A CARE CENTRE FOR HUNTINGTON’S DISEASE: CONFRONTING ATTITUDES TO CHRONIC TERMINAL ILLNESS

A CARE CENTRE FOR HUNTINGTON’S DISEASE: CONFRONTING ATTITUDES TO CHRONIC TERMINAL ILLNESS.
Published in the Australian Journal on Ageing, Vol.1 No.3, August 1982.

This paper was written by me, Alison Ball, and published during the time that I worked at the Arthur Preston Centre for Huntington’s Disease in Balwyn, Melbourne Australia. The beautiful old home that had originally been built by the entrpreneur Oliver Gilpin during the depression of the early 1930’s, became the very first centre in the world devoted entirely to the care of people who had Huntington’s Disease. I was the first social worker employed by Wesley Central Mission who owned and operated the Centre at that time.

Abstract

This paper first describes the Arthur Preston Centre at Mary’s Mount in Balwyn, Victoria and what Huntington’s Disease is. It then discusses the old attitudes to this chronic, terminal illness and the confrontation with these attitudes presented to sufferers and families. The type of care offered at the Centre is described and the ways in which the experience at the centre calls into question the old professional attitudes and assumptions relating to the sufferers of this disease. Finally a challenge is made for the adoption of a new viewpoint.

Introduction

The Arthur Preston Centre at Mary’s Mount in Balwyn Victoria, was officially opened by the Governor General of Australia, Sir Zelman Cowen on October 25th 1981. Wesley Central Mission of the Uniting Church in Australia have thus provided the world’s first centre specifically devoted to the care of sufferers from Huntington’s Disease. Wesley’s help came in response to the needs expressed by the Australian Huntington’s Disease Association and the professional work already done in the field by Mrs Betty Teltscher and Dr. Edmond Chiu through the University of Melbourne Department of Psychiatry. The spacious property and residence purchased for the Centre had been owned by the Catholic Church and had been built in the early 1930’s by the late Oliver Gilpin. With the assistance of the Commonwealth Government, it now comprises a Deficit Funded Nursing Home for up to 25 permanent residents aged 32 to 73 years and has four other beds which are used for short term family relief care. Additionally, sufferers who live at home or in other institutions attend on a daily basis for socialization groups and approved para-medical services.

Huntington’s Disease is an inherited disease which, after onset, results in rapid, progressive degeneration of brain cells. It is characterised by uncoordinated, involuntary movements, speech difficulties and varying degrees of physical and cognitive incapacity. Onset is usually in the middle years of life though childhood forms are known and some sufferers are not diagnosed until as late as their seventies. The disease does not skip generations and each child of a sufferer has a 5007o chance of inheriting or not inheriting the gene which causes this disease. At this time there is no cure and no test can predict which children will inherit the illness. Death, generally through pneumonia or heart failure usually occurs some 15 to 20 years or more after onset.

The Old Attitudes

In the past in Victoria, and still in most places in the world, sufferers from Huntington’s Disease have been the forgotten people, housed in the back wards of Mental Institutions; the skeleton almost literally in the cupboard for hundreds of families and shunned as frightening and hopeless by communities and professionals alike. Sufferers themselves have been given no hope and families have felt shamed by this disease which has often wrecked their marriages and their lives. They have felt angry that their parents have brought this blight upon them and guilty that they may have passed it on to their children. The community and the professionals have reinforced their shame, and, in the face of their own helplessness in combating this disease have in the main, turned their backs on sufferers as being untreatable because their illness is incurable.

Communities, families and sufferers follow the lead of professionals in attitudes toward particular illnesses and, all too often right to this moment, the attitudes of professionals exude helplessness and hopelessness when this disease is mentioned. In much the same way as they do at mention of Senile Dementia many professionals still literally turn their backs and lose all affect as they say “Huntington’s Disease? They can’t be treated.” Where the old attitudes prevail the assumption is that it is not worth treating someone who has a limited time to live or whose quality of life seems poor. In the case of this devastating, chronic, terminal illness it is thought rather better or kinder to withhold any treatment which may prolong that life.

