Title: Effective Strategies for Home and School for the Student with Usher Syndrome
1Effective Strategies for Home and School for the
Student with Usher Syndrome
- Molly McLaughlin OTR/L, M.A.
- Project for NM Children and Youth who are
Deaf-blind - UNM Center for Development and Disability
2Usher Syndrome Overview
- Usher syndrome- defined as hearing loss and an
eye disorder called retinitis pigmentosa, or RP. - Inherited recessive genetic disorder- must get
the gene from both parents. 1-4 chance of having
a child with US if both parents carry the gene - About 25,000 people in the U.S. are affected by
(US) - Estimated that 3-6 of the deaf/hh population
have US - Usually leads to legal, if not complete blindness
from RP - At least three types exist (Type l, ll, and lll)
3Retinitis Pigmentosa RP
- RP causes night-blindness and a loss of
peripheral vision (side vision) through the
progressive degeneration of the retina. - RP may be diagnosed at any age and can lead to
complete blindness - RP affects the photoreceptor cells (rods and
cones) in the retina. These cells gradually
deteriorate and die. - Rods help us see in dim light and at night
- Cones help us with our day vision, seeing fine
details and color
4RP-continued
- As RP progresses, the field of vision narrowsa
condition known as tunnel vision -until only
central vision (the ability to see straight
ahead) remains. - With RP the rods deteriorate first- first symptom
is difficulty seeing in dim light or at night - Later as cones start to die, will get blind spots
in the peripheral vision - With tunnel vision good vision may remain in
the center- (central vision)
5RP- continued
- Person is considered legally blind when only 20
degrees of central vision remain. - Some people may retain 5-10 degrees of good
vision into old age. - Testing
- USM Chip-microchip that can test a saliva sample
(65-75 accurate) - ERG-Electroretinography measurement of nerve
impulses in the retina (95 accurate) - Visual Field Test- side/peripheral vision
6Usher Syndrome- Type l
- Most common-estimated at 90
- Born with severe to profound hearing loss in both
ears - Balance problems- absent vestibular function
- Night blindness in infancy or early childhood
- Usually ASL signers
- Delayed developmental milestones of lifting head,
crawling, walking - Tunnel vision usually by age 16
7Usher Syndrome- Type ll
- Born with moderate to severe hearing loss-both
ears show similar loss - Benefit from hearing aids
- RP- Night blindness begins in teenage years- with
tunnel vision present by late teens to early
20s. - No balance issues
- Normal motor milestones
- Usually oral communicators- rely on lip-reading
8Usher Syndrome- Type lll
- Born with good hearing or mild hearing loss
- Hearing loss progresses over time- hearing aids
may need to be changed. - Begin as oral communicators- as hearing loss
progresses- ASL communicators. - RP- Night blindness in childhood or teens- tunnel
vision in the 20s- 30s - Can have a progressive balance disturbance
9Behavioral Symptoms for Parents and Teachers-
(US-l)
- Young children - harder time learning how to sit
without support, crawl and walk. May prefer
rolling to being on all fours. May have 5-point
crawl-head down - Walking usually delayed (18 months or later)
- Seen as clumsy children
- May love twirling, spinning- dont get dizzy
-
10Behavioral Picture - continued
- As RP progresses
- May naturally or automatically turn their head to
scan visual field- not fully recognizing the
extent of vision loss. - Bumps into things in front of their feet.
- Tripping over curbs, stairs, people
- May reach for something in front of them that
they see clearly and knock something else over. - Spills when pouring liquids
11Behavioral Picture- continued
- Bump into open doors- not see the edge of the
door, or hits head on kitchen cabinet - Reluctance to play in low light or outdoors at
twilight/dark - May request that lights be left on at night, in
hallways etc. - Difficulty adjusting to changes in light- going
from low light to sunny outdoors- vice versa. - Avoids outdoor sports when sun is bright
12Behavioral Picture- continued
- Wears sunglasses- even inside
- Likes to enter a room that is dark early- movie
theater. - Avoids conversations in a darkened area.
- Difficulty with riding a bicycle
- When walking along a road at night, may stagger
or lose balance after an oncoming car has passed.
13School Behaviors and Considerations
- Needs good contrast- has difficulty reading light
copies - Turns head while reading
- Uses fingers to mark place
- Holds book close to the eyes or bends to read
- Places face close to desk while writing
- Sits near blackboard
- Fails to understand or miss group instruction-
may position self to one side of the group. Often
last at completing group activities.
