Macular Degeneration What is Macular Degeneration?

Macular degeneration is the name BLOOD given to the group of degenerative LIGHT VESSELS MACULA retinal diseases that cause progressive loss of central vision, leaving only CORNEA 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 IRIS LENS RETINA OPTIC medication toxicities. NERVE 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.

2 MACULAR DEGENERATION Early stage AMD This 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. Drusen 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 Normal OCT scan 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 OCT scan with Drusen or die resulting in loss of vision.

3 Late stage AMD This is the stage at which most people form of AMD. If untreated, this causes with AMD will present to an eye doctor scarring of the retina and severe loss because of vision loss due to nerve of vision over a period of months. This cell injury or death within the retina. type of late AMD may also be seen in There are two types of late stage AMD: up to one per cent of those aged 55 geographic atrophy and neovascular years or over. (or new vessel growth, also commonly People with AMD may develop new known as the wet form). blood vessels followed by onset of In some people, increasing difficulty geographic atrophy or geographic with reading or face recognition may atrophy first followed by new blood be the first symptom of geographic vessels formation. Geographic atrophy atrophy. In this type of late AMD, there and new blood vessels can also each is progressive, patchy loss of nerve occur alone without the other. 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 Geographic atrophy 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 Late stage AMD it is often referred to as the “wet”

4 MACULAR DEGENERATION 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.

5 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.

6 MACULAR DEGENERATION 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 This image shows 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 This image shows distortion of the and almost all patients can see well Amsler Grid enough to take care of themselves and continue activities that do not require detailed vision.

7 Optical coherence tomography Fundus fluorescein and (OCT) indocyanine green angiography An optical coherence tomography (FFA & ICGA) (OCT) scan captures a detailed Angiography enables doctors to study picture of the retina similar to blood vessel structures in the retina ultrasound. It is completely non- to assist with accurate diagnosis. invasive and provides a very high Fluorescein and indocyanine green resolution image of the various layers are commonly used dyes that are of nerve cells in your macula. This injected into the vein of the arm which scan will allow your ophthalmologist then circulate to the eye to highlight to compare measurements over time blood vessels within the retina during and assess how the treatment is angiography. Different dyes are working and whether more frequent required to see different layers of administration is required. It may even circulation in the back of the eye and to detect fluid build-up due to new blood distinguish between choroidal polyps, vessels before you become aware of central serous retinopathy and AMD- vision impairment. related new blood vessels. 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 is an OCT scan This test is critical in differentiating showing a normal eye. 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 This OCT scan shows late stage on FAF photography. AMD. See the deviation of the retina in the macular region.

8 MACULAR DEGENERATION

Wet AMD 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 AMD Late stage AMD All individuals with early signs of There have been major advances in AMD must stop smoking. Recent the treatment of new blood vessels studies suggest that some vitamins, in the late stage of AMD that have minerals and antioxidants may slow enabled many patients with this type the progression of early AMD into late of complication to regain lost vision. AMD. Your ophthalmologist can advise The majority of patients with active whether these preparations may be new blood vessels can now be offered helpful and discuss new clinical trials treatment with drugs that are injected in novel treatments aiming to reduce into the vitreous cavity of the eye. the harmful effects of drusen. Periodic These drugs reduce leakage from the examinations to monitor you for signs abnormal blood vessels under the of progression may be recommended. 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

9 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 This is FAF photo showing early vessels that may have stronger action stage AMD 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 This FAF photo shows geography macular blood vessels who have not atrophy from AMD 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 This FAF photo shows more cells have new trials that may be enrolling died after 2 years patients with expanding geographic atrophy as documented on FAF multi-centre randomised controlled photography. There are usually one trials demonstrated that up to half of or more treatment trials at the Lions all patients treated with intravitreal Eye Institute recruiting patients with injections achieve improvement in ® ® geographic atrophy. vision. Lucentis and Eylea treatment achieved similar results in vision gain. The Lions Eye Institute was one of ® many eye research centres worldwide Bevacizumab (Avastin ) was developed to investigate the effects of the as an intravenous infusion to treat drug ranibizumab (Lucentis®) and patients with advanced bowel cancer. aflibercept (Eylea®) in treating patients Its potential for treating patients with with wet AMD. The results of large, eye diseases was recognised early. It

10 MACULAR DEGENERATION 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 injection frequency required for an degeneration, retinal inflammation and individual eye to achieve the best vision other similar conditions not classified outcome. Most patients with abnormal as AMD, Avastin remains the only blood vessels due to AMD are judged available drug to treat abnormal blood to be suitable for treatment and they vessels. Myopic macular degeneration will receive an initial loading dose of complicated by new blood vessels three or more injections, once per tends to respond differently compared month. The retreatment interval will be with AMD and hence monthly determined based on assessment of monitoring and a “treat-as-required” fluid status on the OCT scan by your regimen may be more suitable than an ophthalmologist at each treatment visit. “injection at every visit” approach. If there is no fluid, your doctor may decide to extend the interval by one A major drawback of intravitreal drug to two weeks. If there is recurrence treatment is the need for continuous of fluid, your doctor may shorten the multiple injections in order to maintain interval by one to two weeks or go vision. One of the many areas of active back to a monthly loading regimen research at the Institute is our on-going to control the leakage aggressively. clinical audit of intravitreal therapy that A significant proportion of patients will help us to predict the minimum