Teltscher (1) and Chiu (2) have long expressed their challenges to such attitudes. The establishment of the Arthur Preston Centre represents the testing ground of new attitudes derived from their ten years of interactions with Huntington’s Disease sufferers and their families.

A Concrete Confrontation

A building, even a very large one, with sometimes up to thirty-six sufferers of Huntington’s Disease together under the one roof does represent a very strong experience of this illness and a very concrete confrontation with all the old attitudes and assumptions. At our centre our people are not treated as sick, dying people. During the day they are up and about unless they have a specific other illness at the time. They are up because they have work to do. Their work is to take part in the program of activities which is aimed at keeping their minds and bodies active, and which is, in our view, the only and the best way that we can help them. Our people swim, pot plants, do craft activities, play word games, discuss current affairs, take part in relaxation sessions, music and movement therapy. Our physiotherapists who began work only a short time ago clear their chest congestion and put unused muscles back to work. They are excited by what their assessments of these advanced patients might mean in the future for new sufferers. We do not pretend that our people can be cured or that we can even lengthen the span of their lives. All we say is that we hope to improve the quality of their lives, to help them to live each day to the full and, with a realistic acceptance of the limitations, aim to promote their optimum level of functioning at any particular time.

The Sufferers

The confrontation begins with the sufferers themselves. Daily they find themselves in the presence of others in much more deteriorated states than themselves, bringing into sharp focus all their worst fears of what the future might hold for them. Many do not want to identify with the group but there is a sense of relief in not being the odd one out and isolation is being replaced by caring for one another and by a growing self-esteem. Sufferers have expectations placed upon them and, in an atmosphere which treats them as responsible human beings, are now, in turn, beginning to assert themselves and take pride in their accomplishments.

Families

Families too, and particularly those at risk of contracting Huntington’s Disease have also been confronted with this visible evidence of all their worst fears. Evidence of physical deterioration, loss of speech, brain and motor function, peculiar gait and accompanying loss of role and productive ability all combine with distress and grief to drive home the realities of this disease. Very often, for the first time, sufferers and families have realized that this disease cannot be dismissed as a disease of old age but that quite young people can contract this illness and they comprehend at last that this is what may await themselves or their children.

At the same time their children will grow with a knowledge their parents did not have.Future decision making for them will be all the more difficult because they will not be ignorant and a choice to have children will be all the more agonizing. In the past families have been sheltered from such confrontation but at the same time have been isolated and left alone to cope with their fears and their anger. At our centre they are brought face to face with the worst but, at the same time, they see hope for a better quality of life for sufferers and find support amongst friends.

The Professionals

The old attitude amongst professionals are not a feature of the past. Every week we hear and see further evidence of the strength and depth of the myths. The old attitudes meant that once labelled with the diagnosis, sufferers were placed in the long term chronic wards, treated lovingly perhaps but with no stimulation for their minds or activity for their bodies. The old attitudes meant that it was not much use buying day clothes, testing eyes for reading glasses, fixing teeth or fitting dentures. The old attitudes left gynaecological problems unattended and they meant that it was hardly worth the trouble to rehabilitate an elderly sufferer who had broken a leg nor could it have been conceivable to treat the heart condition of an older man. The old attitudes meant that the paramedical services have had no experience at all of working with sufferers of Huntington’s Disease and that participation in the community was discouraged because of the problems. The old attitudes said sufferers were incontinent, that they wasted away, that they were violent or aggressive. The old attitudes said that because speech was incoherent then there was no thinking and, most damning of all, the old attitudes said that sufferers were demented or dementing.

