Challenges and supports for life with EB

• Primary Researcher: DDr Gudrun Salamon
• Institution: Sigmund Frued University, Vienna, Austria
• Type of EB: All EB types
• Funding amount: Full study funded by Debra Austria, Irish-only analysis funded by Debra Ireland
• Project length: 1 year

The research identified that those with EB and their relatives are impacted by the condition in multiple ways. There were significant findings in relation to the burden of EB on both the individual and their families/caregivers. Such burdens included the visibility of the condition, how severe EB is for that individual, lack of mobility, the impact of EB financially and psychologically. It was identified that the majority of those impacted by EB really value the support services they receive from medical and healthcare providers and from Debra. However, because of the financial responsibilities associated with EB, individuals impacted by EB and their relatives have conveyed they need more financial assistance from the state. The findings provide a foundation for developing focused psychosocial intervention programmes for those with EB and their families in Ireland. The findings aim to enhance current resources and formulate strategies for living with EB.

DDr Gudrun Salamon currently works as a postdoctoral researcher and lecturer at the Department of Psychology, Sigmund Freud University Vienna, and as a lecturer at the Department of Computer Science, Vienna University of Technology. Her research focuses on quality of life, health psychology and psychosomatics, therapeutic methods, psycholinguistics, perception, user modeling, and other topics within the interdisciplinary field in between computer science, linguistics and psychology.

Grant Title: Burdens and helpful aspects for achieving and maintaining quality of life with epidermolysis bullosa

Apart from the medical aspects, quality of life is a crucial factor in the well-being of those living with a chronic condition, such as EB. The balance between individual burdens and resources is essential. In the study, a special focus is placed on what was or still is perceived as helpful assuming that EB does not only have an impact on those affected themselves, but also on their relatives.

Since summer 2019, DDr Gudrun Salamon, researcher in the faculty of psychology at the Sigmund Freud University Vienna has been leading a research team of six people on the project of “Burdens and helpful aspects for achieving and maintaining quality of life with epidermolysis bullosa”.

In cooperation with DEBRA International, DEBRA Austria, and the EB-Haus Austria, the team are currently conducting an online survey on burdens and resources in EB with the international EB population (in German and English).

Participation in the study will provide valuable experience and will help the team to build a better understanding on burdens and helpful factors in dealing with EB with the aim of being able to provide better support to people living with EB as well as their relatives.

Researcher’s final progress update

Findings from the Irish-only analysis of this study highlight the burdens of EB both to those who have EB and their relatives.

• Those impacted by EB with a higher degree of severity more often experience more symptoms of EB, face greater burdens related to the disease, and have fewer resources available to manage it.
• Reduced mobility due to EB significantly impacts quality of life. Individuals with limited mobility, because of EB, experience considerably more medical symptoms compared to those without restrictions, and both the individuals with EB and their relatives are less satisfied with life.
• Regarding the current health status, participants who indicated that their or their relative’s EB was not, or hardly visible reported fewer symptoms compared to those whose EB was somewhat to very visible. The data highlights the emotional impact of EB’s visibility on both those with the condition and their relatives. Unpleasant reactions from the social environment, along with parents’ fear of being perceived as bad parents, evoke feelings of shame and are particularly burdensome.
• Financial concerns emerged as one of the more major themes from the qualitative data, highlighting the broad range of economic costs participants face. These expenses include purchasing appropriate clothing and footwear, securing medical and psychological treatments, and affording leisure activities. Regarding financial support, over half of the participants indicated that the costs of bandages and medication were either fully or partially covered, and they had full or partial access to adequate medical care. However, 41% of participants had to entirely fund their own bandages and medication, and 7% reported inadequate access to these necessities due to financial constraints and/or limited availability.
• The accumulation of multiple stressors related to the psychosocial impact of EB was frequently described as overwhelming. Common coping mechanisms include acceptance, adapting to the situation, or distraction. Emotions significantly affect general well-being; participants who felt more burdened by their emotions were notably less satisfied with life and rated their resources lower.
• Overall, satisfaction with available support varies widely. Medical support is commonly utilised and satisfaction levels are generally high. In contrast, supports like physiotherapy or psychotherapy are less frequently accessed and satisfaction levels are lower. High satisfaction tends to correlate with frequent service use.

Due in 2024