Maculopathy, also known as macular degeneration, is a disease that affects the back of the retina, called the macula. The macula controls the ability to read, see fine detail in objects and recognize faces and colors. Maculopathy is a progressive disease and can lead to central vision loss, usually in both eyes.
People with maculopathy don’t go completely blind, but they can lose central vision. Together, central vision and peripheral vision help people understand what they are looking at and where it is. Central vision helps people see details like individual words when reading, while peripheral vision guides them to the next word in the sentence.
People with central vision loss have difficulty seeing details or colors of an object or person they are looking at, but they can see everything around it. For example, people with AMD looking at a clock with hands might not see the hands, but they will still see the numbers, according to the American Academy of Ophthalmology.
If central vision loss becomes severe — 20/200 or worse with corrective lenses — people may be classified as “legally blind.”
There are several types of maculopathy. It usually affects people ages 50 or older and gets progressively worse with age. But some people may be born with it or develop it because of a disease — such as diabetes.
Some prescription drugs may cause macular damage — these include Plaquenil (hydroxychloroquine), Elmiron (pentosan polysulfate sodium) or corticosteroids including prednisone.
There are no cures for maculopathy, but treatment may slow progression for some types of disease.
Age-Related Macular Degeneration (AMD)
The most common type of maculopathy is age-related macular degeneration (AMD), also called macular degeneration. It affects people ages 50 and over and is the leading cause of vision loss in the United States. It affects more than 10 million people, according to the American Macular Degeneration Foundation.
Early stage AMD often has mild or unnoticeable symptoms. In late stage AMD, cells in the retina begin to die and affect the macula. There are two types of late stage AMD — dry AMD and wet AMD.
Dry AMD is the most common and it affects about 8 out of 10 people with macular degeneration, according to the American Academy of Ophthalmology. This occurs when the macula gets thinner and clumps of protein called drusen grow on it.
People with dry AMD slowly lose central vision.
Wet AMD is rarer but is more serious than dry AMD. This condition occurs when abnormal blood vessels grow in the retina and leak blood or other fluids. This leads to macular damage. People with wet AMD lose vision more quickly than people with dry AMD.
The first sign of macular degeneration is a blurry area near the center of vision. It gets progressively worse over time and may lead to central vision loss in one or both eyes. When straight lines start to look wavy, this is an example of severe, late AMD. People who experience this should contact their medical providers right away.
- Blurry spots in center of vision
- Blank or dark spots in center of vision
- Difficulty reading
- Loss of clear color vision
- Objects appear distorted
- Straight lines that appear crooked or wavy
Causes and Risk Factors
Deterioration of the macula causes macular degeneration. Researchers believe oxidative stress and inflammation in the eye may contribute to deterioration, according to the Bright Focus Foundation. Oxidative stress comes from a few things, including bright light and a poor diet.
- Are obese
- Have high blood pressure
- Are over 50 years old
- Have a family history of AMD
- Eat a lot of saturated fat
- Have heart disease
- Have high cholesterol
- Are Caucasian (white)
Early AMD currently has no treatments. Typically, eye doctors will just monitor disease for any changes with regular eye exams. But following a healthy diet, quitting smoking and getting regular exercise may help slow down damage, according to the National Eye Institute.
People with intermediate or late AMD may benefit from taking special vitamin and mineral supplements called AREDS and AREDS2 supplements. These supplements contain vitamin C, vitamin E, beta-carotene, copper, lutein, zeaxanthin and zinc. These vitamins don’t cure, reverse or prevent AMD, but they may stop disease progression.
According to NEI, research shows that these supplements may help people with intermediate AMD in one or both eyes or people with late AMD in one eye. Ask your eye doctor about these supplements and if they are right for you.
Treatment for Wet AMD
While there are no treatments for early-stage or dry AMD, people with wet AMD have two options to slow down or stop vision loss: anti-VEGF injections and photodynamic therapy, or PDT.
Anti-VEGF injections contain medicines that help stop blood vessels in the eye from leaking. The eye doctor will numb the eye, sterilize it and inject medication. After the procedure, patients may need antibiotic eye drops to prevent infection. Because these injections only work for a short time, people often need to keep coming back at least once a month at the beginning of treatment.
Research is underway to use gene therapy to stimulate the body to produce its own anti-VEGF compounds. Several gene therapy medications are under investigation for both dry and wet types of AMD.
Photodynamic therapy uses a special laser to break down extra blood vessels in the eye. Doctors will inject the patient’s arm with a medicine called verteporfin. When the doctor shines a cold laser into the eye, the medicine reacts to the light and destroys extra blood vessels.
Patients will receive an eye numbing medicine and a special contact lens to help guide the laser during treatment.
Hereditary maculopathy, also called juvenile macular degeneration, is a type of macula disease that is inherited. Symptoms often start earlier in life.
Examples of inherited maculopathies include Stargardt macular degeneration, vitelliform macular dystrophy (also called Best disease) and bull’s eye maculopathy.
Stargardt Macular Degeneration
Stargardt macular degeneration is the most common type of juvenile macular degeneration. It occurs in about 1 in 8,000 to 10,000 people, according to U.S. National Library of Medicine.
Like AMD, Stargardt macular degeneration causes progressive vision loss. In this condition, a fatty yellow pigment called lipofuscin builds up in cells in the macula. Over time, this damages the cells. This type of degeneration is caused by mutations in two genes — ABCA4 and ELOVL4 — that make proteins in light-sensing cells.
Symptoms occur in late childhood and early adulthood and include central vision loss and problems with low light and night vision. People with this disease may also lose peripheral vision as they get older.
