Editors: Norio Fujiki, M.D. & Darryl R.J. Macer, Ph.D.
Sawako Kawamura
Professor, Division of Nursing, Tokyo Medical & Dental University, Tokyo, Japan
The national government secures and provides health care services for the health and welfare of all the Japanese people through various health care systems.
There are public health centers distributed throughout the country, administered by the prefectures or municipalities (cities, towns, villages) which provide services for health promotion and prevention of diseases. Of the two, municipal public health centers provide health care services aiming at individuals.
For these medical services, in accordance with people's medical needs an effort to classify organisations and facilities according to their functions is now under way. 'Hospitals with Special Functions' are to deal with those who need highly advanced medical treatment, while clinics and general hospitals will treat patients with rather common diseases in their acute phase. 'Long-term care facilities' are, on the other hand, for the patients in chronically ill states. Home medical and nursing care service are also available for those needing them. Also palliative care units and facilities are growing in number.
One of the major health problems in present Japan is how to deal with the rapidly aging society. As of October, 1992, people over 65-year-old occupied 13.1% of the whole population. Health care services for the aged include day care activities at public health centers and the organisation of self-help groups and their networks. Social welfare services supporting them include meal delivery, home help services, bathing, and short term admission programs, which are becoming more comprehensive. Care providers engaged in these services are scanty in number and rather inexperienced as yet. Therefore, much more effort is needed to increase their number and train them. Recently educational programs for visiting nurses and professional care helpers are becoming much sophisticated. In addition, such systems and facilities as 'Visiting Nurse Stations' and 'Centers for Supporting Home Care' have been officially approved and their construction encouraged.
As I have briefly sketched here, in recent years health care systems as a whole in Japan are being steadily expanded and enriched.
2. Care systems for people with problems related to hereditary diseases
a) People with problems related to hereditary diseases
Cases given below are those which the author, Kawamura, has experienced in her 20-year career as a visiting nurse.
Case A: Mrs. A whose first grandchild suffered from congenital muscle dystrophy
It was a consultation by telephone and the client seemed to be the patient's grandmother. The main points of the consultation were: the patient was a 15-day-old newborn baby still being taken care of in a hospital. The baby was, according to the diagnosis, suffering from congenital muscle dystrophy. His doctor had explained that he would not grow normally and that the cause was something hereditary. Mrs.A, as well as the other family members, did not have any idea were they to take him home how to feed or take care of that baby who had no prospect of growing. She could find no explicit genetic history either on the side of the baby's paternal (her own) or among maternal relatives. The paternal and maternal sides had quarrelled against each other concerning the baby's serious disorder and after talking it over had decided to force the parents to divorce, but the baby's very existence was a barrier to this. They asked the doctor to take some steps to end the baby's life but were refused. Mrs.A wondered if there was any way of doing this after the baby was brought home.
The client, Mrs.A, was doubly troubled facing two tasks. These two tasks were, 1) to accept the sudden appearance of a grandchild with a hereditary disease and 2) to face her own heredity. The nurse, the author, listened to and tried to understand what the parents thought and expected, and gave Mrs.A information on how to take care of the baby and examples of other parents and families who had raised handicapped babies. She seemed to regain her presence of mind through this process.
Unfortunately, at present there is little comprehensive and systematic support for those who are faced with trouble such as Mrs. A's., which provides consultation, counseling, education and information and concrete help and guidance on how to look after the child, so that unity of the family might not be destroyed. Medical organisations generally fail to provide nursing consultation or case working. Though some public health centers are ready to provide such services, few people know this.
Case B: Miss B, whose mother was suffering from a cerebellar degenerative disease
As the patient (Miss B's mother) wanted to be discharged from hospital and return home, Miss B came to the author to get advice on how to manage home care. But what she really wanted information on was something else. The patient had divorced because of her illness. Miss B had been taking care of her mother for 10 years, while working herself. She knew that her mothers illness was hereditary.
The doctor had recently told Miss B that her mother's general condition was worsening and she would not survive more than a few months. Hearing this explanation, she had come to face up to her mother's impending death. Miss B had found the meaning of her own life in taking care of her mother. So helping her mother helped her in turn. When faced with the harsh reality that she would soon lose her mother and be left to lead a solitary life thereafter, she began to think of marriage. At that time one of her relatives recommended her a man as a spouse, telling her that she should marry after her mother's death, keeping her hereditary illness secret.
