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The study stopped because patients dropped out of the study to participate in the interventional trials. With interventional trials present there is very little interest in participating in a Natural Histoy study.
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This study will assess the progression of RP as seen on newer modalities including spectral-domain optical coherence (SD-OCT) and macular assessment integrity (MAIA) microperimetry to evaluate disease status. Understanding the natural history of the disease is not only essential to monitoring and comparing patient populations in clinical trials. It is also fundamental in the predevelopment phase in order to optimize the study duration needed to observe a statistically significant outcome. Furthermore, since the progression of RP is usually slow, relying on traditional tests can take an unfeasible length of time to observe any meaningful changes and assess therapeutic efficacy for new drugs. Therefore, the results of this study will be beneficial in establishing reliable endpoints and outcome measures for future clinical trials. Such outcome measures may be able to detect treatment response with more precision. More importantly, investigators may be able to detect changes early enough to prevent irreversible vision loss.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Retinitis Pigmentosa | Patients with Retinitis Pigmentosa |
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| Measure | Description | Time Frame |
|---|---|---|
| Change in mean macular sensitivity (dB) over time as assessed by microperimetry | Microperimetry (MAIA) will be used to test whether there is a change in sensitivity (dB) in the macula | Baseline, every six months up to 2 years |
| Measure | Description | Time Frame |
|---|---|---|
| Change in Best Corrected Visual Acuity (BVCA) | Scoring will be determined by the number of letters gained or lost per month using Early Treatment Diabetic Retinopathy Study (ETDRS) Letter Score and visual acuity score together with an overall score range of 0 to 20/20 where 0 is the worst vision and 20/20 is the best. | Baseline, every six months up to 2 years |
| Measure | Description | Time Frame |
|---|---|---|
| Correlation between baseline functional and anatomical measures | Visual function parameters at baseline such as Best Corrected Visual acuity (measured using ETDRS and visual acuity scale), mean macular sensitivity (quantified using MAIA microperimetry), mean retinal sensitivity (quantified using static Octopus perimetry) will be correlated to anatomical parameters at baseline such as Ellipsoid width (measurement on Optical Coherence Tomography) |
Inclusion Criteria:
Exclusion Criteria:
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Patients diagnosed with Retinitis Pigmentosa
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| Name | Affiliation | Role |
|---|---|---|
| Peter A Campochiaro, M.D. | Johns Hopkins University | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Wilmer Eye Institute at Johns Hopkins University | Baltimore | Maryland | 21287 | United States |
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| ID | Term |
|---|---|
| D012174 | Retinitis Pigmentosa |
| ID | Term |
|---|---|
| D015785 | Eye Diseases, Hereditary |
| D005128 | Eye Diseases |
| D058499 | Retinal Dystrophies |
| D012162 | Retinal Degeneration |
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| Change in Ellipsoid Zone (EZ) width | This will be assessed by spectral domain optical coherence tomography (SD-OCT) | Baseline, every six months up to 2 years |
| Change in Quality of Life survey metrics | Scoring will be determined by the National Eye Institute's Visual Function Questionnaire (NEI-VFQ-25). It has 25 question elements each with score ranging from 1(excellent) to 6(very poor), therefore a total minimum score of 25 and maximum score 150. | Baseline, every year up to 2 years |
| Change in mean retinal sensitivity | Static Octopus Perimetry will be used to test whether there is a change in mean retinal sensitivity over time using its 30-2 program with III target | Baseline and at 2 years |
| Correlation between change in visual functional and anatomical measures | Change in visual function parameters such as Best Corrected Visual acuity (measured using ETDRS and visual acuity scale), mean macular sensitivity (quantified using MAIA microperimetry), mean retinal sensitivity (quantified using static Octopus perimetry) will be correlated to anatomical parameters such as Ellipsoid width (measurement on Optical Coherence Tomography) | Baseline, every six months up to 2 years |
| Correlation between change in visual functional measures and Quality of Life survey metrics | Change in Quality of Life survey metrics (Scored using National Eye Institute's Visual Function Questionnaire, NEI-VFQ-25) will be compared to visual function parameters such as Best Corrected Visual acuity (measured using ETDRS and visual acuity scale), mean macular sensitivity (quantified using MAIA microperimetry), mean retinal sensitivity (quantified using static Octopus perimetry) | Baseline, every year up to 2 years |
| Baseline, up to 2 years |
| Correlation between baseline functional measures and Quality of Life survey metrics | Visual function parameters at baseline such as Best Corrected Visual acuity (measured using ETDRS and visual acuity scale), mean macular sensitivity (quantified using MAIA microperimetry), mean retinal sensitivity (quantified using static Octopus perimetry) will be correlated to baseline Quality of Life survey metrics (Scored using National Eye Institute's Visual Function Questionnaire, NEI-VFQ-25) | Baseline, up to 2 years |
| Correlation between functional, anatomic and Quality of Life measures | Visual function parameters such as Best Corrected Visual acuity (measured using ETDRS and visual acuity scale), mean macular sensitivity (quantified using MAIA microperimetry), mean retinal sensitivity (quantified using static Octopus perimetry), anatomical parameters such as Ellipsoid width (measurement on Optical Coherence Tomography) Quality of Life survey metrics (Scored using National Eye Institute's Visual Function Questionnaire, NEI-VFQ-25) will be correlated. | Baseline, up to 2 years |
| Proportion of eyes with ≥ 5 loci that show ≥ 6 decibels (dB) decline in mean macular sensitivity from baseline | This will be measured using MAIA microperimetry | Baseline, every six months up to 2 years |
| Proportion of eyes with ≥ 5 loci that show ≥ 7 decibels (dB) decline in mean retinal sensitivity from baseline | This will be measured by static Octopus perimetry using 30-2 program with III target | Baseline and at 2 years |
| D012164 |
| Retinal Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |