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 narrows—a 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 20’s. • 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 20’s- 30’s • 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- don’t 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 doesn’t 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