Sickle cell disease is a rare, inherited disorder in which the red blood cells become hard, sticky, and change shape, resembling the farm tool. People who have it can sometimes develop vision problems when these sickle-shaped cells get trapped in the small blood vessels as the back of the eye. Sickle retinopathy is an age-dependent process, with older people being at substantially higher risk, than younger patients.

To learn more about how this condition manifests in children, researchers at the University of Tennessee Health Science Center conducted a large, retrospective review at their institution. What they found surprised them. One in three children had retinopathy, of which 9 percent required treatment, suggesting children need to be screened for vision problems as often as adults with sickle cell disease. The study will be presented today at AAO 2023, the 127th annual meeting of the American Academy of Ophthalmology.

Our data underscores the need for patients — including pediatric patients– with sickle cell disease to get routine ophthalmic screenings along with appropriate systemic and ophthalmic treatment."

Mary Ellen Hoehn, MD, lead researcher, professor of Ophthalmology at University of Tennessee Health Science Center

Dr. Hoehn and colleagues also evaluated the effectiveness of different therapies for sickle cell disease. They found that hydroxyurea and chronic transfusions were associated with decreased rates of retinopathy, even when accounting for different genotypes.

To conduct the study, they evaluated records for 652 patients, aged 10 to 25 years (median age: 14) who underwent eye exams (2,240 visits) over a 12-year period. They found:

• 33 percent had nonproliferative retinopathy (NPR)
• 6 percent had proliferative retinopathy (PR).
• 33 eyes were treated with panretinal photocoagulation, most commonly for PR stage 3 (43 percent). Intravitreal anti-VEGF therapy was given to five eyes, all with PR.
• Other complications included retinal detachment and retinal artery occlusion in two patients each.
• Vision loss (final best corrected visual acuity 20/60) following complications from sickle cell disease was noted in only one patient with a central retinal artery occlusion.

"We hope that people will use this information to better care for patients with sickle cell disease, and that more timely ophthalmic screen exams will be performed so that vision-threatening complications from this disease are prevented," Dr. Hoehn said.