Maculopathy

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 allows them to find objects that are not in their central vision and navigate through the world.

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.

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 central 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 central vision. There are two types of late stage AMD — atrophic dry AMD and wet AMD.

Dry 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

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.

Symptoms

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 significant central vision loss in one or both eyes. People most often experience this as difficulty reading, even with bright light and updated glasses. This difficulty reading may seem worse than the level of their vision overall.

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, a poor diet and smoking.

Treatments

Early AMD currently has no treatments. Typically, eye doctors will 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.

Supplements

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 may reduce 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.

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 your central vision vision.

Only an eye doctor can give you a diagnosis, and this test is just for reference.

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.

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.

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.