Sickle Cell Retinopathy in Jacksonville, Florida
Sickle cell disease damages the small blood vessels of the peripheral retina. For years it causes no symptoms at all — and then, without warning, an abnormal tuft of new vessels bleeds and vision goes. Annual dilated examination is what prevents that, and most people with sickle cell disease are not getting one.
What happens in the eye
Sickled red cells obstruct the smallest vessels at the far edge of the retina. Those areas lose their blood supply, and the oxygen-starved retina responds by growing fragile new vessels — the fan-shaped tufts called sea fans. Sea fans bleed into the vitreous and can pull the retina off as they contract.
All of this happens in the periphery, far outside the field of view you are aware of. That is why the disease is silent until it is not.
The counterintuitive part: genotype
Proliferative sickle retinopathy is several times more common in HbSC disease than in HbSS disease — even though HbSS causes the more severe systemic illness. This gets stated backwards on a great many websites.
The reason is blood viscosity. Patients with HbSC have a higher haematocrit and thicker blood, with less severe haemolytic anaemia, and that thicker blood promotes vaso-occlusion in the small retinal vessels. The profound anaemia of HbSS is, in the retinal circulation specifically, relatively protective. HbS-beta-thalassaemia sits between the two.
If you have HbSC disease and have been told your sickle cell is the milder kind, that is true systemically and not true for your eyes.
Screening — the single most important thing on this page
Annual dilated retinal examination beginning at age 10, for every genotype. Some recommend starting at 8.
The examination must include the far peripheral retina with scleral depression, because that is where the disease lives. A standard eye examination that does not look out to the edge will miss it.
Screening rates in sickle cell disease are documented to be poor. If you have sickle cell disease and have not had a dilated eye exam in the last year, that is the reason to call, not symptoms.
Symptoms — and why they arrive late
- Sudden floaters, or a shower of dark specks — vitreous haemorrhage
- A shadow or curtain across the vision — retinal detachment
- Sudden painless loss of vision
- Rarely, sudden loss from a central retinal artery occlusion
These are all late findings. Any of them is a same-day call.
Separately, a hyphaema — blood in the front chamber of the eye after trauma — is a genuine emergency in anyone with sickle cell trait or disease, because sickled cells block the eye's drainage and the pressure can rise fast enough to damage the optic nerve within hours. That includes people with sickle trait, who otherwise have no eye risk.
How we examine and image the eye
Dilated examination with scleral depression, plus imaging performed in our office the same visit:
- Ultra-widefield fundus photography — captures the far periphery in a single image, which is exactly where this disease is, and gives a baseline to compare against year on year
- Fluorescein angiography — shows non-perfused peripheral retina and confirms active sea fans
- OCT and OCT angiography — detect macular thinning and small areas of capillary loss at the centre, which occur in sickle disease and can affect vision without any visible sea fan
More about our imaging and diagnostic testing.
Treatment
Observation
Not passivity. A substantial proportion of sea fans — reported between 20 and 60 percent — close off spontaneously. That is a real phenomenon called autoinfarction, and it is why limited neovascularisation is often watched rather than treated.
Scatter laser photocoagulation
We perform scatter laser around areas of sea fan neovascularisation in proliferative disease. The randomised evidence shows a real but modest benefit: treated eyes had prolonged visual loss from vitreous haemorrhage in about 1 percent of cases against nearly 7 percent of untreated eyes, and a lower rate of vitreous haemorrhage overall. New sea fans still formed in about a third of treated eyes.
Scatter technique around the sea fan has replaced the older feeder-vessel approach, which carried more complications.
Anti-VEGF injections
Anti-VEGF can cause neovascularisation to regress and is used in selected cases, particularly with vitreous haemorrhage. It is off-label for this indication and the prospective randomised evidence is not yet in — a trial is ongoing. We would use it as an adjunct in the right circumstance, not as established therapy, and we would tell you that is what we were doing.
Vitrectomy
For vitreous haemorrhage that does not clear and for tractional retinal detachment. Surgery in sickle eyes requires particular care with intraoperative pressure and oxygenation, and it is one of the situations where having the systemic disease well understood matters.
Working with haematology
Dr. Elkeeb is board certified in Internal Medicine as well as Ophthalmology, and we coordinate directly with the haematologists managing your sickle cell disease rather than sending a form letter. Your eye findings are part of your overall disease picture, and your systemic management — hydroxyurea, transfusion history, crisis frequency — is part of ours.
Questions patients ask
My sickle cell has never caused me problems. Do I still need eye exams?
Yes, and if you have HbSC disease, especially so. Retinal disease tracks the opposite way from systemic severity, and it is silent until it bleeds.
I have sickle cell trait, not disease. Does this apply to me?
Proliferative retinopathy is very uncommon in trait alone. The one thing that does apply is eye trauma: a hyphaema in someone with sickle trait is an emergency, because the pressure can rise dangerously fast.
Will laser fix it?
Laser reduces the risk of bleeding and of losing vision. It does not cure the underlying vascular disease, and new sea fans can still appear, which is why treatment does not end the need for follow-up.
Can sickle cell affect the centre of my vision?
Yes. Small areas of capillary loss at the macula are common in sickle disease and can be seen on OCT angiography even when the peripheral retina looks manageable. They can subtly reduce vision without any obvious cause on a standard exam.
Getting seen
We accept urgent referrals and see urgent cases within 24 hours. Call (904) 666-5050. Our office is open 8:00 am to 5:00 pm, Monday to Friday, at 3627 University Blvd S, Suite 605, Jacksonville, FL 32216. Our staff speak English, Arabic, and French.
Dr. Elkeeb operates at HCA Florida Memorial Hospital and Baptist Medical Center Jacksonville.
Referring physicians and optometrists: our referral information is here.
Sources: EyeWiki, Sickle Cell Retinopathy (American Academy of Ophthalmology); Retinopathy in Hemoglobinopathies, StatPearls, 2023; American Society of Retina Specialists patient information, Sickle Cell Retinopathy; update on sickle cell retinopathy management, Retina Today, November/December 2025.
This page is general information about a medical condition and is not a substitute for examination and advice from your own physician. Do not start, stop, or change any medication based on what you read here.