Retinitis Pigmentosa: Age of Onset

November 26, 2019

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Having a healthy pair of eyes is surely a blessing in disguise, the importance of which is often undermined. But what do you do when you become afflicted with an eye disease?

It may become rather distressing when the disease in question is one such as Retinitis Pigmentosa ; which is a result of genetic inheritance rather than nutritional deficiency or negligence in health care.

However, the best strategy to cope with this challenge head-on is to be as well informed about it as you can be. As wise people say

Knowledge is power.

As suggested in one of the researches conducted through the platform of Health Promotion International, attaining health literacy and a thorough understanding of your chronic disease is essential for successful self-management.

Having sufficient knowledge about Retinitis Pigmentosa (RP) will equip you with skills to access, comprehend, evaluate and act in accordance for improved health outcomes and personal well-being.

Let’s get started.

What is Retinitis Pigmentosa?

Retinitis Pigmentosa (RP) is a chronic disease of the eye, which is characterized by a steady deterioration of vision. This visual problem is basically a hereditary disorder (which means that you inherit it from your parents), and it occurs due to a constant and progressive damage caused to the retinal cells of the eye – known also as the photosensitive cells.

The main function of these photosensitive cells is to collect and convert the visual signals from a person’s surroundings into neural signals that can be intercepted and translated by the brain in the form of coherent images.

The degeneration of rod cells (which are responsible for vision in darkness) and cone cells (which control visual acuity as well as colored vision) ultimately leads to a loss of a person’s night vision and peripheral vision.

Here’s more about Retinitis Pigmentosa.

What Causes Retinitis Pigmentosa?

Before approaching the symptomatic conditions of Retinitis Pigmentosa, it is important to understand the causality of this disease.

Retinitis Pigmentosa is basically a genetic disease, rooted in genes and is passed down in a child whose either one or both parents had the same condition.

There are more than 60 different types of problem genes which can cause this eye condition. These defective genes, if possessed by parents, can subsequently be passed down to their offspring.

What is the likelihood of inheriting RP

Retinitis Pigmentosa can be passed down in various ways. It can often be inherited through an autosomal dominant inheritance pattern.

It means that if either of your parents has a dominant gene for this disorder (a parent has symptoms of Retinitis Pigmentosa) then you have a 1 in 2 chance of contracting it i.e. you have a 50% chance of having the condition as well.

However, if both your parents carry the recessive gene for RP, and do not have any symptoms of the condition themselves, then you have slightly lesser risk of contracting it.

It is known as the autosomal recessive inheritance pattern. In this pattern, there is only a 1 in 4 chance of them passing it down to you i.e. a 25% chance of you being affected by Retinitis pigmentosa.

Onset of Symptoms

The onset of symptoms usually begins in childhood and can be identified in a child as young as 10 years of age, but for some people the symptoms may not be manifested until later in adulthood.

The age of onset of symptoms and the progressive speed of the disease, both vary from person to person.

RP begins with deterioration to the rod cells of the retina first, thus causing a loss of night vision for the individual. The person may find it increasingly difficult to see and recognize figures during night hours, or at places with dim lighting.

In the progressive stages of RP, the damage eventually extends toward the cone cells and leads to a loss of visual clarity and vividity, in addition to the inability of differentiating colored objects in the surroundings.

In the later stages, individuals with RP tend to have tunnel vision as their ability of peripheral vision deteriorates.

Manifestation of Symptoms of RP

Typically, as the condition first begins to manifest itself, individuals experience the following symptoms of Retinitis Pigmentosa:

Reduced night vision
Blurred vision / lack of visual acuity
Poor Color Differentiation
Tunnel Vision
Photophobia (increased visual sensitivity and experiencing discomfort/ pain when exposed to bright lights)

People rarely experience complete blindness due to RP, however, chances do prevail that the extent of vision damage may cause individuals to become legally blind at an older age.

Symptom Manifestation; How does the world Look like in RP?

Loss of Night VisionPeople with RP cannot see properly in the dark. They may not have much difficulty carrying out the routine chores during the day time, but damaged night vision makes it impossible to stroll around, drive or do other work during the dark.To begin with, these people first notice that they find it relatively harder to adjust to light changes, in comparison to an average person. If a light is switched off, it would take their eyes a longer period yo adjust to this level of light in the room.They may stumble over objects and bump into doors while trying to navigate through the dark spaces. So, places like movie theaters or dimly lit rooms may become uncomfortable places for them.
Loss of Peripheral VisionPeripheral vision refers to side vision, or the view you see away from the center of your gaze. Loss of peripheral vision constricts the visual field to only those objects which lie straight in the center of one’s line of sight.It may feel like seeing through a tunnel, hence termed as tunnel vision. For a person with tunnel vision, moving around can become quite difficult as they do not get a full view of their surroundings unless they move and line the desired object within visual field straight in line with their central vision.
Loss of Central VisionAlthough RP begins with a loss of peripheral vision, it may slowly start affecting central vision as it progresses. This makes it difficult to perform detailed tasks that require focus, such as reading or sewing etc. Loss of central vision is experienced by the person as having a blurred spot in the center of their vision.
Loss of Color VisionIn some cases, individuals with retinitis pigmentosa may experience some form of color blindness. Since the retinal cells become damaged, they become unable to distinguish between two colors of objects, or simply lose the vividity with which they could see before the onset of RP.

Frequently Asked Questions About Retinitis Pigmentosa

While dealing with such a health condition, it is not unlikely to encounter puzzling questions that may leave you wondering what measures should you take and which ones to stay away from. Here is a helpful list of FAQs you can consult for further concerns.