Specialist perinatal palliative care: a retrospective review of antenatal referrals to a children's palliative care service over 14 years.

BACKGROUND: Perinatal palliative care is an emerging branch of children's palliative care. This study sought to better understand the pattern of antenatal referrals and the role of a specialist paediatric palliative care (PPC) team in supporting families throughout the antenatal period. METHODS: A single-centre retrospective chart review of all antenatal referrals to a quaternary children's palliative care service over a 14-year period from 2007 to 2021. RESULTS: One hundred fifty-nine antenatal referrals were made to the PPC team over a 14-year period, with increasing referrals over time. Referrals were made for a broad spectrum of diagnoses with cardiac conditions (29% of referrals) and Trisomy 18 (28% of referrals) being the most prevalent. 129 referrals had contact with the PPC team prior to birth and 60 had a personalised symptom management plan prepared for the baby prior to birth. Approximately one third (48/159) died in utero or were stillborn. Only a small number of babies died at home (n = 10) or in a hospice (n = 6) and the largest number died in hospital (n = 72). 30 (19% of all referrals) were still alive at the time of the study aged between 8 months and 8 years. CONCLUSIONS: Specialist PPC teams can play an important role in supporting families during the antenatal period following a diagnosis of a life-limiting fetal condition and demand for this service is increasing. A large proportion of the cases referred will not survive to the point of delivery and a number of babies may survive much longer than predicted. PPC teams can be particularly helpful navigating the uncertainty that exists in the antenatal period and ensuring that plans are made for the full spectrum of possible outcomes.

Embracing paediatric palliative care in paediatric oncology from diagnosis onwards.

Paediatric palliative care aims to support children and young people with life-limiting or life-threatening conditions, and their families, from the time of diagnosis. Early integration within oncology has been recognised as having benefits for all involved, whatever the outcome may be. Through improved communication and advance care planning, it enables user-centred care, where concerns about quality of life, preferences and values are given the same relevance as cutting-edge therapy. Challenges to the integration of palliative care within paediatric oncology include raising awareness and providing education, whilst searching for the best care model and adapting to an ever-changing therapeutic scenario.

Paediatric palliative care in the NICU: A new era of integration.

We are entering a new era of integration between neonatal medicine and paediatric palliative care, with increasing recognition that the role and skills of palliative care extend beyond care of only the terminally ill infant. This paper addresses the principles of paediatric palliative care and how they apply in the NICU, considers who provides palliative care in this setting and outlines the key components of care. We consider how the international standards of palliative care pertain to neonatal medicine and how a fully integrated approach to care may be realised across these two disciplines. Palliative care is so much more than end-of-life care, offering a proactive and holistic approach which addresses the physical, emotional, spiritual and social needs of the infant and family. This is a truly interdisciplinary endeavour, relying on a harmonisation of the skills from both the neonatal and palliative care teams to deliver high-quality coordinated care.

International Standards for Pediatric Palliative Care: From IMPaCCT to GO-PPaCS.

CONTEXT: Since the publication of the IMPaCCT project in 2007, much effort has been made to develop new approaches to pediatric palliative care (PPC). Fifteen years later, it is time to redefine the standards in PPC. OBJECTIVES: An international group of experts in PPC has revised the standards in PPC through the GO-PPaCS project (Global Overview - PPC Standards). The goal was to update the PPC standards considering the specificity of different settings, resources, and emerging challenges. The present document is intended to reach all people directly or indirectly involved in PPC. METHODS: A literature review in MEDLINE was conducted to expand on the fundamental points and current standards on PPC and to cover an international setting. The literature search (updated on the 15th of April 2021) was carried out using different combinations of keywords and focusing on papers published in English over the past 5 years (2016-2020), but older articles were considered when relevant. The consensus on the fundamental points, standards of care and paper contents was reached by open discussion. RESULTS: Fundamental points were defined regarding the definition of PPC, eligibility criteria and the magnitude of the need for PPC, while standards were redefined for the following six areas: 1) clinical, developmental, psychological, social, ethical and spiritual needs; 2) end-of-life care; 3) care models and settings of care; 4) PPC in humanitarian emergencies; 5) care tools; and 6) education and training for healthcare providers. CONCLUSION: The present document, developed with the contribution of an international group of experts from different countries, experiences and models of care, provides fundamental points and standards for a wider implementation of PPC worldwide.

