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Sickle cell disease in Cape Town: a perspective from two regional hospitals
Background: Inherited hemoglobinopathies are a global health burden. Sickle cell disease (SCD) is the most common genetic disorder of haemoglobin in Africa but is uncommon in the South African population. Objectives This study aimed to describe the presentation and experience managing paediatric pat...
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| Format: | Thesis |
| Language: | English |
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Department of Paediatrics and Child Health
2022
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| _version_ | 1867613542259097600 |
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| access_status_str | Open Access |
| author | Vahed, Anisa |
| author2 | van Eyssen, Ann |
| author_browse | Vahed, Anisa van Eyssen, Ann |
| author_facet | van Eyssen, Ann Vahed, Anisa |
| author_sort | Vahed, Anisa |
| collection | Thesis |
| description | Background: Inherited hemoglobinopathies are a global health burden. Sickle cell disease (SCD) is the most common genetic disorder of haemoglobin in Africa but is uncommon in the South African population. Objectives This study aimed to describe the presentation and experience managing paediatric patients with SCD at two regional hospitals in Cape Town. Methods This retrospective study used routine data of children aged 0-13 years with SCD managed at New Somerset and Victoria Hospitals in Cape Town from January 2010 to December 2018. Data analysed included demographics, diagnosis, out- and in-patient episodes, and the need for transfers to tertiary level. Results We identified 63 patients of which three did not fit the study criteria and four had missing clinical records. Of the 56 children included, most were diagnosed at the regional hospital (n=32 [58%]) and only a third (n=18 [32%]) were down-referrals from tertiary level facilities. The annual number of new patients per year varied with the most in 2009 and 2015. The median age at first presentation was 20.1 months (interquartile range [IQR] 7.1-43.9 months). There was a male predominance. The majority (n=39 [67%]) were born in South Africa, eleven were born in the Democratic Republic of Congo (DRC) and six in other African countries. None of the parents were born in South Africa. The majority of parents were from DRC (73/110) and Nigeria (11/110). Approximately one third (39%) of patients had a family history of SCD and a quarter (25%) had an affected sibling. Most children were diagnosed incidentally via full blood count findings (66%); others presented with symptomatic anaemia (28%) or were screened because of an affected sibling (11%). The mean number of hospital days was 3.0 (range 0-13). Infections, bone crises and symptomatic anaemia accounted for the majority of hospital admissions. Referral for tertiary level care occurred in 23/56 (41%) patients with most requiring specialist opinion from haematology and/or other specialist disciplines. Nine (16%) children were lost to follow-up, one was transferred to another province and one died at another institution. Conclusion An increasing number of children diagnosed with SCD are being seen in health facilities in Cape Town and probably other parts of South Africa due to migration and children being born to families with SCD ancestries. Recognising the presentation and complications of SCD and developing competency at all levels of care in providing appropriate, protocolised management are important to reduce morbidity and mortality among children with SCD in our setting |
| format | Thesis |
| id | oai:open.uct.ac.za:11427/36174 |
| institution | University of Cape Town (South Africa) |
| language | eng |
| last_indexed | 2026-06-10T12:37:48.225Z |
| license_str | Not specified — see source repository |
| provenance_str_mv | Harvested via OAI-PMH from UCTD — University of Cape Town Open Access Repository |
| publishDate | 2022 |
| publishDateRange | 2022 |
| publishDateSort | 2022 |
| publisher | Department of Paediatrics and Child Health |
| publisherStr | Department of Paediatrics and Child Health |
| record_format | dspace |
| source_str | UCTD — University of Cape Town Open Access Repository |
| spelling | oai:open.uct.ac.za:11427/36174 Sickle cell disease in Cape Town: a perspective from two regional hospitals Vahed, Anisa van Eyssen, Ann Westwood, Anthony Child health Background: Inherited hemoglobinopathies are a global health burden. Sickle cell disease (SCD) is the most common genetic disorder of haemoglobin in Africa but is uncommon in the South African population. Objectives This study aimed to describe the presentation and experience managing paediatric patients with SCD at two regional hospitals in Cape Town. Methods This retrospective study used routine data of children aged 0-13 years with SCD managed at New Somerset and Victoria Hospitals in Cape Town from January 2010 to December 2018. Data analysed included demographics, diagnosis, out- and in-patient episodes, and the need for transfers to tertiary level. Results We identified 63 patients of which three did not fit the study criteria and four had missing clinical records. Of the 56 children included, most were diagnosed at the regional hospital (n=32 [58%]) and only a third (n=18 [32%]) were down-referrals from tertiary level facilities. The annual number of new patients per year varied with the most in 2009 and 2015. The median age at first presentation was 20.1 months (interquartile range [IQR] 7.1-43.9 months). There was a male predominance. The majority (n=39 [67%]) were born in South Africa, eleven were born in the Democratic Republic of Congo (DRC) and six in other African countries. None of the parents were born in South Africa. The majority of parents were from DRC (73/110) and Nigeria (11/110). Approximately one third (39%) of patients had a family history of SCD and a quarter (25%) had an affected sibling. Most children were diagnosed incidentally via full blood count findings (66%); others presented with symptomatic anaemia (28%) or were screened because of an affected sibling (11%). The mean number of hospital days was 3.0 (range 0-13). Infections, bone crises and symptomatic anaemia accounted for the majority of hospital admissions. Referral for tertiary level care occurred in 23/56 (41%) patients with most requiring specialist opinion from haematology and/or other specialist disciplines. Nine (16%) children were lost to follow-up, one was transferred to another province and one died at another institution. Conclusion An increasing number of children diagnosed with SCD are being seen in health facilities in Cape Town and probably other parts of South Africa due to migration and children being born to families with SCD ancestries. Recognising the presentation and complications of SCD and developing competency at all levels of care in providing appropriate, protocolised management are important to reduce morbidity and mortality among children with SCD in our setting 2022-03-17T11:54:58Z 2022-03-17T11:54:58Z 2020 2022-03-17T11:50:56Z Master Thesis Masters MMed http://hdl.handle.net/11427/36174 eng application/pdf Department of Paediatrics and Child Health Faculty of Health Sciences |
| spellingShingle | Child health Vahed, Anisa Sickle cell disease in Cape Town: a perspective from two regional hospitals |
| thesis_degree_str | Master's |
| title | Sickle cell disease in Cape Town: a perspective from two regional hospitals |
| title_full | Sickle cell disease in Cape Town: a perspective from two regional hospitals |
| title_fullStr | Sickle cell disease in Cape Town: a perspective from two regional hospitals |
| title_full_unstemmed | Sickle cell disease in Cape Town: a perspective from two regional hospitals |
| title_short | Sickle cell disease in Cape Town: a perspective from two regional hospitals |
| title_sort | sickle cell disease in cape town a perspective from two regional hospitals |
| topic | Child health |
| url | http://hdl.handle.net/11427/36174 |
| work_keys_str_mv | AT vahedanisa sicklecelldiseaseincapetownaperspectivefromtworegionalhospitals |