Sickle cell disease can impact your or your loved one’s health in ways you may not expect. If you notice vision changes, you might not immediately link them to the blood disorder. But as a condition that affects blood cells and blood flow, sickle cell disease can potentially affect almost any part of your body, including your eyes.

Here’s what makes things complicated: Sickle cell retinopathy can develop for years without causing vision problems, so eye care might not be at the forefront of your mind. With frequent doctor appointments for sickle cell disease, ongoing health uncertainty, and unpredictable pain crises, it’s easy for eye care to drop further down the list of priorities.

Here’s what you need to know about protecting your eye health with sickle cell disease.

🗳️ When was the last time you (or your child with sickle cell disease) had a comprehensive eye exam?

Within the past year

With the past two years

Between two and five years ago

Longer than five years ago

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What Is Sickle Cell Retinopathy?

Sickle cell retinopathy is a complication of sickle cell disease that can develop when sickle-shaped red blood cells damage the tiny, delicate blood vessels in your eyes. The changes that occur in your eyes because of sickle cell retinopathy may lead to vision loss over time.

How Sickle Cell Retinopathy Develops

Sickle cell retinopathy starts in the retina, which is the layer of light-sensitive cells in the back of each eye. Sickle-shaped red blood cells can cause blockages in the small blood vessels of your retina and stop the flow of blood. The resulting changes can lead to nonproliferative or proliferative sickle cell retinopathy.

Nonproliferative Sickle Cell Retinopathy

In nonproliferative sickle cell retinopathy (NPSR), the retina develops round bleeding spots called salmon patches. These occur when a blood vessel in the retina bursts after being blocked by sickled red blood cells. Salmon patches are red at first, but they fade over time from red to orange to white or yellow.

The retina can develop circular scars called black sunbursts in NPSR. A person with NPSR may also develop iridescent spots on the retina, which can be seen during an eye exam and appear glittery or glistening. Iridescent spots can appear in place of salmon patches as they heal.

Most people with NPSR don’t notice any symptoms. Signs in the eye are usually detected during routine eye exams. One study estimated 1.4 out of every 1,000 eyes with NPSR experiences vision loss.

Proliferative Sickle Cell Retinopathy

Proliferative sickle cell retinopathy (PSR) is more likely to cause eyesight changes than NPSR. The same study estimated 31 per 1,000 eyes with PSR experience vision loss. You may start out with NPSR and eventually develop PSR.

There are five stages of PSR:

• Stage 1 — Blood vessels in the retina are blocked by sickled red blood cells, which prevents the retina from receiving the oxygen it needs.
• Stage 2 — Since blood can no longer move through the original blood vessels, the eye starts to build new vessels.
• Stage 3 — New blood vessels develop on the surface of the retina, branching out into a “sea fan” shape.
• Stage 4 — The new blood vessels are fragile, so they can rupture and bleed into the eye.
• Stage 5 — After the blood vessels rupture, scarring can develop and cause retinal detachment, when the retina separates from the inner wall of the eye like wallpaper coming off a wall.

Warning Signs of Sickle Cell Retinopathy

Sickle cell retinopathy can progress for a long time before you ever notice symptoms. Your eye doctor might tell you that you have eye changes that indicate sickle cell retinopathy, such as comma-shaped blood vessels or black sunbursts.

If they occur, vision changes usually don’t start until stage 4 or 5 when there’s bleeding in the retina. You’re also more likely to notice vision changes if bleeding in sickle cell retinopathy affects the macula, or center part of the retina.

Tell your eye doctor if you or a loved one with sickle cell disease notices any of these vision changes:

• Eye floaters
• Dark shadows in your vision
• Flashes

Risk Factors for Sickle Cell Retinopathy

People with certain types of sickle cell disease are at a higher risk of having it affect their eyes.

Males with sickle cell disease have a slightly higher risk of developing proliferative sickle cell retinopathy than females, according to the American Academy of Ophthalmology. The risk of sickle cell retinopathy also gets higher with age, so visits with your eye doctor become increasingly important as you get older.

Risk can also depend on your genes. PSR affects about 40 percent of people with heterozygous type of sickle cell disease (only one copy of the HBB gene makes abnormal hemoglobin), whereas around 20 percent of people with homozygous sickle cell disease (both copies of the HBB gene make abnormal hemoglobin) develop PSR.

How Eye Specialists Treat Sickle Cell Retinopathy

Treatments for sickle cell disease might help prevent sickle cell retinopathy or slow PSR progression by protecting the blood vessels in the retina from being blocked in the first place. However, the exact effect of sickle cell disease treatment on PSR isn’t fully understood.

If you or a loved one has been diagnosed with sickle cell retinopathy, your eye doctor can recommend treatment based on the stage of your PSR and any complications that occur because of it. Most eye doctors first recommend treatment for PSR during stage 3 or later.

Currently, there’s no treatment for NPSR.

Laser Treatments

Laser photocoagulation is the most widely used treatment for PSR. Your eye doctor might recommend laser treatments if you’ve developed sea fan blood vessels in stage 3 PSR. A laser can cause the sea fans to shrivel up, often preventing them from bleeding.

Injections

Your eye doctor might recommend injections to reduce the amount of vascular endothelial growth factor (VEGF) in your eyes. VEGF is a protein that makes abnormal new blood vessels grow when normal retinal blood vessels have been damaged.

By stopping abnormal blood vessel growth, anti-VEGF injections help prevent bleeding in the eye and the complications that can occur because of it.

Your eye doctor administers anti-VEGF injections in their office after numbing your eye with special eye drops. Most people start off getting them every four to six weeks, but your treatment plan will depend on your needs.

Surgery

Surgery for PSR usually isn’t recommended unless someone reaches later stages when complications leading to vision loss occur. Your eye doctor might recommend surgery if you have retinal detachment, a macular hole, or excessive bleeding in the eye that doesn’t improve with other treatments.

Eye doctors plan surgery for PSR very carefully and may recommend one of several techniques. Your eye doctor can work closely with you to decide on the type of surgery to use. They may recommend a blood transfusion to increase hemoglobin in your blood before surgery for PSR.

Prioritizing Your Eye Care

While there’s currently no known way to prevent sickle cell retinopathy, routine eye care can help you monitor your eyes for changes and start treatment when the time is right.

Living with sickle cell disease already involves a lot of steps in caring for your health, which can become overwhelming or difficult to track. Try these tips to help make eye care a priority:

• Book annual comprehensive eye exam appointments with a trusted eye doctor starting in childhood (and mark your calendar so you won’t forget).
• Tell your eye doctor about your sickle cell disease diagnosis.
• Report any changes in your eyesight to your eye doctor as soon as you notice them.
• Stay on track with your overall sickle cell disease treatment plan.
• Keep up with preventive healthcare, including annual physical exams.
• Coordinate care between your eye doctor and other specialists, like your primary care provider and hematologist.

Ask your eye doctor about any other steps you can take to protect your eye health.