Successful Transition of Students with Usher Syndrome from School to Work or Continuing Education
Center for the Study and Treatment of Usher Syndrome
Boys Town National Research Hospital
226 Split Rock Road
The Woodlands TX USA 77381
Dr. Roseanna Davidson
Texas Tech University
Lubbock TX 79409
Issues related to the education of children and teens with Usher Syndrome have changed rapidly over the past decade as scientific advances have lead to earlier diagnosis.† It is now possible to screen newborns for Usher Syndrome before they leave the hospital.† Molecular genetic diagnosis of certain types of Usher Syndrome can be made at any age.† Earlier diagnosis brings many advantages but may also bring complex decisions for the parents of the baby with Usher Syndrome.† Will the diagnosis influence their decision to have other children?† Should they inform the school of their childís Usher Syndrome?†† Will a cochlear implant benefit their childís educational and social assimilation?† Clearly, the need for information about the options available to children with this progressive and combined sensory loss is immediate and necessary following the diagnosis of Usher Syndrome.† Early diagnosis brings with it opportunities for earlier educational interventions that can academically benefit future job seekers who have Usher Syndrome.
Early educational intervention for students with Usher Syndrome should not be limited to specialists working with children who are deaf.†† Educators of students with visual impairments have an equally important role in developing educational programs and managing the successful academic progress of learners with Usher Syndrome.† The early infusion of skills and opportunities offered by low vision devices, orientation and mobility lessons, and proper lighting in the school environment, can provide psychological reassurance and determine a defining role in the studentís academic and employment success.† When introduced early in life, vision skills are regarded as a natural, supporting activity rather than something that will happen in the future when the child can no longer adequately function as a person who is hard of hearing or deaf.† The result is something positive and helpful rather than something in the future to be dreaded or feared.††
The life of a child with Usher Syndrome is full of transitions.† As with children and teens with retinitis pigmentosa, her vision may stabilize but then may diminish due to a reduction in her visual field.† Each loss of vision brings with it a new period of mourning that is experienced by the child and her family.† The preexisting knowledge of vision skills makes each transition easier, less frightening, and provides hope that the student can and will function independently.†† Students with Usher syndrome can use their knowledge of orientation, trailing, and sighted guide techniques when they encounter difficulty traveling due to night blindness.† Children with Usher Syndrome almost always recall difficulty playing night games such as capture the flag, tag, or hide and seek.† Often, these signs were noticed prior to their diagnosis and served as a first indication that something was different in their lives compared to their friends.
In recent years, the rapid scientific advances of genetic researchers in the field of Usher Syndrome have further refined definitions and prognosis for the three types of Usher Syndrome.† Each type of Usher Syndrome has different educational issues that must be further individualized with each childís specific academic program.† Each type of Usher Syndrome also has specific implications for job placement that can be addressed beginning in a studentís school years.
Children with Type I Usher Syndrome usually do not begin to walk until the age of eighteen to twenty-four months.† Type I Usher Syndrome is characterized by an early and profound hearing loss, retinitis pigmentosa, and vestibular problems.†† Usually these children use sign language or have cochlear implants.
Type II Usher Syndrome is characterized a moderate hearing loss in the lower frequencies and a severe to profound loss in the higher frequencies.† There are no vestibular problems in childhood, but the vestibular system becomes less reliable as the individual looses vision from the accompanying retinitis pigmentosa.
Type III Usher Syndrome has a progressive hearing loss and the progressive loss of visual fields and night blindness of retinitis pigmentosa.† In Type III Usher Syndrome the vestibular system gets worse as the person ages.† Children with Type III Usher Syndrome may begin classes with students who are hard of hearing but may experience significant communication delays if their hearing and vision deteriorates.† Educators must constantly reassess the communication needs of these children and remain open to classroom modifications recommended by the VI team members.
Students with Usher Syndrome may benefit from the use of tactile sign language to communicate at night or in low light conditions.†† In all types of Usher Syndrome, the teachers must refrain from moving about the classroom while speaking and should not stand in front of bright windows.†† It is helpful to say the name of each child who is speaking during class to permit successful conversation tracking of the child with limited visual fields.† Solid color clothing that contrasts with skin color should be worn by sign language interpreters and by teachers who sign.† Busy patterns not only make sign language less discernible, but also cause some students with Usher Syndrome to become dizzy.††††††††
At a time when their classmates are expanding their world by learning to drive and staying out later at night, the teenager with Usher Syndrome is often experiencing an increasingly restrictive field of vision and night blindness making these activities more difficult or unsafe without appropriate professional intervention.† In the United States teens with Usher Syndrome often pass driving tests and obtain their driverís license without restriction.† The quickly administered vision test at the driving test site may not pick up the ramifications of night blindness or sudden darkening of the sky due to rain or snowstorms.† Often potential drivers look into a small box that tests visual acuity but not field restriction.† Driverís exams are given during the day when night blindness is not an issue.† A driver with Usher Syndrome may not see a child in the road or hear an approaching ambulance or emergency vehicle siren.† Even if a young driver leaves home on a sunny afternoon, a sudden storm may quickly darken the sky and reduce her useful vision.† Parents of children with Usher Syndrome often permit their child to drive, especially in rural areas where public transportation is limited or unavailable.† Teens with Usher Syndrome, like all teens, want to be like their peers and willingly accept this opportunity for increased freedom.† Some parents and teens think that driving is essential to employment.† It is recommended that a knowledgeable vision professional explain the studentís functional vision to the parent and to the student in a realistic but sensitive manner offering hope through shared knowledge of travel skill instruction, orientation and mobility, and travel enhancing assistive devices.†† Public transportation should be offered as a viable alternative whenever appropriate and available.
