Haemophilia Community Research Articles

Hemophilia: a story of success--disaster and the perseverance of the human spirit, Part 2.

IIn retrospect, it is now clear that the hemophilia holocaust began to take shape early in 1983, when people started getting sick at various hemophilia treatment centers around the country. It was in that year, upon visiting the Los Angeles Hemophilia Treatment Center, that I ran into my old friend, Steve Emerson. Just by looking at him, I could tell that he was not doing well. He looked very thin and sickly, and I was taken aback by how distant he seemed. That was the last time I saw Steve, as he became progressively sicker and died. His death was shocking because he was a close friend and one of the first at the Los Angeles treatment center to be struck by this new killer, now called Acquired Immune Deficiency Syndrome (AIDS). I remember the night I got the news that Steve had died. I sat alone all night grieving, while trying to understand what this meant for the rest of us with hemophilia. When I was tested for HIV in 1985 and was told that I was positive for the antibodies for HIV, it was one of those life-defining moments when everything one is as a person comes into question. On a visit to the treatment center in Los Angeles, I was asked by the staff nurses if I understood the meaning of my positive HIV test. I was then told that I would “probably be dead within 5 years.” When I failed to accept this perspective as the gospel, I was then accused of being in denial. I later found out that this scene was repeated at treatment centers across this country and that the message of death was being conveyed on a daily basis. With this interaction began my formal relation with what we, who had hemophilia, would later label the “hemophilia holocaust.” Between 1985 and 1989, I and others close to me attended many funerals and always stood around the grave wondering who would be next. We were all very angry with what had happened; however, we had not yet uncovered the reality of what had occurred. Many were left believing that this was a terrible accident that would kill us all. I was also consistently struck by the lack of services or discussion related to AIDS within the hemophilia treatment centers and the National Hemophilia Foundation (NHF). It was as if we were simply expected to quietly go into the darkness and die, even though we were looking at the death of more than half of our entire community. The early 1990s was a time of hopelessness and despair for the hemophilia community, with many people feeling that they had been abandoned by their doctors, their advocacy organizations, and the federal government. However, the seed of change was being planted in the Boston area by Jonathan Wadleigh and Tom Fahey. Seeing the need for advocacy and support for those with hemophilia who were HIV infected,

Hemophilia: A Story of Success—Disaster and the Perseverance of the Human Spirit. Part 1: Background and Overview

Until the 1980s, persons with hemophilia were a small, insular, and relatively unknown population within the United States. They generally lived quiet lives, attempted to blend in with the general population, and went about their lives while coping with a physically devastating illness in relative anonymity. Through the first eight decades of this century, hemophilia was an obscure, misunderstood, and basically unknown disease to the society at large. This situation remained true until the outbreak of the AIDS epidemic and of the mass infection of persons with hemophilia through the infusion of HIV-tainted blood products. It is estimated that 50% of persons with hemophilia are currently infected with HIV, and among those with the most severe form of hemophilia, more than 90% are HIV infected. Also, as a result of infusion of tainted blood products, more than 90% of all persons with hemophilia are infected with hepatitis C. This makes the hemophilia community the largest HIV/HCV coinfected group in the United States. There are two types of hemophilia. Of those with hemophilia, 80% have classic hemophilia (type A), in which the eighth factor in the clotting cascade is deficient or virtually nonexistent. The remaining 20% of persons with hemophilia have type B, in which the ninth factor in the clotting cascade is deficient. Within these two types of hemophilia, persons are classified as having severe hemophilia (60% of cases), moderate hemophilia (15% of cases), or mild hemophilia (25% of cases). These classifications are based on the severity of the factor VIII or factor IX deficiency that exists. The latest data from the Centers for Disease Control and Prevention report the incidence of hemophilia at 1 per 5,032 male births in the United States. Worldwide, the incidence of hemophilia is estimated to be 15 to 20 per 100,000 male births. Statistics from the hemophilia treatment centers indicate that there are currently 13,321 persons with hemophilia A and 3,638 persons with hemophilia B in the United States.

HEMOPHILIA: A New Approach to an Old Disease

The history of hemophilia diagnosis and therapy has been a turbulent one. We are coming full circle, back to the use of genetics as the main diagnostic tool for this disease. Therapeutically, the retroviruses that ravaged one generation of hemophiliac patients now may participate in the cure for the next generation. The hemophilia community hopes that the future of hemophilia care will follow a course guided by this modified quote from James Russell Lowell: "New times demand new measures, and men [and women]. As the world advances and in time outgrows the laws that in our fathers' [and mothers'] days were the best, doubtless after us some purer scheme will be shaped out by wiser man [and women] than we, made wiser by the steady growth of truth."

HEMOPHILIA 1996: New Approach to an Old Disease

The history of hemophilia diagnosis and therapy has been a turbulent one. We are coming full circle, back to the use of genetics as the main diagnostic tool for this disease. Therapeutically, the retroviruses that ravaged one generation of hemophiliac patients now may participate in the cure for the next generation. The hemophilia community hopes that the future of hemophilia care will follow a course guided by this modified quote from James Russell Lowell: "New times demand new measures, and men [and women]. As the world advances and in time outgrows the laws that in our fathers' [and mothers'] days were the best, doubtless after us some purer scheme will be shaped out by wiser men [and women] than we, made wiser by the steady growth of truth."

