"It was the best of times, it was the warst of times, it was the age ofwisdom, it was the age of foolishness, U was the epoch of belief, it was the epoch of incredulity, it was the season of Light, it was the season ofDarkness, it was the spring ofhope, it was the winter ofdespaii; we had everything before us, we had nothing before us, we were all going direct to Heaven, we were all going direct the

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Hemophilia treatment in historical perspective: a review of medical and social developments

The concepts of “identity” and “representation” have had a long history both in mundane and philosophical thought; over aeons of time, they have both retained some stable characteristics, but they have also changed. Questions like “who am I?” “who are we?” and “who are they?” as well as “what do we know about the world and how do we represent it?” have been everlasting. However, answers to these questions have been continuously changing throughout history.

Social Identities and Social Representations

Providing comprehensive care, counselling and support to haemophilic patients, and their parents have always been quite complex for haemophilia treatment centres. Nowadays, starting with recent developments in genetic counselling, prenatal diagnosis and carrier testing, the psychological burden on patients and parents might possibly have increased, compared with even the recent past. The emotional strains and worries associated with a possibly affected newborn and his care through childhood and adolescence may also have a grievous impact on couple dynamics and marital relationship. The impact may be even higher in families in which haemophilia is newly diagnosed. The main psychological problems faced by parents and then by affected individuals are herein chronologically reviewed, starting from genetic counselling before conception through childhood, adolescence and adulthood. Aware of the psychosocial burden on patients and their families associated with haemophilia, from prenatal diagnosis and carrier testing until later stages of life of the affected individual, a board of Italian haemophilia specialists and psychologists is designing and organizing an innovative network of psychological support services in some Italian haemophilia centres and promoting specific educational programmes in this setting.

Confronting the psychological burden of haemophilia

The research study described in this chapter forms part of a larger study on ‘Factors affecting labour pain’ (Niven and Gijsbers, 1984; Niven, 1985; 1986) and was particularly concerned with the role played by psychological factors in modulating labour pain. The study was informed by current theories of pain perception, such as the gate theory (Melzack and Wall, 1965; 1988), which predict that psychological factors as well as physiological ones can affect pain perception. Accordingly the study examined the effects of obstetric factors such as parity, induction of labour, duration of labour and weight of the baby as well as the effects of psychological factors such as the desirability of pregnancy, expectations of childbirth, antenatal education, and the presence of the partner during labour. Other factors examined included analgesic use (pethidine and Entonox), social class and previous pain experience. These factors were examined in the first phase of the study which involved 101 labouring women. In the second phase of the study the coping strategies of 51 of these women were examined in some detail.

Coping with labour pain: the midwife’s role

Summary. In a prospective study, we tested the hypothesis that an already reduced quality of life in haemophilia patients is further diminished in those haemophilia patients who contracted the human immunodeficiency virus (HIV) as a result of transfusion of coagulation factor preparations. From an available pool of 92 males with haemophilia A or B, 18 patients seropositive for HIV infection and 11 seronegative patients were randomly selected for the study.



We applied two instruments to measure the quality of life (QOL) in our patients. The first instrument was the quality of well-being (QWB) scale that unifies QOL into a single score based upon an assessment of the patient's symptoms and health-related reductions in mobility, physical activity and social activity. The second instrument was SF-36, the questionnaire from the Medical Outcome Study (MOS) that measures six dimensions of health status (physical functioning, role functioning, social functioning, pain, mental health and health perception). Measurements were obtained with both instruments at three interviews with each patient over a 1-year interval.



As expected, HIV disease reduces QOL in haemophiliacs. The number of bleeding episodes within 2 months of interview was increased in the HIV-positive cohort but not within 6 days of interview, indicating that HIV disease independently affects QOL in haemophilia patients. In a typical 30-year-old patient, haemophilia itself has reduced quality of their lives by 9.3 years, and HIV disease additionally from 8.5 to 20 years. On the MOS scales, the two patient groups differed significantly only in the dimensions of health perception and pain magnitude. Although HIV disease led to a decrement in QOL of haemophilia patients, it also appears that haemophilia patients are able to develop coping skills to prevent more drastic effects of HIV disease on their QOL. Future studies will need to explore the nature and mechanisms of this ‘buffering’ effect.

Comparison of the quality of life between HIV-positive haemophilia patients and HIV-negative haemophilia patients.

Semi-structured interviews concerning various aspects of mental health were carried out with 13 employed and 13 unemployed haemophiliacs 20-35 years old. Significant differences in patients' responses were related to their employment status and the degree of severity of haemophilia. Unemployed patients with milder forms of haemophilia appeared to be least adjusted and suffered most from psychosomatic symptoms.

Ruth Lockyer

Papers

Self-perception of employed and unemployed haemophiliacs