Retinitis Pigmentosa: Causes, Symptoms, Diagnosis, and Treatments

What Does Retinitis Pigmentosa Look Like?

Retinitis pigmentosa may look like an individual who struggles to see at night or during low-light conditions. Retinitis pigmentosa from the patient’s view may look like tunnel vision due to impaired peripheral vision according to UVA Health. The image below shows how retinitis pigmentosa can affect your vision, where peripheral vision gradually reduces, leaving you with central vision.

What is the Medical Term for Retinitis Pigmentosa?

There is no other medical term for retinitis pigmentosa as retinitis pigmentosa is the medical term for this condition.

What Age Does Retinitis Pigmentosa Usually Appear?

The age at which retinitis pigmentosa usually appears can differ according to how it was inherited. The autosomal recessive form can present its symptoms in the early teen years, autosomal dominant retinitis pigmentosa may not trigger symptoms until your mid or late 20s and more than 75% of patients with retinitis pigmentosa are symptomatic by the age of 30 according to Teri B. O'Neal and Euil E. Luther (2023).

Is Retinitis Pigmentosa a Disability?

Yes, retinitis pigmentosa is considered a disability. According to the Australian Government, retinitis pigmentosa is one of the many eye conditions that are commonly assessed to determine the level of impact on visual functions. Consideration of your condition will require a diagnosis from a qualified medical practitioner with corroborating evidence from an ophthalmologist, optometrist, neurosurgeon or neurologist.

Is Retinitis Pigmentosa Genetic?

Yes, retinitis pigmentosa is genetic. Retinitis pigmentosa can be inherited through four inheritance patterns, which include autosomal recessive, autosomal dominant, x-linked recessive and mitochondrial inheritance according to the National Institutes of Health. Autosomal recessive is where both parents carry the altered gene which can then be passed onto their children. Autosomal dominant is when just one parent carrying the gene is sufficient to pass it on to their children. X-linked recessive is where the gene is carried by healthy females and only passed on to their sons and mitochondrial inheritance is where the gene is only inherited from the mother according to MedlinePlus.

1. Trouble Seeing in Dim Light and Poor Night Vision

Trouble seeing in dim light and poor night vision may occur as part of the first signs of retinitis pigmentosa. Trouble seeing in dim light and poor night vision can develop as retinitis pigmentosa can cause the rod cells to break down over time. The rod cells in the retina are what help us see at night and during low light conditions according to the American Academy of Ophthalmology. Children may typically start to exhibit loss of night vision when they struggle with moving around in the dark according to the National Eye Institute. It is a common first sign of the condition and should be examined by an optometrist to know next steps.

2. Feeling of Shimmering or Flickering Light in Vision

Feeling of shimmering or flickering light in your vision, also referred to as photopsia, may be a possible symptom of retinitis pigmentosa and can take place when the retina is damaged. Shimmering or flickering light may appear in your vision suddenly and only last for a brief time. According to the Investigative Ophthalmology and Visual Science (IOVS) Journal, patients in the early stages of retinitis pigmentosa had reported their experience of shimmering or flickering lights appearing in their peripheral vision. Those with advanced retinitis pigmentosa as well as loss of visual acuity reported experiencing shimmering or flickering in the areas where their vision remained according to IOVS.

3. Loss of Side Vision (peripheral) and Blind Spots

Loss of side vision (peripheral) and blind spots may develop as a result of photokeratitis following the occurrence of poor night vision and low light vision. Peripheral vision loss and blind spots can occur as they cause the cells in the retina to gradually deteriorate according to the National Eye Institute. Rod cells are one of the four photoreceptor cells that assist with both our peripheral vision and night vision. As a result, patients can start to develop low vision as your vision may be more narrow, leaving you with just your central vision. This may be referred to as tunnel vision according to the National Eye Institute. If you experience loss of vision to any degree, it is important to seek prompt medical attention from your local doctor or optometrist.

