Caring
"Caring must take precedence over curing when no cure is available.  That doesn't mean we don't go and look for a cure. While we're looking for the cure, let's continue the care."  Dr. Edmond Chiu, September 1989



 

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Standards for HD Family Care
  

On September 12, 1989 Dr. Edmond Chiu presented his lecture Understanding Behavioral Changes In Huntington's Disease to the HDSA. Dr. Chiu's simple and profound standards for family care have not been met in the US.

Phillip Hardt renews Dr. Chiu's call for reform. The message was very well received. Phil was interrupted about 6-7 times with applause after each section and then received a rousing standing ovation at the end. It was overwhelming.
-- Jerry Lampson



Families Are Bleeding In TheTrenches

 Phil Hardt

INTRODUCTION

Good morning. I wish to thank HDSA and Athena Diagnostics for this opportunity to participate on this Family Services and Care Forum panel.

My name is Phil Hardt and I'm living my inheritance- HD. I wish my genes would have come pre-shrunk, but they didn't!

I am 45 years old and have been unable to drive for five years and work for four. I also have eight children. Three are biological (one negative and two still at risk) and five adopted. Of the five that we have adopted, three have severe handicaps. Six of my eight children are still living at home and my youngest daughter is only five years old.

First of all I want to praise HDSA, under the leadership and direction of Barbara Boyle, for the momentum at which new discoveries are beginning to make the headlines! These are very exciting indeed. I would also like to personally thank all of the researchers, scientists and doctors here today whose valuable contributions have brought us closer to the goal of everyone here - a cure!! We would not be where we are without you!

However, don't start resting on your laurels yet as I want to talk candidly about a few other areas of concern where I feel improvement needs to be made immediately.

THE PROBLEM AND THE CHALLENGE

I believe we are overlooking many HD-affected individuals and families who are "bleeding in the trenches," at this very moment while focusing primarily on finding a cure! I agree with Dr. Edmund Chiu, Senior Lecturer in Psychiatry University of Melbourne, Australia, whole-heartedly when he said: "Caring takes precedence over curing when no cure is available. In the United States as well as Australia, too much emphasis is placed upon curing a disease and not enough on caring for a disease."

I believe we have too many families falling apart at the seams because of two main problems, 1) lack of education for everyone regarding the "softer" symptoms of HD and the destruction they cause, and 2) lack of resources and medication available to confront what I like to call these "softer" symptoms of HD, which, in my opinion, are far more devastating than chorea to the individuals and their families.

When I talk about the "softer" symptoms of HD I don't mean the physical hallmark of easily-diagnosed chorea. What I like to call the "softer" symptoms of HD are the emotional, cognitive, behavioral, personality change, and loss of social inhibitions, those symptoms that start subtly and are not easily recognizable, but that perhaps do the most immediate and long-lasting damage to the Phd or person with HD and their family. At least these are the ones that I've had the most trouble from. I believe that everyone wins when these "softer" symptoms are addressed. The Phd's quality of life is improved and the caregiver's quality of life will improve.

DEPRESSION/SUICIDE

We need a national program to educate everyone about depression and suicide. It is so easy to treat but so devastating if left unchecked. HD-related depression packs a double whammy so-to-speak, depression is caused not only from the biological effects of the disease but also as a reaction to it. A reaction to losing everything which has helped define you as a productive and worthwhile member of society such as your work, freedom and role within your family. We need to help everyone to understand very early that untreated depression can lead to suicide. We should continue to educate and reeducate, and not be satisfied with our efforts until the appalling suicide rates of those with HD are not 6 to 7 times the national average!

ASSISTED LIVING/NURSING HOMES

We need to provide our expertise, training and funding to establish HD-friendly 'assisted living homes,' staffed by competent, HD knowledgeable professionals, around the country to help those who are still capable of living by themselves to maintain their independence. In addition, we need to provide our expertise, training and funding to establish regional HD-friendly nursing homes so that no family will be too far away to visit their loved ones frequently, and so that they can be assured that their loved ones are being cared for by compassionate professionals who thoroughly understand this disease and its complexities. These can also be used as respite care providers when loved ones need time to recharge their batteries! I would hope that when we do so, we would use the enlightening philosophies of Dr. Chiu from Australia as our guiding light to run them in order to assure that our loved ones receive the highest "quality of life" possible.

MEDICATION

I think we need to begin new clinical studies, thinking 'out of the box' as far as new medications are concerned. I would like to see additional medications that are already being used successfully to improve cognition in Alzheimer's Disease and other related disorders, such as Aricept, be tried to improve the "quality of life" of persons with HD. More emphasis needs to be placed on meds to help the family cope with the softer symptoms of HD, the emotional, cognitive, behavioral, personality changes, and loss of social inhibitions, while still maintaining the highest "quality of life" possible for the person with HD.

LEGAL ASSISTANCE

We need to provide more assistance and guidance to those families who get a call from their son or daughter, who has Juvenile HD, while they're in jail for shoplifting or drug and alcohol abuses, or families who need guidance because their father got apprehended for masturbating in public. These are all real needs of real people with HD today.

COUNSELING

We need counseling manuals specifically for use by those counselors and therapists in our own communities to help those families with HD-shattered marriages or relationships. Manuals could also contain useful information to discuss with those going through the pre-testing protocol too. I know when I went, I found out that I knew more than they did, and believe me, that wasn't a whole heck-of-a-lot! I ended up "counseling" them on what HD was, not them counseling me. The Centers of Excellence are too few and far between for many to receive this type of ongoing counseling.

LONG-TERM DISABILITY INSURANCE

We need to increase our efforts with national emphasis on the growing number of those at risk and encourage them to purchase as much long-term disability and care insurance as they can immediately, not as an admission that they may get the disease, but as just good common sense that any other person would do.

SOCIAL SECURITY DISABILITY

We need to assist those individuals who need help completing a social security disability application in such a manner that they don't get turned down and denied these needed monies. They should not be subjected to the stress of having to reapply or have to subject themselves to a Social Security-appointed doctor who knows nothing about HD.

LIVING WILLS, TRUSTS, ETC.

We need to do a better job educating everyone regarding the necessary legal planning to protect our spouses and families such as living wills so you don't have to subject them to uncomfortable decisions about whether-or-not you want a feeding tube or to be kept alive artificially later. Durable powers of attorney are needed to make the transition easy after dementia progresses, family trusts to prevent our life insurance from going into probate for a year. These costs are insurmountable to many.

PROFESSIONAL EDUCATION

The focus needs to continue regarding the education of ALL neurologists, psychologists and psychiatrists. This will go a long way to eliminate misdiagnosis and inappropriate medications prescribed which in turn would greatly enhance everyone's "quality of life."

I also believe that there should be input by those with HD into all of the recommendations I am making here today. There are many Phds who would be more than happy to contribute their knowledge of living daily with this disease.

CONCLUSION

As each generation passes, the next has almost exponentially more individuals at risk. We need to prepare better NOW for our children and grandchildren's generations or we have failed them indeed. I believe the numbers reported to be at risk are grossly understated and even as stated are staggering to contemplate. Will we be prepared for this onslaught of additional persons "bleeding in the trenches" or will insight, education, and improved caring increase their "quality of life" far beyond ours? Thank you for your valuable time and may God bless everyone affected by HD!


Response to this article:
P.S. The response to the "Reform" article is still overwhelming. Tremendous grassroots support all over the country. Even had two brothers send me a note stating that they would be splitting up everything that they raised and donating half to research and half to care after reading the article! Isn't that wonderful?...Phil

Source: HDlighthouse.org  - published -0-5--2002


 

CARE

&

QUALITY OF LIFE

 

 

Firstly, the HD family has the right to all available care. This is not a privilege; it's a right.

We hope and pray for a cure but there is no cure for Huntington's Disease, not yet. Good people are working on it of course. In the meantime, other good people are working on the only alternative available- care. The kind of care that doesn't abandon the Huntington's Disease person.

