The profile of patients with haemophilia managed at a haemophilia treatment centre in Pretoria, Gauteng

South African Family Practice

Field Value
Title The profile of patients with haemophilia managed at a haemophilia treatment centre in Pretoria, Gauteng
Creator Mafisa, Lethukuthula Dlova, Abegail N. Moodley, Vanessa
Subject family medicine; rural health; education haemophilia; home therapy; prophylaxis; comprehensive management; inhibitors; coagulation factor deficiency
Description Background: Haemophilia A and B are X-linked recessive bleeding disorders resulting from a deficiency of factors VIII and IX, respectively. Early diagnosis and a comprehensive approach to management is mandatory. This study aimed to describe the profile of patients with haemophilia (PWH) managed at Dr George Mukhari Academic Hospital (DGMAH) with the view to identify potential areas to improve haemophilia care.Methods: A cross-sectional, descriptive study that retrospectively reviewed clinical and laboratory records of PWH managed at DGMAH haemophilia treatment centre from 01 January 2003 to 31 December 2017.Results: Forty-four males were identified, with the majority being adults (~61%). Haemophilia A patients (~82%) outnumbered those with haemophilia B (~18%). Spontaneous mucocutaneous bleeding was the most frequent presenting feature followed by haemarthrosis. Disease-related complications included joint complications and life-threatening bleeds. There was a delay in diagnosis in 11% PWH. Management included episodic plasma-derived factor replacement and bypassing agents for patients with inhibitors. Only 13% of PWH were on home therapy. Prevalence of inhibitor development was 18%. There was a paucity of recorded data regarding prophylaxis, genetic counselling, psychological and physiotherapy support.Conclusion: The majority of PWH were adults, and haemophilia A was more prevalent than haemophilia B. A delay in haemophilia diagnosis could be addressed by increasing the awareness of haemophilia in health facilities. Expanding home therapy and introducing prophylaxis will likely improve the quality of life in PWH. Study outputs have included compilation of diagnostic and management algorithms to optimise haemophilia care at DGMAH.
Publisher AOSIS
Contributor Haemophilia Unit at Dr George Mukhari Hospital
Date 2022-10-13
Type info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion Peer-reviewed Article Survey
Format text/html application/epub+zip text/xml application/pdf
Identifier 10.4102/safp.v64i1.5551
Source South African Family Practice; Vol 64, No 1 (2022): Part 4; 7 pages 2078-6204 2078-6190
Language eng
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Coverage South Africa 2003-2017 Age; gender; Ethnicity; Haemophilia patients
Rights Copyright (c) 2022 Lethukuthula Mafisa, Abegail N. Dlova, Vanessa Moodley