Ageing processes and late manifestations in congenitally
deafblind adults in
Main
results from survey conducted by the Information Centre on Congenital Deafblindness
From: http://www.nud.dk/CA3FE0DA-3DA3-4717-B77B-75D40F2B55BA
The Information Centre on Congenital Deafblindness in
All
persons aged 18 and more diagnosed with congenital deafblindness
in
with
other aetiologies. The statistical data is therefore organised in a "rubella group" and a
"non-rubella group". 53 of the 58 informants stated that the aetiology was known. 2/3 of the target group live in
residential settings for congenitally deafblind
persons, the last third live together with person with other impairments.
In
this article some of the main results from the survey are highlighted. When in
the following the term both groups is used, variables applicable to both the
rubella group and the non-rubella group are referred to
Medical and physiological information
In
both group there is a high incidence of persons born small for date (born at term,
but with lower birth weight than expected). The rubella group has more males
than the non-rubella group. In both groups there are many individuals who later
in life experience weight problems. In
the group non-rubella, it is typically a problem of undesired weight loss. In
the persons with congenital rubella syndrome the problem is rather overweight
in adult age According to the data collected the problems of weight gain is of
a scale which calls for further investigation to identify the causes of this.
Congenital hart failure primarily
occurs in the rubella group. With early surgery, the heart defects poses no
problems for the individuals later in life.
If
we look at the motor functions and physical impairments present at birth, these
only occur in the non-rubella group. In both groups there are individuals who
later in life experience physiological deterioration to an extent that they
need assistive devices for mobility functions. In the group non rubella this
deterioration takes place early in life, as the development of physical
impairments in the rubella group only takes place from age
30
and onwards. This may indicate the presence of a late manifestation. In both
groups there is a relatively high incidence (15,5%) of muscular deterioration
and poor balance in adult age. This underpins that congenitally deafblind persons need treatment from physiotherapists and
occupational therapists in their adult age.
Vision and hearing
The
majority of the informants in both groups have a visual impairment which was
diagnosed during the first year of life. Around 2/3 of the population with
congenital deafblindness have a progressive vision
loss (like glaucoma, retinal detachment, decreasing visual field, and removal
of eye), just like we also can rate the generally occurring age related changes
in vision to much the same level in both groups. In both groups individuals have
severe hearing losses or
deafness (the
latter is more frequent). Moderate hearing losses are only identified in the
group non-rubella. Both groups show that the hearing impairments are identified
later than the visual impairments. Individuals in the group non-rubella are
generally diagnosed earlier than individuals
in
the rubella group. This finding may surprise, as the congenital cataract,
possibly in combination with low birth weight and heart failure should cause
suspicion of a concurrent hearing loss caused by a rubella virus infection.
According to our information 50 persons have had a hearing aid at the time they
were diagnosed. Today only 16 individuals still wear their hearing aids. Progressive hearing losses are
identified in both groups. However, the prevalence of progressive hearing
losses may be higher than shown in the data collected, as unlike progressions
in vision losses, the progressions in hearing losses will not immediately be
detected in observations of changes in behaviour. For
this very reason it seems alarming that control examinations are not being
undertaken at regular intervals. With regard to hearing and vision losses the
survey documents that the awareness of the risk of progression in the sensory
impairments is lacking in the environments where congenitally deafblind adults live. The survey also indicates that there
is more awareness of the possibilities of decrease in vision, as ever second
individual regularly is taken for eye examinations, and only one in five gets
the hearing checked.
Hormonal conditions
Problems with metabolism are
a known late manifestation of Down syndrome, and have been mentioned as a late
manifestation of congenital rubella syndrome as well in foreign studies. None
of the informants in the Danish survey has reported any diagnoses with regard
to metabolism, so it may seem not to be a matter of late manifestation.
Nevertheless we must state from the data collected that 10 out of the 20
individuals in the rubella group have overweight and in the same 10 persons
between 2 and 6 symptoms of low metabolism (e.g. tiredness, sadness, increase
in emotional instability, passivity,
less
stamina and weight gain) are reported. Foreign studies demonstrate that
congenital rubella syndrome entails a major risk of developing diabetes. In our survey we
identified a few cases of diabetes (3), and in the rubella group only. Whether
this finding is a statistical coincidence, or it actually expresses a late
manifestation cannot be judged from the present study. Growth retardation has been
mentioned as a specific problem in persons born with rubella syndrome. We have
identified cases of growth retardation in both groups, but the prevalence is
clearly higher in the rubella
group.
Neuropsychological conditions
Epilepsy according to our
information occurs with almost identical frequency in both groups, but the age
of diagnosis is different. In the group non-rubella the epilepsy is diagnosed
at an early age and must be considered a part of the clinical picture. The
reports on individuals having epilepsy in the rubella group confirm the
assumption that epilepsy is a late manifestation likely to appear in the teens.
In our survey we have also asked for information on changes in
neuropsychological phenomena like attention,
concentration and
memory. Here
we speak of distinct differences in the two groups, as decreased ability in
these three functions is remarkably more frequent in the rubella group. The
Danish survey, which comprises all aetiologies
present in our country among persons with congenital deafblindness,
shows that reductions in memory and attention are late manifestations
significant to the diagnosis congenital rubella syndrome. A number of
individuals in the group non-rubella also report increasing problems with
concentration, thus the difference between the two groups is less significant.
