THROMBOSIS RESEARCH 67; 339-344,1992 0049-3646/92 $5.00 + .OOPrinted in the USA. Copyright (c) 1992 Pergamon Press Ltd. All rights reserved.
THE HAEMOPHILIAC
IN THE SOCIETY
Peter F. Hjort Department of Health and Society, State Institute of Public Health, Geitmyrsveien 75, 0462 Oslo 4, Norway (Received 12.6.1992; accepted in revised form 7.7.1992 by Editor Editor-in-Chief
B. BKjmback)
For three years (195557) Helge Stormorken and I shared a little room in Paul Owren’s clotting laboratory, which became The Institute for Thrombosis Research. Later, our roads departed. He stayed with haemostasis, I went to clinical haematology, to medical education and later to health services research, prevention and social medicine. There has always been a close relationship between the haemophiliacs and the research in haemostasis at the University Clinic of Oslo. The researchers have been involved in clinical and practical work for and with the haemophiliacs. In 1966, we started together the Norwegian Association for Haemophilia. At the 25th anniversary the new generation of haemophiliacs wanted to learn about their past and to discuss their future, and I was asked to present ideas for discussion.
1. THE PAST The past started one day in 1943, when a young woman was admitted to Medical Depattment A of the University Clinic in Oslo. She had bled severely all her life and obviously had a congenital defect of her haemostatic system. She was received by the resident, Paul A. Owren. At that time, the Morawitz clotting theory had been unchallenged for 40 years: Thromboplastin and calcium converted prothrombin to thrombin, which again converted fibrinogen to fibrin. Owren demonstrated that his patient lacked a previously unknown clotting factor which he called proaccellerin or Factor V. This was a scientific breakthrough, and Owren spent the rest of his life in research on clotting and haemostasis. He became professor and chief of Medical Department A and developed a research group which grew rapidly in size and fame. In our daily work we came in close contact with the patients in two ways. They were admitted for diagnosis and treatment, and we depended on their blood for our assay systems. We tried of course to improve the treatment, and the great leap forward came with Judy Pool’s cryoprecipitate. The situation of the haemophiliacs impressed me greatly. They had frequent and very painful bleeding episodes, and they had learned from sad experience that the treatment did 339
340
HAEMOPHILIAC
IN THE SOCIETY
Vol. 67, No. 3
not make much difference. Due to the bleeding episodes they had been kept home from school for long periods. Their joints and teeth were in bad condition, and most of them had very little education and no job. I saw them as people whose life, selfrespect and future had been crushed by the disease. Furthermore, most of them lived in isolation with their terrible problems. Norway had about 300 haemophiliacs in a population of 4 million living in a country with very large distances. We hoped, of course, that research should bring an end to this misery. But we realized that weeping solutions were far off and we were forced to see that we had to open a second front - a human, social and political front. Therefore, the Association for haemophiliacs was started, copying the model from England. The association started to press for the rights of the haemophiliacs in the society, e.g., the right to home-teaching during protracted illness, dental treatment etc. However, very soon it became clear that there were two different fields of action. One was to make demands for social rights, the other was to build up internal friendship and support within the group in order to break the isolation of the haemophiliacs. Quite soon the second field of action became the most important, Gradually, the “haemophilia model” took form. It had four parts: i) The first was a specialty ward for haematological diseases. Here we could build up clinical experience, and we could develop specialty teams which collaborated on clinical problems, e.g. dental services and orthopedic surgery. All surgery in patients with bleeding disorders is still done at the University Clinic of Oslo. ii) The second was research in all aspects of haemostasis and its disorders. Studies in anticoagulant therapy have been an important part of this research. iii) The third was an “Institute for haemophilia” which works on all the practical problems of haemophilia, including the education of parents and patients. It was started to offer the boys a place to live during studies or vocational training. This was important, since the boys haJ to have an education in order to develop an independent social life. Every family was invited to a family course on haemophilia, including home transfusions. Summer camps also proved to be important. Social workers and nurses were trained to make home visits even in remote places to set up local arrangements for care and education. There is no doubt that the institute has played a decisive role in the life of the haemophiliacs and their families. iv) The fourth part was the association of haemophilia which is completely managed by the patients and their families. These four parts have worked together for more than 25 years. The haemophiliacs themselves play a key role in the organization. This model has been quite successful, and it has become the standard goal - and the envy - of other handicap-groups in Norway. The haemophiliacs have been lucky and privileged, probably for two reasons. First, the organization was developed during a period of rapid growth in the health services in Norway. Secondly, haemophilia is a “statusdisorder”. It has been easier to argue for haemophilia than for epilepsy and mental
Vol. 67, No. 3
HAEMOPHILIAC
IN THE SOCIETY
disorders. I believe, however, that many other chronic diseases and disabilities should develop similar models. The effect on the patients and their families has been remarkable. Thanks to better treatment, especially home transfusions, the young population of haemophiliacs have much smaller disabilities. Fortunately, only very few patients have become HIV-positive. Education has further changed their social situation. The haemophiliacs now become doctors, engineers, lawyers and accountants. They are socially independent, marry and manage their own lives. It is almost unbelievable to compare the past and the present. The haemophiliacs have straightened up and are busy with life, not the disease.
