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Trouble with vision as
      we get older...
• Is not always due to Age-
  related macular
  degeneration !
• Your doctor needs to
  perform a complete eye
  exam before reaching a
  diagnosis
Eye Examination -
      Diagnosis


Visual Acuity, Color Vision, Amsler Grid,
Fundus Camera, Fluorescein Angiography,
    Optical Coherence Tomography
Amsler Grid Testing
Drusen, the yellow spots so characteristic
  of Age-related Macular Degeneration
Drusen are sometimes more easily detected
On Color Fundus photos than doctor’s exam
Fluorescein
Angiography
Colour photography is
routinely undertaken with
angiography. It helps to
determine the nature of
changes seen on the
angiogram… particularly
the cause of blocked
fluorescence due to
haemorrhage, pigment or
other cause.
Side by side
    comparison
of the color photo
        and
  the fluorescein
      picture
often complement
        each
       other !
-
Optical Coherence
  Tomography
• Diagnostic imaging technique that examines
  living tissue non-invasively. It is based on a
  complex analysis of the reflection of low
  coherence radiation from the tissue under
  examination.

• Real time cross sectional analysis
•       Macular Degeneration
    Leading cause of
    blindness in people
    over 55
    •   1:10 people over
        55
    •   Increases by a
        factor of 6 in
        people over 80
    •   1.75 million
        Americans over
        age 40 have severe
        AMD
    •   Estimated that 2.95
        million people
        affected by 2020
How Does the Eye
    Work?
•      What is Macular
  Idiopathic
• Central vision
          Degeneration?
  loss
 •   Peripheral vision
     preserved

• Two forms
 •   Dry
     (Nonexudative)
     •   90% of people

 •   Wet (Exudative)
     •   Affects 10% of
         people
What does my Doctor
•
  •
    Drusen
          see?
        Metabolic waste
        products
        accumulating under
        the retina

•   Pigment clumping
    and atrophy
    •   Death of support
        cells under retina

•   Geographic atrophy
    •   Large areas of cell
        death

•   Hemorrhage
    (blood)
    •   Hallmark of wet
        AMD
My Doctor told me I have Macular
   Degeneration. Will I go blind?
• Not necessarily
•   Only 10% develop
    the wet form which
    is responsible for
    90% of blindness
    from AMD
    •   Newer treatments
        may reduce risk of
        blindness

•   Only 10% of the dry
    patients will develop
    severe vision loss
Risk factors for Vision
• Non-modifiable:
  things we cannot loss
  change
 •   Age
     •   (If you figure out how
         to stop aging, please let
         me know)

 •   Caucasian race
 •   Female gender
 •   Family history
     •   2.5x increased risk of
         first degree family
         relative
Risk factors for Vision
• Modifiable
 • Smoking   Loss
    • Hypertension
    • High
      Cholesterol
    • Obesity
    • Sedentary
      Lifestyle


•   Heart Health= Eye
What about those
AREDS study

•    Vitamins? II
    Followed 3640 patients
    for average 6.3 years
          • AREDS
•   High doses of
    antioxidant vitamins         •   Started in 2006 –
                                     ongoing
•   Hope they will reduce
    oxidative stress in          •   Added
    macula
                                     •   Lutein: forms macular
    •   Vitamin A                        pigment

    •   Vitamin C                    •   Zeaxanthin

    •   Vitamin E                •   Subtracted

    •   Zinc: antioxidant in         •   Vitamin A: Potential
        conjunction with C and           increased risk of lung
        E                                cancer in smokers

    •   Copper
•                     Vitamins
    Not found helpful for
    everyone
•   No benefit
    •   No AMD                   •   Benefit:
    •   Mild AMD                     •   If everyone took AREDS,

•   Beneficial
                                         300,000 people could be
                                         prevented from developing

    •   Moderate AMD
                                         advanced AMD over 5 year


    •   Severe AMD

    •   Persons with wet AMD         •   Unfortunately will not stop
                                         progression of AMD in all
•   Benefit                              patients

    •   Reduced risk of
        progression of AMD by
        25%

    •   Reduced risk of vision
Which is the best
         Multivitamin?
•   They are all basically the
    same as long as they have
    the AREDS formula

•   Differences in brands
    •   AREDS I vs AREDS II formula

    •   Dosing

    •   Price

    •   Packaging

    •   Advertising
•   Stop Smoking      Summary
•   Exercise
•   Heart Healthy Diet
•   Eat leafy green
    vegetables
•   Control blood
    pressure and
    cholesterol
•   AREDS
    Multivitamin if
    indicated by your
    eye doctor
•   Monitor vision and
    regular eye checks
•       Macular Degeneration
    Leading cause of
    blindness in people
    over 55
    •   1:10 people over
        55
    •   Increases by a
        factor of 6 in
        people over 80
    •   1.75 million
        Americans over
        age 40 have severe
        AMD
    •   Estimated that 2.95
        million people
        affected by 2020
How Does the Eye
    Work?
•      What is Macular
  Idiopathic
• Central vision
          Degeneration?
  loss
 •   Peripheral vision
     preserved

• Two forms
 •   Dry
     (Nonexudative)
     •   90% of people

 •   Wet (Exudative)
     •   Affects 10% of
         people
What does my Doctor
•
  •
    Drusen
          see?
        Metabolic waste
        products
        accumulating under
        the retina

•   Pigment clumping
    and atrophy
    •   Death of support
        cells under retina

•   Geographic atrophy
    •   Large areas of cell
        death

•   Hemorrhage
    (blood)
    •   Hallmark of wet
        AMD
My Doctor told me I have Macular
   Degeneration. Will I go blind?
• Not necessarily
•   Only 10% develop
    the wet form which
    is responsible for
    90% of blindness
    from AMD
    •   Newer treatments
        may reduce risk of
        blindness

•   Only 10% of the dry
    patients will develop
    severe vision loss
Risk factors for Vision
• Non-modifiable:
  things we cannot loss
  change
 •   Age
     •   (If you figure out how
         to stop aging, please let
         me know)

 •   Caucasian race
 •   Female gender
 •   Family history
     •   2.5x increased risk of
         first degree family
         relative
Risk factors for Vision
• Modifiable
 • Smoking   Loss
    • Hypertension
    • High
      Cholesterol
    • Obesity
    • Sedentary
      Lifestyle


•   Heart Health= Eye
What about those
AREDS study

•    Vitamins? II
    Followed 3640 patients
    for average 6.3 years
          • AREDS
•   High doses of
    antioxidant vitamins         •   Started in 2006 –
                                     ongoing
•   Hope they will reduce
    oxidative stress in          •   Added
    macula
                                     •   Lutein: forms macular
    •   Vitamin A                        pigment

    •   Vitamin C                    •   Zeaxanthin

    •   Vitamin E                •   Subtracted

    •   Zinc: antioxidant in         •   Vitamin A: Potential
        conjunction with C and           increased risk of lung
        E                                cancer in smokers

    •   Copper
•                     Vitamins
    Not found helpful for
    everyone
•   No benefit
    •   No AMD                   •   Benefit:
    •   Mild AMD                     •   If everyone took AREDS,

•   Beneficial
                                         300,000 people could be
                                         prevented from developing

    •   Moderate AMD
                                         advanced AMD over 5 year


    •   Severe AMD

    •   Persons with wet AMD         •   Unfortunately will not stop
                                         progression of AMD in all
•   Benefit                              patients

    •   Reduced risk of
        progression of AMD by
        25%

    •   Reduced risk of vision
Which is the best
         Multivitamin?
•   They are all basically the
    same as long as they have
    the AREDS formula

•   Differences in brands
    •   AREDS I vs AREDS II formula

    •   Dosing

    •   Price

    •   Packaging

    •   Advertising
•   Stop Smoking      Summary
•   Exercise
•   Heart Healthy Diet
•   Eat leafy green
    vegetables
•   Control blood
    pressure and
    cholesterol
•   AREDS
    Multivitamin if
    indicated by your
    eye doctor
•   Monitor vision and
    regular eye checks
Age-related Macular
  Degeneration
What is Age-Related Macular
            Degeneration?
• Age-related macular degeneration (AMD) is a
  deterioration or breakdown of the eye's macula.
• The macula is a small area in the retina — the
  light-sensitive tissue lining the back of the eye.
• The macula is the part of the retina that is
  responsible for your central vision, allowing you
  to see fine details clearly.

