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Table 1 Recommendations summary for the Psychosocial care of individuals living with EB

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

RecommendationsPopulationGrade strengthQuality of evidence (Average)Quality of evidenceKey references
i. We strongly recommend easy access to psychosocial support to improve Quality of life (QoL)
A multidisciplinary approach in treating EB improves QoL for individuals with EB
 • Psychological support and close monitoring of EB improves QoL.
 • They facilitate participation in social activities.
 • Patients with all types of EB including EBS report a great impairment in QoL due to restrictions in physical and social activities.

Adults, children (n = 12/185) EBS; JEB; DDEB; RDEB; KS
Unclear if adults, children or both (n = 43/134) EBS; JEB; DDEB; RDEB;
Adults, children (n = 120/248) EBS; JEB; DDEB; RDEB
Review of inherited & autoimmune blistering diseases
B2++1-
2+
2+
2-
1-
[29]
[30]
[31]
[28]
[32]
ii. We strongly recommend psychosocial support to improve well-being
To promote self-efficacy and support around body image to aid psychological well-being
 • Having access to knowledge and resources about EB can help people have a greater role in managing their EB. This self-management can help improve well-being.
 • Improved self-efficacy and locus of control, as well as support around body-image could help to develop a more positive sense of well-being.
For support during transition periods in life (school transitions, transition into adulthood)
 • Communication and education about EB to improve people’s understanding.
 • Support from families, EB healthcare professionals and DEBRA.
Review of inherited &autoimmune blistering diseases
Adults (n = 87) RDEB, DEB, EBS
Children 10–14 years old (n = 11) EBS (autosomal recessive)
Young male adults (aged 21–35 years) with RDEB (n = 5) and EBS (n = 2)
Observational report
C2+1-
2+
2+
4
4
[32]
[33]
[34]
[35]
[36]
iii. We strongly recommend gaining access to psychosocial support for the whole family
People diagnosed with EB should be referred for psychosocial support as early as possible in childhood or in adulthood, if the person with EB wishes
• To support the family unit.
Encourage supportive network for the family, for example:
 • Education about EB for others
 • Provide access to DEBRA (or other EB support groups)

Children (n = 11/82) EBS; JEB; DDEB; RDEB
Children (n = 16) JEB Adult (n = 1) RDEB
C2-2-
2-
4
[37]
[38]
[39]α
iv. We recommend psychosocial support to help with pain
Pain is present for most children and adults with EB (all types) with profound psychosocial impact:
 • Activity related pain can significantly affect psychosocial well-being and QoL (e.g. fear of/actual pain restricting social activities, affecting relationships with family and friends).
 • Treatment related pain can make managing EB harder and link to procedural anxiety.
Adequate holistic pain management is essential as a focus for helping people with EB:
 • Following pain guidelines.
 • Offering approaches to help people with EB cope emotionally.
 • Help with managing the impact of pain and the interlinked cycle of pain and psychosocial challenges.
Adults, children(n = 374) EBS, JEB, DDEB, RDEB
Adults (n = 6) JEB, DDEB
Children; (n = 11) EBS, JEB, DDEB, RDEB,
Adults (n = 30) children (n = 27) EBS
Children/families (n = 70) type of EB unclear
Adults (n = 43) EBS, JEB, DDED, RDEB
Unclear if adult/child (n = 40)
EBS, JEB, DDEB, RDEB
Best practice guideline
Children (n = 11) EBS, JEB, DDEB, RDEB
Adult (n = 1) RDEB
C2-2+
2-
2-
2-
2-
2+
2+

2+
3
[3]
[40]
[37]
[42]
[41]
[30]
[44]
[7]α
[17]
[43]
v. We strongly recommend psychosocial support to help cope with living with EB
People with EB need support to cope with EB, and their ways of coping need to be supported by others: participation in social life needs to be supported
 • Such as at school, the community, friendships, employment.
 • Aid access to supportive networks.
 • Public education campaigns to help those around them to understand EB and their needs.
Promote a sense of self-management of their EB
 • This can help bring a sense of control over certain aspects of the disease/treatment and pain.
Build social skills and communication
 • Help in learning how to communicate about EB to others and within the family unit.

Children (n = 27) DDEB; (n = 28) RDEB
Children 10–14 years old (n = 11) EBS (autosomal recessive)
Children (n = 11/82)
EBS; JEB; DDEB; RDEB
Children (n = 24)
EBS; JEB; DDEB; RDEB
C2-2+
2+
2-
2-
[45]
[34]
[37]
[46]
vi. We strongly recommend psychosocial support from a multidisciplinary Health Care Team
Encourage access to, and a collaborative ‘working together’ relationship with, an expert multi-disciplinary team of professionals.
 • Facilitate access to multidisciplinary professional support for medical and psychosocial care across the lifespan.
 • At both specialist centres and community services

Adults, children (n = 15) RDEB
Children (n = 21) EBS; JEB; DDEB; RDEB
Children (n = 11/82) EBS; JEB; DDEB; RDEB
HCPs (n = 33) 30 stakeholders (HCPs, and 9 with EB RDEB, DDEB, EBS)
Adults (n = 6) JEB, DDEB
Children and Adults (n = 20) EBS, JEB, DEB
Children (n = 16) JEB
Children (n = 20) EBS, JEB, RDEB
C2-1-
2+
2+
2+
2-
2-
2-
2-
2-
2+
[29]
[48]
[51]
[17]
[46]
[49]
[40]
[38]
[48]
[47]
Key: EB: Epidermolysis Bullosa; RDEB: Recessive Dystrophic Epidermolysis Bullosa; JEB: Junctional Epidermolysis Bullosa; DDEB: Dominant Dystrophic Epidermolysis Bullosa; EBS: Epidermolysis Bullosa Simplex EBS-I: Localised form of EBS; KS Kindler Syndrome; QoL: quality of life; n: number of; α: gray literature; this is an EB guideline
GradesDescriptions in accordance to SIGN [22]
 BA body of evidence including studies rated as 2++, directly applicable to the target population, and demonstrating overall consistency of results; or Extrapolated evidence from studies rated as 1++ or 1+
 CA body of evidence including studies rated as 2+, directly applicable to the target population and demonstrating overall consistency of results; or Extrapolated evidence from studies rated as 2++
Ratings Descriptions in accordance to SIGN [22]
 1-Meta-analyses, systematic reviews, or RCTs with a high risk of bias
 2++High quality case control or cohort studies with a very low risk of confounding or bias and a high probability that the relationship is causal
 2+Well conducted case control or cohort studies with a low risk of confounding or bias and a moderate probability that the relationship is causal
 2-Case control or cohort studies with a high risk of confounding or bias and a significant risk
 3Non-analytic studies, e.g. case reports, case series
 4Expert opinion
 ✔ Recommended best practice based on the clinical experience of the guideline development group [22]