Retinopathy in Children with Sickle Cell Disease

What is sickle cell retinopathy?

Retinopathy is damage to the blood vessels in the retina of the eye, located in the back part of the eye. Retinopathy occurs in 33 percent of people with Hb SC disease and in 3 percent of people with Hb SS disease. It occurs in older children and adults.

What causes retinopathy?

Sickled red blood cells become trapped in the small vessels of the eye, resulting in decreased blood flow to that area of the eye. This may cause loss of vision. This damage may be permanent and blindness can occur in later stages. 

What are the symptoms of retinopathy?

In the early stages of retinopathy, there may be no symptoms.  For this reason, it is important to have your child’s eyes checked once a year by an eye doctor.  The eye exam includes dilation of the pupil in order to see if the vessels appear normal or if changes or scarring is occurring.

In later stages, your child may complain of:

• Seeing dark spots or shadows in his field of vision
• Blurry vision
• Sudden loss of vision
• Pain in the eyes

How is retinopathy treated?

Early treatment by an eye doctor may include more frequent eye check ups to monitor the progression, laser treatment or surgery.

What should I do to prevent retinopathy?

When your child turns 10 years old, her or she should have an eye exam by an ophthalmologist once a year.  Ask your hematology provider what is needed to schedule an appointment.