Hans F. Lessmann, O.D., F.C.O.V.D. Vision Development Institute, P.C. Trisomy 21 Research Conference September 13-14, 2003. New Orleans, LA

Reproduced with the permission of the author. © 2002. All rights reserved. This material may not be published, broadcast, rewritten or redistributed by any means mechanical or electronic without the explicit written permission of the author.

Enhancement because:

• Vision is not static, therefore changeable
• Vision can improve
• True for all of us including people with Down syndrome
• Given vision is alterable, it can also get worse, and we want to avoid that

The Dreaded "Lazy Eye" Syndrome

Today's Objective

• To provide a sense of what it is like to see through the eyes of a person with Down syndrome
• To virtually crawl inside their head and look out as they would with the same sense, perception, and understanding
• Such that we may understand them better

Take Home Points on Vision and Down syndrome

• Vision is complex, hence problematic for DS
• They often have blur especially at near
• Strabismics often have chronic double vision
• Simple optometric interventions may make a big difference in subtle ways
  • Bifocals for near blur
  • Patching for diplopia
  • And yes, even contact lenses
• Glasses can be assessed accurately without consistent cooperation from the patient!!!
• What to ask your eye doctor?

About the Presenter

• Developmental Optometrist
• Specialize in Vision Therapy
• Consultant to:
  • Easter Seals School for children with special needs
  • Warner House Clinic for the study & Tx of Trisomy Disorders
• See few patients, yet spend lots of time with each
• Shared information is from both Clinical and Personal Experience
• Theoretical Basis: Piaget - focus on the person
• Father of Jessica (13) with Down syndrome

Dedication

• My daughter "Jessica"
• and all individuals, organizations, and families involved with intellectual disabilities
• And the Refreshing Perspective they give us
• Scooby-Doo

Why Scooby Doo?

• Gets lost
• Gets scared
• Often misunderstood
• Talks funny
• Happy to help
• Happy to be found
• Likes Scooby-Snacks
• Like people with Down syndrome

Key Question

Scooby Doo
Where are you?

To see what they see we must first:

• Know where they are
• Understand what they know and don't know
• Understand their values, concerns, and confusions
• In short, we must live where they live to see what they see
• This should then help us, help them

What is the cause?

• Their conceptual gap/deficit is difficult for us to relate to.
• Their perceived values and/or problems differ from ours.
• Their ability to adapt and reconcile their confusions is compromised.
• Thus, our prescribed interventions may exceed their level or pursue disparate paths.

Premise for People with Down syndrome

• Behaviors are logical and justified when we consider the concepts and perceptions they operate from.
• However, these concepts and perceptions are often inaccurate and therefore misleading.
• They mislead themselves as well as others.
• They are as mature as their development.
• They can only do and what they know.
• And they can only know what they have experienced.

Perspective

• It is not necessary to have a family member to understand people with Down syndrome
• People with Down syndrome need to grow.
• People with Down syndrome need to be accepted.
• Improvements are possible with appropriate interventions and understanding by all involved.

Why be intelligent?

What is the impetus for growth? —Jean Piaget

Jessica's Team

Common Issues

• Activities of daily living:
  • Eating, Dressing, Hygiene
• Limited energy and physical strength
• Effective communication
• Appropriate social behavior
• Confusion over money and quantities
• "Time management"-big problem

Money, Math & Quantities

• Concepts are highly abstract
• A function of visual thinking/visualization and logic
• Hence, such understanding difficult if not impossible to acquire
• Math performance generally resigned to counting
  • like reciting a bland poem over & over
• What's up with this smaller dime trumping the bigger nickel?

Time Concept

• A further abstraction of Quantity
• Difficulty managing Math, even with manipulatives, which is something concrete
• How can we possible understand the intangible passage, management, and adherence to the quantification of time — the clock?
• Other than straight, specific memorization of tasks "I'll see you at 10 o'clock any time"?!?!

Time Perception & Behavior

• Stuck in the Present!
• Difficulty reflecting off the Past
• And projecting into the future
• Hence, nothing else can be done until the present issue is resolved.
• Time marked by events (i.e. definition of time)
• Need for strict routines
• Difficulty adapting to new schedules
• Difficulty being on time (i.e. morning prep)
• Definitely does not relate to the care givers temporal orientation, hence conflict!

