Challenges of palliative care in children with inborn metabolic diseases
decision making; gastrointestinal tract; palliative therapy; major clinical study; retrospective study; preschool child; cohort analysis; home visit; patient referral; human; article; child; female; male; controlled study; adult; gastrointestinal symptom; attention; young adult; resuscitation; advance care planning; neurologic disease; metabolic disorder
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.Copyright � 2018 The Author(s).
Hoell JI; Warfsmann J; Distelmaier F; Borkhardt A; Janssen G; Kuhlen M
Orphanet Journal of Rare Diseases
2018
<a href="http://doi.org/10.1186/s13023-018-0868-5" target="_blank" rel="noreferrer noopener">10.1186/s13023-018-0868-5</a>
Impact of Pediatric palliative care initiative at tertiary cancer care hospital of nepal
quality of life; hospice; analgesia; terminal care; cancer center; hematologic malignancy; palliative therapy; dyspnea; major clinical study; practice guideline; case study; brain tumor; conference abstract; human; child; female; male; diagnosis; gastrointestinal symptom; wound; sarcoma; Nepal
Sharma KS; Panthee S
Pediatric Blood and Cancer
2018
<a href="http://doi.org/10.1002/pbc.27455" target="_blank" rel="noreferrer noopener">10.1002/pbc.27455</a>
Survey on Neonatal End-of-Life Comfort Care Guidelines Across America
anxiety; compassion fatigue; pediatrics; intensive care; comfort; palliative therapy; Canada; Infant; Newborn; Palliative Care; bodily secretions; newborn; satisfaction; institutional review; neonatologist; practice guideline; human; terminal care; article; pain; gastrointestinal symptom; geographic distribution; neonatal nurse practitioner; perinatal care
Context: Infants of age less than one year have the highest mortality rate in pediatrics. The American Academy of Pediatrics published guidelines for palliative care in 2013; however, significant variation persists among local protocols addressing neonatal comfort care at the end-of-life (EOL). Objectives: The purpose of this study was to evaluate current neonatal EOL comfort care practices and clinician satisfaction across America. Methods: After institutional review board approval (516005), an anonymous, electronic survey was sent to members of the American Academy of Pediatrics Section on Neonatal-Perinatal Medicine. Members of the listserv include neonatologists, neonatal fellow physicians, neonatal nurses, and neonatal nurse practitioners from across America (U.S. and Canada). Results: There were 346/3000 (11.5%) responses with wide geographic distribution and high levels of intensive care responding (46.1% Level IV, 50.9% Level III, 3.0% Level II). Nearly half (45.2%) reported that their primary institution did not have neonatal comfort care guidelines. Of those reporting institutional neonatal comfort care guidelines, 19.1% do not address pain symptom management. Most guidelines also do not address gastrointestinal distress, anxiety, or secretions. Thirty-nine percent of respondents stated that their institution did not address physician compassion fatigue. Overall, 91.8% of respondents felt that their institution would benefit from further education/training in neonatal EOL care. Conclusion: Across America, respondents confirmed significant variation and verified many institutions do not formally address neonatal EOL comfort care. Institutions with guidelines commonly appear to lack crucial areas of palliative care including patient symptom management and provider compassion fatigue. The overwhelming majority of respondents felt that their institutions would benefit from further neonatal EOL care training.
Haug S; Farooqi S; Wilson CG; Hopper A; Oei G; Carter B
Journal of Pain and Symptom Management
2018
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1016/j.jpainsymman.2017.10.023" target="_blank" rel="noreferrer noopener">10.1016/j.jpainsymman.2017.10.023</a>
Surgical Intervention In Trisomy 18-supporting Pallative Care
Trisomy 18; Birth Weight; Clinical Article; Diagnosis; Female; Gastrointestinal Symptom; Heart; Hospital; Human; Infant; Intestine Obstruction; Male; Meningomyelocele; Palliative Therapy; Pathology; Pregnancy; Prognosis; Surgery; Tracheoesophageal Fistula
Aims Patients with Trisomy 18 have a poor prognosis-only 5%-10% of children survive beyond the first year; however various clinical features of Trisomy 18 such as gastrointestinal, cardiac and central nervous system anomalies may benefit from surgery. Providing intensive care including surgery must be carefully considered. We aimed to review surgical intervention in babies with Trisomy 18 and its outcome. Methods A retrospective case note review was undertaken of babies with a confirmed diagnosis of Trisomy 18 admitted to NICU over a 5 year period (01/09/2011 to 31/08/2016). Results 15 babies with Trisomy 18 were identified, 12 male and 3 female. Mean birth weight was 1.91 kg. Babies were born at a mean gestation of 39 weeks (range 31-41 weeks) (Tables 1, 2 and 3). Out of the 8 babies who had surgical pathology, 6 were stable enough to receive surgical intervention. 1 infant with a TOF/OA and 1 with complex cardiac problems were too unstable for surgery and died in hospital. Of the patients operated on, 1 baby died in theatre but the remainder were discharged home. Gastrointestinal problems were the main indications for surgery. 3 operations were undertaken for tracheo-oesophageal fistula, 2 for intestinal obstruction and 1 myelomeningocoele repair. Three of the 6 operations took place before a diagnosis of Trisomy 18 was confirmed. Conclusion Patients with Trisomy 18 who had surgical lesions that were amenable to intervention received surgery and had good short term outcomes with the majority of these patients discharged home. Surgery was performed both before and after the diagnosis of Trisomy 18 was confirmed, although it was suspected in all but one case. We therefore consider that a diagnosis of Trisomy 18 should not be a contraindication to surgical intervention. Our data shows that despite the poor long term prognosis of Trisomy 18, surgery should be considered to enable good quality palliative care, enable discharge home and improve the quality of a short life. (Table presented).
Masood Y; Spiers H; Cragie R; Edi-Osagie N
Archives Of Disease In Childhood
2017
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
10.1136/archdischild-2017-313087.503