Jan Aldridge

Rising National Prevalence of Life-Limiting Conditions in Children in England

BACKGROUND: Life-limiting conditions (LLCs) describe diseases with no reasonable hope of cure that will ultimately be fatal. For children with these diseases, palliative care services should be available but few data are available to estimate the burden of these conditions. METHODS: Children (0–19 years) with LLCs were identified within an English Hospital Episode Statistics dataset (2000/2001–2009/2010) by applying a customized coding framework of the International Classification of Diseases, 10th Revision, disease codes. Prevalence per 10u2009000 population (0–19 years) was calculated by age, diagnostic group, ethnicity, deprivation, and region for each year. RESULTS: The Hospital Episode Statistics extract contained 175u2009286 individuals with 1 or more LLCs of which congenital anomalies were the most common (31%). Prevalence increased over 10 years from 25 to 32 per 10u2009000 population. Prevalence in the South Asian (48 per 10u2009000); black (42 per 10u2009000); and Chinese, mixed, and “other” (31 per 10u2009000) populations were statistically significantly higher compared with the white population (27 per 10u2009000). Prevalence shows an inverse J-shaped relationship with 5 categories of deprivation, with the highest prevalence in the most deprived areas and the lowest in the second least deprived. CONCLUSIONS: In 2010, the prevalence of LLCs in children in England was double the previously reported estimates and had increased annually in all areas over the past decade. This clearly identifies an escalating need for specialist pediatric palliative care services. When planning services for these increasing needs, the excess prevalence in ethnic minority groups, especially in deprived areas, needs to be considered.

Palliative care in Yorkshire, UK 1987–2008: survival and mortality in a hospice

Objective To provide new epidemiological evidence base of information on models of hospice care for children and young adults. Design Retrospective cohort study of children referred to a hospice. Setting Martin House Children’s and Young Person’s Hospice in Boston Spa, North Yorkshire, UK. Participants All children who had been referred for care at Martin House Children’s Hospice since it opened in August 1987, until May 2008. Main outcome measures Demographic profiles and survival times overall and by diagnostic group classified by the Association of Children’s Palliative Care (ACT) Diagnostic Categories, calculated using the Kaplan– Meier and log rank pair-wise methodology. Results Over a 20-year period, 1554 children aged from birth to 19 years were referred to Martin House, of whom 89.5% (mean age 7.45 years) were accepted. The deprivation profile, referral source and distribution of diagnoses of these children have changed over time with recently increasing numbers of non-progressive disorders (ACT category 4). The ethnicity profile has changed with an increase in the numbers of South Asian children. The overall mean survival time was 5.6 years (95% CI 5.1 to 6.1) but this differed by ACT category. Diagnostic category was significantly associated with differing survival patterns. Conclusions There are a disproportionate number of children from areas of higher deprivation being referred for palliative care services. There has been a recent increase in the number of children from South Asian families being referred to palliative care services in Yorkshire. Survival times for children and young people receiving care from a hospice can vary from hours and days to more than 20 years.

Patterns of diagnoses among children and young adults with life-limiting conditions: a secondary analysis of a national dataset

Background: Numbers of children and young people with life-limiting conditions are rising, and increasing lifespans require young adults with life-limiting condition to transit to appropriate adult services. Aim: To describe the prevalence of life-limiting condition in children and young adults by age, sex, diagnostic group, ethnicity and deprivation. Design: A secondary analysis of the English Hospital Episode Statistics dataset was undertaken to calculate prevalence per 10,000 population. Setting/participants: Individuals (0–40 years) with life-limiting conditions were identified within an English Hospital Episode Statistics dataset by applying a customised coding framework of International Classification of Diseases, 10th Edition, disease codes. Results: There were 462,962 inpatient hospital admissions for 92,129 individual patients with a life-limiting condition. Prevalence-by-age group curve is U shaped with the highest overall prevalence in the under 1-year age group (127.3 per 10,000), decreasing until age 21–25 years (21.1 per 10,000) before rising steeply to reach 55.5 per 10,000 in the 36–40 -year age group. The distribution by diagnostic group varies by age: congenital anomalies are most prevalent in children until age 16–20 years with oncology diagnoses then becoming the most prevalent. Conclusion: Non-malignant diagnoses are common in children and young adults, and services that have historically focussed on oncological care will need to widen their remit to serve this population of life-limited patients. The diagnosis determining a patient’s life-limiting condition will strongly influence their palliative care service needs. Therefore, understanding the diagnostic and demographic breakdown of this population of teenagers and young adults is crucial for planning future service provision.

Does referral to specialist paediatric palliative care services reduce hospital admissions in oncology patients at the end of life

Background:Despite advances in the treatment of childhood cancer, some children continue to die from their disease. This study aimed to assess the impact of specialist paediatric palliative care services (SPPCSs) on the number of hospital admissions in children who subsequently died from cancer in Yorkshire, UK.Methods:An extract of patients aged 0–19 years from the Yorkshire Specialist Register of Cancer in Children and Young People (YSRCCYP) diagnosed from 1990 to 2009 were linked to inpatient hospital episodes data and a SPPCS database. Deaths were included if they occurred before 31 August 2011. Differences in hospital admission patterns were assessed using negative binomial regression and presented as incidence rate ratios (IRRs).Results:Of 2508 children on the YSRCCYP, 657 (26%) had died by the censoring date. A total of 211 children had been referred to the local SPPCS, of whom 182 (86%) had subsequently died. Referral to SPPCS was associated with a significant reduction in the rate of planned hospital admissions (IRR=0.60, 95% CI 0.43–0.85). Central nervous system tumours showed significant decreases for all planned and emergency admissions compared with all other diagnostic groups.Conclusion:Referral to SPPCS significantly reduced the number of planned hospital admissions for children and young people with cancer before their death, which are often integral to paediatric oncology treatment regimens. Overall, our findings show that SPPCS have a role in reducing hospital admissions during end of life care of paediatric cancer patients with potential personal, social and economic benefits.