Palliative care for children and young people with stage 5 chronic kidney disease.

Death from stage 5 chronic kidney disease (CKD 5) in childhood or adolescence is rare, but something that all paediatric renal physicians and most paediatricians will encounter. In this paper, we present the literature on three key areas of palliative care practice essential to good clinical management: shared decision-making, advance care planning, and symptom management, with particular reference to CKD 5 where kidney transplant is not an option and where a decision has been made to withdraw or withhold dialysis. Some areas of care, particularly with regard to symptom management, have not been well-studied in children and young people (CYP) with CKD 5 and recommendations with regard to drug choice and dose modification are based on adult literature, known pharmacokinetics, and clinical experience.

Experiences of children with trisomy 18 referred to pediatric palliative care services on two continents.

Children with trisomy 18 that survive beyond the neonatal period have multiple congenital anomalies, neurodevelopmental disability, and high mortality rates. The experience of children with trisomy 18 who receive pediatric palliative care services is largely unknown. We conducted a retrospective review of children with trisomy 18 receiving pediatric palliative care services at both Boston Children's Hospital, USA and Great Ormond Street Hospital, UK from January 1, 2004 to January 1, 2015. Fifty-eight children with trisomy 18 were referred to pediatric palliative care, 38 in the United Kingdom, 20 in the United States. Median age at referral was 19 days (2-89) in the United Kingdom, and 25 days (1-463) in the United States. Median length of time being followed by pediatric palliative care was 32 days (1-1,637) in the United Kingdom and 67 days (3-2,442) in the United States. The only significant difference in the two cohorts (p = .001) was in likelihood of receiving cardiac surgical intervention-37% in the United States, 0% the United Kingdom. Children with trisomy 18 receive pediatric palliative care services, with variable age at referral and for a variable length of time. Further research is needed to understand the experience of children with trisomy 18 and their families receiving pediatric palliative care services.

Palliative medicines for children - a new frontier in paediatric research.

OBJECTIVES: This paper seeks to highlight from a UK perspective the current lack of a research evidence base in paediatric palliative care that has resulted in a paucity of available medicines with appropriate formulations (strength and dosage form) to provide symptom management for children with life-limiting illnesses and to raise awareness of this group of 'therapeutic orphans'. Currently, clinicians have limited, often unsuitable medication choices for their paediatric palliative care patients, with little hope of moving away from the status quo. KEY FINDINGS: Most medicines used in children receiving palliative care are old and off-patent drugs, developed for and tested in an adult population. Many are not available in suitable formulations (dosage form and strength) for administration to children, and there are often no age-related profiles of adverse drug reactions or for safe dosing. SUMMARY: Existing regional paediatric palliative care networks and support organisations should lobby funding bodies and the academic community to support appropriate research for this group of therapeutic orphans. Support must also be provided to pharmaceutical companies in the development of suitable products with appropriate formulations.

Palliative and end-of-life care for children with diffuse intrinsic pontine glioma: results from a London cohort study and international survey.

BACKGROUND: More than 90% of patients with diffuse intrinsic pontine glioma (DIPG) will die within 2 years of diagnosis. Patients deteriorate rapidly during the disease course, which severely impairs their quality of life. To date, no specific research on this clinically important subject has been conducted. This study aimed to compile an inventory of symptoms experienced, interventions applied, and current service provision in end-of-life care for DIPG. METHODS: We performed a retrospective cohort study of children with DIPG, aged 0-18 years, who received treatment under the care of 2 London hospitals. Symptoms, interventions, and services applied during the 12 weeks before death were analyzed. In addition, we conducted a global questionnaire-study among health care professionals. RESULTS: In more than 78% of DIPG patients, problems concerning mobility, swallowing, communication, consciousness, and breathing arose during end-stage disease. Supportive drugs were widely prescribed. The use of medical aids was only documented in <15% of patients. Palliative and end-of-life care was mostly based on the health care professional's experience; only 21% of the questionnaire respondents reported to have a disease-specific palliative care guideline available. CONCLUSIONS: This research assessed the current state of palliative and end-of-life care for children with DIPG. Our results show the variability and complexity of symptoms at end-stage disease and the current lack of disease-specific guidelines for this vulnerable group of patients. This first descriptive paper is intended to act as a solid basis for developing an international clinical trial and subsequent guideline to support high-quality palliative and end-of-life care.

