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Macular Disease - information booklet

Produced by Wales Council for the Blind and the Macular Disease Society UK with support from AMD Alliance International

CONTENTS

Copies of this leaflet are available in braille and on audio-tape.

INTRODUCTION

The production and distribution of this leaflet is part of the AMD Alliance International campaign to raise awareness of AMD and retinal degeneration across the world to reach as wide an audience as possible.

Age Related Macular Degeneration is the leading cause of registered blindness in people over 50 in the Western world yet awareness remains low. Approximately 25-30 million people are affected by some form of AMD (if current trends continue, this is estimated to double in the next 35 years) are affected by some form of AMD. 

This leaflet helps explain the facts about AMD in order to make people aware of what needs to be done. The campaign needs the support of all those who are in a position to promote its aims. The very good reasons for doing so are explained below.

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WHAT IS AMD?

Age-related macular degeneration (AMD) is a degenerative retinal eye disease that causes progressive loss of central vision, leaving only peripheral, or side, vision in tact. AMD affects the macula (see diagram), the central part of the retina responsible for clear, central vision needed for daily activities like reading or driving.

AMD is the leading cause of registered blindness for people over the age of 50 in the Western world.

Clinical Definition

Central vision loss from AMD is caused by the degeneration of light-sensing cells in the macula called photoreceptors. The macula is the central portion of the retina responsible for perceiving fine visual detail. As these photoreceptors begin to degenerate, so does the individual's central vision. The extent of vision loss varies widely and is related to the type of AMD, its severity and other individual characteristics.

AMD appears to come in two types - the "dry" form and the more severe "wet" form. Dry AMD, the more common and milder form of AMD, accounts for 85% to 90% of all cases. Dry AMD results in varying forms of sight loss and may or may not eventually develop into the wet form. One key identifier for AMD is the collection of small, round, white-yellow, fatty deposits called drusen in the central part of the retina. Drusen accumulate in the Retina Pigment Epithelium

(RPE) tissue beneath the macula (see diagram).

Diagram of the Eye

Although the wet form of AMD accounts for only 10 -15 percent of all AMD, the chance for severe sight loss is much greater. It is responsible for 90 percent of severe vision loss associated with AMD.  Approximately 70% of those with wet AMD become registerably blind within two years of diagnosis. Wet AMD is caused by the growth of abnormal blood vessels, or choroidal neovascularisation (CNV), under the central part of the retina, the macula. These abnormal vessels leak fluid and blood into the tissue at the back of the eye, causing a blister to form in the retina. This progression eventually leads to scar tissue, distortion and a loss of central vision.

What are the symptoms?

The progression of the disease can be slow or rapid, but the deterioration of central vision generally occurs over a period of a few years. Peripheral vision usually remains normal, but the individual will have difficulty seeing at a

distance or doing detailed work like sewing or reading fine print. Faces may begin to blur, and it becomes harder to distinguish colours. Distortion that can cause edges or lines to appear wavy may accompany or precede the blurred vision. Those with more advanced forms of AMD may begin to see dark or empty spaces that block the centre of vision.

View through the eyes of an individual with AMD.

Courtesy of Prevent Blindness America

Does AMD cause blindness?

While AMD rarely causes total blindness, the loss of central vision may make everyday activities extremely difficult. Visual deterioration associated with wet AMD is generally severe and can be rapid. Unlike dry AMD, the patient may begin to notice reduced vision. Distortions may appear due to the formation of leaky, abnormal blood vessels on the overlying retina.

Vision loss with dry AMD is usually gradual. A patient may begin to notice blank areas in central visual field. Over time, dry AMD can cause a large loss of detail vision; however, vision loss is generally not as severe compared to the wet form. In either form of AMD, once retinal tissue is damaged, lost vision cannot be regained.

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How do I know if I have AMD?

A chart called the Amsler Grid may be used in some cases to highlight visual distortions symptomatic of AMD. Of course, only a professional eye exam by an eye care specialist can properly diagnose AMD. According to the American Academy of Ophthalmology (AAO), "asymptomatic individuals 40 to 64 years of age who have had a previously comprehensive examination, the recommended interval for interim evaluations is two to four years. For the age group of 65 or older, the AAO recommends an examination every one to two years, even in the absence of symptoms." (AAO Policy Statement, September 15, 1990)

What causes AMD?

