Haemostasis 1992;22:299-304
J. Martin-Villar
La Paz Hemophilia Center,
Hematology Service, Hemophilia Center, La Paz Hospital, Madrid, Spain
1967- 1992: People, Events, and Memories
Key Words
Abstract
Hemophilia Hemophilia centers Comprehensive care AIDS
In 1967, once urgent blood requirements had been resolved in La Paz Hospital, Madrid, Spain, and adequate supplies of plasma for cryoprecipitation, therefore, being available, a He mophilia Unit (later Hemophilia Center) was set up to attend to the comprehensive care of hemophilia patients and patients with other coagulopathies. This Center covers a regional area and also a national area for referrals and normally attends 600 patients. In this Center the Spanish Social Security Hemo philia Association was created. In 1983 the first cases of AIDS appeared, occasioning the change from standard factor VIII concentrates to the heat-treated concentrates that had recently appeared on the market. At present the Center is responsible for the medical attention of those hemophilia patients affected by HIV.
It is nearly 26 years since I was appointed Head of the Service of Hematology and Hemotherapy of the La Paz Hospital. In a few days I shall be leaving the position, owing to what is generally termed ‘biological reasons’, in this case fortunately only age. A group of friends and collaborators decided to mark this occasion by organizing a symposium dedi
cated to the field of hemophilia, an area to which the Hemostasia Section - and the He matology Service in general - has dedicated much time and effort which does not imply that it has been its only activity nor that it is most important. In keeping with the theme of the symposium, then, I should like to relate briefly the short history of the Hemophilia Unit of La Paz, now known as La Paz Hemo philia Center.
J. Martin-Villar Hematology Service Hemophilia Center La Paz Hospital E-28046 Madrid (Spain)
© 1992 S. Karger AG, Basel 0301-0147/92/ 0225-0299S2.75/0
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 1/24/2019 10:22:38 AM
Introduction
300
Martin-Villar
mind attempting to define and encompass a mysterious yet fascinating phenomenon; and also the human heart responding to the chal lenge of repeated adversity’ [2], But let me return to our first group of hemophiliacs in Ottawa, in the 50s. In order to care for them we were obliged to reorganize our small coag ulation laboratory and to become slaves to ‘accuracy’ and ‘reproducibility’ and to main tain a fine tuning of some of those classical tests in the history of the disease, TGT, factor VIII, and inhibitor quantitation, while on the shelf stood our ‘Bible’ - ‘Human Blood Coag ulation’ of MacFarlane and Biggs. As for ther apy, good relations with the blood bank al lowed us to make limited quantities of freshfrozen plasma. I remember that patients would say, when referring to their treatment, that after frozen plasma came the dedication of their doctors and the love of their families. The last two are still just as important in the care for our patients at the Center.
Organization of a Hemophilia Unit for Comprehensive Treatment in La Paz: Autumn 1967
The years 1966 and 1967 had been largely spent in an intensive effort to resolve the urgent requirements of hemotherapy in La Paz, a general hospital with 2,000 beds. Once this was achieved we had to consider the crea tion of a hemophilia unit to supply compre hensive care for the large numbers of hemo philic patients that came for treatment, and for this we had a reasonable supply of plasma for cryoprecipitation. The responsibility and weight of this undertaking was born by a small group of individuals: Dr. José Miguel Cuesta Inclán, Director of La Paz, who spared no effort in personal or official assistance; José María García de Viedma, President of the Association of Blood Donors of La Paz,
La Paz Hemophilia Center, 1967-1992
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 1/24/2019 10:22:38 AM
‘There is properly no history, only biogra phy’, according to Emerson, and in fact I have decided to single out those persons who have been the protagonists of this tale. Not all will be included, I fear, and memory will have its failings, so I apologize for any omissions. ‘Every beginning is a consequence, every beginning ends something’ (Valéry). In this case the beginning was the culmination of 10 years spent in the General Hospital Faculty of Medicine, Ottawa, Canada (1955-1965), with Prof. R.K. Smiley of the Department of Medi cine where I saw my first hemophilia patient. Oslerian in character, Prof. Smiley I consider one of my two teachers in my career as a doc tor. His principles are quite simple, and I can recommend them to any young doctor at the start of his career: any task should be well done and, if possible, perfect; and remember what Osier considered the masterword in medicine: ‘What makes an undistinguished man useful, and a distinguished man brilliant - hard work!’ [1]. I remember that was what Dr. Smiley expected of one’s work - that it be perfect. There is much to be said for and against perfectionism, but in everyday work there are two things to be said: one that it is hard to find and the second that it always pays. We had collected a group of about 50 hemophiliacs in the Ottawa area, and these became very special patients whose problems, personal as well as clinical, soon had us ‘caught’. And I use the word caught intention ally remembering what the late Dr. A. Pavlowski said to me in 1975 when I asked him: ‘How is it that you have dedicated so much of your time to hemophilia?’ ‘Because I was caught by it’, he replied. To a greater or lesser extent, I believe this is what has happened to all those who have participated in this sympo sium. With reason. Prof. Ingram of London stated in an historical study of the disease: ‘The history of hemophilia shows the human
Social Activities - Creation of the Spanish Social Security Hemophilia Association
The Medical Hemophilia Group, the Di rection of La Paz Hospital, and the Blood Donors Association, all contributed to the formation of a Hemophilia Association within the Spanish Social Security. As other bodies of this kind in other countries, the aim of the Association was to bring to the notice of individuals, groups, and institutions the edu cational, professional, labor, and other prob lems of the hemophilic population. The sta tutes received final approval in 1971, and the legal domicile was La Paz Hospital. Prior to this, with provisional statutes drawn up by the medical staff of the Hematology Service, the Association had been accepted by and incor porated into the World Federation of Hemo philia at the World Congress in Moscow in 1969. At this point I should like to pay affec tionate tribute to Mr. Frank Schnabel who founded the World Federation of Hemophilia in 1963. This Organization has done so much in the past, and continues today, to care for the problems and comprehensive attention of hemophiliacs, worldwide. In this country, the Spanish Hemophilia Association - now the Spanish Hemophilia Federation - has played an important part in achieving social and medical benefits for the hemophiliac.
Medicosurgical Activities
In the second half of the 60s, blood sup plies in La Paz made it possible for us to use the necessary plasma to produce abundant cryoprecipitates of factor VIII to meet medi cal as well as surgical requirements. Some years nearly 20,000 cryoprecipitates were ob tained. Surgery had now become a safe proce dure for the hemophiliac, and with their in
301
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 1/24/2019 10:22:38 AM
whose philosophical convictions led him to consider all the innumerable hours spent in the organization of the Association as a sim ple service to society, and all the members of the Hematology and Hemotherapy Service who understood that supplying the extensive requirements for hemotherapy in the Hospi tal was the most urgent priority at that time. In this way, and with an adequate therapeutic capacity, we proceeded to set up what we called ‘La Paz Hemophilia Group’. We formed a group of various medical spe cialists, in hematology (hemostasia) - Drs. Ortega and Magallon were the pioneers in this Section - orthopedics and traumatology, re habilitation, odontology, radiology, and psy chology and set to work as a team, united by a common enthusiasm to bring all the benefits these specialities could afford to the welfare of the hemophiliac. It was a great step foward when the Hema tology Service was given a 20-bed hospitaliza tion unit in 1975, together with attached labo ratories, thanks to the good services of Dr. Cuesta Inclan, Director of La Paz, and Drs. Lamas Lopez and Martinez Estrada of the Institute of Public Health. Until then we had been obliged to make use of the ‘hospitality’ of Dr. Palacios Carvajal, Head of the Depart ment of Traumatology and Orthopedics, who was kind enough to allow his beds for the use of hemophilic patients. Today, with the same team work, we care for the daily needs of 465 hemophiliacs, together with 111 patients with von Willebrand disease. Before continuing with our history, let us place ourselves relatively in time. In 1967, when we formed our Hemophilia Group in La Paz, the Oxford Group had been in existence for 30 years. That would put us 30 years in arrears [3]. Let us now consider some of the more important medical and social land marks in these 25 years of the Hemophilia Center.
