Chapter 9 - Ichi Rittoru No Namida (Part 9)

Hospital life at Nagoya Health University Hospital

In April, 1980, I finished my doctoral thesis at Nagoya University. I moved to take up a new post at Nagoya Health University Hospital - now called Fujita Health University Hospital - in Toyoake, Aichi Prefecture.

By then Aya required the use of an electric wheelchair, and she could only travel to the hospital by car.

Because Toyoake was closer to her home than Nagoya, she moved to the same hospital where I was now posted.

As I examined Aya in the consulting room at the new hospital, I compared her condition to the day when I had first met her in Nagoya. Her cheeks had been much fuller then, and I had been able to understand what she said much better. Even though she had claimed she was swaying, she was walking quite normally to other person's eyes... After just five years, however, she needed someone to push her around in a wheelchair, she couldn't utter words quickly even though she tried hard, she could only speak by twisting and streching her thin neck, and her way of speaking was hard to understand for someone not accustomed to it... I was shocked by her deterioration.

After leaving the school for the handicapped, Aya stayed at home while the other members of her family were out at work or school. She had lunch on her own and looked after herself. Her mother was worried about possible accidents while the others were out; Aya often fell over inside the house even if she was holding on to something. In fact, every time she came to the Outpatients' Department, she had injuries from falls which had caused internal bleeding on her face, arms and legs. There were more of them than before and they were becoming more serious.

She entered the internal medicine ward on the eighths floor of Building #2 of the hospital in order to have treatment and rehabilitation for the second time. She was the first spinocerebellar degeneration patient in that ward.

There were seven or eight other patients there that I was in charge of, plus some others, all with heart or blood disorders. Many of the nurses were young and some of them were younger than Aya. I had gotten into the habit of calling her 'Little Aya'. It sounded funny to hear the nurses who were younger than her calling her 'Little Aya' as well. But it shows the affection everyone had for her.

Aya operated her wheelchair herself. She washed her face using her disabled hands, went to the toilet, and cleaned the table for meals. She went for rehabilitation without fail, and read books sitting on a chair or on her bed during the daytime. She got interested in handicrafts and the origami that the other patients in her ward were teaching each other. But she was distressed at not being able to do as she wanted. The head nurse was touched when she quietly watched her at those times.

More than anyone else in the same part of the hospital, it was the older patients who were moved by Aya.

They were paralyzed on one side because they had had strokes - their blood vessels had suddenly become restricted or broken. They couldn't move their hands and legs as they wished. They got very annoyed and sometimes skipped rehabilitation sessions. Some of them had almost lost their desire not only for exercising but for life itself. However, when they saw the serious efforts made by Aya, who could have been their granddaughter, they were encouraged to do their own training again. They started bending and stretching their arms and legs on their beds.

Bo th their families and the nurses were pleased. As their doctor, I couldn't ask for more. I had explained the benefits of rehabilitation over and over every time I made a round of visits. I had tried to say various things to motivate them. But I realized that what I said had less effect than the way Aya looked as she pushed herself as hard as she could in her wheelchair.

The examination and treatment of patients is not the only role of a university hospital. It also has to carry out research and educate medical students, teaching them how to become good doctors. After studying about diseases in a general way, the students are divided into small groups of six or seven. They make a round of visits to a different department every one or two weeks to examine the patients. They read the relevant textbooks and receive guidance from the doctor in charge of the patients. This is the curriculum called 'porikuri' (polyclinic). Two groups often have to remain in the hospital at night, and sometimes even sleep over in the special 'porikuri' rooms: the students of the surgery-oriented departments, who have to observe operations, and those of the obstetrics department, who also have to attend the birth of babies.

I feel sorry for the patients who cooperate in this curriculum, but I always ask them because I think it is an important way to foster good doctors. The patients all kindly agree. When the visits are repeated, patients get used to them. They even acquire better knowledge by glancing at the textbooks carried by the students and listening to what the doctor explains to them. Reversing roles, the patients sometimes even teach things to the students in the next group that comes around - which is no laughing matter.

Aya was in the same age group as the students. I was a little concerned about her state of mind, but I wanted the students to get some understanding of her disease. I made up my mind to ask for her cooperation.

She nodded with a wet little smile.