It is generally unknown as to what causes AMD. The only clinically proven risk factor is smoking. Several studies have identified possible additional risk factors:

  • Age
  • Genetics
  • A recent study identified a hereditary link, and determined that first-degree relatives of patients with wet AMD may have three times the risk of developing the disorder. The American Academy of Ophthalmology (AAO) recommends that patients who have blood relatives with AMD have their eyes checked every two years.
  • Cataracts
  • Hypertension
  • Patients with dry AMD on anti-hypertensive drug therapy coupled with high serum cholesterol levels and low serum carotenoid are at a greater risk for developing wet AMD.
  • Sun exposure
  • Farsightedness
  • Light skin or eye colour
  • Women
  • Postmenopausal women who do not undergo oestrogen therapy are also at a greater risk of developing AMD.
  • In general, the incidence of AMD is most prevalent in white women over 60.

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How many people are affected by AMD?

Approximately 500,000 new cases of wet AMD are diagnosed annually world-wide and approximately 25-30 million individuals are affected by some form of AMD. This number is expected to triple over the next 25 years.

If AMD is such a serious disease, why are people not aware of it?

Even with the severity of AMD, global awareness of the disease remains minimal. In a recent survey commissioned by the AMD Alliance International, only two percent of adults surveyed think the leading cause of severe sight loss among adults age 50 or older is Age-related Macular Degeneration. Eighty-two percent of adults surveyed were unfamiliar with AMD.

Is AMD treatable?

There is currently no treatment for dry AMD. There are two clinically proven treatments for wet AMD. The first treatment is laser photocoagulation, a surgical procedure involving the application of a hot laser to seal and halt or slow the progression of abnormal blood vessels. However, this procedure may produce a blind spot on the retina.

The new Photodynamic Therapy, the second available option, is a treatment that uses a non-thermal (or cold) laser with an intravenous light sensitive drug to halt or slow the progression of abnormal retina blood vessels. This treatment does not leave a blind spot on the retina.

While many photodynamic therapies are in clinical trials, verteporfin (Visudyne TM) has recently received regulatory approval in over 40 countries covering Europe, North & South America as well as the Asia Pacific Region. Other therapies are still under clinical investigation.

Early diagnosis is key, because once vision is lost due to of the growth of abnormal blood vessels, it cannot be reclaimed.

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How can I avoid losing vision to AMD?

There are steps to take to minimise risk factors and decrease the chances of developing AMD.

  • Make Regular Eye Exams/Tests a Priority: Early detection of AMD through regular medical eye exams/tests (every two years) is crucial so that options for treatment, rehabilitation and support services can be administered early enough to make the greatest impact.
  • Quit Smoking: Smokers are at a greater risk of developing macular degeneration than non-smokers.
  • Control High Blood Pressure: A link has been found between high blood pressure and AMD
  • Protect Your Eyes From Ultraviolet (UV) Light: UV may also damage your retina. This increases your chances of developing macular degeneration and may also speed up the development of AMD. Wear a hat or visor when you are outside, even on cloudy days, and buy sunglasses that block out 99-100% of all ultraviolet rays.
  • Eat a Healthy Diet: A diet rich in fruits and vegetables and low in saturated fat seems to help people with AMD. Some studies have found dark green leafy vegetables such as spinach, kale and collard greens may also be beneficial in lowering the risk of wet AMD.
  • Exercise Regularly: Exercise contributes to both eye health and overall well being. The role of vitamins, minerals, and antioxidants in the prevention of macular degeneration has been a source of controversy and conflicting information; high serum levels of vitamins E, A, and C, as well as zinc, may be beneficial, but currently their efficacy is unproven.

    The National Eye Institute sponsored a study called AREDS (The Age Related Eye Disease Study) which tested the use of antioxidants and zinc on the progression of AMD. The study showed that those with a high risk of developing advanced AMD could reduce that risk by 25% by taking a daily supplement containing anti-oxidant vitamins E (400 IU), C (500mg), Beta carotene (15mg) and 80mg of zinc with 2mg of copper. Those with medium to large drusen in the retina or with advanced AMD in one eye only fall in to this high-risk category. The supplement did not provide any benefit for those with no AMD or early stage AMD, nor could it restore central vision once lost.  Individuals should be cautious in using any nutritional supplements without their doctor's approval. Some of these supplements can cause harmful interactions with other medications and some vitamins can be toxic in excess. Smokers taking high doses of beta-carotene have an increased risk of lung cancer.

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What rehabilitation options are available for AMD patients?

Although there is not a cure for AMD, hope does exist. Those diagnosed with AMD may maintain independent lifestyles through treatment and rehabilitation options such as low vision aids (e.g. strong reading or telescopic lenses, etc.), support services and learned activities of daily living. These options should be discussed with an eye care specialist.

