Is your vision poor at night? Can't see from both sides? Let's learn about Retinitis Pigmentosa (RP)!

Do you or your child feel like your vision is a little blurry at night? Do you sometimes bump into things inside your house when the light is low in the evening? Or do you see vehicles coming from both sides suddenly approaching you while walking on the road? You may not have paid much attention to these things. But these may be symptoms that you should be a little concerned about. Today we are going to talk about an eye disease that causes such symptoms, but which many people in our country are not exactly aware of. That is Retinitis Pigmentosa, which we call (RP) for short.

Simply put, what is Retinitis Pigmentosa (RP)?

Retinitis Pigmentosa (RP) is not a single disease. It is actually a general name for a group of eye diseases that are inherited, that is, genetically inherited. In this condition, the most sensitive part of your eye, at the back, is called the retina.

Imagine that your eye is like a good camera. The film roll of this camera is called the `(Retina)`. When the light coming from outside enters the eye and hits this `(Retina)`, that is, the film, it creates electrical signals and goes to the brain. The brain interprets those signals as a picture and gives us the feeling of 'seeing'. Now imagine, no matter how good your camera is, if the film in it is damaged, will the photo you take come out clear? It won't come out, right? That's what happens in RP. The cells in the `(Retina)` gradually weaken and become non-functional. Then our vision gradually disappears.

This is a condition that we are born with. But the symptoms often begin to appear in late childhood or adolescence. First, night vision begins to decrease. Then, peripheral vision gradually disappears. Over time, this vision becomes even narrower, and by the age of 40, many people have a significant decrease in vision. Some may even become legally blind. But the change is so severe that it takes some people a while to realize that this is a disease.

What does this name mean?

The name "Retinitis Pigmentosa" is actually a bit misleading. In medicine, if a word ends in "-itis", it means 'inflammation' or 'inflammation'. For example, like `(Appendicitis)`. But RP is not an inflammation of the `(Retina)`. What happens here is that the cells of the `(Retina)` gradually weaken and atrophy. Therefore, a more appropriate name for this is `(Retinal Dystrophy).`

So what does the word "Pigmentosa" mean? It has to do with this condition. When the photoreceptor cells in our retina die, they release a pigment. This pigment is deposited on the retina. When a doctor examines the eye, he or she can see these deposited pigment spots. That's why the word "Pigmentosa" is added to the name.

What are the symptoms of RP?

As we've discussed before, these symptoms appear very slowly. They may develop gradually over years. So it can be difficult to recognize them in the early stages.

Let's see what these symptoms are. To understand clearly, let's divide this into two parts.

Why does this situation occur?

The main reason for this is genetic variations . Simply put, our genes contain the complete set of instructions for how our bodies are made and how they work. This is like the blueprint used to build a house. If there is a change or defect in this blueprint, that is, in the genes, then what is made of it, that is, the parts of our body, may not grow or work properly.

Scientists have identified nearly 100 such genetic changes that can cause RP. You can inherit this genetic defect from your mother or father. Or, a new gene mutation can occur in your body by chance, without anyone in your family having it.

Because there are many types of genes, each gene damages the retina differently. That's why RP varies from person to person. Some people lose their vision very quickly. Others lose their vision very slowly. Sometimes RP can be accompanied by many other symptoms and can appear as another condition, such as Usher syndrome.

What really happens inside the eye?

Let's go a little deeper and see what happens inside the eye. In the ``Retina`` that we talked about earlier, there is a special type of cell that detects light. We call these ``Photoreceptors``. These are the cells that take the light that enters the eye, convert it into an electrical signal, and send it to the brain.

There are two main types of photoreceptors:

• Rods: These are what give us vision in the dark, that is, in low light. They cannot distinguish colors, they only give us black and white vision. There are millions of these `(Rods)` in our `(Retina)`, especially in the periphery of the `(Retina)`, that is, on both sides.
• Cones: These are what help us see colors and fine details clearly in good light, that is, during the day.

In RP , the rods are often damaged first . That's why the first symptoms are reduced night vision and loss of peripheral vision. Over time, the cones also start to be damaged. That's when things like seeing colors and working at close range are affected.

How does a doctor diagnose this disease?

If you have these symptoms, you should see an eye surgeon (Ophthalmologist). He or she will examine your eyes. A routine eye exam can detect the symptoms of this condition.

There are several tests that are usually done to confirm the disease:

• Visual Field Test: This measures your peripheral vision, which is how far you can see to the sides. This can help determine if you have tunnel vision.
• Slit Lamp and Fundoscopy: A special instrument is used to examine the inside of your eye (retina). This can check for pigment deposits associated with RP.
• Retinal Imaging: High-resolution images of the retina are taken to study changes in it.
• ERG Test (Electroretinogram): This is a slightly special test. What is done here is to measure the electrical signals emitted by your `(Retina)` to see how it responds to light. In RP, these signals are very weak.
• Genetic Testing: This test can be done by taking a blood sample and finding out exactly what genetic defect is causing the disease.

What are the treatments for this?

This is the part I hate to hear. Unfortunately, there is no cure for Retinitis Pigmentosa yet. But, don't give up hope. There are many ways and means to help those living with this disease and make life easier.

Your doctor may refer you to vision rehabilitation services. These may include:

• Vision aids - magnifying glasses, special software.
• Occupational therapy - Training in how to perform daily tasks with visual impairment.
• Special education or vocational services.
• Counseling or support groups.

In the meantime, scientists continue to research treatments for this condition. There are currently several experimental treatments that have shown promising results:

• Gene Therapy: This is the biggest hope. What is being done here is to try to correct the defective gene. Currently, there is only one approved treatment for one gene type, `(RPE65).` But research is rapidly underway for other genes as well.
• Vitamin A supplements: In some types of RP, taking the right dose of vitamin A has been found to slow the progression of the disease. But this is very important: Never take vitamin A without consulting your doctor. Some people may be harmed by taking too much vitamin A. So this is a decision that only your doctor can make.
• Retinal Prosthesis: This is an electronic device that is implanted inside the eye. It can provide some vision. However, results vary from person to person. You can ask your doctor if this is a good option for you.