Adult Patients with Minimal Language Skills and/or Multiple Impairments
Patients with either minimal language skills and/or multiple impairments require an interpreter who has experience in this rare type of medical interpretation. These patients also require that the ophthalmologist has more than the usual experience in language development, communication techniques and clinical neuropsychology in order to be able to recognize or at least suspect that the problem is:
a technical problem related to the dual sensory impairment (especially visual impairment)
a combination of the sensory impairment and minimal language skills
a combination of dual sensory impairment and neurological problems affecting either receptive or expressive communication but with normal language comprehension
a combination of dual sensory impairment and neurological problems affecting communication with limited language (either sign language or English)
a combination of the sensory impairment and mental retardation.
In the examination of these patients we are at the mercy of the interpreter even more so than usual. We have to learn to appreciate their diagnostic skills in assessing communication, but we must remain aware that their diagnosis in the early stages of an examination is as uncertain as is our own. Difficulties are typically present when we see a new patient and do not have information about the level of communication and language skills, or when the information given in the referral is incorrect.
If a deafblind patient has had a recent change in vision, it is often the case that the previously used visual sign language is no longer effective. If the patient is inexperienced in tactile sign language, he may appear to see in a large enough visual area and, when asked whether he sees comfortably, may answer "yes". However, he may miss so much of the visual information that he misunderstands some usual signs and/or finger spelled words. Even an experienced deafblind interpreter may misjudge the patient's comprehension in such a situation and thus inform you that the communication should be kept at a rather basic level. During the examination it often becomes apparent that the patient is unlikely to see what is signed and that another mode of communication has to be chosen.
If the patient is accustomed to English, writing large enough letters with a felt-tip pen, using a CCTV, a Viewscan or other magnifying devices can often get the most important messages across. A patient who is primarily an ASL-user can get some information through two-handed tactile signing even without previous experience. In both cases it is wise to limit the examination to tests that do not require complicated communication. After the first examination, the patient and the interpreter can work with the usual sample questions as their homework and come back after a few days or weeks for further assessment.
A deafblind patient with known minimal language skills is a challenge if something important and complicated has to be discussed with him, for example symptomless angle closure glaucoma requiring immediate treatment in the hospital. Since it may take half an hour to find out how to describe the situation to the patient, I usually explain it as if I was speaking to a third or fourth grade student. I then ask the interpreter to tell it back to me in English as close as possible to the expressions that will be used in sign language. After clarifying the words that I would stress differently, the patient and the interpreter are left to discuss that part of the information while I am doing something else. When I come back, I repeat the sentences and they are signed again to the patient. Depending on whether the interpreter and I think that the patient has understood the information, we either rephrase the information once more or ask for a decision about what to do. Sometimes the patient has to make the decision on rather inadequate information, that is, actually knowing only that if the treatment is not instituted immediately blindness may ensue.
During routine examinations of deafblind patients with minimal language skills (MLS) the counsellor and the interpreter have usually discussed the worries of the patient before the examination, and we choose only one new important problem to discuss during each appointment.
Whenever we are dealing with an additional neurological impairment, we have to work together with an experienced clinical neuropsychologist who preferably has had experience in deafness, if the patient is prelingually deaf. Otherwise, some tests may be chosen that are misleading when examining a visually impaired, prelingually deaf person. Likewise, the effect of restricted vision on some results has to be clearly understood. In these cases it is mandatory that we use the same two or three skilled interpreters during several months or years in order to learn the most reliable ways to transfer information. Because of the limited number of interpreters in this field, scheduling is sometimes a problem. In order to get the only interpreter who understands a deafblind person we may need to arrange the visit on a day that fits her/his schedule even if it is not the best day for us.
The patient with dual sensory impairment, neurological impairment and minimal language skills is often diagnosed as being mentally retarded. These individuals, however, are able to learn complicated tasks and develop modifications to techniques taught to them. However, they have specific difficulty in comprehension on top of the difficulties in receptive or expressive language. The amount of information that can be conveyed becomes quite limited, and therefore we have to use techniques that we usually use in the examination of prelingual children. These tests have to be used with the understanding that we are examining a grown-up person with an emotional status and attention span different from that of an infant or child.