To provide hope. Not hope for a cure but hope for a better life. And then, hope for the cure to follow.

The risk-taking policy is talking about enabling an HD person to take the highest risk to maintain quality of life. I believe, unless we can take risks with Huntington's persons, we're not giving that person the quality of life in the sense of control and autonomy he or she has a right to; not deserved but has a right to.

We adopted a risk-taking policy right from the beginning. We'll take risks with family and individuals. For instance -- this might be very controversial in this context -- none of our patients in Melbourne has any form of tube in his or her stomach. It's not an option. It's not considered…We will take the risk of patients choking and they know it. We will do all we can to prevent choking…We use all kinds of strategies: occupational therapy, use of Valium, all sorts of physical strategies combined to help that person to eat normally, without tubes. In ten years, we've only had six incidents of inhalation pneumonia. No patient so far has died of inhalation pneumonia. Now that's risk-taking…

We let them walk as much as possible. If they fall, we pick them up and get them walking again. If they fracture a bone, that's the risk we have to take. We haven't had too many fractures so far. Lots of bruises but not so many broken bones. We accept the bruises for the price you have to pay for autonomy and independence.


Dr. Edmond Chiu: Senior Lecturer in Psychiatry, University of Melbourne, Australia September 12, 1989  (Full talk is below.)


 



UNDERSTANDING BEHAVIORAL CHANGES IN
HUNTINGTON'S DISEASE


A Presentation by:
Dr. Edmond Chiu,
Senior Lecturer in Psychiatry
University of Melbourne, Australia
New York University Medical Center


September 12, 1989


Courtesy of: Huntington's Disease Society of America, Inc.
140 W. 22nd St., New York, NY 10011-1420
(212) 242-1968

INTRODUCTION



Patsy Marr, Chairperson,
HDSA Patient and Family Services Committee

I was given the privilege of introducing the keynote speaker, the person you
all came to hear. Dr. Edmond Chiu was born in Hong Kong and migrated to
Australia in 1952. He went on to basic medical qualifications at the
University of Queensland and moved to Melbourne to obtain his post-graduate
training in psychiatry. He continued his post-graduate experience in Hong
Kong and England, where he qualified with a Diploma of Psychological
Medicine from the Royal College of Surgeons and Physicians in 1971.
When he returned to Melbourne in 1972 he was volunteered --and he has that
in parentheses -- by Professor Brian Davies to become a clinician member of
the Huntington's research team and that has, in turn, become the Arthur
Preston Centre which most of us know is the world renowned center for the
care of HD. This makes 18 years that Dr. Chiu has had extensive experience
in the day-to-day care of HD persons.

All expenses for Dr. Chiu's trip are being funded by the Foundation for the
Care and Cure of Huntington's Disease, which is also supporting a return
visit of Dr. Chiu's next year. In fact, right before this meeting, we were
meeting with him to make the final arrangements for that tour. So, even
though we're privileged to hear him tonight, we're going to get him back
another time.


UNDERSTANDING BEHAVIORAL CHANGES IN HUNTINGTON'S DISEASE

Dr. Edmond Chiu

Thank you, Patsy. It's always a pleasure to come to the United States to
talk about Huntington's Disease because it is my way of repaying a personal
debt to the late Marjorie Guthrie the widow of Woody Guthrie. For the
younger generation here, and I see a large number of younger people who may not have been into folk music, Woody Guthrie was the folk music hero of my era. That dates me. He was mistakenly diagnosed with all kinds of horrible
things including alcoholism, schizophrenia etc., when, in fact, he had
Huntington's Disease. Marjorie fought a strong battle to put Huntington's
Disease in its proper place with regard to care and research, and it was
Marjorie Guthrie's enthusiasm, upon a visit to Australia in 1976, that kept
me working on HD.

I started in 1972 and by 1976 I was beginning to feel a little burned out.  I wasn't quite sure what I was doing working with HD when my career path was
in psychiatry anyway. And I wasn't making any headway in my academic career in HD. And as a young psychiatrist, I said, "Well, what on earth am I
doing?" Marjorie told me what I was doing. She came to Australia to
launch the film called "Bound for Glory," a biographical film on Woody -- at
least the first half of Woody's life -- and she sat me down and spent a lot
of time with me and persuaded me that I should continue to commit myself to
HD together with Mrs. Betty Teltscher, who is a sort of a 'mother figure'
and supported me through 18 years work on HD.

So I'm coming back to New York and the United States to repay the debt to
Marjorie. And those who look at my face and realize that I'm Chinese. We
Chinese have a funny cultural habit of visiting people's graves, particularly graves of our teachers and those who have been valuable to us in our lives. It's a sort of hangover from ancestor worship. Although I'm a Christian, I still have the cultural thing about paying respect to one's teachers. And every time I come to New York, I always go to Coney Island and pay my respects to the ashes of Marjorie Guthrie scattered outside Coney Island Pier. And for those of you who really want to do that, may I suggest you do so regularly, because she is the person who really got a lot of us into the work of HD.

So what I'm telling you today has its origin back in the days of Marjorie
Guthrie's fight for HD. And I would at least like to acknowledge that when
I speak to you all.

My experience with HD has been a very rewarding one, particularly in my
professional career, because what I learned from the patients and the families has indeed given me very good training in my practice of psychiatry. A lot of the things I learned have now been translated to my students. Hopefully when they graduate as doctors in Australia, they will carry these principles with them in their psychiatric and medical practice.

Some of the biggest problems in HD, in the care of HD, are the so-called
'behavioral problems.' You hear that all the time. Nursing homes and hospitals say, "What do we do with the behavioral problems of HD patients?"  The way they say it is usually pejorative. It's always very negative. And it's always implied that there's something 'crazy' about HD patients. And, in some way, I'm trying to get rid of the myth about HD patients by trying myself to understand the behavioral difficulties, what they mean, what is the basis for them, and how we can handle them.

But before I talk about the behavioral problems, I want to establish a few
principles. Because without those principles, you may not fully understand
how I explain the understanding of behavioral problems of HD. These are the
philosophies of care for HD which I have been able to establish through our
working together.

Firstly, the HD family has the right to all available care. This is not a
privilege; it's a right.

S
econdly, caring takes precedence over curing when no cure is available. In the United States as well as Australia, too much emphasis is placed upon
curing a disease and not enough on caring for a disease. I'm very glad to
hear, last week when I was at a conference in Tokyo on my way here, the
Deputy Director of the National Institute on Aging, Dr. Gene Cohen, spoke to
the gathering on the philosophies, directions and policies of the National
Institute on Aging. He mentioned Alzheimer's Disease, which has no cure.
The National Institute on Aging is now proceeding with a policy of modification in caring for the person with Alzheimer's Disease. I said to myself, "Cheers!" and finally woke up to the fact that I've been practicing this for the past 18 years. Caring must take precedence over curing when no cure is available.

That doesn't mean we don't go and look for a cure. While we're looking for
the cure, let's continue the care.

When we talk about care, we talk about total, comprehensive, holistic and
balanced caring. It's not just caring in the medical or nursing manner or
the PT manner - we put everything together into caring for the individual.

Quality of life is more to be pursued than quantity of life. We can do little about quantity of life. It's not our prerogative to decide how long a person shall live. But we can do a lot about how well that person can live. So the most important guiding principle when looking at an HD patient is how well that person can live. It's better to live a happy and short life than a long and miserable one.

Ability is to be maintained and optimized. Self-explanatory. Ability development is more relevant than working with irreversible disabilities.  To a large extent, Huntington's patients have a lot of ability that we still can develop with them.


Lastly, clinical and anecdotal assumptions of HD need to be constantly
challenged. The moral and theological term, "demythologization" is a basis
for research and understanding and education. We must not take assumptions
written in medical textbooks and circulating around as 'gospel truth' about
HD without challenging each one of them. So if someone says, "HD is XYZ,"
the answer is "prove it." If you want me to prove it, I shall take it away
and try to prove it.

