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Parents

Parent Tips for Usher

Behavior Indicators for Usher:

  • Possible difficulty maintaining balance
  • Tripping or bumping while walking in changes in lighting (e.g., entering and leaving building on sunny day, etc.), in dim light, or at night; may be accompanied by heightened fear in darkness
  • Sensitivity to bright light or sun or snow; sensitivity to glare (e.g., windows, etc.)
  • Hesitating or stumbling on curbs, steps or unfamiliar terrain
  • Unresponsive to people’s nonverbal or verbal language (e.g., waving “Hello”, etc.) from the left or right or in front

Physical Manifestations of Usher (no way to predict the timing or progression of the vision loss, and progression and visual acuity/visual fields vary from student to student):

  • Deaf, hard-of-hearing or initially hearing
  • Loss of night vision
  • Progressive loss of peripheral vision to the left, right, above and/or below (“tunnel vision”); may still be able to see clearly within the small area of remaining vision
  • May be some loss of central vision

* Above involve continual readjustment to a progressive visual (and hearing) condition for the individual and significant others

Possible Emotional Adjustment to Usher for Individual (may experience few, some or most feelings; with support and training there is a bright future (smile)! :

  • Tripping and bumping into objects and people (startled [surprised, scared], angry, embarrassed ) and missing nonverbal and/or verbal information such as greeting (perplexed, embarrassed)
  • teasing by peers (hurt, angry, sad, lonely)
  • withdrawing and isolating self so other not witness clumsiness/feeling not belong to peer group (sad, lonely), shunning by peers because seemingly “contagious” and/or different (confused, hurt, lonely) and/or struggling to fit in with and be accepted by dominant identity group (anxious, tired)
  • Learning Usher diagnosis (relieved about difficulty being explained, shocked at the news, denied deafblindness exist because reality of Usher so painful, devastated about shattered dreams [e.g., driving], grieved the loss of vision [communication, travel, social, leisure, identity], angry at the injustice [not fair], bargained for a cure, sad about being different, scared about the unknown future [how to communicate and travel with diminished vision; how to maintain relationships; how to be independent and not be burden on family])
  • With approaching graduation thinking about leaving familiar environments and moving to the new and unknown and living independently, going to college and/or work, keeping old friends and forming new ones and/or future marriage and family (scared, overwhelmed, excited)
  • More tripping and bumping and missing information with world seeming to be smaller and smaller (e.g., going from banging head to bumping eye; going from visual sign to tracking sign) and possible temporary loss of (partner, parent, friend, worker) role and dependency on others (demoralized, frustrated, scared, guilt, sad, lonely, tired)

*person needs love, respect, dignity, sense of belonging, and knowledge during continual readjustment to progressive visual (and hearing) condition

Possible Family Adjustment:

  • Behavioral indicators of not hearing and initial diagnosis of deafness in childhood may be associated with shock at the news, denial deafness exist, grieving the loss of hearing, angry at the injustice, bargaining for a cure, sad about shattered dreams (normal child), scared about the future; with (unintentional) passing on deafness, may experience guilt, blame, shame; faced with confusing and overwhelming communication and education options and decisions
  • Later diagnosis of blindness bring devastation, helplessness, isolation; never heard of Usher and frightened of future; may not share diagnosis with family and friends for fear of rejection > need to meet Usher families and adults
  • Parents faced with task of sharing diagnosis with child: when? How? What? All at once? Little by little? Not at all? Ideal to tell at time of diagnosis because child already experience difficulties and need explanation; best not to give initial lengthy information nor prognosis of total blindness; child’s questions and comments indicate level of readiness; respond honestly and according to developmental level over multiple times ( may seek assistance of counselors; assure child will be cared by family; do not share all own thoughts and feelings with child to maintain boundaries (confide mature issues in empathetic adult listeners)
  • With approaching graduation, RESURFACING of anticipatory loss and unresolved issues (angry, GUILT, scared)

Diverse Communication Styles (students may use one or more of these styles, and depend on skill and comfort level; may vary according to settings, changing vision, health, stress, fatigue, etc.):

  • -Speechreading unaided or aided (e.g., hearing aids, cochlear implant, etc.) in well lit areas; good speechreader understand 30% -40% of spoken words
  • -Hearing (e.g., assistive listening systems, etc.) in relatively quiet settings
  • -Signing
    • No adaptations
    • At appropriate distance, position and within smaller central space (e.g., mouth to chest area, etc.)
    • Tracking with hand on signer’s wrist and visually following signs
    • Tactile with hand(s) on signer’s hand(s) and tactually following signs (if welcomed in dim or dark settings)
  • Other (e.g., total fingerspelling, large print TTY, tellebraille, etc.)

