Researchers reported that smoking and blood hemoglobin levels were among the modifiable risk factors for retinopathy related to sickle cell disease (SCD), according to a study published in the International Journal of Retina and Vitreous.

Patients with SCD have sickle-shaped erythrocytes that often lead to painful vaso-occlusive crises, with sickle cell retinopathy (SCR) being one of the more severe manifestations of the disease. 

Studies demonstrate that ocular complications of SCD increase with age; they are hence more common in adults than in children. To prevent disease progression to retinopathy, it is important to identify risk factors for SCR, especially ones that are modifiable. 

The authors of this study therefore set out to understand the risk factors associated with SCR to devise adequate disease prevention strategies. They analyzed data from the Cooperative Study of Sickle Cell Disease, a study conducted from 1977 to 1995 aimed at determining the clinical course of SCD from birth to death. The research team analyzed data of patients who had undergone an ophthalmic evaluation by a trained ophthalmologist. 

Read more about SCD etiology

The results showed that around half of the patients recruited had SCR. In addition, the researchers discovered a number of risk factors associated with proliferative SCR: smoking, older age, higher white blood cell count, higher reticulocyte count, higher hemoglobin levels, and previous blood transfusions. SCR was also more common in men than in women. 

“Several risk factors capable of being modified are associated with development of SCR and its risk of progression to proliferative disease,” the authors of the study wrote. “In particular, promoting smoking cessation and efforts to optimize hematologic profiles are important for reducing the morbidity associated with the development of SCR.”

SCD affects around 100,000 individuals in the United States alone. The disease carries with it significant morbidity and mortality.


Nawaiseh M, Roto A, Nawaiseh Y, et al. Risk factors associated with sickle cell retinopathy: findings from the Cooperative Study of Sickle Cell DiseaseInt J Retina Vitreous. 2022;8(1):68. doi:10.1186/s40942-022-00419-8