Three students, two young men and a young woman, were responsible for Aya. They carefully examined her and studied hard about her disease. Though their visits finished after one week, one of the men sometimes went to see Aya in the evening while he was studying in a different department. He was blessed with good health and came from the kind of family in which it was only natural to study medicine. I could imagine he was shocked to learn about Aya's circumstances: entering a high school aiming at university study, and then having to move to a school for the handicapped because of her disease. And he knew that the disease was 'slow but progressive.' I was pleased to hear that he found time to visit Aya not just because of his interest in the disease but because of his kindness. It suggested to me he would make a good doctor.

One day, I was walking along the corridor after finishing my round of ward visits. Aya suddenly came out of her ward in her wheelchair, just as if she had been waiting for me. She stopped beside a fire hydrant on the dimly-lit wall and asked me a question out of the blue: "Dr. Yamamoto, can I... get married?" I automatically answered, "No, Little Aya, you can't."

Then I thought for a moment. Why had she asked that question? Maybe there was someone she liked... could it be that medical student who had been visiting her? Thinking I should listen to her carefully, I crouched down and looked into her face as she sat there in her wheelchair. I was shocked to see the look of surprise in her eyes. She had clearly been startled by my firm reply.

Aya was in a state where she had to struggle even over small things, and she knew that her disease was gradually getting worse. I had assumed that she would never even think about marriage in general, let alone think about whether she could get married or not.

Now I realized, however, that reality was different: she had become taller, her breasts had developed, and she was having her period regularly. It always bothered her because it made her sway more. I'd watched Aya grow from a young girl into a woman. So why did I assume that she would never think about getting married and having a family? I felt ashamed of myself. I had decided on that dogmatically. Even though we had been deeply associated with each other for so long, I hadn't fully understood her.

That made me reflect on my conduct. It was the biggest shock that I had ever had from one of my patients. I will never forget Aya's large, shivering eyes and surprised expression at that moment.

I suppose my answer had caught her off her guard.

"Why can't I?" she asked. "Is it because my children would have the same disease?"

"Well, you need someone to get married to," I answered as cheerfully as possible. "First of all, you'll have to find someone who fully understands your condition and will agree to marry you. Do you have anyone in mind?"

It was a very cruel answer. But I didn't want to give her a vague reply that would encourage her to cherish an illusion that would soon be dashed.

I was moved to tears as she shook her head and said, "No." I don't know which came first - her face becoming hazy because of my tears or her eyes filling with tears.

For a while, I couldn't move.

For several days after this incident, I could still hear her voice asking, "Dr. Yamamoto, can I... get married?"

The student who had visited her from time to time gradually stopped going to see her. I suppose he got too busy. Perhaps partly because of that, Aya committed herself to rehabilitation as if nothing in particular had happened. And she seemed cheerful in her ward.

Around the end of her stay in the hospital, Aya began to suffer from orthostatic hypotension. She would get a headache and feel nausea whenever she got up. Then one of the patients in the same room died suddenly. That made Aya's anxiety about dying stronger. She spent several days looking very depressed. Again I explained to her what would happen to her as the disease progressed, but I said it was a long time before she would have to face death herself. She nodded. Little by little, she became cheerful again.

However, she started needing other people to look after her. She moved to a hospital that permitted a caregiver to stay with her. I sometimes go there to see patients in my special field. Later she moved to a hospital closer to her home in Toyohashi.

Although I haven't seen her mother for more than two years, she keeps me updated on Aya's condition. She consults me and a young doctor from my university who has been sent to the hospital where Aya is staying now. So I have a good grasp of how she is doing. I hear she is loved by everyone wherever she goes, and her caregiver looks after her with warmth and compassion.

Whenever my patients with this disease start getting discouraged, I encourage them by talking about Aya. Recently, I've been thinking that in fact I am the one who has been encouraged by her most of all.

Hiroko Yamamoto

Assistant Professor,

(Now Professor)

Department of Neurology,

Fujita Health University Hospital

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Chapter 9 - Ichi Rittoru No Namida (Part 8)

Changing to a school for the handicapped

Aya's high school requested that she leave the school because she was causing trouble for the whole class.

It was just what we had feared would happen. Bitterly disappointed, Aya's mother told me that her classmates were helping her go up and down the stairs when she moved to another classroom and saying. "It's no trouble, Aya!" We'll help you in the same way in the future."

I felt brighter when I heard that her classmates were supporting her.

Her mother told me that she was going to ask the school to let her daughter stay.

