How to inform about visual impairment
Information regarding any type of visual impairment is often difficult to discuss with a person who has, or is going to have, that impairment, or with the family of a visually impaired child. The main difficulty is usually related to the scanty knowledge we have about that person's history and values, his family background and their personal concept of disability. Information carelessly delivered may function like a bomb. On the other hand, if given with support, appropriate information may shorten the period of primary shock and help the person and family deal with the problem.
The most commonly expressed criticism about the medical professional relates to the manner in which a diagnosis is communicated. It might be possible to negate this criticism by saying that the traumatic experience itself causes the patient or family to have negative feelings about the person who had to relate the unpleasant news. In most cases, however, this is not true. While some of us can handle this situation without overly upsetting the families, most of us don't. A story that we hear all too often is that the diagnosis was told by a resident saying "You (or your child) have such and such a disease which causes a serious decrease in vision. Unfortunately, nothing can be done." After delivering the information, the doctor quickly disappears before the patient or parents have recovered enough to ask any questions. There are much worse scenarios, of course.
The expression "There is nothing to be done" should disappear from the vocabulary of ophthalmologists altogether. We do not deal with the situation when there is nothing to be done, that is, when the EEG recording shows a straight line. Whenever we encounter a visual rehabilitation problem, there is plenty to be done immediately and even more to be planned for the future.
A basic requirement is that this extremely important communication in the life of the patient should not be dealt with by anyone who is inexperienced in low vision services and the problems of the visually impaired. We do not allow residents to be in charge of complicated operations before they have sufficient experience with senior staff members operating. Similarly, a resident should not be responsible for communicating the news about visual impairment because he does not understand the full implications of the news. Indeed, not all staff members can handle this crucial initial communication properly. A clinician may be experienced in many fields of ophthalmology without having adequate experience in rehabilitation.
Some colleagues seem to bear feelings of guilt, much like some parents, which prevents them from having a positive approach. Any professional who does not feel comfortable about his or her contact with visually impaired patients, and in this particular case patients with dual sensory impairment, should first seek help for his or her own rehabilitation. We must learn to regard even the dual impairment as an inconvenience, a feature of the life of a particular person, and nothing more than that. We also have to understand that there is great variation in the intellectual abilities of these individuals, resulting in many different careers and lifestyles. Last, but not least, we have to learn to understand and appreciate the small steps in the very slow development of many multi-handicapped, deafblind patients, and treat these patients in the same way as we treat our temporarily able-bodied patients.
The first rule of the medical profession is "non nocere", not to harm. Since we know that the information related to visual impairment is always traumatic, we must plan that situation carefully and cushion the bad news with all the support services that are available.
The diagnosis of visual impairment of a deaf or hard-of-hearing person may happen at different ages. Typically, we have to break the news of visual impairment
Most often the diagnosis is not quite certain after the first examination. Even if it was obvious, it is advisable to describe the situation rather briefly and arrange an additional examination as soon as possible when the person responsible for the (re)habilitation of the patient can meet with the patient or the whole family before the examination and be with the patient during the discussion so that he or she knows where to start when the doctor has finished. If we are going to tell the diagnosis to a deaf patient, an experienced interpreter is necessary.
The amount of information given during any appointment should be kept within the limits of coping. It is much better to answer questions than to give lectures that the patients and their families are seldom accustomed to. We should not plan to tell the "whole story" of the disease process with all the possible variations before the patient or the parents have some experience of living with the new facts.
Parents of deafblind infants
A great majority of deafblind infants have several other health problems. The child usually has been examined by paediatricians, audiologists, paediatric neurologists, and possibly several other professionals before coming to the ophthalmologist. When we plan the first discussion on visual impairment, it is a great help if we can include the developmental paediatrician who is in charge of the child's habilitation in the discussion. When professionals share this situation, they support each other and support the family as well by conveying the message that not even professional people are able to cope with this situation alone, but need to get help and support from others.
The parents usually remember the general atmosphere of the discussion, but not many of the details told to them. Therefore it is important that we knowingly communicate with the child with an appreciation of any functions the child may have. The developmental neurologist is usually much better than the ophthalmologist at demonstrating to the parents the strong sides of the infant. The ophthalmologist may be very helpful in explaining why some actions or reactions are not present because the visual information is not available to the infant.
