Exploring the quality of life and care needs of children and families affected by sickle cell anaemia in Angola

Student thesis: Doctoral ThesisDoctor of Philosophy

Abstract

Sickle cell anaemia (SCA) is the most common and severe type of sickle cell disease (SCD) – a recessively inherited chronic blood disorder associated with increased childhood morbidity and mortality. Children, including adolescents, are particularly susceptible to physical and clinical complications that involve intensive medical care and frequent hospitalisations that can adversely impact the quality of life (QoL) of both the affected children and their caregivers and families. Although a significant body of research has previously shown evidence of the psychosocial burden of SCA, including in Sub-Sahara Africa (SSA), this did not appear to have previously been addressed in the literature concerning Angola. This PhD study was therefore designed to explore and address the gap in knowledge and understanding regarding the QoL and the care needs of children and families affected by SCA in Cabinda, Angola. Additionally, the study assessed the opportunities to improve the health care services and delivery for SCA in Angola.

The introduction chapter (Chapter 1) set out to establish, biomedical, psychosocial, and historical-epidemiological facts about the condition providing an overview of the underpinning foci of the study comprehensively described in Chapters 2-5. Chapter 2 highlights a systematic review and meta-analysis that analysed the incidence and prevalence of SCA globally, and how the rates in Angola compare to other countries. Four electronic databases (Pubmed, Medline, Embase, and Web of Science) were searched up to November 2018. Quality assessment was conducted using the Joanna Briggs Institute (JBI) Critical Appraisal Checklist for prevalence studies with statistical analysis conducted using Stata 14.0.

The impact of SCA on the health and wellbeing (QoL) of both affected children (Chapter 3) and caregivers and families (Chapter 4) was explored through the views of informal caregivers (ICGs). While a survey was used to assess and describe the health-related quality of life (HRQL) of the affected children, the financial and psychosocial burdens on the families were explored through a mixed-methods approach. 244. ICGs were recruited consecutively, using a convenient method, as they visited the sickle cell clinic in Cabinda for their children’s routine appointments. All ICGs who participated in the HRQL study also completed the survey regarding the psychosocial burden of disease on the families. Qualitative data were collected from a sample of ICGs (n=12) using a one-to-one interview to capture a broader insight into the impact of SCA. Similarly, health care experiences and needs were explored in-depth through the views of ICGs (Chapter 4) and health care providers (HCP) in Chapter 5. This exploratory assessment concerned with establishing whether the health care and support services available are sufficiently adequate to satisfy the care needs of people affected by SCA in Angola. HCPs were recruited using a purposive method.

Quantitative data from both the children and their caregivers were analysed using Stata 14.0 based on respective PedsQLTM SCD module and SCDBI questionnaires psychometric properties. Meanwhile, qualitative data were transcribed verbatim and transcripts analysed manually using a phenomenological thematic analysis approach. Themes and relevant sub-themes were identified into three categories.

Findings from the systemic review confirmed that the incidence rate of SCA was highest in endemic malaria regions, with Africa having the highest incidence rates compared to other regions. Although Angola had had the lowest incidence rate in the region, the estimates may not be representative of all cases of SCA in Angola due to maternity practices with not all children being routinely tested at birth.

Findings of the study on children (n=244 ICGs reporting on 260 children) revealed that HRQL, in terms of pain and hurt and emotions, significantly declined with increasing age of the child. However, communication abilities of the children with SCA improved significantly as they got older. Many socio-demographic factors influenced reporting of HRQL particularly age, family role, transport system used, and working status of the ICGs. Similarly, the mixed-methods study findings indicated that SCA is associated with an increased financial and psychosocial burden for caregivers and families. Lastly, ICGs and all participating HCPs (n= 7) identified a lack, or limited availability, of healthcare resources and services for a holistic care provision for SCA in Cabinda and Angola in general.

Overall, the main findings identified contextual factors affecting health care resources and services, informing intervention development and research aimed at improving the healthcare services and provision for SCA in Angola and SSA. Recommendations for practice and research focus mainly on further assessment of HRQL of children, community and HCPs knowledge and understanding of the disease and staff development.

This novel study identified funding opportunities and successfully secured an Externally Funded Research Grant from the Internal Pump Priming fund at the University of Aberdeen, Scotland, United Kingdom (UK). This internationally collaborative project aims to evaluate the opportunities and barriers to establishing an autonomous and functional SCA unit in Cabinda. Improvements in the management of SCA in Cabinda, and Angola in general, would help to address the poverty caused by the diseases (United Nations Sustainable Development Goal 1 (SDG 1) and the ability for children to receive equal educational opportunities (SDG 4).
Date of AwardJul 2021
Original languageEnglish
Awarding Institution
  • Queen's University Belfast
SponsorsThe Angolan Ministry of Higher Education & Universidade Onze de Novembro
SupervisorLesley Anderson (Supervisor), Ken Mills (Supervisor) & Olinda Santin (Supervisor)

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