Macular Degeneration

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What is Macular Degeneration?Macular degeneration is the name given to the group of degenerative retinal diseases that cause progressive loss of central vision, leaving only peripheral or side vision intact. It is most commonly related to aging, hence the name, age-related macular degeneration (ARMD or AMD).

Less commonly, degeneration of the macula occurs in younger people. This can be due to inherited conditions called retinal or macular dystrophy, short-sightedness called myopic macular degeneration, and other rarer disease processes such as choroidal polyps, angioid streaks, central serous retinopathy, eye inflammation and medication toxicities.

Macular degeneration is usually progressive, meaning that it worsens with time. Although it never causes complete blindness, it affects central vision, impairing a person’s ability to do many daily tasks, such as reading, recognising faces, driving and shopping. The ability to recognise colours and contrasts is also affected.

CORNEA

IRIS LENS

LIGHT

RETINA

MACULA

OPTIC NERVE

BLOOD VESSELS

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Early stage AMDThis is the most common form and is present in one in 20 people aged 50 years or over. Typically there is no noticeable vision loss but some people may gradually become aware of increasing difficulty with reading in dim light and delayed adaptation to seeing in the dark. As the disease progresses, mild distortion or a blurry patch in the central vision may develop.

In this early stage, there is accumulation of debris in the retina at the level above, within or below the retinal pigment epithelium (RPE), a specialised layer of cells that nourishes the nerve cells in the retina. Over a period of many years, this debris, also called drusen, accumulates and causes RPE damage. As the disease progresses to the late stage, nerve cells in the retina will become injured or die resulting in loss of vision.

Normal OCT scan

Drusen

OCT scan with Drusen

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Late stage AMDThis is the stage at which most people with AMD will present to an eye doctor because of vision loss due to nerve cell injury or death within the retina. There are two types of late stage AMD: geographic atrophy and neovascular (or new vessel growth, also commonly known as the wet form).

In some people, increasing difficulty with reading or face recognition may be the first symptom of geographic atrophy. In this type of late AMD, there is progressive, patchy loss of nerve cells and RPE in the retina resulting in the formation of several blind spots in the vision. This affects recognition of all the letters in a word or details in a face. Over a period of several years, the ability to read may be completely lost due to blurriness in the central vision. This type of late AMD may be seen in up to one per cent of those aged 55 years or over.

Independent of geographic atrophy formation, some people may also develop abnormal new blood vessels from beneath the retina. Upon entry into the retina, these new blood vessels often grow wildly, and may bleed or leak fluid under the retina and hence it is often referred to as the “wet”

form of AMD. If untreated, this causes scarring of the retina and severe loss of vision over a period of months. This type of late AMD may also be seen in up to one per cent of those aged 55 years or over.

People with AMD may develop new blood vessels followed by onset of geographic atrophy or geographic atrophy first followed by new blood vessels formation. Geographic atrophy and new blood vessels can also each occur alone without the other.

Late stage AMD

Geographic atrophy

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Macular degeneration not due to AMD There are other forms of macular degeneration that are not related to aging. These can occur at a younger age and they are due to a variety of causes such as short-sightedness (also called myopic macular degeneration), leakiness of blood vessels (also called central serous retinopathy), balloon-like bulge in the blood vessel wall (also called choroidal polyps), genetic eye disease and medication side effects.

Many of these conditions can also lead to central vision loss through the development of geographic atrophy or new blood vessel formation. It is important that special tests are performed to distinguish these conditions from AMD because their treatments may be very different.

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What causes Macular Degeneration?The causes and mechanisms of macular degeneration are not fully understood. The major risk factor for AMD is increasing age. This disease can cluster in certain families, suggesting that some persons are more genetically susceptible to the disorder than others. Cigarette smoking is known to increase the risk of AMD. Racial, dietary, hormonal and vascular factors appear also to play a role in an individual’s risk of developing AMD. Choroidal polyps cause 10 to 20 per cent of the abnormal new blood vessels seen in Caucasians but up to 50 per cent of the new blood vessels seen in the Asian population. You cannot change your parents, your race, your sex (AMD is more common in females) or your age, but you can reduce your risk of developing AMD.

Things you can do:

• Stop smoking

• Eat a diet rich in fresh fruit and dark green leafy vegetables

• In consultation with your doctor, supplement your diet with vitamins, minerals and antioxidants

• Check your vision regularly

It is important to realise that using the eyes for reading and other daily tasks will not cause or worsen macular degeneration.

Risk factors for other types of macular degeneration vary significantly. Increasing short-sightedness is associated with increasing risk of developing myopic macular degeneration. Central serous retinopathy is more likely to develop in people who are long-sighted. Retinal dystrophy and angioid streaks are usually inherited from parents.

