Lee, C. A. ; Kessler, C. M. ; Varon, D. ; Martinowitz, U. ; Heim, M. ; Srivastava, A. ; Chuansumrit, A. ; Chandy, M. ; Duraiswamy, G. ; Karagus, C. (1998) Management of haemophilia in the developing world Haemophilia, 4 (4). pp. 474-480. ISSN 1351-8216
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Official URL: http://onlinelibrary.wiley.com/doi/10.1046/j.1365-...
Related URL: http://dx.doi.org/10.1046/j.1365-2516.1998.440474.x
Abstract
The problems with management of haemophilia in developing countries are poor awareness, inadequate diagnostic facilities and scarce factor concentrates for therapy. The priorities in establishing services for haemophilia include training care providers, setting up care centres, initiating a registry, educating affected people and their families about the condition, providing low-cost factor concentrates, improving social awareness and developing a comprehensive care team. A coagulation laboratory capable of reliably performing clotting times with correction studies using normal pooled, FVIII and FIX deficient patient plasma and factor assay is most essential for diagnosis. More advanced centralized laboratories are also needed. Molecular biology techniques for mutation detection and gene tracking should be established in each country for accurate carrier detection and antenatal diagnosis. Different models of haemophilia care exist. In India, there is no support from the government. Services, including import of factor concentrates, are organized by the haemophilia Federation of India, with support from other institutions. Haemophilia is managed with minimal replacement therapy (about 2000 i.u./PWH/year). In Malaysia, where the system is fully supported by the government, facilities are available at all public hospitals and moderate levels of factor concentrates are available 'on-demand" (about 11,000 i.u./PWH/year) at the hospitals. Haemophilia care in South Africa is provided through major public hospitals. Intermediate purity factor concentrates are locally produced (about 12,000 i.u./PWH/year) at low cost. The combined experience in the developing world in providing haemophilia services should be used to define standards for care and set achievable goals.
Item Type: | Article |
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Source: | Copyright of this article belongs to World Federation of Hemophilia. |
Keywords: | Haemophilia; Management; Developing; World |
ID Code: | 5774 |
Deposited On: | 19 Oct 2010 11:00 |
Last Modified: | 28 May 2011 06:46 |
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