302
Martin-Villar
self-therapy using concentrates - with cryoprecipitates this was a difficult and risky busi ness - made it possible for the patient or his family to control and treat the disease at home. Not only did this relieve the constant necessity of hospital visits, but such indepen dence made it possible for the hemophiliac to travel and go on holiday and lead a relatively normal life. Prophylaxis made it possible to anticipate the disease; regular infusion of the necessary factor could prevent hemorrhages. Prophylaxis from infancy to 16 or 18 years of age gave these patients a new life; children with severe hemophilias could now lead the same life as other children of their age attend school regularly, practise sports, etc. Through experience and observation we found that it was possible to avoid almost completely the musculoskeletal problems of the hemophiliac. The use of lyophilized coag ulation factors brought resounding thera peutic success that constituted a culmination of the medical, social, economic, and political battles we had first started in 1967. Lowresponse inhibitors had been eliminated in many cases during prophylaxis, and highresponse ones were viewed optimistically something since corroborated by experience with treatments aimed at inducing immuno logical tolerance. An important landmark in the history of the progress in comprehensive treatment of the hemophilic patient was the World Federation of Hemophilia Congress in Bonn (co-organized by Dr. Brackmann, here with us today). In the psychosocial field progress has been immense. In very few years we witnessed pro found changes in the relations of these pa tients in personal, family, professional, and social aspects. In fact, the bonds that had lim ited their lives were eliminated with substitu tive therapy. At this point I have to recognize our debt of gratitude to the Pharmacy Service of the Hospital (Dr. Fernandez Caballero and
La Paz Hemophilia Center, 1967-1992
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 1/24/2019 10:22:38 AM
valuable services in this field, Drs. Soleto, Lis, and Barreiro have been witnesses to this. The Group of Orthopedic Surgery of Drs. J. Palacios and J. Pardo and later that of Drs. E. Galindo and C. Merchan were now able to repair the damage suffered by these patients in their locomotor system as a consequence of frequent articular and muscular hemorrhages. It goes without saying that the Department of Rehabilitation - Drs. Cano, Lopez Cavarcos, and others - played a large part in these suc cesses, not only with postoperative rehabilita tion, but also in the field of physiotherapy now made accessible with the use of thera peutic products. The Odontology Group, led by the late Dr. Verdu and Dr. Municio, were kept very busy, too, repairing all the accumu lated disorders of the past when dental proce dures were notoriously perilous. A long suffer ing and silent collaborator, then as now, has been the Radiology Group - Drs. A. Lorente and A. Perez Gil - assisting us constantly in our daily work, as is amply confirmed by the thousands of X-rays carefully conserved in our files. The decade of the 70s was a fascinating period of therapeutic success. For the first time in the history of the disease, there were sufficient supplies of coagulation factor con centrates obtained from human plasma, thanks to years of joint efforts by dedicated scientists and the pharmaceutical industry. Not only could we make use of these products in modes of therapy previously difficult or impossible to achieve, but the generosity of the Social Security Services made their use gratis for all hemophiliacs, whether or not they were covered by social security. I refer to such therapies - still now in use - as home treatment, self-therapy, and continuous pro phylaxis to combat those hemorrhages that are generally invalidating and to achieve im munological tolerance in the case of inhibi tors. The possibility of home treatment and
AIDS Confusion now hath made his masterpiece. Macbeth
As Dr. Magallon has treated this theme amply this morning, I shall not go into much detail. In June 1983 we had our 1st case of AIDS. The previous year and a half we had witnessed a high incidence of strange and illdefined clinical symptoms and viral infec tions. At that moment and in consideration of the confused situation then existing, we took two precautionary measures: prophylactic treatment was discontinued, and therapy was changed to heat-treated concentrates which then had recently appeared on the market. During the last few years, we have concen trated our clinical and epidemiological inter est on the following points which were pre sented in London in 1988 at a symposium on ‘The Present and Future of Haemophilia Care’ [4]: The extent of HIV infection amongst our patients; the secondary effects of concentrate therapy and their interrelation, in quantity and quality; the clinical evolution and treatment of patients with HIV infection; an evaluation of the psychological impact on the infected hemophilic population, and the pursuit of beneficial strategies in a situation that has presented many problems to the Psy chology Section. Our social worker has also had to cope with the new social and educa tional problems created by HIV infection. As may be supposed, the infection of a large proportion of the hemophilic patients with the HIV-1 virus has been a tragedy, for the patients and their families and for their doctors. But the situation existed and had to
