Download Psychosocial Effects of Low Vision

Session 10: Wednesday, November 18, 2015:
Psychosocial Effects of Low Vision
Student Presentations
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Presentations continue next week!
◦ Those presenting should email Rhiannon with your
Power Point by Tuesday, November 24th, 2015
 I will not have time on Wednesday to put the PowerPoint
together before class!
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Guest Speaker: Peter Parsons
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Student Presentations:
◦ Alstrom Syndrome – Laura Glass
◦ Coloboma - Carla Giesbrecht
◦ Peter’s Anomaly - Catherine Tellier
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Current: APSEA O&M Instructor
Past: CNIB O&M Instructor and
Manager of Programs and
Services
Has an 8 year-old son
Is a goalball superstar and
part-time rapper 
Peter on a recent visit to
CNIB Halifax
A very rare genetic disease with just over 1000 identified
cases worldwide
By Laura Glass
“Alstrom Syndrome is a rare complex genetic disorder
associated with a wide variety of symptoms affecting
multiple organ systems of the body.”, (NORD, 2013).
 Carl-Henry Alstrom was born in
1907 in Sweden
 received doctorate in psychology in 1935
 conducted genetic research in the 40’s
 In 1959 he described 3 patients in his
manuscript describing the symptoms of Alstom Syndrome
 Is recessively inherited; therefore both of the parents
are carriers but will not exhibit the features of
Alstrom.
 Not much is known regarding how this gene works but
we do know that when something goes wrong within
this gene, the consequences are many and encompass
multiple organ systems.
 Because the features of Alstrom can be so wide-spread
and also variable, misdiagnosis is common, especially
early on before some of the classic symptoms develop.
• Bardet Biedl Syndrome (Blue)
• Achromatopsia (Red)
• Leber Congenital Amaurosis
(Green)
• Mitochondrial Disorder (Purple)
• Usher (Teal)
• Retinitis Pigmentosa (Orange)
• Niemann-Pick (Blue)
 Vision loss
 Hearing loss
 Obesity which can lead to Type 2 Diabetes (as early as age 4)
 Cardiomyopathy – enlargement of the heart’s lower chambers due to a
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weakened heart muscle
Acanthosis Nigricans – a skin disorder characterized by a increased
pigmentation and velvety thickening
Pancreatitis – due to elevated levels of certain fats in the blood
Enlarged liver and spleen
Kidney disease, including kidney failure
*Not all patients develop all symptoms. They do vary from person to
person.
• The neural retinal layer contains
millions of cells, called the
photoreceptors, which are able to
respond to light with an electrical
signal.
• Cone cells, concentrated in the
central portion of the retina,
contain light-sensitive pigments
that enable us to see fine detail and
color.
• Rod cells, absent from the center of
the retina, but dense everywhere
else, enable peripheral vision and
vision in dim light or darkness.
• Retinal dystrophy is a general
term to describe a condition where
there is degeneration of the cells in
the retina.
 Some of the first vision symptoms, nystagmus and photophobia, are
revealed at birth. They are caused by the slow degeneration of the
retina. Retinal Dystrophy.
 Typically the cones deteriorate first in the eyes of children who have
Alstrom Syndrome, so the vision that they experience comes only from
the rods. As they get older the rods may also stop working.
 About 39% of people with Alstrom Syndrom develop cataracts. The
most likely cause of cataract formation is the substances released from
the degenerating retina that damage the lens, causing it to become
opaque from the back.
Normal Vision
Early Visual
Defects
Late Visual
Defects
 1. Photophobia and Nystagmus
 2. Low Visual Acuity
 3. Visual Fields
 4. Color Vision
 Many individuals with Alstrom Syndrome develop photophobia,
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light sensitivity. This happens because the lack of function in the
cone cells causes the rods to become over saturated, especially in
bright light.
As the the retinal dystrophy progresses, the rods become less
sensitive and the photophobia diminishes.
Red/orange tinted prescription lenses can help reduce light
sensitivity.
