Retinitis pigmentosa

Retinitis pigmentosa (RP) is a type of retinal dystrophy and is a genetic condition. It affects the retina at the back of the eye and causes the retinal cells that process light to stop working over time, leading to vision loss. It typically first affects your peripheral vision (side vision) and makes it difficult to see in low light.

Retinitis pigmentosa symptoms

Symptoms can develop at any age, though they are often first noticed between the ages of ten and 30. There is currently no treatment for RP, but there are ways you can manage your condition.

Symptoms of retinitis pigmentosa often begin in childhood, progressing gradually over years into adulthood.

Early symptoms

One of the first signs of retinitis pigmentosa is difficulty seeing in the dark or dim light. You might become sensitive to bright light and have trouble adjusting to changing light levels, such as going from a bright sunny day into a dark room indoors. 

Another early symptom is having trouble with peripheral vision (side vision). This reduces your visual field and makes it harder to see things at the edge of your vision without actually turning to look at them. You might begin bumping into things you wouldn't have had a problem with before. This can progress to tunnel vision in later stages, leaving a small clear area in the centre of your vision.

In rare types of RP, the central vision may be affected first.

Later symptoms

As retinal degeneration develops over time, it can eventually cause problems with central vision. This affects visual acuity making it harder to read, watch TV, and recognise people’s faces. Some people also have trouble seeing different colours.

As RP progresses, it can lead to significant visual impairment, though how long this takes varies from person to person. 

Living with retinitis pigmentosa can mean adjusting to changing levels of vision over time. You can get support to make the most of your useful vision and adapt to changes in your sight.

Retinitis pigmentosa in children

Symptoms of retinitis pigmentosa can develop from a young age. Children should have regular eye tests, and if there’s a family history of RP, mention this to your child’s optician. Children with RP may have particular difficulty seeing in the dark and adjusting to changes in light. Speak to your child’s GP or optician if you notice they have any problems like this.

Treatment of retinitis pigmentosa

There is currently no treatment to cure or slow the progression of retinitis pigmentosa. Clinical trials are underway into possible treatments such as gene therapy and stem cell treatment, but these aren’t yet available. 

People with RP can be at risk of developing cataracts. Cataracts can be treated with surgery to ensure vision remains as clear as possible. There is also a chance with RP of developing macular swelling (oedema), which is treated with eye drops.  

What causes retinitis pigmentosa?

Retinitis pigmentosa is caused by a fault in one of the genes responsible for the normal function of the retina. The condition is inherited, and it's not possible to prevent it. If there is a history of retinitis pigmentosa in your family, mention this to your optician or ophthalmologist when you go for eye tests.

There are different ways of inheriting RP - autosomal dominant inheritance, autosomal recessive inheritance, and X-linked RP. The condition can be due to a new mutation of a gene in the person who has RP, and around half of people with the condition have no family history of it (Source: NORD). The way it is inherited influences the likelihood of inheriting it, the severity of the condition, and the chance of passing it on to your children.

There are different types of retinitis pigmentosa linked to the exact gene responsible for the condition. As genetic science advances, more is being discovered about these different forms of RP. You might be given a more detailed diagnosis after genetic testing and have the option of genetic counselling through the NHS. This might be something to consider if planning a family, to help understand the implications of passing on the condition.

Getting a retinitis pigmentosa diagnosis

Your optician (also known as an optometrist) can check for signs of RP by looking closely at your retina. They will look for signs of pigmentation on the retina. They can do a visual field test to check your peripheral vision. You should tell your optician if you have a family history of RP and of any concerns about your eyesight. 

The optician can refer you to an ophthalmologist (eye specialist) if necessary. They use a range of tests to check for RP, including an electroretinogram (ERG) to measure how your retina responds to light, and they can arrange for genetic testing which can confirm the exact type of RP you have. This can take some time, and they may not arrange all of these tests straight away.

When to get medical advice

Regular eye tests monitor eyesight and eye health and can detect many eye diseases before noticeable symptoms occur. The NHS recommends having an eye test at least every two years. If you have a family history of RP, it’s even more important to have regular eye tests.

See your optician or GP as soon as possible if you notice changes in your vision or have any worries about your child's sight. You can also call 111 for advice if you’re concerned.

Living with retinitis pigmentosa

Because RP is a progressive condition, it involves adapting to changing levels of vision over many years. A diagnosis like this can be tough to deal with, and it's natural to feel a whole range of emotions when facing sight loss. It can help to hear from other people with RP for practical tips and to understand how they feel about living with retinitis pigmentosa.

Retina UK also has some useful information.

Frequently asked questions