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Rubella Handicapped Children: An Everlasting Problem?
A Lecture to the European Federation Teachers of the Deaf in Essen West Germany.
Reprint permission, courtesy of Dr. Jan van Dijk, 2001
INSTITUUT VOOR DOVEN
Essen (West Germany) 19.09.1985
Almost 20 years ago three American Scientists Meyer, Parkman and Panos announced the development of a vaccine against rubella, which afforded immunity for life. This was 25 years after the Australian ophthalmologist sir Norman Gregg had discovered the relationship between rubella infection of the mother during pregnancy and damages to the foetus.
For some time there was a discussion whether the vaccine would indeed protect women throughout life. Generally it can be said, that in case where the vaccination has been properly carried out, it can be expected that the woman will not produce a handicapped baby because of rubella-infection. In many countries health-authorities have launched campaigns to vaccinate girls in the beginning of puberty. Gradually it became clear however that this procedure would have only a very slow effect on decreasing the number of rubella handicapped children. It is computed for a country as the Netherlands with a natural immunity of 80-90% by the age of 21 that it will take until the year 2000 before all women of childbearing age are protected. In a recent study of Dutch health authorities it is stated that with the procedure of vaccinating 11-12 year old girls only, every year 40-100 rubella children will be born in this country.
The procedure which is accepted in the United States and for instance Sweden, where all children at the age of 2-4 years are vaccinated leads to better results. By this procedure where all boys and girls are protected against rubella, the disease is completely eradicated.
Figures from the United States show indeed a considerable decrease in the number of rubella infected babies.
In countries where this procedure is not possible, active campaigns are carried out to stimulate all women of childbearing age to see the doctor and ask him information about the level of antibodies. This can be established by a small test. Very often the woman relies on the information given to her that she had been exposed to rubella as a child. Since it is sometimes difficult to distinguish rubella from other diseases, this type of information is not always reliable.
In countries such as the United Kingdom, where there is an active immunisation programme and in the U.S., one sees clearly the decreasing number of rubella children. Hopefully many countries in Europe are going to follow a similar procedure. My prediction is, that it still will take quite some time, perhaps until the turn of the century, until the rubella problem will be solved. According to virologists the disease will not be eradicated completely, such as is the case with polio-melitus, because it seems that some women have a defect in their immunisation system, therefore no anti-bodies are built up after vaccination. It has been reported that two of these unfortunate ladies already have given birth to a rubella baby (Forrest and Menser).
Despite this hopeful outlook that the rubellaproblem finally will be solved, there are still many many victims of this disease in centers for deaf children to be educated.
It is not my purpose in this paper to spell out all the problems of rubella deaf children in detail, which might make you think that all rubella victims are in a poor condition physically and mentally. The opposite is true. Despite the enormous influence the virus has on the developing embryo, many of the children who had been infected turn out to be fine men and women, who lead a full productive life. At this moment many deaf people who are the victims of maternal rubella of the epidemy of 1964-1965 are studying at the colleges for the deaf in the United States and it seems that these students do not differ significantly from other deaf persons. We should not overlook however, that this is a rather selected group, and that in many instances rubella handicapped deaf people do show in their development some intellectual, emotional or scholastic delay.
So far we do not have a good analysis of the typical (if there are!) problems of rubella deaf people. Although some studies have been carried out, which have outlined the problems which might be associated with this early infection, none of the studies to my knowledge can be called representative for the total population of rubella handicapped. I have even the impression that it is doubtful whether it is correct to compare rubella people from one country with another. It seems for instance, that deaf children whose mother had rubella in 1964-1965 on Iceland, were in general less handicapped than their peers who were born in the same period in the United States. Why this is so, is still very unclear (literature).
The data I will present to you in this paper on rubella hearing impaired children, I collected 9 years ago in Australia. This country had suffered from an outbrake of rubella in 1969-1970 and than had left many handicapped children. The mean age was 6 when I studied this group of children (N=81). Since it was very hard to collect all the data I had to return to that continent in 1980. The results of my study were published in 1982. I will report here the most important findings. It is my goal to make you aware how deeply the organism can be involved because of such an attack of a virus and how carefully these youngsters should be followed in their development.
