Retinitis Pigmentosa: Symptoms, Causes, and Treatment

Written by Beth Axtell, Matt McMillen
Medically Reviewed by Timothy G. Murray, MBA, MD, FACS on May 13, 2025
14 min read

Retinitis pigmentosa (RP) is a term for a group of eye diseases that can lead to loss of sight. What they have in common are specific changes your doctor sees when they look at your retina – a bundle of tissue at the back of your eye that transforms the light that enters your eye into electrical signals that travel to your brain. This process lets you see what's around you. When you have RP, cells in the retina called photoreceptors don't work the way they're supposed to, and over time, you lose your sight.

It's a rare disorder that's passed from parent to child. Only 1 out of every 3,500 to 4,000 people have it in the United States, where an estimated 100,000 people have RP. About half of all people with RP have a family member who also has it. Variants in nearly 100 genes have been linked to RP.

The retina has two types of cells that gather light: rods and cones. The rods are around the outer ring of the retina and are active in dim light. Most forms of retinitis pigmentosa affect the rods first. Your night vision and your ability to see to the side – peripheral vision – go away.

Cones are mostly in the center of your retina. They help you see color and fine detail. When RP affects them, you slowly lose your central vision and your ability to see color.

Retinitis pigmentosa usually starts in childhood. But exactly when it starts and how quickly it gets worse varies from person to person. For some people, the disease progresses slowly. But for others, RP causes rapid and severe loss of vision. RP also can worsen at a varying pace over the years, speeding up and slowing down at various points. Most people with RP lose much of their sight by early adulthood. Then by age 40, they are often legally blind. But RP only rarely causes complete loss of vision in both eyes.

What is the first sign of retinitis pigmentosa?

Loss of night vision is usually the first symptom of RP. That's because RP typically affects the rods first. This results in your eyes taking longer to adjust to darkness (called night blindness). For example, you may notice it when you walk from bright sunshine into a dimly lit theater. You may trip over objects in the dark or not be able to drive at night. Because RP often begins in childhood, parents may notice that a child with the disease has a hard time moving through dark rooms or getting used to being in dim light.

Other symptoms of retinitis pigmentosa

  • Progressive loss of peripheral vision is another common early sign. This means you have worsening trouble seeing out of the corner of your eyes.
  • Tunnel vision, which happens when you've lost your peripheral vision and only some central vision remains. You can't see things to the side without turning your head to look at them directly.
  • Loss of central vision as the disease becomes advanced. This can result in blurred or missing vision. This happens if the cones become affected. That will make it harder for you to do detailed work.

Later symptoms of RP may include:

  • Sensitivity to light, a symptom your doctor may call photophobia
  • Loss of color vision, called dyschromatopsia
  • Sensations of flashing or twinkling lights, called photopsia, and you may have trouble seeing colors. It's rare, but sometimes the cones die first.

Broadly, there are two types of RP:

Non-syndromic RP. Also called isolated or simple RP, this type of RP happens on its own and affects only the eyes. 

Syndromic RP. This is RP that comes along with symptoms or conditions that affect other parts of your body along with your eyes. The most common form of syndromic RP is an inherited condition called Usher's syndrome, which causes hearing loss along with RP. Retinitis pigmentosa can happen with many other rare genetic conditions, including:

  • Bardet-Biedl syndrome
  • Alström syndrome
  • Joubert syndrome
  • Senior-Løken syndrome
  • Jeune syndrome

Overall, syndromic RP is much less common than non-syndromic RP, which accounts for as many as 4 out of 5 cases of RP.

Variants or mutations in nearly 100 genes can cause the different types of retinitis pigmentosa. Parents can pass the problem genes on to their children in three ways:

Autosomal recessive RP: Each parent has one problem copy and one normal copy of the gene that's responsible, but they don't have any symptoms. A child that inherits two problem copies of the gene (one from each parent) will develop this type of retinitis pigmentosa. Since two copies of the problem gene are needed, each child in the family has a 25% chance of being affected.

Autosomal dominant RP: This type of retinitis pigmentosa requires only one copy of the problem gene to develop. A parent with that gene has a 50% chance of passing it to each child.