Move the student to a position within the class where the glare is
not reflected off windows, mirrors, wet or shiny surfaces, or
white and brightly coloured surfaces.
Sunglasses and a hat are worn outdoors and perhaps indoors as
well.
 Usually less than 20/200 by age 3 (Malm, E et al,
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2008)
By 9 years of age, approximately one-third of
patients are totally blind; 50% by age 12, and 90%
by age 16 (JD Marshall et al, 2007)
Dual media is often used, however Braille literacy
must be introduced at a young age
Some individuals can read large print into their
third decade, although this is rare
May benefit from a CCTV and magnifiers
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Increasing constriction of visual fields
Orientation and mobility training
Keep the classroom layout as static as possible
Seating in the classroom should be where the child’s seeing field is
maximized for visual materials presented and to view the teacher
during instruction, usually in the front and centre
 Due to developing cone-rod dystrophy, color
perception is affected
 Present materials on backgrounds that offer high
contrast to the objects being viewed.
 Students can print or draw using black markers on
white paper.
 Label pictures with color words such as maps or
diagrams.
 Alstrom Syndrome International: They provide support,
information, and coordination world-wide to families and
professionals in order to treat and cure Alström Syndrome.
https://www.alstrom.org/
 NORD: National Organization for Rare Diseases.
https://rarediseases.org/rare-diseases/alstrom-syndrome/
 Our textbook! Foundations of Low Vision. Regardless of
the diagnosis we use adaptations/accommodations that fit
the specific functional vision implications for each of our
students.
……that intelligence is usually unaffected
in individuals with Alstrom Syndrome!!
 Alstrom Syndrome International website. (2015).
Supporting those affected by Alstom Syndrome. Retrieved from
https://www.alstrom.org/
 Practical Genetics. (2007). Alstom Syndrome. Retrieved from
http://web.b.ebscohost.com.ezproxy.msvu.ca/ehost/pdfviewer/p
dfviewer?sid=120edb8d-d9c6-43b3-bb0b-9e78
3cacce8b%40sessionmgr198&vid=1&hid=109
 Malm, E et al. (2008). Full-Field Electroretinography and
Marked Variability in Clinical Phenotype of Alström Syndrome.
Retrieved from
http://archopht.jamanetwork.com/article.aspx?articleid=420223
 NORD. (2015). Alstom Syndrom. Retrieved from
https://rarediseases.org/rare-diseases/alstrom-syndrome/
Coloboma
Presented by Carla Giesbrecht
What is a Coloboma
Coloboma comes from the Greek word meaning
“curtailed” or “unfinished”.
Corn and Erin (2012) define a coloboma as a congenital
malformation of one or both eyes.
• A coloboma forms when the eye fails to develop causing a
gap/notch in one or more structures of the eye.
• Colobomas affect 1/10 000 infants.
Causes of Coloboma
Colobomas:
• Are an inherited condition that is autosomal-dominant in
nature.
• Can occur with no previous family history.
• May be caused by environmental issues such as fetal
alcohol exposure.
The Diagnosis of Coloboma
The diagnosis is made by an ophthalmologist.
• The ophthalmologist uses an ophthalmoscope to examine the
eye.
• Visual acuity may not be initially assessed because of the age
of the child.
• Symptoms of the condition occur on a spectrum from mild to
severe.
• A pediatrician may perform an assessment if
an associated syndrome is suspected.
https://s-media-cache-ak0.pinimg.com/236x/62/3e/be/623ebe4bd321b8bafa8f0eebe3836279.jpg
Parts of the Eye Affected
A number of structures of the eye may be affected:
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Iris
Lens
Retina
Optic Nerve
National Eye Institute
A coloboma can also form on the eyelid, however the cause may
be different than for the structures affected in the globe of the
eye.
Iris Coloboma
Iris colobomas are the most common form of colobomas.
• The pupil of the eye may appear like a teardrop or keyhole.
• The iris and vision may be only minimally affected.
• The child may experience photophobia, double vision, blurring
or ghosting.
http://ohiolionseyeresearch.com/files/glossary/coloboma.jpg
Lens Coloboma
Lens Colobomas:
• Affect the lens of the eye.
