Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa

Orphanet Journal of Rare Diseases

Review

Simone Baird¹  Anna Carolina Ferreira da Rocha²  Vlasta Zmazek³  Kattya Mayre-Chilton⁴  Mo Blishen⁵  Faiza Ambreen⁶  Toni Roberts⁷  Mark P. Popenhagen⁸  Gideon Stitt⁹  Anja Diem^1,10  Dilini Rajapakse^1,11  Caroline Mackenzie^1,12  Alex King^1,13  Jennifer Chan^1,14  Paola Genovese^1,15  Harper Price^1,15  Kellie Badger^1,15  Paul Murphy^1,16  Zena Moore^1,16  Declan Patton^1,16  Eduard Pellicer Arasa^1,17 

[1] DEBRA Australia, Pittsworth, QLD, Australia;Melbourne, Australia;DEBRA Brazil, Blumenau, Santa Catarina, Brazil;Santa Catarina, Brazil;DEBRA Croatia, Zagreb, Croatia;Zagreb, Croatia;DEBRA International, Vienna, Austria;Mildmay Mission Hospital, London, UK;DEBRA New Zealand, Newtown, Wellington, New Zealand;DEBRA Pakistan, Lahore, Punjab, Pakistan;London, UK;DEBRA South Africa, Western Cape, Cape Town, South Africa;Cape Town, South Africa;Department of Anesthesiology B090, Children’s Hospital Colorado, University of Colorado School of Medicine, Anschutz Medical Campus, 13123 E 16Th Ave, 80045, Aurora, CO, USA;Section of Pediatric Anesthesiology, Children’s Hospital Colorado, Aurora, CO, USA;Division of Clinical Pharmacology, University of Utah, Salt Lake City, UT, USA;EB House Austria, Department of Dermatology and Allergology, University Hospital of the Paracelsus Medical University, Salzburg, Austria;Great Ormond Street Hospital Trust, London, UK;Guys and St Thomas’ Foundation NHS Foundation Trust, EB Adult Service, East Hampshire, England, UK;Human Sense, LLC, Phoenix, AZ, USA;Lucile Packard Children’s Hospital, Stanford, Menlo Park, CA, USA;Phoenix Children’s Hospital, Phoenix, AZ, USA;Royal College of Surgeons in Ireland, University of Medicine and Health Sciences, Dublin, Ireland;Sant Joan de Déu Barcelona Hospital, Barcelona, Spain;

关键词: Epidermolysis bullosa;    Palliative care;    End-of-life;    Quality-of-life;    Clinical practice guidelines;    Consensus guidelines;   

DOI  :  10.1186/s13023-023-02870-8

 received in 2023-01-27, accepted in 2023-08-23,  发布年份 2023

来源: Springer

【 摘 要 】

BackgroundInherited 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.MethodsThis 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.ResultsThis 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.ConclusionsBecause 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.

【 授权许可】

CC BY   
© Institut National de la Santé et de la Recherche Médicale (INSERM) 2023

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