Usher Syndrome in the School Setting
Usher Syndrome in the School Setting
Ilene Miner, CSW Joe Cioffi, M. Ed.
- Published by
- Helen Keller National Center
- Technical Assistance Center
- Sands Point, NY 11050
This document also available in Ascii Text
The development and dissemination of this monograph was supported by cooperative agreement #H025E50001 from the U.S. Department of Education. The opinions expressed in this monograph are solely those of the authors. No official endorsement by the U.S. Department of Education is intended or should be inferred.
About the Authors
Ilene Miner, CSW, ACSW, is a certified social worker who has worked with deaf and deaf-blind youth for 17 years. She is fluent in American Sign Language. She is currently consulting clinical social worker at Helen Keller National Center in Sands Point, New York where she provides individual counseling, psychosocial assessments, and co-facilitates the Usher Support Group. In addition, she is consultant to a mental health program for deaf, deaf-blind and hard-of-hearing people in New York City. She has published several articles on the psycho-social aspects of Usher syndrome, has just completed a chapter for a book on psychotherapy with deaf-blind people, is consultant to state deaf-blind children's programs, and presents at workshops related to Usher syndrome around the country and abroad.
Joe Cioffi M.Ed. is a deaf-blind education specialist and a certified Orientation and Mobility specialist. He is fluent in ASL and has worked with deaf-blind adults and children for 17 years. Currently, he is working as a free-lance consultant for a variety of schools and state agencies which provide services to deaf-blind adults and children. He has published articles previously on the subject of Orientation and Mobility and deaf-blindness, and has traveled extensively in the USA and abroad providing training to professionals in Usher syndrome and Orientation and Mobility.
Table of Contents
III. Common Experiences for People with Usher Syndrome
IV. Usher Syndrome and the Student
V. Usher Syndrome and the Family
VI. Family and School Interaction
VIII. Vision Assessment in the Classroom Environment
Introduction
The Interaction And Relationship of Vision, Mobility and Psychosocial Needs
The issues and services discussed and detailed here are inextricably bound together. Students need early intervention and early contact with adults with Usher syndrome if they are to feel good about themselves and if they are to have any sense of their own selves in the future. Those who are left without will have a harder time feeling confident that they can in fact take care of themselves and manage their lives. If one reviews any public health and prevention literature on chronic disability, one notes that education and preparation for self care are absolute keys to success.
For a host of reasons, this early intervention or involvement of the person with the disability in the process of problem solving, introducing role models, and encouraging the student to take care of him or herself and make independent decisions has rarely been implemented in work with children, youth, and young adults with Usher syndrome.
From our perspectives, as counselor and as teacher, we cannot emphasize enough the importance of our two disciplines working together to have the maximum impact on the lives of youth with Usher syndrome. A counselor cannot work with young people who are worried about whether or not they can take care of themselves if they are not at the same time having instruction that will teach them the skills they need to do just that. Many students have been languishing in school feeling totally at the mercy of their retinitis pigmentosa. Early intervention offers action and success instead of passivity and failure. The current system of education and rehabilitation waits too long and allows a succession of failures and discomfort in many situations to pervade the lives of young people with Usher syndrome.
One young person we have worked with had been very frightened about the future and quite depressed said, after several mobility lessons, "I know now that I am learning to care for myself and I don't have to be frightened anymore."
It's best we heed her words. Early intervention is the answer. We must give students the information skills they need to become functional independent adult partners with us. They, after all, have Usher syndrome; we don't.
We want to thank all the people with Usher syndrome who have provided us with a rich education which we are hoping to pass on to still more people. Every minute has been a learning one, thanks to all.
Ilene Miner 1506 Venice Blvd # 115 Venice, CA 90291 310-740-7304 Email: ileneminer@gmail.comJoe Cioffi P.O. Box 1159 Old Chelsea Station New York, NY 10011 V & TTY 212-677-4575 joecioffi@mac.com
Usher Syndrome in the School Setting
I. Defining Usher Syndrome
Usher syndrome is the name given to a group of genetic conditions involving both hearing loss and retinitis pigmentosa, a progressive, degenerative eye disease. There are three types of Usher syndrome described in the chart that follows. Some people with Usher syndrome are profoundly deaf and some are hard-of-hearing. Some may be born hearing or hard-of-hearing and lose their hearing later. One group has significant balance problems in addition to vision and hearing loss. There are seven genes already identified that cause Usher syndrome and there may be more.
Does a child inherit Usher syndrome from his or her parents?
The Usher Syndromes are all autosomal (not sex linked) recessive conditions. This means that both parents are asymptomatic carriers (having no symptoms of Usher syndrome). Neither parent knows that he or she is a carrier of this condition until they produce a child with Usher syndrome. Both parents must be carriers; each pregnancy of this couple carries a one in four chance that the child born will have Usher syndrome. If only one parent is a carrier, then the child born cannot have Usher syndrome.
Parents find out they are carriers of Usher syndrome only when their child is diagnosed. Typically they have never heard of the condition before and are offered little information. Consequently the diagnosis, which means that their deaf or hard-of-hearing child may well become blind, is a terrible and overwhelming shock.
How common is Usher syndrome?
Among children who are born profoundly deaf, 3 to 6 percent can be expected to have Usher syndrome, Type 1 (see note 1). Among children born hard of hearing, Usher syndrome, Type 2, probably occurs at the same rate (see note 2). Vision screenings for retinitis pigmentosa are not routinely performed in the school setting, resulting in an under-identification of students with Usher syndrome.
Note 1. Kimberling W. J., Möller, C. (1995), Clinical and Molecular Genetics of Usher Syndrome, Journal of the American Academy of Audiology, 6, 63-72 (1995)
Note 2. Personal communication with Sandra Davenport, MD
Types of Usher Syndrome
The following was set in a table of 4 rows and 5 columns. The column headings are Symptom, Type 1, Type 2, and Type 3.
Symptom: Hearing Loss. Type 1: Born deaf with profound hearing loss and has a "corner audiogram" with responses only to very loud, low tones. Type 2: Born hard-of-hearing with a sloping sensorineural loss from mild loss in low frequencies to severe-profound loss in high frequencies. Type 3: Onset of hearing loss 0 - 40 years; progresses rapidly (often over 10 - 15 years) to profound deafness; ski-slope audiogram with speed bump.
Symptom: Balance. Type 1: Absent inner ear balance. Type 2: Normal inner ear balance. Type 3: Some balance disturbance, possibly progressive.
Symptom: Vision loss from Retinitis Pigmentosa. Type 1: Night blindness in infancy or early childhood. Blind spots by late or early teens. Legally blind by early adulthood. Type 2: Blind spots by late childhood or teens. Legally blind by early adulthood. Type 3: Similar to Type 2.
