Abstract
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Purpose:Dystrophic epidermolysis bullosa (DEB) is a devastating condition that causes painful corneal abrasions and vision loss. Epidermolysis Bullosa Eye Disease Index (EB-EDI) for the first time captures and quantifies EB-specific assessment of ocular symptoms and activities of daily living scales. This survey will become critical in developing new interventions on patients' quality of life.Methods:Three-part set of the EB-EDI baseline, EB-EDI interval, and Ocular Surface Disease Index (OSDI) survey was distributed to 92 patients with DEB who previously reported eye symptoms on previous surveys. It was then posted online through several EB patient organizations. We compared the EB-EDI with the gold standard OSDI and examined the repeatability of the EB-EDI over a 7- to 15-day interval.Results:Of the 45 individuals who initially responded, 30 of 45 (67%) completed the surveys sent 7 to 15 days later. The age of participants ranged from 6 to 51 years (mean 21 ± 15 years), and 60% (18 of 30) of participants were younger than 18 years. The overall Cronbach alpha values for the subscales of EB-EDI baseline and interval tools presented a good internal consistency (≥0.7). From 2 visits, the domain scores of EB-EDI baseline (0.94) and interval tools (0.83) were shown to have excellent test-retest reliability (intraclass correlation coefficient >0.8). By comparison, OSDI had the intraclass correlation coefficient score of 0.72 ± 0.11. The convergent validation analysis showed that correlations between the domain scores of EB-EDI baseline and interval tools and the subscales of the OSDI reached the hypothesized strength.Conclusions:Based on a 30-person repeated-measures study, we found that the EB-EDI has excellent reliability and validity specifically in patients with DEB.