1. What are Retinal Degenerative Diseases?
  2. What is Retinitis Pigmentosa?
  3. What causes Retinitis Pigmentosa?
  4. What are the symptoms of Retinitis Pigmentosa?
  5. Can my Ophthalmologist Diagnose Retinitis Pigmentosa?
  6. Are There Special Tests to Diagnose Retinitis Pigmentosa?
  7. How do I Find a Doctor Familiar with Retinal Degenerations?
  8. How Quickly Does Retinitis Pigmentosa Progress?
  9. Can RP Lead to Total Blindness?
  10. Is there a Treatment for Retinitis Pigmentosa?
  11. What Are Some of the Questions I Should Ask My Eye Doctor?

What are Retinal Degenerative Diseases?

Retinal degenerative diseases are a family of disorders that cause degeneration of the retina.

What is Retinitis Pigmentosa?

Retinitis pigmentosa (RP) is the name given to a group of inherited eye diseases that affect the retina. Retinitis pigmentosa causes the degeneration of photoreceptor cells in the retina. Photoreceptor cells capture and process light helping us to see. As these cells degenerate and die, patients experience progressive vision loss.

There are types of photoreceptor cells: rod cells and cone cells. Rod cells are concentrated along the outer perimeter of the retina. Rod cells help us to see images that come into our peripheral or side vision. They also help us to see in dark and dimly lit environments. Cone cells are concentrated in the macula, the center of the retina, and allow us to see fine visual detail in the center of our vision. Cone cells also allow us to perceive color. Together, rods and cones are the cells responsible for converting light into electrical impulses that are transmitted to the brain where "seeing" actually occurs.

What causes Retinitis Pigmentosa?

Retinitis Pigmentosa is a genetic disorder. It is programmed into your cells, not caused by injury, infection or any other external agent. Genetic defects (mutations) send faulty messages to the retinal cells which leads to their progressive degeneration, which in turn, leads to vision loss.

Because Retinitis Pigmentosa is an inherited disorder, it is strongly recommended that if someone in your family is diagnosed with a retinal degenerative disease that all members of the family contact a retinal specialist.

What are the symptoms of Retinitis Pigmentosa?

  • Loss of or diminished night vision. One of the earliest & most frequent
  • Loss of or diminished peripheral vision

Can my Ophthalmologist Diagnose Retinitis Pigmentosa?

Possibly, however, if you experience either of the symptoms noted above and your ophthalmologist isn't certain, you should consult with a retinal specialist. The Foundation maintains a list of doctors who specialize in retinal degenerative diseases.

Are There Special Tests to Diagnose Retinitis Pigmentosa?

Yes.

Acuity Tests: This test measures the accuracy of your central vision at specific distances in specific lighting situations.

Color Testing: This can help determine the status of your cone cells, the retinal cells that interpret color.

Visual Field Test: This test uses a machine to measure how much peripheral vision you have.

Dark Adaptation Test: This test will measure how well your eyes adjust to changes in lighting and can help the doctor better understand the current function of your rod cells, which are the retinal cells responsible for night vision.

ERG Test: The ERG (electrophysiological test) records the electrical currents produced by the retina due to a light stimulus. The intensity and speed of the electrical signal becomes reduced as the photoreceptor cells degenerate.

How do I Find a Doctor Familiar with Retinal Degenerations?

The FFB maintains a list of retinal specialists. All of the eye care professionals on this referral list have indicated their interest in treating people with inherited retinal degenerative diseases. For a copy of the list for your home state, please contact The Foundation at 1-800-683-5555.

How Quickly Does Retinitis Pigmentosa Progress?

It's different for every patient.

Can RP Lead to Total Blindness?

A majority of people with RP are legally blind by the age of 40. Legally blind individuals are those whose best visual sharpness or acuity (with glasses or contact lenses, if needed) is 20/200 or worse in their better eye; or whose visual field, regardless of acuity, is restricted to a 20 degree diameter (10 degree radius).

Is there a Treatment for Retinitis Pigmentosa?

A daily dose of 15,000 I.U. of Vitamin A can moderately slow the progression of RP. There are also several areas of research that offer tremendous promise for halting the disease or restoring vision loss:

  • Retinal Cell Transplantation
  • Gene Therapy
  • Pharmaceutical Therapy
  • Nutritional Therapy

For additional information on these or any other areas of our research, please scroll back to the Home Page or to the Master RP Page.

What Are Some of the Questions I Should Ask My Eye Doctor?

  1. What changes can I expect in my vision?
  2. Will my vision loss get worse? How much of my vision will I lose?
  3. Will regular eyeglasses improve my vision?
  4. What medical/surgical treatments are available for my condition?
  5. What can I do to protect or prolong my vision?
  6. Will diet, exercise, or other lifestyle changes help?
  7. If my vision can't be corrected, can you refer me to a specialist in low vision?
  8. Where can I get a low vision examination and evaluation? Where can I get vision rehabilitation?
  9. What is the name of my eye condition?
  10. What is my visual acuity (Central vision)?
  11. What is my visual field (Side vision)?
  12. Are there other tests?
  13. What about a second opinion?
  14. Are there any optical or non-optical aids that can help me use my remaining vision to the fullest extent possible?
  15. Are there any mobility aids to help me move around more safely?
  16. Do any of your other patients have this condition? Would they be willing to talk with my family and me?
  17. Do you have any educational materials that describe this condition?
  18. Are there any organizations that provide services to individuals with this or related eye conditions?

Input from the National Eye Institute