• Will appear as a notch or gap in the lens.
• May affect the ciliary body
and not involve the lens at all.
National Eye Institute
Retinal Coloboma
Retinal Colobomas:
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Occur when the development of the retina is disturbed.
Can be bilateral or symmetrical.
Can be asymptomatic or cause vision loss.
May reduce visual acuity.
May cause a visual field loss.
May cause a retinal detachment.
May cause strabisumus or nystagmaus.
Macular Coloboma
National Eye Institute
Optic Nerve Coloboma
The optic nerve can be affected in two
ways:
1. The optic nerve is hollowed often
called the optic nerve pit.
Optic Nerve Coloboma
2. A retinal or iris coloboma that is large enough that it
includes the optic nerve.
National Eye Institute
Morning glory disc anomaly is
another coloboma associated
with the optic nerve.
http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-27302008000800004
Effects on Vision
The effect of the coloboma is due to the extent of the gap
and were it is located in the structure of the eye.
• If the coloboma occurs in the eye vision may not be
affected.
• If the coloboma occurs in the retina or the optic nerve
vision could be significantly affected.
• Children with colobomas may also experience cataracts,
glaucoma, myopia, nystagmus and retinal detachment.
Treatment
There is currently no medication or surgery to repair the
part of the eye that fails to close.
• Treatment is dependent upon the extent of the
coloboma and varies for individuals.
• Management of the coloboma may include:
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Glasses or contact lenses
Patching
Treatment of other eye conditions
Treating complications
Use of low vision devices
Early intervention
Genetic counseling
Other Effects that May Occur
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Heterochromia
Microphthalmia
Increased thickness of the cornea
Cataracts
Glaucoma
Retinal dysplasia
Myopia or hyperopia
Nystagmus
Infant with microphthalmia
http://ocularpro.com/prosthetic-eye-services-los-angeles/anophthalmia-and-microphthalmia/
Associated Syndromes
Colobomas can be associated with a number of
syndromes:
• CHARGE Syndrome
• A syndrome with multiple organs affected, including heart
defects, blocked nasal passage, retarded growth or development,
genital abnormalities and ear abnormalities and deafness.
http://chargesyndrome.org/index.asp
Associated Syndromes
Continued
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Epidermal Naevus Syndrome
Cat Eye Syndrome
Kabuki Syndrome
Pataus’ Syndrome
Edward’s Syndrome
Wolf-Hirschhorn Syndrome
MIDAS Syndrome
• Michrophthalmia
• Dermal Aplasia
• Sclerocornea
Infant with Kabuki Syndrome
http://syndromespedia.com/kabuki-syndrome.html
Seeing Better
There are a number of things parents can do to ensure
that that their child’s vision develops as well as it can
before entering school.
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Ensure that their child wears prescribed eyewear
Observe their child in play
Minimize visual clutter
Seek early intervention
Expose their child to as many
activities and experiences as
possible
http://www.telegraph.co.uk/women/mother-tongue/familyadvice/10528194/Pictures-of-our-children-at-play-will-soon-be-museum-pieces.html
Vision At School
It is important that school staff develops a good understanding
of the student’s visual needs.
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Communicate with parents
Complete a Functional Vision Assessment if required
Complete a Learning Media Assessment if required
Implement recommendations
Provide service from the TSVI if required
https://edueval.wordpress.com/2015/01/25/progressive-vision-in-dubai-private-schools-at-what-works-2015/
Optimizing Student Visual
Performance
The Texas School for the Blind and Visually Impaired
recommend considering the following when optimizing
student’s visual performance in the classroom.
• Consider the etiology
• Effects of light
• Field deficits
• Eye motor
• Posture
• Organization
• Lighting on work surfaces, projectors and screens
• Writing tools and materials
• Optical devices
Classroom Adaptions and
Accommodations
Low vision
• Use large print materials.
• Provide with preferential seating- close to the board and central Verbalize
what is been written.
• Provide the student with a printed copy of notes.