Symptom: Location of gene on which chromosome? Type 1: 1A - Long arm of 14, 1B - Long arm of 11 (most common), 1C - Short arm of 11 (most of Louisiana Acadian descent), 1D - Long arm of 10 (one family), 1E - Long arm of 21 (one family). Type 2: 2A - Long arm of 1, 2B - Ln unknown. Type 3: 3 - Long arm of 3.
Thanks to Sandra L. H. Davenport, MD for designing this chart and permitting its use.
II. Looking for Signs
What things might be noticed first about a child with Usher syndrome?
Although many students with Usher syndrome may be unaware that they have a visual loss, they do realize, and often at an early age, that they have difficulties with vision that other kids don't seem to have.
A common first sign of Usher syndrome is the inability to see clearly at night. Parents or teachers may notice a number of things: that the child trips or bumps into objects; has problems walking around the home in dim light; is uncomfortable with unfamiliar steps or curbs; and may have a heightened fear of the dark.
A second sign is the inability to see peripherally (above, below, and to the sides) under any lighting conditions. This is often described as tunnel vision. A student with Usher syndrome may not notice another student waving hello from the side, an obstacle on the floor, a step or curb, a street pole off to the side of one shoulder, etc.
Another sign is difficulty adjusting to changes in lighting: entering or leaving a darkened movie theater; entering or leaving a building on a bright sunny day.
Additional signs are problems in maintaining balance, blind spots (scotoma), sensitivity to glare (under snow, bright light or sun conditions), and problems with clarity of central vision (acuity).
To summarize, the common signs of Usher syndrome are:
- Night blindness
- Peripheral vision loss
- Difficulties adapting to lighting changes
- Blind spots
- Glare discomfort (often related to developing)
- Balance problems
- Acuity problem
Why do students with Usher syndrome become night blind?
All students with Usher syndrome have hearing loss and retinitis pigmentosa (RP). In the typical course of RP, blood vessels constrict decreasing the flow of vital nutrients to the peripheral retina. The retina of the human eye contains two types of light-receptor cells, rods and cones. Cones allow us to see clearly under daylight conditions and give us our color vision. Rods allow us to see clearly under evening light conditions, and give vision only in shades of gray. The loss of night vision is caused by the deterioration of rods.
The peripheral part of the retina is where there the highest concentration of rods exists. As these cells die, the ability to see clearly in the evening decreases. This is what most students with Usher syndrome experience first, the loss of night vision, or what is commonly called night blindness.
Is there a cure for retinitis pigmentosa?
At this time (June 1997) there is no cure for the degenerative eye disease known as retinitis pigmentosa. Research is moving rapidly and focuses on two main areas: genetic research and transplantation of retinal tissue.
III. Common Experiences of People with Usher Syndrome
What common experiences do people with Usher syndrome share?
People with Usher syndrome often talk about how their parents kept the diagnosis of Usher syndrome from them.
- My parents found out about my Usher when I was 8 but didn't tell me till I was 24. An adult with Usher syndrome.
- My parents found out when I was 11, but never told me. I found out when I finally went to the doctor alone at age 24.
People with Usher syndrome often don't meet another person like themselves until they are adults. They often say they feel as though they have come home when they meet large numbers of others with Usher syndrome such as when attending meetings of the American Association of Deaf-Blind (AADB).
- I never met another person with Usher until I went to the Helen Keller National Center when I was 30.
- I have never seen my child just be so "normal" before. A mother commenting on her teen age daughter's interactions at an Usher syndrome Family weekend.
- When I finally met someone with Usher I felt like this was my sister. We could really talk about important things. A teen with Usher syndrome.
- It is such a relief to meet so many people with Usher. I can just relax and have fun. I don't have to explain myself over and over.
People with Usher syndrome may be resistant to using certain communication techniques and equipment needed to compensate for their vision loss. They often discuss the pain of starting to use a cane for independence in mobility or tactile communication for sign communication in dim light and later even in daylight.
- I was so uncomfortable when I started using tactile communication around acquaintances. I could no longer deny that I have a serious problem. An adult with Usher syndrome, Type 1.
- I miss my deaf friends but I am now more comfortable around deaf-blind people who don't stare at my cane.
- From a 19 year old young man: Oh, I can use the subway just fine without the cane, it's not a problem. I don't need the cane. When asked, But do you think it would help you stay safe? he answered, Yes, but I don't like people staring at me.
People with Usher syndrome are not always offered the training in skills they need early enough to assist them in dealing with their vision loss and its impact on their lives.
- This cane is wonderful. I am embarrassed when I think of how many years I spent bumping into things and people while I was working. An adult commenting on his mobility lessons. Now that I have had mobility lessons, I know I can protect myself if I get caught in the dark. I don't need to be afraid anymore.
People with Usher syndrome say that sometimes their peers avoid or reject them because they are clumsy and bump into things. They may be the target of jokes or teasing.
- People teased me and made fun of me. I was always being picked on and considered killing myself. A young adult describing his high school experience.
Adults with Usher syndrome complain about avoidance and lack of patience of other adults in the Deaf community.
- When I put my hand out for tactile communication, some (deaf) people pull away quickly, hurting my feelings. They act like Usher is contagious. I often sit alone at parties. An adult with Usher syndrome, Type 1.
People with Usher syndrome state that they often have to explain their vision needs over and over again, even to people who interact with them frequently.
- I work with the same interpreters every day, but they can't remember to keep their signs small for me. A teenager in secondary school.
- I have to keep reminding my friends to offer me an elbow in dim light. Why can't they remember?
IV. Usher Syndrome and the Student
What happens to students with Usher syndrome in school and community activities?
Group activities are often avoided because of decreasing ability to handle group communication and increasing inability to move around safely. It is critical that students with Usher syndrome be offered training in skills to help them maintain their independence. When this does not occur, students often start to isolate themselves and to avoid going out. They tend to become dependent on others to take care of all their community needs.
Students who know about their Usher syndrome may be fearful of telling friends because they fear rejection. In schools, teens have reported that there is really no one to talk to, that people tend to avoid addressing the issue. Some students, especially those with Usher syndrome, Type 1, learn quite young that the members of the Deaf community are not always comfortable and sensitive to a student with a vision loss. In addition, their academic performance often declines because of their inability to see everything that takes place in the classroom.
When students avoid group activities, experience stress in the classroom, and are unable to talk about their feelings, the result can be depression.
Should all students with Usher syndrome be in counseling?
Not every student with Usher syndrome requires counseling, but certainly all students should have access to a counselor who is familiar with Usher syndrome. There should also be a regular and periodic psycho-social assessment done with attention to the student's mood, affect, social interactions and feelings. Regular weekly meetings with a counselor who offers support and interest and who keeps in touch with how the student is coping is very helpful.