                                    •   American Academy of Ophthalmology
What is going on in the
         eye?
Where exactly?
Where exactly?
Genetics
• DNA
• All information of life encoded in DNA and
  expressed by proteins.
• James Dewey Watson (US) & Francis Crick
  (UK) – Co-discoverers Structure of DNA
  1953
• Awarded Nobel Prize in Physiology or
  Medicine 1962
Before…
…After…Age-related
     changes
Genetic Basics…get
          small…
• The human body is made up of trillions of Cells,
• and with a few exceptions, each cell contains a
  Nucleus
• and with a few exceptions, each nucleus contains
  a COMPLETE set of Chromosomes
• And the chromosomes are made of DNA
From DNA…to You
    and Me…
…and smaller…From Cells to
     Nucleus to Chromosomes

• Every human cell contains 23 pairs of
  chromosomes, for a total of 46 chromosomes.
• Each Chromosome is made up of
• a double- stranded,
• Tightly-coiled,
• helix of DNA
Human Chromosomes
…and smaller…
• A CHROMOSOME contains hundreds to
  thousands of genes.
• A GENE is a segment of DNA containing
  the code used to synthesize a protein.
• A TRAIT is any gene-determined
  characteristic and is usually determined by
  more than one gene.
• Some traits are caused by abnormal genes
  that are inherited or that are the result of
  a mutation.
From Chromosomes to
       Genes
WhatStudies we know?…
• Genetic do
• 1988-2010 so far
    • DNA and Gene-Environment Data
      Collection
    • Twin Study – WWII Registry
•   1997 Familial Aggregation Study – Relatives of
    Cases with Wet AMD. Relatives of controls.
•   1997 Phenotypic Heterogeneity in Families
    with AMD


                         •   Johanna M. Seddon, M.D., Sc.M.
What do we know?…
        so far
• From the US Twin Study, we’ve learned
• Substantial Genetic Component –
 • 46 to 71% of the variation in
   phenotype
 • Important Environmental Influence:
   19 to 37%
OK…what “Genetic
    Factors”?...
• It’s not so much as a “bad” gene, but rather
• Variations of “normal genes” that influence
  the risk of developing AMD.
• Specific DNA chromosomes associated
  with the defective genes are located on
  chromosomes
• 1q and 10q
From Chromosomes to
       Genes
Genes associated…so
• CFH – Y402H far
•   CFH – rs1410996
                              •   Hepatic Lipase C (LIPC)

•   CFB
                              •   CETP

•   C2
                              •   ABCA1

•   Complement Component
                              •   TIMP 3 / SYN3
    3 (C3)                    •   VEGFA

•   Complement Factor I CFI   •   COL10A1

•   ARMS2/HTRA1 gene          •   COL8A1
    region
                              •   TNFRSF10A
Gene location… leads
     to therapy
• For example, the VEGFA locus and the
  development of
• Avastin
• Lucentis and recently
• Eylea
Antigen/Antibody cell
     membrane
      Bayer Healthcare – Bayer Schering Pharma
Surface
antigens/antibodies
        Eylea.com
Relative risk of Genetic
           loci
        Nature Genetics 38: 1057, 2006
Epidemiology

•   Epidemiology is the study of
•   the distribution and patterns of health-events,
•   health-characteristics and
•   their causes or influences in well-defined
    populations.
Epidemiology
• It is our principal method of public health
  research, and
• helps us make policy decisions as well as
  evidence-based medicine
• by identifying risk factors for disease and
  helps us with recommendations for
  appropriate preventive medicine.
Epidemiologic studies looking at
• Meta-analysis of 15 genome-wide
       the genetics of AMD
  association studies
• Began in 2009
• 8,000 cases and 50,000 controls
• 19 groups from 13 countries
• 19 genomic loci associated with AMD
• Six novel loci not previously associated
  with disease
• Pathways similar to other known genes.
                                •   ASHG Oct 2011
Epidemiology and
             Causation
Risk factors for cardiovascular disease (CVD)
   are associated with age-related AMD;
  • Smoking
  • Dietary antioxidants, fats, omega-3 fatty
    acids
  • Physical activity
  • Obesity, abdominal adiposity
  • Hypertension
  • Cholesterol
  • CRP (C-reactive protein)
Epidemiology and
            Causation
• For the homozygous (pure trait) risk
  genotype
• Risk of AMD was

 • Increased for BMI (Body Mass
    Index) and


 • Increased with smoking
So what can we say?
• Genetic mutations make us susceptible to
  the development of AMD
• Multiple genetic factors are at work
• Risk profiles help us to change life style to
  decrease the chance of AMD development
• Which leads us to…
The big question (at least for the
          retina specialists)
• What information from
• TV,
• Radio,
• Internet,
• YouTube,
• Email,
• Fax, Photocopy, Text, Blog,
  Wikipedia….etc
• Should You believe?
Types of Optical Devices




         Near Devices
Stand Magnifier
Dome Magnifier
Illuminated Stand
     Magnifier
Hand Held Pocket
   Magnifier
Hand Held
Illuminated
Microscopes or Reading Glasses
Types of Optical Devices




     Distance Devices
Binoculars
Monocular
Full-Field Telescopic
       Systems
Bioptic Systems
Sunfilters
Night Vision Scopes
Getting the Right
               Device(s)devices
• Because there is such a variety of
    available, it can be somewhat overwhelming to
    locate the correct device.
•   Individuals should receive an evaluation from an
    Optometrist who specializes in treating
    individuals with low vision.
•   This assures that the individual receives the
    correct device with consideration of all goals
    and needs.
What’s New in
Low Vision Technology
Handheld Magnifying Cameras

•   Miracle Mouse
    Electronic TV
    Magnifier
•   MonoMouse-RM B&W
    Electronic Magnifier
•   Max Digital Color
    Magnifier
•   Primer 2 Color
    Electronic Magnifier
Traditional CCTV Video
           Magnifiers reading
                 • Increased
                              speed
                          •   Increased duration
                          •   Reduced visual effort
                          •   Comfortable
•   Desktop systems           working position

•   Variable
    Magnification
•   Color, W/B, B/W
•   Large Field of View
Magnifiers
      Integrated LCD
• Available from all major vendors
               Displays
• Sizes: 17” to 24” + with WS
•   Adjustable height screens
•   High contrast
•   Flicker free
•   Improved response rates
•   Well received by consumers
Integrated LCD Systems
Video
             Magnification
• Variable     Systems
  Magnification
• Adjustable brightness
• Selectable contrast
  setting
• Wide field of view
  for better viewing
  abilities
Magnification
Brightness
Contrast
Windowing and
 Underlining
Portable Video
• More Compact, 7-inch screens
         Magnifiers
• Extensive reading tasks
• Two handed use
• Optelec Traveler
• FS Sapphire
• EV Amigo
• Fusion 7” LCD Portable Video Magnifier
Compact Video
           Magnifiers:
• Variable Magnification
•   Replaceable Batteries
•   Longer battery life
•   Image Freeze
•   Color Select
•   Light off mode: for reading cell phones, other
    displays
•   Writing Cameras
•   Distance Viewing
•   Smaller Designs
Compact/Pocket
Video Magnifiers
        • Advantages
          •     Wide field of view

            •   Image at display level

            •   Use at arms-length

            •   Reverse contrast available

            •   Cell phone, PDA displays!

            •   Short-term writing tasks

            •   Kids reading comic books
                under the blanket
Compact Systems with
 Distance Capabilities
• Near and distance capabilities
• Read mail, products on a shelf and get a
  better view of distance object.
• Allows users to see an object that is at
  distance close up on the screen
• Limited clarity and magnification at a
  distance
GW Micro
           Sense View Duo
•   Two cameras
•   Stores up to 20 images
•   3X to 13X magnification
•   Lights off mode
•   Image panning
•   7 Viewing modes, 4
    Brightness levels
•   4.5 hour battery life,
    integrated, rechargeable
•   Cost: $1299.00
Ash Technology
Quicklook Focus
Desktop Video Magnifiers
• Full Size screen
                 Features
• Full Features
• Remote Controllable
• Near and Distance Viewing
• For Classroom, Conference or In- Service
  Training Activities
• Transportable “Luggable”
• AC or optional battery
Vision Technology: VIEW
Enhanced Vision
 Acrobat LCD
Onyx Deskset
Laptop Compatible Video
       Magnifiers
•   Most students
    use laptop
    computers
•   Video
    magnification
    could be helpful
•   Portable camera
    using notebook
    display
Clarity PCMate
•   Powered by USB
•   Requires 2 USB 2
    ports
    •   Sequence, setup

•   Remote control
    camera
•   Keyboard
    controlled
    software
Optelec
ClearNote
      • Ease of camera
        aiming

