From Chapter 3 - LEARNING THE DIAGNOSIS
Telling the children
Those of you who are parents face another worry, what to tell your children. Much will depend on what age they are, and whether or not they are living at home. If you decided knowing the risk, to have your children and if the presence of HD has not been hidden in your family, then your task may be easier.
Even so, all parents are likely to feel guilty that they may have been responsible for passing on the gene as well as anxious that the lives of their children may be threatened. It is probably wiser to accept from the start that at some time in the future your children or young people will get to know anyway, and that keeping important facts from them may injure family relationships.
Indeed, children who are deliberately kept in ignorance can turn against parents if they discover the truth for themselves in a way which shocks and distresses them. Nowadays there is discussion about genetic illnesses in school and on television and radio, so the truth will come out eventually whether you wish it or not.
In deciding how much to tell children, it is important always to tell the truth but not necessarily the whole truth and certainly not all at once. Don't lie to children, but don't burden them with information beyond their understanding. That is why it is so important not to wait for 'the right time' to tell.
There is no 'right time', and the danger of waiting until adolescence or later is that either the young person will find out in a distressing way or else you will find yourself forced to tell because your young person has a steady boy or girl friend, is planning marriage, or is already pregnant.
It is better for children to learn about difficult facts in their lives as young as possible, so that they can get used to them before they come to realize the full implications. If parents can create the kind of atmosphere in which it is alright to ask questions, then children will generally ask as much as they want to know and no more. Ideally, children should absorb the facts about HD with other important facts about their lives.
Some of you may be able to face this task on your own, but most parents will find it difficult and may need to find someone with whom to talk over their own feelings and the needs of the child. This could be a relative or close friend, doctor, social worker or other professional. There are also genetic counseling clinics in each health authority region where help is available. The important thing is to find someone with an understanding of both of HD and of children, who will give good advice and help you work out the best approach.
It may help too, to get together with other parents and share your ideas, provided you don't feel that everyone must agree on how to act, because each family will have different circumstances. You can meet other parents through joining the HDA or contacting its Family Services.
Children can cope better than adults think, but parents need to be sensitive to the way in which children react to information which makes them anxious. If the child is old enough, and especially if there is some reason why you must explain quickly, distressing information about HD needs to be balanced by more optimistic descriptions of the research into HD now and possible future developments in predictive tests, care and treatment. It is a fact that many young people can cope with knowledge of HD without undue anxiety and find a partner willing to share the risk.
There may be few who feel overwhelmed, even seriously depressed, who may need professional help to see them over a bad period. If you have talked over this possibility beforehand with someone outside the family, you will be able to get help quickly if needed.
Chapter 4 - YOUNG PEOPLE AT RISK
Facing the reality - a balanced view
Being at risk to HD obviously presents a number of
difficulties, but perhaps the basic one is learning to
live with the knowledge realistically
Some people experience a kind of see-saw effect;
on some days they feel sure that they will get HD,
and on others they are sure they won't. Either
extreme distorts reality
If you are too pessimistic, you live in continual dread
and are unable to plan a career or a rewarding relationship,
or cultivate interests. You may also be constantly
looking for symptoms and the smallest accidents take
on a different meaning.
Being too optimistic, on the other hand, may lead to
denial and irresponsibility, such as not telling your partner
and having children without making the decision rationally.
This can lead to misery later on.
The more knowledge you have about the disease, then the more you are able to view your situation realistically and plan your life accordingly. Being prepared for both getting it, and not getting it, can mean that you can enjoy a satisfying life which is not destroyed if you are unfortunate enough to get HD.
This is, of course, easier said than done, but a positive attitude can be helped by remembering that you are not alone, that keeping it to yourself makes it worse, and that even after the onset of the disease life can have meaning and happiness.
Learning that you are at risk
However you learn that you are at risk to HD, you are going to worry about the possible consequences, but the manner in which you were told will to some extent affect your outlook.