Counselling challenges in haemophilia and HIV infection/AIDS.

The advent of AIDS has had such a deep-reaching effect on the international haemophilia community that one can make a reasonable distinction between a pre-AIDS and a post-AIDS era in haemophilia management. In the context of counselling, however, talking about a 'before' and an 'after' in haemophilia does not (and, in our opinion, should not) necessarily imply a separation of the past from the present. Dealing with the psycho-social implications of haemophilia and HIV infection does not mean focusing exclusively on HIV-generated problems at the expense of haemophilia-related issues. Since the HIV crisis, counselling has posed the multiple challenge of: (a) assessing and alleviating the more immediate emotional effects of HIV infection; (b) paying due attention to the underlying influence of haemophilia on reaction, defence and coping; (c) formulating a flexible approach that is based on close cooperation with the medical staff and effective interpersonal communication with the counsellees.* In practice, the flexibility and effectiveness of the counselling model are promoted by means of: (a) ongoing counselling, (b) multiple counselling sites (i.e. the Haemophilia Centre and other appropriate locations), (c) interdisciplinary team-work, (d) respect for individual/ ethnic values, (e) maintenance of exo-empathy (i.e. neutrality), and (f) transfer of coping skills. The above framework can help maximize the effectiveness of counselling sessions through a personalized rapport of mutual trust and confidence between the counselling team and the counsellees.

The risk of suicide in people with haemophilia who are HIV infected.

The incidence of suicide in the HIV infected haemophilic population of the United Kingdom is low. Between 1985, when most patients were tested for HIV infection, and 1993 3 possibly HIV-related suicides were reported to Haemophilia Centre Directors. It is argued that haemophilia comprehensive care may contribute to reducing some of the factors associated with suicide risk, and thus be a reason for this low incidence. It is suggested that the pattern of health care delivery developed for the haemophilia community might serve as a template for those treating others with HIV infection.

Changes in longevity and causes of death among persons with hemophilia A

To examine recent changes in longevity and the causes of death among persons with hemophilia A, we evaluated death certificate data for persons who died in the United States from 1968 through 1989 and had hemophilia A or congenital Factor VIII disorder (ICD code 286.0) listed on the death certificate as one of the multiple causes of death. Multiple-cause-of-death mortality data for the United States from 1968 to 1989 were examined to compare death rates by year, focusing on death rates and causes of death for 1979-1981, 1983-1985, and 1987-1989. Gender, age group, race, geographic region, and median age at death of persons with hemophilia A and human immunodeficiency virus (HIV)-related disease listed as a cause of death were compared with those with hemophilia A without HIV-related disease. From 1968 through 1989, 2,792 hemophilia A deaths were reported. The death rate increased from 0.5 to 1.3 per 1,000,000 persons. From 1979-1981 through 1987-1989, mortality increased in all age groups above 9 years of age and age at death shifted markedly to lower ages. Median age at death decreased from 57 years in 1979-1981 to 40 years in 1987-1989. The percentage of deaths due to hemorrhage or diseases of the circulatory system decreased markedly as the result of the increase in deaths associated with HIV infection or infections other than HIV infection. Spread of HIV-1 infection in persons with hemophilia A has disrupted the reduction in mortality seen with factor replacement therapy, implementation of home care, and use of comprehensive hemophilia treatment centers. It is hoped that advances in the care of HIV-infected persons will improve survival in the hemophilia community.

The contributions of social support and coping methods to stress resiliency in couples facing hemophilia and HIV

The psychosocial impact of HIV on the hemophilia community has been largely ignored in the literature. The current study examined the contributions of social support and coping methods to resiliency against HIV-related stress among hemophilic men and their long-term female partners. Main effect and stress buffering models of stress resiliency were tested. Criterion variables included subjective psychological distress and physical symptoms. Results revealed support for the main effect model only. Marital satisfaction and support from friends predicted husbands' functioning and support from friends predicted partners' functioning. For both spouses, avoidant coping was associated with poorer functioning. The findings of this study and other research with this sample have provided the foundation for a clinical intervention which is summarized in an appendix.

A psycho-clinical investigation in adult hemophiliacs

The human immunodeficiency virus (HIV) has had a fundamental impact on quality of life, delivery of care, and directions of research efforts for the hemophilia community. Studies of psychosocial issues in hemophilia before and after the HIV crisis are reviewed. Before this crisis, research addressed personality factors and coping, family functioning, impact of psychological factors on bleeding, effects of home treatment, and school functioning. After HIV problems surfaced, research focused on psychosocial impact of the epidemic, HIV knowledge assessments, and HIV transmission prevention efforts. HIV-positive children with hemophilia have not received sufficient attention. Future directions for research are suggested.