         •   Built in Light source

         •   Camera control
             positioning

         •   Keyboard controlled
             software

         •   Keyboard controlled
             camera

         •   Dolphin, Zoom Text
             compatible
Freedom Scientific Onyx
      PC Edition arm and flex
            • Swing
                   arm models
               •   Remote control
               •   MAGic with
                   EyeMerge software
                   included
               •   Camera can be
                   powered by USB port
               •   Distance, desktop, self
                   view modes
               •   Cost: $2895.00
Specialty Video
        Magnifiers

• Flipper
• Shoppa
Headborne Low Vision
      System
          •   Eyetonomy SightMate LV920

          •   www.vuzix.com/medical/lv920.h
              tml

          •   Zoom at near: 3X optical, 4X
              digital

          •   Zoom at distance: 0.5X to 6X

          •   Edge contrasting, Inverse mode

          •   Sleek design, 8 ounces

          •   $3499.00

          •   Not yet available for purchase
              or review
Scan and Read Devices
• Kurzweil 1000 (software)
• ABiSee Zoom-Ex, EyePal Vision (portable,
  Solo)
• FS Pearl (laptop compatible)
• KNFB Reader (smartphone)
• AI Squared ZoomReader (app)
Assessment functioning
• Evaluation of visual Factors
•   Response to low vision approaches
•   Analysis of tasks, task specific
•   Required performance level
•   Integration of systems
•   Training
•   Trial of system; speed, duration,
    productivity
•   Modifications
•   Provision
Types of Optical Devices




         Near Devices
Stand Magnifier
Dome Magnifier
Illuminated Stand
     Magnifier
Hand Held Pocket
   Magnifier
Hand Held
Illuminated
Microscopes or Reading Glasses
Types of Optical Devices




     Distance Devices
Binoculars
Monocular
Full-Field Telescopic
       Systems
Bioptic Systems
Sunfilters
Night Vision Scopes
Getting the Right
               Device(s)devices
• Because there is such a variety of
    available, it can be somewhat overwhelming to
    locate the correct device.
•   Individuals should receive an evaluation from an
    Optometrist who specializes in treating
    individuals with low vision.
•   This assures that the individual receives the
    correct device with consideration of all goals
    and needs.
What’s New in
Low Vision Technology
Handheld Magnifying Cameras

•   Miracle Mouse
    Electronic TV
    Magnifier
•   MonoMouse-RM B&W
    Electronic Magnifier
•   Max Digital Color
    Magnifier
•   Primer 2 Color
    Electronic Magnifier
Traditional CCTV Video
           Magnifiers reading
                 • Increased
                              speed
                          •   Increased duration
                          •   Reduced visual effort
                          •   Comfortable
•   Desktop systems           working position

•   Variable
    Magnification
•   Color, W/B, B/W
•   Large Field of View
Magnifiers
      Integrated LCD
• Available from all major vendors
               Displays
• Sizes: 17” to 24” + with WS
•   Adjustable height screens
•   High contrast
•   Flicker free
•   Improved response rates
•   Well received by consumers
Integrated LCD Systems
Video
             Magnification
• Variable     Systems
  Magnification
• Adjustable brightness
• Selectable contrast
  setting
• Wide field of view
  for better viewing
  abilities
Magnification
Brightness
Contrast
Windowing and
 Underlining
Portable Video
• More Compact, 7-inch screens
         Magnifiers
• Extensive reading tasks
• Two handed use
• Optelec Traveler
• FS Sapphire
• EV Amigo
• Fusion 7” LCD Portable Video Magnifier
Compact Video
           Magnifiers:
• Variable Magnification
•   Replaceable Batteries
•   Longer battery life
•   Image Freeze
•   Color Select
•   Light off mode: for reading cell phones, other
    displays
•   Writing Cameras
•   Distance Viewing
•   Smaller Designs
Compact/Pocket
Video Magnifiers
        • Advantages
          •     Wide field of view

            •   Image at display level

            •   Use at arms-length

            •   Reverse contrast available

            •   Cell phone, PDA displays!

            •   Short-term writing tasks

            •   Kids reading comic books
                under the blanket
Compact Systems with
 Distance Capabilities
• Near and distance capabilities
• Read mail, products on a shelf and get a
  better view of distance object.
• Allows users to see an object that is at
  distance close up on the screen
• Limited clarity and magnification at a
  distance
GW Micro
           Sense View Duo
•   Two cameras
•   Stores up to 20 images
•   3X to 13X magnification
•   Lights off mode
•   Image panning
•   7 Viewing modes, 4
    Brightness levels
•   4.5 hour battery life,
    integrated, rechargeable
•   Cost: $1299.00
Ash Technology
Quicklook Focus
Desktop Video Magnifiers
• Full Size screen
                 Features
• Full Features
• Remote Controllable
• Near and Distance Viewing
• For Classroom, Conference or In- Service
  Training Activities
• Transportable “Luggable”
• AC or optional battery
Vision Technology: VIEW
Enhanced Vision
 Acrobat LCD
Onyx Deskset
Laptop Compatible Video
       Magnifiers
•   Most students
    use laptop
    computers
•   Video
    magnification
    could be helpful
•   Portable camera
    using notebook
    display
Clarity PCMate
•   Powered by USB
•   Requires 2 USB 2
    ports
    •   Sequence, setup

•   Remote control
    camera
•   Keyboard
    controlled
    software
Optelec
ClearNote
      • Ease of camera
        aiming

         •   Built in Light source

         •   Camera control
             positioning

         •   Keyboard controlled
             software

         •   Keyboard controlled
             camera

         •   Dolphin, Zoom Text
             compatible
Freedom Scientific Onyx
      PC Edition arm and flex
            • Swing
                   arm models
               •   Remote control
               •   MAGic with
                   EyeMerge software
                   included
               •   Camera can be
                   powered by USB port
               •   Distance, desktop, self
                   view modes
               •   Cost: $2895.00
Specialty Video
        Magnifiers

• Flipper
• Shoppa
Headborne Low Vision
      System
          •   Eyetonomy SightMate LV920

          •   www.vuzix.com/medical/lv920.h
              tml

          •   Zoom at near: 3X optical, 4X
              digital

          •   Zoom at distance: 0.5X to 6X

          •   Edge contrasting, Inverse mode

          •   Sleek design, 8 ounces

          •   $3499.00

          •   Not yet available for purchase
              or review
Scan and Read Devices
• Kurzweil 1000 (software)
• ABiSee Zoom-Ex, EyePal Vision (portable,
  Solo)
• FS Pearl (laptop compatible)
• KNFB Reader (smartphone)
• AI Squared ZoomReader (app)
Assessment functioning
• Evaluation of visual Factors
•   Response to low vision approaches
•   Analysis of tasks, task specific
•   Required performance level
•   Integration of systems
•   Training
•   Trial of system; speed, duration,
    productivity
•   Modifications
•   Provision
Pharmacogenomics
• Treatment based on
    genetic profile

•   “Personalized
    medicine”

•   AREDS – 70% with
    certain gene did not
    progress (vs. 11%
    without)

•   Relationship between
    certain genes and
    outcomes with
    Lucentis and Avastin
Gene Therapy for
 retinal diseases
•                       Trials
    Phase I Dose Escalation Safety Study
    of RetinoStat in Advanced Age-
    Related Macular Degeneration
    (AMD)     Oxford BioMedica
    •   Subretinal injection of virus vector that produces
        anti-VEGF proteins

•   Safety and Efficacy Study of
    rAAV.sFlt-1 in Patients With
    Exudative Age-Related Macular
    Degeneration
    •   Intravitreal injection of a non-pathogenic virus to
        express a therapeutic protein within the eye
Wet




Dry AMD




          Atrophi
             c
So What Have We
    Learned?
 Lory C. Snady-McCoy, MD
Answers to Common
      Questions
What defines macular degeneration?
What tests are used?
Why are they necessary in the
 diagnosis and management?
  Not everything that looks like
      macular degeneration
     is macular degeneration
   Importance of continued
      monitoring and care
50% chance of developing wet AMD
     in fellow eye over 5 years
What Should I Eat?
• Foods rich in antioxidants may delay the
  progression of AMD
• Green leafy vegetables, berries, nuts, seaweed
  (?) Beta-carotene, omega 3 fatty acids, lutein
Which vitamins should I use?
• AREDS vitamins shown to be beneficial in
  patients with high risk AMD
• Vitamins slow the progression; they do not
  stop it
• Benefits of AREDS II not proven yet
Will the high dose of vitamins in the
 AREDS formula have side effects?
•   Vitamin A in smokers - lung cancer
•   Vitamins A and E stored in the liver - potential
    liver toxicity
•   Zinc - anemia if not taken with copper
•   Vitamin E - cardiovascular issues not typical in
    dose prescribed, prostate cancer?
•   AREDS II - High dose Vitamin A removed; Lutein
    and Zeaxanthin added
Is AMD hereditary?
Are my children at risk?
•   Genetics of AMD is complex with many
    associated genes
•   Hereditary forms exist and can express
    themselves at different ages and in different
    degrees of severity
•   Environmental factors may trigger the disease in
    susceptible people
Known risk factors:
• Smoking, diet, exercise, obesity (BMI)
• Hypertension, Hypercholesterolemia