2.
FUTURE
There are reasons to hope for new and fundamental progress in the management of haemophilia. Yet, many problems will remain, and I shall discuss some of them. The situation of the haemonhiliac in the society. The demands on the haemophiliacs will increase. They will have to take more and more responsibility for treatment, care, prevention, education and work. The haemophilia families have the same problems as other families with children, but they are of course larger and more difficult. It will be especially important to motivate the boys for education, systematic work and independent responsibility. Increasingly, we approach the so-called 20-80 society, i.e., a society in which 80% are both well and well off, while 20% have increasing problems with health, work and living. The prevailing philosophy is more competition and greater differences. The struggle for success, personal control and happiness will become tougher. Unemployment is a severe and increasing problem. At present, the prospects are that at least 10% of the young will never get employment. These problems will of course be tougher for the handicapped. Many of them will experience severe problems and will be forced into a permanent “sick role”. It should of course be the responsibility of the society to adjust conditions for the handicapped, but the chances are that they will be squeezed out by “market forces”, unless they are able to stand up for themselves, individually and as a group. These thoughts have two implications for the haemophiliacs. First, every one of them must remember that they carry a responsibility, not only for themselves and not only for the haemophiliacs, but for all the handicapped. The society must be made to see that it needs the disabled and that it can depend on them. These are severe demands on the disabled, but it is important to face them. Unfortunately, people are often unfair. If an ordinary youth shirks his responsibility, the blame is on him. If a disabled does the same, the whole group must often take the blame and the discrimination. Secondly, the haemophiliac - like all disabled - must work for a better and more fair society. The society must be made to realize that the care and the responsibility for the unfortunate is an important cultural value, the value of fellowship and solidarity. Everybody will in reality profit from this idea, because human life is such that all of us sooner or later will need public support. Nobody is able to completely escape difficult problems and crushing demands. This is simply the nature of human life. It is entirely wrong,
341
342
HAEMOPHILIAC IN THE SOCIETY
Vol. 67, No. 3
therefore, to speak about & who are disabled and lls. who are well. We shah all depend on someone or on society, sooner or later. Therefore, it is important to work for the idea of solidarity, both individually and through our organizations. The haemoohilia model. Experience has shown that the model works for the haemophiliacs, and the task now is to adapt it to other groups of disabled. This has proved to be difficult, because time and money have run out for the other groups. The haemophiliacs came first and were served fast. It is important, therefore, that the haemophiliacs and their doctors constantly work to improve quality and efficiency of their model, evaluate and publish their experiences, and support other disability groups in their struggle to achieve a similar model. Health-Drevention and oromotion. The World Health Organization has coined its famous “utopian” definition of health as the total absence of disease and impairment and the complete physical, mental and social well-being. It has served well as a political slogan, but it is far from real life. Experience has shown that many people live their lives with chronic diseases and disabilities. At the age of sixtyfive, 70% of the Norwegian population claim to have a chronic health problem, and about 17% of our population have a permanent and substantial disability. Experience shows, however, that many of them - like many of the haemophiliacs - cope with their problems and live useful and happy lives. Thus, health in spite of disease is an important and common experience. This leads to a different view of health which I have tried to illustrate in Fig.1
‘I
I
I
4 8
Promotion of health 0
.I
Pevention and treatment of disease
-I
FIG. 1
From Disease to Health: the two Different Dimensions
HAEMOPHILIAC
Vol. 67, No. 3
IN THE SOCIETY
To the left is a vertical scale, starting far down in the negative - disease and ill-health. It passes through zero - not ill, and ends high up in the positive - health. Below zero are disease, illness, disability, injuries and difficult life problems. Above zero is something entirely different - strength, happiness, optimism, well-being and fellowship. Thus, we must work: -
below zero to prevent disease and injuries, to remove risk factors and to treat diseases
-
above zero to promote health, improve resistance, build up reserves and learn to cope
It is especially important to understand the field above zero in order to understand and promote health.