§1. Embryological and Embryopathic Data
Studying this figure it becomes rather clear why the classical triad of deafness, heartproblems and eyeproblems are often present at the same.time in rubella children. Embryologically speaking these organs develop somewhat at the same time. It is clear that the first 12 weeks of pregnancy are the most dangerous ones, although this should not be stated too dogmatically. In my sample of rubella hearing impaired children it is true that in most cases in which the time of infection could be detected and who were deaf and blind, infection had taken place within the first 3 months, but that in the group of children who are "only" deaf, 30% had been infected after the first trimester of pregnancy.
Figure 3 shows too, that children who have cataract, that means that their eyelens is involved, were all infected in the first 3 months of pregnancy. This is quite understandable, because after this period physiologically the lens is protected against further attacks of the virus, which is not so much the case with the hearing organ. It can be read from the table that I make a clear distinction between children who are hearing impaired because of maternal rubella, who have at the same time cataracts at both eyes, and the ones who have clear media. The children with cataract are infected significantly earlier in pregnancy than the ones without cataract. Both groups differ in many ways: the birthweight is significantly lower in cataract children than in non cataract rubella children, so is their headcircumference.
I have pursued a number of behavioural variables as well. Here again the hearing impaired children with cataract showed significantly more abnormal behaviour patterns, such as stereotyped behaviour and autism, than the non-cataract children. We are used to refer to the "rubella cataract children" as deaf-blind. It is true that these children suffer from a great visual loss despite the fact that early operation can to some extense restore their vision, so that with glasses or contact lenses they can move freely about, but their visual acuity is often very limited (0.2-0.3) and there are often many more ocular abnormalities, which really restrict their visual functioning. Many of these youngsters have severe nystagmus and the eyes are often too small for their age. Sometimes other breaking media such as the cornea are clouded, the retina often shows an abnormal picture and last but not least many youngsters suffer from too high eyepressure, which can really lead to total blindness later in the course of development. In most countries of Europe these cataract children are placed in so-called deaf-blind departments, where people try to stimulate the development of these very handicapped children, who are physically, emotionally and very often also intellectually very damaged. It must be said here, that the educators of these children in Europe and all over the world work very closely together, have their own magazine and at regular times their world conferences. People visit eachother often and I think the deaf-blind educators are an example how people can work together internationally and profit from the mutual experiences. Through this fruitful cooperation there is a lively exchange of literature, sometimes it is of an extremely practical value. Although further comparisons between the deaf child with and without visual involvement shed a very interesting light on the different aspects of development, I leave out in my further discussion the hearing impaired child with cataract and I limit myself to the hearing-impaired-"only" rubella child. Before giving you further information I would like to stress the fact that even in a rubella hearing impaired child with clear lenses the eyes should be checked at regular times, especially the eyepressure (glaucome). Many of the "deaf-only" rubella children, despite normal vision, have a so-called pepper and salt retina. So far there is no indication that the strange structure of the retina, sometimes called Rubella Retinitis Pigmentosa, has any effect on vision, but there are cases reported, that this symptom is associated with formation of new vessels in the eye, so-called neovascularization (Deutman and Grizzard 1978). I would like to plead here for a very careful, regular check up of the child who is deaf because of rubella.
§ 2. The hearing of rubella children
It has been claimed (Ziring 1974) that hearing impairment may be the only defect in cases of maternal rubella after the eight week of pregnancy. The mechanism which causes hearing impairment in rubella is rather complicated. Desmond and his co-workers in 1970 report middle-ear damage in rubella victims as well as central neuro damage and destruction of the cochleacells. In some cases the hearingloss is conductive in origin, but later in the development there is progression because of a continuing destruction of cochlea tissue. In my sample of rubella subjects about 1/3 of the children's hearingloss progressed during the years. It is also reported in connection with rubella (Ames 1970), that some rubella children appear to be deaf, but in some instances respond to sound. Ames introduced for this phenomenon the concept of "auditory imperception". In such cases there is a mild normal response to pure tone, but children failed to develop language and speech. As far as the audiometric status of rubella children is concerned, I was able to compare the very good John Hopkins study on rubella children with my own findings. I had to come to the conclusion that when a child's hearing is impaired because of congenital rubella there is a great chance that the impairment will be rather severe or profound. The audiometric information gives us only a general idea about the audiological status of the rubella group. We lack information on issues such as the type of hearing impairment and vestibular functions of these children. Taking the mean hearingloss on Hz 250-500-1000-2000-4000 I could in a rather crude way construct the following audiogram.
