operations’. http://congenital-heart-defects.co.uk/typesofchdandoperations.aspx (2011,accessed June 2011) Department of Health (2006) Adult Congenital Heart Disease. A Commissioning Guide. London: Department of Health British Cardiac Society (2002) Grown-up congenital heart (GUCH) disease: current needs and provision of service for adolescents and adults with congenital heart disease in the UK. Heart, 88 (Suppl I): i1-i14. Hawker S, Payne S, Kerr C, Hardy M, Powell J. (2002) Appraising the Evidence: Reviewing Disparate Data Systematically. 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