Major Article
Evaluation of vision-specific quality-of-life in albinism

https://doi.org/10.1016/j.jaapos.2008.10.008Get rights and content

Introduction

Human albinism is a genetic condition associated with visual impairment that affects many aspects of daily life. Office measurements of visual acuity do not necessarily reflect daily visual function and health status. This study used the National Eye Institute Visual Function Questionnaire (NEI-VFQ) to determine the effect of albinism-associated ophthalmopathy on quality of life (QOL).

Methods

We administered the NEI-VFQ, which consists of 25 questions about QOL (VFQ-25) and 14 questions about well-being (VFQ-39), to 44 consenting participants with albinism older than the age of 18 years.

Results

Nineteen male and 25 female subjects completed the study. Median age was 30.5 years (range, 18–79 years). Mean best-corrected visual acuity was 20/83 (range, 20/20 to 20/320). Forty-eight percent reported that they were currently able to drive with their condition. Participants perceived midscale problems with their general vision (median subscale score 60.0). Visual acuity correlated moderately with overall NEI-VFQ composite scores (rs = 0.40, p = 0.01 for VFQ-25 and rs = 0.36, p = 0.02 for VFQ-39). Most notable impairment was recorded for distance acuity, vision-specific mental health, and vision-specific role difficulties (VFQ-39 median subscale scores 66.7, 70.0, and 75.0, respectively). Differences by sex were insignificant. Greater ceiling effects were noted for the VFQ-25 than for the VFQ-39.

Conclusions

The NEI-VFQ-39 is a method to evaluate self-reported effects of vision-related QOL in albinism and may be used as a baseline for evaluating outcomes in interventional studies in these patients.

Section snippets

Methods

Individuals with albinism who were at least 18 years of age were recruited at the 11th meeting of the National Organization of Albinism and Hypopigmentation in Minneapolis, Minnesota, July 2006, or from the ophthalmology practice at the University of Minnesota, Minneapolis. Participants provided written informed consent. The project was approved by the Institutional Review Board at the University of Minnesota and was conducted in compliance with the Health Insurance Portability and

Results

Forty-four adults participated in this study. Demographic information about this cohort is presented in Table 1. All but one had graduated from high school, 35 attended college, and 10 finished graduate school. At the time of participation, all were either in college, employed full-time, or retired from a full-time vocation. Forty-eight percent (21 of 44) were able to drive with their condition. Approximately 52% of participants were affected by a condition listed on the general health

Discussion

The authors of several studies have used the NEI-VFQ-25 to study quality of life in ophthalmic disorders. Although this means of evaluation was designed to evaluate health-related QOL in persons with common ocular pathology, it has also been shown to be effective in the assessment of patients with less common ophthalmic conditions.2, 5, 6 The vision-related subscale results reported here for individuals with albinism are similar to those reported for individuals with moderate-to-severe diabetic

References (15)

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Presented in part at the 33rd Annual Meeting of the American Association for Pediatric Ophthalmology and Strabismus, Seattle, Washington, April 12–15, 2007.

Supported by an unrestricted grant from Research to Prevent Blindness, Inc., New York, NY.

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