Top Ten Tips Palliative Care Clinicians Should Know About Prognostication in Children.

Pediatric palliative care (PPC) is different from palliative care (PC) for adults. However, conceptualizing PPC remains cumbersome due to the high heterogeneity of often rare diseases, the high diversity of disease trajectories, and the particular difficulty to predict the future of an individual, severely ill child. This article aims to provide an overview and critical reflection of different aspects of prognostication in children with PC needs. This includes different diseases from neurology to oncology, from the unborn baby to the young adult, new approaches in treatment, advance care planning, and, most important, communication with the affected child as well as parents.

A mixed-methods systematic review and meta-analysis of barriers and facilitators to paediatric symptom management at end of life.

BACKGROUND: Symptom management for infants, children and young people at end of life is complex and challenging due to the range of conditions and differing care needs of individuals of different ages. A greater understanding of these challenges could inform the development of effective interventions. AIM: To investigate the barriers and facilitators experienced by patients, carers and healthcare professionals managing symptoms in infants, children and young people at end of life. DESIGN: A mixed-methods systematic review and meta-analysis was undertaken (PROSPERO ID: CRD42019124797). DATA SOURCES: The Cochrane Library, PROSPERO, CINAHL, MEDLINE, PsycINFO, Web of Science Core Collection, ProQuest Dissertations & Theses Database, Evidence Search and OpenGrey were electronically searched from the inception of each database for qualitative, quantitative or mixed-methods studies that included data from patients, carers or healthcare professionals referring to barriers or facilitators to paediatric end-of-life symptom management. Studies underwent data extraction, quality appraisal, narrative thematic synthesis and meta-analysis. RESULTS: A total of 64 studies were included (32 quantitative, 18 qualitative and 14 mixed-methods) of medium-low quality. Themes were generated encompassing barriers/facilitators experienced by carers (treatment efficacy, treatment side effects, healthcare professionals' attitudes, hospice care, home care, families' symptom management strategies) and healthcare professionals (medicine access, treatment efficacy, healthcare professionals' demographics, treatment side effects, specialist support, healthcare professionals' training, health services delivery, home care). Only one study included patients' views. CONCLUSION: There is a need for effective communication between healthcare professionals and families, more training for healthcare professionals, improved symptom management planning including anticipatory prescribing, and urgent attention paid to the patients' perspective.

Barriers and facilitators experienced by patients, carers and healthcare professionals when managing symptoms in infants, children and young people at end-of-life: a mixed methods systematic review protocol.

INTRODUCTION: This protocol describes the objective and methods of a systematic review of barriers and facilitators experienced by patients, carers and healthcare professionals when managing symptoms in infants, children and young people (ICYP) at end-of-life. METHODS AND ANALYSIS: The Cochrane Library, PROSPERO, CINAHL, MEDLINE, PsycINFO, Web of Science Core Collection, ProQuest Dissertations & Theses Database, Evidence Search and OpenGrey will be electronically searched. Reference screening of relevant articles and inquiries to researchers in the field will be undertaken. Studies will be selected if they apply qualitative, quantitative or mixed-methods designs to explore barriers and facilitators experienced by patients, carers and healthcare professionals when managing symptoms in ICYP at end-of-life.Articles will be screened by title and abstract by one reviewer with a second reviewer assessing 10% of the articles. Both reviewers will read and screen all remaining potentially relevant articles. For included articles, one reviewer will extract study characteristics and one will check this.Both reviewers will undertake independent quality assessments of included studies using established and appropriate checklists including The Critical Appraisal Skills Programme Qualitative Checklist; The evaluative criteria of credibility, transferability, dependability and confirmability; The Quality Assessment Tool for Quantitative Studies, and The Mixed Methods Appraisal Tool. Data synthesis methods will be decided after data extraction and assessment. ETHICS AND DISSEMINATION: This review will inform our understanding of symptom management in ICYP at end-of-life. The findings will be reported in a peer-reviewed journal and presented at conferences. The study raises no ethical issues. PROSPERO REGISTRATION NUMBER: CRD42019124797.

Denosumab Therapy for Refractory Hypercalcemia Secondary to Squamous Cell Carcinoma of Skin in Epidermolysis Bullosa.