There will be other people after me who will test out a lot of the clinical
observations we all make. So if I say anything that you think is not relevant or not useful, it is up to you to challenge all of us by saying, "Hey, I think you're not right."

The Huntington's patient and family has the right to remind us professionals
that we need to demonstrate that what we say can be proven, and is relevant
and useful. It's your job to do that because we often do not challenge
ourselves. You have to do that for us.

In developing a care center like Arthur Preston Centre or the Cardinal Cooke
Center, we have the following goals:

1) To provide the best quality of care so as to give the best quality of life possible. Notice that quality of life is always the issue.

2) To provide hope. Not hope for a cure but hope for a better life. And then, hope for the cure to follow.


3) To establish a concrete confrontation of the disease, thereby resolving the existence of denial, shame and guilt by fostering pride, self-esteem and a genuine sense of worth. And when we say concrete confrontation, we do
mean concrete confrontation: bricks and mortar. Because without bricks and
mortar, it's very difficult to provide care to people. Winter in New York, without bricks and mortar, is not the way for healthy people. So many people die in freezing cold New York when there's no where to go. So when I say 'concrete, ' I mean not only concrete in the psychological, emotional term, but concrete in terms of bricks and mortar. A place to live.

4) No one can do HD care by himself or herself. It's a cooperative effort with all kinds of organizations networking together and sharing care. And sharing expertise also with other disciplines. I work not only in a multi-disciplinary manner but in a cross-disciplinary manner.

I was explaining to some of the staff at Cardinal Cooke this week about the
way I operate. If the patient happens to need his or her chest 'thumped'
because they're not able to cough, and the physiotherapist happened to be
elsewhere, I take the job of doing it myself. If I don't know, I ask the staff or the therapist to teach me for the next time. If the patient happens to wet himself or herself, and no nurses happen to be available, it's my job to help that patient to get dry, to go to a place to change if the patient is a male. If it's a female, I'd rather not because it can be embarrassing, even though I'm a doctor. It's my job. So l do not have a demarcation with other staff - the nurses, the PT's, the OT's. If the person has a spill and needs cleaning up and I happen to be the first available person, I do it. So that's working cooperatively and sharing the
task.

5) To provide a base for education to the community at large, educational
institutions and self-help groups in a continuing effort to destigmatize HD.
And certainly at Arthur Preston Centre, for those of you who've ever seen the building, you realize that HD got destigmatized very rapidly. It's one of the most prestigious properties in Melbourne. The church had sold the surrounding area which they did not need and the average block of land -- suburban land, that is -- which is roughly 150 by 120 feet, sold for $857,000. And on those blocks of land now sits one and a half to two million Australian dollar properties. So the Arthur Preston Centre is surrounded by the 'nouveau riche' who came after the Huntington's patients.  They decided to join us. How many of you can afford an address on Fifth Avenue, New York? That's a good destigmatizing strategy!


6) To provide a base for research and evaluation. I'm very glad that the
Cardinal Cooke Center is associated with the New York Medical College and I
hope the association will continue. The research and evaluation will proceed out of that.

7) To provide a focal point for the stimulation of research.

8) And to provide the best possible care to develop a model for the caring of related brain disorders and services for the elderly.

One of the characteristics of caring in Melbourne led me to try to understand 'behavior problems' of HD. The characteristic is the rights of the patients. I've taken the position that patients are always right; the customer is always right. I ask the patient if I'm right, if my interpretation of the behavior is right. The Interactive Model is something I modified from Marjorie Guthrie. Some of you may remember from an early CCHD publication of three intersecting circles of schizophrenia, manic depressive illness, and HD. What I've divided the interactive model of HD into are interacting elements of personality, social changes, changes in anatomy, changes in physiology, physical illness that HD patients have, all interacting with the HD person, to produce a complex array of symptoms and behavior patterns with personality changes in exaggeration of the worst of premorbid traits.

I keep telling HD families that if you want to be nice when you get HD, you'd better get nice now! Because if you're nasty now, you'll be double nasty when you're old or when you get HD. Either way, you're going to get nasty. Whether you get HD or not, it's good to be nice now and nice later. Otherwise, your family will desert you.


Personality change also involves loss of control. There is anxiety in losing control. Primary gratification is another issue. Because of personality change as a result of anatomical change, HD persons are the kind of people who want something 'not now but yesterday. ' You remember the days of the anti-Vietnam marches when people shouted slogans like, "What do you want?" "XYZ!" "When do you want it?" "Now!" That's called primary gratification. HD patients don't want it now; they want it yesterday. And you can understand that. These changes interact with a whole lot of social changes of losses. They've lost their role in life. They've lost their usefulness; at least they feel so. They've lost control over their environment. And also there is loss of autonomy over everything.

All these changes have their basis in loss of neurons: the caudate nucleus,
frontal lobe, and cortex generally. This leads to the loss of neurotransmitters and other unknown substances which combine to produce a lot of personality changes. And if that's not bad enough, frequently they've got physical concurrent illnesses -- pneumonia, for instance. This aggravates the whole situation further. All of that produces a very complex array of changes of behavior in an individual.

Now, interestingly enough, this is the same diagram I've used for teaching
psycho geriatrics. Only the different parts change. So it's no different.
What I wanted to say to you is that HD is a disease of the brain. It has a
lot of commonality with other diseases of the brain in the elderly and in
other people.


The next one is the Ripple Effect. This is strictly from Marjorie Guthrie.  You must understand that the effects of HD are not just on the individual.  It involves other people in the family and in the community. If you imagine the HD person is a stone to throw into a still pond, the ripple effect affects the nuclear family, extended family, neighbors, co-workers -- at the time the person's still employed -- carers -- family or professional carers -- friends, and the ripple goes on. Employers, schools, the media, politicians, community, bureaucracy, and churches.

It's interesting that the two major HD care centers in the world -- the one
in Melbourne and the one on Fifth Avenue here -- involve churches. The
Melbourne one is the United Church of Australia and the one here is the
Catholic Archdiocese of New York. When Marjorie taught me about this ripple
effect, little did she realize the influence of the churches and how the effect works in both directions. You can see that the Huntington's person is not a person alone. HD affects both positively and negatively all the people in the community.

We tend to think of negative effects, but there are a lot of positive effects as well in interacting with HD people. For instance. I'm one of the carers who've been 'adopted' into HD families. Nancy Wexler is a genuine daughter of an HD family while I'm only an adopted son. So she has a little precedence over me in the family hierarchy. But we see ourselves dragged into the situation in a positive manner. This ripple effect is VERY important to understand when we talk about behavioral changes in HD and its effect on individuals.

THE MELBOURNE CHARACTERISTICS OF CARE

In the Melbourne caring situation, quality of life is preeminent. I keep repeating that until it really sinks in to everybody's head.

Risk-taking Policy; The risk-taking policy is talking about enabling an HD person to take the highest risk to maintain quality of life. I believe, unless we can take risks with Huntington's persons, we're not giving that person the quality of life in the sense of control and autonomy he or she has a right to; not deserved but has a right to. We adopted a risk-taking policy right from the beginning. We'll take risks with family and individuals. For instance -- this might be very controversial in this context -- none of our patients in Melbourne has any form of tube in his or her stomach. It's not an option. It's not considered. We will only do that if that person has a gastric esophageal lesion which in any other medical situation requires a gastroscopy or gastric tube. It's not for
Huntington's Disease but for a genuine gastroenterological condition.

We will take the risk of patients choking and they know it. We will do all we can to prevent choking. It's a labor-intensive area of helping the patient to eat. We use all kinds of strategies: occupational therapy, use of Valium, all sorts of physical strategies combined to help that person to eat normally, without tubes. In ten years, we've only had six incidents of inhalation pneumonia. No patient so far has died of inhalation pneumonia.  Now that's risk-taking.

