Treatment satisfaction in erpidermolysis bullosa patients: the impact of demographic and clinical factors

Authors : Abdulkadir Calavul

Pages : 246-254

Doi:10.18663/tjcl.1624016

View : 32 | Download : 22

Publication Date : 2025-06-30

Article Type : Research Paper

Abstract :Aim: Epidermolysis bullosa (EB) is a hereditary disease which causes skin and mucous membrane blistering. While both standard and multidisciplinary care approaches exist for managing EB, the impact of care type on treatment outcomes remains incompletely understood, particularly across different EB subtypes. To compare treatment satisfaction and quality of life outcomes between standard versus multidisciplinary care approaches in pediatric EB patients, and to identify key demographic and clinical factors influencing these outcomes. Material and Methods: This retrospective study evaluated 32 pediatric EB patients (age <16 years) receiving either multidisciplinary care (n = 18) or standard care (n = 14). Multidisciplinary care involved coordinated management by dermatologists, wound care specialists, pain management experts, psychologists and dedicated nurses, while standard care consisted of routine outpatient follow-up. Treatment outcomes were assessed using the validated Epidermolysis Bullosa Quality of Life (EB-QoL) scale at baseline and 6 months. Statistical analysis included repeated measures ANOVA, independent t-tests, and multiple regression analysis, with Levene\\\'s test confirming variance homogeneity. Results: While baseline EB-QoL scores were comparable (44.8 ± 8.1 vs 45.2 ± 7.8, p = 0.876), the multidisciplinary care group showed significantly higher scores at 6 months (68.4 ± 9.2 vs 52.3 ± 8.7, p = 0.003). The magnitude of improvement varied by EB subtype, with Simplex patients showing the largest gains (baseline: 60.4 ± 7.2, 6-month: 71.2 ± 8.4) and Dystrophic patients the smallest (baseline: 38.6 ± 6.8, 6-month: 45.3 ± 7.8). Multiple regression analysis identified age (β = 0.324), BMI (β = 0.195), and multidisciplinary care (β = 0.468) as positive predictors of satisfaction, while disease duration (β = -0.286) and comorbidities (β = -0.245) had negative effects. Conclusions: Multidisciplinary approaches to pediatric EB patients benefited from comprehensive care models. These results banner the magnitude of benefit, which relies heavily on skeletal structure. The severity of treatment outcomes was noticeably improved through the effect of structured multidisciplinary care. Each sub-type of EB affliction had improved treatment results but each diverged in the level of gain, which further enhances the need for individual tailored treatment protocols based on EB subtype classification and other parameters.
Keywords : Epidermolizis Bülloza, Pediatrik Hastalar, Multidisipliner Bakım, Yaşam Kalitesi, Psikososyal Destek

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