Supportive care in children with spinal muscular atrophy type 1: Results from a french multicentric study
death; child; female; human; major clinical study; male; palliative therapy; controlled study; conference abstract; prospective study; caregiver; clinical research; drug therapy; France; gastrostomy; history; intensive care unit; new drug; noninvasive ventilation; nusinersen; nutrition supplement; physician; sedation; sedative agent; standardization; Werdnig Hoffmann disease
Introduction: This National Hospital Clinical Research Program (PHRC) was conducted in France between 2012 and 2016 to depict palliative practices in spinal muscular atrophy type 1 (SMA-1). New drugs (Nusinersen) have been developed and may modify its natural history. We thus present data about supportive care for patients included in that PHRC, comparing them to patients not included concomitantly. Patients and Methods: Supportive care data (enteral nutrition, non-invasive ventilation [NIV], sedation), age and place of death were collected prospectively from a specific health-book and a survey about conditions of death for the patients included in the PHRC and retrospectively by questioning physicians of the French Pediatric Neuromuscular Network for the other patients, some of them receiving Nusinersen. Result(s): In 18 centres, 38 patients were included in the PHRC, 43 were not; including 7 receiving Nusinersen. Mean age at diagnosis was 3.9 months (SD 2.4). 77 patients died at 7.5 months (SD 4,96), 32% at home, 8% in an intensive care unit. 85% patients received enteral nutrition, some through a gastrostomy (8%). 16% had a NIV. 70% received sedative treatment. No statistical difference was found between the two groups. However only all 3 patients (4%) receiving Nusinersen had both a gastrostomy and a NIV, without any sedation. Conclusion(s): Our data confirm that palliative care is essential in the management of ASI-1 patients, that is so far still a fatal disorder. Our data suggest that Nusinersen treatment was accompanied by more invasive supportive care, claiming for a standardization of practices in the children and caregivers' best-interest.
Hully M; Barnerias C; Chabrol B; Vuillerot C; Sabouraud P; Cuisset J M; Cances C; Ropars J; Rivier F; Isapoff A; Desguerre I
Developmental Medicine and Child Neurology
2019
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/10.1111/dmcn.14244" target="_blank" rel="noreferrer noopener">10.1111/dmcn.14244</a>
Pediatric palliative care and pediatric medical ethics: opportunities and challenges
adolescent; Child; Humans; infant; Pediatrics; Withholding Treatment; Communication; Resuscitation Orders; Ethics; Medical; Palliative Care; 20th Century; History
The fields of pediatric palliative care (PPC) and pediatric medical ethics (PME) overlap substantially, owing to a variety of historical, cultural, and social factors. This entwined relationship provides opportunities for leveraging the strong communication skills of both sets of providers, as well as the potential for resource sharing and research collaboration. At the same time, the personal and professional relationships between PPC and PME present challenges, including potential conflict with colleagues, perceived or actual bias toward a palliative care perspective in resolving ethical problems, potential delay or underuse of PME services, and a potential undervaluing of the medical expertise required for PPC consultation. We recommend that these challenges be managed by: (1) clearly defining and communicating clinical roles of PPC and PME staff, (2) developing questions that may prompt PPC and PME teams to request consultation from the other service, (3) developing explicit recusal criteria for PPC providers who also provide PME consultation, (4) ensuring that PPC and PME services remain organizationally distinct, and (5) developing well-defined and broad scopes of practice. Overall, the rich relationship between PPC and PME offers substantial opportunities to better serve patients and families facing difficult decisions.
2014-02
Feudtner C; Nathanson PG
Pediatrics
2014
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).
Journal Article
<a href="http://doi.org/10.1542/peds.2013-3608B" target="_blank" rel="noreferrer">10.1542/peds.2013-3608B</a>
Judging the quality of mercy: drawing a line between palliation and euthanasia
Child; Humans; Pediatrics; Euthanasia; Ethics; Suicide; Medical; Palliative Care; Assisted; Passive; 20th Century; History
Clinicians frequently worry that medications used to treat pain and suffering at the end of life might also hasten death. Intentionally hastening death, or euthanasia, is neither legal nor ethically appropriate in children. In this article, we explore some of the historical and legal background regarding appropriate end-of-life care and outline what distinguishes it from euthanasia. Good principles include clarity of goals and assessments, titration of medications to effect, and open communication. When used appropriately, medications to treat symptoms should rarely hasten death significantly. Medications and interventions that are not justifiable are also discussed, as are the implications of palliative sedation and withholding fluids or nutrition. It is imperative that clinicians know how to justify and use such medications to adequately treat suffering at the end of life within a relevant clinical and legal framework.
2014-02
Morrison WE; Kang TI
Pediatrics
2014
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).