If your parent developed HD early on, you may have
grown up with its constant presence so that you are
never actually told about HD - though the knowledge
is around. You may have been subjected to the stresses
of living with HD from an early age and though this
may have had a profound effect on you, knowledge of
the disease does not come as a shock.
If you are a little older when your parent is diagnosed,
then again the knowledge of its effects comes gradually.
You may experience great sorrow at a loved parent
changing physically and mentally until perhaps roles are
reversed and the child cares for the parent. It needs to
be remembered that not all people suffering from HD are
affected in the same way. If there is one aspect of the
disease that you find particularly distressing in your
parent it does not mean that you would suffer in a similar
way. Seeing a dependant parent may also make you fearful
for your own future and possible loss of independence, a
thought which is difficult to live with
Where this is no evidence of HD in the family, perhaps
because a parent is at risk but has not shown any symptoms,
it can be a great shock for a teenager suddenly to be told
the facts of the disease. It may seem at first to disrupt
completely all your plans for a career, marriage and the way
you want to live your life.
However you learned about HD, you will have a number of
anxieties about the future. Perhaps you may even be worried
about your present health - whether in fact you could already
be showing symptoms. You may be concerned that you look
like or have a similar personality to your parent with HD and
irrationally fear that you are therefore mor at risk. These are
common and very understandable anxieties which can be
dealt with if brought out into the open and discussed, but
can be overwhelming if kept to yourself.
Knowledge of the real facts of the disease, rather than the
belief in some of the myths that surround it, leads to a realistic
assessment of your situation. For instance having a parent
who is at risk, puts you in a different position from those whose
parent is already affected, and this may influence your outlook.
An appointment with a neurologist or geneticist organised by
your family doctor would give you the chance to ask questions
and clarify the information you have already received.
Some common reactions
The temptation to blame parents is also strong, and it is wise to remember that parents themselves may be feeling guilty. They may or may not have known about HD when they decided to have children. If they didn't know, they may be blaming their own parents for this; if they did know, they may well have taken the risks into consideration and still thought it was worthwhile for their own and their children's sake. A frank discussion and explanation is the obvious approach to the problem, but children and/or parents may find this difficult and it may take time to fully understand each other's feelings.
You may also be worried that even if your friends know about HD they may not understand the pressure you are under or that they will treat you differently. You may feel that boy- or girlfriends will be discouraged and you will never meet anyone who wants to marry you, which also brings out the problem of exactly when, in terms of a relationship, is the right time to tell someone.
If you are already engaged or married, the problem could be how much to share the knowledge with your partner and how the relationship will be affected. There is no easy answer except perhaps that the information has to be shared whatever its consequences. To keep such a secret would place an enormous burden on you, and the fact that there is something hiddenis bound to cause difficulties with your partner.
You may choose to limit the number of people you tell, but those close to you really need to know. Even of the knowledge should cause a breakdown in a relationship, it is better to establish early on that your future partner or friend cannot cope with the implication of the disease, rather than at a crisis later. Not having been given the facts can lead to bitterness in husband or wife and greater likelihood of rejection. Sharing your anxieties with other people can help you to see that some of these worries are normal in all young people whilst others are peculiar to HD. Such understanding and reassurance can help.
Marrying someone at risk
Thinking of marrying someone at risk can pose problems. If you have never seen or even heard of HD, you may try to ignore the risks and possible consequences. Some of you will feel so frightened that you break off the relationship almose immediately without going further into the subject.
Learning the real facts and weighing up the risks and what they mean to you, against the quality of the relationship, is the only way a proper decision can be taken. If you understand the implications and can work out plans for the possibility that your partner both may and may not get HD, then your marriage can be happy and a sucess whetever happens.
After careful consideration, however, you may feel that you do not want to risk having to care for an ill person, or you may particularly want children without having to put them at risk too. It is difficult to reject someone for reasons that are just a possibility in the future, but unless you are convinced that you can live with the thought or the fact of HD, then it is better and kinder in the long run to decide against the marriage early on.