• Does this sound familiar??
Will I go totally blind?
•   AMD causes central vision loss with
    preservation of peripheral vision
•   90% of severe vision loss occurs in the wet
    (neovascular) type. 10% of patients have wet
    AMD
•   90% of patients have dry (atrophic) AMD. 10%
    of these with develop severe vision loss
Are there any
new treatments
 in the making?
•   Millions of dollars have been appropriated to
    AMD research
•   Medications based on a person’s individual
    genetic profile (pharmacogenomics)
•   Gene therapy to enhance a person’s own
    production of beneficial compounds
•   Retina implant / retinal prosthesis
•   Stem cells
•   Antioxidant eye drops for dry / atrophic AMD
Which treatment is best for me
                     and why?
•   Thermal “Hot” Laser (1970’s)
•   PDT “Cold” Laser (Visudyne)
•   Macugen - First anti-VEGF intraocular treatment
•   Lucentis - First to show improved vision
    95% stabilized 40% improved
•   Avastin - Cost
•   Eylea - Less frequent treatment
Argon/Krypton/Dye/Diode Laser

                                PDT Laser/Visudyne
Macugen
Lucentis/Avas
     tin
    Eylea
How many injections will I need? Is there a limit to the
  number of treatments I can get? What is the side
  effect of long-term treatment?
Lucentis Study - injection every 4 weeks. May still give best
   outcome

Treat and Extend protocol
Double-dosing

Combined PDT with anti-VEGF therapy
Risks: Infection, Retinal detachment, Cataract, Inflammation,
    Glaucoma

Therapy for a chronic condition requires long term treatment.
   Underlying degenerative changes will continue with potential
   gradual vision loss even with treatment.
What will happen if I do nothing?
• Natural history of AMD is to cause significant
  and permanent loss of central vision leading to
  legal blindness
Will changing my glasses help?
Multiple aids to help people with decreased vision with AMD:

•   Spectacle-mounted magnifiers

•   Hand-held and spectacle-mounted telescopes

•   Hand-held and stand magnifiers

•   Video magnification / Computer devices

•   Importance of illumination, brightness and increased contrast

•   Writings devices, voice command, “gadgets”

•   Importance of early evaluation when moderate vision loss by a
    low vision specialist

•   Devices require training; use of eccentric vision
Congratulations!

           You have now
            earned your
              diploma!
So What Have We
    Learned?
 Lory C. Snady-McCoy, MD
Answers to Common
      Questions
What defines macular degeneration?
What tests are used?
Why are they necessary in the
 diagnosis and management?
  Not everything that looks like
      macular degeneration
     is macular degeneration
   Importance of continued
      monitoring and care
50% chance of developing wet AMD
     in fellow eye over 5 years
What Should I Eat?
• Foods rich in antioxidants may delay the
  progression of AMD
• Green leafy vegetables, berries, nuts, seaweed
  (?) Beta-carotene, omega 3 fatty acids, lutein
Which vitamins should I use?
• AREDS vitamins shown to be beneficial in
  patients with high risk AMD
• Vitamins slow the progression; they do not
  stop it
• Benefits of AREDS II not proven yet
Will the high dose of vitamins in the
 AREDS formula have side effects?
•   Vitamin A in smokers - lung cancer
•   Vitamins A and E stored in the liver - potential
    liver toxicity
•   Zinc - anemia if not taken with copper
•   Vitamin E - cardiovascular issues not typical in
    dose prescribed, prostate cancer?
•   AREDS II - High dose Vitamin A removed; Lutein
    and Zeaxanthin added
Is AMD hereditary?
Are my children at risk?
•   Genetics of AMD is complex with many
    associated genes
•   Hereditary forms exist and can express
    themselves at different ages and in different
    degrees of severity
•   Environmental factors may trigger the disease in
    susceptible people
Known risk factors:
• Smoking, diet, exercise, obesity (BMI)
• Hypertension, Hypercholesterolemia

• Does this sound familiar??
Will I go totally blind?
•   AMD causes central vision loss with
    preservation of peripheral vision
•   90% of severe vision loss occurs in the wet
    (neovascular) type. 10% of patients have wet
    AMD
•   90% of patients have dry (atrophic) AMD. 10%
    of these with develop severe vision loss
Are there any
new treatments
 in the making?
•   Millions of dollars have been appropriated to
    AMD research
•   Medications based on a person’s individual
    genetic profile (pharmacogenomics)
•   Gene therapy to enhance a person’s own
    production of beneficial compounds
•   Retina implant / retinal prosthesis
•   Stem cells
•   Antioxidant eye drops for dry / atrophic AMD
Which treatment is best for me
                     and why?
•   Thermal “Hot” Laser (1970’s)
•   PDT “Cold” Laser (Visudyne)
•   Macugen - First anti-VEGF intraocular treatment
•   Lucentis - First to show improved vision
    95% stabilized 40% improved
•   Avastin - Cost
•   Eylea - Less frequent treatment
Argon/Krypton/Dye/Diode Laser

                                PDT Laser/Visudyne
Macugen
Lucentis/Avas
     tin
    Eylea
How many injections will I need? Is there a limit to the
  number of treatments I can get? What is the side
  effect of long-term treatment?
Lucentis Study - injection every 4 weeks. May still give best
   outcome

Treat and Extend protocol
Double-dosing

Combined PDT with anti-VEGF therapy
Risks: Infection, Retinal detachment, Cataract, Inflammation,
    Glaucoma

Therapy for a chronic condition requires long term treatment.
   Underlying degenerative changes will continue with potential
   gradual vision loss even with treatment.
What will happen if I do nothing?
• Natural history of AMD is to cause significant
  and permanent loss of central vision leading to
  legal blindness
Will changing my glasses help?
Multiple aids to help people with decreased vision with AMD:

•   Spectacle-mounted magnifiers

•   Hand-held and spectacle-mounted telescopes

•   Hand-held and stand magnifiers

•   Video magnification / Computer devices

•   Importance of illumination, brightness and increased contrast

•   Writings devices, voice command, “gadgets”

•   Importance of early evaluation when moderate vision loss by a
    low vision specialist

•   Devices require training; use of eccentric vision
Congratulations!

           You have now
            earned your
              diploma!
• Can only be used in patients with functioning inner
         retinal cells – for example AMD and RP
    • Uses an artificial means to detect light (video
                          camera)
    • Converts light energy into an electrical signal
  • Deliver the electrical signal to the retinal neurons
   other than photoreceptors to elicit activity that is
                  interpreted as vision
            • Vision equivalent to 20/1260
Retinal Implant
ARGUS II Implant




 60 electrodes (16 in
      ARGUS I)
250 electrode array in
    development
Subretinal implant
Microprocessor power
Emerging treatments for
        Dry AMD
Atrophic AMD   AL-8309B (Alcon Laboratories)



                •   2 year study evaluating an Eye drop
                    for dry AMD

                •   Primary endpoint is size of the
                    atrophic area

                •   Randomized, double blind

                •   772 patients

                •   Study completed July 2011
ACU-4429 - Acucela
    Phase II granted fast track status

Fenretinide
    Phase III began 2011
Emerging treatments for dry
     AMD

• Encapsulated human cells genetically modified
to secrete ciliary neurotrophic factor (CNTF)



• CNTF is a growth factor capable of rescuing
dying photoreceptors and protecting them from
degeneration
Emerging treatments
• OT- 551 Antioxidant Eye drops
       for Dry AMD
 • Othera Pharmaceuticals/ National Eye Institute
• A Multicenter, Proof-Of-Concept Study
 Of Intravitreal AL-78898A In Patients
 With Geographic Atrophy
 • Alcon
Human embryonic
   stem • Can differentiate into any of
        cells
                    the 220 cell type in the
                    human body
                •   Cells acquired from
                    unwanted or discarded
                    embryo
                •   Can propagate indefinitely
                •   Proposed for regenerative
                    medicine and tissue
                    replacement after injury or
                    disease
Stem Cell Research
• hES Cells modified to become RPE cells which
nourish damaged photorecptors