OTHER RETINAL DEGENERATIONS

In addition to AMD there are many other forms of Retinal Degeneration and although they are not as common, many of them are more severe and affect patients at a much earlier age.  Indeed some of these conditions can affect children even before they reach their teens. In general terms the earlier the onset of these diseases the more severe they are and the more likely they are to cause blindness at an early age. 

The most common of these diseases are:

  • Retinitis Pigmentosa

  • Ushers Syndrome

  • Bests Macular Degeneration

  • Stargardts Disease

All of these conditions are inherited.

Retinitis Pigmentosa (RP)

Retinitis Pigmentosa affects 1 in 3,000-4,000 people and its incidence appears to be constant in all populations.  Depending on its mode of inheritance it normally begins to manifest in late teens or early twenties and can cause total blindness as early as 30. In the less severe forms useful sight can be retained into the 50's and sometimes even into the 60's. There is no known treatment for this condition but it is the subject of very extensive research throughout the world, primarily with a view to developing a gene based therapy. 

Much of the research into Retinal Degeneration has been focused on Retinitis Pigmentosa, since the establishment of the Foundation Fighting Blindness in the United States in 1971 because that organisation was originally founded to find a treatment for RP. Similar organisations were subsequently founded in 50 countries around the world and now form a world wide umbrella group under the heading Retina International which now promotes research in many countries into all forms of Retinal Degeneration.

Usher's Syndrome

Usher's Syndrome is a form of Retinitis Pigmentosa which also involves hearing loss.  It is a recessively inherited condition and its effects are devastating as it attacks people in their teens who suffer from a progressive sight loss in addition to their existing hearing loss and can suffer total sight loss around the 40-50 age group.  A great deal of work has been done to identify the genes which cause Ushers Syndrome and it is hoped to develop a gene based therapy over the coming 5-10 years.

In both of the above conditions the peripheral retinal cells known as the Rod Cells are first affected and people who suffer from these conditions often  retain very useful central vision until the very advanced stages of  degeneration. This is in marked contrast to Macular Degeneration where the central vision is affected from the very beginning.

Bests Macular Degeneration and Stargardts Disease

Both of these conditions are inherited forms of Macular Degeneration and affect children usually from their teens.  The normal symptoms are difficulties in reading and subsequently problems relating to employment. Latest  research results proved that there is also a form of Stargadts disease with late onset affecting people of age 70 and more which could be misdiagnosed as Age-related Macular Degeneration (AMD).

These conditions are genetically inherited and research has focused on attempts to identify the genes in the hope that it might be possible to establish gene therapy, initially to prevent any further deterioration and at some stage perhaps to attempt the reversal of the damage.

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TOP TEN QUESTIONS AMD PATIENTS SHOULD ASK AND TOPLINE ANSWERS

An AMD patient's ability to ask the right questions and receive accurate information and options at the time of diagnosis is crucial. This way, together the AMD patient and the eye care provider can address appropriate options for treatment, rehabilitation and support services in order to maintain the highest possible quality of life for the patient. The AMD Alliance International Board of Directors recommends the following:

1. Question: How did I get this disease -- are there risk factors?

Topline Answer: The cause of AMD is still unknown, and the only clinically proven risk factor is smoking. However, possible risk factors include: genetics, cataracts, hypertension, sun exposure, farsightedness, light skin or eye colour, and a diet low in certain vitamins, minerals and antioxidants. These potential risk factors vary widely from one patient to the next. No two pairs of eyes are the same.

2.  Question: Is AMD hereditary - - will my children be affected?

Topline Answer: Several studies have found that family history of AMD increases the risk of developing the disease. Therefore, if you have AMD, it is even more crucial that your adult children over the age of 40 have regularly scheduled eye exams. Early diagnosis through medical eye exams at least every two years offers eye care specialists the opportunity to provide counsel about appropriate options for possible treatment, rehabilitation and support services.

3. Question: Will both eyes be affected - - what can I do to protect the vision I have?

Topline Answer: It is very difficult to predict what will happen to the second eye if AMD affects one eye. In some instances, vision is not affected. However, vision may change in the second eye over time. Approximately 42 % of patients with wet AMD in one eye will develop the same in the second eye over a period of five years. But again, it is difficult to predict the extent of the vision loss. IT IS THEREFORE IMPORTANT TO ROUTINELY VISIT YOUR EYE CARE PROFESSIONAL. It is important to consult your eye care provider to find out where you can find a low vision specialist who may be able to assist you with your specific needs.