Happiness Defined

• Defined as a function of reconciling ones conflicts and/or confusions.
• They are often confused and may or may not know it.
• If they know it or their supervising adults bagger them about it, they are UNHAPPY!
• If they don't know it they are HAPPY!
• Ignorance is Bliss!
• But, they often get stuck on a conflict/confusion

Well, What about Vision?

What Problems do we see with Vision and Down Syndrome?

Ocular Stats on Children with Down Syndrome

• 70% poor visual acuity
• 38% strabismus
• 60% astigmatism
• 40% hypermetropia — farsighted
• 3% myopia — nearsighted
• 15% kerataconus
• www.specialolympics.org Health Research status Report, Yale Study 2000
• Stats highly variable between studies

Hearing Loss in Children with DS

• 1993 Journal of Pediatrics Vol. 123, Roizen NJ.
  • 47 patients ages 2 mos to 3.5 yrs
  • Tested brain stem responses as opposed to behavioral testing
• Moderate loss in 27%
• Profound loss in 6%

Out of Focus & Instability

• Much like a camcorder in dim light the automatic focus becomes unstable
• This can happen any time or all the time
• At such time one must wait until it stabilizes before recording, just as a child might look away, become stubborn, just stop, or plug along inaccurately and inefficiently
• The person with DS may be unaware of the problem and hence may be confused and disgruntled by the insistence of adult supervisors to complete some task
• Generally best visual acuity ~ 20/40

Demonstration of Poor Fixation and /or Tracking

Demonstration of Poor Eye Teaming

Binocularity — "it's a miracle"

• Aiming: Both eyes must precisely hit the target
  • like two people aiming camcorders in the exact same spot
• Focusing: Both must be in focus
  • like focusing binoculars
• Z-axis convergence & divergence
  • while maintaining the aiming and focusing
• Yoked movements all positions in space
• Execute the above while maintaining over time
• And holding attention to the task at hand

Key Visual Skills, besides Acuity

• Fixation
• Tracking
• Eye Teaming-convergence
• Focus Accuracy
• Near Acuity
• Depth Perception
• Visual Perception
• Efficiency — maintain over time!

Vision Facts

• 60%-80% of learning is mediated visually
• 50% of all the sensory cells in the body lie in the two retini.
• The retina is the most metabolically active tissue in the body.
• The Optic Nerve is by far the thickest Cranial Nerve (~4mm vs. a sliver for the vestibular)
• Most highly developed sense

A Quote from Psychology

...."Vision is our most dominant sense, and this is reflected in our own internal hardware, since more of the brain is devoted to processing vision than all the other senses put together." p 151

"The Right Mind" published by Harcourt 1997 by psychologist, Dr. Robert Ornstein

Vision's Uniqueness

• Dual input — single precept
• Tube demonstration
• Allows passive distal monitoring
  • i.e. inactive, quiet objects
  • Just must reflect light
• Auditory — object must make noise
• Tactile — object must be within reach
• Nasal — object must emit an odor
• Vision will incorporate all above sensations in one visualization

How Does Vision Develop?

• Last to develop
• Must rise as the dominant manager of sensory and information processing
• Only starts to refine itself after age 10!
• First to go when person compromised
  • fatigue or toxicity
• Although it is dominant,
• It is NOT independent!

Synaptic Density as an Indicator of Sensory Development Chronology

Vision Development Depends On:

• Sound biological infrastructure
  • hence nutrition is vital
• Biofeedback, Biofeedback, Biofeedback
• Oral Thinking - first exploratory tool (i.e. hand regard)
• General & Fine Motor Thinking
• Sound body schema as the body is the primary object to reference all other objects spatially and otherwise
• Lots of movement required!!! Hence problem with low tone
• Vestibular development
• Sound image optics of the eye
  • hence need optical correction early as required
• Straight eyes fusion of the two images

Optometric Significance

• Visual development summates and emerges from sensory and cognitive growth to ultimately be expressed in human function.
• Therefore:
• Visual Function ~ Human Function

What two human functions are frequently most affected in challenged children?

1. Speech
2. Vision
3. However, I would reverse the above
4. Speech is obvious, because we hear it
5. But Vision is covert in it's function as well as it's dysfunction,
6. Hence it's dysfunction is hidden

What is covert about vision?

Can you see this?

Sight

• Equates or approximates to visual acuity (i.e. 20/20)
• The biological process of converting light energy to neural energy
• Refers to the integrity of the sensory end organ the eye

Vision

• What is done with the neural energy
• A learned and developmental process
• More than mere visual acuity
• Is a human process that utilizes light energy to direct and guide meaningful action and thought.
• Incorporates all other senses
• Coordinates and feeds back with Vestibular as well as other senses
• Must become the dominant sense!