A cohort study of children and young people with progressive neuromuscular disorders: clinical and demographic profiles and changing patterns of referral for palliative care.

Background: Progressive neuromuscular disease in children is life limiting and these children and young people would benefit from palliative care services, but data are limited on the number and demography of these children. Aim: To describe the clinical and demographic profile of children referred to a Children’s hospice in the UK with progressive neuromuscular disease. Setting/participants: All children and young people with progressive neuromuscular disorders referred to Martin House Children’s Hospice between 1987 and 2010. Design: Retrospective cohort study Results: 300 children with progressive neuromuscular disease were referred to the hospice. Seventy percent (210) of these children had Duchenne Muscular Dystrophy, 22% (67) had Spinal Muscular Atrophy (34 with Type I) and 8% had other neuromuscular diseases. Numbers of referrals have not significantly increased over the last 15 years, although an increasing number come from a South Asian background (from 4% to 32%) and a higher number of children have conditions other than Duchenne Muscular Dystrophy. A total of 55.3% (166) of all referrals came from areas of the highest deprivation. Survival patterns varied by diagnostic group, but ethnicity and deprivation were not associated with survival in these children. Conclusions: The profile of children with progressive neuromuscular conditions who were referred for palliative care has changed over the last 20 years, with a different spectrum of underlying diagnoses and a greater number from a South Asian background. The higher than expected proportion of children living in areas of high deprivation has been consistent over time.

Hospice provision and usage amongst young people with neuromuscular disease in the United Kingdom

AIMnTo identify the nature of services for children and young people with progressive neuromuscular disorders (NMD) provided by Childrens Hospices in the UK.nnnMETHODSnA questionnaire requesting aggregate data on the number of patients with a neuromuscular condition was sent to all childrens hospices in the UK, in addition, specific data was collected on services for young people with DMD presenting to a single local hospice.nnnRESULTSn87% of eligible hospices responded (27/31). 756 young people with an NM condition were being cared for by the hospices. These patients accounted for a mean of 17% of the total hospice population (range 5-35%). The age at which young people were required to leave the childrens hospices varied from 18 up to 35 years. 73% of visits were described as planned stays. Although end of life care is provided, few young people with NMD died in a hospice.nnnCONCLUSIONSnChildren and young people with NMD form a large proportion of the Childrens Hospices caseload. Many valued services provided by childrens hospices are not available through NHS funding. The lack of similar adult based services is a concern as increasing numbers of young people are surviving into adulthood.

‘I can't tell my child they are dying’. Helping parents have conversations with their child

This paper explores the challenges of resolving conflicting feelings around talking with a child about their terminal prognosis. When children are left out of such conversations it is usually done with good intent, with a parent wishing to protect their child from anxiety or loss of hope. There is however growing evidence that sensitive, timely, age appropriate information from those with whom children have a good relationship is helpful both for the child and their family. There is no evidence that involving children in sensitive and timely discussions creates significant problems, rather that withholding information may lead to confusion, frustration, distress and anger. The authors discuss ways in which families can be supported to have these significant conversations with their children.

Fifteen-minute consultation: Not the whole story—considering children’s spirituality and advance care planning

In a society of diverse views, faiths and beliefs, what can paediatric palliative care contribute to our understanding of children’s spirituality? By failing to recognise and respond to their spirituality in this work, we risk missing something of profound importance to children and their families. We overlook their search for wholeness in the absence of cure and fall short of offering truly holistic care. This paper explores how developments in advance care planning and related documentation are addressing these issues. Since children’s spirituality is elusive and rarely explored in practice, it aims to clarify our understanding of it with a variety of examples and contains suggestions for hearing the voice of the child amid the needs of parents and professionals.

Exploring the rewards and challenges of paediatric palliative care work – a qualitative study of a multi-disciplinary children’s hospice care team

BackgroundChildren’s hospices are a key provider of palliative care for children and young people with life-limiting and life-threatening conditions. However, despite recent policy attention to the provision of paediatric palliative care, little is known about the role of children’s hospice staff and the factors that may impact on their wellbeing at work. This study explored the rewards and challenges of working in a children’s hospice with an aim to identify staff support and development needs.MethodsWe conducted an exploratory, qualitative study involving thematic analysis of semi-structured interviews with 34 staff and three focus groups with 17 staff working in a multi-disciplinary care team in a UK children’s hospice.ResultsParticipants identified rewards and challenges related to the direct work of caring for children and their families; team dynamics and organisational structures; and individual resilience and job motivation. Participants described the work as emotionally intensive and multi-faceted; ‘getting it right’ for children was identified as a strong motivator and reward, but also a potential stressor as staff strived to maintain high standards of personalised and emotional care. Other factors were identified as both a reward and stressor, including team functioning, the allocation of work, meeting parent expectations, and the hospice environment. Many participants identified training needs for different aspects of the role to help them feel more confident and competent. Participants also expressed concerns about work-related stress, both for themselves and for colleagues, but felt unable to discuss this at work. Informal support from colleagues and group clinical reflection were identified as primary resources to reflect on and learn from work and for emotional support. However, opportunities for this were limited.ConclusionsProviding regular, structured, and dedicated clinical reflection provides a mechanism through which children’s hospice staff can come together for support and learning, and demonstrates an organisational commitment to staff wellbeing and development. Being aware of children’s hospice specific rewards and challenges can help to ensure that staff feel supported and competent in their role. Breaking down barriers to discussing work-related stress and enhancing awareness about early signs of burnout is also important.