The Reality

Huntington’s Disease does have gross effects upon its victims and many of these effects are a direct consequence of the disease. Brain cells do die off at a rapid rate, functioning in many areas is greatly reduced and for many sufferers some of the problems above are prevalent for some of their years with this disease. But, by writing off sufferers from almost the beginnings of this disease because their illness is incurable, many of the attendant complications have long been ignored and untreated, dismissed as an inevitable concomitant of this disease.
Living day by day with our people forces us to question the old professional assumptions. Do Huntington’s sufferers really have to waste away? We encourage as many as possible to continue to feed themselves even if much of the food ends up on the floor. None of our residents as yet eat vitamised food even though some came in doing so and other daypatients return to nursing homes to eat vitamised food after a normal lunch. Great vigilance must, of course, be exercised in the dining room but most of our residents have maintained weight or gained in weight. Is the supposed incontinence really an integral part of this disease? Our experience suggests that it is rather more a feature of lack of speech clarity which makes it difficult to express needs or in other cases the acting out of anger which is not able to be verbally expressed. Is the aggression and violence we so often hear about also an inherent part of this illness? Our sufferers at Balwyn, in the main, are no more aggressive than you or I. The very few who sometimes are seen to be acting out of fear of what may be happening to them or sometimes it seems the anger attached to the grief which is naturally felt when one is afflicted with any chronic, terminal illness. Often, too, it seems a reaction to finding oneself totally dependent upon others for even the most basic needs.
And is it true that sufferers from Huntington’s Disease are demented or dementing? Certainly the functioning of our people deteriorates in many ways, but dementia? Do demented people listen to the football or cricket, discuss the scores and compare them with previous years? Do demented people cooperate, concentrate and follow instructions to the letter for sometimes up to three or four hours when being examined and assessed by optometrists or physiotherapists? Do demented people laugh at our jokes, or, having been absent for two months, ask us how was our two week holiday which we had said we would be taking when last we met? And do demented people show care, concern and grief for the suffering of one of their friends whose brother or son has died? Dementia may be the medical, technically correct term to describe death of brain cells but, when used in conjunction with the old attitudes it is a perjorative term which, in one foul swoop, is used to write off whole categories of people as not worth bothering about. Chiu(3) has coined a new term ‘Dysmentia’ which expresses a more positive approach to the intellectual deterioration of Huntington’s Disease sufferers.
The Challenge

The reality of this disease can, indeed, be frightening but our advanced sufferers at Balwyn are an inspiration. The battles against the old attitudes which are being fought are not new for the sufferers of this particular disease. Professional and community attitudes have changed in relation to many disadvantaged groups in our society but that the battles must be fought for our people is indicative of how far along the road we still must travel. We do not want extraordinary measures or excessive medical interventions used to resuscitate a sufferer when a heart attack or pneumonia will give a quick and easy release from this world. Our sufferers themselves may well think it a blessing when the lingering has ended. But we do want our people to be given reasonable care for what ails them, we do want to ask the physiotherapists, the occupational therapists, the speech therapists and the doctors to do their best to help the sufferer maintain whatever may be their optimum level of functioning at any particular time. At the Arthur Preston Centre we are in the business of helping people to live each day to the full. Most will presumably reach a point beyond which they will never improve and certainly most could not survive outside a protected environment. All will eventually deteriorate and die no matter what we do. But we are in the business of trying to abolish the sense of helplessness and hopelessness that has accompanied this disease. Our sufferers are people again; people who have rights and responsibilities; people who are able to stand up for themselves and people who are beginning to realise that they still may have a contribution to make to this world. Our sufferers from Huntington’s Disease may be dying; so are we all. At the Arthur Preston Centre they are showing us how to live with a chronic terminal illness. We have the challenge to offer realistic hope and all of us have a responsibility to look not at a disease, a sick organ, at old age or chronic illness but at the person who, in the presence of established disabilities, still has ‘a lot of living to do’.

References

1.Telscher, B., Huntington’s Disease. Some Recent Developments, Australian Social Work. March 1981 Vol. 34, No.1.
2.Chiu,E., Notes on the Management of Huntington’s Disease: A brief guide for family physicians. Australian Family Physician Vol.8 February 1979.
3.Chiu, E.,& deL. Horne, D.J. Dysmentia in Huntington’s Disease; Paper presented at the first Australian Huntington’s Disease conference, 1979.

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