Presented by

Chino AnyanwuLynn BaudoinMona L. BellEmily Diaz

Wendy LewisKathy Sanders

1. Understanding Retinitis Pigmentosa2. Common Problems of Retinitis Pigmentosa3. The Effects of Retinitis Pigmentosa on the Visual

System4. Treatment of Retinitis Pigmentosa5. Medications used when treating Retinitis Pigmentosa6. Surgical Care when treating Retinitis Pigmentosa7. Functional Implications of Retinitis Pigmentosa8. Low Vision Aids for People with Retinitis Pigmentosa9. Americans with Disabilities Act

Retinitis Pigmentosa (RP) is also known as Rod/Cone Dystrophy.

RP affects 1 in 3,500 people in the United States. Worldwide, RP is thought to affect roughly 1 in 5,000 people.

RP is defined as a hereditary and progressive group of disorders that gradually leads to visual field loss and retinal degeneration.

The name “retinitis pigmentosa” refers to what the retina looks like in individuals diagnosed with RP.

When viewed with a specialized tool, the RP retina often shows clusters of pigment that are not seen in a normal retina.

The word “pigmentosa” refers to the pigment deposits seen during the eye exam.

The term “retinitis pigmentosa” refers to a group of eye conditions that affect the retina, or the layer of nerve cells at the back of the eye.

Tunnel Visual Field  - Progressive loss of visual fields is a characteristic of RP. The visual field loss often begins as a donut-like ring in mid-periphery. As it progresses both centrally and peripherally, the resultant tunnel vision begins to affect the patient’s activities, driving and mobility. In severe tunnel vision cases, the patient may report seeing “two separate world.”

Night Blindness - Another characteristic of RP is night blindness. It may be the first presenting sign.

Sensitivity to Light & Glare- When RP patients go into bright sunlight, they are often overwhelmed by “white-out” debilitating glare. They are aided by the use of dark plum and amber filters used often with side shields. RP patient also frequently report glare problems inside. Many find a light amber filter in their general wear eyeglasses improves their tolerance. A variety of filters are available. 

Double Vision - Tunnel vision interferes with the brain’s ability to accurately control alignment of the eyes. To treat the resultant double vision, prisms and orthoptic therapy may be used.

Cataracts - RP patients frequently develop a form of

cataract. It may reduce visual acuity and increase glare.

Decreased Visual Acuity -  A portion of RP patients, will experience a loss of central vision. The macular area may deteriorate making it difficult for the patient to see detail as in seeing a faces or reading. Simple magnification and increased task lighting may be helpful.  

A person with RP may experience:a reduction in depth perceptiondifficulty with discerning fine detailsdifficulty entering or leaving different environments due to slow adjustment to light changesa reduction in peripheral vision

Lighting will be a major factor in visual functioning.

Low vision aids may be necessary as vision reduces.

Normal VisionNormal Vision Tunnel Tunnel VisionVision

Presently, there is no therapy available for the vast majority of individuals with RP.

Attention is directed towards low vision aids, and proper social and governmental programs for the visually-impaired.

Because much remains unknown about RP conditions, research is very active in this area.

While therapies are limited, physicians should emphasize the therapies that are available and as new therapies emerge, routine evaluation can keep patients informed of clinical trials and new treatments.

Some research indicates that daily use of microcurrent stimulation may help preserve vision.

Medication Medical Benefit

Acetazolamide Useful in reducing macular edema, which can reduce vision in the later stages of RP

Corticosteroids May be useful for macular edema but has not been well studied

Lutein and Zeaxanthin Useful in protecting the macula from oxidative damage

Vitamins A&E, Omega-3 Fatty Acids, other antioxidants, and COQ10

May help slow down RP and preserve or even improve vision

Cataract extraction can often be

beneficial in the later stages of RP.

Most cases of retinitis pigmentosa are inherited. However, some people develop the disease even if they have no family history. Others may develop the condition as part of another disorder.

Signs of RP can usually be detected during a routine eye exam when the patient is around 10 years old. However, symptoms usually do not develop until adolescence.

Although the disease worsens over time, most patients retain at least partial vision, and complete blindness is rare.

It is perfectly natural for someone with vision loss to feel anxious, fearful, angry, or unhappy. It is also natural for them to have feelings of uncertainty, anxiety, and fear over what the future may hold.

Most adults describe living a full and interesting life despite the practical and social problems that can arise because of RP.

Although many individuals with RP are diagnosed in childhood, some may not be diagnosed until they are adults.

Others might not have a need for low vision adaptation until they are adults with established careers. When this happens, they may feel as though they can no longer perform their job functions. However, individuals with RP can and do have a wide variety of jobs, from computer programmer to musician, and often find it unnecessary to change careers because of RP.

Some people may benefit from using special lighting to complete their daily tasks. There are also devices and programs that make it easier to use computers.

Some computer-based programs can act as magnifiers, or even convert text to audio formats.

Patients with very late-stage disease are often helped by learning how to use a cane to find objects or curbs in their path when walking. They may also find a guide

dog helpful. Some patients ask if it is necessary to learn Braille. Only a small number of patients in very

late stages of RP need Braille for reading.

The Americans with Disabilities Act gives civil rights protections to individuals with disabilities, such as low vision due to RP.

Its policies prevent employment discrimination and allow equal access to education, healthcare, and public transportation.

For more information, you can call 800-514-

0301 or visit www.ada.gov.

Resources: Texas School For the Blind: tsbvi.eduAmerican printing House for the blind: aph.orgThe Nova Clinic of Houston: opt.uh.edu/uei/services/pediatrics.com

References:Wikipedia. (2010, April 3).Cone Dystrophy. Retrieved July 5, 2010, from http://en.wikipedia.org/wiki.conedystrophyEye Conditions and Other Retinal Diseases. (2004-2010). Cone-Rod Dysrophy. Retrieved July 5, 2010, from http://www.blindness.org/index.php?view=article&catid=38%3Aother-retnal-diseases&id=...Selected Anomalies and Diseases of the Eye. (1990). Cone-Rod Dystrophy. Retrieved July 5, 2010, from http://www.tsbvi.edu/education/anomalies/nystagmus. htmUniversity of Edinburg. (n.d.). SSC Scottish Sensory CentreRod-Cone Dystrophy. Retrieved July 5, 2010, from http://www.ssc.education.ed.ac.uk/resources/vi&multi/eyeconds/RcDyst/htmlAmerican Academy of Ophthalmology: Berkley. (2006). High Resolution Retinal Imaging of Cone –Rod Dystophy. Retrieved July 5, 2010, from http://www.berkely.edu/imagingofroordalab/pubs/wolfing.pdf

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