Table 3 Recommendations Table: Mental health, quality of life, and life satisfaction
No. | Recommendation | Strength of recommendation | Key references* |
|---|---|---|---|
General | |||
R1 | See R1 in Table 2 above | ↑↑ | |
R36 | Both the symptoms and treatments associated with EB require equal consideration when providing care necessitating that medical providers consider the whole person living with EB that is beyond the visible lesions | ↑↑ |  → [10] |
R39 | People living with EB must have the same opportunities to feel that they are a useful and contributing member of society as those individuals unaffected by EB by allowing them to grow emotionally, spiritually, and intellectually rather than holding them back because of any actual or perceived physical challenges that they may have | GPP | Â |
R41 | Medical providers should help reduce the costs to the person living with EB and his/her family through judicious use of materials | ↑↑ | [62] |
R44 | Increasing public awareness of the biopsychosocial challenges caused by EB as well as addressing fears of contagion or abuse is necessary | ↑↑ | |
R49 | Health care providers should provide multi-faceted health care education to benefit patients and carers to encourage active engagement in the process of one’s own medical decision making throughout the lifespan | ↑↑ | |
R50 | Established models for practice should be utilized | ↑↑ | |
R51 | Interventions provided by healthcare teams should target the development of personal support systems for people living with EB | ↑↑ | |
Patient, family, and carers mental health | |||
R37 | The mental health of parents, carers, and providers should be addressed | ↑↑ |  → [10] |
R38 | Focus should be placed on the emotional needs of the family and of the parent/child relationship | GPP | |
R40 | Children living with EB should be encouraged to develop psychosocially and live their lives despite having EB while either accepting or distancing themselves from the disease | ↑↑ |  → [10] [60] |
R43 | Formal and informal support and advocacy groups and solutions-focused psychotherapy, while being mindful of trauma, can help with coping related to EB, stigma, bullying loss, challenging decision-making, and bereavement while encouraging fuller participation in society, a sense of self-realization, having a fulfilling social life, and good peer support | ↑↑ |  → [10] |
R45 | Family members need long-term support while helping to improve coping with the emotional burden associated with providing care and with bringing in respite | ↑↑ |  → [10] |
R46 | Psychological support of the person living with EB, their families, and carers is very important after a diagnosis of SCC, in end-of-life decision-making, or following bereavement | ↑↑ |  → [9] |
Provider mental health | |||
R34 | See R34 in Table 2 above | ↑↑ | |
Quality of life | |||
R42 | QoL and other psychological assessment/screening tools should be considered to help evaluate the impact of EB | ↑↑ | [13, 15, 16, 40, 50, 51, 55, 56, 59, 63,64,65,66,67,68,69,70,71] |
R47 | Involvement of an IDT (where each member has expertise in treating EB) that provides a wholistic treatment approach aids in the improvement of overall QoL, especially when the person living with EB decides their own therapeutic goals | ↑↑ | [2, 12, 13, 23, 26, 29, 32,33,34, 40, 53, 59, 61, 63, 74,75,76,77,78,79,80,81,82,83,84, 87] |
R48 | To improve QoL, symptoms should be managed through medical or surgical interventions | ↑↑ | |