Retinitis pigmentosa (RP) was first described as a condition in 1853, but the name wasn’t used until 1857. Although "retinitis" suggests inflammation, it's actually a misnomer since inflammation plays a minimal role in the disease. This glossary will explain what retinitis pigmentosa is and address concerns about it.

What Is Retinitis Pigmentosa?

Retinitis pigmentosa (RP) refers to inherited eye disorders that affect the retina (the layer of cells at the back of the eye that captures light and transmits signals to the brain, allowing us to see). It’s a condition that gradually causes retinal deterioration, leading to slow vision loss.

People with RP usually start noticing symptoms in childhood or early adulthood, with their night vision and peripheral vision being the first to go.

RP affects approximately 1 in 3,000 to 1 in 4,000 people worldwide, which adds up to around two million people. An estimated 100,000 people have the condition in the US.

Causes

What causes retinitis pigmentosa? The condition is primarily genetic, passed down from parents to their children. Mutations in the genes that help keep cells in the retina healthy can cause it to break down over time. Since it's inherited, your family history significantly influences your chances of developing RP. There’s no single cause, but understanding your genetic background can provide insight into your risk.

Symptoms

Retinitis pigmentosa often begins with these common symptoms:

  • Night Blindness: Trouble seeing in low light, like at dusk, in dimly lit rooms, or in inclement weather
  • Blind Spots: Losing the ability to see in parts of your peripheral vision

As the condition progresses, you might also notice:

  • Tunnel Vision: A gradual narrowing of your field of vision
  • Photophobia: Developing abnormal sensitivity to bright light
  • Color Blindness: Losing the ability to detect or differentiate colors

Eventually, RP can impact central vision, making everyday tasks like reading or recognizing faces more difficult.

Diagnosis

Diagnosing retinitis pigmentosa usually involves a series of eye exams. Your eye doctor may use visual field tests to assess peripheral vision and an electroretinogram (ERG) to measure how well the retina responds to light.

Your doctor might also suggest genetic testing to pinpoint the exact mutations behind your RP, offering valuable insights into how the condition could progress.

Treatment Options

Currently, there’s no cure for retinitis pigmentosa, but there are ways to manage the condition and slow its progression. Wearing sunglasses to protect your eyes from UV light, taking certain vitamins or supplements as recommended by your doctor, and using low-vision aids can all help maintain your quality of life. New treatments like gene therapy are being explored as potential options to restore or preserve vision in some cases.

Frequently Asked Questions

Maintaining good vision is essential to enjoying a good quality of life, as it impacts daily activities, independence, and overall well-being. If you or a loved one is dealing with retinitis pigmentosa, stay informed and consult with your Visionworks eye care professional regularly.