Management of respiratory symptoms in paediatric palliative care.

PURPOSE OF REVIEW: Respiratory symptoms in children with life-limiting and life-threatening conditions are common, distressing and have a lasting impact, yet there is a paucity of evidence to guide clinicians in their management. This article makes a series of recommendations for the management of the most frequent and distressing respiratory symptoms encountered in paediatrics (dyspnoea, cough, haemoptysis and retained secretions) with attention to the evidence from research. RECENT FINDINGS: There are very few paediatric studies exploring any aspect of respiratory symptoms in paediatric palliative care, so much of the evidence base has been drawn from adult studies, few of which have been published in the past 12-18 months. SUMMARY: In the absence of well designed paediatric studies we need to judiciously apply what we can extrapolate from adult studies to each child we are treating; selecting interventions and approaches carefully, adjusting them when there is no evidence of individual benefit.

Experiences of healthcare professionals in the community dealing with the spiritual needs of children and young people with life-threatening and life-limiting conditions and their families: report of a workshop.

OBJECTIVE: We sought to understand how healthcare professionals (HCP) conceptualise spirituality among seriously ill children and young people (CYP) and their families, and their experiences in dealing with spiritual issues that emerge in practice. METHOD: We analysed thematically presentations and small group discussions with HCP that took place as part of a day-long workshop exploring the place of spirituality in paediatric healthcare. RESULTS: (1) HCP conceptualised spirituality as highly individualised searches for meaning, hope and connectedness to self, others and the world. They saw spirituality within a developmental context. (2) HCP described spiritual concerns that were tied to their own conceptualisations of spirituality, centring on ideas of loss, including loss of hope or meaning. (3) HCP approached spiritual concerns of CYP and families by 'being there' and supporting spiritual enquiry. (4) Challenges to their work included managing hopes of CYP and families in the face of poor prognoses, discussions about miracles and issues with their own faith. Spiritual care was seen as different to other areas of care which HCP felt had a greater prescription in delivery. CONCLUSIONS: The findings underscore the complexity of spirituality in times of illness and the challenges faced in its management. HCP should be alerted to the myriad ways spirituality emerges in serious illness and opportunities for developing confidence in attending to spiritual issues with CYP and families through training. Research should explore with patients, families and HCP how tensions among CYP, autonomy, the maintenance of hope and miracle beliefs are best approached within care.

Withdrawal of ventilatory support outside the intensive care unit: guidance for practice.

OBJECTIVE: To review the work of one tertiary paediatric palliative care service in facilitating planned withdrawal of ventilatory support outside the intensive care setting, with the purpose of developing local guidance for practice. METHODS: Retrospective 10-year (2003-2012) case note review of intensive care patients whose parents elected to withdraw ventilation in another setting. Demographic and clinical data revealed common themes and specific incidents relevant to local guideline development. RESULTS: 18 children (aged 2 weeks to 16 years) were considered. Three died prior to transfer. Transfer locations included home (5), hospice (8) and other (2). Primary pathologies included malignant, neurological, renal and respiratory diseases. Collaborative working was evidenced in the review including multidisciplinary team meetings with the palliative care team prior to discharge. Planning included development of symptom management plans and emergency care plans in the event of longer than anticipated survival. Transfer of children and management of extubations demonstrated the benefits of planning and recognition that unexpected events occur despite detailed planning. We identified the need for local written guidance supporting healthcare professionals planning and undertaking extubation outside the intensive care setting, addressing the following phases: (i) introduction of withdrawal, (ii) preparation pretransfer, (iii) extubation, (iv) care postextubation and (v) care postdeath. CONCLUSIONS: Planned withdrawal of ventilatory support outside the intensive care setting is challenging and resource intensive. The development of local collaborations and guidance can enable parents of children dependent on intensive care to consider a preferred place of death for their child, which may be outside the intensive care unit.

