Haemophilia : the official journal of the World Federation of Hemophilia
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Currently, there is no single haemostasis laboratory test that has the capacity to accurately illustrate the clinical effects of procoagulant or anticoagulant interventions. Although the time course of thrombin generation in plasma and the endogenous thrombin potential (ETP) may be useful coagulation parameters, clotting involves components other than thrombin (e.g. platelets, fibrinogen). The continuous coagulation profiles of thrombelastography may provide a more accurate reflection of in vivo biology, covering initiation, development and final clot strength during whole blood clot formation. ⋯ Thrombelastography has been shown to reflect the clinical efficacy of activated prothrombin complex concentrate (aPCC) and recombinant activated factor VII (rFVIIa) in patients with haemophilia A with inhibitors and in patients with acquired haemophilia. Overall, tailoring laboratory assays to illustrate and correlate with clinical phenotypes is essential for effective coagulation monitoring. Applying an algorithm of preoperative, perioperative and postoperative tests, including thrombelastography, may enable physicians to achieve this.
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Preoperative and postoperative rehabilitation may be useful for improving the recovery of patients undergoing orthopaedic surgery, particularly in those with co-morbidity or special requirements. This case study, of a patient with haemophilia and inhibitors to factor VIII undergoing total knee replacement, demonstrates the benefits of 6 weeks' preoperative physiotherapy ('prehabilitation') combined with 6 weeks' postoperative rehabilitation. The supervised physiotherapy regimen was individually tailored to specifically increase range of motion and muscle strength, enabling rapid mobilization and recovery of function, whilst minimizing the risk of bleeding.
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Multicenter Study
Dental procedures in adult patients with hereditary bleeding disorders: 10 years experience in three Italian Hemophilia Centers.
Excessive bleeding after dental procedures are one of the most frequent complications occurring in patients with hereditary bleeding disorders. In this retrospective study we collected data from 10 years of experience in the oral care of patients with congenital haemorrhagic disorders in three Italian Hemophilia Centers. ⋯ We recorded 10 bleeding complications (1.9%), most of which occurred in patients with severe/moderate haemophilia A undergoing multiple dental extractions. Thus, our protocol of management of patients with hereditary bleeding tendency undergoing oral treatment or surgery has been shown to be effective in preventing haemorrhagic complications.
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Current challenges facing haemophilia care were identified and reviewed by an interdisciplinary group of experts in haemostasis and thrombosis, infectious disease, epidemiology, pharmacoeconomics and public health who met in February 2005 in Brussels. The outcome of this meeting was a series of consensus recommendations proposed to address the following three challenges: (i) developing the next generation of haemophilia specialists; (ii) reducing the risk that emerging pathogens present to safe haemophilia care and (iii) providing haemophilia care in an environment of cost constraint. It is intended that these consensus recommendations will form the basis of a concerted effort by leading haemophilia clinicians to secure future resources for the development and improvement of haemophilia care throughout Europe.
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In developing countries like India, where walking is the primary, preferred and feasible mode of transport, the implications following quadriceps weakness poses a serious threat to ones functional independence. This has been a challenge for professionals while ambulating individuals with haemophilia, where quadriceps weakness is very common. ⋯ It provides prolonged stretch to contracted tissue, allows for being used as a serial cast in improving the knee range and is cosmetically acceptable. Above all, it is user-friendly, thus enhancing compliance and superior outcome in haemophilic knee rehabilitation.