What Is Retinitis Pigmentosa?

Retinitis pigmentosa (RP) is a genetic condition that leads to vision loss over time. A person first loses their night vision, then their daytime vision.

Often, people with RP have other eye problems that affect vision, such as cloudy lenses (cataracts) or swelling of the retina.

What Are the Signs & Symptoms of Retinitis Pigmentosa?

In most types of retinitis pigmentosa (ret-in-EYE-tis pig-men-TOE-suh), the loss of night vision begins around age 10 and continues into adulthood. Sometimes it can begin earlier or later.

Vision loss happens slowly. When it starts, a child may:

• see shimmers, blinking lights, or flashes
• be slow to adjust to darkness
• have trouble seeing things to the far left and right

These problems usually show up when the child is doing normal activities. For example, a child may start to:

• have trouble seeing the ball or other players when playing sports
• find it hard to move around in low-light places, such as movie theaters
• bump into things

What Happens in Retinitis Pigmentosa?

The retina is a thin layer of tissue at the back of the eye. It receives light and changes it into signals that travel to the brain, letting us see images. The retina is made of light-sensitive cells called rod cells and cone cells.

In retinitis pigmentosa, over time the rod cells, which help us see at night, die off. Then, the cone cells, which help us see colors in daylight, die off.

Who Gets Retinitis Pigmentosa?

Sometimes RP runs in a family or happens in people with some types of health syndromes. Other people get it without a family history or a syndrome.

How Is Retinitis Pigmentosa Diagnosed?

If the condition runs in a family, genetic testing can tell whether a child is likely to get RP before symptoms start.

When a child shows symptoms of RP, an eye specialist will:

• do an eye exam
• check to see if another condition (such as an infection) is causing the symptoms

The eye specialist may order tests, including:

• electroretinography (ERG) to check the eye's response to light
• optical coherence tomography (OCT) to examine the retina
• genetic tests

How Is Retinitis Pigmentosa Treated?

There's no treatment to cure retinitis pigmentosa or bring back lost vision. In some cases, diet changes and vitamins can slow the loss of vision a little. Doctors may recommend that kids with RP wear sunglasses to avoid bright sunlight when outdoors.

If kids with RP have other eye problems that can make their vision worse (such as cataracts or swelling), those can be treated.

How Can Parents Help?

If your child has retinitis pigmentosa, talk with a genetic counselor to find out:

• which symptoms your child is likely to have
• how the RP gene runs in families
• if other children in your family are likely to have RP
• which treatments work best for your child
• if a clinical trial might be right for your child

Follow the eye doctor's instructions for any diet changes, vitamins, or protection from bright light.

You also can find more information and support online at:

• The Foundation Fighting Blindness

Reviewed by: Jonathan H. Salvin, MD
Date Reviewed: 11-11-2019