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

NCT05248503 | Completed

• 1 other identifier: APHP211522
• Study Type: observational
• Target: 19
• Locations: 1 country
• Sites: 1

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.

Conditions

Hereditary Epidermolysis Bullosa

Keywords

hereditary epidermolysis bullosa; nursing care; care training; child

Outcome Measures

Primary Outcomes (1)

• Change of anxiety level

  Anxiety will be assessed using the Anxiety - Spielberger State self-quiz, rated from 20 to 80 (80 indicating maximum anxiety state).

  Change form baseline (before training) and at the end of the second day of training

Secondary Outcomes (5)

• Anxiety evolution

  3 months after training

• Anxiety auto-evaluation

  Before training. 3 and 6 months after the training

• Feeling of skills

  Before training. 3 and 6 months after the training

• Nurse knowledge about Epidermolysis bullosa

  Before and after the training. 3 and 6 months after the training

• Satisfaction questionnaire

  At the end of the second day of training.

Study Arms (1)

Nurse

Pediatric nurses taking charge of the care of patients with epidermolysis bullosa at Necker Hospital

Behavioral: Complex Care Training of Hereditary Epidermolysis Bullosa

Interventions

Complex Care Training of Hereditary Epidermolysis Bullosa BEHAVIORAL

Training in the care of epidermolysis bullosa by combining theoretical content on the disease and practical workshops.

Nurse

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Any caregiver who, within their department, must take charge of the complex care of patients with EBH (nurse, caregiver, pediatric nurse, childcare assistant) is eligible.

You may qualify if:

• 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.

You may not qualify if:

• Person under tutorship or curatorship.
• Other health professionals (physiotherapist, doctor, etc.).

Sponsors & Collaborators

• Assistance Publique - Hôpitaux de Paris: lead
• URC-CIC Paris Descartes Necker Cochin: collaborator

Study Sites (1)

Assistance Publique - Hôpitaux de Paris

Paris, 75015, France

Location

Related Publications (9)

• Ingen-Housz-Oro S, Blanchet-Bardon C. Épidermolyses bulleuses héréditaires. EMC - Dermatol-Cosmétologie. 1 févr 2004 ; 1(1) :2 18.

  BACKGROUND

• Chiaverini C, Bourrat E, Mazereeuw-Hautier J, Hadj-Rabia S, Bodemer C, Lacour JP. [Hereditary epidermolysis bullosa: French national guidelines (PNDS) for diagnosis and treatment]. Ann Dermatol Venereol. 2017 Jan;144(1):6-35. doi: 10.1016/j.annder.2016.07.016. Epub 2016 Dec 5. French.

  PMID: 27931749 BACKGROUND

• Lustre A, Levé-Corset I, Leeuwin G, Launay C, Blondy MF, Michaud MT, et al. Soins infirmiers de l'épidermolyse bulleuse. Rev Fr Allergol Immunol Clin. 2001 ; 41(7) : 659-663.

  BACKGROUND

• Jérôme Palazzolo, Julie Arnaud, Anxiété et performance : de la théorie à la pratique, Annales Médico-psychologiques, revue psychiatrique, Elsevier, Volume 171, Issue 6, July 2013, Pages 382-388.

  BACKGROUND

• Bandura, A. Auto-efficacité. Le sentiment d'efficacité personnelle (Trad. J. Lecomte). Bruxelles : De Boeck. 2003.

  BACKGROUND

• Gadbois, C., Aides-soignantes et infirmières de nuit, conditions de travail et vie quotidienne, Paris : Etudes et Recherches, 1981.

  BACKGROUND

• Van Daele A., Le stress chez les médecins généralistes : une approche transactionnelle, In B. Gangloff (Ed.), satisfactions et souffrances au travail (60-67), Paris : l'Harmattan.

  BACKGROUND

• Verquerre R., Rusinek-Nisot, Etude du stress chez des infirmiers, In R.Jacob et R. Laflamme (Eds), Stress, santé et intervention au travail, (61-71), Québec : Presses Inter Universitaires.

  BACKGROUND

• Borteyrou X, Bruchon-Schweitzer M, Spielberger CD. [The French adaptation of the STAXI-2, C.D. Spielberger's State-trait anger expression inventory]. Encephale. 2008 Jun;34(3):249-55. doi: 10.1016/j.encep.2007.06.001. Epub 2007 Oct 10. French.

  PMID: 18558145 BACKGROUND

Study Officials

• Sandrine COMPAIN, RN

  Necker Hospital, APHP

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: January 17, 2022
• First Posted: February 21, 2022
• Study Start: June 1, 2023
• Primary Completion: November 15, 2023
• Study Completion: May 20, 2025
• Last Updated: May 6, 2026

Record last verified: 2026-04

Data Sharing

• IPD Sharing: Will not share

Locations

FR (1)