be dealt with. First of all we decided that, as far as possible, we would be responsible for the primary care of the infected hemophiliacs, and this would be carried out in the Hemo philia Center, both in the case of hospitaliza tion and for outpatients. The same doctors who had for years cared for their problems derived from hemophilia would continue to do so with this new pathology. In order to be able to do this, we could count on the invalu able assistance of specialists in those fields most implicated in HIV. To these doctors I should like to express our sincere gratitude. There is no doubt that undertaking the constant care of HIV infection in hemophilic patients by doctors and nurses in a big hemo philia center is a decision that must be made by each group according to the circumstances. In our case the decision has been welcomed by the patients and their families, and it has helped to mitigate the shock suffered by those infected. It has also been a great help in evalu ating new and changing therapeutic proce dures. I have to emphasize the considerable increase in the work load and the emotional stress involved not only for doctors and psy chologists, but for all the members of the Hematology Service who attend these pa tients during their long stays in hospital. Hope that such catastrophes may be avoided in the future depends on the advances being evolved in therapeutic material and on new proce dures aimed at ‘curing’ hemophilia that have been so brilliantly dealt with during these ses sions. In the meantime, in my opinion all the objectives achieved in the fields of self-treat ment, prophylaxis, and immunological toler ance in the treatment of patients with inhibi tors remain valid today.
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others) for their constant support throughout these 25 years that has aided us in navigating the difficult sea of the choice of therapeutic concentrates.
From the early days, when the Hemophilia Unit was formed, we established relations with important international groups and cen ters who had been pioneers in the field of hemostasia. With the years these links have become closer and have developed into bonds of friendship and cooperation. Three scientific meetings on hemophilia have taken place in La Paz: in the years 1973, 1977, and 1981. Today I should like to take the opportunity of thanking once again all those who took part and especially those who took time and trouble to come from other countries. Many of those participants are here with us and have been presented: Drs. L.M. Aledort, M. Blombäck, H.H. Brackmann, P.M. Mannucci, H. Roberts, and M. Tezanos. We miss the company of Drs. J.P. Soulier and J.P. Allain of the National Transfusion Cen ter, Paris; Dr. Ahlberg of the Orthopedic Clinic of Malmö; Dr. P. Hoffmann, Bonn University Hospital; Dr. K. Schimpf of Hei delberg, and last of all two outstanding ladies; Dr. E. Bidwell of the Oxford Haemophilia Centre and the late Dr. J. Pool of Stanford
References
i Osler W: Aequanimitas : With other
2
304
University, California, who in her great mod esty attributed her discovery of cryoprecipitates to just ‘serendipity’. Lastly, I should like to dedicate a sincere tribute to Prof. Miguel Carmena Villarta, Va lencia University, who was my first professor of medicine. During his long career he has worked to inspire his students with the virtues of dedication, order, self-discipline, and affec tion for the patient. As this meeting has been organized to mark my retirement from the Hematology and Hemotherapy Service of La Paz in a few days, I should like to take the opportunity to bid farewell to all those with whom I have had professional relations during the last 25 years, within and outside the Hospital. To the mem bers of the Service, who have done so much in helping to create and maintain it, I say a par ticularly warm good-bye. Very many have passed through it, bringing or taking with them knowledge and experience - or both at the same time. I should like to tell them that I gave them what I could of myself, and yet remembering their collaboration and affec tion, I feel I have received more than I have given, and for this I am humbly grateful.
adresses to medical students, nurses and practioners of medicine. Phila delphia, Blakiston, 1904, pp 362— 388. Ingram GIC: The history of hemo philia. J Clin Pathol 1976;29:1-13.
Martin-Villar
3
4
Biggs R: Thirty tears of haemophilia treatment in Oxford. Br J Haematol 1967;13:452-463. Martin Villar J, Magallon M, Ortega F, Arranz P: The preent and future of haemophilia care. Proc Int Symp. Alpha Ther UK 1988:45-53.
