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Sickle Cell Retinopathy

What is sickle cell retinopathy? 

Sickle cell retinopathy is an eye problem that can happen in people with sickle cell disease. Retinopathy is damage to the retina, the part of the eye that senses light. Retinopathy can affect 1 or both eyes.  

In the early stages, sickle cell retinopathy usually does not cause vision problems. But over time, it can get worse and cause vision problems if not treated.  

Retinopathy is common in people with sickle cell disease. The best ways to help protect vision are to manage sickle cell disease, keep regular medical appointments, and get regular eye exams.  

Eye exams can find signs of sickle cell retinopathy early, before symptoms develop and when treatment is most effective. 

Eye anatomy showing cornea, iris, pupil, lens, sclera, choroid, retina, central retinal artery, and central retinal vein

Retinopathy is damage to the retina, a thin layer of tissue in the back of the eye.

Proliferative sickle cell retinopathy 

Proliferative sickle cell retinopathy (PSR) is the most serious type of sickle cell retinopathy. It happens when new, abnormal blood vessels grow in the retina. These blood vessels are weak and can cause serious eye problems, including: 

  • Bleeding in the eye 
  • Retinal detachment, when the retina separates from its normal position 
  • Vision problems 
  • Blindness (in rare cases)  

Symptoms of sickle cell retinopathy

Sickle cell retinopathy often does not cause symptoms at first. Signs and symptoms may include:

  • Seeing spots, floaters, or shadows
  • Blurred vision
  • Trouble seeing colors
  • Vision loss

Causes of sickle cell retinopathy

In sickle cell disease, red blood cells become hard, sticky, and shaped like a crescent or banana (sickle-shaped).  

Sickle cells clump together and clog blood vessels. Less blood and oxygen go to the body’s tissues and organs. Reduced blood flow and oxygen to the retina can damage it over time. 

Anyone with sickle cell disease can develop sickle cell retinopathy. But it is more common in people with sickle hemoglobin C (HbSC) disease, hemoglobin SS (HbSS) disease, and sickle beta zero (HbS beta0) thalassemia. These conditions also increase the risk of the most serious type of retinopathy, proliferative sickle cell retinopathy.  

Diagnosis of sickle cell retinopathy

People with sickle cell disease should have regular eye exams with an ophthalmologist (a medical doctor who specializes in eye health) experienced in caring for people with sickle cell disease.  

Most children with sickle cell disease need eye exams every 1-2 years starting around age 10. 

The schedule depends on the type of sickle cell disease and the findings on previous eye exams. Your child's care team will recommend the right schedule. In general: 

  • Children with sickle hemoglobin C (HbSC) disease need an eye exam every year.  
  • Children with other types of sickle cell disease need an eye exam every other year.  

Ask your care team how often your child should have these exams.  

The doctor will do a dilated retinal exam. They will place special drops in the eyes to widen the pupil. This lets the doctor examine the retina, optic nerve, and blood vessels.  

The doctor may also order imaging tests such as: 

  • optical coherence tomography (OCT) 
  • OCT angiography 
  • fluorescein angiography 

If your child has signs of retinopathy, they may need more frequent exams. It is important to have these eye exams on schedule.  

Severe vision loss is uncommon when retinopathy is found early and treated. 

Treatment for sickle cell retinopathy

Managing sickle cell disease helps reduce the risk of retinopathy and other problems. Keep all medical appointments and follow your child’s recommended treatment plan. 

Treatments for more severe sickle cell retinopathy may include: 

  • Laser treatment: Helps control or stop the growth of abnormal blood vessels  
  • Medicines: Help slow or stop the growth of abnormal blood vessels  
  • Surgery: May be needed to treat certain eye problems, such as a detached retina or bleeding into the eye 

Speak with your child’s care team if you have questions or concerns. They are available to help you.  

Questions to ask your care team

  • What is my child’s risk for developing sickle cell retinopathy? 
  • How often should my child have eye exams? 
  • What symptoms or vision changes should we watch for? 
  • If you find signs of retinopathy, what happens next? 
  • What treatments are available for sickle cell retinopathy? 
  • How can we help manage sickle cell disease to lower the risk of eye problems? 
  • When should we call the clinic or seek emergency care for eye symptoms? 

Key points about sickle cell retinopathy

  • Sickle cell retinopathy is an eye problem that happens in some people with sickle cell disease. 
  • Most people do not have symptoms in the early stages, so regular eye exams are important. 
  • Eye exams can find retinopathy early, before it causes vision problems. 
  • Your child should have regular eye exams with an ophthalmologist as recommended by their care team. 
  • Managing sickle cell disease and keeping regular eye appointments are important ways to protect your child's vision. 

—
Reviewed: September 2026

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