"If the teachers have any questions about Little Aya's disease," I said to her, "I'll be happy to explain.

Or I could go with you to the school."

But her mother replied that she would prefer to go by herself. She went to the school many times, despite her busy work schedule, and made a strong appeal to the school authorities for Aya to stay there. In the end, however, it was decided that Aya would move to a school for the handicapped. The grounds of that school are designed so that the students can freely move around in wheelchairs. It also has a rehabilitation facility where they can study while having treatment. But I think it was a big shock for Aya's mother who,

supported by many of Aya's classmates, had been fighting against the move. When she told me in a sad voice that Aya was going to move, I felt a lump in my throat.

I suppose that the high school administrators did not know how to deal with Aya. Their conclusion was that if there was a school designed for children like her, why shouldn't Aya go there? But I wonder if causing trouble was the only effect Aya had on the school. From what I heard, the desire to care for a disabled friend was emerging very naturally among her classmates. They could learn a lot from the serious attitude of a friend who was trying so hard to live. I was very disappointed with those evolved in her education.

They didn't even inquire about er disease. They just did things by the book. Today, the issue of bullying is talked about a great deal, but I believe there was no hint of a dark shadow over Aya's classmates.

Much later, when Aya entered the hospital again, I remember her saying to me happily, "Please give me permission to go out because I'm going to meet my high school friends."

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Chapter 9 - Ichi Rittoru No Namida (Part 7)

Little Aya's admission to the hospital

Aya stayed in Ward 4A at Nagoya University Hospital. She was very popular with the nurses. Although she was a high school student, her childlike face gave her an angelic look. She obediently followed everyone's instructions, hoping to get better, even if only a little. She planned and practiced various exercises for her hands and legs. It was impossible not to be fond of her. The new remedy had a slight effect, but it didn't reduce her daily inconveniences. The nurses complained to me, saying "Dr. Yamamoto, Little Aya is working so hard. Why can't you do something to help her?" I felt a loss.

Around that time, word was going around that the professor at the hospital was an authority on spinocerebellar degeneration. Many patients came to our hospital from all over the country. Aya and U-kun, a boy one year younger than her, were young and cheerful patients. But some of the patients were bedridden, only getting up to go to the toilet in a wheelchair. Sharp-eyed Aya mentioned the names of the seriously ill patients to me and asked, "Will I be like them soon?"

I knew Aya had various dreams for her future. During my rounds, she would carefully check my reactions as she told me about them. I had begun feeling that it was about time to tell her properly about her disease.

So I answered, "It will be a long time from now, Little Aya, but yes, you'll be like that eventually."

I explained to her in detail what would happen to her as time passed: her swaying would gradually increase; eventually, it would be difficult to walk; her speach would become unclear and would not be understood; and writing and using her hands for any kind of handwork would become dificult.

For several days after that, she was very depressed. But soon she started asking me positive questions again: "Dr. Yamamoto how much longer will I be able to walk?" or "Do you think I could manage this kind of work?" I felt sorry for her, but I thought it was good that I had explained everything.

In fact, after that, our mental bond became stronger. We could talk openly about the very serious symptoms of the disease, and knowing in advance what would come next made it easier to decide what to do next.

Her stay at the hospital that time couldn't do much to improve her condition. However, I believe she left the hospital understanding what was most important for the long life under medical treatment that she would have to face.

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Chapter 9 - Ichi Rittoru No Namida (Part 6)

First meeting with Little Aya

I had just returned from a three-year stay in the United States. I was working in the 4th Study Room of the 1st Department of Internal Medicine at Nagoya University Hospital (now the Department of Neurology) with Professor Itsuro Sofue. I was helping to analyze nationwide data on sinocerebellar degeneration. I also wrote the professor's findings about the outpatients in their case records.

One Monday, a junior high school girl with a short bob was brought to the examination room by her mother. In recent years, the number of specialists who examine nervous diseases in pediatric departments has increased.

So it was unusual for a child to come for a consultation at the Department of Neurology. I learned later that Aya's mother was a nurse working for a health center in Toyohashi. She had discovered that the professor was the leader of 'the research team on sinocerebellar degeneration, a disease specified by the Ministry of Health and Welfare.' That explained why Aya had bothered to come all the way to our Department of Internal Medicine as an outpatient. It had been her mother's decision.

