Retinitis Pigmentosa

Retinitis pigmentosa (RP) is a group of inherited eye diseases that cause the light-sensing cells of the retina to break down over time. The result is a slow, progressive loss of vision, typically beginning with difficulty seeing in dim light and a gradual narrowing of peripheral vision, and in some cases advancing to significant vision loss or blindness.

Unlike many other causes of vision loss, RP is genetic in origin. It can affect people of any age and background, and the degree of vision loss can vary widely between individuals, even within the same family. While there is currently no cure for most forms of RP, early diagnosis, the right support and emerging treatments are giving people with RP more options than ever before.

Retinitis pigmentosa is caused by mutations in genes that provide instructions for the proteins needed by the retina’s photoreceptor cells. When these genes are faulty, the rod cells – which detect low light and peripheral vision – begin to die. Over time, cone cells (which support central and colour vision) are affected too. The retina gradually loses its ability to process light, and vision deteriorates.

Autosomal recessive RP is the most common inheritance pattern, accounting for roughly 60–70% of cases. Both parents carry a faulty gene but are typically unaffected themselves; each of their children has a 25% chance of developing the condition.

Autosomal dominant RP accounts for approximately 30–40% of cases. One affected parent passes on the gene mutation, and each child has a 50% chance of inheriting the condition. Symptoms in dominant RP tend to appear later in life.
Retinitis pigmentosa is estimated to affect around 8,900 Australians,¹ and inherited retinal diseases (IRDs) as a whole affect approximately one in every 3,000 Australians.² Retinitis pigmentosa is the most common form of IRD and is the leading cause of blindness in working-age adults in Australia.³

Symptoms of retinitis pigmentosa usually appear gradually and may be so subtle at first that they go unnoticed. The pattern of vision loss is fairly consistent: peripheral and night vision are affected first, often followed by loss of central vision. Common retinitis pigmentosa symptoms include:

Night blindness (nyctalopia) – difficulty seeing in low light or darkness or a slow adjustment when moving from a bright to a dim environment is typically the first symptom of RP.

Loss of peripheral (side) vision – as the rod cells around the edges of the retina deteriorate, the visual field gradually narrows, creating a characteristic ‘tunnel vision’ effect.

Difficulty adjusting to changes in light – moving between different lighting conditions, such as entering a dark room from a bright environment or stepping outside on a sunny day, can take noticeably longer than usual.

Difficulty with contrast and detail in dim light – faces, steps, kerbs and other features may become harder to distinguish in low-contrast or dim environments, increasing the risk of trips and falls.

Loss of colour vision – as cone cells – which are responsible for colour perception and fine detail – are affected in later stages, colours may appear washed out or less vivid.

Light sensitivity– some people with RP experience increased sensitivity to bright light or glare, finding sunlight or artificial lighting uncomfortable or disorienting.

Gradual loss of central vision – in advanced RP, central vision – used for reading, recognising faces and fine detail tasks – also becomes affected.

Retinitis pigmentosa vision is often described as looking through a narrow tube, with the world visible only in the centre and darkness or blur around the edges. In low light, even this central window may become unreliable. People with RP often develop sophisticated strategies to compensate, using head movements to scan their environment, or relying on familiar surroundings to navigate. Because vision loss is gradual, many people adapt over time without fully realising how much their field of view has changed.

Some changes that can signal retinitis pigmentosa is progressing include:

• Missing objects or people at the edges of your visual field
• Needing more time to adjust when entering a dark building from bright sunlight
• Bumping into objects or people that others would have seen in their peripheral vision
• Increasing difficulty reading in dim environments or on low-contrast backgrounds

If you notice any of these signs, speak to your GP or paediatrician. Guide Dogs Australia’s early childhood services are available from the point of diagnosis.

There is currently no cure for most forms of retinitis pigmentosa, and no standard treatment that halts progression in the majority of patients. For most people, management focuses on slowing the rate of vision loss, managing associated conditions, and maximising remaining vision through low vision aids and rehabilitation.

Guide Dogs Australia supports people at all stages of retinitis pigmentosa, including:

• Orientation and mobility training – building the confidence and practical skills to move safely and independently as vision changes.
• Assistive technology support – screen magnification, audio tools and low vision aids to help maintain independence in daily life.
• Early childhood services – for children and adults adjusting to vision changes caused by RP.
• Psychology and counselling – for people navigating the emotional impact of a progressive, inherited vision condition.
• Peer support – connecting with others who understand the experience of living with vision loss firsthand.

Beyond Guide Dogs Australia, Retina Australia and Vision Australia are valuable community resources offering education, research updates, advocacy and peer support for people living with RP and other inherited retinal diseases.

A retinitis pigmentosa diagnosis changes things, but it does not define what is possible. Many people with RP live active, fulfilling lives with the right support around them. Guide Dogs Australia is here to help at every stage of that journey.

¹Retina Australia. Retinitis Pigmentosa (RP). Available at: https://retinaaustralia.com.au/resources/retinitis-pigmentosa-rp/

²Better Health Channel (Victorian Department of Health). Eyes – retinitis pigmentosa. Available at: https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/eyes-retinitis-pigmentosa

³Jolly JK, et al. Driving with retinitis pigmentosa. Ophthalmic Genetics. 2023. Available at: https://www.tandfonline.com/doi/full/10.1080/13816810.2023.2196338