Eye Problems and Sickle Cell Disease

An eye problem called sickle cell retinopathy (SCR) is a common complication of sickle cell disease (SCD). It happens when blood flow to the retina is blocked by sickle cells. SCR can be asymptomatic for a while before complications begin to affect vision.

People with SCD should be examined regularly for signs of SCR to avoid permanent vision loss. Hydroxyurea and blood transfusion therapy may prevent SCR. Laser treatment is the most common therapy.

What eye problems do people with sickle cell disease experience?

People with SCD can experience vision loss, usually due to sickle cell retinopathy. About 33 to 40 percent of people with sickle hemoglobin C (HbSC) develop SCR. About 15 to 20 percent of people with sickle cell anemia (HbSS) develop SCR. If untreated, SCR can lead to vision loss.1

SCR can be classified as either proliferative (PSCR) or non-proliferative (NPSCR). PSCR is less common than non-proliferative sickle cell retinopathy, but PSCR is more frequent among people with HbSC than HbSS.2

Proliferative sickle cell retinopathy is more severe than non-proliferative sickle cell retinopathy. It begins during childhood but remains asymptomatic until “vitreous hemorrhage” or “retinal detachment” occurs. Vitreous hemorrhage is the leakage of blood into the area between the retina and the lens of the eye. Retinal detachment is the separation of the retina from the layer underneath. Both complications of PSCR lead to vision loss.3

SCR can be diagnosed by a doctor with expertise in eye conditions (ophthalmologist). They can perform tests such as ophthalmoscopy and angiography to see inside the blood vessels of the eye.2

What causes eye problems?

People with SCD experience eye problems like SCR when sickle cells block blood vessels in the eye. Blockage can occur in any part of the eye, but the retina is most commonly affected. The retina is the light-sensitive part of the eye that sends visual messages to the brain.4

Blocked blood flow to the retina leads to inflammation and low oxygen levels. This affects the retina and causes complications like SCR. Some risk factors increase the chance of developing eye problems, such as:2

  • Older age (above 35 years old)
  • Lung disease
  • Deafness or tinnitus
  • Male gender
  • High hemoglobin levels
  • Low white blood cell count
  • Other genetic and environmental factors

Symptoms

Sickle cell retinopathy can lead to many different symptoms, even among people with the same type of SCD. Symptoms usually occur because of retinal detachment or vitreous hemorrhage. Some common symptoms and signs of SCR include:5

  • Flashes
  • Floaters
  • Dark shadows
  • Sudden vision loss

These are not all the symptoms of SCR, and other eye complications can cause these symptoms. If you experience any sudden changes in your vision, talk to your doctor. They can refer you to an eye specialist to perform tests to diagnose the complication.

What are treatment and prevention options?

People with all types of SCD should be screened regularly for vision changes and other signs of sickle cell retinopathy. Screening helps prevent serious complications of SCR. Hydroxyurea treatment or chronic blood transfusions may also prevent SCR.2,6,7

Treatment options include:2,5

  • Anti-vascular endothelial growth factor (anti-VEGF) injections into the eye
  • Laser treatment
  • Surgical procedures to address retinal detachment or vitreous hemorrhage

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Written by: Matthew Zajac | Last reviewed: January 2021