On the medical chart of the girl who sat in the middle of that bright consulting room in the early afternoon was written 'Aya Kito, 14 years old.' Her small round face with eyes wide open gave the impression that she was a bright girl. Her eyes looked worried as she glanced in turns at the professor and her mother as they talked.

After the first examination, the professor diagnosed spinocerebellar degeneration. He explained to Aya's mother about the disease. He gave instructions on how to get a CT scan for Aya to check the inside of her brain, a center of gravity oscillation test, and an eyball motion test. Those tests would provide a better analysis of the symptoms. He asked Aya to come back as an outpatient about once a month to check her condition.

I was impressed with the positive attitude of both Aya and her mother despite the great anxiety pressing down on them. I felt a strong affinity with them. Soon I came to have my own outpatients in a different consulting room, so I no longer had the chance of seeing Aya being examined. But we often went to the hospital on the same day and met each other in the corridor.

Aya's mother grasped the progress of the disease very well. She sometimes told me in a sad voice that Aya's swaying was increasing and she had stumbled, or that her writing had become more disordered. Sometimes she also reported happily that Aya was doing well at school, was getting good grades, and had had an internal school report praising her good attitude, so she could enter a public high school. She also told me Aya had passed the 3rd level of the Practical English Proficiency Test. I kind of championed Aya who was doing her best to fight against her disease. "The third level of the Practical English Proficiency Test is quite difficult, you know," I said proudly to my colleagues, as if she was my own daughter.

One day, around the time when the cherry blossoms in Tsurumai Park were budding and the light pink petals were started to emerge, Aya's smiling face appeared round the corner of the curtain of my consulting room.

"Dr. Yamamoto," she said, "I passed the entrance exam!" As I replied "Congratulations and good luck!", I couldn't help wishing that her disease would at least not develop too far before her high school graduation. That also encouraged me to press on with my research on a possible remedy that was being developed.

The high school that Aya entered was located in Toyohashi in Aichi Prefecture. It was oriented toward preparation for university entrance examinations. Her lively high school life began. But soon Aya's balance worsened. She could no longer commute to school an a crowded bus every morning. Even though her mother was busy as a nurse, she took Aya to school by car every day. Aya sometimes fell over in her school days and came to the Outpatients' Department with cuts on her knees or a lump on her forehead. Her mother's face darkened momentarily as she told me that Aya's scores at school seemed to be getting worse little by little.

But immediately after that she smiled cheerfully and said, "But because her writing is so slow during tests, she runs out of time. So it can't be helped, can it?"

In fact, Aya couldn't take notes properly. She also had to change classrooms for different lessons, and was always late for classes because her movements were so slow. Her high school apparently regarded that as a big problem. However, her classmates helped her a lot, carrying her textbooks or holding her hand when they saw her desperately trying to walk. I can imagine how grateful she was, but also how frustrated she must have felt about her disabled body. However, she was always smiling and her big eyes were active in her face as it gradually went thinner and smaller.

It was decided she should enter the hospital during her summer holiday in order to try out the new medicine.

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Chapter 9 - Ichi Rittoru No Namida (Part 5)

How do you explain about the disease to patients?

It is not difficult for medical specialists to diagnose the disease. However, they tend to agonize over how they should explain it to the patients and their families. Some doctors prefer not to tell the patients that there is no chance of them getting better. Instead, they encourage their patients with words like, "Don't worry, you will be cured." However, they know that the patient's condition will gradually get worse, and it's impossible for them to recover. They may explain this to the patient's family to a certain degree. Other doctors give up in despair and all they do is tell the patients and thair families that it's a very serious disease for which there is no cure.

What I say to patients is this: "It is very difficult to cure this disease. There is also the possibility it will slowly get worse. However, today various types of remedy are being developed." Then I explain in detail how many more years the patient will probably be able to walk, and how long they will be able to sit and move their hands and legs.

Patients and their families tend to be temporarily shocked by the news. But they soon recover their normal spirit and start planning their new life and how to spend their social life while coping with the disease. Some patients, however, visit various hospitals hoping to get more reassuring words about a cure. They never come back to me as outpatients. That discourages me, because I start worrying I couldn't make myself understood properly. I have to conclude that to begin with we didn't have a good rapport.

I guess those patients and their families who stay with me have thoughts similar to mine as their doctor. Little Aya Kito (referring to a grown woman like this may sound strange, but to me she is still 'Little Aya') and her mother were among them.