TABLE I The Positive Aspects of Health
Well-being
coping
Reserves
Social support
A good life Joy of life Cheerfulness Optimism Hope Fellowship Love Happiness
Potency Stamina Internal coherence Functional fitness Adaptation Positive self image Self assurance Self esteem Self reliance
Capital Fitness Vitality Energy
Helpfulness Friendliness Faithfulness
In Tab.1 I have listed some of the words people use when I talk to them about these ideas and also some of the concepts scientists use when they write about them. I have divided them arbitrarily into four obviously partly overlapping groups. The last group - social support - is usually not considered as a part of individual characteristics, but I have included it, since there am close relations between such characteristics and the social support and individual goals. The important idea is that many health problems cannot be prevented or removed. They must be lived with and coped with. Experience shows that many people, probably the majority, learn to cope. This is the message I have taken from many of the haemophiliacs too. The big problem for health promotion is to find out how the families and the society can learn to cope in order to achieve health in spite of disease.
343
344
HAEMOPHILIAC
IN THE SOCIETY
Vol. 67, No. 3
3. CONCLUSIONS
I have tried to spin together two threads - past and future. The haemophiliacs have been lucky: they have been able to harvest the fruits of research, and they have succeeded to build an effective organization for prevention and treatment of bleedings and for coping with the disease. However, they have deserved their results, through their courage and stamina. I believe the experiences of the haemophiliacs are useful for the organization of treatment and care for other disabilities. Finally, the message of health in spite of disease is basic for promotion of health.
THE HAEMOPHILIAC
IN THE SOCIETY
Peter F. Hjort Department of Health and Society, State Institute of Public Health, Geitmyrsveien 75, 0462 Oslo 4, Norway (Received 12.6.1992; accepted in revised form 7.7.1992 by Editor Editor-in-Chief
B. BKjmback)
For three years (195557) Helge Stormorken and I shared a little room in Paul Owren’s clotting laboratory, which became The Institute for Thrombosis Research. Later, our roads departed. He stayed with haemostasis, I went to clinical haematology, to medical education and later to health services research, prevention and social medicine. There has always been a close relationship between the haemophiliacs and the research in haemostasis at the University Clinic of Oslo. The researchers have been involved in clinical and practical work for and with the haemophiliacs. In 1966, we started together the Norwegian Association for Haemophilia. At the 25th anniversary the new generation of haemophiliacs wanted to learn about their past and to discuss their future, and I was asked to present ideas for discussion.