Possible Suggestions upon Initial Diagnosis of Usher:

  • Watch for emotional/behavioral changes (e.g., grief, anger, isolation, decreased self-image, etc.); may receive counseling-related services (have periodic psychological evaluations with attention to adjustment to Usher)
  • If student is willing, may provide in-service on symptoms and needs of Usher to classroom or school to foster understanding
  • If clumsy walking, may learn confident, safe travel through Orientation and Mobility (O & M: e.g., sighted guide, self-protective techniques, scanning, trailing, cane, etc.) (Have periodic O & M evaluations to address changing travel needs)
  • If difficulty accessing information, may use adapted communication techniques (e.g., scanning, signing confined to small window, visual tracking, tactile sign language, etc.), adapted devices (e.g., hearing aids/FM listening systems, magnifiers, monoculars, etc.), as well as other helpful items (e.g., sunglasses, caps with bims, etc.) (Have periodic language evaluations to address changing communication needs)
  • ASL (if hard-of-hearing/initially hearing)? Easier to learn visually and with better vision, augment with speechreading/hearing
  • Braille (if have adequate reading vision)? Exposure at young age as fun activity at time of stability and strength provide foundation and sense of control about future, take time to be comfortable and to master skill; if choose not to learn Braille, important to learn about possible future modifications/accommodations and adaptive equipment
  • Transition age? Explore preferred career choices; may remain in first occupation for many years with modifications/accommodations and adaptive equipment later on; may be need for career change
  • May maintain contact with service providers to support generalization of newly learned skills
  • May obtain resources for contact with other children, teenagers and adults with Usher and families so as to ask questions, to share feelings and experiences, to discuss concerns, to shape positive self-image, to feel socially accepted, to be receptive to adaptive techniques and to learn future options, such as post-secondary education, employment and living independently (e.g., deafblind family weekends, deafblind consumer organizations, DB Link, etc.)

Possible Suggestions for Helpful Environmental Accommodations (to provide an environment that is safe, predictable, accessible, and interesting):

 

  • Use appropriate lighting across rooms for access and safety; when traveling in areas of changing lighting (e.g., bright sun to dim entrance, etc.); allow time for vision to adjust
  • Not stand where bright light or direct light (e.g., from window, etc.) shine in student’s eyes to minimize fingerspelling or speechreading difficulties & fatigue, if possible, close curtains or shades to minimize glare
  • Minimize clutter of walls and floor
  • Furniture should be arranged to provide easy movement in open space; limit furniture rearrangement as much as possible and when necessary inform child beforehand; keep drawers and doors closed
  • If welcomed and as necessary, convey visual information, such as physical description of people, body language and facial expressions, physical description of room (e.g., location of doors, etc.), when someone leaves or enters room, etc.; if child not respond visually or auditorily, touch shoulder/arm/hand to indicate presence and/or redirection
  • Keep desired distance and position for speechreading/signing according to child’s needs (e.g., stand 5 feet away facing student and confine signs and facial expressions to small window space of face and chest area, etc. ); provide tracking and tactile sign if child welcomes it
  • At times of social interactions, wear solid color clothing that contrast with skin tone (light skin tone: black, charcoal, navy, dark green; dark skin tone: white, cream, buff); if wearing appropriately colored clothing is not possible, use a smock when with child; no jewelry
  • Use slower, clear signs and/or speech
  • Minimize interactions in dimly lit room or noisy rooms

Bibliography

  • Bagley, M., Mascia, J. and Rothstein, R. (1996). Blind and Severely Profoundly Hard-of-Hearing. Proceedings of the Fifth Canadian Conference on Deaf-Blindness. Living and Learning: A Lifelong Adventure, May 8-11. Vancouver, B.C., Canada, 43-53.
  • Baumgarner, J. Tips for Students with Usher Syndrome. Pennsylvania Deafblind Project.
  • Jordan, B. (2000). Functional Implication & Environmental Modifications with Students Who Have Usher Syndrome. Helen Keller National Center, Sands Point, NY.
  • McInnes, J.M. (1999). Guide to Planning and Support for Individuals Who Are Deafblind. University of Toronto Press, Toronto, ON, Canada.
  • Miles, B. and Riggio, M. (1999). Understanding Deafblindness. Remarkable Conversations. Perkins School for the Blind, Watertown, MA.
  • Miner, I. (1997). People with Usher Syndrome, Type II: Issues and Adaptations. Journal of Visual Impairment & Blindness, November-December. American Foundation for the Blind. New York, NY 579-589.
  • Miner, I. (1995). PsychoSocial Implications of Usher Syndrome, Type I, Throughout the Life Cycle. JOURNAL OF VISUAL IMPAIRMENT AND BLINDNESS, May-June. American Foundation for the Blind. New York, NY, 287-296.
  • Miner, I. (1997). When to Tell Children about Usher Syndrome. Internet, Usher-List.
  • Miner, I. and Cioffi, J. (1997). Usher Syndrome in the School Setting. Helen Keller National Center, Sands Point, NY.
  • Myer, L.H., Park, H.-S., Grenot-Schwartz, M., Schwartz, I.S. and Harry, B. Supporting Deaf-Blind Students to Develop Social Relationships. Making Friends: The Influence of Culture and Development. Baltimore, Paul H. Brooks