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.
Subject(s)
Anemia , Epidermolysis Bullosa , Adult , Humans , Retrospective Studies , Ferric CompoundsABSTRACT
Pruritus is one of the most debilitating symptoms for patients with epidermolysis bullosa (EB). This study aimed to assess the burden of itch and to address its dimensions across patients with EB. Forty-six patients with EB were recruited from the Saudi EB registry to participate. All participants completed the Leuven Itch Scale. The sample included 5 patients with EB simplex (EBS), 3 with junctional EB (JEB), 34 with dystrophic EB (DEB), and 4 patients had unknown type. Overall, 97.8% patients reported itch. In patients with itch, 73.3% reported that it was often or always present, longer than 2h Itch episodes was reported by JEB (66.7%) and recessive DEB (3.2%). Itch, in all its dimensions, was worst in patients with JEB and DEB than EBS. Itch occurred mostly in a hot environment (80%), when sweating (71.1%), in healing wounds (40%), and during dressing change (35.6%) whereas cold environment resulted in itch in only (2.2%). The burden of pruritus increased with increasing age. This study highlights a challenging area in EB care with a need for specific treatments.
