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

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)

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

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)

RP- continued Testing: 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

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

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

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

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

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

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

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.

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.

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

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

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

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.

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.

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.

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

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

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

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

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

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