Any care center or nursing home here that will do that has to consult a battalion of lawyers. I'm not sure what you're going to do about it. You live in your society and I live in mine. This is your society and you'll have to deal with it. You may have to consult a battalion of lawyers on that one. But I feel very sorry for professional carers who are in nursing homes and care centers. What are they going to do if the family doesn't want to have any tubes? What are their legal liabilities if they follow your suggestions and something happens to the patient? You only need one in a hundred patients that it happens to and that institution is down the drain. You press suit. What about the other family members? What's going to happen? So I leave it to you to think this one over. I have no solutions. I'm just telling you that in Australia, we hold dear a high risk-taking policy.


We let them walk as much as possible. If they fall, we pick them up and get
them walking again. If they fracture a bone, that's the risk we have to take. We haven't had too many fractures so far. Lots of bruises but not so many broken bones. We accept the bruises for the price you have to pay for autonomy and independence. That's the policy we have. Whether you'll have it in the United States, we'll have to wait and see.

We also have a policy of not scapegoating HD. In other words, not blaming the HD patient for a lot of things like behavioral problems. We also do not blame HD for everything and anything happening to the individual. Too often, any illness or changes in life, 'it's the HD doing it. ' We say, "is there any other explanation? Would it happen if that individual hasn't got HD?" And frequently we find that that's the explanation. If the HD person has a chest pain, it's not HD at all. We have to do an EKG. Or maybe the patient has gastric ulcers. But too often people blame every medical condition on HD. We don't do that. We don't scapegoat the disease nor do we scapegoat the person.

In terms of behavioral change, we will say, "Okay, it's not the person who's bad. It's something happening in the brain." We accept the behavior for what it is. We don't put a value judgment on the behavior.  I also have a matter of policy that we keep telling the HD person, "You're not sick; you've only got HD." That is to avoid the difficulty of a 'sick-role' adoption. We do not want HD persons to say, "I'm a sick person." Rather, "I'm a person afflicted with HD, but I'm not sick."  Because the corollary of that is the person can control his or her own behavior. If he or she says, "I'm sick," then the responsibility will be
passed on to the physician or nurses. I will not allow them to pass the buck. That person must accept the behavior the way I accept the behavior.

I talk about multi-disciplinary and cross-disciplinary operations. We also have a very strong advocacy role. We're able to do better for HD persons as much as we're given the power to do so. We take it as far as we can within a democratic constitution. All Huntington's families should take on advocacy on behalf of family members and on behalf of families at large.


To quickly go on, we try to understand the behavior in more detail. I would
remind you that HD is a disease of brain cells. A lot of people forget that. In Australia, HD is cared for by psychiatrists, whereas in the United States it's cared for by neurologists. This is just an historical thing that happened. In Australia, the last generation of neurologists made the diagnosis and then passed the patient on to the psychiatrist and then the mental hospital. Here your neurologist does everything. Psychiatrists, until recently, did not like touching patients because they're trained to do psychoanalysis and psychotherapy. And they sit there and listen very carefully. "Um, ah, yes, no." Recently, they found that there's a human being there with all kinds of organs and neurotransmitters and enzymes.  There's hope for U.S. psychiatry yet!

We psychiatrists in Australia 'got lumbered' with the task and we said, "look, we are doctors first and we happen to be psychiatrists. What the hell? We don't mind." And because of acceptance of caring for that problem, a lot of people say, "Ah, it's a psychiatric problem - he must be treated by a 'shrink." People in Australia tend to think of HD as a psychiatric problem, rather than a brain cell problem. And I keep saying to my students that this is a disease of brain cells. The brain cells die gradually.

Professor Dom in Düsseldorf counted the rate of cell death - six cells per second. From onset to death a Huntington's person loses six cells per second. We've got billions of them, so we can afford to lose six cells per second for a little while. When an HD person dies, there's still billions of brain cells unused. And that's the part I want to tap into when I say ability development is important. Let's get on to those parts of brains not dying and do something with that part of the brain.

Brain cells from the caudate nucleus and cerebral cortex are the brain cells that die. And the six cells that die per second are from those areas. At the present, we don't know what causes cells to die. But once Dr. Gusella in Boston and other people like Dr. Michael Hayden in Vancouver and elsewhere find the gene, then we may be able to tell you how the brain cells die.


Now, what does the caudate nucleus do? The caudate nucleus controls
movements. The cerebral cortex controls the major high intellectual, emotional, abstract, motor, sensory and complex human functions. We are what we are because our brain has developed in a way in which we have a large cortex and the front part of the brain which differentiates us as human beings - the highest order of animals, from the lowest order. You know the saying 'bird brain. Birds have very small frontal lobes. Sheep don't think. They just follow the leader. Sheep also have very small frontal lobes. We've got a huge one.

So our cortex, our frontal lobe and the rest of the brain control all these complex functions. And when the cells die, these complex functions begin to deteriorate. What are the frontal lobe and cerebral cortex responsible for?  Some things that they control are emotion, judgment, planning, organization, social inhibitions, abstract thinking, encoding of memory, problem solving, logical thinking.

Starting to ring a bell for those of you who care for HD persons? Let's look at another condition and this is not my slide. This is one I borrowed last week from Professor Gustafson who presented a paper on Frontal Temporal Atrophy in a group of patients he followed for twenty years in Sweden.  Picks Disease is the general term; there is another condition which is not  Picks Disease but it's a frontal temporal atrophy. The front and side part of the brain die. The reason is unknown; the symptoms are slow progression, change of personality, lack of judgment, euphoria, disinhibition, agitation, emotional unconcern and inertia, depressive episodes, suicidal ideation, reduced speech and phrases, fainting attacks, epileptic seizures, etc. Some of these things are common with HD and some are not. But the change of personality and cognitive changes are very similar.

So, my hypothesis -- and I can't prove it at this stage, it's a clinical observation waiting to be proven -- is that behavioral changes in HD are the result of death of brain cells leading to altered personality functions and should be viewed as part of the disability suffered by the patient who should not be held totally 'culpable' for such changes.

I'll give you an example. In my practice of psychogeriatrics, I see a lot of patients who suffer from psychiatric problems after a stroke. Usually such patients are referred to me as "behavioral disorder; please assess and advise treatment." After a stroke, they usually have paralysis and behavioral presentation that reminds me a lot of HD patients.

When a person has a stroke, that part of the brain bleeds, cells die, and frequently a lot of swelling occurs in that area. And the swelling effects that area in front responsible for movement. And when that's affected, the patient has these behavioral changes. And when the swelling settles down, some of these behavioral changes will settle down.

Sometimes it annoys me that the nursing staff will have no difficulty coping with patient's paralysis. If the patient can't use the right arm, you never hear them say, "You naughty boy, why don't you use your right arm?" No one dares say that. They'd be sacked. But if the patient has behavioral symptoms, "Naughty, naughty, naughty."

If you explain to nursing staff and others, "Hey, this is part of the stroke," they say, "Oh, no problem." We accept it. Same behavior but different level of acceptance, different level of understanding. HD patients should not be held totally culpable, because the brain cells have died.  Some of these behaviors are brain-cell based.

Let's take another approach. Don't say, "Naughty, naughty, naughty" or "Bad boy, bad girl." Let's say, "Alright, the brain cells have died. I don't like the behavior but let's put it in the context of understanding. Part of the brain cells have died."

So, what are the behavioral consequences of cortical cell death in HD? I mentioned personality changes. Some of the symptoms are well-known to you
for those of you who are carers: irritability, intolerance, childish regressions, inappropriate social interaction, usually in a public situation. Behavior which we call in Australia, a real 'bastard. . You become a bastard when you have HD or Alzheimer's. It has three meanings.  It can mean you're a nasty piece of work or it can mean you're a great friend of mine. Australia has a very interesting language. HD persons can be very stubborn people, and egocentric -- "Me first, me last, and me in between." They're self-absorbed. The world ends with whatever's around them. They're also very demanding. These are the kinds of behaviors that we see in any patient who has cortical-cell death of the frontal lobe.