In those cases where Usher Syndrome is diagnosed in infancy the parents need reassuring that the child "will do well", that most children with Usher Syndrome are above average in intelligence, very pleasant to work with, and that visual symptoms will not disturb them for several years except, perhaps, at dusk. If a child has very pronounced balance problems the parents may think that this is a sign of poor vision. It is important to clarify for the parents that balance will improve noticeably before the child is in kindergarten, and that it will not get worse later. It is not the ophthalmologist's work to inform about the contents of training programs for improved balance, but it is a good idea that you know they exist. With the diagnosis of retinitis pigmentosa, it seems to lessen the impact if the ophthalmologist stresses the importance of visual communication and creativity in finding ways to express feelings.
Parents of severely multi-handicapped infants need very special treatment. Due to the presence of multiple health problems, the child and the parents have to see so many specialists that the number of strangers getting involved with the child actually becomes an additional impairment. To reduce the impact of this problem we can improve the situation by scheduling appointments in collaboration with other professionals and choose someone who will be primarily in charge of communication with the family; usually the paediatrician.
There is a massive amount of new knowledge that the parents have to learn during the subsequent months and years, but during the first few weeks they need and can cope with only rather basic information. The booklet "Vision in Children - Normal and Abnormal" LINKKI MYÖHEMMIN? (Hyvärinen 1988) was written for parents and other caregivers of visually impaired children to be part of this early information. Dual sensory impairment causes very specific problems which are often highly individualized and thus we have to warn the parents to read all the information with reservation and a critical mind. Many parents are likely to read medical books and to be very confused because of the conflicting information in different texts. When the knowledge of the parents increases it is easier to discuss the specific features of their child's disorder and the disabilities and abilities related to the condition. The common thread throughout all of these discussions should be to emphasize what strengths the child has, not what he has lost.
It cannot be stressed strongly enough that we should avoid the expression "blind" whenever there is even a slight possibility that the child will have some visual functions. We see so often that children behave as if they were blind during the first few months of their life, but learn to use their vision during the end of the first year, resulting in quite useful vision at the age of two to three years. Sometimes it is still possible to have a remarkable improvement in visual functions after three years of life.
The parents should be encouraged to observe their infant during different activities and at different luminance levels for any new signs of the beginning of visual functioning. The observations of the special educator during home visits are, of course, very important and should be reported regularly. As we encourage stimulation of vision, we must not overemphasize the value of visual functioning alone, since the child must learn the use of other modalities as well.
By involving the special educator in the first discussion of the visual problem we make the concept of such special services more acceptable to the parents. If the parents are told about the visual impairment and asked to contact the services themselves, it may take weeks or months before they have the courage to meet with one more professional who represents the loss that they fear the most.
Parents of hearing impaired children 4-9 years of age
In this age group the diagnosis of Usher's Syndrome is the most common one. Although it would be possible to make the diagnosis during the first year of life, the suspicion of abnormal vision is usually formed when the child behaves abnormally at low luminance levels and this leads to investigations at an eye clinic.
In most cases it is impossible to obtain reliable visual fields during the first visit, so there is good reason for arranging another appointment a few days later, making sure that the parents will have ample time with the special educator of the habilitation team and with the ophthalmologist.
When we discuss a slowly progressive eye disorder like retinitis pigmentosa, it is important to remember the time scale and not to overemphasize the end stages of the disease. The often-used statement "You are going to be blind" should be carefully avoided because neither the parents nor the child will be able to understand the present situation if they are overwhelmed by the term "blind".
For those who insist that we should tell the whole "truth" at once I want to say that it does not make sense. Every one of our patients is going to die some day but we do not tell a paediatric patient that he is going to die some 50 years from now because the child does not know what 50 years means. To a five to 10 year old, anything beyond 25 years is almost beyond the grave.
If the parents ask a direct question about blindness I always state that no one can answer that question without several years of followup to determine the type and rate of progress of the disorder. In addition, I add that during those years we hope to be able to develop new treatments to affect the disease process itself. I know that some of my colleagues feel that this gives the families "false hope". Since I believe that the vast research in this field will lead to some clinical applications in the near future, it seems correct to let the parents share this belief. Also, when the parents learn about the joint research projects in retinitis pigmentosa they are less likely to start the exhausting and expensive hunt for a cure in less-respected places which claim to have treatment available. It also motivates them to accept the time-consuming tests we have to make for exact diagnosis.
