The retina is the light sensitive part of your eye. It contains millions of visual cells responsible for transmitting visual stimuli to the brain. Retinitis Pigmentosa (RP) is a form of retinal degeneration that is associated with loss of the visual cells and loss of the normal function of the retina. Overtime, the retina becomes irreversibly damaged with severe loss of vision.
RP can start at any age although it typically starts during childhood or teenage. The disease is characterized by poor visual function especially at night. Patients may describe decreased night vision, bumping into things and tripping over things at night. They may also suffer from decreased peripheral vision and glare. Although the disease generally runs in families, many patients do not have a family history of this condition.

This disease is rare. Therefore, general ophthalmologists are usually not experienced enough to diagnose and manage patients with the disease. The disease may also be associated with life threatening systemic conditions such as heart and kidney problems. It is recommended that a retina specialist and not a general ophthalmologist manage patients with RP. Not every ophthalmologist is a retina specialist that is experienced in managing this condition.

The first step to achieve an accurate diagnosis is through a comprehensive eye exam. The findings in RP maybe typical of the disease that no further testing is required for diagnosis. Sometimes your retina specialist will order additional tests or procedures to confirm your diagnosis. Some of the tests are not usually done for other more common retinal problems. For example, color vision maybe tested and this involves using special tests to identify colors.

Visual field testing may also be obtained to assess the magnitude if peripheral vision loss in this disease.

An optical coherence tomography (OCT) maybe obtained to assess the magnitude of retinal atrophy caused by the disease. The OCT allows us to obtain a cross sectional views of the macula with three-dimensional images. It is can be helpful for measuring the amount of loss of visual cells. Fluorescein angiography may also be performed. This test shows changes in the structure or function of retinal blood vessels. In this procedure, a dye is injected into a vein in your arm, and then the dye circulates through your body. As it transits through your eyes, photographs of your retina are taken as the dye outlines your retinal blood vessels.

Another test that is helpful in the diagnosis as well as monitoring progression of the disease and treatment outcomes is the electroretinogram (ERG). This test measures the electrical activity of the visual cells of the retina. If the visual cells are absent or malfunctioning because of the disease, the results of the test will be abnormal. This may get worse as the disease progresses or better if treatment if successful.

Treatment options:
Unfortunately, to date there is no effective treatment to stop retinitis pigmentosa or cure it. Several potential treatments are currently under investigation worldwide. These involve attempts at replacing the visual cells with stem cells or at replacing malfunctioning genes. In the meantime, many people benefit from low vision aids and therapy provided by our eye care team.
Some patients with advanced RP and severe or total loss of vision may benefit from a retinal prosthesis called the Argus II Retinal Prosthesis. This surgically-implanted device delivers electrical stimulation to the retina to restore visual perception. Although this may improve visual perception in some patients, the level to which the visual function improves varies among patients, but is generally minimal. Rarely, the improvement maybe significant.