Currently there is no treatment for Stargardt macular degeneration, but wearing dark glasses and hats when out in bright light reduces the buildup of lipofuscin.
Vitelliform Macular Dystrophy (Best Disease)
Vitelliform macular dystrophy is similar to Stargardt macular degeneration but is much rarer. Researchers have found it occurs in about 1 in 16,500 to 1 in 21,000.
This condition causes lipofuscin to build up in the macula, and it’s caused by mutations in two genes: BEST1 or PRPH2.
Symptoms usually occur within the first 20 years of life, but some people might not have symptoms until they are older. These include blurry and distorted vision. Best disease doesn’t usually affect night vision.
Vitelliform macular dystrophy doesn’t have any treatments, but some types may respond to anti-VEGF injections and photodynamic therapy (PDT).
Bull’s Eye Maculopathy
Bull’s eye maculopathy isn’t a diagnosis. It’s a name given to the visual representation of the damaged retina in people with different types of maculopathy.
The “bull’s eye” is a ring of pale-looking damage around a darker area of the macula. Circular bands of different shades of pink and orange may also appear.
Diabetic maculopathy is a complication of diabetic retinopathy, a condition that causes vision loss and blindness in people with diabetes. Over time, having too much sugar in the blood damages the retina and affects vision.
The most dominant sign of diabetic maculopathy is diabetic macular edema, or DME. DME occurs when blood vessels in the retina leak, causing swelling in the macula. The fluid causes blurry and spotty vision. Some people with later stages of disease may see streaks that look like cobwebs.
Treatment for diabetic maculopathy and retinopathy includes anti-VEGF drugs, corticosteroids, laser treatment to reduce swelling and eye surgery to remove scarring in the eye.
The most common eye exam is a dilated eye exam. The doctor will use drops to dilate the pupil and study the retina for signs of disease. But the doctor may use other types of tests to check for macular degeneration. Some of these tests are listed below.
- Doctors use a special blue light that causes certain parts of the eye to glow without dye. They can use this technique to check retina health.
- Fundus Photography
- The doctor shines a bright light into the eye after dilating the pupil. He or she uses a special camera to take pictures of the back of the eye to look for disease in the macula, retina and optic nerve.
- Optical Coherence Tomography (OCT)
- OCT is a noninvasive way to image the retina. The doctor can use this to measure the thickness of different layers of the retina. A thick retina could lead to a diagnosis of dry AMD.
Amsler Grid for At-Home Testing
The Amsler grid can help patients keep track of their visual health at home. The Amsler grid is a simple square grid with a dot in the middle. Using it once a day can help determine if you have problem spots in you field of vision.
Only an eye doctor can give you a diagnosis, and this test is just for reference.
- Wear your glasses that you normally use to read.
- In good light, hold the grid 12 to 15 inches away.
- Cover one of your eyes.
- Keep your uncovered eye focused directly at the center dot.
- Without moving your focus from the dot, see if the lines on the grid are wavy, blurry, dark or blank.
- Repeat the steps with your other eye.
Seeing blank spots, wavy lines, blurry lines or darker areas of the grid may mean you need treatment for AMD. Contact your eye doctor right away.
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Elmiron and Pigmentary Maculopathy
Pigmentary maculopathy is a unique type of maculopathy linked to the interstitial cystitis drug, Elmiron (pentosan polysulfate sodium). Some people who used the drug long-term for treating IC and bladder pain developed pigmented deposits that resemble little specks in the macula, according to a 2018 study by Drs. William A Pearce, Rui Chen and Nieraj Jain in Ophthalmology.
Since the study by Pearce and colleagues, a handful of other studies have provided evidence that the IC drug increases the risk of developing this type of maculopathy.
People in these studies complained of several symptoms including dim vision, difficulty reading, difficulty adjusting to the dark or low lighting and areas of vision loss. In some cases, vision loss is permanent.
Some people filed Elmiron lawsuits against the drug’s manufacturer claiming they were never warned about the risk of vision loss.
The malaria and lupus drug Plaquenil (hydroxychloroquine) can cause macula damage that resembles a bull’s eye when examined by doctors. This side effect is rare, but some people have irreversible vision damage that progresses even after stopping the drug.
Early on, most people don’t notice symptoms. But early signs of Plaquenil maculopathy include trouble reading and diminished color vision. Over time, the damage becomes more severe and leads to loss of peripheral vision, night vision and vision acuity.
Living with Maculopathy
Because of vision distortion, loss of central vision and blind spots, people with maculopathy or macular degeneration face challenges doing daily tasks. Maculopathy is irreversible. But people with the disease can still use their peripheral vision and adapt their surroundings to live productive and safe lives.
- Ask your eye doctor for special low vision glasses and tips for using lighting and electronic devices to help make up for vision loss.
- Mark floors with contrasting, bright-colored tape to make changes in floor levels or steps easier to see.
- Use grip bars in the shower.
- Use night-lights.
- Use bright, warm-colored LED lighting.
- In the kitchen, mark stove dials with bright tape or use glue to make bumps to indicate specific oven temperature settings.
- Use large print for medications. Use bright lighting and magnifying glasses to read drug labels.
- High-powered reading glasses, hand-held magnifiers and closed captions on TVs may help reading.
- Try turning your head or eyes to the side when viewing an object to help bypass central vision loss.
- Try using large print books, audio books and voice recognition software on computers and cell phones.
- Emotional side effects of AMD including helplessness, depression and anger are common when dealing with vision loss. Take care of your mental health as well as your physical health.
- You may need to learn new skills to adjust to living with low vision. Try to stay positive and learn about resources and rehabilitation services that can help you.
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