Study finds that 1 in 3 children with sickle cell disease has retinopathy.
While the inherited disorder in rare, people with sickle cell disease can develop serious vision complications. The disease gets its name from the shape of the red blood cells that become hardened and change shape. Sickle retinopathy is an age-dependent process, with older people being at substantially higher risk, than younger patients.
Researchers at the University of Tennessee Health Science Center conducted a large, retrospective review at their institution with surprising results: 1 in 3 children had retinopathy, of which 9 percent required treatment. These findings suggest children need to be screened for vision problems as often as adults with sickle cell disease.1
Lead researcher, Mary Ellen Hoehn, MD, professor of Ophthalmology at University of Tennessee Health Science Center shared the importance of “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.” 1
In an evaluation of therapies for this condition, Hoehn and colleagues also found that hydroxyurea and chronic transfusions were associated with decreased rates of retinopathy, even when accounting for different genotypes.1
To conduct the study, the team 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:
“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.1