AimsChildren with severe neurodisability presenting with pain behaviour, retching, bloating, abdominal distension, and constipation/pseudo-obstruction can be referred to as having Gastro-Intestinal ...Dystonia. Management can be extremely challenging, with evidence base for therapy extremely limited, and a spectrum of symptomology that can be severely debilitating.1 Nabilone is a synthetic cannabinoid designed to mimic the activity of delta-9-Tetrahydrocannabinol (THC). Nabilone is a partial agonist of both CB1 and CB2 receptors, and is licensed, and indicated by NICE guidance, for use in adults as adjunctive therapy alongside standard anti-emetic for chemotherapy induced nausea and vomiting. It has also been used widely in paediatrics for the same indication, despite not being licensed for use in children.2 There has been significant interest in the use of medical cannabis to treat the spectrum of symptomology that manifest in gut dystonia yet limited published work in reporting the effectiveness of nabilone in treating gut dystonia. However, there is emerging anecdotal and case reports from clinical practice suggesting a role for nabilone.1 MethodFrom October 2022 to June 2023, patients referred and accepted to the Great Ormond Street Hospital palliative care service, for end-of-life care, were considered for use of nabilone to manage gut dystonia that had proven resistant to normal dystonia management strategies (e.g., optimisation of gabapentin, clonidine, baclofen etc.). Nabilone was initiated following approval for use by trust DTC and in discussion with the neuro-disability team. Families of patients commenced on nabilone were requested to complete a dystonia diary and reviewed weekly assessing for effectiveness and toxicity. Notes and charts were reviewed in triplicate by consultant in paediatric palliative, specialty registrar and specialist pharmacist, to ensure consensus agreement on the effectiveness of nabilone as well as any potential incidence of toxicity.ResultsOne patient was initiated on nabilone for gut dystonia during the study period. Weight at initiation of nabilone was 16.45 kg. Weight after 9 months (June 2023) was 19.45 kg. Observed dystonia and feed tolerance improved significantly, enabling the child to return to school, and there was a significant reduction in number of dystonic breakthrough medications required, from daily use of chloral hydrate to infrequent use at night. Following optimisation of nabilone, gabapentin has been weaned with no observed increase in dystonic episodes. Additionally, despite increased weight there has been no requirement to increase doses of clonidine. There were no parenteral or professional observations of any adverse effects associated with nabilone initiation or titration.ConclusionNabilone can be safely initiated for children with gut dystonia that has proven resistant to other conventional medications used for dystonia management. Use of nabilone has demonstrated a subjective and objective improvement in tone and an increase in ability to tolerate feeds with weight gain, that has persisted despite weaning of other concomitant medications.ReferencesMcConnel N, Beattie LM, Richards CE, Protheroe S, Barclay AR. Nabilone for gastro-intestinal dystonia. a single centre case series. Journal of Paediatric Gastroenterology and Nutrition 2018;66(suppl 2):1002.Nabilone capsules 250 micrograms and 1 mg capsules Summary of Product Characteristics, UK. Last Updated 07/2021. www.medicines.org.uk (Accessed 09 March 2023).
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.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
Abstract
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.
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IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
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.
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DOBA, EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, IZUM, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, SIK, UILJ, UKNU, UL, UM, UPUK, VKSCE, VSZLJ, ZAGLJ
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.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
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.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
Summary 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.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
Contents Preface s3 - - Executive summary s4 - - Introduction s6 - - Background considerations s6 Definitions s6 The extent of withholding and withdrawal of LST in paediatric practice s6 The legal ...framework s7 Statutes s7 The child and young adult s8 Parental Responsibility s8 The role of the courts in end-of-life decision making s9 Best interests s9 Quality of life and legal decisions s9 Withdrawing treatment s9 The ethical framework s10 Fundamental considerations s10 The interests of the child s10 Parental discretion responsibilities, rights, duties and power s10 Parental interests, wishes and professional duties s11 Involving children s11 Children with disabilities s11 Transition to adult services s12 Axioms on which to base best practice s12 - - The process of decision making s13 Practical considerations; substantial issues in decision making s13 To withhold, to withdraw or to limit? s13 Dealing with uncertainty s13 Situations in which it is appropriate to limit treatment s13 Spectrum of decisions and parental discretion s14 - - Practical aspects of end-of-life care: responsibilities, treatments that may be limited, appropriate or permissible actions s15 Clinical responsibilities of the healthcare team s15 The range of treatments that may be withdrawn s15 Cardiopulmonary resuscitation s15 Clinically assisted nutrition and hydration s16 Muscle relaxants and end-of-life care s16 Palliative care s16 Organ donation s17 - - Practical aspects of decision making s17 The basis of decision making by teams s17 Obtaining second opinions s17 Resource considerations s18 Communication as part of decision making s18 Resolution of different opinions s19 Medical input s19 - - Bereavement s20 Families s20 The healthcare team s20 Key goals in the provision of bereavement support s20 - - Future practicalities s21 Training s21 Resources s21 Research/audit s21 Clinical ethics services s21 - - References s21 - - Appendix 1: Suggested bereavement resources s23 Preface The first edition of the Royal College of Paediatrics and Child Health (RCPCH) document 'Withholding or Withdrawing Life Saving Treatment in Children: A Framework for Practice' was published in 1997 and was one of the first documents produced by the newly-formed College.
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