ABSTRACT
Introduction
Sickle cell disease is a genetic autosomal blood disorder that causes red blood cells to turn rigid and crescent shaped. The condition ultimately results to several complications leading to organ damage. This study aimed at assessing attitudes and practices of unmarried adults towards sickle cell disease.
Methods
A cross sectional study was undertaken in the Nigerian Federal Capital Territory. Data were collected using a well-structured questionnaire. Descriptive and inferential statistics were undertaken.
Results
Male and female participants were of a similar proportion as represented by 52% and 48%. The overall mean score for attitude towards sickle disease was 6.60 ± 2.583 (range; 0–11). A higher attitude score was observed among female participants (p = 0.012), older participants reported a better attitude towards sickle cell disease (p < 0.001), and level of education also influenced perception towards the disease (p < 0.001). Three-quarters of the participants (73.4%) supported the need for contextual legislation to prohibit marriage between two sickle cell carriers. More than one-tenth of the sample (14.4%) indicated that they would marry someone with sickle cell disease irrespective of their own genotype. Whilst two-thirds of the study participants (67.1%) indicated that they would prioritise sickle cell screening in their relationships, a third of them (33.3%) expressed a contrary standpoint.
Conclusion
Findings emanating from this research can guide Government and other critical Stakeholders in developing contextual policies and practices that will reduce the burden of sickle cell disorder.
Introduction
Sickle cell disease refers to a group of blood disorders inherited from a person’s parents [Citation1]. The most common form is known as sickle cell anaemia. It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. This consequently leads to a rigid sickle-like shape under certain conditions. Sickle cell disorder alongside its health system consequences has remained a condition of international concern [Citation2,Citation3], with especially significant public health implications for Africa [Citation4].
Globally, the prevalence of the disease is highest in sub-Saharan Africa, with Nigeria saddled with the highest burden of carrier prevalence ranging from 25% to 30% [Citation5,Citation6]. Furthermore, in the country, sickle cell anaemia occurs in approximately 3% of all births [Citation7–Citation9]. Some of the common characteristics of sickle cell disease include chronic haemolytic anaemia and recurrent vaso-occlusion, with the latter being responsible for painful crises associated with the disease. Another common feature of the condition is chronic vasculopathy triggered by free heme resulting in nitric oxide scavenging and upregulation of adhesion molecules in reticulocytes neutrophils and endothelial cells [Citation10,Citation11]. One of the major causes of mortality among children is overwhelming bacterial infections occurring due to encapsulated organisms, particularly pneumococcus [Citation12Citation13]. Other common causes of death include splenic sequestration [Citation14], acute chest syndrome [Citation15], stroke [Citation16,Citation17] and multiple organ failure [Citation18]. There is therefore substantial evidence that the disease constitutes a high mortality risk for under-five sickle cell disease patients who live in areas without adequate access to healthcare [Citation19]. This is further complicated by the fact that empirical evidence now correlates malaria episodes with increased risk of crises, alongside the possible morbidity and mortality associated with these events [Citation20,Citation21].
The perception of people towards a particular disease can influence their decision-making processes regarding the development of preventive mechanisms. Despite the large number of people affected with sickle cell disease in Nigeria, there is some evidence of negative attitudes amongst the populace [Citation22]. This has necessitated the call for more emphasis on health education through programmes promoting sickle cell education. Other interventions identified in the literature, include the development of multifaceted education programmes as well as the intensification of screening for the control of sickle cell disease, particularly during routine hospital visits and premarital medical examinations [Citation23]. Currently however, newborn screening facilities for sickle cell disease and other genetic disorders are lacking in Nigeria, as a result, diagnosis is usually made during hospitalisation for childhood ailment or when serious life-threatening complications occur. Evidence suggests that cost-effective strategies to reduce the burden of sickle cell disease include primary prevention of the disease through public education and health awareness. It therefore means that a lack of appropriate attitude and practice towards sickle cell disease can result in increased prevalence of the condition as well as worsening the quality of life amongst persons living with sickle cell disorder [Citation24–Citation26]. With a better understanding of various critical demographies’ attitudes and practices, some of these interventions can help save lives. From a public health perspective, this is especially important in individuals that are yet to procreate. So far however, knowledge gap exists in area of these concepts amongst this demography, who arguably, are the most critical for the condition. This study therefore aimed at assessing attitudes and practices of unmarried adults towards sickle cell disease in Federal Capital Territory, Nigeria.
Methods
A cross sectional design was adopted by this study which was undertaken in the capital city of Nigeria in August 2021. The data collection instrument was adapted from similar studies [Citation1,Citation27], with little modifications on some of the items. The questionnaire validation process was carried out using an expert panel comprising faculty members that are engaged in teaching and research activities in this field. Face and content validation were undertaken, this was then followed by pilot testing. The questionnaire was administered to an initial cohort of 40 participants that were randomly selected, the feedback received did not necessitate changes in the data collection tool.
The participants were selected using stratified random sampling across different districts in Federal Capital Territory. In each district, a number of participants were recruited using convenience sampling technique [Citation28]. Strategic locations which include motor parks, worship centres, and corporate offices were included for data collection. Inclusion criteria included marital status and residency in the Federal Capital Territory. Participants were asked to indicate their marital status prior to questionnaire administration, and those that were married were excluded from the study.
