Living with epidermolysis bullosa: Daily challenges and health‐care needs

Abstract

Background & Objective Epidermolysis bullosa (EB) is the term used for a group of genetic skin fragility disorders. For those living with EB, pain represents a constant challenge, with blistering and tasks such as changing dressings, adding to the distress. This paper focuses on describing and exploring the health‐care needs of children, adults and families who are affected by EB. The specific aim of the paper is to identify the needs of the EB population with a view towards informing the development of a community liaison service to support adults living with EB and the parents/carers of children living with EB. Setting and Participants Interviews with six adults and the parents of eight children with EB were conducted. The data were analysed thematically. All participants were resident on the island of Ireland and are therefore reflecting on services in this geographic region. Results Participants’ needs were grouped into five themes: support managing physical health‐care issues; access to community/home‐based services; EB‐specific information and psychosocial support; effective interaction with health‐care professionals; and advice regarding benefits and entitlements. Discussion and Conclusions This article represents the health‐care needs and preferences of a broad spectrum of those with EB, highlighting the need for a comprehensive service regardless of the severity of the condition.

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

“…To the best of our knowledge, this is the first study of EB to implement a survey that assesses patient benefit outcomes weighted by individual needs. The results in this study complement existing qualitative and cross-sectional research on patient needs and consequences of living with EB [6,9,26,63]. The response rate in this study was high compared to other cross-sectional EB studies in our centre and demonstrates participants' willingness to be involved in research that addresses their individual needs [4,24,26].…”

It’s more than just lubrication of the skin: parents’ experiences of caring for a child with ichthyosis

“…However, when the child is affected by ichthyosis, parents are the ones exposing them to pain, hence depriving the child of the comforting parent in painful situations. This could create distress in parents, and demanding emotional and behavioral dynamics between children and parents, as has been described also by parents of children with epidermolysis bullosa and other complex chronic conditions (Kearney et al, 2020 ; Miller et al, 2009 ), which may require provision of support or psychological interventions. However, most of the parents in our study reported to cope well, although some of them felt uncertainty regarding touching their child and finding alternative ways of showing affection and love for it.…”

“…This is in line with parents of children with osteogenesis imperfecta, who also report fear of injuring their child or causing pain, and therefore, as a consequence, limit cuddling and physical contact (Deatrick, Knafl, & Walsh, 1988 ). Similarly, parents of children with epidermolysis bullosa, also share the challenging emotional impact of witnessing their child in pain (Kearney et al, 2020 ). In our study, parents described some factors that could possibly affect parental touch-related behaviors, like for instance experiencing depressive symptoms, feeling anxious and shocked, and displaying high levels of psychological distress, as they understood that there was something wrong with their newborn child.…”

“…Hence, parents may struggle with difficult feelings without support from health care services or others. As demonstrated also in parents of children with epidermolysis bullosa, the social and emotional impact of complex skin conditions on parents is understudied, and need to be addressed by appropriate support within health care services (Kearney et al, 2020 ; Wu & Cohen, 2019 ).…”

“…Despite this, only a few studies have investigated health-related quality of life (HRQoL) for people with ichthyosis (Troiano & Lazzeri, 2020 ); even fewer studies have explored how parents adapt to the many challenges associated with their child’s diagnosis. A small number of studies have addressed the difficulties faced by the parents of children suffering from another skin disorder, epidermolysis bullosa, which also involves painful and time-consuming skin care (Kearney, Donohoe, & McAuliffe, 2020 ; Van Scheppingen, Lettinga, Duipmans, Maathuis, & Jonkman, 2008 ). A Swedish study showed that HRQoL was impaired for both children with congenital ichthyosis and their parents (Gånemo, 2010 ).…”