The profile of patients with haemophilia managed at a haemophilia treatment centre in Pretoria, Gauteng
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...
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AOSIS
2022-10-01
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| Series: | South African Family Practice |
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| Online Access: | https://safpj.co.za/index.php/safpj/article/view/5551 |
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| author | Lethukuthula Mafisa Abegail N. Dlova Vanessa Moodley |
| author_facet | Lethukuthula Mafisa Abegail N. Dlova Vanessa Moodley |
| author_sort | Lethukuthula Mafisa |
| collection | DOAJ |
| 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. |
| format | Article |
| id | doaj-art-7fca4edaff454125a38dfc6bfe2fdc50 |
| institution | DOAJ |
| issn | 2078-6190 2078-6204 |
| language | English |
| publishDate | 2022-10-01 |
| publisher | AOSIS |
| record_format | Article |
| series | South African Family Practice |
| spelling | doaj-art-7fca4edaff454125a38dfc6bfe2fdc502025-08-20T03:06:51ZengAOSISSouth African Family Practice2078-61902078-62042022-10-01641e1e710.4102/safp.v64i1.55514267The profile of patients with haemophilia managed at a haemophilia treatment centre in Pretoria, GautengLethukuthula Mafisa0Abegail N. Dlova1Vanessa Moodley2Department of Haematological Pathology, School of Medicine, Sefako Makgatho Health Sciences University, Pretoria, South Africa; and, Department of Haematological Pathology, Dr George Mukhari Academic Laboratory, National Health Laboratory Service, PretoriaDepartment of Haematological Pathology, School of Medicine, Sefako Makgatho Health Sciences University, Pretoria, South Africa; and, Department of Haematological Pathology, Dr George Mukhari Academic Laboratory, National Health Laboratory Service, PretoriaDepartment of Haematological Pathology, School of Medicine, Sefako Makgatho Health Sciences University, Pretoria, South Africa; and, Department of Haematological Pathology, Dr George Mukhari Academic Laboratory, National Health Laboratory Service, PretoriaBackground: 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.https://safpj.co.za/index.php/safpj/article/view/5551haemophiliahome therapyprophylaxiscomprehensive managementinhibitorscoagulation factor deficiency |
| spellingShingle | Lethukuthula Mafisa Abegail N. Dlova Vanessa Moodley The profile of patients with haemophilia managed at a haemophilia treatment centre in Pretoria, Gauteng South African Family Practice haemophilia home therapy prophylaxis comprehensive management inhibitors coagulation factor deficiency |
| title | The profile of patients with haemophilia managed at a haemophilia treatment centre in Pretoria, Gauteng |
| title_full | The profile of patients with haemophilia managed at a haemophilia treatment centre in Pretoria, Gauteng |
| title_fullStr | The profile of patients with haemophilia managed at a haemophilia treatment centre in Pretoria, Gauteng |
| title_full_unstemmed | The profile of patients with haemophilia managed at a haemophilia treatment centre in Pretoria, Gauteng |
| title_short | The profile of patients with haemophilia managed at a haemophilia treatment centre in Pretoria, Gauteng |
| title_sort | profile of patients with haemophilia managed at a haemophilia treatment centre in pretoria gauteng |
| topic | haemophilia home therapy prophylaxis comprehensive management inhibitors coagulation factor deficiency |
| url | https://safpj.co.za/index.php/safpj/article/view/5551 |
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