Challenges of palliative care in children with inborn metabolic diseases
Abstract Background 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 retrospective cohort study of patients in the care of a large specialized PPC team. Results Betwee...
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| Format: | Article |
| Language: | English |
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BMC
2018-07-01
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| Series: | Orphanet Journal of Rare Diseases |
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| Online Access: | http://link.springer.com/article/10.1186/s13023-018-0868-5 |
| _version_ | 1828885869613809664 |
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| author | Jessica I. Hoell Jens Warfsmann Felix Distelmaier Arndt Borkhardt Gisela Janßen Michaela Kuhlen |
| author_facet | Jessica I. Hoell Jens Warfsmann Felix Distelmaier Arndt Borkhardt Gisela Janßen Michaela Kuhlen |
| author_sort | Jessica I. Hoell |
| collection | DOAJ |
| description | Abstract Background 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 retrospective cohort study of patients in the care of a large specialized PPC team. Results Between 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. Conclusions About 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. |
| first_indexed | 2024-12-13T11:34:27Z |
| format | Article |
| id | doaj.art-3eae427a7ab5457b8c404e6377df3f2b |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-12-13T11:34:27Z |
| publishDate | 2018-07-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-3eae427a7ab5457b8c404e6377df3f2b2022-12-21T23:47:50ZengBMCOrphanet Journal of Rare Diseases1750-11722018-07-011311710.1186/s13023-018-0868-5Challenges of palliative care in children with inborn metabolic diseasesJessica I. Hoell0Jens Warfsmann1Felix Distelmaier2Arndt Borkhardt3Gisela Janßen4Michaela Kuhlen5Medical Faculty, Department of Paediatric Oncology, Haematology and Clinical Immunology, Centre for Child and Adolescent Health, University of DuesseldorfMedical Faculty, Department of Paediatric Oncology, Haematology and Clinical Immunology, Centre for Child and Adolescent Health, University of DuesseldorfMedical Faculty, Department of General Paediatrics, Neonatology and Paediatric Cardiology, Centre for Child and Adolescent Health, University of DuesseldorfMedical Faculty, Department of Paediatric Oncology, Haematology and Clinical Immunology, Centre for Child and Adolescent Health, University of DuesseldorfMedical Faculty, Department of Paediatric Oncology, Haematology and Clinical Immunology, Centre for Child and Adolescent Health, University of DuesseldorfMedical Faculty, Department of Paediatric Oncology, Haematology and Clinical Immunology, Centre for Child and Adolescent Health, University of DuesseldorfAbstract Background 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 retrospective cohort study of patients in the care of a large specialized PPC team. Results Between 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. Conclusions About 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.http://link.springer.com/article/10.1186/s13023-018-0868-5End-of-life careChildrenMetabolic disease, neurological diseasePalliative care |
| spellingShingle | Jessica I. Hoell Jens Warfsmann Felix Distelmaier Arndt Borkhardt Gisela Janßen Michaela Kuhlen Challenges of palliative care in children with inborn metabolic diseases Orphanet Journal of Rare Diseases End-of-life care Children Metabolic disease, neurological disease Palliative care |
| title | Challenges of palliative care in children with inborn metabolic diseases |
| title_full | Challenges of palliative care in children with inborn metabolic diseases |
| title_fullStr | Challenges of palliative care in children with inborn metabolic diseases |
| title_full_unstemmed | Challenges of palliative care in children with inborn metabolic diseases |
| title_short | Challenges of palliative care in children with inborn metabolic diseases |
| title_sort | challenges of palliative care in children with inborn metabolic diseases |
| topic | End-of-life care Children Metabolic disease, neurological disease Palliative care |
| url | http://link.springer.com/article/10.1186/s13023-018-0868-5 |
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