Impact of Complex Care Training of Hereditary Epidermolysis Bullosa on Caregiver Burden (FIREB)

Hereditary epidermolysis bullosa (EBH) is a rare, orphan disease characterized by skin and mucous membrane fragility. The latest scientific data show that the proposed treatments are still in the experimental stage and that no curative treatment is available. The repercussions of this chronic disease, with neonatal onset, are major. Epidermolysis bullosa requires multidisciplinary medical management, nursing care, psychological and social care. Skin care involves preventing and treating chronic wounds and identifying their complications. The very great cutaneous-mucous fragility makes these treatments painful, long and complex, the caring hand itself being able to cause new wounds. Analgesics of different levels are not effective enough during treatment. Along with counseling and education, nursing takes a central role in multi-professional accompaniment interventions to support and relieve families. Parents became home caregivers out of necessity, and developed specific skills in epidermolysis bullosa, their child and dressings. They have great and demanding expectations of caregivers facing this rare disease, for which they are not trained in their degree course. Despite the severe nature of the disease, few studies have been carried out on the impact and psychosocial consequences on patients and their families, yet there is an expressed need for support. The burden on parents is heavy, assessed by specific scales, but to date there are no studies examining the impact of epidermolysis bullosa care on caregiver stress.