4. Question: How fast will wet AMD progress - - will I become totally blind?

Topline Answer: While 85-90 percent of AMD patients have the less sight-threatening dry form of AMD, approximately 70% of those diagnosed with wet AMD become registerably blind within two years of diagnosis. However, with AMD, you generally will maintain your peripheral, or side, vision, even into the latest stages of the disease. You should not become completely blind.

5. Question: Are there any current or promising treatments - - can I enrol in a clinical trial?

Topline Answer: There is currently no treatment for dry AMD; however, there are two clinically proven treatments for wet AMD:

  • Laser photocoagulation: can be used in about 15 percent of wet AMD patients (those in which the area of leakage is small, well defined and outside the central area). It involves the use of a thermal (or hot) laser, which burns or cauterises the abnormal, leaky blood vessels. Because this treatment also damages the overlying retina, patients experience some immediate permanent vision loss following treatment; however, this loss is preferable to total vision loss.
  • Photodynamic Therapy: can be used in about 36 percent of wet AMD cases (those in which the area of leakage is due to a predominately classic lesion located in the centre) and uses a non-thermal (or cold) laser with an intravenous light-sensitive drug. The cold laser activates the drug to seal the abnormal vessels without damaging the overlying retina.
  • There are a number of other treatments in development. Your eye care provider can discuss these with you and inform you of any clinical trials for which you may be eligible.

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6. Question: Is there anything I can do to enhance the vision I have left (e.g., technology, vision aids, rehabilitation, etc.)?

Topline Answer: Yes! There are a number of things you can do to maintain an independent lifestyle. Your eye care provider can discuss with you available treatments, vision rehabilitation options such as low vision aids (e.g., strong reading or telescopic lenses, electronic devices, etc.), support services, talking books (available at your local public library) and learned activities of daily living.

7. Question: Is AMD rare - - how many people are affected by this and are there any support groups in my area?

Topline Answer:  AMD is the leading cause of registerable blindness for people over 50 in the Western world. Approximately 25-30 million people around the world are affected by some form of AMD. In the U.K. there are many local support groups that may assist you. You can find a local group in your area by contacting the Macular Disease Society whose contact details are listed at the end of this leaflet. For lobbying and campaigning groups, contact the other organisations listed.

8. Question: How will my daily life change - - can I drive a car, cook, read, sew, write letters, live an active life, etc.?

Topline Answer: Although peripheral vision usually remains normal, typical symptoms of both wet and dry AMD may include difficulty seeing at a distance, difficulty doing detailed work like sewing or reading fine print, blurring of faces and difficulty distinguishing colours, distortion causing edges or lines to appear wavy; and dark or empty spaces blocking the centre of vision. However, through the proper use of low vision aids and rehabilitation to learn new skills to enhance daily living, an active lifestyle can be maintained. Although you may not be able to drive a car, you can learn new ways to cook, read, sew, write letters, etc.

9. Question: Where can I get more information about AMD?

Topline Answer: There are a host of resources. Contact details of the main organisations are listed at the end of this leaflet. These organisations are good places to look for more information.

10.  Question: What would you do if you were me?

Topline Answer: This is an excellent question to discuss in detail with your eye care provider, a low vision specialist as well as others who have been diagnosed with AMD and their family and friends to see how they deal with this every day.

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THIS CAMPAIGN

The Aims

The campaign is focused around five calls for action.

Access to Regular Eye Exams

  • Early detection
  • Prevention of avoidable blindness
  • Promote cost-effective management of eye diseases

Access to Treatment Options

  • Equal access
  • Quality of life
  • Prevention of avoidable blindness

Access to Research

  • Expedite development of treatment for all retinal degenerative diseases
  • Accelerate treatment trials
  • Enhance hope for people with visual impairment and for their families

Access to Rehabilitation

  • Maintain independent living
  • Prevent social exclusion
  • Maintain employability of an increasingly ageing population

Access to Public Environment and Information

  • Maintain mobility
  • Protect human and civil rights
  • Prevent avoidable accidents

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AMD ALLIANCE INTERNATIONAL - HISTORY AND DATA

The AMD Alliance International is a non-profit alliance of international vision and seniors organizations working to raise the awareness level of Age-related Macular Degeneration (AMD) and an understanding of available options for treatment, rehabilitation and support services.

Mission

To bring knowledge, help and hope to individuals and families around the world affected by Age-related Macular Degeneration (AMD) by:

  • Generating awareness and understanding of Age-related Macular Degeneration
  • Promoting the importance of education, early detection, knowledge of treatment and rehabilitation options
  • Preserving vision and improving the quality of life of individuals affected by Age-related Macular Degeneration.

