Abstract
BackgroundOur objective was to evaluate children with metabolic diseases in paediatric palliative home care (PPC) and the process of decision-making. This study was conducted as single-centre retrospective cohort study of patients in the care of a large specialized PPC team.ResultsBetween 01/2013 and 09/2016, 198 children, adolescents and young adults were in the care of our PPC team. Twenty-nine (14.6%) of these patients had metabolic conditions. Median age at referral was 2.6 years (0–24), median duration of care 352 days (3–2248) and median number of home visits 13 (1–80). Most patients are still alive (16; 55.2%). Median number of drugs administered was 5 (range 0–12), antiepileptics were given most frequently.Symptom burden was high in all children with metabolic disorders at referral and remained high throughout care. Predominant symptoms were gastrointestinal, respiratory and neurologic symptoms.Children with metabolic conditions, who were referred to PPC younger than 1 year of age had a shorter period of care and died earlier compared to those children, who were referred to PPC later in their lives (older than 10 years of age).Eleven (37.9%) of the children initially had no resuscitation restrictions and 7 (53.8%) of those who died, did so on ICU.ConclusionsAbout 15% of children with life-limiting conditions in PPC present with metabolic diseases. Symptom burden is high with neurologic, respiratory and gastrointestinal symptoms being the most frequent and most of those being difficult to treat. In these children, particular attention needs to be addressed to advance care planning.
Highlights
Our objective was to evaluate children with metabolic diseases in paediatric palliative home care (PPC) and the process of decision-making
This study was conducted as single centre analysis of patients referred to a specialised paediatric palliative care team (PPCT) for community care between 01.01.2013– 15.09.2016
Besides children and adolescents, who are diagnosed with any life-limiting conditions (LLCs), young adults are eligible for care if the LLC was diagnosed during childhood and adolescence and is unfamiliar to adult palliative care teams [8]
Summary
Our objective was to evaluate children with metabolic diseases in paediatric palliative home care (PPC) and the process of decision-making. The number of children, adolescents and young adults with life-limiting conditions (LLCs) and, a right of paediatric palliative care (PPC) is increasing [1]. Most data on symptoms and needs of children in PPC refer to those with cancer, whereas the majority of the children is living with or dying from LLCs other than cancer [2]. In the subgroup of children with metabolic diseases such as mitochondrial disorders, Phases of disease stability change with times of acute deterioration. Often, such acute episodes come along with incomplete recovery and the children remain on a reduced new health plateau. Likewise the disease trajectory remains highly uncertain, with an overall decline of health, which often occurs slowly over months to years [5]
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