•First study of   human transplantation reported in
January 2012

• Study of safety and tolerability of subretinal
transplantation

•  2 patients studied, one with AMD and one with
Stargardt’s disease, vision did not worsen in either one

• No tumor formation or signs of rejection
Stem Cells – Current
• Rejection Problems
• Tumor formation
• Expense – producing and cultivating cell
  lines is costly
• Intellectual property rights
• Ethics
• No good animal models of macular
  degeneration

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Age related macula degen

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  • 5. Trouble with vision as we get older... • Is not always due to Age- related macular degeneration ! • Your doctor needs to perform a complete eye exam before reaching a diagnosis
  • 6. Eye Examination - Diagnosis Visual Acuity, Color Vision, Amsler Grid, Fundus Camera, Fluorescein Angiography, Optical Coherence Tomography
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  • 11. Drusen, the yellow spots so characteristic of Age-related Macular Degeneration
  • 12. Drusen are sometimes more easily detected On Color Fundus photos than doctor’s exam
  • 14. Colour photography is routinely undertaken with angiography. It helps to determine the nature of changes seen on the angiogram… particularly the cause of blocked fluorescence due to haemorrhage, pigment or other cause.
  • 15. Side by side comparison of the color photo and the fluorescein picture often complement each other !
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  • 20. Optical Coherence Tomography
  • 21. • Diagnostic imaging technique that examines living tissue non-invasively. It is based on a complex analysis of the reflection of low coherence radiation from the tissue under examination. • Real time cross sectional analysis
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  • 37. Macular Degeneration Leading cause of blindness in people over 55 • 1:10 people over 55 • Increases by a factor of 6 in people over 80 • 1.75 million Americans over age 40 have severe AMD • Estimated that 2.95 million people affected by 2020
  • 38. How Does the Eye Work?
  • 39. What is Macular Idiopathic • Central vision Degeneration? loss • Peripheral vision preserved • Two forms • Dry (Nonexudative) • 90% of people • Wet (Exudative) • Affects 10% of people
  • 40. What does my Doctor • • Drusen see? Metabolic waste products accumulating under the retina • Pigment clumping and atrophy • Death of support cells under retina • Geographic atrophy • Large areas of cell death • Hemorrhage (blood) • Hallmark of wet AMD
  • 41. My Doctor told me I have Macular Degeneration. Will I go blind? • Not necessarily • Only 10% develop the wet form which is responsible for 90% of blindness from AMD • Newer treatments may reduce risk of blindness • Only 10% of the dry patients will develop severe vision loss
  • 42. Risk factors for Vision • Non-modifiable: things we cannot loss change • Age • (If you figure out how to stop aging, please let me know) • Caucasian race • Female gender • Family history • 2.5x increased risk of first degree family relative
  • 43. Risk factors for Vision • Modifiable • Smoking Loss • Hypertension • High Cholesterol • Obesity • Sedentary Lifestyle • Heart Health= Eye
  • 44. What about those AREDS study • Vitamins? II Followed 3640 patients for average 6.3 years • AREDS • High doses of antioxidant vitamins • Started in 2006 – ongoing • Hope they will reduce oxidative stress in • Added macula • Lutein: forms macular • Vitamin A pigment • Vitamin C • Zeaxanthin • Vitamin E • Subtracted • Zinc: antioxidant in • Vitamin A: Potential conjunction with C and increased risk of lung E cancer in smokers • Copper
  • 45. Vitamins Not found helpful for everyone • No benefit • No AMD • Benefit: • Mild AMD • If everyone took AREDS, • Beneficial 300,000 people could be prevented from developing • Moderate AMD advanced AMD over 5 year • Severe AMD • Persons with wet AMD • Unfortunately will not stop progression of AMD in all • Benefit patients • Reduced risk of progression of AMD by 25% • Reduced risk of vision
  • 46. Which is the best Multivitamin? • They are all basically the same as long as they have the AREDS formula • Differences in brands • AREDS I vs AREDS II formula • Dosing • Price • Packaging • Advertising
  • 47. Stop Smoking Summary • Exercise • Heart Healthy Diet • Eat leafy green vegetables • Control blood pressure and cholesterol • AREDS Multivitamin if indicated by your eye doctor • Monitor vision and regular eye checks
  • 48.
  • 49.
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  • 52.
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  • 54.
  • 55.
  • 56. Macular Degeneration Leading cause of blindness in people over 55 • 1:10 people over 55 • Increases by a factor of 6 in people over 80 • 1.75 million Americans over age 40 have severe AMD • Estimated that 2.95 million people affected by 2020
  • 57. How Does the Eye Work?
  • 58. What is Macular Idiopathic • Central vision Degeneration? loss • Peripheral vision preserved • Two forms • Dry (Nonexudative) • 90% of people • Wet (Exudative) • Affects 10% of people
  • 59. What does my Doctor • • Drusen see? Metabolic waste products accumulating under the retina • Pigment clumping and atrophy • Death of support cells under retina • Geographic atrophy • Large areas of cell death • Hemorrhage (blood) • Hallmark of wet AMD
  • 60. My Doctor told me I have Macular Degeneration. Will I go blind? • Not necessarily • Only 10% develop the wet form which is responsible for 90% of blindness from AMD • Newer treatments may reduce risk of blindness • Only 10% of the dry patients will develop severe vision loss
  • 61. Risk factors for Vision • Non-modifiable: things we cannot loss change • Age • (If you figure out how to stop aging, please let me know) • Caucasian race • Female gender • Family history • 2.5x increased risk of first degree family relative
  • 62. Risk factors for Vision • Modifiable • Smoking Loss • Hypertension • High Cholesterol • Obesity • Sedentary Lifestyle • Heart Health= Eye
  • 63. What about those AREDS study • Vitamins? II Followed 3640 patients for average 6.3 years • AREDS • High doses of antioxidant vitamins • Started in 2006 – ongoing • Hope they will reduce oxidative stress in • Added macula • Lutein: forms macular • Vitamin A pigment • Vitamin C • Zeaxanthin • Vitamin E • Subtracted • Zinc: antioxidant in • Vitamin A: Potential conjunction with C and increased risk of lung E cancer in smokers • Copper
  • 64. Vitamins Not found helpful for everyone • No benefit • No AMD • Benefit: • Mild AMD • If everyone took AREDS, • Beneficial 300,000 people could be prevented from developing • Moderate AMD advanced AMD over 5 year • Severe AMD • Persons with wet AMD • Unfortunately will not stop progression of AMD in all • Benefit patients • Reduced risk of progression of AMD by 25% • Reduced risk of vision
  • 65. Which is the best Multivitamin? • They are all basically the same as long as they have the AREDS formula • Differences in brands • AREDS I vs AREDS II formula • Dosing • Price • Packaging • Advertising
  • 66. Stop Smoking Summary • Exercise • Heart Healthy Diet • Eat leafy green vegetables • Control blood pressure and cholesterol • AREDS Multivitamin if indicated by your eye doctor • Monitor vision and regular eye checks
  • 67. Age-related Macular Degeneration
  • 68. What is Age-Related Macular Degeneration? • Age-related macular degeneration (AMD) is a deterioration or breakdown of the eye's macula. • The macula is a small area in the retina — the light-sensitive tissue lining the back of the eye. • The macula is the part of the retina that is responsible for your central vision, allowing you to see fine details clearly. • American Academy of Ophthalmology
  • 69. What is going on in the eye?
  • 72. Genetics • DNA • All information of life encoded in DNA and expressed by proteins. • James Dewey Watson (US) & Francis Crick (UK) – Co-discoverers Structure of DNA 1953 • Awarded Nobel Prize in Physiology or Medicine 1962
  • 75. Genetic Basics…get small… • The human body is made up of trillions of Cells, • and with a few exceptions, each cell contains a Nucleus • and with a few exceptions, each nucleus contains a COMPLETE set of Chromosomes • And the chromosomes are made of DNA
  • 76. From DNA…to You and Me…
  • 77. …and smaller…From Cells to Nucleus to Chromosomes • Every human cell contains 23 pairs of chromosomes, for a total of 46 chromosomes. • Each Chromosome is made up of • a double- stranded, • Tightly-coiled, • helix of DNA
  • 79. …and smaller… • A CHROMOSOME contains hundreds to thousands of genes. • A GENE is a segment of DNA containing the code used to synthesize a protein. • A TRAIT is any gene-determined characteristic and is usually determined by more than one gene. • Some traits are caused by abnormal genes that are inherited or that are the result of a mutation.
  • 81. WhatStudies we know?… • Genetic do • 1988-2010 so far • DNA and Gene-Environment Data Collection • Twin Study – WWII Registry • 1997 Familial Aggregation Study – Relatives of Cases with Wet AMD. Relatives of controls. • 1997 Phenotypic Heterogeneity in Families with AMD • Johanna M. Seddon, M.D., Sc.M.
  • 82. What do we know?… so far • From the US Twin Study, we’ve learned • Substantial Genetic Component – • 46 to 71% of the variation in phenotype • Important Environmental Influence: 19 to 37%
  • 83. OK…what “Genetic Factors”?... • It’s not so much as a “bad” gene, but rather • Variations of “normal genes” that influence the risk of developing AMD. • Specific DNA chromosomes associated with the defective genes are located on chromosomes • 1q and 10q
  • 85. Genes associated…so • CFH – Y402H far • CFH – rs1410996 • Hepatic Lipase C (LIPC) • CFB • CETP • C2 • ABCA1 • Complement Component • TIMP 3 / SYN3 3 (C3) • VEGFA • Complement Factor I CFI • COL10A1 • ARMS2/HTRA1 gene • COL8A1 region • TNFRSF10A
  • 86. Gene location… leads to therapy • For example, the VEGFA locus and the development of • Avastin • Lucentis and recently • Eylea
  • 87. Antigen/Antibody cell membrane Bayer Healthcare – Bayer Schering Pharma
  • 89. Relative risk of Genetic loci Nature Genetics 38: 1057, 2006
  • 90. Epidemiology • Epidemiology is the study of • the distribution and patterns of health-events, • health-characteristics and • their causes or influences in well-defined populations.