The classical bell-shape audiogram shows up here again, which means that the child has the best residual hearing in the lower and higher frequencies and his greatest hearingloss in the speecharea.
Because of the particular problem in rubella of deterioration of hearing and fluctuation of audiograms the fact should be stressed that the regular check up of the child's hearing and adjustment of hearingaids should be carefully carried out. Since an attack of the rubella virus on the developing embryo has such devastating effects, something that interests us all is how this disease influences the child's intelligence and learning potential. In order to assess this, the well known Hiskey Nebraska test for learning aptitude was chosen as the instrument. For those who are not familiar with this test I can give you the following information. The test is standardized on 466 deaf and 380 hearing children. The unit of measurement is age norms. These represent the avarage amount of mental development in a year. Hiskey uses the term learning age to indicate age type score. For the young children of our sample 8 parts of the scales were used; beadstringing, memory for colours, identifying pictures, folding paper, blockbuilding, completion of drawings, to remember series of pictures and identification of pictures. Although this test needs a little bit of "modernisation" for deaf children it is a rather attractive test. Although it does not give an I.Q., Hiskey states that his test can be used with confidence as a measurement of intelligence (Hiskey 1966, page 7). We find this test so important in predicting deaf children's learning potential, because the test contains so many different types of memory tasks, which play such an important role in learning.
Our sample of testable children was 50, on whom we could get reliable scores. The non testable children were the ones with cataracts. In order to make our results of a more general value, we compared our study again with the ones from Johns Hopkins.
It can be read from the figure, that in our study 2/3 of the rubella children we tested had an average or above average level of intelligence. This number was lower in the Hopkins study (38%). However the percentage ofboth studies of intellectually defected children was similar. It should, however, be kept in mind, that the subjects in our studies were two years older. This is not unimportant to note, because there are some indications that the development of rubella children after a very slow start seems to "speed up". It is interesting to refer to a follow-up study by Koh on 70 post rubella children aged from 2 - 5 years. The children in this study were enrolled in a special programme and were tested every 6 months for 3 years with the Merill Palmer Scale of mental tests. All children were hearing impaired, but apart from this were comparatively free of other physical handicaps. Koh reported that the age of 2 - 5 year the mental age scores of the children were not only higher than the mental age norms of hearing children, but that they accilerated as they grew older, as much as 18 months higher than the norm at age 5.
In another study in which 34 rubella children and 40 not rubella children were compared (Hicks 1970), a mean intelligence of 112 was found for the rubella group and 114 for the non rubella group. Similar findings are reported by Lehman and Simmons, who report on (normal) intellectual ability in the group of rubella hearing impaired children, but their scholastic achievement was significantly below the level of the non rubella group. By scholastic achievement is meant the level of reading, language, reasoning and computation. Also on the performance of lipreading the rubella children were significantly behind.
Similar findings are reported in Vernon's research in which he compared 5 etiological categories of deafness. The conclusion is that "although the rubella subjects' mean I.Q. was similar to premature, meningitis and Rh-factor children, their schoolachievement and emotional status was far below the level reached by these groups". The conclusion is rather obvious. It seems that rubella children's intelligence is not inferior, but that their scholastic achievement and their behaviour is rather a problem. Unfortunately our sample was too young, which made it very hard to collect data concerning their educational achievement, because it is an interesting fact to pursue further what causes the delay in their acquisition of speech and language.
I have tried however, with a great effort, to shed some light on this phenomenon. I will give you my findings and than try later to explain what are the most important underlying factors which cause the delay in the scholastic achievements of these children. I was able to get a reliable specimen of the speech of 36 rubella children. I selected 8 colours and made first sure that the child could name these colours without help. If the trial was positive, the child was requested to name the colours 3 times. The phonological analysis showed that these 8 colour names contain 10 vowels and diphthongs and 19 consonants. Two assessors were involved in the speech analysis of these children (for an exact procedure see my study of rubella children), who were especially interested in the articulation errors. We came to the following categories:
- phoneme omissions
- phoneme additions
- phoneme substitutions
- phoneme order change.