Underlying barriers to referral to paediatric palliative care services: knowledge and attitudes of health care professionals in a paediatric tertiary care centre in the United Kingdom.

Referrals to children's palliative care services typically occur late in the illness trajectory, with many children who would benefit not referred at all. Previous studies report health care professionals' (HCPs) assessment of various parent-related factors as barriers to referral. We conducted a cross-sectional survey of HCPs working in a paediatric tertiary care hospital in the United Kingdom, with an established paediatric palliative care team, to explore staff perceptions of barriers, knowledge and attitudes, with the aim of developing interventions to increase patient access to palliative care services. Survey respondents evidenced good knowledge of the principles of palliative care in closed questions, but their attitudes expressed in open-text questions and reported reasons to refer to a palliative care service demonstrated an association of palliative care with death and dying. We suggest that the association of palliative care with end of life may be a modifiable factor relevant to late and non-referral and deserving of further investigation and attention in education and training.

Palliative care education in neonatal units: impact on knowledge and attitudes.

OBJECTIVE: To facilitate improved and earlier access to palliative care for babies, a 4-h workshop on the principles, practice and resources in palliative care was delivered in 21 neonatal units across London. This paper assesses the impact of these workshops. DESIGN: As part of mixed methods, processual evaluation of a series of education workshops, a pre and post survey instrument was developed, consisting of open and closed questions assessing knowledge of services and attitudes towards palliative care. Before and after each workshop attendees were invited to complete the survey. SETTING: Neonatal units in hospitals across London, including examples of intensive care, high dependency and special care units. RESULTS: 331 healthcare professionals attended the sessions, and 264 (80%) completed questionnaires. The majority of attendees were experienced neonatal nurses working in neonatal intensive care  (61% more than 7 years) but with limited experience of palliative care services. Over 79% of respondents reported that the workshop completely met their learning needs. Post-session 80% of respondents felt confident referring to palliative care services, compared with 46% before (p<0.001). Analysis of open and closed text responses revealed a shift in attitude after the sessions from a focus on dying/end-of-life, towards integrating palliative care as part of a holistic treatment plan. CONCLUSIONS: A short (half day), locally delivered workshop can change attitudes and increase knowledge in neonatal staff. Such a workshop can serve as a first step in facilitating access to and utilisation of a variety of palliative care services.

Can we truly offer a choice of place of death in neonatal palliative care?

In the developed world, more than 90% of neonatal deaths occur in hospital and most deaths on the neonatal unit follow the planned withdrawal of ventilation. In this paper we look at what choices of place of death can be considered, the support that is available outside the hospital environment and the practicalities of achieving parental choice. We conclude that choices of place of death are usually possible, although there may be practical or resource restraints that affect which choices are available or can be achieved. Where choice is currently offered, the proportion of hospital deaths is much lower than national statistics suggest. Sadly, it is likely that the high proportion of hospital deaths currently reported reflects not that choice is unavailable, but that choice is not offered.

Pediatric palliative care: use of opioids for the management of pain.