Miss B, however, thought otherwise. She did not mean to get married without telling her partner of her hereditary potential. She herself did not want to have a child who would inherit the same genetic problem. Marriage did not necessarily aim at child-bearing and child-rearing, she thought. She hoped to find a man who would understand her hereditary background and agree to a childless marital life. She developed her ideas even further and began to think that it would be quite difficult for general people who did not have a idea of hereditary problems to understand her. Then she thought that there could be someone with a similar genetic problem to her, and that she would be able to create meaningful life with such a person. She was looking forward to joining a self-help group or a patient group.
For those who may have hereditary predispositions, even though symptoms or disorders are not manifest, their quality of life (QOL) is threatened as in this case. Social support should be provided in order to help clients get through these hardships and improve their QOL. The organisation of self-help groups is one example of how this may be done.
Case C: Family members of a person with a cerebellar degenerative disease
The number of clinics and hospitals that provide home care services or home visits is increasing. Mr. C has been suffering from a hereditary cerebellar disease for 15 years now. His gait disorder is markedly worsening. As his wife could not by herself help him all the way to the hospital's outpatient's department, medical staff visited and treated him at his home.
His wife called up the doctor and the visiting nurses each and every day. This suggested that one of the major needs in the case was psychosocial support for the wife. Mr. and Mrs. C had three children : the older son was married and living independently without much interaction with them; the daughter was also married and living apart from them with her children; the younger son had made up his mind to live with his parents and support them, and got a job in the nearby area.
Before Mr. C got gait disorder, Mrs. C's major complaint was that her children would abandon her and Mr. C, as their older son and the daughter had already left home. But this complaint vanished as their younger son was living with and helping them.
As Mr. C's gait disorder progressed, psychiatric symptoms began to appear. All of sudden, he would shout "let's go out!" and start banging at the wall mistaking it for a door, screaming "It won't open !", which began to wake and disturb their neighbours. Mrs. C kept watch over him all through the night almost without sleeping. Whenever Mr. C rose up and tried to go out, she restrained him, distracted him and calmed him. Being much troubled and bewildered by these episodes, the younger son could not help but begin to think that he, as Mr. C's son, was also doomed to collapse mentally, as well as physically. Though he said he had given up the idea of marriage, he was not really resolute. He once told the visiting nurse that there was something deep down in his mind that could not be settled down by reason or logic.
Then Mr. C got into the habit of sleeping well during 10 to 12 o'clock at night. Around this time the younger son came back drunken and started groaning at Mrs.C, his mother, saying that he had no idea how to get on in the world, "Dad has a wife to take care of him, as he has married. He could carry on as a man. He can get you to spoil him when he feels bad. But what should I do? You may not be alive when I reach Dad's age. When the illness strikes me and makes me behave like Dad, whose going to be kind to me, and look at me as a human being? I can't live. I've been thinking of jumping in front of a train, but I can't. I can't even die. I don't know, should I buy myself a woman?" His agony was endless. Mrs. C, though listening to him, fell into a doze as she was very tired. At this, the younger son got furious, accusing her "Even you don't care about me" . Mrs. C, not knowing how to care for her own fatigue, called up the visiting nurse as early as 8:30 the next morning just as the home visit section opened, and poured out her own grief over a long phone call.
Mr. C's care was managed fairly well from the treatment and nursing point of view, but his wife and children, though physically well, were heavily overburdened and deeply hurt inside. What kind of support would be appropriate for the family? It would be very helpful for Mr. C's younger son to get in touch with people like Miss B in the Case B described above. Mrs. C needs some nurses to take care of her husband for her everyday, so that she can get enough time to sleep.
Case D: A daughter whose mother had Huntington's chorea
Mrs. D, the patient, had a gentle husband who was in business, and a daughter. This daughter changed suddenly after she entered high school, starting to wear extremely long skirts and put on red lipstick. After Mrs. D told her not to dressed like that as she was still at school, she ran away from home. She was nowhere to be found. A rumour reached the caseworker at the hospital where Mrs. D was taken care of that the daughter was working in a certain bar in downtown Tokyo. The caseworker called on her in secret. On hearing that her mother was anxious about her, she burst into tears. She said, "After we entered high school, my friends started talking about marriage. When I thought of my marriage, I was suddenly reminded of my mother's illness. In that instant I thought I can never marry. My mother once told me that she got ill right after my birth. That was when she was 20 years old. There are only three years of health and liberty left for me before I reach that age. When I realized this, I turned pale and couldn't stop trembling with fear. I don't care what happens to me. Now is the last chance for me to act as a healthy woman. Leave me alone!"