  • 91. Epidemiology • It is our principal method of public health research, and • helps us make policy decisions as well as evidence-based medicine • by identifying risk factors for disease and helps us with recommendations for appropriate preventive medicine.
  • 92. Epidemiologic studies looking at • Meta-analysis of 15 genome-wide the genetics of AMD association studies • Began in 2009 • 8,000 cases and 50,000 controls • 19 groups from 13 countries • 19 genomic loci associated with AMD • Six novel loci not previously associated with disease • Pathways similar to other known genes. • ASHG Oct 2011
  • 93. Epidemiology and Causation Risk factors for cardiovascular disease (CVD) are associated with age-related AMD; • Smoking • Dietary antioxidants, fats, omega-3 fatty acids • Physical activity • Obesity, abdominal adiposity • Hypertension • Cholesterol • CRP (C-reactive protein)
  • 94. Epidemiology and Causation • For the homozygous (pure trait) risk genotype • Risk of AMD was • Increased for BMI (Body Mass Index) and • Increased with smoking
  • 95. So what can we say? • Genetic mutations make us susceptible to the development of AMD • Multiple genetic factors are at work • Risk profiles help us to change life style to decrease the chance of AMD development • Which leads us to…
  • 96. The big question (at least for the retina specialists) • What information from • TV, • Radio, • Internet, • YouTube, • Email, • Fax, Photocopy, Text, Blog, Wikipedia….etc • Should You believe?
  • 97. Types of Optical Devices Near Devices
  • 100. Illuminated Stand Magnifier
  • 101. Hand Held Pocket Magnifier
  • 104. Types of Optical Devices Distance Devices
  • 111. Getting the Right Device(s)devices • Because there is such a variety of available, it can be somewhat overwhelming to locate the correct device. • Individuals should receive an evaluation from an Optometrist who specializes in treating individuals with low vision. • This assures that the individual receives the correct device with consideration of all goals and needs.
  • 112. What’s New in Low Vision Technology
  • 113. Handheld Magnifying Cameras • Miracle Mouse Electronic TV Magnifier • MonoMouse-RM B&W Electronic Magnifier • Max Digital Color Magnifier • Primer 2 Color Electronic Magnifier
  • 114. Traditional CCTV Video Magnifiers reading • Increased speed • Increased duration • Reduced visual effort • Comfortable • Desktop systems working position • Variable Magnification • Color, W/B, B/W • Large Field of View
  • 115. Magnifiers Integrated LCD • Available from all major vendors Displays • Sizes: 17” to 24” + with WS • Adjustable height screens • High contrast • Flicker free • Improved response rates • Well received by consumers
  • 117. Video Magnification • Variable Systems Magnification • Adjustable brightness • Selectable contrast setting • Wide field of view for better viewing abilities
  • 122. Portable Video • More Compact, 7-inch screens Magnifiers • Extensive reading tasks • Two handed use • Optelec Traveler • FS Sapphire • EV Amigo • Fusion 7” LCD Portable Video Magnifier
  • 123. Compact Video Magnifiers: • Variable Magnification • Replaceable Batteries • Longer battery life • Image Freeze • Color Select • Light off mode: for reading cell phones, other displays • Writing Cameras • Distance Viewing • Smaller Designs
  • 124. Compact/Pocket Video Magnifiers • Advantages • Wide field of view • Image at display level • Use at arms-length • Reverse contrast available • Cell phone, PDA displays! • Short-term writing tasks • Kids reading comic books under the blanket
  • 125. Compact Systems with Distance Capabilities • Near and distance capabilities • Read mail, products on a shelf and get a better view of distance object. • Allows users to see an object that is at distance close up on the screen • Limited clarity and magnification at a distance
  • 126. GW Micro Sense View Duo • Two cameras • Stores up to 20 images • 3X to 13X magnification • Lights off mode • Image panning • 7 Viewing modes, 4 Brightness levels • 4.5 hour battery life, integrated, rechargeable • Cost: $1299.00
  • 128. Desktop Video Magnifiers • Full Size screen Features • Full Features • Remote Controllable • Near and Distance Viewing • For Classroom, Conference or In- Service Training Activities • Transportable “Luggable” • AC or optional battery
  • 132. Laptop Compatible Video Magnifiers • Most students use laptop computers • Video magnification could be helpful • Portable camera using notebook display
  • 133. Clarity PCMate • Powered by USB • Requires 2 USB 2 ports • Sequence, setup • Remote control camera • Keyboard controlled software
  • 134. Optelec ClearNote • Ease of camera aiming • Built in Light source • Camera control positioning • Keyboard controlled software • Keyboard controlled camera • Dolphin, Zoom Text compatible
  • 135. Freedom Scientific Onyx PC Edition arm and flex • Swing arm models • Remote control • MAGic with EyeMerge software included • Camera can be powered by USB port • Distance, desktop, self view modes • Cost: $2895.00
  • 136. Specialty Video Magnifiers • Flipper • Shoppa
  • 137. Headborne Low Vision System • Eyetonomy SightMate LV920 • www.vuzix.com/medical/lv920.h tml • Zoom at near: 3X optical, 4X digital • Zoom at distance: 0.5X to 6X • Edge contrasting, Inverse mode • Sleek design, 8 ounces • $3499.00 • Not yet available for purchase or review
  • 138.
  • 139. Scan and Read Devices • Kurzweil 1000 (software) • ABiSee Zoom-Ex, EyePal Vision (portable, Solo) • FS Pearl (laptop compatible) • KNFB Reader (smartphone) • AI Squared ZoomReader (app)
  • 140. Assessment functioning • Evaluation of visual Factors • Response to low vision approaches • Analysis of tasks, task specific • Required performance level • Integration of systems • Training • Trial of system; speed, duration, productivity • Modifications • Provision
  • 141. Types of Optical Devices Near Devices
  • 144. Illuminated Stand Magnifier
  • 145. Hand Held Pocket Magnifier
  • 148. Types of Optical Devices Distance Devices
  • 155. Getting the Right Device(s)devices • Because there is such a variety of available, it can be somewhat overwhelming to locate the correct device. • Individuals should receive an evaluation from an Optometrist who specializes in treating individuals with low vision. • This assures that the individual receives the correct device with consideration of all goals and needs.
  • 156. What’s New in Low Vision Technology
  • 157. Handheld Magnifying Cameras • Miracle Mouse Electronic TV Magnifier • MonoMouse-RM B&W Electronic Magnifier • Max Digital Color Magnifier • Primer 2 Color Electronic Magnifier
  • 158. Traditional CCTV Video Magnifiers reading • Increased speed • Increased duration • Reduced visual effort • Comfortable • Desktop systems working position • Variable Magnification • Color, W/B, B/W • Large Field of View
  • 159. Magnifiers Integrated LCD • Available from all major vendors Displays • Sizes: 17” to 24” + with WS • Adjustable height screens • High contrast • Flicker free • Improved response rates • Well received by consumers
  • 161. Video Magnification • Variable Systems Magnification • Adjustable brightness • Selectable contrast setting • Wide field of view for better viewing abilities
  • 166. Portable Video • More Compact, 7-inch screens Magnifiers • Extensive reading tasks • Two handed use • Optelec Traveler • FS Sapphire • EV Amigo • Fusion 7” LCD Portable Video Magnifier
  • 167. Compact Video Magnifiers: • Variable Magnification • Replaceable Batteries • Longer battery life • Image Freeze • Color Select • Light off mode: for reading cell phones, other displays • Writing Cameras • Distance Viewing • Smaller Designs
  • 168. Compact/Pocket Video Magnifiers • Advantages • Wide field of view • Image at display level • Use at arms-length • Reverse contrast available • Cell phone, PDA displays! • Short-term writing tasks • Kids reading comic books under the blanket
  • 169. Compact Systems with Distance Capabilities • Near and distance capabilities • Read mail, products on a shelf and get a better view of distance object. • Allows users to see an object that is at distance close up on the screen • Limited clarity and magnification at a distance
  • 170. GW Micro Sense View Duo • Two cameras • Stores up to 20 images • 3X to 13X magnification • Lights off mode • Image panning • 7 Viewing modes, 4 Brightness levels • 4.5 hour battery life, integrated, rechargeable • Cost: $1299.00
  • 172. Desktop Video Magnifiers • Full Size screen Features • Full Features • Remote Controllable • Near and Distance Viewing • For Classroom, Conference or In- Service Training Activities • Transportable “Luggable” • AC or optional battery
  • 176. Laptop Compatible Video Magnifiers • Most students use laptop computers • Video magnification could be helpful • Portable camera using notebook display
  • 177. Clarity PCMate • Powered by USB • Requires 2 USB 2 ports • Sequence, setup • Remote control camera • Keyboard controlled software
  • 178. Optelec ClearNote • Ease of camera aiming • Built in Light source • Camera control positioning • Keyboard controlled software • Keyboard controlled camera • Dolphin, Zoom Text compatible
  • 179. Freedom Scientific Onyx PC Edition arm and flex • Swing arm models • Remote control • MAGic with EyeMerge software included • Camera can be powered by USB port • Distance, desktop, self view modes • Cost: $2895.00
  • 180. Specialty Video Magnifiers • Flipper • Shoppa
  • 181. Headborne Low Vision System • Eyetonomy SightMate LV920 • www.vuzix.com/medical/lv920.h tml • Zoom at near: 3X optical, 4X digital • Zoom at distance: 0.5X to 6X • Edge contrasting, Inverse mode • Sleek design, 8 ounces • $3499.00 • Not yet available for purchase or review
  • 182.
  • 183. Scan and Read Devices • Kurzweil 1000 (software) • ABiSee Zoom-Ex, EyePal Vision (portable, Solo) • FS Pearl (laptop compatible) • KNFB Reader (smartphone) • AI Squared ZoomReader (app)
  • 184. Assessment functioning • Evaluation of visual Factors • Response to low vision approaches • Analysis of tasks, task specific • Required performance level • Integration of systems • Training • Trial of system; speed, duration, productivity • Modifications • Provision