X-linked RP: A mother who carries the problem gene can pass it down to their children. Each one of them has a 50% chance of getting it. Most women who carry the gene won't have any symptoms. But about 1 out of every 5 will have mild symptoms. Most men who get it will have more severe cases.

The only known risk factor for the disease is having a gene variant that causes RP. But certain things may affect how the disease gets worse, though much more research is needed to confirm that the following play a role in RP.

  • Age. The younger you are when symptoms begin, the faster the disease is likely to get worse.
  • Sex. Men and people assigned male at birth (AMAB) may be more likely to have the disease worsen to legal blindness.
  • Smoking. Some researchers think smoking may make RP get worse faster.

An eye doctor can tell you if you have retinitis pigmentosa. Your doctor will talk to you about your symptoms, review your medical history and your family's medical history (including a history of eye problems), and have you get a comprehensive eye exam in order to:

  • Evaluate your current vision
  • Check how well the rods in your eyes function
  • Look for any changes to your retina

Tests for retinitis pigmentosa

The tests your eye doctor will use to diagnose RP include:

  • Ophthalmoscope. The doctor will put drops into your eyes to make your pupil wider to get a better look at your retina. They will use a handheld tool to look into the back of your eye. If you have RP, there will be specific kinds of dark spots on your retina.
  • Visual field test. You'll look through a tabletop machine at a point in the center of your vision. While staring at that point, objects or lights will appear to the side. You'll press a button when you see them, and the machine will create a map of how far to the side you can see. This test helps evaluate your peripheral vision.
  • Electroretinogram. The eye doctor will put a film of gold foil or a special contact lens on your eye. Then they will measure how your retina responds to flashes of light.
  • Optical coherence tomography scan. In this non-invasive test, you'll focus your eye on a target while your doctor takes a picture of the back of your eye. This is done to measure the thickness of your retina and evaluate its integrity.
  • Fundus autofluorescence test. This non-invasive imaging test uses blue light to take pictures of your retina to see how healthy it is.
  • Genetic test. You'll submit a DNA sample to find out which form of RP you have, which may tell you how the disease will progress over time.

If you are diagnosed with retinitis pigmentosa, other family members may benefit from genetic testing. But first, they should speak with their doctor or a genetic counselor to understand the benefits and risks of screening. 

What to know when you're first diagnosed

You should know that RP is a progressive disease that gets worse over time. For that reason, you should find an eye doctor who specializes in RP. Such doctors, known as retina specialists, can help you understand your disease, manage its complications, track its progress with specific eye tests, and refer you to clinical trials that are looking into promising treatments.

You also should know that RP is a genetic disease that runs in families. You may want to discuss RP with your doctor or a genetic counselor if you plan to have children.

It can be helpful to connect with others who have RP or another condition that causes vision loss. Your doctor can suggest organizations that have active communities.

How to deal with a sudden diagnosis

Learning that you may lose your vision can be frightening, and it can lead to grief and depression not unlike what you have after the death of a loved one. Healthy ways to cope can help.

  • Consider talking to a therapist trained in grief counseling.
  • Be open with friends and family about your condition.
  • Connect with others facing vision loss.
  • Try meditation and mindfulness to ease your grief and anxiety.
  • Seek out lifestyle adjustment classes to learn how to adapt your daily life to your vision loss.
  • Think about getting a service animal, such as a dog, both for helping you get around and for companionship.

You'll receive care from many types of health care professionals after your diagnosis of RP. Your team may include:

Ophthalmologist/retina specialist. An ophthalmologist is a medical doctor who treats eye diseases. A retina specialist is an ophthalmologist with advanced training in diseases and surgery of the retina and the vitreous body of the eye, between the eye's lens and retina.

Genetic counselor. Specially trained in medical genetics, your genetic counselor will help you understand how genes affect RP as well as guide you and your family through the genetic testing process – its benefits, risks, and limitations.

Occupational therapist (OT). Your OT will help you learn new ways to safely do everyday tasks as your RP progresses and your vision gets worse. Your OT will train you to use vision aids and assistive tech as well as teach you new visual skills, including those that can help compensate for the loss of peripheral vision.