Often ophthalmologists including retinal specialists are not knowledgeable about educational topics including travel skills, low vision devices, classroom modifications, and eventual employment potentials of people who have Usher Syndrome.
In interviews with eighty families of children with Usher Syndrome, Miles found that forty-six families reported that the diagnosing ophthalmologist told their child to learn Braille without inquiring about the childís reading level.† Twenty-nine were told by ophthalmologists at the time of diagnosis that their children could not work in gainful employment as adults.†† Clearly, the message is for educators in the field of visual impairments to start with the basics when working with a student with any type of Usher Syndrome and her family.† Emphasis on the possibilities available through the acquisition of travel skills, low vision training, career planning, and communication training provides hope.
The early involvement of professionals in adult rehabilitation and transition services for individuals who are visually impaired including those with Usher Syndrome, is essential to form a transitional pathway to lead to hope in adulthood.†† Students need to hear success stories of adults with Usher Syndrome, who have hobbies, jobs, and who are involved in community and family activities.† It is valuable to arrange meetings between teens who have Usher syndrome and adults who have the same type of Usher syndrome.† This allows for independent communication and exchange of information.††
The knowledge of travel skills should be accompanied by the opportunity for the student and his family to acquire the methodology, skills, and practice necessary for safe and independent travel in indoor and outdoor environments.† Ideally, the indoor instruction of the school campus should be offered each year prior to the start of the academic year when the campus is not session.† If the student needs to learn how to travel to new classrooms during class changes it is important to introduce the new routes when the hallways are not crowded and when they are free of students. Open locker doors, books sitting on the floors, classroom doors opening into the hallway, and other obstacles must be dealt with during the school year.† However, the special communication requirements of sign language for students with Usher Syndrome I or Usher Syndrome III, or quiet hallways permitting optimal use of residual hearing for students with Usher Syndrome Type II or Usher Type III, cannot occur successfully during chaotic, crowded class changes.† It is recommended that the initial instruction be conducted in a quiet, not crowded school building for optimum communication and understanding to take place.† After the essential communication is conveyed, the student can be introduced to increasingly congested hallways or school common areas.†† Orientation and mobility instructors who cannot sign fluently should use a sign language interpreter for all travel skill lessons for students who have Type I Usher Syndrome.† All travel lessons must incorporate considerations for the loss of auditory information from approaching cars, audible traffic signals, and nearby pedestrians.
Students who are regularly introduced to new travel patterns and skills learn to see orientation and mobility skills as a positive and life-enhancing tool for ongoing and increased independence.† Adults with Usher Syndrome who have not been acquainted with orientation and mobility and travel skills during school years, have reported that they view these skills as a dreaded and future necessity only when and if they experience restricted fields or a visual loss that makes independent travel impossible. (Miles survey)† Adults who have come to regard travel skills as an ongoing learning experience are able to transfer this practice to employment settings.† If a new office space design is introduced, or if the employee is transferred to a new floor, he knows the benefits of transferring or modifying his travel skills to the new setting.
After school hours work-study programs allow the student with Usher Syndrome to obtain valuable work experience while still experiencing the support of her schoolís educational team.†† Academic skills required on the job can be strengthened at school.†
The parents of students with Type I Usher Syndrome are increasingly choosing cochlear implants for their children whenever the appropriate rehabilitative services are available in their community.† This development, as well as many other issues, requires ongoing teamwork of educators from the field of visual impairments and those from deaf education to share their expertise.† Education team members familiar with functional benefits and limitations of a studentís cochlear implant must convey this information to members of the VI team.† One young man with Usher Syndrome was on a mobility lesson with an instructor who thought that his cochlear implant meant normal hearing.† The young man could not hear his instructorís verbal directions and was injured and terrified when he fell into a fifteen-foot canal.† But with ongoing team building, student can benefit from the knowledge of both the VI field and the field of deafness.
With ongoing opportunities for independence, self-confidence, and communication the student with Usher Syndrome is able to realize her strengths.† Successful job placement occurs when these strengths are combined with assistive devices, education and rehabilitation professionals, and opportunities that serve as a bridge from the school to work environment.† Once the bridge is crossed, the student has an opportunity to use the skills that she acquired during her school years.† As Helen Keller said, ďAlone, we can do so little, together we can do so much.Ē
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