  • 185. Pharmacogenomics • Treatment based on genetic profile • “Personalized medicine” • AREDS – 70% with certain gene did not progress (vs. 11% without) • Relationship between certain genes and outcomes with Lucentis and Avastin
  • 186. Gene Therapy for retinal diseases
  • 187. Trials Phase I Dose Escalation Safety Study of RetinoStat in Advanced Age- Related Macular Degeneration (AMD) Oxford BioMedica • Subretinal injection of virus vector that produces anti-VEGF proteins • Safety and Efficacy Study of rAAV.sFlt-1 in Patients With Exudative Age-Related Macular Degeneration • Intravitreal injection of a non-pathogenic virus to express a therapeutic protein within the eye
  • 188. Wet Dry AMD Atrophi c
  • 189. So What Have We Learned? Lory C. Snady-McCoy, MD
  • 190. Answers to Common Questions What defines macular degeneration?
  • 191. What tests are used? Why are they necessary in the diagnosis and management? Not everything that looks like macular degeneration is macular degeneration Importance of continued monitoring and care 50% chance of developing wet AMD in fellow eye over 5 years
  • 192. What Should I Eat?
  • 193. • Foods rich in antioxidants may delay the progression of AMD • Green leafy vegetables, berries, nuts, seaweed (?) Beta-carotene, omega 3 fatty acids, lutein
  • 195. • AREDS vitamins shown to be beneficial in patients with high risk AMD • Vitamins slow the progression; they do not stop it • Benefits of AREDS II not proven yet
  • 196. Will the high dose of vitamins in the AREDS formula have side effects? • Vitamin A in smokers - lung cancer • Vitamins A and E stored in the liver - potential liver toxicity • Zinc - anemia if not taken with copper • Vitamin E - cardiovascular issues not typical in dose prescribed, prostate cancer? • AREDS II - High dose Vitamin A removed; Lutein and Zeaxanthin added
  • 197. Is AMD hereditary? Are my children at risk?
  • 198. Genetics of AMD is complex with many associated genes • Hereditary forms exist and can express themselves at different ages and in different degrees of severity • Environmental factors may trigger the disease in susceptible people
  • 199. Known risk factors: • Smoking, diet, exercise, obesity (BMI) • Hypertension, Hypercholesterolemia • Does this sound familiar??
  • 200. Will I go totally blind? • AMD causes central vision loss with preservation of peripheral vision • 90% of severe vision loss occurs in the wet (neovascular) type. 10% of patients have wet AMD • 90% of patients have dry (atrophic) AMD. 10% of these with develop severe vision loss
  • 201. Are there any new treatments in the making?
  • 202. Millions of dollars have been appropriated to AMD research • Medications based on a person’s individual genetic profile (pharmacogenomics) • Gene therapy to enhance a person’s own production of beneficial compounds • Retina implant / retinal prosthesis • Stem cells • Antioxidant eye drops for dry / atrophic AMD
  • 203. Which treatment is best for me and why? • Thermal “Hot” Laser (1970’s) • PDT “Cold” Laser (Visudyne) • Macugen - First anti-VEGF intraocular treatment • Lucentis - First to show improved vision 95% stabilized 40% improved • Avastin - Cost • Eylea - Less frequent treatment
  • 204. Argon/Krypton/Dye/Diode Laser PDT Laser/Visudyne
  • 205. Macugen Lucentis/Avas tin Eylea
  • 206. How many injections will I need? Is there a limit to the number of treatments I can get? What is the side effect of long-term treatment? Lucentis Study - injection every 4 weeks. May still give best outcome Treat and Extend protocol Double-dosing Combined PDT with anti-VEGF therapy Risks: Infection, Retinal detachment, Cataract, Inflammation, Glaucoma Therapy for a chronic condition requires long term treatment. Underlying degenerative changes will continue with potential gradual vision loss even with treatment.
  • 207. What will happen if I do nothing? • Natural history of AMD is to cause significant and permanent loss of central vision leading to legal blindness
  • 208. Will changing my glasses help? Multiple aids to help people with decreased vision with AMD: • Spectacle-mounted magnifiers • Hand-held and spectacle-mounted telescopes • Hand-held and stand magnifiers • Video magnification / Computer devices • Importance of illumination, brightness and increased contrast • Writings devices, voice command, “gadgets” • Importance of early evaluation when moderate vision loss by a low vision specialist • Devices require training; use of eccentric vision
  • 209. Congratulations! You have now earned your diploma!
  • 210. So What Have We Learned? Lory C. Snady-McCoy, MD
  • 211. Answers to Common Questions What defines macular degeneration?
  • 212. What tests are used? Why are they necessary in the diagnosis and management? Not everything that looks like macular degeneration is macular degeneration Importance of continued monitoring and care 50% chance of developing wet AMD in fellow eye over 5 years
  • 213. What Should I Eat?
  • 214. • Foods rich in antioxidants may delay the progression of AMD • Green leafy vegetables, berries, nuts, seaweed (?) Beta-carotene, omega 3 fatty acids, lutein
  • 216. • AREDS vitamins shown to be beneficial in patients with high risk AMD • Vitamins slow the progression; they do not stop it • Benefits of AREDS II not proven yet
  • 217. Will the high dose of vitamins in the AREDS formula have side effects? • Vitamin A in smokers - lung cancer • Vitamins A and E stored in the liver - potential liver toxicity • Zinc - anemia if not taken with copper • Vitamin E - cardiovascular issues not typical in dose prescribed, prostate cancer? • AREDS II - High dose Vitamin A removed; Lutein and Zeaxanthin added
  • 218. Is AMD hereditary? Are my children at risk?
  • 219. Genetics of AMD is complex with many associated genes • Hereditary forms exist and can express themselves at different ages and in different degrees of severity • Environmental factors may trigger the disease in susceptible people
  • 220. Known risk factors: • Smoking, diet, exercise, obesity (BMI) • Hypertension, Hypercholesterolemia • Does this sound familiar??
  • 221. Will I go totally blind? • AMD causes central vision loss with preservation of peripheral vision • 90% of severe vision loss occurs in the wet (neovascular) type. 10% of patients have wet AMD • 90% of patients have dry (atrophic) AMD. 10% of these with develop severe vision loss
  • 222. Are there any new treatments in the making?
  • 223. Millions of dollars have been appropriated to AMD research • Medications based on a person’s individual genetic profile (pharmacogenomics) • Gene therapy to enhance a person’s own production of beneficial compounds • Retina implant / retinal prosthesis • Stem cells • Antioxidant eye drops for dry / atrophic AMD
  • 224. Which treatment is best for me and why? • Thermal “Hot” Laser (1970’s) • PDT “Cold” Laser (Visudyne) • Macugen - First anti-VEGF intraocular treatment • Lucentis - First to show improved vision 95% stabilized 40% improved • Avastin - Cost • Eylea - Less frequent treatment
  • 225. Argon/Krypton/Dye/Diode Laser PDT Laser/Visudyne
  • 226. Macugen Lucentis/Avas tin Eylea
  • 227. How many injections will I need? Is there a limit to the number of treatments I can get? What is the side effect of long-term treatment? Lucentis Study - injection every 4 weeks. May still give best outcome Treat and Extend protocol Double-dosing Combined PDT with anti-VEGF therapy Risks: Infection, Retinal detachment, Cataract, Inflammation, Glaucoma Therapy for a chronic condition requires long term treatment. Underlying degenerative changes will continue with potential gradual vision loss even with treatment.
  • 228. What will happen if I do nothing? • Natural history of AMD is to cause significant and permanent loss of central vision leading to legal blindness
  • 229. Will changing my glasses help? Multiple aids to help people with decreased vision with AMD: • Spectacle-mounted magnifiers • Hand-held and spectacle-mounted telescopes • Hand-held and stand magnifiers • Video magnification / Computer devices • Importance of illumination, brightness and increased contrast • Writings devices, voice command, “gadgets” • Importance of early evaluation when moderate vision loss by a low vision specialist • Devices require training; use of eccentric vision
  • 230. Congratulations! You have now earned your diploma!
  • 231. • Can only be used in patients with functioning inner retinal cells – for example AMD and RP • Uses an artificial means to detect light (video camera) • Converts light energy into an electrical signal • Deliver the electrical signal to the retinal neurons other than photoreceptors to elicit activity that is interpreted as vision • Vision equivalent to 20/1260
  • 233. ARGUS II Implant 60 electrodes (16 in ARGUS I) 250 electrode array in development
  • 236.
  • 237. Emerging treatments for Dry AMD Atrophic AMD AL-8309B (Alcon Laboratories) • 2 year study evaluating an Eye drop for dry AMD • Primary endpoint is size of the atrophic area • Randomized, double blind • 772 patients • Study completed July 2011
  • 238. ACU-4429 - Acucela  Phase II granted fast track status Fenretinide  Phase III began 2011
  • 239. Emerging treatments for dry AMD • Encapsulated human cells genetically modified to secrete ciliary neurotrophic factor (CNTF) • CNTF is a growth factor capable of rescuing dying photoreceptors and protecting them from degeneration
  • 240. Emerging treatments • OT- 551 Antioxidant Eye drops for Dry AMD • Othera Pharmaceuticals/ National Eye Institute • A Multicenter, Proof-Of-Concept Study Of Intravitreal AL-78898A In Patients With Geographic Atrophy • Alcon
  • 241. Human embryonic stem • Can differentiate into any of cells the 220 cell type in the human body • Cells acquired from unwanted or discarded embryo • Can propagate indefinitely • Proposed for regenerative medicine and tissue replacement after injury or disease
  • 243. • hES Cells modified to become RPE cells which nourish damaged photorecptors •First study of human transplantation reported in January 2012 • Study of safety and tolerability of subretinal transplantation • 2 patients studied, one with AMD and one with Stargardt’s disease, vision did not worsen in either one • No tumor formation or signs of rejection
  • 244. Stem Cells – Current • Rejection Problems • Tumor formation • Expense – producing and cultivating cell lines is costly • Intellectual property rights • Ethics • No good animal models of macular degeneration