Does a counselor working with students with Usher syndrome need special training?
It is not enough for the counselor to be familiar with the problems of deafness or being hard-of-hearing or of blindness and vision impairment. It is important for the counselor to be cognizant of the interaction between deafness and blindness and to be aware of the psychosocial aspects of Usher syndrome.
A counselor working with students with Usher syndrome should actually know people with Usher syndrome and should be involved with the deaf-blind community. In this way the counselor can better understand the issues and feelings of people with Usher syndrome.
Is it important for students with Usher syndrome to be informed or to know that they have a problem with their eyes?
Yes, there are several reasons why students should be informed that they have a problem with their eyes:
First, they already know there is a problem. Many teens and adults have reported that they knew around age 12 to 14 that something was wrong with their eyes.
Second, they can receive related educational services (orientation and mobility) and increase their understanding of their vision loss and use the information to keep themselves safe.
Third, eventually, students find out about their diagnosis. If they later realize that parents and counselors knew and kept information from them, there can be a significant disruption in their ability to trust others. They need to perceive adults who are involved with them as trustworthy.
Fourth, students need to develop a sense that Usher syndrome is a part of them and they own it. Young people with Usher syndrome cannot develop a sense of mastery and competence if they are at the mercy of a condition about which they know nothing. Students must know what they are dealing with in order to have a sense of control over their lives.
What should they be told and when?
The responsibility for informing the child or teen about Usher syndrome belongs to the parents. Parents may wish to join in partnership with professionals in the school or health care setting in providing this information. Professionals can clarify information for the student or parents. Professionals can also work directly with the parents on how and when the student should be informed, what to say and can even, if the family desires, be present at the telling. This issue is discussed more fully in the section on parent counseling.
When children are told about Usher syndrome, they need to receive the information in a developmentally appropriate and functional way. They need not be told they will be blind; they should be told that they have a vision problem, not that they will be blind (see section on Usher syndrome and the Family). A young child cannot really understand the concept of future. Children will build on this basic information as they mature. Additionally, teenagers should learn about the genetics of the condition. Older teens typically ask more questions and should have multiple contacts with people with Usher syndrome.
What should students who ask if they are going blind be told ?
The answer to this question must be, "I don't know." Some people with Usher syndrome keep good central vision well into their late adult life. Some lose their vision in their 30s. Others still have vision in their 60s. There is absolutely no way to predict what will happen to any one particular child.
What role can adults with Usher syndrome play in the lives of children and teens with Usher syndrome?
Teens with Usher syndrome connect to adults with Usher syndrome because they perceive that these adults have answers and wisdom to share with them. Including adults in the lives of these young people normalizes the experience of living with Usher syndrome. Being with others who have the same condition allows the students to be identified by their own individual personalities instead of solely as the "Usher kid."
Teens are already thinking and talking about the future and worrying about the time when they may lose their vision. What they imagine possible or probable is often not what does, in fact, happen to people with Usher syndrome. If they meet people who are working and/or going to school, who have families, and who are leading full and busy lives, they can incorporate these experiences into their concept of what the future will be like.
Do teenagers with Usher syndrome have a high incidence of depression?
There has been no study of the incidence of depression in teenagers with Usher syndrome. Although it is clearly not true for every teen, many teenagers with Usher syndrome report being depressed and frightened, having thought about or actually tried to commit suicide. Adults, when interviewed, also report depression and thoughts of suicide, as well as some suicide attempts in their teen years.
Teens report that people don't want to discuss their fears or sadness with them; that people withdraw; that they cannot imagine their future and feel that none of their dreams will come true. This is another reason why it is so important for adults with Usher syndrome to be involved in their lives.
Why isn't this issue of depression mentioned more frequently?
Many people are afraid to raise the issue of depression and thoughts of suicide because they believe that this might put ideas into young people's heads. This is not true. Asking about depression and ideas of suicide may save the life of a depressed teenager.
What other issues do students with Usher syndrome worry about?
Teenagers worry about losing their friends. They may feel embarrassed by their vision problem which is already making its impact felt; they may feel damaged and diminished; they may have trouble following conversation in groups; they may avoid people and at the same time report that friends avoid them; they may be angry because they can't do some of the things that their friends do, like driving; they may be angry at parents for giving them Usher syndrome and jealous of siblings and friends who don't have Usher syndrome.
Some say they never want to have children because they don't want them to go through what they are going through. Others want to hurry up and fall in love and have children quickly while they can still see. They think they will never have a normal life, a family or a job. They often don't know what is available for them in terms of skills and equipment. Without role models and examples of successful people in their lives, they think that nothing is possible for them.
They may be very sad about being different. They know their lives are not the same as the lives of their friends without Usher syndrome, and they don't know what the future will bring.
Teens with Usher syndrome also worry about abandonment by their caretakers. They are aware that they won't have parents forever. They also have tremendous concerns about communicating with their family after they start to lose their vision, especially if their parents don't know sign language.
Do students with Usher syndrome have other topics they want to discuss in counseling?
At the same time students with Usher syndrome are worrying about their decreasing visual fields, they are still students who share the concerns of all young people of their age: popularity and attraction to others, sports, ambitions for the future. Students need a place and a person with whom they feel safe to discuss all their concerns.
Can't students with Usher syndrome discuss these issues with their parents?
Of course some do discuss these issues with their parents, but many teens also report that they do not. Some teens feel a strong need to protect parents by not adding to their pain and worry. Some teens blame themselves for parental depression, illness, or divorce. In these situations, intervention for parents and the student can be helpful in clarifying issues.
Is group or individual counseling best?
Both individual and group counseling have a place in the life of the student, and both should be offered if at all possible. Individual counseling can provide a place to be safe and a single person with whom the student can explore issues, ideas, and fears.
At the same time there is enormous power in groups. Group counseling can offer opportunities to compare feelings and thoughts with others experiencing similar problems. Shared problem solving can bring about changes in their school and home lives, e.g. recommendations for better lighting or a workshop to discuss problems with staff.
V. Usher Syndrome and the Family
What leads the family to suspect their child has an eye problem?
Parents report noticing clumsiness and mobility problems in dim light and the dark. They notice that their child has bicycle accidents and bumps into people frequently. Sometimes teachers notice that the child is missing signed communication and friends trying to get their attention. Teachers and parents may try at first to dismiss what they notice or they may discuss what they have seen with the child's primary care doctor who may then reassure parents that nothing is wrong.
What kinds of experiences do parents have in terms of finding out about Usher syndrome?
Many parents report very stressful experiences in dealing with the medical profession during the process of trying to determine what is wrong with their child.
- "My doctor told me my child would be blind within the year."