Mrs. D's daughter is much younger than Miss B or Mr. C's younger son. Counseling and guidance should be provided in an educational setting to help those with hereditary problems to think of their future and make the most of their present lives.
Case E: A wife whose husband had cerebellar degeneration
One evening, Mrs. E suddenly charged into the home visit office looking desperate. Mrs. E was a really genteel 70-year-old lady, who had been devotedly taking care of her husband who had gait, speech, swallowing and mictrition disorders which required a lot of attention. That lady was now spiting out, "Shit, I've been cheated !" Asked what the trouble was, she said again in a strangled voice, "I've been cheated for 50 years!" Her story went back to the days when she got married to Mr. E. He was an orphan, who was a quiet hard-worker respected by every one at work. Moved by that character, Mrs. E married him. He was supposed to have no relatives but the other day, there had been a telephone call to Mr.E from an ex colleague. Previously Mr. E had answered the phone, but now that he had difficulty in movement and in speech, Mrs.E answered the call for him and so got to know an unbelievable fact. The caller was Mr. E's nephew, telling her that his father (Mr. E's brother) had just died of stomach cancer. Mrs. E was quite astonished as she had been told and believed that Mr. E had no relatives at all. She left for the funeral to be held in Mr. E's home town that was several hours trip from Tokyo and found many relatives of Mr. E's. There she learned that though Mr.E's brother had died of cancer, he also had had the same disease as Mr. E and some other of his relatives were suffering from that same disease,too. His relatives had stopped contacting Mr. E out of consideration for his position and Mr. E had kept up a one-way communication with them. They told Mrs. E that they were very worried as Mr. E had failed to contact them recently.
Mrs. E told the visiting nurse, "My husband has been kept his hereditary disease secret from me. It is just too cruel to find out now we already have children and grandchildren. On the way back I thought of killing myself, or of divorce, or of throttling him. But whatever I might do, that genetic trait passed on to my descendants cannot be wiped out. When I realized that, I went cold and couldn't stop crying. I have no idea what to do. Suddenly I wanted to talk to you and I came here."
There are so many people with hereditary diseases who, suffering alone, cannot disclose the fact even to their family members. This would be fine if the symptoms were never to appear and the facts never to be known to others. But things never go that way. Sometimes the facts are revealed, as in Mr. E's case, when the one who is affected has totally lost their capacity for self-care and when the hereditary factors may have been handed over and spread to following generations.
b) Measures to help families with problems related to hereditary diseases
Both physically and mentally healthy family members are one of the crucial factors in a supportive environment for the diseased. Also it is important for all members of the family, especially the young ones, to be able to have hope in their own future. QOL for the patients is achieved through the QOL of their family members, and vice versa.
As we have seen in the cases described above, in principle every family's members are very caring and supportive. But they are overburdened with distress which they cannot tell the patient, and so they tell it to the visiting nurses instead. Thus it is one part of the work of visiting nurses to spend time listening to them. Topics concerning genetic traits often come up on such occasions, and thus they often become the opportunity for the care team to improve support.
Unfortunately, visiting nurses usually do not have enough time to solve such problems or alleviate the client's distress. There is seldom a sufficient number of staff to provide continuous support for family members with hereditary problems, either. Below are suggestions for additional measures and for members to be included in our care team :
- consultation and counseling services for hereditary diseases and related problems; these services should be provided at medical, health care and home visit organisations
- marriage counseling for those with hereditary diseases and their relatives
- self-help groups for those with hereditary diseases and their relatives
- educational activities
In Japan, ethical problems related to hereditary diseases have not yet been subject to open discussion. This makes it difficult for health care providers to deliver their services, even though some of those problems might be solved or at least eased effectively through such services. An effort should be paid to alter this situation. Systems should be developed to share the fruits of genetic sciences to all that need them.
1. Final Report on Studies of the Ethical and Legal Problems in Medicine and Biomedical and Behavioral Research, President's Commision for the Study of Ethical Problems in Medicine and Biomedical and Behavioral Research, (Washington D.C., U.S.G.P.O. 1983).
2. Loeb,S., et al., eds., Law & Ethics (Springhouse 1992).
3. Leininger,M.L. et al., eds., Ethical and Moral Dimensions of Care (Wayne State Univ. Press 1990).