Exploring the Impact of Epidermolysis Bullosa on Parents and Caregivers: A Cross-Cultural Validation of the Epidermolysis Bullosa Burden of Disease Questionnaire

Abstract

Purpose

Epidermolysis bullosa (EB) is a heterogeneous group of genetically inherited skin and mucosal fragility disorders. EB may have a profound impact on parental physical and psychosocial health. This study was designed to evaluate the disease burden in parents of patients with EB and identify out-of-pocket (OOP) expenditures for EB care in Saudi Arabia.

Patients and Methods

Thirty-eight caregivers of patients with EB were recruited from the Saudi EB registry to participate. All participants completed the EB Burden of Disease (EB-BoD) questionnaire. Data were collected between May 2020 and December 2020. The sample included 10 patients with EB simplex (EBS), 10 with junctional EB (JEB), 14 with dystrophic EB (DEB), and 4 with an unknown type.

Results

Mothers were the primary caregivers in 89.5% of cases. The mean EB-BoD score was 53 ± 21.5. The family-life and child’s life dimensions had the higher burden. The mean EB-BoD score observed in patients with DEB was 62.4±16.8 versus 45.7 ± 19.42 for EBS. The EB-BoD score was correlated with the patient’s family income. Most caregivers (97.4%) reported OOP expenditure, with a mean monthly OOP expenditure of $575.5± $701.1. OOP expenses increased with the severity of the condition.

Conclusion

This study highlights the need for support services for parents caring for patients with EB.

Keywords:

• burden
• caregivers
• epidermolysis bullosa
• quality of life

Acknowledgments

The authors thank all parents and caregivers who contributed valuable time to complete this questionnaire.

Disclosure

The authors report no conflicts of interest in this work.