20/20 is not Perfect Vision!!!

• "Acuity is not the sum total of visual function"
• However it is primarily the only standard of visual function universally utilized
• Used to qualify for vision services
• Other than eye health it is the primary focus of eye care

What's else is the Problem?

• Ocular Health Dx =/= Academic Performance
• Often the Medical Ocular Diagnosis has little if any correlation to ocular function or academic performance.
• Glaucoma, Field loss, Conjunctivitis, even strabismus, means what in terms of classroom performance?

What are the Unique Optometric Considerations for individuals with DS?

The Problem of Blur & The Problem of Double Vision

The Problem of Blur

• Blur is not a problem
• Blur is accepted
• Things just are
• Not a function of the person, their eyes, or the glasses.
• No awareness of cause and effect
• Hence, no impetus to correct
• They just don't do
• Or they do poorly without realizing it

Close Working Distance

Contrast Problem: Pencil vs. Print

Cardiff University, University of Wales College of Medicine, & others DS Accommodation Study

• "Accommodative and Refractive Error in Children with Down Syndrome: Cross-Sectional and Longitudinal Studies" by Mary Cregg, J. Margaret Woodhouse & others (Investigative Ophthalmology and Visual Science. 2001;42:55-63)
• "Conclusions: In children with Down syndrome, underaccommodation is substantial, (regardless of their glasses correction) ...(they) may have the physical capacity to respond...but the neural control of the system has an anomalous (inaccurate) set point... This has important implications...in a learning environment, because near vision is consistently out of focus."

How would I then characterize the visual system of one with DS

Unstable & Fragile

Focus Analogy

Like an end stage adult presbyope
One who has worn bifocals for a while

Why are bifocals generally not prescribed?

• No near point complaints
• Not old enough for bifocals
• No diagnosis of a near point problem
• Does not read anyhow
• The MR/DS expectations trap

Jessica's Optometric Findings

• Rx +4.00DS / +2.00 add OU
• VA's ~ 20/40 OD, OS, OU
• Phorias: ortho
• Response to minus: "ZILCH"
• Stereo: adequate

Bifocals in Action

Bifocals a Must!!!

• Critical for near point and reading
• Generally a +2.00D add
• Must be a standard bifocal i.e. executive or FT (i.e. a lined bifocal!)
• No progressives! (i.e. no-line bifocals)
  • unaware of blur areas of the lens
• High seg height (lower pupillary margin)

A Case Study

• Old Rx
  • OD -6.00
  • OS -9.00
  • Visual Stim Behavior
  • Head Posture Down
• New Rx
  • OD -4.00
  • OS -1.00
  • Eyes open
  • Better eye contact
  • Improved head posture

How does your eye doctor know what to prescribe?

When they say yes to everything.

Static & Dynamic Retinoscopy

• Very accurate by a skilled optometrist
• Can monitor instability and adjust
• Static is for distance fixation
• Dynamic is for near fixation
• Best done in a trial frame
• And repeated several times

What to avoid?

• Computer refractors
• Phoroptor
• Shortened mirrored Rooms
• Cycloplegic (wet) refractions

Small Room / Big Room

What is the problem?

• Poor fixation, thus off axis measurement
• poor focus i.e. may be focusing at near yet the computer assumes distance
• Reduced peripheral vision in instrument adds to instability
• Mirrors confuse distance perception "objects in mirror are closer than they appear" (statement on passenger side view mirrors)

What to ask for?

A dry trial frame refraction based on retinoscopy

Will you get it?

• Breaks most office routines
• Request will likely offend the doctor
• Many rely on the computer
• Drops routinely given before glasses testing
• May not have twenty foot room
• Most Developmental/Behavioral Optometrist
• Least likely Pediatric Ophthalmologist
• Option: examine in hallway

What about Double Vision?

Signs, Symptoms, & Behaviors

• Blinks frequently and/or hard
• Alternates fixation between the eyes
• Angle or degree of eye turn varies
• May shake, turn, or tilt head
• Tentative gait!!!
• Gross and Fine motor skills stagnant
• Avoids working on midline
• May occasionally fixate off to the side
• Poor attention to desk work or easily distracted
• May see improvement with patching
• Easy to write off on their mental retardation

Problem for Surgeon & Prescribing

• How to adjust muscles with variable angle
• Likewise for Rx amount of correcting prism
• Hence results often unpredictable and require repeat procedures or glasses redo

What is Suppression?

