Hereditary Epidermolysis Bullosa
Conditions
Keywords
hereditary epidermolysis bullosa, nursing care, care training, child
Brief summary
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.
Interventions
BEHAVIORALComplex Care Training of Hereditary Epidermolysis Bullosa
Training in the care of epidermolysis bullosa by combining theoretical content on the disease and practical workshops.
Sponsors
Assistance Publique - Hôpitaux de Paris
URC-CIC Paris Descartes Necker Cochin
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Nurse, nursing assistant, nursery nurse, childcare assistant, state graduates caring for patients with EBH and working at Necker Hospital. * Having participated in the entire training program specific to the complex care of epidermolysis bullosa. * Informed and not opposed to their participation in research.
Exclusion criteria
* Person under tutorship or curatorship. * Other health professionals (physiotherapist, doctor, etc.).
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Change of anxiety level | Change form baseline (before training) and at the end of the second day of training | Anxiety will be assessed using the Anxiety - Spielberger State self-quiz, rated from 20 to 80 (80 indicating maximum anxiety state). |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Anxiety evolution | 3 months after training | Anxiety will be assessed using the Anxiety - Spielberger State self-quiz, rated from 20 to 80 (80 indicating maximum anxiety state). |
| Anxiety auto-evaluation | Before training. 3 and 6 months after the training | Self-assessment of factors that may induce anxiety measured on a Likert Scale from 0 to 4 (4 indicating a factors induces an high level of anxiety) |
| Feeling of skills | Before training. 3 and 6 months after the training | Self-assessment of feeling of skills on Likert scales from 0 to 4 (4 indicating a factors induces an high level of anxiety) |
| Nurse knowledge about Epidermolysis bullosa | Before and after the training. 3 and 6 months after the training | A true/false questionnaire with degree of certainty assessing knowledge about epidermolysis bullosa |
| Satisfaction questionnaire | At the end of the second day of training. | Satisfaction questionnaire on the quality and content of the training with a Likert scale ( 0 to 5; an higher score indicates better outcome) and open questions. |
Countries
France
Contacts
PRINCIPAL_INVESTIGATORSandrine COMPAIN, RN
Necker Hospital, APHP
Outcome results
None listed