July 2019
Volume 60, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2019
The Visual Function Index (VF-14) reflects a notable loss in Vision-Related Quality of Life (VRQOL) in Stargardt Disease
Author Affiliations & Notes
  • Justin Tyler Zaremba
    Ophthalmology, Alkeus Pharmaceuticals, Somerville, Massachusetts, United States
  • Zach Strecker
    Ophthalmology, Alkeus Pharmaceuticals, Somerville, Massachusetts, United States
  • Gabrielle DeBartolomeo
    Ophthalmology, Alkeus Pharmaceuticals, Somerville, Massachusetts, United States
  • Stephen H Tsang
    Ophthalmology, Columbia University Medical Center, New York, New York, United States
  • Christine Nichols Kay
    Ophthalmology, Vitreoretinal Associates, Gainesville, Florida, United States
  • Thomas B. Connor
    Ophthalmology, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
  • Michael B Gorin
    Ophthalmology, Stein Eye, Los Angeles, California, United States
  • Paul S Bernstein
    Ophthalmology, Univ of Utah/Moran Eye Center, Salt Lake City, Utah, United States
  • Byron L Lam
    Ophthalmology, Bascom Palmer Eye Institute, Miami, Florida, United States
  • Ilyas Washington
    Ophthalmology, Alkeus Pharmaceuticals, Somerville, Massachusetts, United States
  • Hendrik P Scholl
    Dept. of Ophthalmology, University of Basel, Basel, Switzerland
    Institute of Molecular and Clinical Ophthalmology (IOB), Basel, Switzerland
  • Leonide Saad
    Ophthalmology, Alkeus Pharmaceuticals, Somerville, Massachusetts, United States
  • Footnotes
    Commercial Relationships   Justin Zaremba, Alkeus Pharmaceuticals (E); Zach Strecker, Alkeus Pharmaceuticals (E); Gabrielle DeBartolomeo, Alkeus Pharmaceuticals (E); Stephen Tsang, None; Christine Kay, None; Thomas Connor, None; Michael Gorin, None; Paul Bernstein, None; Byron Lam, None; Ilyas Washington, Alkeus Pharmaceuticals (C), Alkeus Pharmaceuticals (P); Hendrik Scholl, None; Leonide Saad, Alkeus Pharmaceuticals (E), Alkeus Pharmaceuticals (S), Alkeus Pharmaceuticals (I)
  • Footnotes
    Support  FDA Grant FD004098
Investigative Ophthalmology & Visual Science July 2019, Vol.60, 4970. doi:
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      Justin Tyler Zaremba, Zach Strecker, Gabrielle DeBartolomeo, Stephen H Tsang, Christine Nichols Kay, Thomas B. Connor, Michael B Gorin, Paul S Bernstein, Byron L Lam, Ilyas Washington, Hendrik P Scholl, Leonide Saad; The Visual Function Index (VF-14) reflects a notable loss in Vision-Related Quality of Life (VRQOL) in Stargardt Disease. Invest. Ophthalmol. Vis. Sci. 2019;60(9):4970.

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      © ARVO (1962-2015); The Authors (2016-present)

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Purpose : The VF-14 is a 14-item questionnaire designed to assess visual function impairment and generate an aggregate score (AS) between 0 and 100 measuring the difficulty due to decreased vision, to complete ordinary daily tasks. Healthy controls score above 95 points on average. Research indicates that advanced age-related macular degeneration (AMD), preoperative cataract and glaucoma have approximate AS of 62, 73, and 79, respectively. Stargardt disease (STGD1) is a common juvenile macular degeneration. To date, there have been no reports of aggregated VF-14 data in STGD1. Here, we present VF-14 data collected from subjects with ABCA4-confirmed STGD1. We hypothesize a correlation between AS and psychophysical tests.

Methods : 48 of 50 subjects with STGD1, taking part in a Phase 2 prospective clinical trial, completed a paper, self-reported VF-14 as part of a baseline visit, usually administered before pupillary dilation and as one of the first procedures. Other baseline data include age (mean: 44 years, range:12-60), age of onset (32 years, 9-56), best-corrected visual acuity (BCVA, 20/80, 20/20-20/1000), reading speed (104 words/minute [wpm],12-199), atrophic lesion size (6.8mm2, 0.1-28.2), mean retinal sensitivity by fundus-tracking microperimetry (MP) (8.3 dB, 1.7-16.6), mean central 2° MP fixation (60%, 12-99%), and central 4° MP fixation (80%, 34-100%). Descriptive statistics and linear regression analyses are performed between AS and baseline data.

Results : Mean AS was 60 (range: 25-93, SD: ±18) with a minority (10%) of subjects scoring greater than 80. Pearson correlations are displayed in Table 1. Of note, the highest correlation we found was between AS and the absolute difference in BCVA between eyes (r=0.42). Consistent with data from cataract, a moderate correlation was found between AS and BCVA in the better eye (r=0.3), but not with the worse eye (r=-0.02). Table 2 provides detailed descriptions of item-specific VF-14 showing which tasks STGD1 subjects reported most and least difficult.

Conclusions : This is the first report of VF-14 in subjects with STGD1. VF-14 score in STGD1 was found to be similar to that of advanced AMD, and one of the lowest reported scores among eye diseases. The VF-14 should continue being used to further explore the impact of STGD1 on VRQOL.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.




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