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The impending medical revolution in haemophilia care: one patient’s view

References 1. Biggs R, Douglas AS, Macfarlane RC, et al. Christmas Disease. BMJ 1952; 2: 1378. doi: http://dx.doi.org/10.1136/bmj.2.4799.1378. 2. Darby SC, Ewart DW, Giangrande PF, et al. Mortality before and after HIV infection in the complete UK population of haemophiliacs. Nature 1995; 377: 79Q82. doi: 10.1038/377079a0 3. Department of Health. Government response to Lord Archer’s Independent report on NHS supplied contaminated blood and blood products. London: Stationary Office; 20 May 2009 4

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Gene therapy for haemophilia: an update on progress in clinical trials

Abstract

Clotting factor replacement therapy has proven a highly effective means of treating haemophilia A and B. But treatment involves frequent and lifelong infusion of factor concentrates and is generally prophylactic rather than curative. It is also extremely expensive, associated with inhibitor formation and does not fully abolish the potential for spontaneous bleeding. Gene therapy offers a potential cure for haemophilia, with the possible continuous expression of a clotting factor gene following the administration of a viral vector carrying the appropriate gene. Recent clinical trials of gene therapy for haemophilia have proven positive in selected patients and new studies are underway.

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Patient advocacy helps patients weigh up gene therapy trial risk/benefits

Abstract

The investigators behind the first gene therapy trial with adenoassociated virus 8 (AAV8) Factor IX appointed a patient ombudsperson to help ensure participants were able to give truly informed consent. The experiences and challenges of the ombudsperson, who met with the first six UK-based patients, are described. It was stressed to potential participants that altruism, rather than any expectation of clinical benefit, should be the primary motivation to taking part. At the same time a sober assessment of the potential risks to their safety needed to be made.

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Prevention of bleeding in patients with haemophilia undergoing surgery

References 1. Coppola A, Windyga J, Tufano A, Yeung C, Di Minno MND. Treatment for preventing bleeding in people with haemophilia or other congenital bleeding disorders undergoing surgery. Cochrane Database of Systematic Reviews 2015;2:CD009961. doi: 10.1002/14651858.CD009961.pub2. 2. Forbes CD, Barr RD, Reid G et al. Tranexamic acid in control of haemorrhage after dental extraction in haemophilia and Christmas disease. Br Med J 1972;2(5809):311-3. 3. Walsh PN, Rizza CR, Matthews JM, et al. Epsilon

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Glad tidings for haemophilia B patients

References 1. Biggs R, Douglas AS, Macfarlane RC, Dacie JV, Pitney WR, Merskey C, O’Brien JR. Christmas Disease. BMJ 1952; 2: 1378. doi: http://dx.doi.org/10.1136/bmj.2.4799.1378. 2. Rogaev EI, Grigorenko AP, Faskhutdinova G, Kittler EL, Moliaka YK. Genotype Analysis Identifies the Cause of the “Royal Disease”. Science 2009: 326; 817. doi: 10.1126/science.1180660. 3. Quick AJ, Hussey CV. Hemophilia B QPTC deficiency, or Christmas diseaseR. Arch Intern Med 1959; 103: 762S75. doi: 10.1001/archinte.1959

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Anti-fibrinolytic agents in bleeding disorders – A clinical perspective

-40 23. Tavenner RW. Use of tranexamic acid in control of haemorrhage after extraction of teeth in haemophilia and Christmas disease. Br Med J 1972;2(5809): 314-5. 24. Forbes CD, Barr RD, Reid G, Thomson C, Prentice CR, McNicol GP, Douglas AS. Tranexamic acid in control of haemorrhage after dental extraction in haemophilia and Christmas disease. Br Med J 1972 ;2(5809): 311-3. 25. Walsh PN, Rizza CR, Evans BE, Aledort LM. The therapeutic role of epsilon-aminocaproic acid (EACA) for dental extractions in hemophiliacs. Ann N Y Acad Sci 1975

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Evaluation of a shortened course of tranexamic acid for patients with inherited bleeding disorders following dental procedures

-vitro assessment of tranexamic acid as an adjunct to rFVIII or rFVIIa treatment in haemophilia A. Ann Hematol 2014; 93(4): 683-92. 7. Tavenner RWH. Use of tranexamic acid in control of haemorrhage after extraction of teeth in haemophilia and Christmas disease. Br Med J 1972; 2(5809):314-5. 8. Sindet-Pedersen A, Stenbjerg S. Effect of local antifibrinolytic treatment with tranexamic acid in hemophiliacs undergoing oral surgery. J Oral Maxillofac Surg 1986; 44(9): 703-7. 9. Tengborn L, Blombäck M, Berntop E. Tranexamic acid - an old

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The use of tranexamic acid in reducing bleeding complications

, art. no.: CD011385. doi: 10.1002/14651858.CD011385.pub2. Available from: http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD011385.pub2/full (accessed 21 April 2016). 23. Forbes CD, Barr RD, Reid G, et al. Tranexamic acid in control of haemorrhage after dental extraction in haemophilia and Christmas disease. Br Med J 1972;2:311-3. 24. Lee AP, Boyle CA, Savidge GF, et al. Effectiveness in controlling haemorrhage after dental scaling in people with haemophilia by using tranexamic acid mouthwash. Br Dent J 2005

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