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Table 2 Recommendations summary for the Psychosocial care of Family and caregivers of people 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 access to psychosocial family support to improve the family QoL
Early psychosocial support to improve QoL of the family unit for all subtypes EB and children with high infantile mortality:
 • As caregivers QoL may also be impacted.
 • Psychological support and close monitoring helps.
 • Support is essential for family of palliative patients with EB.
Adults, children; 11 studies
EBS; JEB; DDEB; RDEB
Adults, children (n = 125/185)
EBS; JEB; DDEB; RDEB; KS
Children (n = 16) JEB
B2+1-
2+
2-
[29]
[30]
[38]
ii. We strongly recommend psychosocial support to improve the family well-being
Support for the family to reduce emotional burden of caring for someone with EB and improve well-being for the family unit:
 • Home nursing can provide much needed relief and support for primary caregivers and could reduce the need for hospital admission.
 • Actively assist in seeking counselling before the family unit is irreparably destroyed.
 • Provide information about the nature, course and outcome of EB.
 • Provide training in the management of patient symptoms.
 • Access to Social media and face to face EB support groups might be beneficial for families.
Promoting family well-being can help the family enhance their strong and positive influence for those living with EB
 • The way the family reacts to EB can be psychologically assimilated by the person with EB, particularly children.
 • Acceptance of the EB by the family is important and can make it more bearable for the patient.
Adults, children (n = 15) RDEB
Children, Adults (n = 374/ 425)
EBS; JEB; DDEB; RDEB
Adults, children (n = 125/185)
EBS; JEB; DDEB; RDEB; KS
Adults, children (n = 25, 14 children, 11 adults) RDEB, EBS
Adult – personal experience
C2-2+
2+
2-
4
2+
4
[50]
[52]
[30]
[36]
[53]
[54]
iii. We strongly recommend family counselling in order to prevent family breakdown
To help prevent the family unit breakdown, for the family of all EB subtypes:
 • Strengthen family relationships.
To prevent family emotional breakdown or distress
 • Support in managing life with EB.
To prevent parents’ emotional breakdown or distress
 • Specially provide support for single parents with a child living with EB.
Children, Adults (n = 374/ 425)
EBS; JEB; DDEB; RDEB
Adults, children (n = 15) RDEB
Children (n = 11/82)
EBS; JEB; DDEB; RDEB
C2-2+
2+
2-
[52]
[50]
[17]
Adults, children (n = 28/42)
EBS; JEB; DDEB; RDEB
Children (n = 16) JEB
Children, Young Adults; (n = 63/138) EBS; DDEB; RDEB+
2-
2-
2-
[16]
[38]
[55]
Adults, children (n = 125/185)
EBS; JEB; DDEB; RDEB; KS
  2-[30]
iv. We strongly recommend psychosocial support to help the whole family to cope with living with EB
Specialist home based psychosocial support for the family of all EB subtypes can help promote strategies to cope:
 • Help access counselling to promote the intra-family communication.
 • Access help to manage EB and economic burden.
 • Promote good relationships between the family
 • Provide a home care program for respite, or support handing over physical care to others.
Children (n = 21)
EBS; JEB; DDEB; RDEB
Adults, children (n = 125/185)
EBS; JEB; DDEB; RDEB; KS
Adults, children (n = 28/42)
EBS; JEB; DDEB; RDEB
Children (n = 11/82)
EBS; JEB; DDEB; RDEB
C2-2+
2-
2-
2-
[51]
[30]
[16]
[17]
v. We recommend psychosocial support to reduce emotional burden during daily painful procedures
Psychosocial support needs for parents and family to reduce the emotional burden of caring for someone living with EB who has severe pain:
 • Offer psychological support for caregivers. Parents/care givers can struggle with ‘causing pain’ due to dressing changes and wanting to protect their child from pain. This is very difficult emotionally for parents.
 • Pain can negatively affect relationships within the family and with friends. Help optimise pain management techniques.
 • Parents/carers can find it difficult to see people in severe pain. Aid access to respite, independent carers and promote independence with dressings.

Children (n = 11/82)
EBS; JEB; DDEB; RDEB
Adults, children; (n = 57) EBS-I
Adult (n = 6/20) JEB, DDEB
C2-2+
2-
2-
[17]
[42]
[40]
vi. We strongly recommend easy access to a multidisciplinary expert team for the whole family
Provide access to recognised expert support and training for the whole family
 • Provide appropriate treatment and training or refer to national EB experts.
 • Referring to with the DEBRA or EB support network may help.
Adults, children; 11 studies
EBS; JEB; DDEB; RDEB
Adults, children (n = 15) RDEB
Children (n = 21)
EBS; JEB; DDEB; RDEB
Children (n = 11/82)
EBS; JEB; DDEB; RDEB
Adult (n = 1) RDEB
C2-1-
2+
2+
2+
4
[29]
[50]
[51]
[17]
[39]α
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+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
 4-Expert opinion
 ✔ Recommended best practice based on the clinical experience of the guideline development group [22]