Emotional changes: They cry one moment and laugh the next. Their emotions just don't match what's around them. They're superficial. They're very shallow. You can't relate to them deeply. They're disinhibited, which adds to the social inappropriate problems. They're very facile and very childish. They also have impaired judgment. They can't judge what is socially acceptable behavior. They can't judge what's appropriate to do at that time. They're frequently disorganized. They can't concentrate and finish anything. They keep moving around and changing things. They can't attempt a conversation for more than a short period of time. They want to go on to something else. They can't plan -- they're incapable of planning ahead. They demand primary gratification.

One of the questions asked by our nursing staff is why HD persons always want things now. "If I'm attending to someone else, what am I to do?" A common experience. This is part of the brain change. Inhibition and social control is impaired. Frequently they have no drive. They're withdrawn and depressed and usually referred to as 'lazy.' But they're not lazy - they just haven't got the drive, the self-starter is not working. So these are the behavioral consequences of cortical cell death in HD and in other frontal lobe diseases. It's not peculiar to HD. Let's look at the behavior of HD this way: that before the disease, you have a whole lot of personality, behavior, interactional relationships that existed long before HD comes. Cell death leads to impaired communication function and also personality changes. In addition, you have altered environment and altered relationships as a result of the disease. The social environment changes and relationships change. The person is no longer in control and is now controlled by other people. Autonomy has changed. All has changed. The enhanced behaviors may be the behaviors that are not so nice.

There are a lot of behaviors still maintained. When you cut away all the
behaviors, inside there's still the core of a very nice person. That's why
I stuck to HD. It's because I think HD persons are basically very nice
people, despite the fact that they have these enhanced, exaggerated
behaviors. There's a lot of released behavior which wasn't there before.
But because of brain cell death, they've now got this new behavior released
from control by the frontal lobes. When the frontal lobe cells die, all
these inappropriate social things come up which are no longer under the
control of the frontal lobe.

Despite the combination of enhanced 'bad' behavior and the released 'bad'
behavior, if you take away that shell, there's still a very nice individual.
We have to continue to look for the maintained behaviors which are
appropriate and nice to work with.

It is very difficult to look beyond that. Maybe I'm too optimistic or naive but after 18 years, I can honestly say that there isn't any HD patient of mine that isn't, underneath it all, a nice person. So, what do we mean by 'behavior problems'? What is the problem? Who is the problem -- the patient or me? Frequently, when I'm asked to see HD patients or psychogeriatric patients, the problem's not really with the patient. The problem's the carer, and I treat the carer before I treat the patient. So who needs the treatment -- the patient or the carer? I'm not saying that the carer is at fault. Frequently the carer wants and needs treatment as much or more than the patient. And who's asking for help?

When the nurse rings me from the ward and says, "Dr. Chiu, such and such is
behaving badly," I say to myself, "Well, who wants the help? Does she? Or the patient?" What's the expected outcome? What do we expect the HD patient to be? And who wants that outcome? Me? You? Who is it? We must examine what's going on. Who wants the outcome? What's the outcome they want? What are the solutions? Are there any solutions? What kind of person do we want the HD person to be? Do we want that person to be like he or she was fifteen years ago? And I would think: that we'd want the HD person to be the best person he or she can be at that stage of the disease.  Not what the person was 15 or 20 years ago.  I had a patient who died about six months ago who was a gorgeous girl when she was young. Her photograph album made me feel I should be 20 or 30 years younger. She was one of the chorus girls in our most famous musical troupe.  When she got HD she was, to quote journalistic jargon, "a miserable shell of her former self." But if I keep thinking of her as that chorus girl, of course I'm going to be disappointed. I have to teach myself that I have to treat her as the kind of person she is now: in the late stages of HD. She still loved music. She had the TV on when musicals were on, and she listened on the radio to the songs she sang in the chorus line. That's the kind of person she is. And not the person who was a beautiful girl. I will work with her on that level. And I expect her to behave at that level, so let's not go back over these years and carry those old messages around.

What kind of person do we want the HD person to be now? And what kind of
person do we want ourselves to be in relationship to that person? When we talk about behavioral problems, what kind of person do we want ourselves to be? By handling the problem in a way that can be helpful, am I able to make myself a better person as a result? It sounds like I'm preaching a sermon, doesn't it? I'm not. Because I spent 18 years working with HD patients, I know I'm a better person for it. Not because I've done anything, but because they taught me to be a better person. I believe I'm a better person for looking after my Huntington's patients and my psychogeriatric patients.  I learn from them. They teach me.  When we talk about behavioral problems, we may say some of those behavioral problems can have a very positive value on the kind of person we want ourselves to be. I'm not saying if we have problems we should not run away from them. Maybe the thing to do to be a better person is walk away from it, to let someone else handle it. I know people who do that. Families have said, "Dr. Chin, take this patient for a little while while I go and recharge my batteries." I say, "Okay, don't feel guilty. That's okay. If that helps you to be a better person, that's fine. At the same time. I'm a better person if you've passed the patient onto me. So don't lose any sleep over it."

We have to look at these questions. When we say behavioral problems, really, is there a problem? If so, whose problem is it? The next question is what really disturbs you about HP? Have you ever asked yourself that question? They can't remember and they can't talk to you. At least they can't talk back to you and sometimes that's good. The mobility problems and behavioral changes, loss of weight, loss of social graces -- you can't take the person out to expensive Fifth Avenue restaurants -- you have to worry about that.

We had a male nurse, who was at Preston Centre for a few years who was a
member of the Royal Melbourne Yacht Club. This is quite a prestigious club.  And he would take one of our patients for Sunday brunch at the Royal Melbourne Yacht Club. And despite the fact that this patient could mess up the beautiful tablecloth and so on -- the Yacht Club didn't mind. They recognized that this was a patient with a disability and the member was kind and gracious enough to invite this lady to lunch at a yacht club, so it was okay.

A lot of people worry about social graces but it's really not a real problem. We think it's a problem. Look, if you think it's a problem taking HD persons out to dinner, take them to a Chinese restaurant. They won't mind. You can make as much mess as you like and they love it! One thing they hate when you eat in a Chinese restaurant is if you leave food untouched and the tablecloth is spotless. You have not enjoyed your food.  I think Italian restaurants don't mind, either. Make a mess. Go ahead!

Change of normal relationships? That's a tough one. That disturbs people.  But on the other hand, life is full of changes in normal relationships. Who was it that said the only certain things in life are death and taxes?  Nothing is certain. Our relationships change all the time. We have to roll with the punches. And out of changes, we get to be better people.

Appearance? What about appearance? I've been asked in Australia to organize a symposium for 40 people in November next year in Canaberra to talk about Environment and the Aged. I was speaking last week in Tokyo with a colleague from the World Health Organization in Geneva, inviting him to this conference. He said, "Oh I can come, if my time allows me. By the way, WHO wants to do something about that subject in 1991 so it's going to be very appropriate for me to come. I think you should ask a cosmetician to come." What a brilliant idea! I've never thought about that, but I think we should. Helena Rubenstein for the ladies and Calvin Klein for the blokes.

We look at appearances. Social stigma? Yes, it's a problem. It disturbs me. But why does it disturb us? We ask these questions. If those things disturb us, why? Is it behavior, is it me, is it society? Having asked those questions, the next questions is, what can we do about it? And the answer should never be, "There's nothing we can do about it." There's always something we can do about it. A lot of the strategy I have for helping Huntington's patients does not come from between my ears. It comes from the patients and the families. I'm only a conduit to transmit them to everybody else.

Way back in the beginning when microwave ovens first came out in Australia, a patient's husband taught me about the use of microwave ovens in caring for HD persons. He was a lighthouse keeper and lived away from the lighthouse.  He had to drive to the lighthouse and couldn't return to serve his wife her lunch. After a few years of cold sandwiches in winter, he said, "Look, I don't want to eat cold sandwiches, why should my wife be eating cold sandwiches?" And he says, "I can't ask her to cook things because she'll burn herself." As he was looking through catalogs, he saw a microwave oven.  So he purchased a microwave oven, and he discovered it could make a hot lunch for his wife every day in winter. Not only that, she could make herself a cup of coffee or tea using the microwave oven, without scalding herself boiling a kettle.