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What are the symptoms of Macular Degeneration?The symptoms are visual and there is no associated pain or discomfort. Many patients note difficulty with reading as words become blurred or crowded. There may be a blurred, dark, or empty spot in the central vision, similar to the after-effect of a flashbulb. Distortion is a frequent and notable symptom. Straight lines such as door frames or telephone poles may appear bent or wavy. One eye may be affected more than the other. Blurred vision in one eye alone sometimes goes unnoticed for a long period of time because the other seeing eye compensates for the vision deficit.

Macular degeneration does not lead to total blindness even if both eyes are affected. The peripheral, or side, vision is unaffected by the condition and almost all patients can see well enough to take care of themselves and continue activities that do not require detailed vision.

This image shows distortion

This image shows distortion of the Amsler Grid

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Optical coherence tomography (OCT)An optical coherence tomography (OCT) scan captures a detailed picture of the retina similar to ultrasound. It is completely non-invasive and provides a very high resolution image of the various layers of nerve cells in your macula. This scan will allow your ophthalmologist to compare measurements over time and assess how the treatment is working and whether more frequent administration is required. It may even detect fluid build-up due to new blood vessels before you become aware of vision impairment.

Fundus autofluorescence (FAF)Fundus autofluorescence (FAF) imaging is a non-invasive photograph of the retina that allows doctors to see those RPE cells under metabolic stress.

This test is critical in differentiating yellow flecks due to genetic retinal disease from drusen in AMD. Repeated FAF photography allows the doctor to measure how fast cells are deteriorating because areas of dead RPE cells are easily seen as dark patches in the macula on FAF photography.

Fundus fluorescein and indocyanine green angiography (FFA & ICGA)Angiography enables doctors to study blood vessel structures in the retina to assist with accurate diagnosis. Fluorescein and indocyanine green are commonly used dyes that are injected into the vein of the arm which then circulate to the eye to highlight blood vessels within the retina during angiography. Different dyes are required to see different layers of circulation in the back of the eye and to distinguish between choroidal polyps, central serous retinopathy and AMD-related new blood vessels.

This is an OCT scan showing a normal eye.

This OCT scan shows late stage AMD. See the deviation of the retina

in the macular region.

Wet AMD

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What is the treatment for Macular Degeneration?There is currently no cure for macular degeneration. Treatment efforts are directed at maintaining useful central vision for as long as possible. The treatment varies depending on the cause and stage of macular degeneration. Many factors will be considered by your ophthalmologist when determining the best treatment for you.

Early stage AMDAll individuals with early signs of AMD must stop smoking. Recent studies suggest that some vitamins, minerals and antioxidants may slow the progression of early AMD into late AMD. Your ophthalmologist can advise whether these preparations may be helpful and discuss new clinical trials in novel treatments aiming to reduce the harmful effects of drusen. Periodic examinations to monitor you for signs of progression may be recommended.

Late stage AMDThere have been major advances in the treatment of new blood vessels in the late stage of AMD that have enabled many patients with this type of complication to regain lost vision. The majority of patients with active new blood vessels can now be offered treatment with drugs that are injected into the vitreous cavity of the eye. These drugs reduce leakage from the abnormal blood vessels under the retina when they are administered regularly at monthly intervals. Once OCT scans show absence of fluid in the macula, the time between retreatments may gradually extend by one to two weeks until the 12 week interval is reached. In many patients, lifelong treatment every 12 weeks may be sufficient to keep the vision stable. In a significant proportion of patients, a prolonged course of four to six weekly retreatment regimens may be required because retinal fluid

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returns or vision loss occurs when the retreatment interval is extended. Your doctor will discuss possible alternative treatments in such situations with you. There are also new drugs being developed for treating new blood vessels that may have stronger action or longer-lasting effects. If you have just been diagnosed with this form of late AMD, you should ask your doctor for information on new trials. There are usually one or more treatment trials at the Lions Eye Institute that are recruiting patients with abnormal macular blood vessels who have not been treated before.

Geographic atrophy that occurs as a complication of AMD is currently untreatable. However, there are many experimental treatments being tested and you should ask your doctor about new trials that may be enrolling patients with expanding geographic atrophy as documented on FAF photography. There are usually one or more treatment trials at the Lions Eye Institute recruiting patients with geographic atrophy.

The Lions Eye Institute was one of many eye research centres worldwide to investigate the effects of the drug ranibizumab (Lucentis®) and aflibercept (Eylea®) in treating patients with wet AMD. The results of large,

multi-centre randomised controlled trials demonstrated that up to half of all patients treated with intravitreal injections achieve improvement in vision. Lucentis® and Eylea® treatment achieved similar results in vision gain.

