Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa.
Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa.
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DOI：
10.1186/s13023-023-02870-8
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发表时间：
2023-09-04
影响因子：
3.7
通讯作者：
Mayre-Chilton, Kattya
中科院分区：
文献类型：
--
作者：
Popenhagen, Mark P.;Genovese, Paola;Blishen, Mo;Rajapakse, Dilini;Diem, Anja;King, Alex;Chan, Jennifer;Pellicer Arasa, Eduard;Baird, Simone;Ferreira da Rocha, Anna Carolina;Stitt, Gideon;Badger, Kellie;Zmazek, Vlasta;Ambreen, Faiza;Mackenzie, Caroline;Price, Harper;Roberts, Toni;Moore, Zena;Patton, Declan;Murphy, Paul;Mayre-Chilton, Kattya
关键词：
遗传性大疱性表皮病（EB）是一组罕见的遗传性皮肤和粘膜脆性疾病，具有多系统和继发性影响，其中因摩擦/机械创伤而发生水疱和糜烂。考虑到这种疾病的不可治愈性和潜在的生命限制性以及其症状带来的挑战，有必要对EB相关护理采取姑息治疗方法。然而，知识和经验有关提供EB姑息治疗是最小的。需要基于证据的最佳护理指南，为从业者建立知识基础，以预防或减轻痛苦，同时改善疾病各个阶段的舒适度，而不仅仅是生命的结束。该共识指南（CG）是应DEBRA国际的要求开始的，DEBRA国际是一个致力于改善EB患者护理，研究和知识传播的国际组织，代表了姑息治疗和EB，EB患者以及为EB患者提供护理的国际医学专家小组的工作。遵循严格的循证指南制定过程，作者小组根据对EB患者、护理人员和该领域医学专家的调查结果以及对文献的详尽和系统评价确定了六种临床结果。通过对现有文献的专家组共识，为EB患者提供最佳的姑息治疗建议。本文提出了提供姑息性医疗服务的循证建议，为跨学科团队方法建立了知识和实践基础，以减轻所有EB患者的痛苦并提高其生活质量。注意到EB亚型之间提供护理的任何具体差异。由于EB尚未治愈，这种循证CG是优化和标准化IDT护理的一种手段，以减少痛苦，同时提高这种罕见且通常具有破坏性的疾病患者的舒适度和整体生活质量。在线版本包含补充材料，可通过10.1186/s13023-023-02870-8获得。
Inherited epidermolysis bullosa (EB) is a cluster of rare, genetic skin and mucosal fragility disorders with multi-system and secondary effects, in which blistering and erosions occur in response to friction/mechanical trauma. Considering the incurable and potentially life-limiting nature of the condition and the challenges posed by its symptoms, a palliative approach to EB-related care is necessary. However, knowledge and experience related to the provision of EB palliative care is minimal. Evidence-based, best care guidelines are needed to establish a base of knowledge for practitioners to prevent or ease suffering while improving comfort at all stages of the illness, not just the end of life. This consensus guideline (CG) was begun at the request of DEBRA International, an international organization dedicated to improvement of care, research, and dissemination of knowledge for EB patients, and represents the work of an international panel of medical experts in palliative care and EB, people living with EB, and people who provide care for individuals living with EB. Following a rigorous, evidence-based guideline development process, the author panel identified six clinical outcomes based on the results of a survey of people living with EB, carers, and medical experts in the field, as well as an exhaustive and systematic evaluation of literature. Recommendations for the best clinical provision of palliative care for people living with EB for each of the outcomes were reached through panel consensus of the available literature. This article presents evidence-based recommendations for the provision of palliative healthcare services that establishes a base of knowledge and practice for an interdisciplinary team approach to ease suffering and improve the quality of life for all people living with EB. Any specific differences in the provision of care between EB subtypes are noted. Because there is yet no cure for EB, this evidence-based CG is a means of optimizing and standardizing the IDT care needed to reduce suffering while improving comfort and overall quality of life for people living with this rare and often devastating condition. The online version contains supplementary material available at 10.1186/s13023-023-02870-8.
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DOI：
10.1097/jdn.0000000000000188
发表时间：
2016-01-01
影响因子：
0.4
作者：
Bodan, Rebecca Cross
通讯作者：
Bodan, Rebecca Cross
影响因子：
10.3
作者：
Dufresne, H.;Hadj-Rabia, S.;Bodemer, C.
通讯作者：
Bodemer, C.
影响因子：
3.7
作者：
Brun, Jennifer;Chiaverini, Christine;Lacour, Jean-Philippe
通讯作者：
Lacour, Jean-Philippe
影响因子：
1.5
作者：
Eismann, Emily A.;Lucky, Anne W.;Cornwall, Roger
通讯作者：
Cornwall, Roger
影响因子：
13.8
作者：
Eng, Victor A.;Solis, Daniel C.;Tang, Jean Y.
通讯作者：
Tang, Jean Y.