- "My doctor told me my child was significantly developmentally delayed, and then he said good-bye."
- "Three different doctors told me there was nothing wrong with my daughter's eyes, the pediatrician, and two eye doctors."
- "My doctor told me, 'There is nothing I can do; take him home and teach him braille'."
Some children are mis-diagnosed or the parent's perceptions are not taken seriously. When they are finally given an accurate diagnosis, it is often brusque and without further referral or connection to support services.
What medical specialists should be involved in making this diagnosis?
If it is suspected, a family should first consult with an ophthalmologist. If possible, the child should also be seen by a retina specialist. The diagnosis is made by examination and an electroretinogram. In addition, families should also be referred to a geneticist who has knowledge and understanding of all the related issues and is especially trained to work with families.
How do parents respond?
A diagnosis of Usher syndrome is always a terrible shock, even when there has been a suspicion that something is wrong. The shock may be complicated by being given little or no information, or wrong information and no helpful referrals. Most parents have never heard of Usher syndrome before.
Parents may have feelings of depression, anxiety and a feeling of confusion. In most cases, the parents must start the search for information on their own. Typically, the next few months are extremely difficult. Parents report not knowing anyone who can offer them support, which often results in their withdrawal from friends and family members. Despite being told that Usher syndrome is genetic, they don't understand that with Usher syndrome both parents must be carriers for the child to have it and they sometimes blame each other for what has happened.
Who provides advice and support to the parents?
At first, there may be little advice and support for the parents. Some parents have been told there was no one who could help them only to find, after many phone calls and letters, that this is not true. It invariably takes time to find out about local services for children who are deaf-blind, although states do have programs. (Programs and services are listed in Resources)
Finding other parents of children with Usher syndrome also takes time, but once found, they can be a tremendous source of help and support. Connecting with parents of children of varying ages can also provide needed information about Usher syndrome through the life cycle.
What are the immediate issues confronting parents?
One immediate issue is what to tell the child when Usher syndrome is first diagnosed. Some professionals wrongly advise parents to tell the child that he or she will probably be blind. This is not what should be done. Parents should not feel they must immediately give a prognosis to their child. They should allow themselves time to begin to integrate what is happening. They will need time to progress in their own understanding and to gather the necessary supports.
What should parents tell their child about Usher syndrome?
Young children won't really grasp the information. Teens who can think in abstract terms may be totally overwhelmed by the diagnosis. Every child must be given information in a developmentally appropriate and functional way. One might explain to younger students that they will need to be very careful when crossing a street or riding a bicycle because they have an eye problem that does not allow them to see well to the sides or up or down. This kind of description avoids the use of words like peripheral which they may not fully understand. Parents can also tell their children that if they have difficulty seeing in the dark, Mom or Dad will hold their hand or offer an arm if they need it. This approach will help prevent children from becoming marooned in a spot without help.
Parents and professionals should anticipate that explanations of Usher syndrome to a child or adolescent may have to occur multiple times. Each explanation provides an opportunity to present a bit more information than the last time. In addition, the genetic information and ideas for experimental treatment are changing rapidly. Information that is valid today may change tomorrow. It is important for parents and professionals to stay current.
What should parents be aware of regarding their children's feelings?
Parents should be aware that their children may be depressed about their decreasing vision and that it is better to talk about these feelings and not ignore them. Parents should also be aware that many children and adolescents with Usher syndrome feel responsible for parental grief and pain even though this may not seem logical to the parent.
What can parents do about their children's feelings?
Parents can encourage children to express their feelings. It helps to assure the child that, although parents may be concerned or even upset, the child did not cause this to happen. Parents, however, cannot and should not share all their feelings and concerns with the child. Parent and child can both be worried about the same issue, but boundaries must be maintained.
If the student is depressed, the parents should listen and offer help. If any thoughts or attempts of suicide surface, parents should seek professional help from a counselor who knows about deaf-blindness. The counselor should understand that having Usher syndrome is not the same as being deaf and sighted, and is not the same as being hearing and blind. If American Sign Language is the child's preferred mode of communication, the counselor should also be able to communicate in that language.
VI. Family and School Interaction
How should school staff initiate contact with the family?
When initiating contact with the family, indicate that you want to discuss the impact of Usher syndrome on their child in the classroom and school environment. You want to know how the family deals with this issue at home. Let the family know that their help and input is crucial. If the parent does not want the child to be told about having Usher syndrome, do not urge the parents to do so, especially during the first interview. In fact, your message should be the opposite. Indicate that you will not push them to do something they cannot or will not do. The first step is to listen.
Parents may need to express their grief and worry before discussing other issues with the school staff. It is crucial to form an alliance with parents, to let them know that you are working with them in the best interest of their child. They are, after all, the primary caretakers.
What should be done if the family refuses to talk about what is happening to their child and will not allow the child or school staff to be informed of the diagnosis of Usher syndrome?
This is a question frequently asked by school personnel. Parents' refusal to talk about their child's diagnosis may indicate that they are frightened, or that their needs are not being met. Parents must know they have the power to make decisions that affect their child. It is crucial not to push parents to the point where they feel they are losing control.
Parents should be asked about their concerns related to school and told that their input is really needed. They should be told that no one is going to insist that they make any particular decisions or changes, but that the school needs to know what is on their minds.
Many parents have been told by school personnel that they must tell their child about Usher syndrome and that he or she is going blind. This is the wrong approach. It is too overwhelming and threatening and often results in avoidance and flight. The process must be allowed to unfold naturally.
Tell parents that you want to hear their reasons for not informing their child. It is also important to ask what the parents think school staff want to tell the child. They may think that school staff wants to tell the child that he or she is going blind. As discussed previously, this is not the message to give the child. Assure the parent that you want to develop a joint plan and that staff will not disclose information without parental permission.
It is crucial to develop a working relationship with parents and to understand their feelings. Once the family realizes that the school staff is not taking over the parental role or recommending a course of action which the parent disapproves, the parents are more likely to work collaboratively with the school.
All of this is a process and sometimes it moves slowly. For example, some parents don't want the term Usher syndrome to be mentioned, but approve Braille and mobility lessons. Compromises can be achieved so the child receives needed services and support. Continue to work with the family and agencies that provide support services. The goal is to assure that all students get the most out of their education.
What should parents know about planning for their child's future career?
Appropriate transition planning is crucial. It is too easy to dismiss planning as unimportant because of the assumption that vision problems will make most career choices impossible. The crux of the issue is: should students be trained for now, trained for later, or trained for both?