Development of behaviour problems In our survey we
have asked for information on changes in behaviour in
11 different areas. In six of the areas there is a dominant score of
individuals with congenital rubella syndrome. Self-stimulating
behaviour, motor agitation and
increased self-abusive behaviour are three types
of behaviour which primarily are identified in the rubella group. This
tendency seems to confirm the hypotheses of a late manifestation with respect
to an increase in these types of behaviour. There is
a significant difference between the two groups. Increased aggressiveness, less tolerance to changes in
routines and increased
level of confusion are three types of behaviour
which have previously been considered as late manifestations of congenital
rubella syndrome. These behaviours are relatively
frequent in both groups, however, with the difference that aggressiveness and
less tolerance to changes in routines are much more significant in the rubella
group, which could indicate a connection with the diagnosis. There is also the
possible explanation that these three types of behaviour
express general deprivation in the congenitally deafblind
persons, or are related to early ageing processes. We know from gerontology
that the behaviours mentioned may be observed also in
the normal ageing process. Increased
restlessness and increased
impulsivity are described as late manifestations of
congenital rubella syndrome in previous foreign studies. Our study cannot
confirm this. According to our data increased restlessness and increased
impulsivity is reported in both groups, but not extensively. These behaviours are also known from gerontology. Less initiative, fall in ability to start activity,
as well as more difficulties in changing fromone activity to another are
also behaviours which previous studies have characterised as late manifestations of congenital rubella
syndrome. However, none of these types of behaviour
seem to be characteristic of any of the two groups in our study. Despite a
small majority of rubella persons showing these behaviours
our data cannot confirm previous assumptions. According to our information
theses variables covariate. As these behaviours are
also described in gerontology, they may be indicators of early ageing
processes. Among people who work with congenitally deafblind
persons it has often been discussed whether these types of problem behaviour could be explained by environmental factors. We
have therefore tried to identify possible
common
features of the persons and their environment when the focused behaviours occur. None of them seems to depend on whether
the setting is deafblind specific or not. Nor can we
state a correlation with the individual's general level of functioning or
ability to communicate.
Psychological state and psycho-socially conditioned
factors
The
study investigated in total 11 types of behaviours.
Two of these are low frequent in both groups. Five of the nine variables left
show moderate frequency in the behaviours scored with
much the same level in the two groups. Four variables have scores in both
groups, but clearly higher for the rubella group.
Emotional withdrawal and
increased separation anxiety are,
according to the findings in our study, not particularly a problem among
congenitally deafblind adults. If, however, we look
at social withdrawal, more frequent
waves of sobs and increased
passivity, these types of behaviour
are a problem to one in five congenitally deafblind
persons, with nearly the same occurrence in the two groups. As these behaviours also are known in gerontology, they may be
tokens of early ageing. Less
energy seems to be a problem to every third
congenitally adult person in both groups. We cannot find a clear explanation to
this. Could it be the lifelong deprivation caused by the deafblindness?
Is this also signs of early ageing? Could the explanation be something so
simple as too little exercise? The risk of developing a psychiatric disease is
relatively high in both groups. The only difference between the groups, which
can be stated, is that the disease is diagnosed at an earlier age in the
non-rubella group. Depression is
the most frequent diagnoses in both groups. Is this caused by a permanent state
of deprivation or is it again due to early ageing? Emotional instability occurs
more frequently in the rubella group than in the non-rubella group, we speak of
every second individual with congenital rubella syndrome and one in four of the
deafblind persons with other aetiologies.
The same is manifested for the variable lowered
frustration threshold; again the problem is present in every
second individual with congenital rubella syndrome and in one in four in the
non-rubella group. Do we speak of a late manifestation in the rubella group? Or
do we speak of a symptom of early ageing in the rubella group?
Sleeping disorders, sleeps only a little and
circadian problems.
Here we see a significant difference between the two groups. Every second
individual in the rubella roup experience
considerable sleeping disorders, whilst in the non-rubella group we speak of
one in six. The absence of residual vision affects the individuals in the
rubella group most. In this group the data monitors a significant correlation
between early infection during gestation and severe sleeping disorders later in
life. The individuals among whom we have rated all these problem behaviours constitute a representative section of the
congenitally deafblind adult population in
Concluding comments
The
changes in behaviour observed in the rubella group to
be significantly different from the non-rubella group may indicate rubella
specific late manifestation of the congenital syndrome. Changes of neurological
or physiological characters take place in some of the organs of these
individuals, which result in the onset of problems at somatic, behavioural and psychosocial levels in adult age. Some
indications may point to earlier ageing in the rubella group than in other
persons. When facing changes in behaviour we must
question whether internal factors, external factors or a combination of those
are the trigger of the changes observed. At first it must be clarified if the
cause could be a yet not discovered somatic condition, a neurological condition
or decrease in hearing and/or vision. Attention must be paid to the either
sudden or gradual character of the changes observed. A neurological and
psychological assessment is also important, and the individuals' interaction
with their environments must be observed and analysed
as a part of the total evaluation of the persons' situation. We still recommend
annual health checks for all persons with congenital rubella syndrome. In its
Appendix 2 the report offers a guiding checklist, which may be presented to the
physician examining the rubella person. Also we again stress the importance of
regular appointments for examinations of hearing and vision. In general there
is a need for far more attention to the risk of deteriorations in the two
senses for the deafblind population as a whole.
If
we look at how quickly changes take place in the two groups studied with
respect to the variables scored, it appears that within the two-year period our
survey went on the positives occur almost exclusively in the rubella group
(reported in Appendix 3). If this is a valid tendency we can estimate that only
in five years the differences between the two groups compared will be even more
important. The Information Centre on Congenital Deafblindness
therefore recommends that the results achieved in the present survey should be
followed-up by a quantitative study of the same group of individuals starting
no later than 2006. Until then qualitatively oriented interviews may contribute
to the development of the existing knowledge in identifying useful details from
selected informants. Experience based development is still needed in this very
important area, with advantage in a combination with medical research. Systematic
information of this kind would be highly valuable to the clinical work in our
field and considerably contribute to an increased quality of life for the
individual adult person with congenital deafblindness.