Dear Editor,
We congratulate Malpas on an eloquent paper but disagree with her
conclusions:
If it is appropriate to tell a child that they are at risk of some illness
in adult life, Malpas argues, then it must be appropriate to tell them if
they are actually going on to develop it. Such an action may of course be
entirely appropriate for conditions which affect children, or where there
is some medical intervention in childhood that may affect the later course
of the disease, but Malpas does not consider such instances, rather the
genetic conditions, such as Huntington’s disease where there is no
available medical intervention and onset usually occurs in adult life.
Many individuals at risk of HD, when allowed to make informed choices as
adults, choose not to know their genetic status, even though they have
lived with the knowledge of their own risk for many years. Indeed only a
minority of those at risk go on to have predictive genetic testing. This
remains the main reason to not test during childhood: Testing during
childhood denies those children the informed choice as adults.
Malpas views the distinction between the knowledge someone is at risk
(through knowing a disease is present in the family) and knowledge of
genetic status, as an arbitrary and illogical cut-off, yet we suggest that
this distinction is in place for very good reasons. Whilst it may be
unhelpful and deleterious to a child to deny that the obvious clinical
features a close relative is exhibiting have any relevance to them, it is
not logical to conclude that they might then as well have a genetic test
to confirm their own status. Imagine the following analogy: Most children
will become aware at some point during their childhood that they are
mortal. Some will struggle with this, and spend some time being very
anxious about it, but at some point during childhood their risk of dying
will be assimilated. Suppose it were possible, through some test, to
predict the actual date of death. It does not follow that, just because
the child is aware in childhood that the risk of death is there, that it
is therefore now also logical to predict its actual date. Furthermore, if
such a test were freely available to adults there might be a variable
uptake: Some will want it to use this information to plan their lives
while others would rather live in ignorance of such knowledge. Such
difficult choices should be left until a time that adequate consent can be
given.
The decisions that the child will want to make on the strength of the
test results may initially appear reasonable but, on closer examination,
the claimed benefits of knowledge vanish into the mist. End of life
decisions cannot sensibly be made by a healthy young child who has some
decades to live … and who is likely to experience good times as well as
bad during those years. Decisions about education, career and
relationships may be made by a young child but are more likely to blight
their healthy years than an uncertain risk of disease at some point in the
future. We all live with death ahead of us; those who may harbour a
genetic disorder realise that this death rather than another death may
await them – but to a child or teenager, their situation may not seem very
different from that of their peers. To know that this particular death is
what awaits you, however, is very different and could well be disturbing.
Early knowledge of genetic status may be helpful and lead on to
important choices – if the test gives a favourable result. To assimilate
an unfavourable result while avoiding the perception that this is a threat
will be much harder if the child’s unfortunate genetic status is already
known. For a child to retain “hope” when his parents are anxious and
distressed about a test result will be difficult indeed. The “good”
outcomes of bad test results arise in circumstances where the individual
has chosen to go through testing as an independent person and in a very
different context from that of a child whose parents are clearly anxious
about their child’s future and eager to resolve their own uncertainty at
the expense of their child’s capacity to make an independent choice in the
future.
Malpas does not like secrets within the family. We can all agree that
difficult information should be discussed as openly as possible but, if
the information causes family members great distress or if the relevant
biology is difficult to understand, there will be limits as to what it is
helpful to share with a young child. Passing on the knowledge requires
that the child is shown how to view it from a helpful perspective. This
will be a real challenge for many families who have been devastated by
disease. While many families often manage this task well, to give
information about hard facts, rather than information about mere risks,
might well prove too difficult for many. If an affected parent (or a
parent at very high risk), has tested a child in the hope of finding that
the child, at least, has been spared, and if this hope has been dashed,
then the support that they can provide to help the child may be limited.
A powerful advantage of discussing the family illness with a child
but explicitly deferring the moment of testing at least to their reaching
adult maturity is that this emphasises both (a) that the decision about
testing is serious, and (b) that this decision is for them to make – that
they are trusted with this. So the family and professionals – if they
agree – are jointly acting to acknowledge the difficult situation of the
child and, at the same time, validating the child’s worth as an
individual. This can be a very powerful message and potentially
therapeutic. To move away from this position does not seem to us to be a
move in the right direction.