Hypercalcemia secondary to malignancy is rare in children and the majority is caused by tumor-produced parathyroid hormone-related protein (PTHrP). We report a case of hypercalcemia refractory to bisphosphonate and corticosteroid therapy, but responsive to denosumab. A 17-year-old boy with epidermolysis bullosa (EB) and advanced squamous cell carcinoma (SCC) of the left leg was referred with severe hypercalcemia (serum calcium, 4.2 mmol/L). The serum parathyroid hormone (PTH) was 0.7 pmol/L (1.1 - 6.9 pmol/L). The hypercalcemia was initially managed with hyperhydration, prednisolone and pamidronate. Following two infusions of pamidronate (1 mg/kg/dose), serum calcium fell to 2.87 mmol/L. However the hypercalcemia relapsed within a week (serum calcium, 3.61 mmol/L) needing aggressive management with intravenous fluids, prednisolone and two further doses of pamidronate. The serum calcium fell to 2.58 mmol/L over the first 4 days, but rose to 3.39 mmol/L 3 days later. As the hypercalcemia was refractory to bisphosphonate treatment, a trial dose of subcutaneous denosumab (60 mg) was administered following which the calcium fell to 2.86 mmol/L within 24 h and normocalcemia was sustained 4 days later. We report a case of refractory hypercalcemia secondary to malignant SCC, which responded well to denosumab therapy. To our knowledge, this is the first case of hypercalcemia of malignancy in an adolescent managed with denosumab.

The Spectrum of Children's Palliative Care Needs: a classification framework for children with life-limiting or life-threatening conditions.

OBJECTIVES: This paper examined the potential of a new classification framework, The Spectrum of Children's Palliative Care Needs, to facilitate identification of children with palliative care needs for the purposes of minimum data set collection and population needs assessment. METHODS: Health and social care professionals (n=50) in a range of paediatric palliative care settings applied The Spectrum to (i) clinical vignettes and (ii) consecutive children on their caseloads. They also provided confidence ratings and written comments about their experiences. Inter-rater reliability, conceptual validity, acceptability, feasibility and sustainability were examined. A subset of professionals (n=9) also participated in semistructured telephone interviews to provide further insight. RESULTS: Inter-rater reliability for the vignettes (κ=0.255) was fair. However, professionals were more confident applying The Spectrum to their caseloads, which included children (n=74) with a range of life-limiting/life-threatening conditions. The Spectrum made conceptual sense in relation to these children and was considered to offer a meaningful way to define the eligible population in service mapping. Benefits for clinical work (eg, facilitating patient review, workload management, clinical audit) and research were also identified. However, important threats to reliability were highlighted. CONCLUSIONS: Preliminary assessment of The Spectrum confirms its potential to promote consistent data set collection in children's palliative care. The results have been used to produce a revised version and user guidelines to address issues raised by participants. However, further research is required to further validate the framework and establish its relevance to families' self-defined needs.

Prognostic indicators for children and young people at the end of life: A Delphi study.

BACKGROUND: Recognizing transitions in end of life care for children is difficult and hinders communication and care planning. AIM: To identify the signs and symptoms that are most useful in signalling which children may have end of life care needs. METHODS: A Delphi study was undertaken with palliative care professionals who rated the extent to which 75 symptoms alerted them that a child/young person may have moved into his or her last (a) weeks/days, (b) 6-12 months of life using a 7-point response scale. Level of support for items was indicated by the median, and consensus was shown by the mean absolute deviation from the median. The impact of the Delphi on final agreement and consensus was also assessed. RESULTS: Second-round questionnaires were completed by 49 (89%) individuals. It was easier to identify prognostic items in the last weeks/days than earlier in the end of life trajectory. Items most indicative included failure of physiological systems, deteriorating level of consciousness, loss of autonomic control (e.g. breathing and peripheral circulation), together with a feeling of the professional that life is ending and an agreement that resuscitation would be futile. Items most indicative of last 6/12 months suggest a progressive decline in disease trajectory, increased chest infections or other complications from which the child has difficulty in making a full recovery and which may require high dependency or critical care. CONCLUSION: This study provides important insight into which signs and symptoms are considered most valuable in identifying children approaching the end of the life.

Developing the Liverpool Care Pathway for the dying child.

In most western societies the death of a child is a rare occurrence. When it does occur, it typically takes place after a period of intensive and often prolonged treatment. In light of the relative infrequency of these events in clinical practice, ensuring that all dying children and their families receive consistent and appropriate care remains a challenge. A retrospective audit of documentation of care for dying children in two paediatric units in the north-west of England illustrated that the care provided was not always documented consistently. This paper highlights work currently underway to develop an integrated care pathway for the care of the dying child based on the Liverpool Care Pathway (LCP). The aim of this work is to facilitate the delivery and recording of optimum care for all dying children and their families.