# SCD REVIVE - Retina to Evaluate Vaso occlusion In the Vasculature of the Eye

> **NIH NIH R01** · ICAHN SCHOOL OF MEDICINE AT MOUNT SINAI · 2024 · $793,654

## Abstract

Sickle Cell Disease (SCD) is a high-morbidity, beta-globin blood disorder that causes hemolysis and vaso-
occlusion, leading to pain, organ damage and premature death. A key barrier to progress is that current
disease-monitoring biomarkers correlate weakly with clinical outcomes because they do not directly measure
the mechanisms that cause clinical pathology. The transparent media of the eye presents the opportunity to
directly visualize the retinal microvasculature, as an indirect representation of the microvascular status of other
organ systems and to quantify transient interruptions in blood flow (a major cause of SCD pathology). Our
group found that new approaches to quantifying retinal perfusion abnormalities, such as mapping variably
perfused areas and comparing them over minutes to hours, can produce reliable metrics of retinal perfusion
that predict SCD severity and mortality better than any currently available clinical biomarker. Using Optical
coherence tomography angiography (OCTA) and adaptive optics scanning light ophthalmoscopy (AOSLO) we
hypothesize that innovations in retinal imaging may be leveraged to create new biomarkers to guide disease
monitoring and improve mechanistic understanding of disease. In our preliminary work, we developed several
highly-reliable retinal perfusion metrics, identified 4 mechanisms of small-vessel occlusion in SCD, and showed
that one novel perfusion metric, between-session intermittent flow index (IFI), outperformed all current
biomarkers as a measure of disease severity and predictor of mortality. We propose to conduct a prospective
cohort study using serial retinal imaging and clinical data collection to 1) develop reliable metrics of retinal
perfusion (as determined by coefficients of variation and indexes of individuality), 2) validate perfusion metrics
as objective indicators of disease severity, treatment response and mortality risk, and 3) compare the
mechanisms that cause microvascular occlusion among the 5 major SCD phenotypes. To ensure maximum
generalizability and potential for harmonization with other data sources, clinical data will be collected using
tools developed from the NHLBI Sickle Cell Implementation Consortium Clinical Data Registry. To accomplish
these important goals, the proposed project brings together expertise in ophthalmology, retinal imaging, high-
efficiency study design and analyses for rare diseases, stakeholder engagement and SCD.

## Key facts

- **NIH application ID:** 10812349
- **Project number:** 5R01HL159116-03
- **Recipient organization:** ICAHN SCHOOL OF MEDICINE AT MOUNT SINAI
- **Principal Investigator:** Yuen Ping Toco Chui
- **Activity code:** R01 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2024
- **Award amount:** $793,654
- **Award type:** 5
- **Project period:** 2022-04-10 → 2027-03-31

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10812349

## Citation

> US National Institutes of Health, RePORTER application 10812349, SCD REVIVE - Retina to Evaluate Vaso occlusion In the Vasculature of the Eye (5R01HL159116-03). Retrieved via AI Analytics 2026-05-21 from https://api.ai-analytics.org/grant/nih/10812349. Licensed CC0.

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