Bevacizumab (Avastin®) was developed as an intravenous infusion to treat patients with advanced bowel cancer. Its potential for treating patients with eye diseases was recognised early. It

This is FAF photo showing early stage AMD

This FAF photo shows geography atrophy from AMD

This FAF photo shows more cells have died after 2 years

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has been used to successfully treat patients with abnormal new vessels in the macula and other eye diseases by injection of a far smaller dose into the vitreous cavity of the eye. However, several studies have demonstrated that Lucentis® is superior to Avastin® in AMD so that Lucentis® and Eylea® are now accepted worldwide as the gold standard treatments for patients with wet form of late AMD, and their use has given patients with this disease real hope of retained or even improved vision.

In patients who have new blood vessels due to myopic macular degeneration, retinal inflammation and other similar conditions not classified as AMD, Avastin remains the only available drug to treat abnormal blood vessels. Myopic macular degeneration complicated by new blood vessels tends to respond differently compared with AMD and hence monthly monitoring and a “treat-as-required” regimen may be more suitable than an “injection at every visit” approach.

A major drawback of intravitreal drug treatment is the need for continuous multiple injections in order to maintain vision. One of the many areas of active research at the Institute is our on-going clinical audit of intravitreal therapy that will help us to predict the minimum

injection frequency required for an individual eye to achieve the best vision outcome. Most patients with abnormal blood vessels due to AMD are judged to be suitable for treatment and they will receive an initial loading dose of three or more injections, once per month. The retreatment interval will be determined based on assessment of fluid status on the OCT scan by your ophthalmologist at each treatment visit. If there is no fluid, your doctor may decide to extend the interval by one to two weeks. If there is recurrence of fluid, your doctor may shorten the interval by one to two weeks or go back to a monthly loading regimen to control the leakage aggressively. A significant proportion of patients

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may not achieve a fluid-free macula on OCT even with 4-weekly injections. It is important that you discuss with your ophthalmologist alternative treatment strategies if this situation persists into the second year of treatment. One option for treatment resistance or failure to extend retreatment interval is to switch from Eylea® to Lucentis® or vice versa. In some patients, switching drugs may be the solution to successfully extending the retreatment interval.

In situations where choroidal polyps or central serous retinopathy are suspected based on FAF photography or OCT scans, further investigation using indocyanine green (ICG) angiography may be required to confirm these diagnoses. Both choroidal polyps and central serous retinopathy can mimic the wet form of AMD but, unlike AMD, they can be effectively treated by photodynamic therapy (PDT) with verteporfin, or sometimes laser photocoagulation alone. Both treatments are performed as an outpatient procedure at the Lions Eye Institute. Neither treatment is suitable for every patient with choroidal polyps or central serous retinopathy, and your retinal specialist will determine if any treatment can be recommended after examination and review of the OCT scan, FAF

photograph and ICG angiography. Neither treatment is a cure, and both aim to preserve central vision for as long as possible.

In PDT, a drug called verteporfin (Visudyne®) is infused into the patient’s bloodstream. The drug has been designed to selectively target the leaky abnormal blood vessels under the macula. A non-thermal laser (a laser that does not burn the retina) is directed at the leaky blood vessels and activates the drug. This in turn seals the leaky vessels without causing damage to other parts of the retina. Because of its selectivity, PDT with verteporfin is typically used when the leaky blood vessel is underneath the centre of the macula. PDT may become a course of therapy, and sometimes several treatments are needed to keep the leaking blood vessels closed and stop the progression of choroidal polyps or recurrence of central serous retinopathy. Close follow-up evaluations with your retinal specialist are needed to determine if retreatment is required. Sometimes, reactivation of the new blood vessels may respond to another course of injections and therefore combining PDT with injections may be necessary to achieve long-term control of the leakage particularly in choroidal polyps.

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Future treatments for Macular DegenerationResearchers at the Lions Eye Institute continue to seek new and more effective treatments for AMD and other forms of macular degeneration. There are usually one or more clinical trials at any time in the Institute recruiting patients with abnormal new blood vessels or geographic atrophy due to AMD. Several retinal specialists at the Institute are currently involved in these large-scale clinical trials, which are being conducted in multiple centres worldwide to investigate the effectiveness of new drugs that protect cells from dying, dampen inflammatory response and block growth factors that promote blood vessel growth. The Institute’s Ocular Tissue Engineering Laboratory is also developing cell transplantation techniques that may ultimately provide a cure as this approach uses stem cells to regenerate retinal cells in the macula of patients with AMD. Genetic studies are also being conducted at the Institute to identify the genes that may predispose individuals to early onset drusen (under the age of 50 years) and myopic macular degeneration. Large epidemiological studies are being conducted to examine environmental factors that may reduce the risk of myopia and hence prevent myopic macular degeneration.