People with Usher syndrome and other deaf-blind conditions are able to go to college and vocational programs and to work at all kinds of jobs. Among them:
| agency administrators | librarians | ||
| high school teachers | chefs | ||
| rehabilitation counselors | medical transcriptionists | ||
| health planners | hospital maintenance workers | ||
| mental health counselors | restaurant workers | ||
| school counselors | clerks | ||
| computer operators | typists | ||
| ministers | artists |
It is imperative that doors not be closed to students before they leave the secondary school setting. A diagnosis of Usher syndrome should not be the only factor when determining a future course of study. People with Usher syndrome may be able to work for many years at their first choice of occupation. Adaptation and use of special equipment may be necessary along the way and, at some point, there may be a need for possible career change. But students should be allowed to explore their preferred choices.
- "A college student may be able to work for 20 years at his or her first choice occupation. Why cut off their preferred choices before they have a chance? Most people don't do the same thing for more than 20 years anyway." A rehabilitation counselor with Usher syndrome, Type 1.
Many parents don't consider American Sign Language important to their children's well-being. What should be done?
Children with Usher syndrome are slowly losing their vision. They become night blind early, in childhood for some and in adolescence for others. This means that they cannot use lip reading to augment what they hear unless they are in perfect lighting conditions. Parents need to know that all children with Usher syndrome benefit from knowing and using American Sign Language. It may not be their only choice of language, but it should be included for all.
Even children with cochlear implants (see note) and children who are hard-of-hearing will need additional communication methods at some time. When they cannot augment their hearing with additional visual methods, they may well need to add tactual sign techniques (hand-over-hand signing or finger-spelling) to their repertoire of communication methods.
Note: Cochlear implants are electronic aids designed to provide people who are deaf with sensations of sound. Cochlear implants differ from hearing aids: hearing aids amplify sounds while cochlear implants change sounds into electrical impulses. These electrical impulses are sent to the brain through 22 electrodes or wires that are implanted in the head.
Many parents do not know American Sign Language and see no reason to learn because they can communicate orally with their children. While this may be true initially, it may not be true when the child loses a significant amount of vision, or when the child and parent are communicating in dim light. To quote an adult with Usher syndrome:
- When my mother found out I had Usher, she didn't learn sign. When I started to lose my vision, she didn't learn sign. When my vision started to get much worse, she didn't learn sign. When I lost my vision, I lost my mother.
Some people with Usher syndrome lose virtually all contact with their families after they lose their vision. It is tragic and preventable.
VII. Orientation and Mobility
Orientation and mobility is a term usually heard when talking about services to people who are visually impaired or blind. Orientation and mobility represents a body of various skills and techniques that can help people with visual loss travel more safely and independently.
Mobility
Mobility refers to the safe movement of the body in any space. As individuals with Usher syndrome experience a loss of vision, they may encounter mobility difficulties walking through indoor and outdoor areas: tripping, falling, bumping and loss of balance. There are specific skills which a mobility teacher can introduce to address and help resolve these mobility problems. To name a few, there are self-protective skills, trailing, cane skills and visual scanning techniques.
Orientation
Orientation refers to the ability to understand the body's position and location relative to other points in the environment. It also refers to the ability to systematically plan routes to other destinations. Most hearing and sighted people orient themselves by relying upon visual landmarks . Most hearing blind people orient themselves primarily through the use of sound cues. Students with Usher syndrome have limited or no access to sound cues and experience increased difficulty using visual landmarks.
There are specific skills that the orientation and mobility teacher can introduce to help resolve these orientation problems. These include alternative land marking skills (using touch, smell, wind, slope and other cues), mapping skills and route planning skills, among others.
What kinds of difficulties in orientation and mobility do students with Usher syndrome typically experience?
Usher syndrome students, who continually need to readjust to a progressive and deteriorating visual condition, will likely experience difficulties in the following areas:
- Mobility - safe movement of the body through the environment.
- Orientation - awareness of the body's location within an environment.
- Communication
- Using decreasing residual vision
How can orientation and mobility services help someone with Usher syndrome?
Students with Usher syndrome usually experience a slow and progressive loss of peripheral vision, and this may be followed by a loss of central vision. As the visual loss becomes more advanced, people with Usher syndrome have increased difficulty getting around safely. Orientation and mobility services can teach students to develop and maintain a high level of safety, independence and confidence as they travel and interact in school, at home and in the community.
Many students with Usher syndrome simply stop going out into the community alone once they experience vision and travel problems. Not knowing what they can do to make travel easier, they avoid what often becomes a frightening or embarrassing experience. They depend increasingly on family and friends. A routine of dependent activity, and sometimes inactivity, develops.
- An 18 year old girl, a high school student with Usher syndrome, spent the last two summers traveling by train to a part-time job. She enjoyed the work, and hoped that continued work experience would help her gain competitive employment after graduation. In the past 6 months, however, she has lost more vision and now has a number of problems traveling and communicating alone. Street crossings have become difficult, and minor collisions in the crowded public transit system have become so frequent that she has decided not to travel alone any longer.
This is a student who can greatly benefit from orientation and mobility instruction. Her ability to work in the future may well depend upon her continued ability to travel alone. Instruction in scanning techniques (systematic and careful use of her residual vision) and basic cane techniques will together allow her to travel through her community environment with maximum safety. A second area of instruction, expressive and receptive communication strategies to be used while traveling, would help her communicate with the public confidently and comfortably.
What is the goal of an orientation and mobility evaluation?
An orientation and mobility evaluation is an assessment of the visual, auditory and/or tactile techniques that a student uses to travel independently and safely. The evaluation should determine if there is, in fact, a need for orientation and mobility instruction and if so, in what specific skill areas. Should instruction be recommended, the student and instructor should mutually develop realistic short and long term goals. A plan of instruction would then be implemented to gradually build the skills necessary to reach those goals.
What would an orientation and mobility instructor do first to evaluate a student with Usher syndrome?
The mobility instructor should review vision reports and school records to gain an understanding of the student's particular vision loss. The instructor should then meet with the educational team to share observations and to explain the characteristics of the student's visual loss and the purpose of the evaluation.
If the student uses sign language, the instructor should also meet with the interpreter to explain the characteristics of the student's visual loss and the purpose of the evaluation, which is an assessment of visual efficiency skills, including scanning and comprehension in the classroom. If the orientation and mobility teacher is not familiar with the use of an interpreter, this is an opportunity for the interpreter to explain his or her role.
What kinds of situations should an orientation and mobility instructor include in the evaluations?
The instructor first observes the student in various natural school settings. Then, after the student and instructor have met and discussed the purpose of the evaluation, the instructor conducts the evaluation in what would be typical problem areas for a student with Usher syndrome. Indoor settings to be evaluated include stairs and stairwells, crowded areas, areas with random obstacles or poor lighting, and typical school settings such as the cafeteria, auditorium or locker rooms. The student would also be observed in outdoor settings under various sun conditions (with the student facing the sun and with the sun at the student's back), in familiar and unfamiliar areas, at street crossings and congested areas, on public transportation systems, etc. A thorough evaluation must include observation of the student in familiar as well as unfamiliar low light areas.