• Pre-teach or review books or movies to be viewed as a whole class.
• When the student is writing a test, ensure that the student can read the font
before beginning the test; provide the student with extra time to write
exams.
• Allow the student to explore new environments.
• At assemblies or large group gatherings provide preferential seating and
explain what is happening.
• APHont can be downloaded to use on worksheets.
•
http://www.aph.org/products/aphont/
• Encourage the use of prescribed glasses.
• Encourage the use of their vision.
• If it is appropriate and has been prescribed by a low vision specialist the
student may benefit from the use of a hand held magnifier of CCTV.
• Be aware of visual fatigue.
Photophobia
• Adjust the distance the student sits from the window or light
source as needed, sitting with the students back to the window.
• Spot light reading material or use of a filter over top of reading
material if bright lighting is an issue.
• Avoid or be aware of glare on surfaces.
• Student may benefit from wearing sunglasses, hat or visor.
• Reduce or diffuse lights.
http://lookfordiagnosis.com/mesh_info.php?term=photophobia&lang=1
Visual Field Loss
• Ensure that the student is made aware of any changes to the
environment.
• Provide contrast on stairs and playground.
• During physical education ensure that the student is aware of
projectiles that are coming towards them.
• Announce yourself when you are approaching the student.
• Allow the student to view materials through their best visual
field.
• Teach the student to stop, look and then move.
• Avoid visual clutter within the environment.
• Tack or tape down scatter mats in the classroom.
• Teach the students to push in their chairs.
• Provide the student with access to and O&M Specialist.
Colour Vision
• Provide the student with high contrast materials.
• Supplement visual tasks with auditory and tactile information.
• Depending on the student they may require similar adaptions as
those with photophobia.
Contrast
• When using a white board write with dark markers such as black.
• Avoid the use of red, orange and yellow on white boards and
anchor charts.
• Provide the student with good photocopies of materials. (Blurred
or fuzzy lines are hard to read, provide white space)
• Worksheets and written materials should be of an appropriate
font size, of high contrast (bold) and contain well-spaced
words/letters.
A Final Word From A Parent
• https://www.youtube.com/watch?v=ZrOgNYCZtDE
If Time…..
• https://www.youtube.com/watch?v=nHksmsCJgpo
Websites
Resources
• Statewide Vision Resource Center
http://www.svrc.vic.edu.au/index.shtml
• Teaching Students with Visual Impairment
http://www.teachingvisuallyimpaired.com
Videos related to CHARGE Syndrome:
http://www.perkinselearning.org/videos/webcast/charge-syndrometeaching-strategies-children
http://www.perkinselearning.org/videos/webcast/charge-syndromeimpact-of-charge-on-communication-and-learning
http://www.perkinselearning.org/videos/webcast/charge-syndromeoverview
References
Blaikie, A.Medical information on coloboma. Retrieved from
http://www.ssc.education.ed.ac.uk/resources/vi&multi/eyeconds/colob.html
Corn, Anne and Erin, Jane (Ed.). (2010). Foundations of low vision: Clinical and functional perspectives
(2nd ed.). New York: American Foundation for the Blind.
Cowan, Chrissy and Texas School for The Blind.Possible accomodations for the student with a visual
impairment. Retrieved from http://www.tsbvi.edu/instructional-resources/62-familyengagement/3657-vision-accommodations
Genetics Home Reference.Coloboma. Retrieved from http://ghr.nlm.nih.gov/condition/coloboma
National Eye Institute.Facts about uveal coloboma. Retrieved from
https://nei.nih.gov/health/coloboma/coloboma
Patient.Coloboma. Retrieved from http://patient.info/doctor/coloboma
Rao, Elsie and Texas School for the Blind.Considerations for low vision students in A classroom.
Retrieved from http://www.tsbvi.edu/program-and-administrative-resources/53-resources/programand-administration-resources/3277-considerations-for-low-vision-students-in-a-classroom
RNIB.Coloboma. Retrieved from http://www.rnib.org.uk/eye-health-eye-conditions-z-eyeconditions/coloboma
Overview of
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It is a congenital condition which occurs in the
first trimester before the anterior chamber of
the eye is completely formed.