In the theory, developed over the years by dr. van Uden, most problems in articulation of deaf children can be diagnosed as dyspractic in nature (see van Uden 1983). Dyspraxia is defined as a disturbance in the cognitive, senso-motoric Gestalt formation. He considers that in the formation of smooth movement patterns (such as in speech), sensomotoric, rhythmical and perceptual factors come to a unity (a Gestalt). If deaf children have problems in this Gestaltformation, it is very likely that that child will have problems in speechdevelopment as well. Van Uden found a strong relationship between certain motoric tests, such as imitations of armmovements and the imitation of fine fingermovements and the ability to speak and to lipread. With our particular group of children we re-applied some of his work and found in this group of children a correlation between dyspraxia and problems in articulation. This relationship can be presented in the following regression equation:
It can be stated that the problems in developing oral skills in rubella children as far as my sample is representative, is very much dependent on the sensory motoric Gestaltformation. Indeed, when one considers the scores obtained by the sample of rubella children in comparison with the group Van Uden has normed, than it becomes clear, that this group of rubella children falls behind in tests which require fine motor coordination. Since in the formation of the smooth sensomotoric Gestalt rhythm plays such an important role, several parts of Van Uden's rhythmtest were carried out. It showed that in our sample a considerable part of the testable rubella children had problems retaining simple rhythmic patterns. This rhythmtest contains also a section which leads into another problem, which might help us to understand the reported problems in reading and writing of rubella children. In that part the child is tested whether he can "switch" from one modality to another, e.g. the experimentor shows the child a card on which dots are drawn (visual motoric response), also the other way around. In this "cross" modal task more than 1/3 of our sample encountered the greatest difficulties. There is a very strong evidence in hearing children that the skills of sensory motor and cross modal integration as assessed in this rhythmtest are developmental precursors for the reading ability (Satsz et al 1978). Delays in the development of these skills reflect a lack of maturation of the brain. If this holds true for the hearing impaired rubella children, then it should be considered as an important factor in further research on the academic development of this group of children. It is indeed a matter of fact that when we are reading and writing, the brain functions as a whole. It is a continuous flow between the different areas of our brain. It is assumed that from our emotional involvement in certain activities the lower brain areas are "at work" and the right hemisphere. These interconnections make it possible that we become "emotionally" aware of the situation. Our left brain helps to analyse the situation. It is the brainpart between the left and the right part of the brain which we call corpus-calosum that plays a great role in this exchange between the two brainareas.
In very few clinical studies on rubella children there are indications that the bridge beween the two parts of the brain just mentioned was to a certain extent damaged, the so-called hypoplasia of the corpus-calosum. There are other studies done, which indicate that the number of morphologically normal cells in the white brainmatter of rubella children are smaller than in the control sample. It is also reported, that the formation of myelinated fibers was retarded in 30% of this sample of rubella children. It is also reported by Rorke and his coworkers, that there were vascular abnormalities in the brain of rubella children in more than 50% of the cases. Bloodvessels of all sizes were affected including the major basal and cortical cell branches. In this respect he speaks about vascular lesions in the brain. Many of these studies relate to very damaged rubella children. It gives us however an indication that some rubella children might have great problems concerning the integration of one part of the brain with the other, although this will be hard to prove, even with the best instruments available at this time.
In my frame of thinking, dealing with this kind of children, I adhere the theory of the so-called "desintegrated brain". The children function sometimes very good with one modality, such as the visual modality. And although I don't have statistical evidence, in my clinical practice I see high functioning rubella children, being completely involved in visual tasks. They are able to make very detailed drawings, are very sensitive to colours, are sometimes very good in copying words, but when it comes to attaching meanings to this words (this means relating one part of the brain with the other) than they run into the greatest difficulties. By the same token sometimes the child imitates a spoken word rather well, but when asked to write it down, runs into problems. If one agrees with this explanation that this has great consequences for the education of the children. If it is true that the brain cannot cope with "too much information at the same time", the way of teaching these children should be choosen in which the child (by nature) is the best. Since sequential information, such as signing, fingerspelling and speech require a total full activity of the brain, this might not be the most favourite way of approaching these children. I found from my years of experience of guiding and helping these children that the visual mode of using pictures, written sentences, is the most fruitful way of educating these children. It is very important to say that I am not advocating the socalled "matching method" where the child has to match words with pictures, but that words and sentences should be taken from the conversation with the child, because conversation requires emotional involvement. The latter should be stressed because it is a fact we very often encounter that these children pick up only language which lies within their field of interest. And this field of interest might be of a very particular sort. I will ellaborate on this more, because it leads us to the problem of the emotional development of rubella children. In order to understand this, and I am not saying I do, but again I have developed some sort of theory over the years, it should not be overlooked how the beginning of life of these children was. Many of them never had one month of normal live. The influence of the virus on the developing human being is enormous. The virus has prevented the normal growth of the foetus, this is the reason for instance why so many rubella children have such a low birthweight. In my study more than 40% had a birthweight under 2500 grams.
