Understanding the Needs of Deaf People with Retinitis Pigmentosa (RP)
Some Considerations for Teachers, Social Workers and others working in a professional role.
By Mary Guest, Head of Usher Services at Sense
When a deaf child is diagnosed with retinitis pigmentosa (RP), the attitudes of those who make up his or her world suddenly change. He* becomes a victim, the object of pity, and as far as his family is concerned the subject of great anguish and concern for the future. Yet the child does not perceive himself any differently from the day before the diagnosis was made; he has not changed nor has his eyesight.
It is important for everybody involved to grasp that the symptoms which finally led to the child being referred to an eye clinic have been present for some time. The problem of not being able to see in the dark for the deaf child diagnosed at, say 15, is not a new one. Indeed some children report that as far as they can remember they have always had difficulty seeing at night. So it is probable that for at least half the child's short life he has been night blind and has, albeit unconsciously, made some adjustments to his behaviour.
Loss of field vision, although not necessarily pronounced in early life, could also have been part of the deaf child's visual experience for some years before the point of diagnosis. It is probable that this child has already made many adjustments to his head and body movement, unconscious of why it is necessary, in order to scan, move about safely and compensate for decreasing field vision. In other words, the deaf child, without knowing it, has begun to learn to live with visual impairment long before the diagnosis and he may, if circumstances allow, continue to employ such adaptive behaviour in making the best use of his sight after diagnosis. However, if he has to bear an inordinate burden of grief engendered by his parents, his own adjustment to RP may be hindered.
How the newly diagnosed deaf person handles his RP condition will depend not only on the extent and rate of his vision loss but also on the reaction of his family.
Parents and Family
Initially it is upon the parents that the full impact of the diagnosis and its implications usually falls. They are the ones who are told that their child has an eye condition which is untreatable medically, which will tend to get worse and which may eventually lead to blindness; they are the ones who must try and break the news to their son or daughter. For most parents this news comes like a bolt from the blue and they are totally unprepared as they go into the consulting room for this bad news.
It does not take much stretch of the imagination to comprehend what this news must mean to the mother and father of the deaf child. These parents suffer much in the cause of their child's deafness both at the point when deafness is confirmed and throughout childhood as they struggle with choices over language forms, lip-reading, hearing aids and all the paraphernalia that a major hearing loss involves. As their youngster reaches a point of maturity and starts to achieve, parents begin to relax and watch with satisfaction as their son or daughter moves on to greater independence. Invariably, it is at this point when all the efforts of the parents seem to be paying off that they learn that he has an incurable eye diseases.
How the family handles the news, their ability to understand the implications of RP, how they perceive blindness, how they handle their own grief, how they convey the news to their child, all these factors play an important part in shaping the attitude the young person with Usher syndrome will have to his visual impairment.
Suggestions on ways in which Deaf Children and Young Adults can be helped after diagnosis of Retinitis Pigmentosa.
When considering how to help the newly diagnosed student it is very important, firstly, to find out if his parents have told him about his RP condition and, secondly, what he himself knows and understands about his vision. Armed with this information the appropriate action can be taken.
At School and College
1. It is important to understand firstly that Usher syndrome has a combined effect on hearing and vision. If a child has a sensory hearing loss any decrease in visual function has a more severe effect on his ability to respond to sensory stimuli.
2. Learn about the condition of RP, and the particular way in which it is affecting him. Observe how he reads, moves about and copes with his surroundings.
3. Talk to your student sympathetically and ask him what he thinks he needs to help him in his daily work at school/college/work place. Make a note of what he suggests.
4. Ask about light - does he need to sit near a window with the light behind him - does he need extra light on his desk? Find out if he is bothered by bright lights. Headaches and eye watering are common; such activities as cricket or tennis on a bright day can disturb the vision of a child with RP.
5. If the student is at school, call on the services of an Advisory Teacher for the visually impaired who could advise about glare, light and aids.
6. Think about improvements which can be made to poorly lit areas in the school/college/work place: better lighting could improve conditions for others too.
7. Emphasise the sports and activities in which he can excel - those which do not depend for success on side vision or exposure to bright lights, e.g. swimming or pottery. It is easy for the deaf person with RP to withdraw from occasions and activities which show up his eye problems, to avoid this tendency he needs to achieve and be successful in some other spheres.
8. Predictions about how long sight will last should be avoided. No one knows accurately when or if RP will cause the individual to go blind and forecasts of five or ten years can cause undue anguish and inappropriate planning.
9. Help the student and family to think realistically about the future when considering a job or training. Driving jobs are not advised.
10. Ascertain what the student wants to do and what he is good at. Look at the job first and the student's aptitude to do it rather than the sensory impairments. Avoid the phrase deafblind when discussing the student's application for a job. To the employer, this term means that he is being asked to employ someone who cannot see or hear anything.
11. Given that the student wants to do the job, at that point look at what aids and adaptations can be utilised to keep him working successfully.
12. Be prepared to listen. Be open to the emotional needs of the young person. He may not be able to express all he is feeling at home because his parents are themselves too upset and sensitive. You may be the one calm and caring person in whom he can confide his fears. Be honest about the situation but avoid predictions about the future.
13. In these early stages reference to blindness and all things associated with this concept such as Braille, white canes and guide dogs can be alarming. Be careful before introducing these subjects.
14. Avoid discussing the student and his problems over the "top of his head". He needs to trust you and not feel that people know more than he does about his situation.
15. At some point it will be necessary to explain to fellow students how RP affects vision. Rather than dwelling on thoughts of blindness, give them practical hints on how best to communicate with a person with restricted field vision e.g. standing with light onto the face: keeping in the line of vision and indicating when conversation is finished.
16. The person with Usher syndrome may have special needs which require outside advice from a social worker with deaf people or a Disablement Employment Advisor with the local PAC team. The employer, the personnel officer and employees need to understand how their deaf colleague communicates and the best lighting conditions for him to see speech, lip-reading, signing and gesture. Very simple guidelines which are easy for fellow workers to absorb can be given, such as: always stand with the light on your face, speak more slowly without exaggerating, let the deaf colleague determine the best distance for conversation.
17. When talking to employers, avoid the term deafblind, to most people this means unable to see or hear anything. With Usher syndrome this is rarely the case, particularly in the young adult. What needs to be asked is can the employee, given the restricted hearing and vision he has, still do the job?
18. Some tact may be needed before introducing agencies for the blind
into the situation. A person who has Usher syndrome is unlikely to regard
himself as blind and one runs the risk of outright rejection of all things
to do with vision if the client feels that he is being pushed into the
world of the blind too hastily.
*For grammatical convenience, he/his/him has been used throughout.
Mary Guest, Head of Usher Service
11-13 Clifton Terrace
London N4 3SR
Telephone: 0171-272 7774
Fax: 0171-272 3862