Pediatric palliative care (PPC) is provided to children experiencing life-limiting diseases (LLD) or life-threatening diseases (LTD). Sixty to 90% of children with LLD/LTD undergoing PPC receive opioids at the end of life. Analgesia is often insufficient. Reasons include a lack of knowledge concerning opioid prescribing and adjustment of opioid dose to changing requirements. The choice of first-line opioid is based on scientific evidence, pain pathophysiology, and available administration modes. Doses are calculated on a bodyweight basis up to a maximum absolute starting dose. Morphine remains the gold standard starting opioid in PPC. Long-term opioid choice and dose administration is determined by the pathology, analgesic effectiveness, and adverse effect profile. Slow-release oral morphine remains the dominant formulation for long-term use in PPC with hydromorphone slow-release preparations being the first rotation opioid when morphine shows severe adverse effects. The recently introduced fentanyl transdermal therapeutic system with a drug-release rate of 12.5 microg/hour matches the lower dose requirements of pediatric cancer pain control. Its use may be associated with less constipation compared with morphine use. Though oral transmucosal fentanyl citrate has reduced bioavailability (25%), it inherits potential for breakthrough pain management. However, the gold standard breakthrough opioid remains immediate-release morphine. Buprenorphine is of special clinical interest as a result of its different administration routes, long duration of action, and metabolism largely independent of renal function. Antihyperalgesic effects, induced through antagonism at the kappa-receptor, may contribute to its effectiveness in neuropathic pain. Methadone also has a long elimination half-life (19 [SD 14] hours) and NMDA receptor activity although dose administration is complicated by highly variable morphine equianalgesic equivalence (1 : 2.5-20). Opioid rotation to methadone requires special protocols that take this into account. Strategies to minimize adverse effects of long-term opioid treatment include dose reduction, symptomatic therapy, opioid rotation, and administration route change. Patient- or nurse-controlled analgesia devices are useful when pain is rapidly changing, or in terminal care where analgesic requirements may escalate. In this article, we present detailed pediatric pharmacokinetic and pharmacodynamic data for opioids, their indications and contraindications, as well as dose-administration regimens that include practical strategies for opioid switching and dose reduction. Additionally, we discuss the problem of hyperalgesia and the use of adjuvant drugs to support opioid therapy.

Paediatric palliative care: coming of age in oncology?

Palliative care in children has been emerging as a clinical subspecialty of paediatrics for many years. It requires the knowledge and experience of a paediatrician, combined with the skills of a palliative care specialist. Both are essential, as a paediatrician may not have advanced knowledge of palliative care and a palliative care specialist is unlikely to be familiar with the complexity of working with families where the child is the patient. This paper reviews recent literature and discusses advances in the development of palliative care services for children and young people with incurable cancer. It highlights key areas where paediatric palliative care differs from that of adults and outlines the barriers to providing palliation and conducting evidence-based research in children and young people dying from cancer.

Characteristics of deaths occurring in hospitalised children: changing trends.

BACKGROUND: Despite a gradual shift in the focus of medical care among terminally ill patients to a palliative model, studies suggest that many children with life-limiting chronic illnesses continue to die in hospital after prolonged periods of inpatient admission and mechanical ventilation. OBJECTIVES: To (1) examine the characteristics and location of death among hospitalised children, (2) investigate yearwise trends in these characteristics and (3) test the hypothesis that professional ethical guidance from the UK Royal College of Paediatrics and Child Health (1997) would lead to significant changes in the characteristics of death among hospitalised children. METHODS: Routine administrative data from one large tertiary-level UK children's hospital was examined over a 7-year period (1997-2004) for children aged 0-18 years. Demographic details, location of deaths, source of admission (within hospital vs external), length of stay and final diagnoses (International Classification of Diseases-10 codes) were studied. Statistical significance was tested by the Kruskal-Wallis analysis of ranks and median test (non-parametric variables), chi(2) test (proportions) and Cochran-Armitage test (linear trends). RESULTS: Of the 1127 deaths occurring in hospital over the 7-year period, the majority (57.7%) were among infants. The main diagnoses at death included congenital malformations (22.2%), perinatal diseases (18.1%), cardiovascular disorders (14.9%) and neoplasms (12.4%). Most deaths occurred in an intensive care unit (ICU) environment (85.7%), with a significant increase over the years (80.1% in 1997 to 90.6% in 2004). There was a clear increase in the proportion of admissions from in-hospital among the ICU cohort (14.8% in 1998 to 24.8% in 2004). Infants with congenital malformations and perinatal conditions were more likely to die in an ICU (OR 2.42, 95% CI 1.65 to 3.55), and older children with malignancy outside the ICU (OR 6.5, 95% CI 4.4 to 9.6). Children stayed for a median of 13 days (interquartile range 4.0-23.25 days) on a hospital ward before being admitted to an ICU where they died. CONCLUSIONS: A greater proportion of hospitalised children are dying in an ICU environment. Our experience indicates that professional ethical guidance by itself may be inadequate in reversing the trends observed in this study.