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This study outlines the development and evaluation of a structural equation model for establishing the consequences of haemophilia. The hereditary disorder is characterized by a high tendency to haemorrhages, with recurrent bleeding into the joints causing irreversible joint damage. The model is, in general, an attempt to answer the following questions: what is the effect of haemophilia on the wellbeing (i.e. satisfaction, health, somatic complaints and self-esteem) of patients and what is the additional or mediating role of other individual characteristics in this pathway? Disease severity, joint impairment and disability are defined as antecedents of well-being and the mediating roles of appraisal (i.e. the personal evaluation of the disease), health beliefs (i.e. locus of control), psychological characteristics (i.e. anxiety, anger, depression and optimism) and social support are investigated. Psychological variables turned out to be the strongest determinants of well-being and partly mediated the detrimental effect of disability on well-being. The role of appraisal remained somewhat unclear, as no significant relationship was established between this personal evaluation of haemophilia and well-being. Nevertheless, appraisal very well reflected the level of disability. An internal locus of control and favourable psychological characteristics appeared to reduce the perceived seriousness of haemophilia. No evidence was found for social support to act as a mediator between disability and well-being. The perception of support did show moderately strong associations with psychological characteristics (i.e. anxiety and depression) and satisfaction ratings. The study merits further research on quantifying the relationships between clinical parameters and psychosocial outcomes in patients with a chronic disease. Key words - haemophilia, well-being, disability, psychological characteristics, social support

The best dosage of anticlotting drugs for patients with artificial heart valves may be one that produces an international normalized ratio (INR) of coagulation between 2.5 and 4.9. Researchers analyzed the medical records of 1608 patients with artificial heart valves. The patients received coumarin derivative anticlotting drugs. The total follow-up time was 6475 patient-years, at an average of 4 years per patient. For patients with a INR between 2.5 and 4.9 the rate of blood vessel blockage or bleeding was about 2 per 100 patient-years. At INR levels below 2.5 the incidence of clotting increased sharply. At INR levels above 4.9 the incidence of hemorrhages increased sharply. An INR between 3.0 and 4.0 may be the best target in administering the drugs.

A retrospective study was performed to explore carrier testing among women who were possible or obligate carriers of the haemophilia gene. Knowledge of the possibility of carrier testing and use of carrier testing were studied separately. In our exploration we were guided by the diffusion theory and the Health Belief Model. Logistic regression analysis showed that four factors were statistically significant related to knowledge of carrier testing: information via mass media, a haemophilic relative in the nuclear family rather than in the extended family, medical severity of haemophilia, and information from the patients' organization. For those women acquainted with carrier testing two of the four factors just mentioned were significantly related to the utilization of carrier testing i.e. having a haemophilic relative in the nuclear family and the medical severity of the haemophilia. In addition the following factors were associated: attitude towards abortion because of haemophilia, educational level, and marital status. Notwithstanding the prominent function of the mass media and the patients' organization, the respondents themselves stated that relatives, especially parents and sisters, were the most important source of information on genetic counselling and carrier testing.

Several observational studies have found that anticoagulants should be given to people with atrial fibrillation at a dosage that leads to an international normalized prothrombin-time ratio (INR) between 2 and 4. People with atrial fibrillation have a high risk of stroke and must take anticoagulants to reduce this risk. However, too much anticoagulation could lead to excessive bleeding. One study found that the risk of stroke increased as the INR dropped below 2. Others have found the risk of bleeding increases as the INR rises above 4.

A study was performed to determine whether improvements in the treatment of haemophilia over the past 20 years have influenced the prospects of these patients in the labour market. Surveys on the medical and social situation of haemophiliacs in The Netherlands were carried out in 1972, 1978 and 1985. Most of the patients participated in these surveys. Trends in employment do not show either an increase in the number of employed haemophiliacs or a decrease in the number administratively defined as disabled. However, considering the influence of the economic recession on the position of the chronically sick on the labour market and the rise in the number administratively defined as disabled. However, considering the influence of the economic recession on the position of the chronically sick on the labour market and the rise in the number administratively defined as disabled in the Dutch population, haemophiliacs perform well. Sick leave has decreased considerably. Although the employment rate for the group of haemophiliacs is lower than that for the general male population, the level of employment in relation to educational achievements is high and most of the employed do not feel limited in the daily job activities by the haemophilia. Physical mobility is a main factor influencing the employment status but other factors, such as the type of occupation or former occupation and prejudice against people with haemophilia, have to be considered.