Background & Aims
Inherited epidermolysis bullosa (EB) is a cluster of rare, heterogeneous, genetic skin and mucosal fragility disorders with multi-system and secondary effects, in which painful blistering and erosions occur in the skin and mucosae 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 needed. However, knowledge and experience related to the provision of palliative care for people living with EB (PLWEB) is minimal. Evidence-based, best care guidelines are needed to establish a base of knowledge for practitioners to prevent/ease suffering while improving comfort at all stages of the illness not just the end of life (EoL). The primary aim was to establish the first internationally actionable clinical practice recommendations for the provision of palliative care that applies to all PLWEB, their caregivers, and their medical practitioners.
Methods
The authors followed a rigorous, evidence-based, guideline development process using the results of a survey of PLWEB, carers, and medical experts in the field, and an exhaustive and systematic literature evaluation to identify 3 broad clinical questions and 6 clinical outcomes. A systematic literature search not limited by language, year published, publishing journal, author/study country of origin, or EB type identified 1,405 potentially relevant articles covering multiple research and participant types. Relevant articles published after the initial search were included. After twice appraising the articles, 90 were used in the synthesis of the CG. Each article was grouped into at least one of the clinical outcome categories, graded, and summarized. Panel consensus of the literature identified recommendations for the best clinical provision of palliative care for PLWEB for each outcome. The authors also incorporated feedback from a panel of 8 independent content-expert reviewers.
Results
The authors identified 73 evidence-based recommendations for the provision of palliative and EoL healthcare services that establishes a knowledge and practice base for an interdisciplinary team (IDT) approach to ease suffering and improve the quality of life for all PLWEB. Key Recommendations:
Use an IDT approach, so team members meet the PLWEB’s evolving needs, keeping the PLWEB/their carers at the care’s center. Focus treatment on achievable goals, including managing pain, itch, and emotional suffering, while respecting the PLWEB’s autonomy and individuality. Tailor an effective and appropriate pharmacologic and non-pharmacologic pain management regimen. The treatment plan for suffering related to pain and itch may change with the PLWEB’s goals and clinical trajectory.
Encourage PLWEB to live despite EB. Providing pain medications with the intent of symptom management is an ethical alternative to euthanasia, even if it hastens death. Provide a strong support system through death.
Conclusions
Palliative care for people living with inherited EB begins at diagnosis and spans their lifetime. Despite the lack of a cure for this disease, understanding that there are many potential interventions that can improve quality of life and reduce suffering. This evidence-based consensus guideline is a means of optimizing and standardizing the interdisciplinary team approach to care needed to reduce suffering while improving comfort and overall quality of life for people living with this rare and often devastating condition.
References
Selected References
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Presenting Author
Mark P. Popenhagen
Poster Authors
Mark Popenhagen
OTHR
University of Colorado School of Medicine
Lead Author
Topics
- Systematic Reviews/Meta-Analysis