Young deaf children are usually not at all interested in the discussion about their condition, since the vocabulary is usually way above their conceptual level. Usually I tell the child that "You know that you see just a little in dim light (if that is true). You have something in your eyes that is different from your friends' eyes but this does not bother you. We will talk about it more when you come to see me in two years." I then let the child go and play with other personnel, accompanied by the interpreter, while the parents discuss the situation with the habilitation team.
The problems related to "legal blindness" and the confusion caused by the expression "tunnel vision" are discussed in the chapter related to visual fields, and do not need to be dealt with here. I would, however, like to stress the importance of explaining the structure of the visual field in detail to the parents. It is also very important to point out to the parents that visual acuity, contrast sensitivity of central vision, and color vision are all normal and that changes are usually very slow during the next ten years.
Invariably the parents want to know the ophthalmologist's opinion regarding special education. It is actually quite common to be asked this question when a visually impaired infant is seen for the first time. Since the special education of deaf children is complicated by many different approaches, e.g. sign language versus signed English or oral communication, it is wise not to step into this trap at all but to suggest that the highly individualized needs of every child must be assessed by an experienced teacher after which the proper type of school placement can be wisely chosen. If psychological tests are used in this process it is very important to stress to the school authorities that the child's placement must be based on tests suitable for testing a child with dual sensory impairment.
Since the word of the medical profession is still much respected, it may be possible to help the family get the best schooling available. Unfortunately, some states have reclassified deafblind children in the more general category of multi-handicapped children. As a result, deafblind children may not be able to have special teachers who have very specific training in deafblindness. In this situation, teachers who have limited or no background in teaching high-functioning children with Usher Syndrome need very well formulated descriptions of the child's visual functions written in plain English.
Reports to the schools should stress the need for increased awareness of a child's dual sensory impairment among the teachers and other personnel as well as the students. It is quite common even today that schools for the deaf have a negative attitude in matters related to visual impairment and theachers often have had very little training related to the visual impairment of a given child. This child's hearing impairment is usually known to its smallest detail as are the hearing aids but knowledge related to vision is usually limited to the dioptric power of the glasses, if the child has glasses. Unfortunately, this is typical in all countries. For some unexplainable reason, the teachers for the visually impaired learn much about vision but almost nothing about hearing and teachers for the deaf learn a great deal about hearing but almost nothing about vision, the very modality that they use in teaching.
In order to improve the quality of life of deaf, visually impaired children we need continuous awareness programs in the schools for the deaf and in all the schools where these children are integrated. We need to know that these children are harassed, even today, simply because they are different. Despite the many nice words about equal rights, our society is not yet ready to accept its disabled members; it is this disability of our society that makes these children handicapped.
Parents and the visually impaired deaf teenager
The diagnosis at this age is most often Usher Syndrome. The late diagnosis is due to a lack of knowledge regarding visual impairment in the child's environment. Typically, the school for the deaf of a given teenager does not have an early screening program for visual impairment. If the student is mainstreamed, the likelihood of missing the early diagnosis is even greater.
Before the diagnosis of RP can be discussed with the teenager and his parents, we must know how much the child knows about the function of the eye. The curriculum in science varies in different schools and many schools for the deaf have rather limited teaching in biology. Quite often we must start with basic information about the structure of the retina and the visual pathways before we can discuss RP, retinitis pigmentosa. Not only the deaf youngsters who have RP need to know more, but also their peers and the whole deaf community need to have a better understanding of visual problems if we want to improve the status of the deafblind members of that community.
When we discuss visual impairment with a teenager, the news of a progressive loss of vision is about the last thing that a deaf teenager wants to know. The plans for a motorcycle and car become unreal, many dreams about future occupations vanish at once, and often the inexperienced family is of little help. Because the diagnosis is such a blow to the teenager and his family, we should find and diagnose these children when they first enter school, at the latest.
In the cases that we have missed, the news should be presented only after the family has met with the special teacher of the rehabilitation team and preferably has also met with a high-functioning young person with Usher Syndrome. Since these well-adjusted youngsters are not that numerous, this is usually possible only in cities where they get their vocational or graduate schooling. It is worthwhile to develop a working relationship with these schools since the time spent in consultations is paid back when we can get the help of their deafblind students to prove to the teenager and his family that there is hope for the future, despite the diagnosis of RP.