1. THE PAST The past started one day in 1943, when a young woman was admitted to Medical Depattment A of the University Clinic in Oslo. She had bled severely all her life and obviously had a congenital defect of her haemostatic system. She was received by the resident, Paul A. Owren. At that time, the Morawitz clotting theory had been unchallenged for 40 years: Thromboplastin and calcium converted prothrombin to thrombin, which again converted fibrinogen to fibrin. Owren demonstrated that his patient lacked a previously unknown clotting factor which he called proaccellerin or Factor V. This was a scientific breakthrough, and Owren spent the rest of his life in research on clotting and haemostasis. He became professor and chief of Medical Department A and developed a research group which grew rapidly in size and fame. In our daily work we came in close contact with the patients in two ways. They were admitted for diagnosis and treatment, and we depended on their blood for our assay systems. We tried of course to improve the treatment, and the great leap forward came with Judy Pool’s cryoprecipitate. The situation of the haemophiliacs impressed me greatly. They had frequent and very painful bleeding episodes, and they had learned from sad experience that the treatment did 339
340
HAEMOPHILIAC
IN THE SOCIETY
Vol. 67, No. 3
not make much difference. Due to the bleeding episodes they had been kept home from school for long periods. Their joints and teeth were in bad condition, and most of them had very little education and no job. I saw them as people whose life, selfrespect and future had been crushed by the disease. Furthermore, most of them lived in isolation with their terrible problems. Norway had about 300 haemophiliacs in a population of 4 million living in a country with very large distances. We hoped, of course, that research should bring an end to this misery. But we realized that weeping solutions were far off and we were forced to see that we had to open a second front - a human, social and political front. Therefore, the Association for haemophiliacs was started, copying the model from England. The association started to press for the rights of the haemophiliacs in the society, e.g., the right to home-teaching during protracted illness, dental treatment etc. However, very soon it became clear that there were two different fields of action. One was to make demands for social rights, the other was to build up internal friendship and support within the group in order to break the isolation of the haemophiliacs. Quite soon the second field of action became the most important, Gradually, the “haemophilia model” took form. It had four parts: i) The first was a specialty ward for haematological diseases. Here we could build up clinical experience, and we could develop specialty teams which collaborated on clinical problems, e.g. dental services and orthopedic surgery. All surgery in patients with bleeding disorders is still done at the University Clinic of Oslo. ii) The second was research in all aspects of haemostasis and its disorders. Studies in anticoagulant therapy have been an important part of this research. iii) The third was an “Institute for haemophilia” which works on all the practical problems of haemophilia, including the education of parents and patients. It was started to offer the boys a place to live during studies or vocational training. This was important, since the boys haJ to have an education in order to develop an independent social life. Every family was invited to a family course on haemophilia, including home transfusions. Summer camps also proved to be important. Social workers and nurses were trained to make home visits even in remote places to set up local arrangements for care and education. There is no doubt that the institute has played a decisive role in the life of the haemophiliacs and their families. iv) The fourth part was the association of haemophilia which is completely managed by the patients and their families. These four parts have worked together for more than 25 years. The haemophiliacs themselves play a key role in the organization. This model has been quite successful, and it has become the standard goal - and the envy - of other handicap-groups in Norway. The haemophiliacs have been lucky and privileged, probably for two reasons. First, the organization was developed during a period of rapid growth in the health services in Norway. Secondly, haemophilia is a “statusdisorder”. It has been easier to argue for haemophilia than for epilepsy and mental
Vol. 67, No. 3
HAEMOPHILIAC
IN THE SOCIETY
disorders. I believe, however, that many other chronic diseases and disabilities should develop similar models. The effect on the patients and their families has been remarkable. Thanks to better treatment, especially home transfusions, the young population of haemophiliacs have much smaller disabilities. Fortunately, only very few patients have become HIV-positive. Education has further changed their social situation. The haemophiliacs now become doctors, engineers, lawyers and accountants. They are socially independent, marry and manage their own lives. It is almost unbelievable to compare the past and the present. The haemophiliacs have straightened up and are busy with life, not the disease.
2.
