Table 2 Recommendations summary for the Psychosocial care of Family and caregivers of people living with EB

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

B

2+

1-

2+

2-

[29]

[30]

[38]

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

C

2-

2+

2+

2-

4

2+

4

[50]

[52]

[30]

[36]

[53]

[54]

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

C

2-

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]

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

C

2-

2+

2-

2-

2-

[51]

[30]

[16]

[17]

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

C

2-

2+

2-

2-

[17]

[42]

[40]

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

C

2-

1-

2+

2+

2+

4

[29]

[50]

[51]

[17]

[39]^α

Grades

 B

 C

 1-

 2+

 2-

 4-