Mentally retarded, deafblind patients without additional handicaps most often cause no major problem in the selection of tests or test situations. They are examined at the level at which they are able to perform. The rate of success in examination is as unpredictable as when we examine young children. We have to be prepared to quickly shift to another test if a certain test does not interest the patient and complete the examination by using indirect information such as VEP-grating acuity values and photorefraction to support or contradict our clinical impression. We must work as a team with teachers, interpreters, caregivers and paediatricians to learn more about the quality of the patient's visual functioning in different tasks.
Patients who have forgotten communication
These patients have typically gone to a deaf school in their childhood and then have lost contact with other deaf persons, often living in an environment with minimal communication, either in their own family or in an institution. If their sensory impairment becomes worse, as in Usher Syndrome, they may become misdiagnosed as catatonic schizophrenics because they do not react to usual communication and tend to sit in the same place for days. Some patients stay in bed and some have even been fed in bed for years before somebody happened to suspect that sensory impairment may be a large component of their behavior.
You will have these very difficult examinations in the beginning of increased activity in deafblindness, since that is usually the time when deafblind individuals are found in institutions and brought to your office. These otherwise healthy individuals have often been for years without any communication and may have forgotten sign language. They may even have forgotten what communication means, so that when some contact has been developed it takes months before they remember that questions are not meant to be repeated back, but answered.
During the first examination there is either minimal or no communication and no information about how much vision might be present. We have to start with simple observations of what the patient seems to notice and whether changes in light levels seem to affect the patient's behavior.
When I read through old records, I ask the accompanying person the usual questions "What do we know about the school and the family of the patient; what was the father's occupation, mother's . . . " and these sentences are signed to the patient with rather small, slow and clear signs even if we have no idea at which distance the interpreter should be. Sometimes one of these common signs awakens the patient to sign something he remembers from the time when signs were first used and we have solved our first piece of the puzzle. When we know that there is some vision, we can start making observation schemes to determine the size of the picture in the sign dictionary the person prefers, which techniques are used to locate in a new environment, etc. We continue the examination at intervals of a few months until we get enough information about the patient's vision.
When you have more experience in dual sensory impairment with or without mental retardation, it becomes rather easy to at least suspect that a certain low-functioning person is not functioning at that abnormal level because of mental retardation or psychosis, but because of a lack of information. It is important that the ophthalmologist helps make this diagnosis because some of these individuals can learn to function rather independently even after 15 to 20 years of deprivation.
Ophthalmologists should be especially cautious so as not to misjudge the patient's cognitive development and intelligence. Our schooling in communication is so inadequate that unintentionally we are apt to make gross errors. I dare say this because I can read my own reports written in early 1980s when I knew how to assess impaired vision, but did not know how to use an interpreter effectively. Very careful planning and consultations with other professionals, including audiologists, special educators, and the interpreter are often needed before the communication with this group of patients is adequate.
The often written statement "Patient does not co-operate" should disappear from the patients' charts. Co-operation is a two-way street. In most cases, the patient tries his best but cannot reach the communication level of the doctor. Thus it is often not the patient who does not co-operate, but the doctor.
It is my personal opinion that it is not necessary to be able to use sign language in more than a very rudimentary form. It is necessary, however, to know enough about the structure of sign language and the deaf culture in order to be comfortable in this cross-cultural situation.
There are several sign languages, and patients may use combinations of different languages. Thus, it is difficult to learn to communicate directly with all patients if one does not have continuous contact with deaf culture for other than professional reasons. Limited sign language skills may be dangerous if the doctor starts to believe that he or she can take care of the communication without an interpreter. On the other hand, even limited knowledge of sign language is helpful. Patients usually experience any effort to communicate directly with them as very positive.
Children become much more relaxed when they realize that the eye doctor knows only a few signs and signs them in a funny fashion. They tend to be less frightened by a doctor who is so apparently inferior in communication skills, and they are usually very helpful and good at guessing even ridiculously signed information.
Interpreters have to learn some ophthalmology and optics in order to work in the eye doctors' offices. It is only fair that the medical professional learns as much about sign languages and deaf culture.
Should the Eye Doctor know Sign Language?