• Suppression is the ability to ignore the second image when seeing double.
• Most mentally intact individuals with a miss aligned eye can and do learn to suppress.
• It is often assumed that those with a miss aligned eye and DS suppress also!
• However this assumed ability in DS is highly suspect.

What is the Problem with Suppression?

• Must first be aware of double vision
• Then acknowledge it as an error
• Then must desire to correct it
• And last must have the capacity to execute such suppression
• Hence suppression is not automatic, but largely cognitive.

Lost Fixation

Patching for Double Vision

• Not Amblyopia therapy!!!
• Occlude poorer functioning eye
  • Maximize performance anyway anyhow
  • Have your eye care professional monitor visual acuity.
• Or alternate patch 24hrs (R-odd, L-even)
• Scotch tape one lens of glasses

Patching for Double Vision

Thick Glasses - Distortive

Crossed Right Eye with Glasses

Crossed Left Eye (alternating) with Glasses

Closed Right Eye with Excessive Blinking to Try and Manage the Diplopia

Uncorrected Crossed Right Eye and Stressed

Contacts and Straight Eyes

Contact Removal

Qualifiers on Visual Performance

• Can read with significant blur
• Can manage even with diplopia
• These do not prevent the execution of the task by themselves, but they do render it more difficult to learn and employ habitually

Visual Perception

• 20% of vision is the physical act of visual sensing, my estimate
• 80% of vision is understanding what we see, thus a very broad & complicated area
• Consumes much of the cortex
• Refers to the capacity to accurately interpret what we see
• Is a direct function of what we know
• Save this talk for next time, but here is a taste

Cautionary Note: Be wary of the high verbal

• They may not know what they speak
• Verbal advantage may be deceptive
• "Sophisticated echolalia"
• Confuses supervising adults
• Check by simply asking them to perform what they speak or read

More Confusion—Who does what? The 3 O's

• Optometrist — works on vision
  • Prescribes glasses and contacts
  • Treats routine eye diseases
  • Specialties, low vision & vision therapy
• Ophthalmologist — works on sight
  • Surgeon & treats complex eye disease
  • Specialties for every part of the eye
• Optician — makes & fits glasses, CL

What is lacking in Vision Care for Down Syndrome

• Functional aspects, glasses and therapy
• Medical considerations well cared for
• Focus is on eyes rather than the person
• Mental limitations assumes less visual needs, when it is just the opposite

Take Home Points

• People with Down syndrome are logical!
• Strive to justify their behaviors
• Little things can make a big difference in subtle ways
• Consider bifocals for reading
• Consider patching for diplopia
• Even try contact lenses
• Consider Vision therapy a later priority

Why Help These People?

• Drag on resources
• Don't pay taxes
• At best minimally productive
• What can they possibly offer us?

The Assets

• Develop an appreciation of the developmental process.
• Develop an understanding how their knowledge and visual confusions influence their behaviors.
• Develop an acceptance of their condition as not bad or good, it just simply is.
• Ultimately, the above helps us to more adequately reflect on ourselves.

Teamwork

So We Can All Be Winners!!!

Contact Information

Vision Development Institute, P.C.

Hans F. Lessmann, OD, FCOVD

1789 S. Braddock Ave.

Suite 130

Pittsburgh, PA 15218

Voice 412-731-5007

Fax 412-731-5251

Email: visiondevelopment1992@yahoo.com

www.optometrists.org/lessmann

Credits

• SCOOBY-DOO Web O'Rama www.scoobysnack.net
  • Scooby Logo
• Sport Graphics http://www.sport-graphics.com
  • Rowing photo
• Pittsburgh Post-Gazatte
  • Medal Photo
• Harry T. Chugani, MD, Wayne State University, Dep't of Pediatric Neurology
  • Synaptic Density Slide
• Thomas Lecoq, double vision reading
• Perceptual Development Corp., double vision seesaw

Additional Web Sites

Developmental Delay Resources

DDR www.devdelay.org

Parents Active for Vision Education

PAVE www.pave-eye.com

College of Optometrists in Vision Development

COVD www.covd.org

Optometric Extension Program Foundation

OEPF www.oep.org

American Foundation for Vision Awareness

AFVA www.afva.com

American Optometric Association

AOA www.aoa.org

References

"Thinking Goes to School"
"Suddenly Successful Student"
ERIC Fact Sheet
DDR
PAVE
AOA
COVD
OEP