# The eye as a window into sickle cell disease morbidity

> **NIH NIH P20** · NEMOURS CHILDREN'S HOSPITAL, DELAWARE · 2021 · $318,573

## Abstract

PROJECT SUMMARY/ABSTRACT
Sickle cell disease (SCD) is the most common genetic mutation among the US population, affecting
approximately 100,000 Americans. As a systemic illness, SCD attacks multiple organs including the eyes.
Although SCD can affect nearly all structures in the eye, sickle cell retinopathy (SCR) is the vision threatening
complication with a reported 10% blindness rate. SCR damage often begins in childhood and progresses with
age. Early in SCR, small peripheral retinal vascular occlusions, also known as “retinal stroke”, are typically
asymptomatic. However, as the disease progresses, abnormal blood vessels begin to grow (neovascularization
or proliferative change). Advanced proliferative SCR can lead to intraocular hemorrhage, retinal detachment and
vision loss. Currently, there are no proven methods to prevent SCR, and treatment options are focused on
deterring the progression of proliferative SCR. Prompt diagnosis of SCR is an important step for early treatment,
which can prevent vision loss. The diagnostic strategies focus on visualizing signs of retinal stroke and abnormal
vessel growth. NIH guidelines recommend annual screening for SCR by dilated funduscopic examination of the
eye beginning at age ten, which has low sensitivity. The pathological process of retinal neovascularization is a
complex phenomenon under active investigation. The research proposed in this application, The Eye as a
Window into Sickle Cell Disease Morbidity, will utilize a combination of standard and novel noninvasive
diagnostic modalities to examine retinal circulation and structure changes in children with SCD. Using these
techniques will improve our ability to accurately diagnose and monitor SCR in children and provide a better
understanding of the mechanism of the disease. Furthermore, the value of sensitive and specific assessments
of ocular changes in SCD is not limited to saving vision. For many neurologic conditions and systemic illnesses,
ocular findings aid in both diagnosis and monitoring of overall disease progression. Thus, findings from the
proposed ophthalmologic examinations may elucidate a link between retinal damage and damage to other at-
risk organs, especially the brain in SCD. Through this study, we will refine our ability to predict progression,
assess therapeutic efficacy, develop more effective monitoring guidelines and initiate early interventions.
Correlating retinal findings with clinical data may provide insight into other vascular complications of SCD, such
as cerebrovascular disease. The long-term goal of this study is to enhance early detection of SCR and develop
treatment strategies to slow the progression of SCR toward proliferative retinopathy and blindness, lowering the
societal burden of SCR and improving the quality of life for these patients.

## Key facts

- **NIH application ID:** 10271046
- **Project number:** 2P20GM109021-06
- **Recipient organization:** NEMOURS CHILDREN'S HOSPITAL, DELAWARE
- **Principal Investigator:** JING JIN
- **Activity code:** P20 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2021
- **Award amount:** $318,573
- **Award type:** 2
- **Project period:** 2014-08-01 → 2024-06-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10271046

## Citation

> US National Institutes of Health, RePORTER application 10271046, The eye as a window into sickle cell disease morbidity (2P20GM109021-06). Retrieved via AI Analytics 2026-05-23 from https://api.ai-analytics.org/grant/nih/10271046. Licensed CC0.

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