Seeing Sickle Cell Retinopathy Anew

Adrienne Scott, M.D., chose to join the ranks of retina specialists because, she says, they are known as “the Marines of the eyes. We’re the people they call in when an urgent problem arises.” An assistant professor of ophthalmology in Wilmer’s Retina Division, Scott also serves as the chief of the Wilmer satellite office in Bel Air.

While she sees patients with all manner of retinal diseases, she has a specific interest in sickle cell retinopathy, a condition caused by sickle cell disease. Inspired by a patient who passed away a few years ago from complications of sickle cell disease who told her “we need to cure this,” Scott decided to dedicate herself to studying sickle cell retinopathy.

The most commonly inherited blood disorder, sickle cell disease occurs when the normally sphericalshaped red blood cells have a sickle shape, which deprives body parts of oxygen. “Sickle cell patients can have problems with every organ system in the body, but the eyes are particularly vulnerable given all the small vessels, especially within the retina,” says Scott.

With the arrival of new imaging techniques—such as optical coherence tomography (OCT) angiography—that can help visualize the retina in finer detail than ever before, Scott believes research into sickle cell retinopathy is poised for a breakthrough. “We’re using OCT angiography to visualize blood flow at a very detailed level. And we can not only visualize blood flow, but also in the case of sickle cell retinopathy, we can visualize lack of blood flow in the retinal circulation,” says Scott. 

Traditionally, sickle cell retinopathy has been considered a disease of the periphery of the retina—and damage to this area causes the most severe vision loss. With OCT angiography, however, Scott has seen changes in the blood flow around the macula—the part of the retina responsible for central vision.

If Scott finds a correlation between lack of blood flow to the macula, which OCT angiography can visualize quite clearly, and lack of blood flow to the periphery of the retina, this could help doctors treat patients more effectively. OCT angiography could serve as a fast and noninvasive screening tool to predict which patients could be most at risk for future vision loss and thus require closer monitoring.

That would be a welcome development for this clinicianscientist whose focus on her patients propels her work. “I hear from people over and over, ‘You can take any sense of mine, but I cannot lose my eyesight,’” Scott says. “It’s a very critical sense and it’s an honor to be able to try to maintain that.”