Retinitis Pigmentosa (RP) is the name given to a group of hereditary eye diseases in the course of which the retina is destroyed. Gradually, the retinal cells die: in all cases, the rods, which are located in the periphery of the retina, die first. The cones in the center of the macula initially remain intact.
Distribution of the RP gene
About 30,000 to 40,000 people in Germany suffer from this disease, and about 3 million worldwide. It is estimated that one in eight carries an “unfavorably” altered RP gene.
So such a gene carries genetic information that can initiate the development of this retinal disease in gene carriers or their offspring.
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Retinitis pigmentosa: symptoms and consequences.
The first consequence of RP, which usually occurs in adolescence or young adulthood, is usually night blindness. Gradually, color and contrast vision are lost, and later visual acuity decreases and the visual field narrows until, after a few years, only a small central visual nerve remains. Hence the term “tunnel vision” or “tube visual field”.
At this stage, the RP patient sees, for example, the glass in front of him on the table, but no longer the bottle standing right next to it. Despite still maintaining central visual acuity, orientation in unfamiliar rooms or on the street is now no longer possible.
Many people who suffer from this disease go completely blind in the course of their lives. Retinitis pigmentosa is the most common cause of vision loss in middle age.
Diagnosis of retinitis pigmentosa
Retinitis pigmentosa is a hereditary disease caused by an altered gene. It is not possible to prevent it. The main method for early detection of this disease is electroretinography (ERG).
This tests the visual field, visual acuity, color sense, and dark adaptation, as well as measuring the retinal current curve and mirroring the back of the eye.
RP: measures and therapy
To date, there is no way surgically, medicinally, or through diet to slow or halt the process of photoreceptor cell death. Great hopes are being placed in molecular genetics to detect the changes in the genetic material that cause such diseases and thus find a possible therapeutic approach.
For many years, various research projects have been underway with the aim of helping people blinded by degenerative retinal diseases to see again. In the meantime, various retinal chips have been developed with which some test persons in clinical studies at least experienced light-dark perception and were even able to recognize shadowy outlines.
Further development should at least enable orientation in the foreseeable future. For all other eye tasks, people suffering from RP remain dependent on electronic visual aids, speech computers, and the additional Braille keyboard on the PC.
