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Paediatric neuro-disability end-of-life care: symptom burden and management
  1. Carol Stephens1,
  2. Zoe Coghlan2,
  3. Louise Gibson1,3,
  4. Niamh McSweeney3,4,
  5. Olivia O'Mahony4 and
  6. Mary Jane O'Leary2,5
  1. 1Department of Paediatrics, Cork University Hospital, Cork, Ireland
  2. 2Department of Palliative Medicine, Cork University Hospital, Cork, Ireland
  3. 3Department of Paediatrics and Child Health, University College Cork, Cork, Ireland
  4. 4Department of Paediatric Neurology, Cork University Hospital, Cork, Ireland
  5. 5Department of Palliative Medicine, Marymount University Hospital & Hospice, Cork, Ireland
  1. Correspondence to Dr Carol Stephens, Department of Paediatrics, Cork University Hospital, Cork, Ireland; c.stephens552{at}

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Dear Editors,

The WHO advocates that paediatric palliative care comprises a multidisciplinary approach, beginning at the onset of disease diagnosis and carrying on throughout the illness.1 Paediatric neuro-disability and palliative care are closely intertwined. Many children with neuro-disability have complex needs, escalating at end of life (EOL). Perception and experience of symptoms can differ with varying symptom burdens depending on age and development. Management can be complicated by other non-neurological symptoms that can be non-specific and difficult to correctly identify. Poorly controlled symptoms impact quality of life, interfere with sleep, and lead to fatigue and distress. A robust team of experts is required to ensure the best care for children living and dying with neuro-disability.

We performed a retrospective single-centre observational study in Cork University Hospital, Ireland. The aim was to review symptom burden and management in children between 0 and 16 years with a background of a neuro-disability, who were referred to the palliative care team and subsequently died between 1 January 2016 and 31 May 2019. Those who died at home but had an inpatient stay within 1 month of death were included.

Twenty-nine children were referred to the service, 14 met inclusion criteria. The median age at death was 11.21 years (IQR: 4.23–15.06). Five (35.7%) died at home. This lower than national average of deaths at home was attributed to the exclusion of oncology patients as they were not cared for in our centre. The median length of time between referral and death was 10 days, (IQR: 1-3) The underlying diagnoses were cerebral palsy (n=5), undiagnosed conditions (n=4), genetic (n=3), metabolic (n=1) and neuromuscular disorder (n=1). Fifty per cent died from respiratory causes, 28.6% from end-organ failure and 7.1% died from gut failure, epileptic encephalopathy and seizures, respectively.

Neurological, pain, respiratory and gastrointestinal (GI) symptoms were reviewed. Twelve (85.7%) had epilepsy, all except one requiring ≥2 anti-epileptic drugs (AEDs). Routes of administration varied from enteral, intravenous, subcutaneous and intramuscular. Agitation and irritability were recorded in 50%, and over 60% with these symptoms also had poor seizure control. Of the patients who were observed to have both pharmaco-resistant seizures and agitation, 80% were prescribed gabapentin or pregabalin. In relation to pain, neuropathic was the most common (31.6%) followed by bone pain (21.1%). Persistent seizure activity (15.8%), dystonia (10.5%), cerebral irritability (5.3%) and GI pain (5.3%) were also described. Respiratory symptoms were encountered in 13 of 14 (92.8%) children, most commonly shortness of breath, cough or hypersalivation. Secretions often predated the EOL care episode but became more problematic. Either hyoscine hydrobromide or glycoporinium bromide was used in all patients with hypersalivation and excessive secretions. They were delivered enterally or subcutaneously. No child was ventilated in an intensive care unit setting. The most common GI symptoms were gut failure (71.4%), constipation (71.4%), and nausea and vomiting (28.6%). Nausea and vomiting was likely underestimated in non-verbal patients. All children had some form of assisted enteral feeding in place in the form of nasogastric tube or percutaneous gastrostomy tube. Given the variety of symptoms described above, it is understandable that 85.7% received some form of input from other specialty team with anaesthetics being the most common for intravenous access.

From our study, we highlight the complexity of needs in children with neuro-disability and how they can escalate at EOL. From our experience, seizure management complicated by progressive gut failure can be one of the most challenging aspects of medical care. Gut failure can be defined as a reduction in gut mass limiting appropriate digestion and absorption required for hydration and nutrition.2 There can be a hypersensitive response to stimuli in the GI tract secondary to visceral hyperalgesia.3 Symptoms can manifest themselves as nausea, vomiting, constipation and gut failure. Assisted feeding in the form of nasogastric or percutaneous gastrostomies and jejunostomies are often warranted and often become the main route of medication administration also. Deterioration in gut health can lead to reduced absorption of medications, increased aspirates, limited routes of administration and subsequent deterioration of symptom control. This can be hugely problematic in the child with complex needs who rely on regular maintenance medications and in particular in the child with epilepsy.

Seizure burden often escalates during this time with seizures becoming refractory. Routine management of seizures can also differ at EOL. Rescue medications are not administered unless there is autonomic instability, distress or a prolonged seizure, so as to limit toxicity, tolerance, oversedation and respiratory compromise. An important but delicate balance exists between seizure control and oversedation. Side-effect profiles of drugs must constantly be revisited. Choice of drug and mode of delivery of seizure medications can be limited by failing GI systems, renal and hepatic failure. Medications should be delivered enterally but failing these intravenous or subcutaneous modes of delivery may be considered. Intravenous access can become problematic and subcutaneous delivery is often the most reliable route. Seizure control can be achieved with a proactive and anticipatory approach and should never preclude a child from dying at home if it is their wish.

A collaborative team approach is essential drawing from all experiences and expertise. There are limited data on optimal drug dosing. Ongoing research and information sharing both at local and national level is key to optimising the care of this young cohort of patients. Palliative care aims to help ordinary people who find themselves and their families in extraordinary situations.4 These extraordinary situations must be matched with clinicians who possess extraordinary commitment and determination to bettering the life of that individual and their family. Our goal must forever be to ensure each child lives and dies well.5

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Patient consent for publication

Ethics approval

This study involves human participants and was approved by the Clinical Research Committee of the Cork Teaching Hospitals (reference number ECM 4(d) 28/06/2019 and ECM 4(d) 28/06/2019). Consent was deemed not required by the local ethics board.



  • Twitter @Carol_Stephens_

  • Contributors LG, NM, OO'M and MJO'L were responsible for planning the design of this study. CS and ZC were responsible for data collection. CS analysed the collected data and completed the first draft of the manuscript. All authors contributed to further edits and proofreading of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; internally peer reviewed.