Sickle cell disease: The importance of awareness and early detection of ocular manifestations

Abstract

World Sickle Cell Awareness Day is celebrated every June 19 to raise awareness for sickle cell disease (SCD). Access to health services remains unequitable in countries affected by the disease and stigma surrounding patients hinders access to therapies. SCD is the most common severe monogenic disease in the world and is characterized by abnormal hemoglobin production. Major complications include vaso-occlusive events, hemolytic anemia, and inflammation. Microvascular events in the eye are namely responsible for sickle cell retinopathy with or without vasoproliferative changes. Methods included the electronic search of peer-reviewed English literature published until 2021, which was screened, appraised in full version, and incorporated into the review as deemed necessary. This review provides a summary of disease mechanisms and ocular manifestations, and highlights the importance of early diagnosis, close management with imaging technology, and therapeutic avenues for patients with SCD. In addition to significant healthcare barriers encountered by patients and their families, early diagnosis for SCD must be posed by physicians. It is crucial for the healthcare community to become better familiarized with the disease manifestations for early recognition and prevention of chronic complications and morbidity.

Keywords

• disease awareness
• early diagnosis
• healthcare barriers
• sickle cell disease
• sickle cell retinopathy