La Paz Hemophilia Center, 1967-1992
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Scientific Meetings
J. Martin-Villar
La Paz Hemophilia Center,
Hematology Service, Hemophilia Center, La Paz Hospital, Madrid, Spain
1967- 1992: People, Events, and Memories
Key Words
Abstract
Hemophilia Hemophilia centers Comprehensive care AIDS
In 1967, once urgent blood requirements had been resolved in La Paz Hospital, Madrid, Spain, and adequate supplies of plasma for cryoprecipitation, therefore, being available, a He mophilia Unit (later Hemophilia Center) was set up to attend to the comprehensive care of hemophilia patients and patients with other coagulopathies. This Center covers a regional area and also a national area for referrals and normally attends 600 patients. In this Center the Spanish Social Security Hemo philia Association was created. In 1983 the first cases of AIDS appeared, occasioning the change from standard factor VIII concentrates to the heat-treated concentrates that had recently appeared on the market. At present the Center is responsible for the medical attention of those hemophilia patients affected by HIV.
It is nearly 26 years since I was appointed Head of the Service of Hematology and Hemotherapy of the La Paz Hospital. In a few days I shall be leaving the position, owing to what is generally termed ‘biological reasons’, in this case fortunately only age. A group of friends and collaborators decided to mark this occasion by organizing a symposium dedi
cated to the field of hemophilia, an area to which the Hemostasia Section - and the He matology Service in general - has dedicated much time and effort which does not imply that it has been its only activity nor that it is most important. In keeping with the theme of the symposium, then, I should like to relate briefly the short history of the Hemophilia Unit of La Paz, now known as La Paz Hemo philia Center.
J. Martin-Villar Hematology Service Hemophilia Center La Paz Hospital E-28046 Madrid (Spain)
© 1992 S. Karger AG, Basel 0301-0147/92/ 0225-0299S2.75/0
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 1/24/2019 10:22:38 AM
Introduction
300
Martin-Villar
mind attempting to define and encompass a mysterious yet fascinating phenomenon; and also the human heart responding to the chal lenge of repeated adversity’ [2], But let me return to our first group of hemophiliacs in Ottawa, in the 50s. In order to care for them we were obliged to reorganize our small coag ulation laboratory and to become slaves to ‘accuracy’ and ‘reproducibility’ and to main tain a fine tuning of some of those classical tests in the history of the disease, TGT, factor VIII, and inhibitor quantitation, while on the shelf stood our ‘Bible’ - ‘Human Blood Coag ulation’ of MacFarlane and Biggs. As for ther apy, good relations with the blood bank al lowed us to make limited quantities of freshfrozen plasma. I remember that patients would say, when referring to their treatment, that after frozen plasma came the dedication of their doctors and the love of their families. The last two are still just as important in the care for our patients at the Center.
Organization of a Hemophilia Unit for Comprehensive Treatment in La Paz: Autumn 1967
The years 1966 and 1967 had been largely spent in an intensive effort to resolve the urgent requirements of hemotherapy in La Paz, a general hospital with 2,000 beds. Once this was achieved we had to consider the crea tion of a hemophilia unit to supply compre hensive care for the large numbers of hemo philic patients that came for treatment, and for this we had a reasonable supply of plasma for cryoprecipitation. The responsibility and weight of this undertaking was born by a small group of individuals: Dr. José Miguel Cuesta Inclán, Director of La Paz, who spared no effort in personal or official assistance; José María García de Viedma, President of the Association of Blood Donors of La Paz,
La Paz Hemophilia Center, 1967-1992
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 1/24/2019 10:22:38 AM
‘There is properly no history, only biogra phy’, according to Emerson, and in fact I have decided to single out those persons who have been the protagonists of this tale. Not all will be included, I fear, and memory will have its failings, so I apologize for any omissions. ‘Every beginning is a consequence, every beginning ends something’ (Valéry). In this case the beginning was the culmination of 10 years spent in the General Hospital Faculty of Medicine, Ottawa, Canada (1955-1965), with Prof. R.K. Smiley of the Department of Medi cine where I saw my first hemophilia patient. Oslerian in character, Prof. Smiley I consider one of my two teachers in my career as a doc tor. His principles are quite simple, and I can recommend them to any young doctor at the start of his career: any task should be well done and, if possible, perfect; and remember what Osier considered the masterword in medicine: ‘What makes an undistinguished man useful, and a distinguished man brilliant - hard work!’ [1]. I remember that was what Dr. Smiley expected of one’s work - that it be perfect. There is much to be said for and against perfectionism, but in everyday work there are two things to be said: one that it is hard to find and the second that it always pays. We had collected a group of about 50 hemophiliacs in the Ottawa area, and these became very special patients whose problems, personal as well as clinical, soon had us ‘caught’. And I use the word caught intention ally remembering what the late Dr. A. Pavlowski said to me in 1975 when I asked him: ‘How is it that you have dedicated so much of your time to hemophilia?’ ‘Because I was caught by it’, he replied. To a greater or lesser extent, I believe this is what has happened to all those who have participated in this sympo sium. With reason. Prof. Ingram of London stated in an historical study of the disease: ‘The history of hemophilia shows the human
Social Activities - Creation of the Spanish Social Security Hemophilia Association
The Medical Hemophilia Group, the Di rection of La Paz Hospital, and the Blood Donors Association, all contributed to the formation of a Hemophilia Association within the Spanish Social Security. As other bodies of this kind in other countries, the aim of the Association was to bring to the notice of individuals, groups, and institutions the edu cational, professional, labor, and other prob lems of the hemophilic population. The sta tutes received final approval in 1971, and the legal domicile was La Paz Hospital. Prior to this, with provisional statutes drawn up by the medical staff of the Hematology Service, the Association had been accepted by and incor porated into the World Federation of Hemo philia at the World Congress in Moscow in 1969. At this point I should like to pay affec tionate tribute to Mr. Frank Schnabel who founded the World Federation of Hemophilia in 1963. This Organization has done so much in the past, and continues today, to care for the problems and comprehensive attention of hemophiliacs, worldwide. In this country, the Spanish Hemophilia Association - now the Spanish Hemophilia Federation - has played an important part in achieving social and medical benefits for the hemophiliac.
Medicosurgical Activities
In the second half of the 60s, blood sup plies in La Paz made it possible for us to use the necessary plasma to produce abundant cryoprecipitates of factor VIII to meet medi cal as well as surgical requirements. Some years nearly 20,000 cryoprecipitates were ob tained. Surgery had now become a safe proce dure for the hemophiliac, and with their in
301
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 1/24/2019 10:22:38 AM
whose philosophical convictions led him to consider all the innumerable hours spent in the organization of the Association as a sim ple service to society, and all the members of the Hematology and Hemotherapy Service who understood that supplying the extensive requirements for hemotherapy in the Hospi tal was the most urgent priority at that time. In this way, and with an adequate therapeutic capacity, we proceeded to set up what we called ‘La Paz Hemophilia Group’. We formed a group of various medical spe cialists, in hematology (hemostasia) - Drs. Ortega and Magallon were the pioneers in this Section - orthopedics and traumatology, re habilitation, odontology, radiology, and psy chology and set to work as a team, united by a common enthusiasm to bring all the benefits these specialities could afford to the welfare of the hemophiliac. It was a great step foward when the Hema tology Service was given a 20-bed hospitaliza tion unit in 1975, together with attached labo ratories, thanks to the good services of Dr. Cuesta Inclan, Director of La Paz, and Drs. Lamas Lopez and Martinez Estrada of the Institute of Public Health. Until then we had been obliged to make use of the ‘hospitality’ of Dr. Palacios Carvajal, Head of the Depart ment of Traumatology and Orthopedics, who was kind enough to allow his beds for the use of hemophilic patients. Today, with the same team work, we care for the daily needs of 465 hemophiliacs, together with 111 patients with von Willebrand disease. Before continuing with our history, let us place ourselves relatively in time. In 1967, when we formed our Hemophilia Group in La Paz, the Oxford Group had been in existence for 30 years. That would put us 30 years in arrears [3]. Let us now consider some of the more important medical and social land marks in these 25 years of the Hemophilia Center.