Every attempt should be made to conduct evaluations in home, school and community environments. It is important to include the family in the process by interviewing them and discussing the purpose of the evaluation and of orientation and mobility education. The teacher should encourage the family to learn as much as possible about Usher syndrome and should provide necessary supports and reinforcement of independence and safety recommendations.
What is the function and purpose of a cane?
There are several reasons for using a cane. Some students with Usher syndrome with residual vision may benefit by selectively applying specific and distinct cane techniques in different lighting, environmental, and/or fluctuating visual conditions. Some won't choose or need a cane until they are much older. The three main functions of a cane are identification, protection, and information.
Identification
A student with Usher syndrome, even one with considerable residual vision, may still benefit by using a cane, even if the cane is only for the purpose of identifying their vision loss to others. When traveling in a crowded city environment, e.g. using public transportation or crossing city streets at rush hour, the presence of a cane will greatly minimize collisions and difficulties. At street crossings, the cane is an important identifier to drivers, making it safer for students who use vision techniques alone to cross streets.
Protection
Some students' visual fields have become so restricted that, even when applying scanning techniques, they occasionally bump into obstacles. In these situations, a cane can offer a great deal of physical protection. Objects which are outside the central field of vision, such as a street pole, a curb or a knee-high sidewalk obstacle, can be safely touched and noted by the cane, thereby avoiding a collision.
Information
When traveling in the evening, or when a student's residual vision requires regular use of a cane, the student learns specific techniques where the cane is used to gather tactile information, such as assisting in identifying landmarks, establishing position at a street corner or on a train platform.
Why is early cane education important?
Information about the purpose of a white cane, and the benefits it can bring with someone with Usher syndrome, should be explained to a student at the earliest opportunity. Even for those who may not yet need to use a cane, simple lessons demonstrating basic cane technique can give a student valuable information for their future safety. Some professionals in blindness education have suggested that if a student has adequate vision to travel without a cane at the time of evaluation, instruction in cane techniques should be postponed until functional vision decreases. This approach is discouraged for the following reasons:
- The condition of retinitis pigmentosa can change rapidly, unpredictably, and substantially.
- For individuals who are deaf-blind, access to state rehabilitation services and professionals is often extremely limited, and these systems can be very slow in processing applications for new cases.
- If the student does, in fact, lose more vision and begins to travel unsafely, this situation may continue for a significant period of time before services are ultimately provided.
The approach should be one of crisis prevention rather than crisis intervention. Students should be encouraged to get all the information they need in order to become active partners in the learning process. If they have the necessary information and skills, then they are likely to note before anyone else when changes happen and when they need more education. This approach is empowering and builds confidence in the student's ability to care for him or herself.
- A young adult with Usher syndrome, employed in a large urban city, had for several years been traveling to and from his home and office. In the space of one year, his vision deteriorated substantially, and he was unable to travel safely across streets, or in the congested and complex environment of the city's transit system. Attempts to initiate orientation and mobility services on his own were extremely delayed, and he was forced to wait an entire year following his initial request for services. During that year, he was hit by a car on two occasions and had several accidents on the subway platforms and stairways of the transit system. He had never been shown or given a cane, despite the fact that his school had known for years that he had Usher syndrome.
Could a model of early intervention and crisis prevention have made this individual's experience safer and less traumatic?
Early intervention would have made a tremendous difference in this young man's life. He was having significant problems in traveling safely, and he knew he needed training. If he had received even the most basic information about a cane and been given one, he might have carried it with him for identification during this waiting period for services, and the accidents he experienced would, in all likelihood, have been avoided.
Are orientation and mobility and vision services routinely provided in schools for students with Usher syndrome?
Most students receive no services in school until vision loss has reached an extreme level. In many cases, the problems and issues of the student with Usher syndrome are not discussed or addressed at all. Often, it is not until the student graduates and begins to explore options for adult life that students come in contact with specialized services. This experience is common and often results in crises occurring rather than being prevented. If some vision services (O&M specialists, vision specialists) are provided it is often by an instructor with little or no experience in deaf-blindness. Interpreters are not always used, making it extremely difficult for rapport and trust to develop between student and teacher and impossible for adequate exchange of information or education to take place.
How can frustrations experienced by students with Usher syndrome in communicating with the public be dealt with effectively?
The inability to communicate comfortably with the hearing public is a common frustration for students with Usher syndrome. Deafness is invisible to the public, and if the student is not using a white cane, his vision impairment is also invisible. The bus drivers or store clerks with whom the student is trying to communicate have no idea that the student has a hearing or vision loss. Misunderstandings can result when the student does not obtain the information he needs.
One strategy that works well to reduce these misunderstandings is to use a simple but individualized communication book. Laminated cards are prepared in bold print at a comfortable font size and can be used with dry erase markers for a variety of community and travel needs. A tiny flashlight can also be attached to the book for use in low light situations. By laminating the cards, they can be used and then erased repeatedly, and can last under different weather conditions. In cases where visual acuity does not allow for the use of large print cards, tactile labels using a highmarker or braille can be used.
Are all mobility instructors prepared to work with students with Usher syndrome?
In general, mobility programs at the graduate level focus on the needs of people who are blind and hearing. They offer little or no training for people with a hearing loss who also have vision problems. The mobility instructor who receives a deaf-blind referral may be intimidated by the communication barrier and not know exactly how to proceed. This is especially true in cases of Usher syndrome, Type 1 where ASL is the primary language and profound deafness prohibits receiving instruction through voice.
Without effective and fluent communication between student and instructor, a wide range of visual abilities and skills will be overlooked. Some of these are visual efficiency skills, problem solving skills, judgment in combination with scanning skills at street crossings, and the ability of the student to follow the pace and maintain the comprehension of a classroom dialogue.
If there is inadequate or unclear communication, students may be given a false sense of security and travel unknowingly at high risk. Poor communication can result in tragic consequences.
In cases of profound deafness where ASL is also the primary language of the student, the orientation and mobility instructor should ensure that a skilled ASL interpreter, preferably certified, is present during the evaluation and any subsequent training. The strengths, weaknesses, needs and potential of a student with Usher syndrome cannot be evaluated independently by any orientation and mobility teacher or vision specialist who does not share the language of the student.
Do interpreters or other staff need any extra training or information to work effectively with a student with Usher syndrome?
Interpreters need information regarding sign methods for students with Usher syndrome and must learn about and be comfortable with tracking and tactile signing, both of which require touch. Interpreters should also learn the techniques of sighted guide and some basic mobility skills as well.
