-
- The Editor:
Dr. Paul Finger - The Editor:
| Patient Information: Retinal
Diseases Contacts: |
|
Retinitis Pigmentosa - RP The term Retinitis Pigmentosa represents a group of retinal diseases characterized by progressive degeneration of the peripheral retina. The term "retin" "itis" is descriptive. It means there are signs of retinal "itis" or inflammation. Similarly, the term "pigmentosa" relates to the spicular pigmentation which forms within affected retina. Though there is inflammation associated with RP, it is not thought to be contagious. RP typically affects the peripheral retina first resulting in loss of peripheral vision. To get an idea what this is like for an RP patient, you might try looking through the hole in a donut. You will see that RP causes tunnel vision. This means that while an RP patient's central vision can be excellent while their ability to simultaneously look to the sides, up or down is markedly impaired. Sometimes RP is associated with hearing loss and this is called Usher's Syndrome. Other times RP can be associated with swelling of the macular retina (called cystoid macular edema) with resultant loss of central vision. There is no typical age of onset for RP or degree of severity. Therefore it is impossible to predict the outcome for any individual patient. Treatment: Unfortunately, there is no currently available treatment to halt the progression of most retinal dystrophies. Many unproven treatments continue to be attempted and research scientists continue to investigate many potential treatments. FAQ's: Q: Does light harm an RP eye? A: No, but long exposures to bright light are avoided to protect these eyes. Patients can work in dim light as much as they like. ------------------------------------------------------------------ Q: If my child has RP should I get an eye examination? A: Yes, it is possible that another member of your family will be found to be affected by RP. You should consult your doctor about genetic testing and professional genetic counseling. ------------------------------------------------------------------ Q: Can I get my cataract removed if I have RP? A: Cataract surgery can be performed on an eye with RP. The results of this surgery depend on the extent of pre-existing retinal degeneration and the eye's reaction to surgery. The Genetics of Retinitis Pigmentosa Autosomal Recessive Inheritance: This is the most common form of RP where both parents are asymptomatic carriers of the gene (not affected by the disease). In this case, 25% of their children will be affected (no matter which sex). Autosomal Dominant Inheritance: In this type of inheritance, one parent has RP and each child has a 50% chance of getting the disease. X-linked Recessive Inheritance: X-linked is the least common form of RP. This form must be carried by the mother who passes the RP gene to her son. There is a 50% chance that the each son will develop RP and a 50% chance that each daughter will be a carrier.
Please
report any problems to: While this WWW site is intended to provide general information as a public service, the content is not intended to represent official policies and practices of the institution or to substitute for health care provided by a clinician. This
Web site and bulletin board has been provided by as a public service.
If you feel that you have benefited from this Web site, Content
and design copyright © Paul
T. Finger, MD 1999-2003 pfinger@macular-degeneration.org ALL RIGHTS RESERVED
|