Search

Optimal haemophilia care is best established and implemented through a well‐coordinated plan guided by clearly defined principles and priorities. A document which enunciates those details is therefore important. A successful example of this approach is the definition of principles of haemophilia care (PHC) outlined by the European Association for Haemophilia and Associated Disorders (EAHAD) and also the World Federation of Hemophilia. A similar document applicable to the Asia‐Pacific region must take into account not only the highly varied healthcare systems but also the tremendous socio‐economic and cultural diversities which impact provision of such care. The Asia‐Pacific Haemophilia Working Group (APHWG), representing the countries in this region, has prepared this perspective of the PHC. While endorsing the overall framework outlined by EAHAD, this APHWG document emphasizes regional priorities on education and training of healthcare personnel in the diagnosis and management of hereditary bleeding disorders. Central coordinating agencies with wide stakeholder input, networks of haemophilia treatment centres and national registries as well as robust processes for procurement and distribution of safe and effective clotting factor concentrates (CFCs), implementation of prophylaxis programmes and management of patients with inhibitors should also be developed. The implementation of these strategies should lead to establishment of good comprehensive care programmes. This document should also be an advocacy tool to lobby for improved care for people with haemophilia (PWH) in the region. We urge national healthcare policy makers to consider these principles and initiate strong and decisive action to reach these goals. [ABSTRACT FROM AUTHOR]

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). Among the ten patients (type 2–5; type 3–5) who underwent major procedures, the pre-operative dose was 35 IU kg−1 of FVIII followed by 10–20 IU kg−1 once daily depending on FVIII:C levels. The mean total dose of FVIII used per procedures was 106 IU kg−1 (30–190) over a mean duration of 7 days (3–11). In this group, pre-infusion FVIII:C, VWF:Ag and VWF: ristocetin cofactor (RCoF) level that were 19.5% (1–64), 20 U dL−1 (0–96) and 12% (0–66) increased to 72% (54–198), 131 U dL−1 (68–206) and 68% (27–108) postinfusion, respectively. Sixteen minor procedures were performed in 11 patients (type 1–3, type 2–6, type 3–2). The preparative dose of FVIII was 10–20 IU kg−1. The average duration of factor support was 2 days (1–3) for a mean total dose of 23 IU kg−1 (9–60). The pre-infusion levels of FVIII:C, VWF:Ag and VWF:ristocetin cofactor (RCo) which were 31% (22–64), 25.5 U dL−1 (0–63) and 21% (0–76), respectively, increased to 76% (27–111), 73 U dL−1 (30–137) and 45% (2–106) postinfusion. 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. [ABSTRACT FROM AUTHOR]