302
Martin-Villar
self-therapy using concentrates - with cryoprecipitates this was a difficult and risky busi ness - made it possible for the patient or his family to control and treat the disease at home. Not only did this relieve the constant necessity of hospital visits, but such indepen dence made it possible for the hemophiliac to travel and go on holiday and lead a relatively normal life. Prophylaxis made it possible to anticipate the disease; regular infusion of the necessary factor could prevent hemorrhages. Prophylaxis from infancy to 16 or 18 years of age gave these patients a new life; children with severe hemophilias could now lead the same life as other children of their age attend school regularly, practise sports, etc. Through experience and observation we found that it was possible to avoid almost completely the musculoskeletal problems of the hemophiliac. The use of lyophilized coag ulation factors brought resounding thera peutic success that constituted a culmination of the medical, social, economic, and political battles we had first started in 1967. Lowresponse inhibitors had been eliminated in many cases during prophylaxis, and highresponse ones were viewed optimistically something since corroborated by experience with treatments aimed at inducing immuno logical tolerance. An important landmark in the history of the progress in comprehensive treatment of the hemophilic patient was the World Federation of Hemophilia Congress in Bonn (co-organized by Dr. Brackmann, here with us today). In the psychosocial field progress has been immense. In very few years we witnessed pro found changes in the relations of these pa tients in personal, family, professional, and social aspects. In fact, the bonds that had lim ited their lives were eliminated with substitu tive therapy. At this point I have to recognize our debt of gratitude to the Pharmacy Service of the Hospital (Dr. Fernandez Caballero and
La Paz Hemophilia Center, 1967-1992
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 1/24/2019 10:22:38 AM
valuable services in this field, Drs. Soleto, Lis, and Barreiro have been witnesses to this. The Group of Orthopedic Surgery of Drs. J. Palacios and J. Pardo and later that of Drs. E. Galindo and C. Merchan were now able to repair the damage suffered by these patients in their locomotor system as a consequence of frequent articular and muscular hemorrhages. It goes without saying that the Department of Rehabilitation - Drs. Cano, Lopez Cavarcos, and others - played a large part in these suc cesses, not only with postoperative rehabilita tion, but also in the field of physiotherapy now made accessible with the use of thera peutic products. The Odontology Group, led by the late Dr. Verdu and Dr. Municio, were kept very busy, too, repairing all the accumu lated disorders of the past when dental proce dures were notoriously perilous. A long suffer ing and silent collaborator, then as now, has been the Radiology Group - Drs. A. Lorente and A. Perez Gil - assisting us constantly in our daily work, as is amply confirmed by the thousands of X-rays carefully conserved in our files. The decade of the 70s was a fascinating period of therapeutic success. For the first time in the history of the disease, there were sufficient supplies of coagulation factor con centrates obtained from human plasma, thanks to years of joint efforts by dedicated scientists and the pharmaceutical industry. Not only could we make use of these products in modes of therapy previously difficult or impossible to achieve, but the generosity of the Social Security Services made their use gratis for all hemophiliacs, whether or not they were covered by social security. I refer to such therapies - still now in use - as home treatment, self-therapy, and continuous pro phylaxis to combat those hemorrhages that are generally invalidating and to achieve im munological tolerance in the case of inhibi tors. The possibility of home treatment and
AIDS Confusion now hath made his masterpiece. Macbeth
As Dr. Magallon has treated this theme amply this morning, I shall not go into much detail. In June 1983 we had our 1st case of AIDS. The previous year and a half we had witnessed a high incidence of strange and illdefined clinical symptoms and viral infec tions. At that moment and in consideration of the confused situation then existing, we took two precautionary measures: prophylactic treatment was discontinued, and therapy was changed to heat-treated concentrates which then had recently appeared on the market. During the last few years, we have concen trated our clinical and epidemiological inter est on the following points which were pre sented in London in 1988 at a symposium on ‘The Present and Future of Haemophilia Care’ [4]: The extent of HIV infection amongst our patients; the secondary effects of concentrate therapy and their interrelation, in quantity and quality; the clinical evolution and treatment of patients with HIV infection; an evaluation of the psychological impact on the infected hemophilic population, and the pursuit of beneficial strategies in a situation that has presented many problems to the Psy chology Section. Our social worker has also had to cope with the new social and educa tional problems created by HIV infection. As may be supposed, the infection of a large proportion of the hemophilic patients with the HIV-1 virus has been a tragedy, for the patients and their families and for their doctors. But the situation existed and had to