Another thing taught to me by another patient is that dental care is a problem. Agree? After a while a patient said to me, "Dr. Chin, what do you think of the idea of electric tooth- brushes?" You can hold an electric toothbrush and you can move your head any way you like. With a toothbrush, you have to move it where your teeth are. Such things are often taught us by the patients.

So, through all these disturbing things, you can learn a lot of things about the person. You should share it with us. Then we can share it with each other. When it's a behavioral problem, we should not get too concerned about some of the behavior in a totally negative manner. We should look at these so-called behavioral problems in a more positive manner as they contribute to your own welfare, to your loved ones, and also to people elsewhere.

Just one more thing for those of you who have to deal with family physicians. I want to show you this diagram which is sometimes life-saving.  This is one I use to teach medical students about Huntington's Disease and psychogeriatrics. The natural history of the disease is this slide ( Error!  Bookmark not defined. ). When an HD person develops "behavioral problems" suddenly thus, then it's not HD.  That is not 'dementia.' That is something else. The causes are these five:

1) Infection -- chest infection, urinary track infection, and other kinds of infection. And patients with HD do not often show high temperatures. They may express in terms of sudden behavioral change.

2) Iatrogenic causes -- pills that we doctors prescribe. They're a very important cause of the sudden confusion and behavioral change.

3) Subdural bleeding -- bleeding in the brain. Often they fall and hit their heads. If it does not show immediately, it may take a few years to show.  It's what we call subdural hygroma. I have a series of six Huntington's patients in Melbourne who have this problem. I sent one patient to general hospital saying, "Please investigate; I think there's something wrong with the brain of the patient in addition to the Huntington's Disease." Some of my colleagues examined this patient from top to toe, right through. I took her to the neurologist and they said, "We can't find any- thing wrong with this patient." I said, "Look, I've known this patient for many years and I notice that the right arm is a little bit weaker than the left arm. Do a CAT scan, keep her there till tomorrow morning, and if the CAT scan is clear, send her back to me." So my colleague said, "Fine, anything to do you a favor." They sent the patient for a CAT scan the next morning. She went straight from the radiology to the operating theater for evacuation subdural hygroma.  Since that time, whenever I send a patient and say, "There's something wrong with the brain," they do not discharge this person unless thorough tests are done. I've had six of them. The latest one died two months ago of subdural bleeding. The other five survived. I'm very aware of the fact that subdural bleeding can be a cause of sudden behavioral change which is not consistent with the way of HD's development.

4) Depression can be a very important cause of behavior change. When we're
depressed, it's very difficult to behave normally. I know I don't. I treat depressed patients in psychiatry all the time and recognize depression can be a cause of a sudden change in the behavior of a person with HD.

5) Common illness: the Huntington's patient is not exempt from heart attacks, strokes, gastric ulcers, thyroid disease, and kidney disease. They can all have it. And frequently the disease. shows itself not in the traditional signs that these diseases show themselves but in sudden behavioral changes quite out of pattern with the usual course of the condition.

As you can see, to understand behavioral change in HD, we encompass not only the brain changes. The other reasons for the behavioral change are the
psychological, social, and medical reasons. The patient may not even be the
real cause of the problem. I'm trying to understand to not really blame the patient and say, "Look, it's all your fault", because sometimes it's not your fault. It's not anybody's fault. It's the way it is. And we should try to understand what's going on and seek a solution that is good for the patient and good for ourselves at the same time. * * *
 


Transcript of Dr. Edmond Chiu Video
Senior Lecturer in Psychiatry,
University of Melbourne, Australia


[I make an assumption here that this videotape was done at the same time Dr. Chiu also gave his speech: Understanding Behavioral Changes In Huntington’s Disease at the New York University Medical Center on September 12, 1989. This first speech has long been one of my favorites and is now joined by this one! The video was done at the Cardinal Cooke Center in New York. If anyone knows the exact date, who did the videotaping, who was on the credits, or any other pertinent information regarding this video, I would be happy to add it to this transcript if you e-mail it to me. Unfortunately, not much is known about it right now. Special thanks go to Bob and Ruth Lentner from Michigan, for preserving and practicing what’s contained in this treasure! Transcript prepared by Phillip Hardt  phardt1@cox.net]

Transcript:

[Kids are shown singing Happy Birthday to their grandmother, Nanny. Dr. Chiu wheels in their grandfather and encourages him to throw birthday balloons back-and-forth to his grandchildren.]

[The commentator says:] That was Nanna’s husband in the wheelchair. He has Huntington’s Disease. I’m Bob Kostas. I wanna tell you that there was a time when Nanna’s birthday would have been celebrated without grandpa because you never know how a Huntington’s Disease person is going to behave. What they might do to disrupt the festivities and maybe even scare the children. Too many grandpas have been written out of too many family scenes needlessly. Too many times love is even turned into fear as family members try to deal with the effects of this strange disease on a sibling, a spouse or parent.

We hope and pray for a cure but there is no cure for Huntington’s Disease, not yet. Good people are working on it of course. In the meantime, other good people are working on the only alternative available- care. The kind of care that doesn’t abandon the Huntington’s Disease person. The kind of care that tries to make full use of every single brain cell the Huntington’s Disease person can command. The kind of care that understands occasional erratic behavior. The kind of care that includes a grandfather in the happy family event we just saw. The kind of care that has been pioneered by one very sensitive and very practical person. The Hong Kong-born Assistant Professor of Psychiatry at the University of Melbourne, Australia, Dr. Edmond Chiu. For almost 20 years Dr. Chiu has been spreading the realistic approach that in the absence of a cure for Huntington’s Disease, all we can do is care, and care a lot- care about and care for. He’d much prefer that we could do something about the “quantity” of life for Huntington’s Disease people, but for now he accepts the fact that we can’t, and he asks us to accept that fact too. He also urges us to realize that through care we can improve the quality of life for the Huntington’s Disease person. Here he is to share his ideas with us, Dr. Edmond Chiu.

[Dr. Edmond Chiu says:] Let’s just spend a few moments talking about Huntington’s Disease. Over the last few years a lot of knowledge has been gained about the condition but it is necessary at this stage to address the given attitude towards the condition which needs to be changed. It is no easy matter to change attitude. Knowledge itself is not enough if we want to provide the best quality of care for our Huntington People. Unless we have the right attitude then all the knowledge in the world will not give them a better quality of life.

For instance. In our medical textbooks we have frequent reference to Huntington’s person having high level of promiscuity, high level of crime, high level of psychopathology and psychiatric conditions. All that is rubbish! Apart from the high level of depression, all the other things do not exist and unless we change our attitude with the knowledge say therefore our people do not have these things and deserve a better quality of life it will not happen.

Another common myth is that Huntington’s person cannon learn new skills and Huntington Person frequently are unable to control their behavior. That is not the case. Despite the death of brain cells they are still able to take responsibility and take control of their lives and be given autonomy. In understanding how Huntington’s person behaves we have to remember that that person is the interaction of a multitude of factors. These factors include the death of brain cells which result in abnormal movement and change in intellectual function. At the same time with the death of those brain cells the chemistry changes and the brain no longer functions the way it used to.

With also Huntington’s Disease there is also a lot of sociological changes that accompany it. They loose their job. They loose their identity as contributing members of society. The relationship with spouses and children or neighbors change because they become a more dependent person, they require more care. The personality changes that accompany brain cell change also interact with that.


In addition, they are entitled, and do have a variety of physical illnesses that attack this person. Therefore we must constantly be reminded that what happens to Huntington’s Disease person is not always just Huntington’s Disease- it’s other things interacting with that individual.

