Retinitis Pigmentosa (RP) consists of several hereditary diseases that cause deterioration of the retina, a thin layer of tissue lining the inside of the back of the eye. RP attacks the photoreceptor cells in the retina which are responsible for capturing and processing light. As these cells deteriorate, vision loss progresses.

There are two types of photoreceptor cells -- rod and cone-shaped. Rod-shaped cells are responsible for peripheral and night vision while cone-shaped cells are responsible for central vision and colors. RP typically assaults the rod-shaped cells robbing patients of their side vision and eventually leaving only tunnel vision.

Since night and peripheral vision are affected, a common symptom of RP is night blindness and the tendency to bump into or trip over objects. As the person’s field of vision continues to narrow towards a tunnel shape there can also be a decreased response to magnification and the constant need for more light.

Due to the hereditary nature of RP, it is imperative to remember the disease can affect another member of the family. If one family member is diagnosed with a hereditary retinal degeneration, it is important for all members of the family to contact an eye care physician. Since RP is rare (1 in 4,000 incidence) and difficult to accurately diagnose, it’s important for symptomatic patients to see an ophthalmologist who specializes in retinal degenerative diseases.

Unfortunately, there is no cure for RP. However, early diagnosis is still important because your eye care physician can help with proper planning for the disease and its progressive developments. Other options include vision rehabilitation and vocational instruction.

Typical Progress of Someone with RP

Symptoms of RP are often recognized in children, adolescents and young adults. The disease then progresses throughout the individual’s life. Following is a typical advancement of rod-cone RP but keep in mind pattern and degree of vision loss vary.

1. Night vision begins to suffer, progressing to night blindness
   • Most forms of RP first attack the rod cells which are responsible for night and peripheral vision
   • Night blindness is similar to what sighted individuals encounter when entering a dark room after being outside in the bright sunlight.
2. Peripheral vision begins to fail
   • As the disease progresses, more rod cells degenerate
   • A ring of vision loss in the mid-periphery is often experienced
   • Small islands of vision in the extreme periphery may remain
3. Tunnel vision develops
   • People with RP usually keep a small degree of central vision their entire life.

Other forms of RP, usually referred to as cone-rod dystrophy, attack the central vision first. Since the cone cells are responsible for central vision and color perception, uncorrectable central vision loss occurs as well as disturbances in color perception.

If significant vision loss has occurred as a result of Retinitis Pigmentosa, vision rehabilitation may be helpful. The goal is to enhance remaining sight so that an individual can continue to do routine tasks and reach meaningful personal goals, e.g., reading, sewing, television viewing.

The process of vision rehabilitation involves an assessment of functional vision and training in adaptive visual skills with special vision devices. Additional services are available to help adjust to vision loss and maintain independence at home and in the community.

For more information about retinitis pigmentosa or vision rehabilitation speak with your eye care physician, CSBPS’s professional staff, The National Society to Prevent Blindness (211 Wacker St, Chicago, IL 60606, (800) 331-2020)), or the Lighthouse National Center for Vision and Aging (The Lighthouse Inc., 111 E 59th Street, New York, NY 10022, 800-334-5497).