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The Dictionary of Eye Terminology describes
Peter’s Anomaly as a “central corneal
malformation characterized by adhering of
the iris to Descemet’s membrane and the
endothelium” (Cassin & Solomon, 1990)
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The cause is unknown but can be:
 Inherited:
▪ Autosomal recessive (CYP1B1 gene)
▪ Autosomal Dominant (FOXC1, PAX6, or PITX2 gene)
 Environmental
 Both
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Two types: Type I , Type II
Very rare
Approximately half of the population with PA have
low vision early in life and a quarter are legally blind
Type I:
▪ may or may not have cataracts; lens does not
adhere to the cornea
▪ 80% bilateral
Type II:
▪ Corneal clouding is typically denser
▪ cataracts present and adhere to the cornea
▪ Usually bilateral
▪ Iris may or may not be present
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Anterior chamber
 Cornea
 Iris
 Pupil
 Lens
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Clouding of the cornea can be partial, central
or complete
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Microcornea
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Cornea plana
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Aniridia
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Coloboma
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Cataracts
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Dislocation of the lens
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Attachment of the lens to the cornea
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Glaucoma (90%)
Microphthalmia
Coloboma of the Choroid
Persistent Hyperplastic Primary Vitreous
(PHPV)
Amblyopia
Nystagmus
Optic Nerve Hypoplasia or Atrophy
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Type II associated with more systemic issues
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developmental delay
congenital heart disease
structural defects of the neurologic system
spinal defects
genitourinary abnormalities
external ear abnormalities and hearing loss
cleft lip and palate
short stature
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Medical care:
 Medication to manage glaucoma
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Surgical care:
 Keratoplasty
 Lensectomy/vitrectomy
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Other Care:
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Genetic counselling
Low Vision Specialist
Near and Distance
Visual Acuity
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Blurred vision
low vision
legal blindness
Blind
 Vision may fluctuate
 May or may not use corrective lenses/protective
lenses
 Implications are varied (FVA/LMA)
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Visual fields:
 Peripheral loss
 Central loss,
 blind spots
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Implications are varied (FVA/LMA/O&M)
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Light sensitivity
Glare sensitivity
May need to wear visor, tinted glasses, light control
(high or low)
- May need time to adjust to lighting changes
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Contrast Sensitivity
- May have difficulty with details, locating objects
- May use materials to increase contrast or highlight
important visual information (eg. light, black placemat)
- CCTV
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Poor colour vision
 May not be able to distinguish between similar
shades.
 May need to label items tactually to identify
colour
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Reduced or absent depth perception
Implications in mobility and motor planning
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Eye fatigue
Fluctuating visual abilities
Eccentric viewing
Diploplia
Poor night vision
Pain or headaches
Corneal Disease Information
https://nei.nih.gov/health/cornealdisease/
Peter’s Anomaly (genetics diagram)
http://disorders.eyes.arizona.edu/handouts/peters-anomaly
Peter’s Anomaly (parent video)
http://childrenseyefoundation.org/meet-littleambassadors/peters-anomaly-in-children/
Low Vision: A Resource Guide with Adaptations for Students
with Visual Impairments (Levack, N.) TSBVI
Cassin, B. & Solomon, S. (1990). Dictionary of Eye Terminology, Second
Edition. Triad Publishing. p. 206.
Corn, A. & Erin, J. (2010). Foundations of Low Vision: Clinical and Functional
Perspectives. AFB Press.
Giri, G. (2015). Peters Anomaly Clinical Presentation. Retrieved from
http://emedicine.medscape.com/article/1200372-clinical#b4
Levack, N. (2004). Low Vision: A Resource Guide with Adaptations for
Students with Visual Impairments, Second Edition. TSBVI.
U.S. National Library of Medicine. (November 9, 2015). Peter’s Anomaly.
Retrieved from http://ghr.nlm.nih.gov/condition/peters-anomaly