11 may not achieve a fluid-free macula on photograph and ICG angiography. OCT even with 4-weekly injections. It Neither treatment is a cure, and both is important that you discuss with your aim to preserve central vision for as ophthalmologist alternative treatment long as possible. strategies if this situation persists into In PDT, a drug called verteporfin the second year of treatment. One (Visudyne®) is infused into the patient’s option for treatment resistance or failure bloodstream. The drug has been to extend retreatment interval is to ® ® designed to selectively target the switch from Eylea to Lucentis or vice leaky abnormal blood vessels under versa. In some patients, switching drugs the macula. A non-thermal laser (a may be the solution to successfully laser that does not burn the retina) extending the retreatment interval. is directed at the leaky blood vessels In situations where choroidal polyps and activates the drug. This in turn or central serous retinopathy are seals the leaky vessels without causing suspected based on FAF photography damage to other parts of the retina. or OCT scans, further investigation Because of its selectivity, PDT with using indocyanine green (ICG) verteporfin is typically used when the angiography may be required to leaky blood vessel is underneath the confirm these diagnoses. Both centre of the macula. PDT may become choroidal polyps and central serous a course of therapy, and sometimes retinopathy can mimic the wet form several treatments are needed to of AMD but, unlike AMD, they can be keep the leaking blood vessels closed effectively treated by photodynamic and stop the progression of choroidal therapy (PDT) with verteporfin, or polyps or recurrence of central serous sometimes laser photocoagulation retinopathy. Close follow-up evaluations alone. Both treatments are performed with your retinal specialist are needed as an outpatient procedure at the to determine if retreatment is required. Lions Eye Institute. Neither treatment Sometimes, reactivation of the new is suitable for every patient with blood vessels may respond to another choroidal polyps or central serous course of injections and therefore retinopathy, and your retinal specialist combining PDT with injections may will determine if any treatment can be necessary to achieve long-term be recommended after examination control of the leakage particularly in and review of the OCT scan, FAF choroidal polyps.

12 MACULAR DEGENERATION Future treatments for Macular Degeneration Researchers 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.

13 Other resources It 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.

Self Monitoring with the Amsler Grid It is important for patients with simple method for patients to monitor AMD and other types of macular their own central vision. An Amsler degeneration to regularly monitor Grid is provided in this brochure for central vision in each eye. The object your convenience. Patients should of self monitoring is to detect any wear their normal reading glasses change in vision as early as possible, and hold the Amsler Grid at normal since this may signify progression reading distance in good light. Each to the late stage of AMD. Because eye should be examined in turn by treatment of abnormal blood vessels covering the opposite eye. The patient is generally more successful if given should attempt to look at the central earlier in the course of the disease, dot of the grid to determine whether it is essential that patients attend all the surrounding lines are straight for examination as soon as possible and regular or whether any areas after noticing any significant change of the grid are blurred, distorted, or in vision. Self-monitoring can be as missing. Any change in the appearance simple as covering each eye in turn of the grid should be reported to your while looking at a familiar object, or ophthalmologist as soon as possible. TV screen. The Amsler Grid provides a

14 MACULAR DEGENERATION Amsler Grid Eye Exam

1. Do not remove glasses or contacts 4. If you see you normally wear for reading. wavy, broken or 2. Stand approximately 13in (33cm) distorted lines, from the grid in a well-lit room. or blurred or missing areas of 3. Cover one eye with your hand and vision, you may focus on the centre dot with your be displaying symptoms of AMD uncovered eye. Repeat with the and should contact your eye care other eye. provider immediately.

15 Will glasses help? Macular Degeneration Patients will gain some benefit from wearing the correct glasses. However, at the Lions Eye a change in prescription is unlikely to Institute significantly improve vision in most people. In late stage AMD special The cause of macular degeneration magnifying spectacles and various is not yet understood and therefore optical instruments are available current treatment cannot prevent or to help people continue their usual cure the condition. The key to future activities. Known as low-vision aids, treatment will be intervention at a these devices enable patients to use much earlier stage, prior to irreversible their remaining vision to its fullest. retinal damage. Rehabilitation programs and other Our Ocular Tissue Engineering community services are also available Laboratory at the Lions Eye Institute through VisAbility (http://www.visability. is developing methods to grow the com.au/), formerly known as the retinal pigment epithelium (RPE) from Association for the Blind of WA. A patients’ own cells. The RPE is a layer referral can be arranged through your of cells underlying the retina that act retinal specialist if necessary. 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.

16 MACULAR DEGENERATION Researchers at the Lions Eye Institute It takes dedicated theorise that replacement of damaged RPE by stem cell-derived RPE may research program to be a curative approach to treat AMD. cure blindness However, reversal of vision loss is only possible if photoreceptor cells and Assessment and management of underlying choroidal blood vessels are macular degeneration is a complex restored as well. Using sophisticated process that requires specialised molecular biology techniques (cellular training in retina and cutting edge reprogramming), they are developing research expertise. Nonetheless, the optimal conditions for growing RPE most challenging obstacle that the from patient-derived stem cells. Lions Eye Institute faces is securing Success in this approach will offer adequate funds to carry on its sight- patients their own RPE cell to replace saving research programs. The Institute the damaged ones in their eye. relies upon the financial support of the The Lions Eye Institute is the leading community for its continued existence ophthalmic research institution in the and impact on improving quality of life southern hemisphere and is amongst through improving vision. Join us in the best in the world. There are many the fight against blindness. Your tax dedicated research teams at work deductible* donation is therefore a investigating macular degeneration welcome and important contribution to and many other unsolved causes of an enterprise from which you or your blindness. Your ophthalmologist is loved-ones may one day benefit. involved with many of these teams, ensuring that you receive the very best and most up-to-date treatment Please telephone that can be provided anywhere in (08) 9381 0777 the world. Additionally, the Institute for more information is involved in community screening projects through the Lions Save-Sight *Donations $2 or more are tax Foundation to detect silent causes of deductible. visual loss such as glaucoma, diabetic retinopathy and trachoma.

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