Journal Article
<a href="http://doi.org/10.1542/peds.2013-3608F" target="_blank" rel="noreferrer">10.1542/peds.2013-3608F</a>
The Neurontin legacy--marketing through misinformation and manipulation
Humans; United States; United States Food and Drug Administration; 20th Century; 21st Century; History; Amines/history; Cyclohexanecarboxylic Acids/history; Drug Industry/ethics/history; Drug Labeling/history/legislation & jurisprudence; gamma-Aminobutyric Acid/history; Hal's Folder; Marketing/ethics/history/legislation & jurisprudence
2009
Landefeld CS; Steinman MA
The New England Journal Of Medicine
2009
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).
Journal Article
<a href="http://doi.org/10.1056/NEJMp0808659" target="_blank" rel="noreferrer">10.1056/NEJMp0808659</a>
The Holocaust as a context for telling life stories
Female; Humans; Male; Aged; Middle Aged; Survivors; Aging; Adolescent Transitions; 20th Century; History; Holocaust; Narration/history
Using a narrative approach, this study explores the role of the Holocaust in the life stories of Survivors, contrasted with two comparison groups (one Jewish and one non-Jewish) whose direct experiences did not include surviving the Holocaust. Using the technique of the life line and measures such as number and type of life events identified, as well as the events marking the beginning and ending of the life story, several differences were found between the three groups. Survivors identified an average of 10 life events, fewer than the non-Jewish comparison group (18) but more than the Jewish comparison group (7). Most of these events were positive, although less so for the Jewish comparison group, with very few future events identified by any of the groups. The War marked the beginning of the life story for most of the survivors and their stories ended at an earlier age than did the stories of the comparison groups. Further, WWII events predominated in the stories of the survivors, as did family births and relationship events (comparably seen in the stories of the Jewish comparisons). In contrast, the comparison groups, and particularly the non-Jewish group, identified greater numbers of career, education, illness, and family death events. It is proposed that the pronounced effect of the Holocaust in the life stories of survivors (and to a lesser, though still evident, degree of the Jewish comparisons) serves as an anchoring and contextual influence on the nature and quality of life stories told. That is, the Holocaust sets the standard for what events merit mention and further determines the nature of the events reported and their distribution.
2005
de Vries B; Suedfeld P; Krell R; Blando JA; Southard P
International Journal Of Aging & Human Development
2005
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).
Journal Article
<a href="http://doi.org/10.2190/tfha-d5k5-kqkk-8de4" target="_blank" rel="noreferrer">10.2190/tfha-d5k5-kqkk-8de4</a>
Family beliefs regarding the nutritional care of a terminally ill relative: a qualitative study
Female; Humans; Male; Palliative Care; Adult; Canada; Middle Aged; Nutritional Support; Practice; adolescent; Non-U.S. Gov't; Research Support; Interviews; Terminally Ill/psychology; Attitudes; Family/psychology; Health Knowledge; Neoplasms/psychology/therapy; History; 18th Century
OBJECTIVE: To develop a conceptual model of family caregiver beliefs and behavior related to nutritional care of the terminally ill by examining the perspectives of family members, patients, and health care providers. DESIGN: Qualitative study using in-depth, open-ended, face to face interviews, participant observation, and content analysis. PARTICIPANTS: A total of 47 participants from 4 groups: hospitalized palliative patients with cancer (n = 13), family members of hospitalized palliative patients with cancer (n = 13), health care providers on a palliative care unit (n = 11), and bereaved family members who had previously experienced the death of a relative on a palliative care unit. OUTCOME MEASURES: Participant views on nutritional care in the terminal phases of illness. RESULTS: There is marked variability in the ways family members respond to a dying cachectic anorexic relative. The overarching theme of "doing what's best" captures this variability and integrates the major categories into the key analytic model emerging from this study. CONCLUSION: We have developed a conceptual model of family beliefs regarding the nutritional care of a hospitalized terminally ill relative. The model has implications for education, practice, and research regarding nutritional care in the palliative care setting.
2003
McClement SE; Degner LF; Harlos MS
Journal Of Palliative Medicine
2003
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).
Journal Article
<a href="http://doi.org/10.1089/109662103322515248" target="_blank" rel="noreferrer">10.1089/109662103322515248</a>
Palliative care in neonatal period and ethical issues
medical ethics; newborn care; palliative therapy; clinical decision making; communication skill; ethical decision making; family attitude; History; Human; morality; Newborn; newborn period; nurse attitude; Perinatal Care; physician attitude; review; Terminal Care
Perinatal palliative care is the active total care of the fetus who has been diagnosed with a life-limiting condition with his/her entirety and also involves giving support to the family. It begins when illness is diagnosed, and continues regardless of whether or not a child receives treatment directed at the disease. Effective palliative care requires a broad multidisciplinary approach that includes the family and makes use of available community resources; it can be successfully implemented even if resources are limited, various barriers to provide palliative care were identified ranging from ethical, moral and attitudinal of physicians, nurses and families. These problems can be solved with providing for the education and training needs of physicians, GPs and nurses in aspects of palliative care, decision making in end-of-life situations, and communication skills.
Turkcapar AF
Anestezi Dergisi
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).
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