Orientation and mobility (O&M) specialist. An O&M specialist will teach you the skills you need to situate yourself in whatever environment you're in. They will also teach you how to move safely through that environment. You'll learn from your specialist how to use a cane, cross a street, ride public transportation, and other skills you need to get around and travel.

Rehabilitation teacher/vision rehabilitation specialist. These specialists help you learn skills that let you live and work independently. They teach you how to best use the vision you have now to improve your quality of life.

Social worker. These professionals provide short-term counseling to address the emotional troubles that come with vision loss, which can include depression, anxiety, sadness, frustration, and anger. This can help you better cope with your vision loss. Your social worker also will help you navigate the benefits, resources, and services available to you.

There's no cure for retinitis pigmentosa, but doctors are working hard to find new treatments. A few options may slow your vision loss and may even restore some sight.

Medications for retinitis pigmentosa

  • Voretigene neparvovec-rzyl (Luxturna). This gene therapy improves vision in people with RP caused by a defect in the RPE65 gene. It is not effective for RP caused by defects in other genes. This treatment is given via injection into each eye. You will only need one shot per eye.
  • Acetazolamide. In the later stages, the tiny area at the center of your retina can swell. This is called macular edema, and it, too, can reduce your vision. This medication can ease swelling and improve your vision.

Surgery for retinitis pigmentosa

  • Cataract surgery. Cataracts, which cloud your eyes' lenses and limit vision, can develop in RP. You may benefit from surgery to remove cataracts.
  • Retinal implants. The FDA has approved a prosthetic retina called Argus II for advanced RP. But studies of the benefits of these surgically implanted devices have shown mixed results. It's implanted into a single eye and paired with glasses equipped with a camera. Images are converted to electrical pulses that are sent to the retina. Many people studied were able to find lights and windows. Some were able to know where other people were in a room, and about half of the subjects were able to read letters that were about 9 inches high.

Natural or alternative treatment for retinitis pigmentosa

  • Vitamin A palmitate. Some research has found that high doses of this compound may slow retinitis pigmentosa by about 2% each year. But recent research suggests that vitamin A has no benefit for RP. If you do take vitamin A, keep in mind that too much can be toxic to your liver. For this reason, you should have regular monitoring of your liver function if you take vitamin A long term. Work closely with your doctor and follow their recommendations.
  • Other supplements. Omega-3 fatty acids (DHA), found in fish oil, and tauroursodeoxycholic acid (TUDCA) may slow vision loss. But more research is needed to support their use for RP. Both are available as over-the-counter supplements.

Home remedies for retinitis pigmentosa

  • Sunglasses. These make your eyes less sensitive to light and protect your eyes from harmful ultraviolet rays that may speed vision loss.
  • Low vision aids. There are many devices that can help you see things better as your vision gets worse, including telescopic and magnifying lenses, as well as adaptive devices for night vision. Your eye doctor or other member of your care team can help you select the right devices for you.

RP does get worse over time, but how quickly and severely it progresses varies, depending on several things:

  • The gene defect responsible for your RP
  • Your age at diagnosis. The younger you are when symptoms begin, the faster the disease is likely to progress.
  • Your sex. RP in men and people assigned male at birth (AMAB) may be more likely to progress to legal blindness.

Some researchers divide the disease into stages.

Early stage

The disease usually starts with the loss of night vision in childhood. 

Middle stage

People with RP begin to lose their peripheral vision, or their ability to see out of the corner of their eyes. They also may have a hard time telling pale colors apart and may become sensitive to light.

Late stage

Eventually, they will likely lose all their peripheral vision, leaving them only with central vision, a condition called tunnel vision. This typically happens slowly. They may become a lot more sensitive to light. Many people with RP have good central vision into their 40s and 50s, and most don't lose all their vision.

In some cases, RP mostly affects the cones in the eyes. This causes a loss of central vision and the ability to tell colors apart early in the disease course.

As RP progresses, it leads to many complications that affect your vision. These include.

Vision loss. RP causes gradual loss of vision, usually starting with your night vision, followed by your peripheral vision, before finally affecting your central vision. This loss takes place over many years.