Most offices that serve a large number of RP patients have one afternoon a week for just this group of patients. It is then rather easy to arrange the second visit of the new patient so that he will meet with an older, well-adjusted Usher youngster in the waiting area. Since they will share the same interpreter they will meet in the waiting area and usually will talk to each other. The interpreter who knows the older patient understands that he is supposed to greet the older patient with something such as "What exciting things have you done lately?", thus getting the patients to talk about all those things that the parents are likely to forbid as soon as they know about the visual impairment! After this type of casual exposure it is much easier to suggest to the family that they meet with other families that have gone through similar experiences.
The teenager is likely to deny the visual impairment in school for quite some time. It thus requires considerable diplomacy informing teachers and peers first about vision and visual impairment in general, and later about Usher Syndrome. This is especially important if there are no other children with Usher Syndrome at that school.
Breaking the news to an adult hearing impaired patient
There is a great variation of causes of vision loss in adults. The most common reasons are retinitis pigmentosa, macular degenerations, optic atrophy, hereditary or related to multiple sclerosis, choroiderernia, diabetes and glaucoma. More rare causes are multiple tumors, like bilateral acusticus neurinomas combined with an additional tumor near visual pathways and causing changes in vision. Explosion accidents, severe traffic accidents, etc. may affect both modalities at once.
The only group of patients that are reasonably easy to tell about their visual impairment are the patients with either retinitis pigmentosa or choroideremia. We know that they must have had the changes for years and they know that they have been able to manage somehow despite the impaired vision. In many cases the patient is relieved to learn that he is not “disabled” in any other way. Deaf persons do not know much about vision in general and seldom anything about hereditary degeneration of the retina. They may have thought that all deaf persons, or all people, see like they do and that there is something wrong in their ability to observe the surroundings as carefully as other people do.
If the patient has had the hearing impairment for a long time, his work may be highly visual and he is dependent on vision in communication. The impending visual impairment will affect at least communication but it may also mean major changes in the workplace. Therefore any significant changes in vision should be discussed early in the course of the disease with a hearing impaired patient. There are now many technical solutions in most types of work to compensate for loss of vision, but the necessary skills require schooling. Since the departments of vocational rehabilitation are slow in their decisions on re-education and it also takes time to get devices, the process of rehabilitation should be started before the situation at work has become disabling.
The different areas of rehabilitation psychological, vocational, social should be remembered. Although the professional help is important, contact with some well-adjusted deaf, visually impaired persons is usually appreciated even more. There are numerous minor everyday problems that can be solved in very many ways. Nobody knows them better than persons who have experienced loss of vision themselves.
A very common reaction among laymen and also among many professionals is that a person who is already hearing impaired and now is developing visual impairment should "have the right to retire". For the persons with a dual sensory impairment, work may be even more important than it is to we "able-bodied" individuals. The decision to work or not to work must be carefully weighed. If the person retires, we should remember to keep an eye on the need of mental health services during this often emotionally stressful time and on the possibility of the person becoming severely isolated.
The hearing impaired elderly person who is losing vision
In the older population, dual sensory impairment is common and its effects on different functions of the individual vary even more than in the younger groups. People with visual impairment may during their lives develop hearing loss, the prelingually deaf may lose vision, but the great majority of new deafblindness after retirement age occurs in persons with no previous experience of sensory impairment. Since these persons are so old, there is a tendency not to even consider rehabilitation or more complicated aids.
Because of limited communication, the nature of the disorder is often explained with only a few words. These older patients deserve to get exactly the same explanations as our younger patients and they may need to get the information several times, with assurance that low vision rehabilitation may help them to be independent.
Sometimes the effect of visual impairment on the receptive communication of a deaf person is not recognized, and the old deaf person is thought to be senile when he does not understand signs as well as before.
It is important to actively start teaching independent living skills, as well as communication and recreation skills to old patients since they often have amazing potential for functioning. If one has not had contact with some of the fiercely independent senior citizens, it is difficult to imagine university students in their 80s, very hard of hearing, with less than 20/200 visual acuity, living independently in the countryside, commuting daily, and preparing for their Ph.D.s.
Nowadays, "elderly" people are accustomed to an active life and may want to continue it despite the sensory impairments. Learning new techniques may take more time than for younger individuals, but basically the same skills should be taught to both groups. On the other hand, we must know that the combined sensory impairment may often lead to changes in such areas as eating habits and daily walks and become a cause of major health problems. Rather simple teaching on how to use impaired vision and hearing in ADL tasks, how to find services, etc., may improve the quality of life of these patients. It is not an ophthalmologist's task to teach these things but it is their task to initiate the rehabilitation process, and reactivate it if things are not developing as they should.