Epidermolysis bullosa: Patient relevant outcomes and helpful factors for the quality of life (Salamon 1)
Completed| Project lead | DDr Gudrun Salamon |
| Organisation | Sigmund Freud University, Vienna, AUSTRIA |
| Project budget | EUR 130,000,00 |
| Start date / Duration | 01. Jul 2019 / 19 months |
| Funder(s) / Co-Funder(s) | DEBRA Austria |
| Research area | Psychology |
Publications related to the projects
Facilitating factors for the quality of life of people living with epidermolysis bullosa and their families, identified by health care professionals and experts Psychosocial aspects of Epidermolysis bullosa and quality of life. A systematic review iscorEB-p: A patient-reported outcome measure for Epidermolysis bullosa. Psychometric properties of the Patient Score from the Instrument for Scoring Clinical Outcome of Research for EB https://pubmed.ncbi.nlm.nih.gov/38773622/Project details
Short lay summary
EB has an enormous impact on all aspects of psychosocial life. In EB, stress is caused by chronic pain, the need of intensive wound management and restrictions in everyday life. Additionally, these restrictions lead to social and emotional challenges. In this project, we focus on patient relevant outcomes of long-term medical and sociopsychological treatment for patients with EB with a special emphasis on what matters most for increasing their quality of life. To do so, we conduct an extensive literature review as well as interviews with EB patients, families and professionals.
Scientific summary
In addition to the rather medical point of view of treatment success, our study focuses on “patient relevant outcome“ and takes the individual concepts of and experiences with quality of life as well as the definition of and the coping strategies with success and failure of treatment and the course of disease. As indicated in previous research, EB has an enormous impact on all aspects of psychosocial life. Hence, quality of life is an important measure in psychological as well as in clinical research. However, quality of life is highly influenced by individual perception. The same applies to the concept of wellbeing, which describes the individual balance between personal resources and challenges faced. Coping strategies are an individual’s mental or behavioural response options to stress and to challenging situations. In EB, stress is caused by chronic pain, the need of intensive wound management and restrictions in everyday life. Additionally, these restrictions lead to social and emotional challenges.
Goals of the project:
- Systematic review of the literature on quality of life with EB
- Conduction and analysis of interviews with EB patients, families and professionals
- Better understanding of what is considered as helpful by EB patients
Strategic relevance
A better understanding of what is considered as helpful by EB patients can be used for adapting or expanding the support offers by DEBRA, especially for
- specific support of EB patients and their families with achieving and maintaining quality of life
- well-directed information and preparation of EB patients and their families in regard to the expected course of disease
- knowledge of a set of coping strategies with success and failure of treatment and the course of disease
What did this project achieve?
For a thorough understanding of psychosocial aspects of EB, Gudrun Salamon and her team first conducted a systematic review of the scientific literature on this topic. They looked through 1113 papers and finally selected 38 to be analysed in detail. For gaining more insights on burdens and helpful aspects of a life with EB, they led 42 interviews. They interviewed EB patients, their relatives, and EB professionals, from Austria, Germany, and Italy, and identified important helpful factors in dealing with EB. They found that, e.g., EB relatives mentioned more than two times more aspects where they wish to have more support than EB patients.
It is essential that people with EB are taken seriously and are not only seen and treated as sufferers and patients, but as highly individual people. Due to the multiple demands of EB, it is essential to get additional professional or private support when needed. Even though EB has a central influence on life, it should not become the only focus of the entire family system. It is helpful to know people who are in the same situation in order to share experiences. However, this does not mean that the same things help everyone. Even with the same disease, people differ in their needs. What works for one person, doesn’t necessarily work for another, so individual solutions need to be found, again and again. In this concern, it helps to consider the patients and their relatives as experts for their own case, and to team up individual and medical expertise for finding the best solution for the individual concerned.
Next steps: The next step is a survey on burdens and helpful aspects with EB. This will allow to better understand the different needs within the EB community. In the future, these results can be used as a basis for the design of support and interventions tailored to the needs of EB patients and their relatives.