Perinatal palliative care

Perinatal palliative care (PNPC) is a holistic multidisciplinary model of care for both baby and family in the event of a perinatal diagnosis of a life-limiting condition. It aims to provide optimal symptom control and end-of-life care to the baby as well as specialized support to families from diagnosis through to birth, death, and bereavement. This chapter aims to address the challenging clinical, ethical, and practical issues specific to perinatal palliative care. It describes antenatal life-limiting diagnoses, the role of anticipatory bereavement care, a palliative care approach to pregnancy, and outlines the complex planning and models of care required to optimally provide for the baby, mother, and family throughout. This chapter ultimately aims to provide management strategies to guide multidisciplinary teams (MDT) to deliver high-quality PNPC to the family as a whole.

Palliative care for children with communicable illnesses

Whilst non-communicable diseases provided the impetus for the development of children’s palliative care (CPC) in the developed world, it was a single communicable illness human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) that was the catalyst for the development of many CPC programmes in the developing world. Whilst considerable gains have been made in preventing and controlling paediatric AIDS globally, there is still benefit to an integrated palliative care approach to the care of these children and especially for those living in countries without access to anti-retrovirals. Furthermore, there are many other communicable diseases associated with significant health-related suffering in children that could benefit from palliative care. This chapter proposes grouping these diseases using the well-known Association for Children with Terminal Conditions (ACT) categorization and discusses examples of important communicable diseases in each category. The need for improved CPC as part of the humanitarian response to acute communicable disease outbreaks such as Ebola virus disease is also explored.

History and epidemiology

There has been a marked reduction in child mortality in the last few decades but there were still 6.6 million deaths in children aged 0–14 years worldwide in 2016. The vast majority of those deaths (5.6 million) occurred in children aged under 5 years and 98% of these deaths occurred in the low- and middle-income (LMCI) countries. After a brief history of the development of children’s palliative care (CPC) and key definitions, this chapter summarizes the epidemiological data on mortality and prevalence of life-limiting (LLC) and life-threatening conditions (LTC). This chapter also highlights the gaps in these data and concludes with advice on how to use local, regional, and national data to plan services.

Using medication in children’s palliative care

Using medication in children’s palliative care (CPC) is fraught with difficulties as a result of an insufficient evidence-base and a lack of child friendly formulations, however this cannot be a reason not to intervene and allow unnecessary suffering. A lack of access in some countries to even essential medicines such as opioids is an infringement of the rights of the child to pain relief and needs urgent redress. Often, the only reasonable option is to extrapolate from adult data and to proceed rationally on the basis of what the prescriber knows about the available drug, the individual child, and the likely effects of their disease. Alternative routes for administration of medicines in children who are no longer able to use the oral route, such as the trans- and sub-cutaneous and buccal route mean symptom control can be provided at home and until end of life.

Impact on the family

In this chapter, we will focus on the importance of caring for the family of a child with a life-limiting condition (LLC) or life-threatening condition as a unit, each of the family members being integral to the well-being and care of the others. We recognize that the family unit itself is embedded within a wider context including the health and social care system, and more broadly within its society and culture. We discuss the concept of family, exploring the impact of having a child with an LLC, and how families adjust to this. We then expand on considerations about how to offer effective and timely support and help. We have included parents’ narratives with the aim of adding depth to the discussion, and in recognition of the truth of families’ own experiences.

Overview of symptoms and their assessment in life-limiting illness

The aims of this chapter are to discuss the prevalence of symptoms in paediatric palliative care, the importance of accurate symptom assessment, as well as the principles of and challenges to assessing symptoms comprehensively. We examine the areas where knowledge and expertise are lacking and the reasons for this. Finally, we discuss novel approaches to assessing symptoms and discuss areas for future study. In 2018 the World Health Organization published a guidance document for healthcare workers delivering paediatric palliative care in which it describes ‘the medical and moral necessity of making palliative care accessible to all children in need and their families’.

Care in the final hours and days

Caring for a child and his or her family during the last hours and days of the child’s life is emotionally charged, with inherent pressure to ‘get things right.’ Anticipation and treatment of the patient’s changing symptoms are essential to stave off emergency situations or, at least, to be optimally prepared to deal with them. Parents must be actively supported during this time, regardless of the setting, and the availability of professional assistance must be continuous, even if this can be only by telephone in some circumstances. Access to emergency medications must be anticipated in this context. Communication must be seamless, and care providers must be prepared for sudden changes in the desired setting of care. It is much easier to plan for every contingency, knowing that some plans will go unused, than to have to cobble together suboptimal arrangements at the last minute.

Multimodal analgesia in paediatric palliative care

Pain in hospitalized children is very common, under-recognized, and under-treated. In paediatric patients the pain might be not be caused by the underlying serious medical conditions alone, but also by their frequent exposure to painful procedures (e.g. bone marrow aspirations, dressing changes, lumbar punctures, blood draws, injections, etc.). Advanced paediatric pain treatment for children with serious illness usually requires multimodal analgesia, which may include medications from different analgesic classes, procedural interventions, rehabilitation, psychological, and/or integrative (‘non-pharmacological’) therapies that usually act synergistically for more effective paediatric pain control with fewer side effects than any single analgesic or modality.

Education and school

Every child has the right to an education. Children with life-limiting illnesses or conditions aspire to normality and will often express a strong desire to return to school or continue their education, despite probable limitations. This chapter looks at how education can be offered to children with a life-limiting diagnosis and suggests how mainstream schools can work collaboratively with the child and the family to integrate or reintegrate them into their school programme. Information is provided on ways in which the school can manage risks, protect the child, and play a pivotal role in the provision of holistic and ongoing support. It also considers the need for in-depth planning and training for school staff in order to achieve safe reintegration and what must be done to provide support to siblings, classmates, and educators while the child attends school and during bereavement, should the child die.

Intensive care units

The goals of intensive care and palliative care teams may at first seem to be polar opposites. The one focuses on advanced technologies directed towards saving or prolonging life, while the other focuses on providing comfort and support, accepting death as a likely outcome. In caring for patients in the paediatric intensive care unit (PICU), however, those two teams are brought into a close working relationship. PCIU provides a natural interface for these teams with different goals. Here children and families face potential or inevitable death, and alongside attempts to save or prolong life, they need access to the kind of care that is at the heart of palliation. Where PICU and palliative care meet, children can receive the most advanced technology alongside skilled, holistic, supportive care, and both teams can work together with families to facilitate appropriate support as goals change. Palliative care is not a separate or different approach, but a continuation of the philosophy that medicine must only intervene in ways that will do more good than harm. Palliative care continues to be integrated into the care of patients and families in both the neonatal and PICU settings. With focused research aimed at healthcare service delivery, effective symptom management, and the role of palliative care consultants in the PICU, the potential to broaden and improve palliative care in the PICU will be further advanced for all children and their families. The surprise is not that these two services should be integrated, but rather that it has taken us so long to realize the importance of such integration.