Retinitis Pigmentosa

What Is Retinitis Pigmentosa?

Retinitis pigmentosa (RP) is a genetic condition that leads to vision loss over time. Typically, someone with RP first loses night vision, followed by a loss of peripheral vision over time.

Often, people with RP have other eye problems that affect vision, such as high refractive errors, cloudy lenses (cataracts), or swelling of the retina. There may be a family history of blindness.

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 in early adolescence 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.

Sometimes RP runs in a family or happens in people with some types of health syndromes. But people can get it without a family history or a health 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 and dark
optical coherence tomography (OCT) to examine the retina
fluorescein angiography (FA) to check blood flow in the retina
visual field testing to look for a loss of peripheral vision
genetic tests

How Is Retinitis Pigmentosa Treated?

Treatment to cure retinitis pigmentosa or bring back lost vision is limited. Genetic therapy can help with retinitis pigmentosa caused by a gene defect (RPE65). 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 refractive error, astigmatism, cataracts, or retinal 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. Also, a low-vision specialist can help optimize your child’s remaining vision and recommend tools to maintain function.

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