Whether or not to plan children
Perhaps the greatest problem that young people at risk have to face is the issue of having children. If you do not want to have the sort of predictive test which was discussed in Chapter 2, the decision has to be taken in the dark.
Some people feel strongly that they should not burden a future generation with the risk of HD and therefore decide not to have any children. Others feel equally strongly that they want to lead as normal a life as possible, which includes having children, and that the risk is worth taking. We should perhaps remind ourselves that not every couple who plans a child will conceive. Overall about 1 couple in 10 cannot conceive and this is nothing to do with HD.
Those that feel that their lives will be incomplete without children may consider some of the following: IVF (in vitro fertilisation); AID (artificial insemination by donor); fostering (couples at risk to HD are not usually allowed to adopt); becoming a social aunt or uncle; limiting family size to one or two children; having children early so that if the parent should get HD the children would be at a reasonable age by then. Pre-natal testing has already been discussed in Chapter 2.
Whatever the outcome, the decision is not easily arrived at and needs a lot of discussion between the two people involved. It is a good idea to seek information and the chance to discuss your personal situation with someone who is knowledgeable in the field. You can be referred by your doctor to a genetic counsellor who will see you, or you and your partner, by appointment. The counsellor will be able to answer your other questions. This could be a good opportunity for you to talk about your anxieties and perhaps sort out any conflicting or questionable information you have already been given. Some genetic counsellors offer further appointments so that you have the chance to go over again any information you are not sure about, and ask questions which have occured to you in the interval or which you forgot to ask the first time.
Your own doctor or a clinic will be able to help you with family planning whether permanent (sterilisation) if you are completely certain about the decision not to have children, or temporary until you are ready to make your decision. You may still feel that you would like to talk more to someone outside the family, if so ask to see a social worker, either from your own Local Services Department or from the hospital where your genetic counsellor is based or where one of your family is being treated.
Responsibility for a parent
Some young people are faced with the dilemma of how far they should be involved in the care of a sick parent. This problem is not, of course, unique to HD, though it may become one earlier than in other families.
The question of care is discussed in Chapter 5, but it needs repeating that you have to consider very carefully the immediate and long-term effects on you before you commit yourself to looking after a mother or father however much you may love them.
This may be right for some people but not for others. You can be under considerable pressure from others, even from your well parent who depends on you to take over at times, but a social worker will help sort out what services are needed, what are available, and what help from you is reasonable given your own feelings and circumstances.
Other practical problems
Other important worries about HD are in relation to insurance, employment, mortgage and so on. Where you have to make a medical statement it is wise to include information about HD as otherwise any documents, such as an insurance policy, could be invalid if it were proved that you knowingly gave false information. Where you give permission for a medical reference which includes family illnesses, your doctor will have to give the relevant details.
It is an unfortunate fact that you can be adversely affected in these matters by being at risk to HD, but not necessarily so, and it is worth shopping around various insurance companies to get the terms. Some jobs will need medical references where health is an important factor. Employers may be prejudiced, but this could largely be through ignorance and a frank discussion about the disease may be helpful. Where no medical recommendation is required, it is up to you whether or not you tell your employer that you are at risk.
Chapter 5 - THE COURSE OF THE ILLNESS
HD in children
Some people dread more than anything that their child will get HD. It is extremely rare in young children, but understandably parents do worry about a child who is clumsy or not getting on as well as expected.
Parents should take advice if they are worried, though usually their anxieties prove to be unfounded. Parents have hopes and ambitions for their children and it is very hard to accept that these may not be fulfilled.
Parents may also feel guilty that they may have caused the child to be ill, whether or not they knew the risk at the start. There are also practical problems, particularly if one parent is already caring for the other.
However, HD in young children is very rare and brings special problems beyond the scope of this guide; specialist advice and help need to be sought