Children need to be screened for vision problems as often as adults with sickle cell disease, study suggests

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

To learn more about how the 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, with 9 percent requiring treatment, suggesting that children need to be screened for vision problems as often as adults with sickle cell disease. The research will be presented today at AAO 2023, the 127th annual meeting of the American Academy of Ophthalmology.

Our data underscore the need for patients — including pediatric patients — with sickle cell disease to receive regular ophthalmologic screening with appropriate systemic and ophthalmic treatment.

Mary Ellen Hoehn, MD, principal investigator, professor of ophthalmology at the 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 reduced rates of retinopathy, even when accounting for different genotypes.

To conduct the study, they evaluated the records of 652 patients aged 10 to 25 years (median age: 14) who underwent eye examinations (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, usually for PR stage 3 (43 percent). Intravitreal anti-VEGF therapy was given in five eyes, all with PR.
  • Other complications included retinal detachment and retinal artery occlusion in two patients.
  • Visual loss (final best corrected visual acuity 20/60) following complications of sickle cell disease was observed in only one patient with central retinal artery occlusion.

“We hope that people will use this information to better care for patients with sickle cell disease, and more timely ophthalmic screening tests to prevent sight-threatening complications from this disease,” said Dr. Hohen said.

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