be dealt with. First of all we decided that, as far as possible, we would be responsible for the primary care of the infected hemophiliacs, and this would be carried out in the Hemo philia Center, both in the case of hospitaliza tion and for outpatients. The same doctors who had for years cared for their problems derived from hemophilia would continue to do so with this new pathology. In order to be able to do this, we could count on the invalu able assistance of specialists in those fields most implicated in HIV. To these doctors I should like to express our sincere gratitude. There is no doubt that undertaking the constant care of HIV infection in hemophilic patients by doctors and nurses in a big hemo philia center is a decision that must be made by each group according to the circumstances. In our case the decision has been welcomed by the patients and their families, and it has helped to mitigate the shock suffered by those infected. It has also been a great help in evalu ating new and changing therapeutic proce dures. I have to emphasize the considerable increase in the work load and the emotional stress involved not only for doctors and psy chologists, but for all the members of the Hematology Service who attend these pa tients during their long stays in hospital. Hope that such catastrophes may be avoided in the future depends on the advances being evolved in therapeutic material and on new proce dures aimed at ‘curing’ hemophilia that have been so brilliantly dealt with during these ses sions. In the meantime, in my opinion all the objectives achieved in the fields of self-treat ment, prophylaxis, and immunological toler ance in the treatment of patients with inhibi tors remain valid today.
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others) for their constant support throughout these 25 years that has aided us in navigating the difficult sea of the choice of therapeutic concentrates.
From the early days, when the Hemophilia Unit was formed, we established relations with important international groups and cen ters who had been pioneers in the field of hemostasia. With the years these links have become closer and have developed into bonds of friendship and cooperation. Three scientific meetings on hemophilia have taken place in La Paz: in the years 1973, 1977, and 1981. Today I should like to take the opportunity of thanking once again all those who took part and especially those who took time and trouble to come from other countries. Many of those participants are here with us and have been presented: Drs. L.M. Aledort, M. Blombäck, H.H. Brackmann, P.M. Mannucci, H. Roberts, and M. Tezanos. We miss the company of Drs. J.P. Soulier and J.P. Allain of the National Transfusion Cen ter, Paris; Dr. Ahlberg of the Orthopedic Clinic of Malmö; Dr. P. Hoffmann, Bonn University Hospital; Dr. K. Schimpf of Hei delberg, and last of all two outstanding ladies; Dr. E. Bidwell of the Oxford Haemophilia Centre and the late Dr. J. Pool of Stanford
References
i Osler W: Aequanimitas : With other
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University, California, who in her great mod esty attributed her discovery of cryoprecipitates to just ‘serendipity’. Lastly, I should like to dedicate a sincere tribute to Prof. Miguel Carmena Villarta, Va lencia University, who was my first professor of medicine. During his long career he has worked to inspire his students with the virtues of dedication, order, self-discipline, and affec tion for the patient. As this meeting has been organized to mark my retirement from the Hematology and Hemotherapy Service of La Paz in a few days, I should like to take the opportunity to bid farewell to all those with whom I have had professional relations during the last 25 years, within and outside the Hospital. To the mem bers of the Service, who have done so much in helping to create and maintain it, I say a par ticularly warm good-bye. Very many have passed through it, bringing or taking with them knowledge and experience - or both at the same time. I should like to tell them that I gave them what I could of myself, and yet remembering their collaboration and affec tion, I feel I have received more than I have given, and for this I am humbly grateful.
adresses to medical students, nurses and practioners of medicine. Phila delphia, Blakiston, 1904, pp 362— 388. Ingram GIC: The history of hemo philia. J Clin Pathol 1976;29:1-13.
Martin-Villar
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Biggs R: Thirty tears of haemophilia treatment in Oxford. Br J Haematol 1967;13:452-463. Martin Villar J, Magallon M, Ortega F, Arranz P: The preent and future of haemophilia care. Proc Int Symp. Alpha Ther UK 1988:45-53.
La Paz Hemophilia Center, 1967-1992
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Scientific Meetings