VIII. Vision Assessment in a Classroom Environment
What is special about the assessment of functional vision of students with Usher syndrome?
The evaluation of functional vision involves communication and comprehension of both signed and spoken language and of everything else that is happening in the classroom. This differs substantially from the evaluation of hearing students who have similar visual problems. For the most part, the orientation and mobility teacher or the vision education specialist has had no previous preparation for this task which includes knowledge and expertise from the fields of both blindness and deafness.
The specialist must evaluate the ability of students with restricted fields of vision to maintain an adequate level of comprehension of the classroom dialogue. This is particularly important for student's with Usher syndrome, Type 1. As that student's already restricted field of vision continues to decrease, the ability to compensate visually by modifying scanning techniques becomes increasingly more difficult. At a certain point, the student is no longer able to scan effectively and follow both the teacher's signs and the signs of other students in the class. If the student has to continue to depend upon scanning alone, he or she will experience visual fatigue, diminishing comprehension and will demonstrate decreased academic performance.
The student may be able to attend to the teacher without a problem, but when there is interaction between students and teacher or among students, students with Usher syndrome, Type 1 have a hard time locating the person who is talking or signing.
When conducting a vision evaluation of a student with Usher syndrome in a school for the deaf, it is imperative that the assessment not be restricted to the students' viewing of the teacher's stationary signs. An evaluation of active scanning and simultaneous comprehension of class dialogue is vital as well.
When working with a student with Usher syndrome Type 1, what other skills must the evaluator have beyond education in orientation and mobility or vision to conduct this type of evaluation?
Ideally, this evaluation would be conducted by a vision or orientation and mobility specialist who is fluent in American Sign Language (ASL). Realistically, however, there are very few specialists with this skill. It should be emphasized that a specialist without ASL skills or with only some familiarity with sign language should not be conducting this part of the evaluation alone.
It is more realistic to have an ASL-fluent member of the vision/education team (possibly the primary advocate or safe person) join the vision or orientation and mobility specialist with this part of the assessment. This person should first develop an understanding of the student's residual vision characteristics, perhaps in consultation with the vision specialist, before attempting any joint assistance.
What factors are involved in determining a student's ability to participate visually in the classroom?
The first step is to determine the best possible placement of the student in the classroom, to find the place from which scanning techniques can be most easily and efficiently applied.
Other factors to consider are: distance from the signer, teacher or blackboard, the location and type of lighting source, the appropriate size and contrast of printed classroom and hand-written blackboard materials, the background and clothing contrast of signer, and the position of the student relative to the lighting source. Once these variables of position, lighting, environment, size of materials, and contrast are addressed, the specialist can observe the student following classroom dialogue and interaction over a period of time.
A deaf student with retinitis pigmentosa (RP) experiences more eye fatigue trying to follow classroom information than does a hearing student with RP. It is through visual scanning alone that he or she receives information all day long.
By comparison, hearing students with RP can still follow classroom information through sound. If they choose to rest their eyes, they can do so easily and without sacrificing comprehension or attention to the class lecture or dialogue. This is not so for the student with Usher syndrome, whose struggle to follow class dialogue is analogous to a spectator at a tennis game watching the match through a straw. It is inevitably a tiring experience, and as visual fields decrease, it may eventually be futile.
Once the actual difficulties of visual participation are understood, the gradually decreasing academic performance of students with Usher syndrome comes as little surprise. Exceptions to this would include students with Usher syndrome, Type 2 or 3 who have sufficient residual hearing to follow voice instruction with amplification equipment or other assistive devices. Taking the above factors into consideration, evaluators should schedule various times during the day and week to make observations.
If it is found that the student cannot visually gather classroom information comfortably and efficiently, what should be done?
If scanning techniques and other strategies to maximize visual efficiency (such as position of the student, lighting and background contrast of the signer) fail to bring the student to a point of reasonably full participation in the classroom, then it is important to explore specialized and individualized interpreting services.
What can be done about communication problems that arise?
Ideally the Usher syndrome student should be able to communicate comfortably and efficiently with both users and non-users of sign language, even as the student's vision loss becomes more advanced.
A variety of skills can be taught to the student with recommendations to teachers and family members to help solve difficulties. Examples might include:
- scanning techniques
- signing confined to a small central space
- using visual tracking
- using tactile sign language
- positioning the student appropriately in relation to the signer
- using appropriate lighting
- keeping an appropriate distance
- using high contrast backgrounds
- using appropriate size and contrast of printed classroom materials
- using interpreters in special situations
- employing equipment that improves visual participation
Some suggestions in the above list are only for those who use American Sign Language and some are for both signing and oral students.
Why should tactile sign language be introduced to high school students with Usher syndrome?
As a general rule, tactile sign language, for those using sign language, should be introduced to students with Usher syndrome at the earliest opportunity. In most cases, this a skill that the student already needs to use in evening environments and with family members under various lighting conditions.
Although individual assessment always needs to be done to determine the student's readiness to accept instruction in these areas, training in tactile sign language skills for those using sign, protective and trailing skills and sighted guide skills can all be introduced in a non-threatening way under very comfortable conditions.
Occasionally students are uncomfortable discussing vision loss, or their parents have refused to give permission to discuss the subject. In such situations, an instructor can pair students with each other (or with the instructor) and play simple games with blindfolds, all structured to facilitate the development of various necessary skills without pressuring the student. For those older students or more mature students, the teacher can use a direct approach doing training sessions under actual evening conditions.
What is meant by specialized individual interpreting services?
Individualized interpreting may require a range of methods, including signing at some distance from the student in a small physical space, using tactile tracking, using fully tactile sign language, or a combination of these methods. A selective and varying use of these three methods will be needed as the student's visual fields narrow (and involve central field and acuity loss). At this stage of vision loss, the student's visual condition and corresponding needs may fluctuate as they are influenced by health, stress, fatigue, available lighting and other factors.
What about Braille education?
Exposure to orientation and mobility instruction and instruction in tactile communication methods are of primary importance. After these needs have been met, the school vision consultant should introduce the student to Braille to see what it looks like and how it works. Students should see a Perkins brailler and should learn as they get older that computers can be operated with refreshable Braille output.
The authors support early exposure to Braille so that it is done at a time of relative stability and strength in the life of the student. Adolescents with the ability to use abstract reasoning will already be wondering how they will use a computer, go to college, or read a book if they lose their remaining vision. The goal is to avoid having the student become dependent and despondent when they experience further vision loss. Students need to know there are ways of managing their lives successfully. They need to be in control and not constantly be place in dependent situations requiring the assistance of hearing/sighted adults. Early exposure to and acquisition of skills in Braille will contribute to the development of a person who believes they can take care of themselves. It is for these reasons that early exposure to Braille is supported.
