This is an international, multicenter study with two components: Registry * A standardized genetic screening and a prospective, standardized, cross-sectional clinical data collection * Enrollment is open to all genes on the RD Rare Gene List Natural History Study * A prospective, standardized, longitudinal Natural History Study * Enrollment opens gene-by-gene, based on funding and within-gene Registry enrollment The study objectives are as follows. Registry Objectives 1. Genotype Characterization 2. Cross-Sectional Phenotype Characterization (within gene) 3. Establish a Link to My Retina Tracker Registry (MRTR) 4. Ancillary Exploratory Studies - Pooling of Genes Natural History Study Objectives 1. Natural History (within gene) 2. Structure-Function Relationship (within gene) 3. Risk Factors for Progression (within gene) 4. Ancillary Exploratory Studies - Pooling of Genes
This study includes multiple phases. 1. Screening Phase The patient's current genetic report will be reviewed. Genetic testing will not be performed in this study. A prior conclusive genetic test will be assessed for screening analysis. Having at least one gene on the RD Rare Gene List meets one of the eligible Genetic Screening Criteria and other eligibility criteria can be evaluated based on medical history. 2. Genetic Screening Phase: Genetic reports for participants enrolled into the genetic screening phase will be uploaded to study website for review and confirmation by Central Genetics Auditor (CGA) as meeting Genetic Screening Criteria.Participants confirmed as meeting those criteria will be considered enrolled into the Registry. 3. Registry Phase: The flow of participants who are enrolled into the Registry depends on whether their causal gene is designated as a Natural History Study (NHS) Target Gene. If they are not Designated as NHS Target Gene, they will receive annual phone calls up to 48 months from the Registry/Screening visit or until the gene is designated as NHS Target Gene. If they are Designated as NHS Target Gene participants will be considered pending enrollment into the NHS. The Registry will establish genetically and clinically well-characterized cohorts of patients across hundreds of genetic variants associated with retinal dystrophy (RD). Characterization of these patients will accelerate eligibility screening for the Natural History Study, provide cross-sectional data on phenotype-genotype associations, and contribute to our knowledge of pathogenicity of these rare disease-causing variants. 4. Natural History Study (NHS) Phase Participants pending enrollment will return to the clinic for the NHS Enrollment/Baseline Visit and return to the clinic for follow-up visits. The Natural History Study will accelerate the identification and development of sensitive, reliable outcome measures for clinical trials, which will facilitate development of treatments for retinal dystrophies due to disease-causing genetic variants. The expected impact of the Natural History Study is as follows: 1. Describe the natural history of retinal degeneration in patients with rare disease-causing genetic variants 2. Identify sensitive structural and functional outcome 3. Identify well-defined subpopulations for future clinical trials of investigative treatments for rare inherited retinal degeneration
Study Type
OBSERVATIONAL
Enrollment
1,500
University of Arkansas, Jones Eye Institute
Little Rock, Arkansas, United States
RECRUITINGUSC Roski Eye Institute
Los Angeles, California, United States
NOT_YET_RECRUITINGUniversity of California San Francisco
San Francisco, California, United States
RECRUITINGUniversity of Florida Health Jacksonville
Jacksonville, Florida, United States
Functional Outcome: Characterize change using Visual field sensitivity measured with quantitative topographic analysis (hill of vision [HOV])
Measured by Static Perimetry (SP) using Octopus 900 Pro
Time frame: Baseline and every year until study completion (4 years)
Functional Outcome: Characterize Change Using Early Treatment of Diabetic Retinopathy Study (ETDRS) / HOTV Best Corrected Visual Acuity (BCVA) letter score
Measured by Electronic Visual Acuity (EVA) system or ETDRS/HOTV charts
Time frame: Baseline and every year until study completion (4 years)
Functional Outcome: Characterize Change Using Low visual acuity test - for participants unable to see ETDRS letters
Measured by Berkeley Rudimentary Vision Test (BRVT) for Low Visual Acuity
Time frame: Baseline and every year until study completion (4 years)
Functional Outcome: Characterize Change Using ETDRS/HOTV best corrected low luminance visual acuity letter score
Measured by Electronic Visual Acuity (EVA) system or ETDRS/HOTV charts
Time frame: Baseline and every year until study completion (4 years)
Functional Outcome: Characterize Change in Mean retinal sensitivity
Measured by Fundus guided Microperimetry (MP) using MAIA
Time frame: Baseline and every year until study completion (4 years)
Functional Outcome: Characterize Change in Contrast sensitivity function
Measured by Contrast sensitivity CSV-1000E chart
Time frame: Baseline and every year until study completion (4 years)
Functional Outcome: Characterize Change in Retinal function using amplitudes and timing in response to rod- and cone-specific stimuli
Measured by Full-field Electroretinogram (ffERG) Diagnosys Espion
Time frame: Baseline and at study completion (4 years)
Functional Outcome: Characterize Change in Full-field retinal sensitivity
Measured by Full-field stimulus threshold (FST) testing to blue, white, and red stimuli using Diagnosys Espion
Time frame: Baseline and every year until study completion (4 years)
Functional Outcome: Characterize Change in Color vision function
Measured by Color vision testing using Lanthony D15
Time frame: Baseline and every year until study completion (4 years)
Structural Outcome: Characterize Change in Ellipsoid zone (EZ) area; outer nuclear layer and ganglion cell layer thicknesses
Measured by Spectral Domain Optical Coherence Tomography (SD-OCT) using Heidelberg Spectralis
Time frame: Baseline and every year until study completion (4 years)
Structural Outcome: Characterize Change Using Qualitative and quantitative assessments of autofluorescence pattern
Measured by Fundus Autofluorescence (FAF) using Optos
Time frame: Baseline and every year until study completion (4 years)
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University of Miami, Bascom Palmer Eye Institute
Miami, Florida, United States
RECRUITINGEmory University, Emory Eye Center
Atlanta, Georgia, United States
RECRUITINGJohns Hopkins University, Wilmer Eye Institute
Baltimore, Maryland, United States
RECRUITINGHarvard Univ., Massachusetts Eye and Ear Infirmary
Boston, Massachusetts, United States
RECRUITINGUniversity of Michigan, Kellogg Eye Center
Ann Arbor, Michigan, United States
RECRUITINGMayo Clinic
Rochester, Minnesota, United States
RECRUITING...and 26 more locations