What is Retinitis Pigmentosa

Retinitis Pigmentosa is a group of eye problems that affect the retina, a thin tissue that lines the inner surface of the back of the eye. This condition changes how the retina responds to light, making it difficult to see, especially at night. While people with RP typically lose their vision gradually over time, most will not become totally blind.

What Causes Retinitis Pigmentosa?

RP is a genetic condition that is passed down in families. It is considered a rare disorder, affecting roughly 1 in 4,000 people worldwide. There are more than 50 genes that can be affected, and harmful changes in any one of them can lead to RP.

Some gene mutations are so severe, the gene is unable to make the required protein. Other changes produce a toxic protein. And others cause an abnormal protein that doesn’t function properly. Either way, the photoreceptors (special cells in the eye’s retina responsible for converting light into signals that travel to the brain) are damaged.

What are the Symptoms of RP?

The symptoms of Retinitis Pigmentosa typically appear in childhood. Children may have a difficult time seeing in the dark, or it may take them a long time to adjust to changes in lighting. Over time, children may appear to be clumsy because they trip over things or miss things in their field of vision. However, this is happening because the visual field is becoming more restricted.

Like other inherited conditions, the progression of RP is different for everyone. It all depends on the type of gene mutations involved in the disorder. Some people retain central vision and a restricted visual field for many years, while others experience significant vision loss by early adulthood. Most people with RP will eventually lose their sight, though not go totally blind.

The symptoms of RP include:

  • Loss of night vision
  • Gradual loss of peripheral (side) vision
  • Loss of central vision
  • Problems with color vision

How is Retinitis Pigmentosa Diagnosed?

A retina specialist can diagnose and measure the severity of RP using the following tests:

  • Genetic testing. This testing looks at your blood and tissues to see if you have certain genes.
  • Electroretinography. This test measures how well the retina responds to light.
  • Visual field testing. Because RP can affect your side vision, this test measures peripheral vision and looks for blind spots.
  • Optical coherence tomography. Known as OCT, this test takes highly detailed pictures of the retina.

How is RP Treated in Children and Adults?

With so many genes responsible for causing RP, there is no single treatment. Some research shows that Vitamin A may slow the progression, but it’s difficult to know how each patient will respond. An artificial vision device called the Argus II has also shown promise in restoring vision for people with late-stage RP.

However, for most people with RP, treatment is focused on helping them live effectively with vision loss. Children and adults may benefit from low vision aids, portable lighting devices, eye scanning techniques and UV-blocking sunglasses.

If you have been diagnosed with Retinitis Pigmentosa, it’s important to have a retina specialist that you trust. Empire Retina Consultants in New York specializes in retinal diseases like RP. Contact us today to schedule an appointment and discuss your options for treatment.