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Retinopathy and Sickle Cell Disease

What causes retinopathy with sickle cell disease? 

Sickle cell disease causes red blood cells to become sickled (banana-shaped). They are also sticky and rigid.  These sickle cells can block blood and oxygen flow to all areas of the body.  

Sickle cells can block the small blood vessels in the eye depriving the eye of oxygen and causing damage. This is called sickle retinopathy. 

Any person with sickle cell disease can develop sickle retinopathy. But it is more common in people with sickle-hemoglobin C disease (SC) and sickle beta plus (Sβ+) thalassemia. Up to 1/3 of children with SC and Sβ+ thalassemia disease may develop sickle retinopathy. 

Sickle retinopathy and eye damage 

If your child has sickle cell disease, they should begin having eye exams to check for sickle retinopathy at age 9 or 10.  

  • Children with sickle hemoglobin C disease will need an exam every year. 
  • Children with other types of sickle cell disease will need an exam every other year. 

Retinopathy can happen in 1 or both eyes. Early damage does not usually impact your child’s vision. 

Retinopathy can go undetected for years without an eye exam by a trained eye doctor. That is why it is important for your child to have regular eye exams with an eye doctor who is familiar with sickle cell disease.  

If your child’s eye doctor notices signs of sickle retinopathy, then they may need to be seen more often for exams. It is very important for your child to have these exams on schedule. 

Sickle retinopathy can progress to severe proliferative sickle cell retinopathy (PSR). PSR can cause bleeding into the eye or detachment of the retina. It can lead to vision changes and, in rare cases, blindness. 

Managing sickle retinopathy 

You can help your child manage sickle retinopathy with regular eye exams. Your child’s eye doctor can monitor both eyes. They can help manage and document if sickle retinopathy progresses. 

If your child’s sickle retinopathy does progress, treatment is available to help prevent vision loss. Blindness is rare in patients who regularly see an eye doctor. If left untreated, 10 percent of people with SR may develop vision problems. 

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

Reviewed: September 2022