Tunnel vision. This develops due to the loss of your peripheral vision, or your ability to see out of the corner of your eye, leaving you with only central vision.

Cataracts. This is clouding of the lenses in your eyes. It commonly occurs in RP.  Surgery can remove cataracts, which may help you see better.

Cystoid macular edema. This is swelling of the central part of the retina. It causes things you see with your central vision to appear blurry or wavy.

Color vision problems. As RP progresses, you may become less able to tell colors apart, a condition called dyschromatopsia. This usually happens in more advanced RP.

Reduced contrast sensitivity. Contrast sensitivity is your ability to see sharp and clear outlines of small objects as well as the ability to distinguish subtle differences in shading and patterns. These abilities decline as RP progresses. That makes it harder to distinguish objects and text from what's in the background.

Other potential complications include:

  • Sensitivity to light
  • Depression and anxiety
  • Reduced quality of life
  • Reduced ability to live independently
  • Myopia (nearsightedness) and astigmatism (a cause of blurred vision)
  • Glaucoma
  • Vitreous hemorrhage, or bleeding in the vitreous, the gel between your lens and retina
  • Detached retina
  • Total blindness, which is uncommon in RP

This disease will be with you for the rest of your life. Staying in close touch with your care team is critical. They can keep track of your RP's progression. Always contact your doctor if your symptoms get worse or new ones develop, such as changes in your vision or new pain or discomfort in your eyes.

How is retinitis pigmentosa managed?

There's a lot you can do to live well with RP and to adapt to changes in your vision, including:

  • Wear high-quality sunglasses to protect your eyes from the sun.
  • Don't smoke.
  • Get a genetic test to identify the type of RP you have. This can tell you how your disease is likely to progress.
  • Work with your care team to learn ways to remain independent for as long as possible.
  • Know the warning signs of depression and talk to your doctor if you have them.
  • Stay informed about treatment developments that may help you.
  • Learn the use of adaptive devices that will help you make the most of your remaining vision.
  • Ask your doctor about taking part in clinical trials of new treatments that may help you.
  • Reach out to your state's rehabilitation department to learn about local resources for the blind.

Retinitis pigmentosa and vision loss

Your vision loss likely will be gradual, but keep your care team informed about changes to your vision. Work with an occupational therapist and other care team members who can help you adapt to vision loss and remain independent as long as possible.

Retinitis pigmentosa and mental health

People with RP are much more likely to have depression and anxiety than people who don't have this disease. Learn the symptoms of these mental health problems and talk to your doctor if you get them.

Numerous organizations can help those with RP and their families. They offer detailed info about the disease, support groups, and more. These include:

The disease is not one-size-fits-all, so everyone will go through it differently. For some people, it progresses quite slowly, while others may lose vision more quickly. Your age when it started and the gene defect that caused it will determine how it progresses. While nearly everyone with the disease eventually will become legally blind, RP does not commonly cause complete blindness.

Can retinitis pigmentosa be cured?

No, there is no cure for RP. But your care team can help you to live and function well with the disease.

What should you expect with retinitis pigmentosa?

If you have RP, you can expect to lose much of your ability to see. This usually starts with loss of night vision and progresses to loss of peripheral vision. Your central vision also likely will be affected, as well as your ability to tell colors apart.

Retinitis pigmentosa is a disease that causes vision loss over time. It usually progresses slowly. Most people with RP may be legally blind by about age 40. 

While there’s no cure for RP, there are numerous treatments and lots of support available to help adapt to and live well with worsening vision.

Is retinitis pigmentosa inherited?

Yes. Defects in nearly 100 genes have been linked to the development of RP.

Is there a cure for retinitis pigmentosa?

No. Treatment focuses on adapting to vision loss with adaptive devices, rehabilitation, and other resources your care team can provide.

How fast does retinitis pigmentosa progress?

This depends on the age when your symptoms first developed and the gene responsible for your RP. For most people, though, the disease progresses slowly, over many years.

What is the average lifespan of someone with retinitis pigmentosa?

If your RP affects only your eyes, your lifespan will be no different from that of the average person. But if your RP develops due to another disease, your lifespan may be affected. For example, those with the rare Hurler syndrome typically live no longer than 10 years.