The term retinitis pigmentosa (RP) refers to a category of rare diseases that happen to the retina, a thin layer of tissue at the back of your eye that helps turn light into signals that get sent to your brain. When you have RP, cells in your retina degrade over a long period of time, which can make it harder for you to see.
Most people with RP are born with it, as it’s a genetic condition passed down in families. You may not notice the most obvious symptoms of RP until later in childhood. But the symptoms often become more noticeable with age and result in a significant amount of vision loss over time.
There is currently no cure for RP, but there are many options to help you improve your vision and train yourself to use your vision in a way that accommodates your vision loss. Read on to learn more about this condition’s causes, symptoms, and treatments.
RP doesn’t have a clear cause yet, but it results from mutations or differences in genes that affect the photoreceptor cells in your retina. These cells are responsible for helping you see colors and adjust to low light.
RP damages these cells, which can make it harder for you to see throughout your life. But researchers and doctors aren’t sure exactly what triggers these changes in your genes.
There’s some research that suggests that RP can also result from:
- Medications: A
2019 research reviewshowed that medications called phosphodiesterase type 5 inhibitors, such as sildenafil (Viagra), may link to worsening conditions like RP in people who may have gene mutations that increase their risk of developing RP.
- Infections: RP involves a
RPGR genemutation. An upper respiratory tract infection, such as chronic sinusitis, can trigger the gene, resulting in RP symptoms.
- Injuries to the eye: A
2012 studysuggested that injuries to the eye can result in long-term retina damage, potentially resulting in the onset of RP symptoms.
- Other genetic conditions:
Usher syndromeis another genetic condition that commonly associates with RP and other symptoms like deafness or difficulty staying balanced while walking.
The symptoms of RP typically become noticeable when you’re still young.
While some people with RP may experience a slow symptom progression, RP symptoms can also appear without warning and progress quickly — especially as you get older.
Here are some of the most common symptoms of RP:
- significant vision loss at night or in dim, low lighting even when your vision during the day or in bright lights seems to remain the same
- eye adjustment difficulties when you go from environments with bright light into environments with dim lighting or no light at all
- peripheral vision loss, which involves vision loss along the top and bottom edges and left and right sides of your visual field
- visual field narrowing until you can only see what’s directly in front of you
- difficulty viewing the differences between colors
- trouble seeing in bright lights or becoming highly sensitive to bright lights
Experts currently do not know exactly how many people with RP will experience total blindness over time. But most people with RP will experience some form of partial vision loss, including a loss of night vision, sometimes called night blindness.
People with Usher syndrome can have a much higher risk of vision loss over time as a result of RP. Usher syndrome is another rare condition that can cause vision loss, hearing loss, and trouble with balance.
There’s currently no cure for RP. But you can treat the symptoms of RP to help you adjust to your gradual loss of vision or difficulty seeing in different levels of lighting.
Here are some treatments and lifestyle changes you can try to help reduce the impact that RP has on your daily life:
- Contact an eye care professional: An ophthalmologist or optometrist can help you decide what your prescription is and find glasses to help you see better.
- Take supplements: These may include vitamin A, fish oil, or lutein to help keep your eyes healthy — just be sure to ask a doctor or eye specialist whether these supplements may increase your risk for liver conditions or other side effects.
- Consume turmeric in your diet: Turmeric contains a substance called curcumin that can help slow the process of vision loss.
- Try eyewear with anti-glare coatings: Use glasses or sunglasses with any features that can help reduce the intensity of bright lights.
- Use adjustable lighting: Using light bulbs or light fixtures with adjustable or dimmable lighting controls can create a comfortable environment to see at home or at work.
- Use a magnifying device: This can help when reading small print.
- Increase screen text size and thickness: Your computer or mobile device settings and accessibility features can help you do this.
- Rearrange your furniture: Rearranging furniture at home and work can help you have clear, wide spaces to walk through and less risk of running into objects that can cause frustration or injury.
There is currently no cure for RP. But there are some promising clinical trials on the horizon looking closely at the genes that result in RP.
First, talk with a genetic counselor to have them analyze your genome so that you can better understand the mutations in your genes that cause RP. The affected genes influence possible future treatments. The genes that RP can affect include:
- RPE65: Researchers are currently studying a medication called voretigene neparvovec-rzyl (Luxturna) that can help stand in for the RPE65 gene that helps the retina sense light.
- RPGR: A doctor or surgeon removes the vitreous, jelly-like fluid in your eye, and injects new RPGR gene versions to improve your retina function.
- USH2A: People with Usher syndrome may benefit from a treatment called QR-421a, which researchers are currently studying. This injection can help improve your sight by replacing USH2A gene mutations that don’t produce the USH2A protein.
Another experimental treatment includes a pair of goggles that provide you with optogenetic therapy — along with an injection, this treatment helps replace the function of your retina in sensing light and seeing contrasts between objects.
RP is a genetic condition passed down in families. It has no clear cause, and medications, infections, or eye injuries trigger it.
Talk with a doctor if you’re concerned that you may be at risk of developing RP or if you notice symptoms of RP, such as vision loss or light sensitivity, that disrupt your daily life.
If you’re a parent who has RP, you may also want to meet with a genetic counselor, who can conduct tests to see if you can pass the genes to your children.