Marriage, Sex and Counseling in Huntington's Chorea

Audrey Tyler, BA, AIMSW

Huntington 's chorea is one of the major inherited diseases. I t is a chronic, incurable condition resulting in progressive mental and physical deterioration, usually characterised by a combination of in- voluntary movements and loss of intellectual faculties such as memory and concentration. Onset is usually in middle life. The gene can be t ransmit ted through either parent, and both sexes are equally af- fected. The sufferer who carries this gene has a 50-50 chance of passing it on to his or her children, and, as far as we know, it never skips a generation.

Thus, children with an affected parent have a 50-50 chance of developing the condition themselves, and, if they do, of passing it on to their children. Truly, it can be said that, they live with a hidden disability. There is no predictive test, so they cannot know whether or not they themselves carry the gene until they reach late middle age at least {unless, of course, they develop symptoms earlier}. Even if they do not develop the complaint, they probably have had to live for many years with the worry that they could develop it, a worry that they often find very difficult to share with anyone else, even members of the same family.

Children at risk of developing Hunt ington 's chorea can face a series of agonizing decisions with regard to marrying and bearing children themselves. First, do they tell their fiance or spouse? This can be par- ticularly difficult where the parent at risk may not yet show signs of the complaint, but has still not lived through the high-risk period; or where the parent has vague symptoms which could be the beginning sign of H.C., but equally could be attributable to other causes.

Secondly, do they have children or not? If they want children but are very worried about the possibility of passing on the complaint do they wait until the diagnosis is confirmed or otherwise in the parent?

Audrey Tyler is associated with the Welsh National School of Medicine, Cardiff, U.K.

160 Sexuality and Disability

Do they wait until the parent has lived through the high-risk period without showing symptoms? Or do they defer having children for a few years in the hope that a reliable predictive test becomes available?

If they decide to have children regardless of the implications, or do not discover the family complaint until after having borne their children, there arise such questions as: do they tell these children; and if so, how, when, and by whom should they be told?

If a couple decide not to have children the alternative choices open to them are very limited. Obviously this is an area which needs further exploration. In our study a few individuals selected partners who already had children, and a few deliberately decided to concentrate on a career.

Counseling: Turning to the ways in which we try to deal with some of these questions and problems in our genetic counseling clinic, first, it can be important to establish that the diagnosis is accurate, or at least highly probable. Secondly, it is very important to make sure that the family fully understands the risks and genetic implications, and this can mean reassuring people at low risk as well as warning those at high risk. We consider that information should be given before mar- riage and that future spouses should be put fully in the picture. Some marriages may not then take place. But it is our experience that if spouses find out after marriage they can harbor a deep, burning and lasting resentment at not being told. In our study of H.C. patients 38 out of 61 spouses questioned were positive that they wished they had known earlier; in the children-at-risk study 20 out of 70 spouses ques- tioned also wished they had been told earlier.

We consider that our task, in genetic counseling, is to help people to come to the decision which is right for them, and we do not advise them not to have children. They may, of course, reach this decision themselves. We do counsel telling children the t ruth about the risks. In our s tudy we found that, difficult and painful as it may be, the vast majority of children at risk felt they should be told, and had a right to know.

A point worth making is that many children are aware that there is a family "secret," and can actually be relieved when it is brought into the open. Ideally, the subject is introduced gradually over a period of time by the parents themselves, with the genetic counseling clinic and general practitioner providing professional backup. Unfortunately, this is not always possible, particularly where a family has broken up, and a professional person may need to take the initiative when the children reach adulthood.

There are no easy or clear-cut answers in dealing with this disease;

161 A. Tyler

parents and children, genetic counselors and other professional person- nel, often face a terrible dilemma. The latter also often have to deal with a web of tangled emotions and try to help parents and children to come to terms with strong feelings of guild and resentment. I t is our hope that by using a combination of methods, including sympathetic and skilled support, and vigorous methods of ascertainment, we shall ultimately reduce the incidence of this devastating disease.