FUTURE
There are reasons to hope for new and fundamental progress in the management of haemophilia. Yet, many problems will remain, and I shall discuss some of them. The situation of the haemonhiliac in the society. The demands on the haemophiliacs will increase. They will have to take more and more responsibility for treatment, care, prevention, education and work. The haemophilia families have the same problems as other families with children, but they are of course larger and more difficult. It will be especially important to motivate the boys for education, systematic work and independent responsibility. Increasingly, we approach the so-called 20-80 society, i.e., a society in which 80% are both well and well off, while 20% have increasing problems with health, work and living. The prevailing philosophy is more competition and greater differences. The struggle for success, personal control and happiness will become tougher. Unemployment is a severe and increasing problem. At present, the prospects are that at least 10% of the young will never get employment. These problems will of course be tougher for the handicapped. Many of them will experience severe problems and will be forced into a permanent “sick role”. It should of course be the responsibility of the society to adjust conditions for the handicapped, but the chances are that they will be squeezed out by “market forces”, unless they are able to stand up for themselves, individually and as a group. These thoughts have two implications for the haemophiliacs. First, every one of them must remember that they carry a responsibility, not only for themselves and not only for the haemophiliacs, but for all the handicapped. The society must be made to see that it needs the disabled and that it can depend on them. These are severe demands on the disabled, but it is important to face them. Unfortunately, people are often unfair. If an ordinary youth shirks his responsibility, the blame is on him. If a disabled does the same, the whole group must often take the blame and the discrimination. Secondly, the haemophiliac - like all disabled - must work for a better and more fair society. The society must be made to realize that the care and the responsibility for the unfortunate is an important cultural value, the value of fellowship and solidarity. Everybody will in reality profit from this idea, because human life is such that all of us sooner or later will need public support. Nobody is able to completely escape difficult problems and crushing demands. This is simply the nature of human life. It is entirely wrong,
341
342
HAEMOPHILIAC IN THE SOCIETY
Vol. 67, No. 3
therefore, to speak about & who are disabled and lls. who are well. We shah all depend on someone or on society, sooner or later. Therefore, it is important to work for the idea of solidarity, both individually and through our organizations. The haemoohilia model. Experience has shown that the model works for the haemophiliacs, and the task now is to adapt it to other groups of disabled. This has proved to be difficult, because time and money have run out for the other groups. The haemophiliacs came first and were served fast. It is important, therefore, that the haemophiliacs and their doctors constantly work to improve quality and efficiency of their model, evaluate and publish their experiences, and support other disability groups in their struggle to achieve a similar model. Health-Drevention and oromotion. The World Health Organization has coined its famous “utopian” definition of health as the total absence of disease and impairment and the complete physical, mental and social well-being. It has served well as a political slogan, but it is far from real life. Experience has shown that many people live their lives with chronic diseases and disabilities. At the age of sixtyfive, 70% of the Norwegian population claim to have a chronic health problem, and about 17% of our population have a permanent and substantial disability. Experience shows, however, that many of them - like many of the haemophiliacs - cope with their problems and live useful and happy lives. Thus, health in spite of disease is an important and common experience. This leads to a different view of health which I have tried to illustrate in Fig.1
‘I
I
I
4 8
Promotion of health 0
.I
Pevention and treatment of disease
-I
FIG. 1
From Disease to Health: the two Different Dimensions
HAEMOPHILIAC
Vol. 67, No. 3
IN THE SOCIETY
To the left is a vertical scale, starting far down in the negative - disease and ill-health. It passes through zero - not ill, and ends high up in the positive - health. Below zero are disease, illness, disability, injuries and difficult life problems. Above zero is something entirely different - strength, happiness, optimism, well-being and fellowship. Thus, we must work: -
below zero to prevent disease and injuries, to remove risk factors and to treat diseases
-
above zero to promote health, improve resistance, build up reserves and learn to cope
It is especially important to understand the field above zero in order to understand and promote health.
TABLE I The Positive Aspects of Health
Well-being
coping
Reserves
Social support
A good life Joy of life Cheerfulness Optimism Hope Fellowship Love Happiness
Potency Stamina Internal coherence Functional fitness Adaptation Positive self image Self assurance Self esteem Self reliance
Capital Fitness Vitality Energy
Helpfulness Friendliness Faithfulness
In Tab.1 I have listed some of the words people use when I talk to them about these ideas and also some of the concepts scientists use when they write about them. I have divided them arbitrarily into four obviously partly overlapping groups. The last group - social support - is usually not considered as a part of individual characteristics, but I have included it, since there am close relations between such characteristics and the social support and individual goals. The important idea is that many health problems cannot be prevented or removed. They must be lived with and coped with. Experience shows that many people, probably the majority, learn to cope. This is the message I have taken from many of the haemophiliacs too. The big problem for health promotion is to find out how the families and the society can learn to cope in order to achieve health in spite of disease.
343
344
HAEMOPHILIAC
IN THE SOCIETY
Vol. 67, No. 3
3. CONCLUSIONS
I have tried to spin together two threads - past and future. The haemophiliacs have been lucky: they have been able to harvest the fruits of research, and they have succeeded to build an effective organization for prevention and treatment of bleedings and for coping with the disease. However, they have deserved their results, through their courage and stamina. I believe the experiences of the haemophiliacs are useful for the organization of treatment and care for other disabilities. Finally, the message of health in spite of disease is basic for promotion of health.