IX. Next Steps
What actions should schools take so they can provide timely and appropriate services to students with Usher syndrome?
There are several steps that schools can take to work with children with Usher syndrome and their families and teachers to ensure that the children have positive feelings about themselves and their school experiences and so they can make optimum use of their vision.
Steps include:
- Comprehensive vision screening for students
- Specialized training for staff
- Monitoring students
- Counseling and support for students
- Establishing Usher syndrome support groups
- Modifications
Vision Screening
Periodic visits to a low vision optometrist or ophthalmologist will monitor changes in students' visual fields or sharpness of vision. Students should also obtain appropriate sun filters, prescription glasses, and any magnification aids or devices that might help them. For students who use sign language, interpreters should be in attendance so that students can participate in their own care.
Staff Training
Teachers need to know the following about Usher syndrome:
- Its impact and effect
- Needs of students with Usher syndrome
- Information about genetics
- Outside services and resources
- Needs of adults with Usher syndrome
Training that covers these issues can be arranged. The Resource section lists a variety of national and local contacts that can provide consultants to work with members of the school staff.
Monitoring Students
Some schools have designed and designated a vision team. Team members include the school nurse, guidance counselors, pupil personnel staff, a member of the administration, some classroom teachers, the school's consulting doctor. Others who are interested or involved might join as well. The team reviews the needs of each child: how he or she is doing in class and socially, the medical history and visual field reports, classroom and other environmental needs, psychosocial needs, and what classroom teachers must do for that child to provide equal access to information. Another topic the team can address is the need for training for school staff about Usher syndrome.
Records should document what is necessary and what is done for each child every year. For example, monitoring visual fields, checking to see if classroom needs are being met or if adaptation is necessary. These services were discussed in detail in the section on Orientation and Mobility.
Counseling and Support
Each child should have a primary advocate, a safe person, who feels comfortable dealing with Usher syndrome issues. This person should be noted in the records kept by the vision team. The issue of Usher syndrome has been overlooked for many reasons, some of which include the resistance to dealing with potential blindness in a deaf or hard of hearing young person. The safe person should be well-informed about Usher syndrome, comfortable with issues of depression and fear as they arise, and who can listen without shutting off expressions of feelings. A common complaint from teens with Usher syndrome is that no one listens or talks to them about their condition. The advocate should be the first line of defense for the student, a haven when the student is upset or frightened.
The safe person can also act as advocate for the student with the vision team, or with classroom teachers and other school personnel. School social workers or counselors are good primary advocates if they are comfortable and knowledgeable about the issues, because they are also trained to look for and evaluate depression. The safe person may be the primary counselor but if not, then the advocate and the student should meet regularly anyway so the advocate is aware at all times of how the student is doing and what problems, if any, have arisen.
Usher Syndrome Support Groups
Young people with Usher syndrome often grow up without meeting other people with Usher syndrome. They often feel that it is not all right to talk about Usher syndrome and that no one really understands their problems.
- I grew up attending a school for the deaf and although we were all deaf and used American Sign Language to communicate, I never met a single other person with Usher except my own sister until I was a junior at college. I was totally isolated, alone, and depressed. My high school years were the worst. A 25 year old graduate student with Usher syndrome, Type 1.
A support group has several functions: it provides a safe and confidential place to express feelings; it can provide accurate information about Usher syndrome that students often do not have; it can invite adults and others with Usher syndrome to visit, to answer questions and to introduce role models to students with Usher syndrome. Support groups should meet regularly and should be facilitated by a safe person or primary advocate who has the necessary skills.
Modifications
Some of these suggestions are ideas for students and some are ideas for school staff. For example, any dim light in classrooms, hallways, stairwells, locker rooms, and any other areas inside school buildings should be evaluated and enhanced. Stairways in the school should have handrails, and proper marking of the first and last step of each series of stairs should be made. If there are streets on the school campus which the students must cross to attend gym or other classes, there should be a crosswalk area prominently designated.
Classroom modifications may include ensuring there is:
- Proper positioning of the student in the classroom, taking into consideration students visual field and acuity, and his/her ability to scan information in a most comfortable manner
- Room lighting, blackboard contrast and appropriately printed materials needs evaluation
- Staff should be instructed to wear clothing with high background contrast and to sign in a small central space
- Specialized sign techniques (tracking or tactile sign) may be required, or it may be determined that individualized and specially trained interpreters are necessary
Many students with Usher syndrome have difficulty expressing their needs and isolate themselves from their peers. However, slight modifications in school activities can help them be more comfortable
Proper lighting in the classroom and cafeteria is a great help to students with Usher syndrome. Those same students will participate more happily in social events if at least one table has extra lighting to help them communicate successfully with friends.
Perhaps most important, a school-wide program to educate other students to the problems and needs of students with Usher syndrome will foster understanding and empathetic behavior. A young person with Usher syndrome is more likely to be offered assistance on a snowy day if classmates understand that people with Usher syndrome are experiencing declining visual fields and balance problems.
In closing . . .
We encourage students with Usher syndrome to meet and spend time with adults with Usher syndrome. Staff who teach and counsel students with Usher syndrome should also participate in activities in the deaf-blind community. It is by being with adults with Usher syndrome in the deaf-blind community at large, that we can all learn about the dignity and resilience of the human spirit.
National and State Resources
National Resources
American Association of the Deaf-Blind 814 Thayer Avenue, Suite 302, Silver Springs, MD 20910-4500 TTY: (301) 588-6545 FAX: (301) 588-8705
American Foundation for the Blind 11 Penn Plaza, Suite 300, New York, NY 10001 PH: (212) 502-7600 TTY: (212) 502-7662 FAX: (212) 502-7777
Boys Town National Research Hosp, Genetics Department 555 N 30th Street, Omaha, NE 68131 PH/TTY: (800) 835-1468 FAX: (402) 498-6331
Helen Keller National Center Headquarters, 141 Middle Neck Road, Sands Point, NY 11050 PH: (516) 944-8900 TTY: (516) 944-8637 FAX: (516) 944-7302
National Association for Visually Handicapped 22 W 21st Street, New York, NY 10010 PH: (212) 889-3141 FAX: (212) 727-2931
National Braille Association 3 Townline Circle, Rochester, NY 14623 PH: (716) 427-8260 FAX: (716) 427-0263
National Family Association for Deaf-Blind
National Consortium on Deaf-Blindness - NCDB Teaching Research Institute- Western Oregon University 345 N Monmouth Avenue, Monmouth, OR 97361 PH: (800) 438-9376 TTY: (800) 854-7013 e-mail: info@nationaldb.org
RP Foundation 1401 Mount Royal Avenue, 4th Floor, Baltimore, MD 21217-4245 PH: (410) 225-9400 FAX: (410) 225-3936
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