Effective Strategies for Home and School for the Student with Usher Syndrome

1
Effective 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

2
Usher 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)

3
Retinitis 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

4
RP-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)

5
RP- 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

6
Usher 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

7
Usher 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

8
Usher 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

9
Behavioral 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

10
Behavioral 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

11
Behavioral 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

12
Behavioral 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.

13
School 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.

14
School 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

15
Educational 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

16
Educational 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

17
Classroom 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.

18
Classroom 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.

19
Classroom 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.

20
Home 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

21
Home 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

22
Home 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

23
Emotional 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

24
Emotional 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

25
Conclusion

• 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