Psychosocial recommendations for the care of children and adults with epidermolysis bullosa and their family: evidence based guidelines

Abstract

Epidermolysis Bullosa (EB) is a group of rare genetic disorders resulting in skin fragility and other symptoms. Commissioned by DEBRA International and funded by DEBRA Norway, this evidence-bases guideline provides recommendations to optimise psychosocial wellbeing in EB. An international multidisciplinary panel of social and health care professionals (HCP) and people living with EB was formed. A systematic international literature review was conducted by the panel following the Scottish Intercollegiate Guidelines Network (SIGN) methodology. The resulting papers underwent systematic selection and critique processes. Included papers were allocated to 6 different outcome groups to allow data synthesis and exploration: quality of life, coping, family, wellbeing, access to HCP and pain. Based on the evidence in those papers, recommendations were made for individuals living with EB, family and caregivers and HCP working in the field. Few studies have investigated interventions and which factors lead to better outcomes, but general recommendations can be made. EB is a complex disease impacting enormously on every aspect of psychosocial life. People and families living with EB need access to multidisciplinary support, including psychological guidance, in order to improve quality of life and psychosocial wellbeing. Interventions should stimulate social participation to prevent isolation. People with EB and their families should be able to access a supportive network. HCP should be well supported and educated about the complexity of EB. They should work collaboratively with those around the individual with EB (e.g. schools, employers etc.) to provide psychosocial opportunity and care. Attention should be paid to the psychosocial impact of EB as well as physical needs. Directions for research are indicated. Electronic supplementary material The online version of this article (10.1186/s13023-019-1086-5) contains supplementary material, which is available to authorized users.

Psychosocial impact of epidermolysis bullosa on patients: A qualitative study

“…Since then, guidelines emphasizing the importance of training clinicians and providing multi-disciplinary support to address the psychosocial needs of EB patients have been published. [20][21][22]24 Similarly, social media campaigns (eg informational DVDs and cards for classmates, celebrity promotion, etc) for health professionals, schools, and the general public have addressed some of these issues identified in this study.…”

“…Research into the QoL of EB patients has increasingly brought more awareness to the challenges that they face. 2,3,10,11,13,[16][17][18][19][20][21][22][23][24] In this qualitative study, we were able to further expand on many of these challenges.…”

“…Recommended coping strategies have been successful in improving EB patients' QoL, including acceptance and distancing from others. 22 Finally, cognitive behavioral therapy has been considered for EB patients 24 and could address many patient concerns identified in our study (Table 4).…”

Clinical practice guidelines: Oral health care for children and adults living with epidermolysis bullosa

“…DEBRA International has supported and funded the development of CPG in skin and wound care, 23,24 pain management, 25 psychosocial care, 26 foot care, 27 as well as the guidelines that have already been cited in this article 8,12,17,18 . Research is also supported to explore gene, protein, and cell therapies.…”

Meeting Report: The First Global Congress on Epidermolysis Bullosa, EB2020 London: Toward Treatment and Cure

“…Other complications, such as anemia, oral and dental problems, esophageal strictures, nutritional compromise, airway involvement, contractures, and eye involvement, provide a significant burden for patients with EB, but a good quality, robust evidence base for treatments and interventions is generally lacking. The development of clinical best practice guidelines over recent years, supported by DEBRA International, has tackled some of these knowledge gaps by assembling professionals, patients, and caregivers to develop expert consensus to give practical guidance in areas including oral health, pain, cancer, wound care, foot health, psychosocial care, and occupational therapy, for example (Chan et al, 2019;Khan et al, 2020;Martin et al, 2019).…”