For many people living with sickle cell disease, the biggest worries are often the painful crises, hospital visits, and complications affecting vital organs. Rarely does vision feature prominently on that list. Yet, doctors say one of the disease’s most serious complications can quietly unfold in the eyes. Ahead of World Sickle Cell Day, observed annually on June 19, experts are highlighting the dangers of sickle cell retinopathy.
To understand why the eyes become vulnerable, it helps to begin with the disease itself and the changes it causes inside the body. “Sickle cell disease affects the eyes because sickling of red blood cells and obstruction of tiny blood arteries affect the retinal blood vessels, leading to disrupted retinal blood flow. This can cause oxygen deprivation, aberrant blood vessel growth, haemorrhage, and scarring that may harm vision,” explains Dr Ganesh Jaishetwar, consultant haematologist, haematologist-oncologist and bone marrow transplant physician at Yashoda Hospitals.
Once those changes begin, a quiet process unfolds inside the eye, often without the patient noticing anything unusual. “Sickle cell retinopathy is an ocular complication of sickle cell disease that occurs when sickled red blood cells repeatedly block small retinal blood vessels, leading to chronic oxygen deficiency in the retinal periphery. Consequently, abnormal and fragile sea fan neovascularisation develops, which may rupture and cause intra-ocular haemorrhage,” says Dr P Muralidhar Rao, senior vitreo-retinal surgeon at Maxivision Superspeciality Eye Hospital, Somajiguda.
As doctors follow patients over the years, they often find that some individuals face a greater threat of serious eye complications than others. “People with some HbS + and HbSC, although HbSS poses a higher risk of additional systemic problems, genotypes with proliferative sickle retinopathy are more common than those with HbSS. Age, a history of vaso-occlusive crises, poorly managed illness, and comorbid illnesses like diabetes or hypertension all raise risk,” notes Dr Ganesh.
What makes the condition especially difficult to detect is that it can remain hidden even while damage is steadily progressing. “Patients may have normal vision while abnormal vascular changes actively develop, and symptoms typically appear only after complications arise, making routine ophthalmic examination essential for detection,” expresses Dr Muralidhar.
This silent progression also sheds light on why routine eye screenings are often missed despite the risks involved. “There are several explanations. Preventive eye exams are frequently subordinated to acute systemic care for sickle cell patients and their families. Inadequate knowledge of eye hazards, practical difficulties (transport, time away from work or school), financial worries, and unequal access to specialised retinal treatments in some areas all play a part,” narrates Dr Ganesh.
The focus then shifts from detection to the possibilities that open up when the disease is identified early. “Early detection of sickle cell retinopathy can help prevent vision loss through regular monitoring and timely treatment, including laser photocoagulation, intravitreal anti-VEGF injections in selected cases, and vitrectomy for complications such as non-resolving vitreous haemorrhage or retinal detachment, while urgent ophthalmic evaluation is required for sudden visual symptoms,” concludes Dr Muralidhar.
Experts stress that protecting sight requires teamwork between patients and healthcare providers. Greater awareness, timely referrals, routine retinal screening, and prompt attention to warning signs such as sudden floaters, flashes of light, blurred vision, or loss of peripheral vision can help prevent avoidable blindness and ensure that eye care keeps pace with advances in sickle cell disease treatment.
