Children With Sickle Cell Disease at Risk for Vision Loss

Clinicians must monitor children with sickle cell disease for eye complications as much as they do for adults, a new research review suggests.

Earlier research indicated that older patients were more at risk for eye complications from sickle cell disease, but the new study found that a full third of young people aged 10-25 years with sickle cell disease had retinopathy, including nonproliferative retinopathy (33%) and proliferative retinopathy (6%), which can progress to vision loss.

Two patients experienced retinal detachment, while two suffered retinal artery occlusion. One patient with retinal artery occlusion lost their vision and had a final best-corrected visual acuity of 20/60, according to the researchers, who presented their findings at the 2023 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, a professor of ophthalmology at the University of Tennessee Health Science Center, Memphis, who led the research, said in a press release.

The review covered records for 652 patients with sickle cell disease aged 10-25 years (median age, 14), who underwent eye exams over a 12-year period.

Besides looking at rates of retinopathy, Hoehn’s group studied which treatments were most effective. They found that hydroxyurea and chronic transfusions best lowered retinopathy rates among all genotypes.

“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,” Hoehn said.

The authors report no relevant financial relationships.

Brittany Vargas is a medicine, mental health, and wellness journalist.