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Chapter 9 - Ichi Rittoru No Namida (Part 4)

Is there no remedy?

Today there is no remedy available. The cause of the disease is still unknown, so we are groping in the dark regarding proper treatment. There is a medicine that can temporarily prevent the disease from advancing or slightly slow down the speed of its advance. It has been drawing a lot of attention, but it hasn't been used for very long and we need to take a long-term view of its efficacy.

But thanks to the remarkable advances in genetic engineering in recent years, it is surely only a question of time before it will be possible to determine the chromosome in which there is a gene that causes the disease - if the disease is hereditary. Then it may be possible to replace that gene with a healthy gene. I sincerely hope that the sad voices of the spinocerebellar degeneration patients and thair families will be fully added to the debate over the rights and wrongs of gene manipulation.

But that is for the future. The most effective guidance for the time being is for patients to continue exercising and training. They must try to maintain the strength of the muscles in their whole body, and look after themselves as well as they can.

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Chapter 9 - Ichi Rittoru No Namida (Part 3)

How the disease progresses

The swaying increases, and you need some kind of support when you are walking. If it gets much worse, you cannot stand by yourself with your legs together. Pronunciation gradually becomes unclear and your speaking rhythm deteriorates, so people cannot understand what you are saying. The movements of your hands and fingers can't be controlled as you wish. That means you have difficulty writing and nobody can read what you write. You can't use

chopsticks when eating, and you can't carry food properly to your mouth, even with a spoon. And even if someone helps you to eat, it takes time to swallow, and you may sometimes choke and spray grains of boiled rice everywhere.

This symptoms progress little by little until you finally become bedridden. Then there are various dangers: bedsores can get infected; you may develop pneumonia because food gets into the trachea due to the failure of swallowing; urine is left in the bladder, and an increase of bacilli leads to cystitis or pyelitis.

As a result of all or some of these symptoms a patient usually dies in five to ten years.

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Chapter 9 - Ichi Rittoru No Namida (Part 2)

What is spinocerebellar degeneration?

A human brain has about 14 billion nerve cells, supported by more then ten times that number of cells. The nerve cells are classified into many groups. Some function when you are exercising and others work when you are observing, hearing, or feeling something. As long as a person is alive, many groups of nerve cells are working.

The nerve cells inside the cerebellum in the brain, the brain stem, and the spinal cord are required for the body to maintain balance reflexively, and to achieve quich and smooth movements. Spinocerebellar degeneration is a disease in wich those nerve cells gradually change and then finally disappear. We haven't yet discovered why they behave like that. According to nationwide statistics there are just over 1000 patients suffering from the disease in Japan. However, it is said that the real number of patients is two or three times that figure.

The most frequent symptom of the disease at the early stage is when you feel your body is swaying. At first you may think it's because you are tired or have anemia. But it gradually develops to the stage where you can't walk straight. People may start asking if you're drunk. Other early symptoms can include: dimmed vision, double vision, or things appearing to shake; difficulty in speaking because you can't get your tongue around certain sounds; difficulty urinating and the sensation of residual urine even after you've been to the toilet; feeling faint when you stand up because your blood pressure suddenly drops; etc.

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Chapter 9 - Ichi Rittoru No Namida (Part 1)

"Can I… get married?" by Hiroko Yamamoto

Introduction

I had a call from Aya's mother one Wednesday afternoon in late September. It was around the time of day when the outpatients waiting to be examined and those who were undergoing an examination were all beginning to get a little tired of having to spend such a long time in the hospital. She told me she was preparing to publish Aya's diary, wich had been written over a long period. She wanted to ask me, as her doctor, about the disease Aya had suffered from and also about my association with her.

While advising Aya to keep her notes in a diary and organize them into a book, I was concerned about the fact that there wasn't much I could do to help her. So I was relieved and very pleased to hear that publication was getting under way. Aya is no longer able to get up by herself and is now bedridden. She has to rely on someone to help her eat and do everything for her.

Her mother said she wanted to complete the book as quickly as possible for the sake of such a daughter. I felt a lump in my throat when I heard her mother's way of talking. I granted her request. At the same time, I thought that looking back would be a good chance for me to put everything together; my encounter with Aya was also deeply connected with my own growth as a doctor.

You may find what I have to say about Aya's incurable disease - spinocerebellar degeneration - a little difficult to follow. But I hope you will read it carefully because it is important to understand her way of live.

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