Sickle cell disease can cause various eye-related complications, including retinopathy. If untreated, sickle cell retinopathy can cause permanent vision loss. Treatments include laser therapy and eye injections.

Sickle cell disease (SCD) is a genetic condition that causes your red blood cells (RBCs) to become rigid and sickle-shaped. Unhealthy RBCs tend to build up in blood vessels throughout your body. RBC buildup in blood vessels in your eyes can potentially lead to vision problems.

Keep reading to learn more about how SCD can affect your eyes and what you can do to prevent vision loss.

SCD can have several effects on your eyes, with sickle cell retinopathy among the most common.

In sickle cell retinopathy, sickle-shaped RBCs block the tiny blood vessels in your retina (a thin layer of tissue that lines the back of your eye on the inside). These blockages disrupt regular blood flow to your retina. Over time, they can cause new, fragile blood vessels to form.

As these new blood vessels expand, they tend to form fan-shaped networks along your retina. This is a serious condition known as proliferative sickle cell retinopathy. The new vessel networks can break, leading to bleeding inside your eye and other complications.

Other eye conditions linked to SCD include:

  • Retinal detachment: A possible complication of sickle cell retinopathy, retinal detachment may occur when the new vessel networks pull on your retina. This action can also cause a retinal tear.
  • Sickle cell maculopathy: This condition occurs when blood supply to the central area of your retina (the macula) is reduced.
  • Retinal artery occlusion: Retinal artery occlusion is a blockage in a blood vessel that supplies blood to your retina.
  • Vitreous hemorrhage: This condition occurs as a result of vaso-occlusive crises. It causes blood vessels to break and blood to leak into the vitreous humor, the clear gel that fills the space between your retina and lens of your eye.

In its early stages, sickle cell retinopathy might not cause any symptoms. But as the condition progresses, you may experience:

Other possible symptoms of sickle cell-related eye conditions include:

What risk factors influence the severity of sickle cell retinopathy?

People with certain risk factors can develop serious complications, including vision loss. These risk factors include:

  • being older
  • being male
  • having hemoglobin SC type SCD (a milder form of SCD in which you have two types of abnormal hemoglobin: hemoglobin S and hemoglobin C)
  • having sickle cell thalassemia

Diagnosing sickle cell-related eye conditions usually involves a thorough eye examination.

An ophthalmologist (a doctor specializing in eye conditions) will likely use several tests, including:

  • Dilated eye exam: This is part of a standard eye exam and allows the doctor to see the back of your eye, where your retina is located.
  • Fluorescein angiography: This is an imaging test doctors can use to view the blood vessels in your retina.
  • Optical coherence tomography: This imaging test provides a detailed view of your retina’s layers to help doctors detect any swelling or damage.

When to contact a doctor

Sickle cell retinopathy may not cause symptoms in the early stages. For that reason, regular checkups with an ophthalmologist are crucial if you have SCD.

The American Academy of Ophthalmology and the National Heart, Lung, and Blood Institute recommend that people with SCD undergo screening for eye conditions every 1 to 2 years.

In addition, be sure to contact a doctor immediately if you have SCD and notice any changes in your vision or the appearance of your eyes.

The treatment for sickle cell retinopathy depends on the severity of the condition. In mild cases, regular monitoring may be all you need. For more advanced cases, treatments may include:

  • laser therapy to shrink abnormal blood vessels in your retina
  • anti-VEGF injections into your eye to stop the growth of new blood vessels
  • vitrectomy, a surgical procedure to remove blood or scar tissue from your eye (often used in cases of retinal detachment)

Treatment for other eye conditions related to SCD may include medications, surgery, or other interventions, depending on the specific issue.

With early detection and proper treatment, the outlook for people with sickle cell retinopathy is usually positive. Regular eye exams are crucial for managing this condition and preventing serious complications.

If left untreated, sickle cell retinopathy can lead to permanent vision loss.

How common is sickle cell retinopathy?

Sickle cell retinopathy affects 15% to 40% of people with SCD in the United States, but not all of them have symptoms. Many people may not know they have this condition.

Why do people with sickle cell have yellow eyes?

Yellowing of the skin and eyes (jaundice) results from a buildup of bilirubin, a substance that is produced when RBCs break down. Jaundice is common in people with SCD because of the increased breakdown of unhealthy RBCs.

Does sickle cell get worse with age?

SCD can worsen with age because complications, including eye conditions, may become more severe over time. Regular medical care is essential to manage these complications.

Does sickle cell trait affect your eyes?

If you have sickle cell trait, it means you carry one copy of the sickle cell hemoglobin gene, while your other copy is unaffected. Sickle cell trait generally does not cause severe symptoms, but there have been rare reports of eye complications, including retinopathy.

Up to 40% of people with SCD in the United States also have sickle cell retinopathy, but many don’t know about it because it may not cause symptoms early on. Regular eye exams are essential for catching any potential issues early.

Sickle cell retinopathy and other eye complications of SCD can lead to serious vision problems, but prompt treatment can help preserve your vision. Don’t wait to contact a doctor if you notice any changes in your vision. Early intervention is key.