Frequently we have to think of Huntington’s Disease person as a pebble dropped into the pond of peaceful society. It’s ripples affect immediate family, extended family, friends, fellow workers, employers, employees, neighbors, even politicians, bureaucrats and churches. Therefore, that person is not alone and the fate of Huntington’s Disease community is the fate of society at large. And in helping a Huntington’s Disease person we have to remember that we have to deal not only with the person, but we deal with society at large.

Frequently I am asked: “How do you handle a Huntington’s Disease person with behavioral changes?” The first principle I like to enunciate is that irrespective of how difficult and how abnormal or how stressful the behavior is on us we accept the behavior and accept the person. We work with the behavior. We do things to change the behavior but, we accept the person in whom the behavior expressed. Huntington’s Disease person is disabled, no doubt about that. He has an illness. He has a disease. But, I do not want to think of Huntington’s Disease person as a sick person. That is, we do not permit the Huntington’s Disease person to hide behind a sick role and be dependent upon us and upon other carers. One of my favorite sayings is that, to a Huntington’s Disease person, is that, ‘You are not suck, you’ve only got HD.’

Now, there are also other myths about Huntington’s Disease. People think Huntington’s Disease person is demented, like say, for instance, dementia of Alzheimer’s type. I do not believe that Huntington’s Disease patients are demented, or suffer from dementia like Alzheimer’s Disease. Huntington’s Disease patients remain oriented through their lives. They retain insight, they are alert, and one of the tragedies of their condition is that they have insight. They know what’s happening to them and they know that they’re loosing the physical ability, loosing the intellectual ability and that their children are at risk for the condition. Sometimes I wish to God that they do no have insight and be more blissfully unaware.

I did mention the Huntington’s Disease person can learn. They can learn by repetitive trials. We must not treat them as someone who is not capable of learning. The mistake we frequently make is we do not give them enough time and patience to learn new skills. Given time and patience they will learn new skills and surprise us. I have one patient who learned pottery after he got HD and he presents me with a piece of his own pottery every Christmas as a present. He has taught me that Huntington’s Disease person can learn. They retain special interests. For instance, if their interest is particularly a ball game they will follow that team loyally and will follow the progress or failure of that team. If we concentrate on that kind of interest and concentrate on newly developed interests I believe that they can have a very satisfying intellectual life. We’re to encourage the learning process by targeting those areas of the special interest and restricting the field of learning.

Huntington’s patients sometimes have difficulty making choices between more than two things but if we target it and summarize it for them so they can concentrate their brain power onto just those things that can be learned and can be learned with repetitive trials then we will be able to be surprised by the capacity to learn.

Some of the physiological changes we need to address that is the result of the loss of cells from the front part of the brain, the frontal lobes. As a result of that they are unable to make, what we call sensible judgments. They have difficulty organizing their lives. They have difficulty planning things. Any activities requiring what we call sequencing have a problem- the activity to go from A to B to C to D and the down to Z. They sometime skip steps. Sometimes they forget steps and we may have to help them in organizational behavior.

Social control and social nuances may be affected. They’re not so nice person when you take them out to dinner. However, if we accept that that is part of the brain change and then give them a life that includes some of those social functions, accepting the behavior, good quality of life can be had.

While we’re talking about dinner, we will remember the Huntington’s Disease person requires twice the calories that we do. They are always hungry. When they are hungry give them food.

A lot of people do not believe the Huntington’s Disease person can eat by mouth. There is a tradition, in this country, that the Huntington’s Disease person is given gastronomy tube. In Australia and in Lake Maricabo, gastronomy tubes are definitely not options. Venezuela Huntington’s Disease populations as with Australian, eat by mouth up to the day they die because we believe they can swallow, and they enjoy the pleasure of eating. They enjoy the social life that surrounds a meal. It’s not much fun and not much dignity to be fed by a machine pumping fluid into your stomach through a tube. We have demonstrated in 10 years of looking after long term care patients in our facility that we do not have a large amount of inhalation pneumonia. We have only six case of inhalation pneumonia in ten years and none of them have died. They have managed to regain the ability to swallow, to eat, to derive pleasure from eating.

We put quality of life as the main criteria for decision making regarding their lives. What makes for quality of life is worth doing. What detracts from quality of life in not worth doing. For instance, we had to make a very difficult decision about smoking. I am an antismoker. I don’t like smoking around me. However, when we had to make the decision whether our people is allowed to smoke it came down to the point they say ‘Smoking is one of the last pleasures we have.” And at the same time we discovered, by our physical therapist, that the Huntington’s Disease patient who smokes, in fact, have better lung function because every time they inhale and exhale they in fact do physical therapy. So the decision was made to let them smoke but we changed their environment so that they’re in a safe environment so that they don’t get burnt and the facility doesn’t get burnt.


Huntington’s patients have difficulty because of the movements, in dealing with buttons and zips. But given comfortable clothing they are able to handle their dressing. Which brings me to the point- that we need to deal very much more with the environmental health of our patients. In other words, its up to our ingenuity to make their environment to suit them, rather than to make them suit our environment. Environment for Huntington’s patient is very important and I’d just like to point out a few things about architectural design as part of a patients environment.

[Dr. Chiu is now in an Phd’s (Person with HD) bedroom.] The first thing you notice is that the bed is empty. That’s very important that during the day time the patient should not be in bed, unless it’s absolutely necessary, but should be up and out about doing normal things as much as possible. So the empty bed during the day time is an essential feature of care of Huntington’s persons. [Looking at the nightstand between the two beds he says:] Some of the more, I suppose, negative aspects of architectural design are prevalent in a lot of nursing homes and facilities and needs to be addressed. Such as, there are sharp corners in this bedside cupboard which will the injure the Huntington’s patient due to their movements. A better design would be to eliminate such bedside cupboard and put up behind the bed head a large mantle piece where personal belongings, photographs, nick knacks, and memorabilia will be placed to remind the patient of his or her identity. The drawers and doors will be behind the bed so injury is minimized. [Dr. Chiu is now in the bathroom.] Over here we have a basin that has two controls for hot and cold temperature. It would be much easier if the Huntington’s patient only had to worry about one control in controlling the temperature and we add to this a thermostatic control device so they don’t scald themselves. Then they don’t have the anxiety of burning themselves and be able to control the water temperature more successfully. One good feature in this bathroom is that the patient’s personal items on the shelf are clearly visible so the patient has this personalized feeling about the bathroom. As far as the toilet is concerned, the standard design of toilets in facilities are always a disaster both for the patient as well as for the poor nurses who look after for them. For example, it’s very difficult for a nurse, or two nurses to go on either side of this bowl to assist the patient in coming up or going down. In designing, there needs to be maximum space on both sides, not just one side of the patient to assistance on both sides. There are facility available that you can bring down here, grab bars, that come from the back of the wall to the front to give patients a grab and a lift and a push. [Looking at the grab bar mounted on the wall he says:] These side bars are not always appropriate if you want to give maximum space to the patient and to the nurses who care for the patients.

[Dr. Chiu is now back in his swivel chair.] One of the major issues in Huntington’s Disease is eating as I’ve mentioned before. In order to enhance eating we have to attend to their dental health. People frequently forget that there is no pleasure eating if your teeth hurt. Therefore one of the earliest things I instruct my Huntington’s patient to do, after seeing me, is to make the next appointment with the dentist, not with me, to get their teeth in good order and don’t allow any teeth to be extracted unless it is absolutely necessary. Dental health is very important. Frequently we forget about that.

Another very important issue with our people, is that for the female patients, so often their gynecological health has not been attended to. Because they’ve got Huntington’s Disease people tend to forget that they are woman as well.

Huntington’s patients need to be encouraged to stay mobile and active as long as possible. They need to be fit. We have workouts. They should have workouts too. But people say- We cannot improve their function. That is not the issue. The issue is quality of life. They need to be given the ability to have exercises so they will feel fitter and psychologically better.

