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Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa
BACKGROUND: 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 natur...
Autores principales: | , , , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10476410/ https://www.ncbi.nlm.nih.gov/pubmed/37667330 http://dx.doi.org/10.1186/s13023-023-02870-8 |
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author | 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 |
author_facet | 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 |
author_sort | Popenhagen, Mark P. |
collection | PubMed |
description | BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s13023-023-02870-8. |
format | Online Article Text |
id | pubmed-10476410 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-104764102023-09-05 Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa 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 Orphanet J Rare Dis Review BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s13023-023-02870-8. BioMed Central 2023-09-04 /pmc/articles/PMC10476410/ /pubmed/37667330 http://dx.doi.org/10.1186/s13023-023-02870-8 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Review 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 Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa |
title | Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa |
title_full | Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa |
title_fullStr | Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa |
title_full_unstemmed | Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa |
title_short | Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa |
title_sort | consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10476410/ https://www.ncbi.nlm.nih.gov/pubmed/37667330 http://dx.doi.org/10.1186/s13023-023-02870-8 |
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