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Other resourcesIt is important to realise that you are not alone. Over 200,000 Australians suffer vision loss from AMD either due to geographic atrophy or abnormal new blood vessels. Even with severe vision loss, most people are able to continue leading independent lives with appropriate medical and social support. Patient support groups can provide further information and resources to assist with the tasks of daily living. Your retinal specialist can help direct you toward these resources.

simple method for patients to monitor their own central vision. An Amsler Grid is provided in this brochure for your convenience. Patients should wear their normal reading glasses and hold the Amsler Grid at normal reading distance in good light. Each eye should be examined in turn by covering the opposite eye. The patient should attempt to look at the central dot of the grid to determine whether all the surrounding lines are straight and regular or whether any areas of the grid are blurred, distorted, or missing. Any change in the appearance of the grid should be reported to your ophthalmologist as soon as possible.

Self Monitoring with the Amsler GridIt is important for patients with AMD and other types of macular degeneration to regularly monitor central vision in each eye. The object of self monitoring is to detect any change in vision as early as possible, since this may signify progression to the late stage of AMD. Because treatment of abnormal blood vessels is generally more successful if given earlier in the course of the disease, it is essential that patients attend for examination as soon as possible after noticing any significant change in vision. Self-monitoring can be as simple as covering each eye in turn while looking at a familiar object, or TV screen. The Amsler Grid provides a

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4. If you see wavy, broken or distorted lines, or blurred or missing areas of vision, you may be displaying symptoms of AMD and should contact your eye care provider immediately.

Amsler Grid Eye Exam

1. Do not remove glasses or contacts you normally wear for reading.

2. Stand approximately 13in (33cm) from the grid in a well-lit room.

3. Cover one eye with your hand and focus on the centre dot with your uncovered eye. Repeat with the other eye.

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Will glasses help?Patients will gain some benefit from wearing the correct glasses. However, a change in prescription is unlikely to significantly improve vision in most people. In late stage AMD special magnifying spectacles and various optical instruments are available to help people continue their usual activities. Known as low-vision aids, these devices enable patients to use their remaining vision to its fullest.

Rehabilitation programs and other community services are also available through VisAbility (http://www.visability.com.au/), formerly known as the Association for the Blind of WA. A referral can be arranged through your retinal specialist if necessary.

Macular Degeneration at the Lions Eye InstituteThe cause of macular degeneration is not yet understood and therefore current treatment cannot prevent or cure the condition. The key to future treatment will be intervention at a much earlier stage, prior to irreversible retinal damage.

Our Ocular Tissue Engineering Laboratory at the Lions Eye Institute is developing methods to grow the retinal pigment epithelium (RPE) from patients’ own cells. The RPE is a layer of cells underlying the retina that act as a metabolic support system for the photoreceptors, which are the light-sensitive cells of the retina. Enzymes in the RPE digest large amounts of debris from the photoreceptors and provide nutrients. If the RPE does not function properly the photoreceptors degenerate and vision is lost. In AMD, RPE and its underlying supporting membrane are damaged early in the disease process. Loss of RPE leads to geographic atrophy and abnormal new vessel formation, i.e. the late stages of AMD.

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Researchers at the Lions Eye Institute theorise that replacement of damaged RPE by stem cell-derived RPE may be a curative approach to treat AMD. However, reversal of vision loss is only possible if photoreceptor cells and underlying choroidal blood vessels are restored as well. Using sophisticated molecular biology techniques (cellular reprogramming), they are developing optimal conditions for growing RPE from patient-derived stem cells. Success in this approach will offer patients their own RPE cell to replace the damaged ones in their eye.

The Lions Eye Institute is the leading ophthalmic research institution in the southern hemisphere and is amongst the best in the world. There are many dedicated research teams at work investigating macular degeneration and many other unsolved causes of blindness. Your ophthalmologist is involved with many of these teams, ensuring that you receive the very best and most up-to-date treatment that can be provided anywhere in the world. Additionally, the Institute is involved in community screening projects through the Lions Save-Sight Foundation to detect silent causes of visual loss such as glaucoma, diabetic retinopathy and trachoma.

It takes dedicated research program to cure blindnessAssessment and management of macular degeneration is a complex process that requires specialised training in retina and cutting edge research expertise. Nonetheless, the most challenging obstacle that the Lions Eye Institute faces is securing adequate funds to carry on its sight-saving research programs. The Institute relies upon the financial support of the community for its continued existence and impact on improving quality of life through improving vision. Join us in the fight against blindness. Your tax deductible* donation is therefore a welcome and important contribution to an enterprise from which you or your loved-ones may one day benefit.

Please telephone (08) 9381 0777 for more information

*Donations $2 or more are tax deductible.

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Version 3. Date Revised: January 2016