Very often in these children feeding problems are reported by the mother. Especially swallowingreflex develops very slowly. Some of the children in their first period after birth are hypotonic, that means that the children are very hard to hold by the mother. Because of the early infection in many instances day and night rhythm are disturbed: the child sleeps during the day and is then awake during the night. Very often the first years of life of a rubella child and this in relationship with his mother can be described as very poor. More and more our attention is brought to the very important aspects of good bonding between mother and child. I am not saying here that generally speaking the bond between a rubella child and his mother is not of a high quality. As far as I know this is not researched, but knowing in what circumstances many of the children with their mother are raised, I dare say that this relationship is very often endangered by the difficult first years of life. In the early period of life one already sees very often that the child concentrates himself more on one aspect of the world in which he lives and not so much in different objects and situations. Parents report that the child is very early fascinated by the wheels of the car, or the opening or closing of doors, or very interested in comic books etc. When these objects are taken away from the child, he often tantrums. The children resist changes. Anyone who is familiar with the syndrom of early infantile autism will recognise this symptom as "preservation of sameness". It is a false assumption to relate congenital rubella too easily with autism, but it cannot be denied that quite a group of rubella children shows socalled autistic symptomatology. I do not consider all of these symptoms as autistic traits, but they can be considered as a way for the organism to compensate for living in such an impoverished world; physically and emotionally the organism is understimulated. When this is the case, it seeks for compensation. Some of the children might do this by provoking their environment, for instance by breaking "on purpose" things in order to get response from their parents. Others go crazy over looking into comic books and are fascinated by drawings of people who are killed etc. Again others always talk about sickness and hospitals and blood. Some tell you the most extraordinary dreams and fantasies they have. We relate this to the deprivation of adequate stimuli in the early years. There must be also a neurological component involved (see above). We have the impression that the level of excitement, the socalled "arousal" is very high, that is to say that the child is only "happy" when he is in a state of excitement. It seems that the childs brain needs to be bombarded by stimuli. When this "bombardment from within" does not come to an end, the child, especially the older one, might act out for instance by attacking a person. That not always the environment in which the child lives is responsible for his behaviour, but that neurological factors play a role as well, might be concluded from our experience. In some instances of these very highly aroused rubella children we treated them with very small doses of medication and immediately it could be reported that the child calmed down. When we changed the medication by a placebo the "aroused behaviour" came back. I would like to warn you again that it is not my purpose to generalize, but there are quite a few of the rubella children whose behaviour is as I just described. As far as the educational approach of these children is concerned, I think the same as said with regard to the language teaching should be mentioned here again. The moment the child is put into too complex situations, without notifying it before, his organism, his brains are unable to cope with the situation. On the other hand, when we prepare the events very carefully with the child, we draw and write down what we call "daily routine", what things are going to happen; who will be present and who will hot be present; when the father or the mother will come to pick him up. And all these things should be drawn and written down very quietly together with the child. This will help him to plan his day better and prevents him from worrying about things, because these children worry. When they are getting older they are worried about their future, they worry about their physical wellbeing. Therefore a very solid education is required, in which the educator suggests certain solutions and does not leave the child in doubt. Do not make these children insecure, because they are unable to cope with it. It is therefore very important that a counsellor is given to the older rubella child with the problems I have just described, who goes over and over the problems again and again.
Very briefly I have tried to present to you 20 years of work and research with a very particular and fascinating group of children. They have all sorts of problems, but also all sorts of possibilities. Only when we study further and stay in report with the child, we may say as Van Uden in his introduction to my book on rubella children: Rubella victims might become rubella victors!!
Dr. J. van Dijk