4. Photophobia

Photophobia refers to the discomfort felt when your eyes are exposed to bright light and can be a possible symptom of retinitis pigmentosa. Photophobia, also known as light sensitivity, may ensue during retinitis pigmentosa as another early sign according to Kathleen B. Digre and K.C. Brennan (2012). Photophobia may be triggered as a result of retinal damage, which can lead to the light that enters the eye to scatter rather than reaching the macula according to the Royal National Institute of Blind People.

5. Colour Vision Problems

Colour vision problems may develop as a consequence of retinitis pigmentosa due to the damage of the cone cells in the eye. Colour vision may be affected during retinitis pigmentosa as the cone cells are what enable our detection of colours according to the National Library of Medicine.

Can a Slit Lamp Exam Diagnose Retinitis Pigmentosa?

Yes, a slit lamp exam can diagnose retinitis pigmentosa. A slit lamp exam is used to assess your overall eye health by dilating a patient's pupils to look closer at the back of the eye. A slit-lamp eye exam is performed using a microscope with a bright light. A slit lamp exam may be carried out not only to diagnose retinitis pigmentosa but also cataracts, glaucoma, dry eye, corneal abrasions, macular degeneration and corneal diseases.

How Long Does Retinitis Pigmentosa Last?

Retinitis pigmentosa can last from childhood to senior age. According to Elliot L. Berson (2007), some cases of retinitis pigmentosa can cause loss of vision in patients at the age of 30. However, other cases of retinitis pigmentosa were able to have functioning vision up to 80 years of age.

What are the Eye Tests for Retinitis Pigmentosa?

The eye tests for retinitis pigmentosa are a comprehensive eye test, visual field test, colour vision testing and digital scans of the eye according to Better Health. The list of eye tests for retinitis pigmentosa are listed below.

• Comprehensive Eye Test: A comprehensive eye test involves a series of tests that help assess different regions of the eye to determine ocular health and diagnose any eye problems.
• Visual Field Test: A visual field tests your peripheral vision
• Colour Vision Test: A colour vision test is for assessing your ability to see and distinguish between colours.
• Digital Scans: Digital scans of the eye or a digital retinal scan is used to capture high-resolution images of structures inside the eye. These can include the retina, optic nerve, blood vessels and the macula.

How is Retinitis Pigmentosa Treated?

Retinitis pigmentosa may be helped with low vision aids to improve residual vision as there is no cure for retinitis pigmentosa. Treatment for retinitis pigmentosa may include resources such as hand-held magnifiers, magnifying reading glasses, prismatic reading glasses and reverse telescopic glasses according to the Optometrists Network. Vitamin A supplements may have previously shown effectiveness in slowing the rate of retinal deterioration. However, more recent studies have reported no difference was made using vitamin A according to Teri B. O'Neal and E. Luther (2023)

Can Eye Drops Help Manage Retinitis Pigmentosa?

No, eye drops cannot help manage retinitis pigmentosa. However, according to Medical News Today, certain eye drops and medication may help to reduce swelling from macular edema. Macular edema is an eye condition that can occur as a consequence of retinitis pigmentosa and refers to the buildup of intraretinal fluid in the macular according to the Nature Journal.

Can People with Retinitis Pigmentosa Wear Glasses?

Yes, people with retinitis pigmentosa can wear glasses, however, it may not improve their vision. People with retinitis pigmentosa may require special glasses depending on the stage of their vision loss according to and on the optometrist’s treatment plan.

What Happens if Retinitis Pigmentosa Goes Untreated?

There is no specific treatment for retinitis pigmentosa, therefore, symptoms may continue to worsen gradually, depending on your condition. Retinitis pigmentosa can lead the cells in the retina to gradually break down resulting in the loss of your peripheral vision or blindness. However, your optometrist may recommend wearing sunglasses to ensure your remaining vision is protected from ultraviolet exposure according to Health Direct.

How Can Ophthalmologists Help Prevent Retinitis Pigmentosa?

Ophthalmologists may not be able to prevent retinitis pigmentosa as it is passed down from your parents. According to Teri B. O'Neal and E. Luther (2023), there is no standard treatment for retinitis pigmentosa. After receiving an assessment from an optometrist, you may likely be referred to an ophthalmologist. They will then be able to examine your condition and determine the rate of progression and/or damage.