14School Behaviors and Considerations-continued
- May have repetitive behavior- likes to do things
the same way. Routines are comforting,
predictable and increase success! - Appears to ignore others standing to the side
- Prefers conversation at 4-6 feet
- Becomes anxious in unfamiliar areas or with new
tasks. - Episodes of anger, frustration, emotional
outbursts-normal grieving process. Can/will
reoccur as vision decreases over time
15Educational Needs
- Schools need to provide
- - academics- with modifications/adaptations
- - orientation and mobility instruction
- - athletics/sports- good for student as long
as it - doesnt compromise safety.
- - vision teacher/consultant- help with
modifications -
16Educational Needs-continued
-
- - communication- ASL, Braille, tactile
- signing, captioning-need black box with
- text for good contrast
- - vocational exploration and training-
skills - for future
- - support/counseling- reduce isolation,
- educate peers
-
17Classroom Modifications
- Teachers need to consider future dual-sensory
impairment when identifying skills to teach- not
just current status. - Full spectrum lighting if possible- helps us see
fine details more easily, color matching, reduces
glare, Helps lesson eye fatigue and strain when
performing visually demanding tasks. - Seat student where they are comfortable-
(front-side) so they can see chalkboard, teacher
and other students in class.
18Classroom Modifications- continued
- Teacher/assistant needs to direct attention of
the student to other students in class that are
asking or answering questions. Can only see 1
person at a time. - Windows should be behind the student. Teacher
should avoid standing in front of window while
communicating - Teacher should provide instruction using a
non-cluttered background area-with good contrast - Furniture arrangement- Keep room the same if
possible. Keep doors and drawers closed. Let the
student know in advance of any changes.
19Classroom Modifications-continued
- Print materials should be maximum contrast. Use
non-glare paper. Yellow transparency overlay is
helpful to reduce glare and eye fatigue - Whiteboard- need dark markers- black, blue or
purple best. Yellow difficult to see. - Students may need individual copies of graphs,
charts, assignments to examine close up. - Students need additional time to complete tasks-
time and a half minimum. May consider reducing
the of questions or problems to equalize the
time spent on task.
20Home Modifications
- Lighting- have child/youth experiment with
different lighting options- full spectrum,
goose-neck lamps, natural light, etc. to figure
out what is best, where, and at what time of day.
Use curtains/ shades to decrease glare - Good contrast helpful- tables, contact paper,
place mats. Black and white usually best - Avoid visual clutter- on tables, bed
- Use contrasting colors to differentiate between
walls and floors- contrast molding can be
helpful. Flat paint provides less glare
21Home Modifications- continued
- Outline doorways with contrasting border of
color- tape, paint - Use paint or tape of contrasting color on edges
of steps. Top and bottom step can be marked with
contrasting stripe. Use of handrail on stairs - Provide a consistent and organized environment.
Keep furniture and objects in consistent places.
Keep walking areas free of low objects to run
into or trip over. - Keep doors and cabinets fully open or closed
22Home Modifications- continued
- Use of flashlight in dimly lit areas
- Plan for and allow more time for eyes to adjust
to changes in light - Go early to events
- Teach child/youth to advocate for themselves and
what they need to function best - Identify strengths and interests
23Emotional Considerations for Individual and Family
- Can be very difficult to cope with initial
diagnosis of US- debate about when to tell a
child. Child may ask questions and want to know.
- Questions should be answered honestly but
constructively. Emphasize strengths. - Most professionals believe that students should
know future implications by high school so that
student can make educational or vocational
choices that will compensate for eventual
dual-sensory loss - Watch for increased isolation, talking or
thinking of suicide - May not be safe to drive- HUGE issue for
teenagers and adults -
24Emotional Considerations for Individual and Family
- Anger, depression and denial- cycle between,
before acceptance. Recycle as vision or hearing
loss changes - When grieving, academic focus may decrease
- Peers need education- deaf friends may think that
the student with US is deliberately ignoring them
or acting rude and start to pull away - Can be helpful to find a older mentor with US to
help support child/youth, provide positive role
model - Take advantage of resources- state and
national-HKNC- 2 summer programs for 16-22 year
olds. - Individual will need to learn self-advocacy
skills
25Conclusion
- Early identification- offers more opportunity for
learning adaptations/modifications - Helps with realistic vocational goals and
learning independent life skills - Genetic counseling- may want-especially when
considering children - Start treatment to slow down or arrest the
progression of US