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

Study Overview

• Status: Recruiting
• Conditions: Hereditary Epidermolysis Bullosa
• Intervention / Treatment: Behavioral: Complex Care Training of Hereditary Epidermolysis Bullosa

• Study Type: Observational
• Enrollment (Estimated): 16

Contacts and Locations

• Study Contact: Name: Sandrine COMPAIN, RN; Phone Number: +33 1 44 49 45 95; Email: sandrine.compain@aphp.fr
• Study Contact Backup: Name: Solimda SOTOU BERE; Phone Number: + 33 1 42 19 28 79; Email: solimda.sotoubere@aphp.fr

Study Locations

• France
  • Paris, France, 75015
    • Recruiting
    • Assistance Publique - Hôpitaux de Paris
    • Contact: Sandrine Compain, nurse; Phone Number: +33 1 44 49 45 95; Email: sandrine.compain@aphp.fr

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No

• 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.

Description

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.).

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Nurse; Pediatric nurses taking charge of the care of patients with epidermolysis bullosa at Necker Hospital	Behavioral: Complex Care Training of Hereditary Epidermolysis Bullosa; Training in the care of epidermolysis bullosa by combining theoretical content on the disease and practical workshops.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
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 Outcome Measures

Outcome Measure	Measure Description	Time Frame
Anxiety evolution	Anxiety will be assessed using the Anxiety - Spielberger State self-quiz, rated from 20 to 80 (80 indicating maximum anxiety state).	3 months after training
Anxiety auto-evaluation	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)	Before training. 3 and 6 months after the training
Feeling of skills	Self-assessment of feeling of skills on Likert scales from 0 to 4 (4 indicating a factors induces an high level of anxiety)	Before training. 3 and 6 months after the training
Nurse knowledge about Epidermolysis bullosa	A true/false questionnaire with degree of certainty assessing knowledge about epidermolysis bullosa	Before and after the training. 3 and 6 months after the training
Satisfaction questionnaire	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.	At the end of the second day of training.

Collaborators and Investigators

• Sponsor: Assistance Publique - Hôpitaux de Paris
• Investigators: Principal Investigator: Sandrine COMPAIN, RN, Necker Hospital, APHP

Publications and helpful links

General Publications

• Ingen-Housz-Oro S, Blanchet-Bardon C. Épidermolyses bulleuses héréditaires. EMC - Dermatol-Cosmétologie. 1 févr 2004 ; 1(1) :2 18.
• 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.
• 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.
• 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.
• Bandura, A. Auto-efficacité. Le sentiment d'efficacité personnelle (Trad. J. Lecomte). Bruxelles : De Boeck. 2003.
• Gadbois, C., Aides-soignantes et infirmières de nuit, conditions de travail et vie quotidienne, Paris : Etudes et Recherches, 1981.
• 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.
• 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.
• 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.

Study record dates

Study Major Dates

• Study Start (Actual): June 1, 2023
• Primary Completion (Estimated): June 1, 2024
• Study Completion (Estimated): December 1, 2024

Study Registration Dates

• First Submitted: January 17, 2022
• First Submitted That Met QC Criteria: February 9, 2022
• First Posted (Actual): February 21, 2022

Study Record Updates

• Last Update Posted (Actual): October 31, 2023
• Last Update Submitted That Met QC Criteria: October 27, 2023
• Last Verified: October 1, 2023

More Information

Terms related to this study

• Keywords: child; nursing care; hereditary epidermolysis bullosa; care training
• Additional Relevant MeSH Terms: Behavioral Symptoms; Skin Diseases; Congenital Abnormalities; Genetic Diseases, Inborn; Stress, Psychological; Skin Diseases, Genetic; Skin Diseases, Vesiculobullous; Skin Abnormalities; Caregiver Burden; Epidermolysis Bullosa
• Other Study ID Numbers: APHP211522

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No