Haemophilia : the official journal of the World Federation of Hemophilia
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Historical Article
Haemate P von Willebrand factor/factor VIII concentrate: 25 years of clinical experience.
Although von Willebrand disease (VWD) has a very long history, our understanding and treatment of the bleeding disorder has only evolved during the past 50 years or so. It was not until the 1920s that VWD was first recognized as a disease separate from that of classical haemophilia. It then took another 30 years before the first effective treatment was developed. ⋯ One of the key milestones in the evolution of the treatment of VWD was the development of Haemate P/Humate-P (CSL Behring) - the first virus-inactivated factor VIII plasma product. For 25 years, this concentrate has demonstrated excellent clinical efficacy and safety for patients with VWD and for those with haemophilia. This article provides an historical overview of the early landmark efforts to ensure a safe plasma-derived replacement product and outlines the clinical evolution in the use of Haemate P.
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Pain coping strategies are important influences on outcomes among people with painful chronic conditions. The pain coping strategies questionnaire (CSQ) was previously adapted for sickle cell disease and haemophilia, but those versions have 80 items, and a briefer version with similar psychometric properties would facilitate research on pain coping. The full-length haemophilia-adapted CSQ, plus measures of pain frequency and intensity, pain acceptance, pain readiness to change, and health-related quality of life were completed by 190 men with haemophilia. ⋯ Test-retest reliabilities were 0.73 for negative thoughts, 0.70 for active coping and 0.64 for passive adherence. Negative thoughts were associated with less readiness to change, less acceptance of pain and more impaired health-related quality of life, whereas active coping was associated with greater readiness to change and more acceptance of pain. The short form is a convenient brief measure of pain coping with good psychometric properties, and could be used to extend research on pain coping in haemophilia.
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Prophylaxis is defined as the regular administration of clotting factor concentrates to prevent bleeding. Extensive data from observational studies and a recent randomized controlled trial (have established that early prophylactic treatment prevents bleeds and arthropathy in boys with severe haemophilia. The initiation of prophylaxis in young children remains challenging. ⋯ The use of prophylactic treatment with bypassing agents in inhibitor patients is increasing. Early studies report in a significant reduction of bleeds, including intracranial bleeds, and improvement in quality of life. Data on results of primary prophylaxis in patients with inhibitors to prevent arthropathy are not yet available.
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A number of articles have investigated the outcomes of total knee arthroplasty (TKA) and causes of prosthetic failure in patients with haemophilic arthropathy. The aims of this retrospective study were to evaluate the clinical and functional outcomes of TKA and causes of prosthetic failure in patients with haemophilic arthropathy. A consecutive series of 35 TKA in 26 patients with haemophilic arthropathy were performed between November 1985 and October 2006 by one experienced surgeon. ⋯ It also showed great pain relief and significant functional gain. Under the circumstance of acceptable infection rate and complication, TKA is an effective method to achieve pain relief and gain better function in patients with haemophilic arthropathy of knee. The data of this study confirm those previously published by many authors.
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Clinical Trial
Successful surgical haemostasis in patients with von Willebrand disease with Koate DVI.
This report describes our experience with Koate DVI, a factor VIII (FVIII) concentrate containing von Willebrand factor (VWF) for surgery in patients with von Willebrand's disease (VWD). Twenty-one patients underwent 26 procedures, 10 of which were major and 16 were minor. The median age was 27 years (3-55) and the mean weight was 52 kg (16-88). ⋯ Whereas surgical haemostasis was achieved in all patients, minor postoperative bleeding occurred after one procedure in each group. Both were controlled with additional doses of factor replacement. We conclude that Koate DVI in modest doses provide adequate haemostasis for surgery in patients with VWD.