Notes de l'éditeur

  1. Amsler grids are everywhere. Check the vision in each eye daily.
  2. Throughout the history of medicine, doctors have observed treatment variability among patients which we know now to be due to genetic differences. Why some people respond well to one drug and others don’t or why some people develop certain side effects and others don’t is due to small differences in genetic make up between us. Some preliminary pharmacogenetic relationships have been reported to exist between genetic risk factors associated with AMD and the response to treatment. These pharmacogenetic associations could be used in the future to create a personalized therapeutic plan whereby patients with different genotypes are offered different treatments.
  3. So how can we use our knowledge of genetics to actually treat patients. Many diseases come about b/c of an abnormal protein produced by a faulty gene. Gene therapy aims to alter or stop the formation of proteins that lead to disease. This approach is still a long way off in treating AMD but is being used to treat other eye diseases. On the other hand creating a so called “biofactory” by injecting genes that produce proteins such as anti-VEGF compounds drugs would obviously be very helpful for patients with wet AMD. An injection every year or two rather than monthly is not an unrealistic possibility.
  4. The wet form of AMD with bleeding and scarring (shown in the top picture) and the Atrophic form (bottom picture) with thinning and and loss of retinal tissue are the primary causes of severe loss of vision in pts with macular degeneration. The treatments I’ll discuss here pertain primarily to this subset of patients. Both of these forms of AMD will become less prevalent as we become better at indentifying those patients who are at greatest risk and as preventative treatments become available.
  5. Three models are now in development or testing. Model 1, with 16 electrodes, has been implanted in six patients. As of mid-July 2009, a second model integrating a 60-electrode array has been implanted in 30 human subjects domestically and internationally. A third model, an array with a higher electrode count, is under development.
  6. May 2012 – details released of a wireless, subretinal 256 channel implant being developed in Boston. Disadvantages: more complex surgical procedure. All the implant designs in development have advantages and disadvantages not the least of which is the complexity of the surgical procedure, the lack of long term follow-up and cost ($100K)
  7. Moore’s law loosely defined says that the power of a computer chip doubles approximately every 2 years. Timeline for progress of the artificial retina. Progress started with the installation of the first 16-electrode device in 2002, which restored light perception and the ability to perform simple visual spatial and motion tasks (hand motion level). Theoretically, there is improvement with 60 and 200+ electrode implants (finger count level) with the possibility of face recognition and reading ability with a 1000+ electrode device. (Left scale) Progression in vision from bare hand motion to face recognition. At 20/200 visual acuity, large letters can be recognized. At 20/20, small letters can be seen with good reading ability. (Right scale) Number of patients potentially helped with the different generations of prosthetic devices.
  8. Pixels (short for picture element) are the smallest controllable element of a digital image. It is a single point in a graphic image. The more pixels on the display the higher the resolution. Examples of pixilated vision. Lower resolution may allow crude shape recognition, but increasing resolution can lead to reading letters on an eye chart and face recognition.
  9. AL-8309 protects the photoreceptors and RPE cells from photo-oxidative stress and may be a useful pharmacotherapy for AMD
  10. Reducing the speed of the visual cycle has been shown to protect the retina from light damage, improve retinal vasculature (the arrangement of blood vessels) and reduce the accumulation of retinal-related toxic by-products, including A2E, which is implicated in AMD. RPE cells continually consume the tips of Photoreceptors as they grow (at a constant rate of speed), accumulating toxic by-products of the visual cycle. ACU 4429 slows Visual cycle modulation resulting in a decrease of toxic by-product accumulation, and a slowing of AMD.
  11. Dry or atrophic AMD has become an orphan disease – we know a lot about it but we have no effective treatments. My point is not to overwhelm you with technical information but to highlight the exciting and extensive ongoing research in dry AMD.
  12. This model consists of rats with a genetic disorder where they do not have one of the enzymes needed for the RPE cell layer to function. As a result, the rats become blind due to deterioration of their photoreceptors. Following transplantation of human ES cells, injected into the sub-retinal area of the eye, rats displayed recovery of their photoreceptors and improved visual ability compared to the control rats, which were injected with a mock treatment and showed no significant improvement.  
  13. RPE dysfunction is the most common cause of blindness in people over 60 in the United States, and it affects over 30 million people worldwide.