Their intellectual needs to be addressed. Maybe they cannot read as well as they ought to, or as they used to but frequently we encourage them to read, to watch television, listen to the radio, and in particular, musical appreciation. Our experience is that the Huntington’s patient retains the ability to appreciate music right to the end. It may be that the part of the brain controlling music ability and music appreciation is not affected by Huntington’s Disease. We don’t know where that part of the brain is, but where ever it is they appreciate music. That is a very important part of their quality of life.

They need companionship. They need new faces around them all the time. We need to bring in volunteers. We need to bring in all sorts of people. We need to take the Huntington’s patient into the community to share the life of the community. Never mind the stigma. Never mind the funny looks. They need to deal with the world at large and receive that kind of relationship and that kind of social life that everybody has.

Their spiritual life needs to be attended too. They need to be able to deal with the potential and the reality of their own death, and deal with it not only in a psychological manner, but in a social manner and in a spiritual manner.

I also want to address the issue of the Huntington’s Disease patient who is in “care.” The family attitude towards the person is important. Frequently families have difficulty letting go of the patient. They have had the patient for so long, they have cared for them so long they have become intermeshed in the relationship with the patient. We need to let the patient go. We need to delegate our caring responsibilities to the professional carers- not allow them to be our substitute because this means you hand over everything to your substitute. But delegate, so that you yourself take responsibility and share the responsibility with the professional carers. That also means that you don’t scapegoat, you do not compete with them, but be patient with your delegates to care for them. You will have also to help them deal with some of the problems which are new to them. The doctors and nurses and therapists are just beginning their journey in caring for the Huntington’s Disease person. Whereas the family members have 20, 30, 40 years of experience. You need to pass this experience on to the professional carers.

We need to help each other in caring. We need to give each other the ideas. Frequently, the junior nurses are reluctant to come up with some ideas in case it sounds crazy. Sometimes the craziest ideas become good ideas. We need to be open and say. Hey, there is another way of dealing with this problem. There are other ways that come from common sense, not necessarily from textbooks. The person with the highest education may not necessarily be the person to come up with the best ideas to provide a better quality of life for our people. For what you’ve learned you share with each other. So, a lot of things I have learned, I learned from the patient, I’ve learned from the families, I’ve learned from other people who have taken care of the patient in an institutional setting, or in a community setting.

Sometimes I’m asked the question: “At what stage of the condition should a Huntington’s Disease person be admitted into residential care?” I don’t have any stages. I do not follow the defined five stages as a decision for admission to residential care. The decision is based on the need of the individual, the need of the family, and the final criteria- if this person comes into residential care am I giving him or her a better quality of life, irrespective of the stage of the disease. If the answer is yes then that person shall be admitted. If the answer is no, no matter how late stage that person might be, I don’t think it’s proper to inflict upon that individual a worst quality of life in a residential facility. So I go back to the same criteria again- quality of life!

[Phds are shown playing Yahtzee and walking. Someone is playing the piano in the background. Next we see Dr. Chiu walking into the chapel. He says:] To sustain the spiritual life of our Huntington’s Disease persons is very important because this is where they will find strength to deal with their disabilities and also to assist the family in handling this very difficult situation. In such a facility as the Cooke Center, we have a multi-faith chapel to attend to those needs. It not only serves the needs of the Catholic population, the Protestant population, but also our Jewish brethren who develop Huntington’s Disease. In this way we can help our people to maintain peace and joy in their life in spite of Huntington’s Disease.

We need to give our patients back their right to determine what they want for themselves. Now, sometimes that is only lip service. However, we need to take this seriously. We need to work with our people to say: Is this what you want for your quality of life? Take for example, in Melbourne we have what we call a high risk-taking policy. We negotiate with our patients and say: Do you want to eat by mouth? Do you want to walk by yourself? And we negotiate if they deteriorate and we will say: Okay, at this stage, do you want now to receive some kind of assistance? Of course we do a lot of persuading when we feel that any future expression of risk taking may provide some damage. That’s why we insist there are no tubes because we want them to have the pleasure of eating and take what of the risk that is consistent with safety. And, they have taught us that it is possible. However, in this country of United States, it is very difficult to do that with out confronting some regulations. We must work creatively to get around or change those regulations. We must not be bound down by regulatory restraints so that our patients can have a better quality of life. We must not allow the bureaucrats to dictate to our patients and make them more dependant than what they are. We may have to be advocates on their behalf to change the regulations. We Australians are rather irreverent the way we approach our authority figures. And I would say if he bureaucrat is wrong you change them. We must learn from our people how they want quality of life for themselves.

Another strategy I like to share with you. In working with Huntington’s Disease persons we need to take an interdisciplinary approach. By that I mean we share with each other our skills and train each other to be minimally competent in our own discipline. I have been taught very well by physical therapists, occupational therapists, and music therapists in what they do. It is important to remember, particularly in a residential setting that staff are not on seven days a week, particularly therapy staff. On weekends the physical therapists and occupational therapists are not on duty and frequently on the weekend the patient will loose what they have learned unless the staff on the weekend has been taught by the physical therapist and the occupational therapist to continue those strategies over the weekend. That requires an interdisciplinary approach so that each person can help each other to make the best for the patients. More than just multidisciplinary or cross disciplinary, it must be interdisciplinary.

We need to remember, if we are going to give our patients that amount of activity they will get very tired. They need time out. In Australia we have this interesting phenomena called the “sickie,” which means that if I want to take a day off I tell the boss I’m taking a “sickie.” I’m not sick, I just want the day off and that is counted against your time off. It is important that if our people are tired from all of their activity that we give them time off. What we do is put them to bed and pamper them for a day. The next day they get up and get on with life. That is also part and parcel of our attitude that they are not sick, they need to get up and get on with life but they are allowed like we are, to have a day off.

We also have to remember, like I said before, not everything that happens to Huntington’s Disease person is Huntington’s Disease. Any sudden decline in the condition is not necessarily HD. It’s usually due to an infection but can be due to the drugs we prescribed or it may be due to a change in the physical condition due to other illnesses and very importantly, due to depression. Not everything that happens to an HD person is due to HD!

Our people need a circle of friends, a circle of companions, a lot of recreation. I would encourage you to think creatively of what kind of recreational activity we can bring to bear to our people. For instance, in New York the Cooke Center has a huge front yard called Central Park. What our people can do in Central Park is only limited by muggers and your imagination. So you can use the environment to the best advantage for our people.

There are other issues involved such as medication. I’m constantly asked: “What are the best medications?” I think we need to remember medication is not the ultimate solution for HD. I would go for environmental solutions first of all. If there’s a problem in Huntington’s Disease you can usually find a creative environmental solution for the problem. The next strategy: I would look for a psychological solution to the problem. Because there are lots of psychological directions between the person, within the person, and with staff members. The next thing I would look at is the kind of social interaction in the facility. We want to create a family atmosphere for our people. If we can have a family atmosphere, a genuine family atmosphere, we will have fights. Families do fight. As a result of some of the social interactions. Some of the problems in residential care are due to these kind of difficulties. Medication doesn’t often help. Having gone through 1) environmental; 2) psychological issues; 3) social interaction issues, then we can go for 4) the medical or pharmacological solution. It may be we have to exercise all four strategies but we must not just depend on medication as a solution.

So, finally, I’d like to remind everybody the attitude you take about Huntington’s Disease is, first of all- quality of life, secondly, enhance the ability that our people have, and thirdly, I’d like to give you a riddle: When a glass is half full of water is it half full or half empty? I’d like you to think of our Huntington’s Disease family members as half full, rather than half empty!

[Bob Kostas closes by saying:] It will be a happy day when this film is no longer needed. When science has finally developed a cure for HD, or at least a medicine to arrest it’s effect. In the meantime though, as we’ve heard from Dr. Chiu, caring comes before curing when there’s no cure available. More importantly, the HD family has the right to all available care, not the privilege- the right. You can exercise this right by keeping fully informed of developments on HD and its care, and the best way to do that is to make and maintain contact with the Huntington’s Disease Society of America. Thanks very much.
 

 

 

 
 
         
   

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