Angus Clarke and Anneke Lucassen
Dear Editor,
We congratulate Malpas on an eloquent paper but disagree with her conclusions: If it is appropriate to tell a child that they are at risk of some illness in adult life, Malpas argues, then it must be appropriate to tell them if they are actually going on to develop it. Such an action may of course be entirely appropriate for conditions which affect children, or where there is some medical intervention in childhood that may affect the later course of the disease, but Malpas does not consider such instances, rather the genetic conditions, such as Huntington’s disease where there is no available medical intervention and onset usually occurs in adult life. Many individuals at risk of HD, when allowed to make informed choices as adults, choose not to know their genetic status, even though they have lived with the knowledge of their own risk for many years. Indeed only a minority of those at risk go on to have predictive genetic testing. This remains the main reason to not test during childhood: Testing during childhood denies those children the informed choice as adults.
Malpas views the distinction between the knowledge someone is at risk (through knowing a disease is present in the family) and knowledge of genetic status, as an arbitrary and illogical cut-off, yet we suggest that this distinction is in place for very good reasons. Whilst it may be unhelpful and deleterious to a child to deny that the obvious clinical features a close relative is exhibiting have any relevance to them, it is not logical to conclude that they might then as well have a genetic test to confirm their own status. Imagine the following analogy: Most children will become aware at some point during their childhood that they are mortal. Some will struggle with this, and spend some time being very anxious about it, but at some point during childhood their risk of dying will be assimilated. Suppose it were possible, through some test, to predict the actual date of death. It does not follow that, just because the child is aware in childhood that the risk of death is there, that it is therefore now also logical to predict its actual date. Furthermore, if such a test were freely available to adults there might be a variable uptake: Some will want it to use this information to plan their lives while others would rather live in ignorance of such knowledge. Such difficult choices should be left until a time that adequate consent can be given.
The decisions that the child will want to make on the strength of the test results may initially appear reasonable but, on closer examination, the claimed benefits of knowledge vanish into the mist. End of life decisions cannot sensibly be made by a healthy young child who has some decades to live … and who is likely to experience good times as well as bad during those years. Decisions about education, career and relationships may be made by a young child but are more likely to blight their healthy years than an uncertain risk of disease at some point in the future. We all live with death ahead of us; those who may harbour a genetic disorder realise that this death rather than another death may await them – but to a child or teenager, their situation may not seem very different from that of their peers. To know that this particular death is what awaits you, however, is very different and could well be disturbing.
Early knowledge of genetic status may be helpful and lead on to important choices – if the test gives a favourable result. To assimilate an unfavourable result while avoiding the perception that this is a threat will be much harder if the child’s unfortunate genetic status is already known. For a child to retain “hope” when his parents are anxious and distressed about a test result will be difficult indeed. The “good” outcomes of bad test results arise in circumstances where the individual has chosen to go through testing as an independent person and in a very different context from that of a child whose parents are clearly anxious about their child’s future and eager to resolve their own uncertainty at the expense of their child’s capacity to make an independent choice in the future.
Malpas does not like secrets within the family. We can all agree that difficult information should be discussed as openly as possible but, if the information causes family members great distress or if the relevant biology is difficult to understand, there will be limits as to what it is helpful to share with a young child. Passing on the knowledge requires that the child is shown how to view it from a helpful perspective. This will be a real challenge for many families who have been devastated by disease. While many families often manage this task well, to give information about hard facts, rather than information about mere risks, might well prove too difficult for many. If an affected parent (or a parent at very high risk), has tested a child in the hope of finding that the child, at least, has been spared, and if this hope has been dashed, then the support that they can provide to help the child may be limited.
A powerful advantage of discussing the family illness with a child but explicitly deferring the moment of testing at least to their reaching adult maturity is that this emphasises both (a) that the decision about testing is serious, and (b) that this decision is for them to make – that they are trusted with this. So the family and professionals – if they agree – are jointly acting to acknowledge the difficult situation of the child and, at the same time, validating the child’s worth as an individual. This can be a very powerful message and potentially therapeutic. To move away from this position does not seem to us to be a move in the right direction.
Angus Clarke and Anneke Lucassen