International Objectives

  • Raise the overall awareness level of AMD and the importance of early detection.
  • Increase number of people 50+ who receive regular eye examinations.
  • Increase percentage of those with AMD who receive treatment and rehabilitation  options.

International Strategies

  1. Launch aggressive international general consumer awareness campaign with a focus on seniors and their caregivers to raise visibility of AMD and ignite effective action.
  2. Mobilise medical community, insurance industry and key governmental decision-makers to recognise and support AMD as a health priority.
  3. Support existing Alliance Members and expand international reach of Alliance through securing additional Members in targeted countries.

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AMD ALLIANCE INTERNATIONAL BOARD & ASSOCIATE MEMBERS

Board of Directors

1.  The Association for Macular Diseases (U.S.) 

Nikolai Stevenson

2.  The Canadian National Institute for the Blind* (Canada)

Gerrard Grace

3.  The Foundation Fighting Blindness (U.S.)

Bob Gray

4.  International Agency for the Prevention of Blindness (Italian Branch) Giuseppe Castronovo

5.  International Federation on Ageing (Canada)

Dr. Yitzhak Brick

6.  LIGHTHOUSE INTERNATIONAL* (U.S.)

Dr. Bruce Rosenthal (Chair, AMD Alliance International)

7.  The Macular Disease Society* (U.K.)

Dr. Bob Thompson

8.  ONCE/Organización Nacional de Ciegos Españoles (Spain) 

Vicente Ruiz

9.  PREVENT BLINDNESS AMERICA (U.S.)

  John Shoemaker

10.  Pro Retina Deutschland* (Germany)

  Dr. Rainald von Gizycki

11.  RETINA FRANCE (France)

  Jean-Jacques Frayssinet

12.  Retina International (Switzerland)

  Christina Fasser

13.  Royal National Institute for the Blind* (U.K.)

  Stephen Winyard

14.  The Seniors Coalition* (U.S.)

  John Powell

15.  Vision Australia Foundation* (Australia)

  Rick Osborn

*Denotes executive committee member

Associate Members

1.  Age Concern England (U.K.)

2.  ASORN/American Society of Ophthalmic Registered Nurses (U.S.)

3.  Belgische Vereniging ter Voorkoming van Blindheid (Belgium)

4.  Berufsverband der Augenaerzte Deutschlands (Germany)

5.  Canadian Association of Optometrists (Canada)

6.  Canadian Ophthalmological Society (Canada)

7.  ESHEL / The Association for the Planning and Development of Services for the Aged in Israel (Israel)

8.  EURAG / European Federation of the Elderly (Austria)

9.  Fighting Blindness Ireland (Ireland)

10.  Finnish Federation of the Visually Impaired (Finland)

11.  The Foundation Fighting Blindness (Canada)

12.  Fundación Oftalmológica Hugo Nano (Argentina)

13.  Hong Kong Retinal Pigmentosa Society (Hong Kong)

14.  Hong Kong Society for the Blind (Hong Kong)

15.  Lions Clubs International (U.S.)

16.  Macular Degeneration Foundation Australia (Australia)

17.  Macular Degeneration Partnership (U.S.)

18.  Macula Degeneratie Vereniging (Netherlands)

19.  National Coalition on Vision Health (Canada)

20.  National Society for the Prevention of Blindness (India)

21.  Retina Australia (Australia)

22.  Retinal Preservation Foundation of South Africa (South Africa)

23.  Retina Suisse (Switzerland)

24.  The Royal New Zealand Foundation for the Blind (New Zealand)

25.  Spanish Vitreous and Retina Society (Spain)

26.  Wales Council for the Blind (U.K.)

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CONTACT DETAILS

The AMD Alliance International

Avenue de Tervuren 402 • 1150 Brussels • Belgium

Phone: +32 2/761 66 59

Fax:  +32 2/777 05 05

E-mail:  mk@intermar.be

Website:  www.amdalliance.org

The Macular Disease Society (U.K.),

Darwin House, 

13A Bridge Street, 

Andover, 

Hampshire 

SP10 1BE.

Helpline: 0845 241 2041 (freephone)

Web-site: www.maculardisease.org

R.N.I.B. Cymru,

Trident Court,

Cardiff, CF24 5TD

Phone: 02920 450440

Fax:  02920 449550

Helpline: 0845 7669999

Wales Council for the Blind,

3rd Floor, Shand House,

20, Newport Rd.,

Cardiff, CF24 0DB.

Phone: 02920 473954

Fax:  02920 470777

E-mail:  staff@wcb-ccd.org.uk 

Web-site: www.wcb-ccd.org.uk

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