Retinitis Pigmentosa is a group of eye diseases that can eventually lead to loss of vision. It is usually passed down in families. A doctor can usually spot it when he looks at your retina. The retina is a thin layer of tissues at the back of your eye, which converts light into electric signals. If you are diagnosed with RP, the photoreceptors cells in the retina stop functioning properly. This can cause permanent blindness as well.
It’s a rare disorder. It is found in only one out of every 4,000 people. The Retina has mainly two types of cells for gathering light, the rods and cones. The rods are present around the outer ring of the retina and function primarily in dim light. Most forms of Retinitis Pigmentosa tend to affect the rods first. It can tamper the night vision and also the peripheral vision (ability to see sideways) of those affected.
Cones are present in the center of the retina. The cells help in identifying color and facilitates fine detailing. So if RP affects the cones, the central vision slowly fades away. The ability to identify color is affected as well.
The symptoms start appearing from childhood. But we cannot exactly predict when it starts or when things can go really bad! It’s usually said that people affected by RP will start losing their eyesight by the time they reach their early adulthood, and might lose their vision completely and turn blind by the age of 40.
One of the first symptoms include ‘night blindness’. The patients will have a hard time seeing things in poor lighting (in the dark). They will have a hard time reading print and might even stumble over objects not seen.
Loss of peripheral vision (tunnel vision), developing photophobia (discomfort in bright light) and photopsia are other symptoms of RP. Photopsia is a condition where one might see flashes of light that shimmer or blink.
Causes
Over 60 genes can cause different types of RP. Parents can pass on the problem genes to their children in several ways.
Autosomal recessive RP
A child who receives two problem copies of the gene (one from each parent) will develop this type of retinitis pigmentosa. Since two copies of the problem gene are required, there is only a 25% chance for the child to be affected in this scenario.
Autosomal dominant RP
Only one copy of the problem gene is required in this case. There is a 50% chance that the parent can pass on the disorder to the child.
X-linked RP
Here, there is a 50% chance for the mother to pass on the disorder to her child. The mothers will mostly be asymptomatic.
Diagnosis
An ophthalmologist does the following tests to diagnose Retinitis Pigmentosa.
Ophthalmoscope
Here,the doctor will put drops into the eyes. This will make the pupil wider, allowing the doctor to have a better look at the retina. RP is confirmed if there are dark spots on the retina.
Visual field test
Your vision will be judged with the help of a table top machine. Visual field tests help in judging your peripheral vision.
Electroretinogram
The doctor will observe how the retina responds to flashes of light. A film of gold foil or special contact lens are put on the eye for the same.
Doing a genetic test by submitting your DNA sample will also help in diagnosing Retinitis Pigmentosa.
Treatment
There is no proper cure for Retinitis Pigmentosa, but certain treatment methods can help in slowing down the vision loss.
Acetazolamide
The medication can ease the swelling on the retina (macular edema). The treatment helps in improving vision.
Vitamin A palmitate
High doses of the compound may slow down Retinitis Pigmentosa. But the treatment shall be taken up only under the prescription of the doctor.
Sunglasses
Using sunglasses will make eyes less sensitive and protect it from harmful UV radiations.
Retinal Implant
In late stages of RP, retinal implants may help in restoring vision partially. Argus II, an implant available in the USA, is widely used. It’s implanted into a single eye. With its help, images get converted to electrical pulses which are eventually sent to the retina.
There are other treatment methods under review. This includes replacement of damaged cells with healthy ones and gene therapy.
Retinitis Pigmentosa is a condition that will take a physical and emotional toll on the people suffering from it. Thanks to the improving technology, there are several devices that can help in boosting the vision of the patients. Counseling and rehab services will help them stay mentally strong.
If anyone in your family gets diagnosed with the condition, the whole family must visit an eye doctor and get a screening test done immediately!