March 11, 2012

What Is Macular Degeneration?

Posted in: Daily Living, Eye Conditions, Health and Nutrition, Low Vision Rehabilitation, Research and Developments, Therapies, Treatments, and Procedures

by Dan Roberts
(Updated April 29, 2015)

(Reviewed by Martin A. Mainster, Ph.D., M.D., FRCOphth)

Audio/visual presentations on all related subjects

Recommended books:

The First Year: Age-Related Macular Degeneration: An Essential Guide for the Newly Diagnosed
by Daniel L. Roberts

Macular Degeneration: The Complete Guide to Saving and Maximizing Your Sight
by Lylas Mogk, M.D. and Marja Mogk, Ph.D.

Definition

Age-related macular degeneration (AMD) is a progressive disease of the retina wherein the light-sensing cells in the central area of vision (the macula) stop working and eventually die. The disease is thought to be caused by a combination of genetic and environmental factors, and it is most common in people who are age sixty and over. AMD is the leading cause of visual impairment in senior citizens. An estimated fifteen million people in the United States have it, and approximately two million new cases are diagnosed annually.

Other less common types of macular degeneration, which are hereditary and can affect younger people, are Best’s disease, Stargardt’s disease, and Sorsby’s disease. Collectively, these types are called juvenile macular degeneration.

Other diseases of the retina and extreme myopia (near-sightedness) can also result in degeneration of the macula. These conditions are not to be confused with AMD, but the end result, loss of central vision, can be the same.

Dry Macular Degeneration

Most cases of macular degeneration are the “dry,” or “atrophic”, form, distinguished by yellowish deposits of debris in the retina. Called “drusen,” the material comprising these deposits is usually carried away by the same blood vessels which bring nutrients to the retina. But for reasons yet unknown, this process is diminished in macular degeneration. Some of the potential causes being studied are inflammation, inadequate blood circulation in the retina, and premature aging of the sight cells due to genetic deficiencies. In addition, environmental, behavioral, and dietary factors are thought to contribute to the progress of the disease in those who are susceptible to it.

Dry AMD may occur in three stages in one or both eyes:

1. “Early.” Identified by several small drusen or a few medium-sized drusen. No obvious symptoms or vision loss.

2. “Intermediate.” Identified by many medium-sized drusen or one or more large, irregular-shaped drusen (called “soft” drusen). Symptoms may include a blurred or blind spot (“scotoma”) or distortion of images in the central field of vision. Also, more light and higher contrast may be needed for seeing.

3. “Advanced Dry.” Also called “Geographic Atrophy” (GA). Identified by drusen as described above, plus a breakdown of light-sensing “photoreceptor cells” and surrounding tissue in the macula. Scotomas may become larger and distortion more severe, and may eventually encompass the entire center field. Detail vision becomes impossible, causing the patient to rely upon the peripheral field for sight.

Treatments and cures for dry AMD and the juvenile forms of MD will likely come from the fields of genetic replacement therapy and stem cell transplantation. A good amount of research is being done in these areas, and we can reasonably expect a cure to be found within a decade.

There is yet no way to actually prevent the dry form of macular degeneration, but studies have shown that a person can take certain steps to help slow its progress:

More details on these recommendations may be found on this site or by contacting the organizations and foundations listed in the resource links. For continually updated information about latest research pertaining to dry AMD, see A Guide to Research in Dry AMD.

Wet Macular Degeneration

About 10-15% of macular degeneration cases are the “wet” (or “exudative”) form, in which newly-formed, immature blood vessels grow from the choroid (“choroidal neovascularization”) and leak into the spaces above and below the photoreceptor cells. This process can damage the photoreceptor cells and cause permanent central vision loss. For illustrations, definitions, and more information about the parts of the eye mentioned in this article, see Anatomy of the Eye.

Nearly 90% of wet MD cases are of the subfoveal type. This means that the offending vessels are beneath the fovea, or very center of the macula. Other types are called “juxtafoveal” and “extrafoveal.”

Two laser treatments have been shown to temporarily stop the leakage. One is laser photocoagulation, in which a hot laser beam cauterizes the vessel. The other is photodynamic therapy (PDT), which requires injection of a light-sensitive drug (Visudyne) into the patient’s veins. The light from a low-voltage laser is then used to coagulate the vessel.

Surgical procedures that have had some success are macular translocation, which involves rotating the macula to a healthier part of the retina, and submacular surgery, which involves removing the leaking membrane.

Pharmaceutical treatments are now leading the field in the treatment of wet AMD. Anti-angiogenic (aka anti-VEGF) drugs are now the most predominant treatment for stopping the development of blood vessels in the retina. The first to be FDA approved was Macugen in February of 2005. In June 2006, a more clinically effective drug, Lucentis, was approved. A third anti-angiogenic drug is Avastin. Similar to Lucentis and made by the same company for treatment of cancer, Avastin is being used off-label. And the most recently-approved drug is Eylea. For information about all of these, including others still in trials, read “Antiangiogenic Drugs Are Stopping Neovascularization in Wet Macular Degeneration“.

What To Expect

At its worst, macular degeneration will damage only central vision, which comes from the very center of the retina at the back of the eye. This area, called the macula, comprises less than 5% of the total retina, but it is responsible for about 35% of the visual field. That means an affected person will find it difficult or impossible to read, drive, or recognize faces. The peripheral vision, however, is left untouched, so macular degeneration does not, by itself, lead to total blindness. (See “Age-related Macular Degeneration Does Not Cause Blindness.”) Many affected people move about with no assistance at all and lead independent, productive lives. The most successful of them have also learned to use a wide variety of visual aids such as magnifiers, closed circuit TV readers, special bioptic glasses, etc., all of which are readily available from distributors listed in the Resources section of this site. It is highly recommended that a person with advanced macular degeneration enroll in a program of low vision rehabilitation. This program will provide evaluation of visual needs, assistance with environmental adaptations, and training in the use of appropriate low vision devices, computer software, and other technology. For more information on coping with visual impairment, see “Learning to Live With Low Vision” and “The T.A.S.K. of Living With Central Vision Loss” in the Audio/Visual Library. For extensive resources related to low vision rehabilitation, see the Resources section of this site.

The risk of developing macular degeneration depends upon a person’s age and whether soft drusen and/or changes in retinal pigment (color) are present. Research has shown that a person with these conditions who is 80 or older has a 42% chance of developing AMD within five years (Arch Ophthalmol 2003;121:519-26). A person who is less than 60 with a healthy retina has a 0.7% chance, and that risk gradually increases to 22.5% as the person reaches 80. Studies have shown that, for people with AMD in one eye, the chance of eventually developing the disease in the partner eye is between 38.7% and 55% (reported by the Rotterdam and Age-Related Eye Disease studies respectively.) For more recent results on risks of developing advanced AMD, see Findings Help Predict AMD Advancement.

Understanding and treatment of macular degeneration in all of its forms is progressing steadily. Meanwhile, education helps people to become discriminating consumers of therapies and services. It also keeps them aware of progress in the continuing battle to slow macular degeneration and improve the quality of life of those who have it.

Recommended additional reading:

Saving Lives: The Impact of Vision Loss in Later Life

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