Patient-Reported Health-Related Quality of Life in Individuals with Inherited Retinal Diseases

Purpose: To evaluate the impact of inherited retinal diseases (IRDs) on quality of life (QoL) using multi -attributable health utilities derived from primary patient data.Design: Cross-sectional observational study.Participants: Seventy adult patients (mean age, 42.7 years) with IRD recruited from state-wide services in Australia.Methods: Health utility values were calculated from the Assessment of Quality of Life 8-Dimension (AQoL-8D). Linear regressions were used to analyze the relationship between the 25-item and 39-item National Eye Institute Visual Function Questionnaires (NEI-VFQ-25 and NEI-VFQ-39, respectively) and health utilities from the AQoL-8D. Main Outcome Measures: The AQoL-8D utility values were compared between the IRD cohort and pop-ulation norms. Regressions were used to determine explanatory power of the NEI-VFQ-25 and NEI-VFQ-39 for health utilities from the AQoL-8D.Results: Average health-related utility for patients with IRD was 0.58, significantly lower than population norms of 0.80. The IRD patient scores were significantly lower than population norms for all 8 domains of the AQoL-8D. Regressions showed a statistically significant relationship between the NEI-VFQ-39 and AQoL-8D, with the NEI-VFQ-39 and other clinical data explaining up to 73% of the variation in AQoL-8D values and 69% of the variation in the NEI-VFQ-25 values.Conclusions: Patients with IRD have significantly lower utility values across all dimensions of QoL, with the largest differences in independent living, senses, and relationships. The NEI-VFQ-25 and NEI-VFQ-39 are highly correlated with overall AQoL-8D utilities and, combined with other data, can reasonably estimate QoL utilities required for cost-effectiveness studies. Ophthalmology Science 2022;2:100106 & COPY; 2022 by the American Academy of Ophthalmology. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/ licenses/by-nc-nd/4.0/).