Supporting sexuality for people living with epidermolysis bullosa: clinical practice guidelines

Abstract

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.

Identifying Epidermolysis Bullosa Patient Needs and Perceived Treatment Benefits: An Explorative Study Using the Patient Benefit Index

“…The PNQ analysis also highlighted more obscure patient needs, such as to be able to have a normal sex-life, which was significantly more important in JEB compared to EBS. Sexuality in relation to EB is underrepresented in the scientific literature; however, new international EB consensus guidelines on sexuality have been published [13,52].…”

Prevalence of delayed puberty and low bone density in patients with epidermolysis bullosa: Insight from a large single center's experience

“…Areas for further research included the development of assessment and interventions strategies relevant to the adolescent with EB related to pubertal maturation. 17 The topic of puberty can be challenging for patients with chronic conditions for several reasons, but early discussion and systematic assessments can help identify abnormalities in pubertal progression before additional consequences on skeletal health become apparent. 21 Our data suggest that this is especially important in children diagnosed with RDEB as the prevalence of delayed puberty is high.…”

“… 16 Experts acknowledge that both delayed puberty and impaired bone health are significant concerns for patients with EB; however, guidance for clinicians on how to assess and when to treat is limited. 16 , 17 …”

Recommendations on pregnancy, childbirth and aftercare in epidermolysis bullosa: a consensus‐based guideline*

“…However, in severe EB subtypes complicated by mucosal fragility (e.g. RDEB, JEB and KEB), vulvovaginal involvement may result in pain during sexual intercourse and, rarely, vaginal stenosis; 12 these factors may impact on sexual function 13 . Use of water‐based lubricants may be helpful for women with vulvovaginal involvement.…”