Department of Rehabilitation, Phoenix Children's Hospital, 1919 East Thomas Rd, Phoenix, AZ, 85016, USA.
DEBRA New Zealand, 123 Daniell Street, Newtown, Wellington, 6021, New Zealand.
Orphanet J Rare Dis. 2021 Jan 6;16(1):9. doi: 10.1186/s13023-020-01640-0.
This article presents evidence-based Clinical Practice Guidelines (CPG) for the provision of healthcare services to address sexuality for people living with epidermolysis bullosa (EB). Currently, a lack of EB-specific research limits these services to sexual health assessment and intervention strategies designed for the general population. Due to the unique challenges of EB, a rare skin-fragility condition causing blistering responses to minor skin trauma and other systemic and secondary complications, condition-specific strategies are needed to support people with EB in achieving valued sexual lifestyles. This CPG represents the work of an international panel comprised of thirteen members including a medical doctor, nurses, psychologists, a social worker, an occupational therapist, and patient population involvement members living with EB. It describes the development of EB-specific recommendations for two primary domains of assessment and intervention related to sexuality: psychosocial and mechanical. Following a rigorous evidence-based guideline development process, this CPG establishes the first internationally actionable clinical practice recommendations for sexuality-related assessment and intervention for this population. Future research priorities are identified. Supplemental materials included provide additional support to clinicians in developing the necessary understanding and skills to promote equity and efficacy in this care domain.
本文提出了循证临床实践指南(CPG),以提供医疗保健服务,解决患有大疱性表皮松解症(EB)患者的性健康问题。目前,由于缺乏针对 EB 的具体研究,这些服务仅限于针对一般人群设计的性健康评估和干预策略。由于 EB 是一种罕见的皮肤脆弱性疾病,会导致轻微皮肤创伤和其他全身及继发性并发症出现水疱反应,因此需要针对特定病情的策略来支持 EB 患者实现有价值的性生活方式。本 CPG 代表了由 13 名成员组成的国际小组的工作成果,包括一名医生、护士、心理学家、社会工作者、职业治疗师和 EB 患者群体的参与成员。它描述了与性健康相关的两个主要评估和干预领域的 EB 特定建议的制定:心理社会和机械。在严格的循证 CPG 制定过程之后,本 CPG 为该人群的与性健康相关的评估和干预建立了第一个具有国际可操作性的临床实践建议。确定了未来的研究重点。补充材料为临床医生提供了更多支持,以帮助他们在这一护理领域发展必要的理解和技能,以促进公平性和有效性。