Presentation on theme: "Molly McLaughlin OTR/L, M.A."— Presentation transcript:
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-blindUNM 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 geneAbout 25,000 people in the U.S. are affected by (US)Estimated that 3-6% of the deaf/hh population have USUsually leads to legal, if not complete blindness from RPAt 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 blindnessRP 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 nightCones help us with our day vision, seeing fine details and color
4 RP-continuedAs 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 nightLater as cones start to die, will get blind spots in the peripheral visionWith “tunnel vision” good vision may remain in the center- (central vision)
5 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
6 Usher Syndrome- Type l Most common-estimated at 90% Born with severe to profound hearing loss in both earsBalance problems- absent vestibular functionNight blindness in infancy or early childhoodUsually ASL signersDelayed developmental milestones of lifting head, crawling, walkingTunnel vision usually by age 16
7 Usher Syndrome- Type ll Born with moderate to severe hearing loss-both ears show similar lossBenefit from hearing aidsRP- Night blindness begins in teenage years- with tunnel vision present by late teens to early 20’s.No balance issuesNormal motor milestonesUsually oral communicators- rely on lip-reading
8 Usher Syndrome- Type lll Born with good hearing or mild hearing lossHearing 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’sCan 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 downWalking usually delayed (18 months or later)Seen as “clumsy” childrenMay 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, peopleMay 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 cabinetReluctance to play in low light or outdoors at twilight/darkMay 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 insideLikes to enter a room that is dark early- movie theater.Avoids conversations in a darkened area.Difficulty with riding a bicycleWhen 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 copiesTurns head while readingUses fingers to mark placeHolds book close to the eyes or bends to readPlaces face close to desk while writingSits near blackboardFails 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 sidePrefers conversation at 4-6 feetBecomes 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 itdoesn’t compromise safety.- vision teacher/consultant- help with modifications
16 Educational Needs-continued - communication- ASL, Braille, tactilesigning, captioning-need black box withtext for good contrast- vocational exploration and training- skillsfor 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 communicatingTeacher should provide instruction using a non-cluttered background area-with good contrastFurniture 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 fatigueWhiteboard- 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 ModificationsLighting- 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 glareGood contrast helpful- tables, contact paper, place mats. Black and white usually bestAvoid visual clutter- on tables, bedUse 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, paintUse paint or tape of contrasting color on edges of steps. Top and bottom step can be marked with contrasting stripe. Use of handrail on stairsProvide 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 areasPlan for and allow more time for eyes to adjust to changes in lightGo early to eventsTeach child/youth to advocate for themselves and what they need to function bestIdentify 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 lossWatch for increased isolation, talking or thinking of suicideMay 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 changesWhen grieving, academic focus may decreasePeers need education- deaf friends may think that the student with US is deliberately ignoring them or acting rude and start to pull awayCan be helpful to find a older mentor with US to help support child/youth, provide positive role modelTake advantage of resources- state and national-HKNC- 2 summer programs for year olds.Individual will need to learn self-advocacy skills
25 ConclusionEarly identification- offers more opportunity for learning adaptations/modificationsHelps with realistic vocational goals and learning independent life skillsGenetic counseling- may want-especially when considering childrenStart treatment to slow down or arrest the progression of US