Ethical approval was obtained from Federal Capital Territory Health Research Ethics Committee before the commencement of data collection. Participation in the study was voluntary. Confidentiality and anonymity were strictly maintained throughout the data collection process, as all information that could link participants to their responses was not included in the data collection instrument. Following the retrieval of questionnaires from participants, data collected were entered into Statistical Package for Social Sciences version 25. Descriptive statistical analyses were undertaken and results were presented as frequencies (n) and percentages (%). Attitude towards sickle cell disease was assessed by responding to 11 statements. Higher scores reflected a positive attitude towards prevention. The calculation of a total cumulative attitude score was carried out for each participant. Questions were assigned one (1) point for positive attitude towards preventing the disease, and zero (0) was assigned for unanswered or negative feedback. The maximum score each participant can accumulate was 11, and the minimum was 0.
Student’s t-test and Analysis of variance (ANOVA) were undertaken to determine the association between mean attitude score and socio-demographic characteristics. Post hoc analysis (LSD) was performed in cases of significant ANOVA test for multiple comparisons. A p-value of 0.05 or less represented the threshold for statistical significance.
Results
Demography
A total of 1700 questionnaires were administered, and 1423 were completed and returned. Response rate was 83.71%. Male and female participants were of a similar proportion as represented by 52.0% and 48.0%, respectively. About half of the respondents (47.9%) were between 21 and 30 years of age, whilst a third of the sample (31.6%) was 20 years and below. Majority of the participants (83.6%) had knowledge of their genotype. Other relevant details about socio-demographic characteristics are presented in .
Table 1. Socio-demographic characteristics.
Attitude towards sickle cell disease
The attitude score towards prevention of sickle cell disease ranged from 0 to 11, with a total mean of 6.60 ± 2.583. Those that had a higher mean score were considered to have positive attitude towards preventing sickle cell disorder.
Whilst findings in this study showed that three-quarters of the participants (73.4%) were of the view that a law should be enacted to prohibit marriage between 2 sickle cell carriers, only a quarter (26.8%) of the respondents however indicated that abortion of an unborn sickle cell disease child should be encouraged by law. Furthermore, majority of the sample (73.2%) were against marriage between 2 sickle cell carriers. Other relevant details relating to attitude towards sickle cell disease are presented in .
Table 2. Attitude towards sickle cell disease.
Practice
Two-thirds of the study participants (67.1%) indicated that they prioritise sickle cell screening in a relationship, whilst a similar proportion (69.5) were willing to undertake relevant screening for sickle cell trait as a prerequisite for marriage. Further details about practice towards sickle cell disease are presented in .
Table 3. Practice relating to sickle cell disease.
Association between demographic characteristics and attitude about sickle cell disease
Inferential statistical analyses undertaken show that attitude towards sickle cell disease was influenced by socio-demographic characteristics of the participants. Female participants reported higher attitude scores compared to their male counterparts (p = 0.012), older respondents also had better attitude towards sickle cell disease prevention (p < 0.001). Additionally, the level of education attained by the participants emerged as having a significant influence on their attitude (p < 0.001). Further details are presented in .
Table 4. Inferential statistical analyses.
Discussion
In this study, it emerged that majority of the participants demonstrated a positive attitude towards preventing sickle cell disease. This finding was similar to previous research undertaken in North-central Nigeria [Citation29] and Saudi Arabia [Citation30]. Interestingly however the findings contrasted the report from a study carried out in South-western Nigeria where majority of the participants had negative attitude towards the disease [Citation22]. This clearly signifies the urgent and critical need for a nationwide study to better understand factors influencing these emergent geographical variations.
Whilst three-quarters of the respondents in this study supported that a law should be enacted to prohibit marriage between two sickle cell carriers, only a quarter of the participants were of the opinion that abortion of unborn sickle cell disease foetus should be encouraged by law. Although this preventive approach may significantly reduce the burden of sickle cell disease, it however raises some ethical concerns [Citation31].
Whilst selective abortion as a method of control of the disease is popular elsewhere [Citation32,Citation33], findings from this study suggest a potential lack of acceptance within the Nigerian setting. These novel findings provide key contextual insights into sociocultural influences underpinning perceptions associated with the disease. Prevention of sickle cell disorder through public education and genetic counselling regarding reproductive choice may therefore be considered a more contextual option.
Three-quarters of the respondents indicated that undergoing sickle cell testing of all family members is important, and it also emerged that a considerable proportion of the participants were not ready to marry someone with sickle cell disease. Although this may be due to the financial burden associated with the management of sickle cell disorder [Citation34,Citation35], it also raises concerns about the potential for discrimination in this setting.
Though, more than half of the participants indicated that they would end their relationship if they discovered that their genotypes predisposes them to having children with sickle cell disease, one-fifth of the participants indicated otherwise, regardless of incompatibility. This study’s findings reflect evidence in the extant literature [Citation36,Citation37], and go further to provide contemporary insights as to specific areas for targeted enlightenment.
Findings from this study revealed that female participants reported higher positive attitude towards prevention of sickle cell disease compared to their male counterparts. The reason behind this is not clearly understood and this phenomenon can be explored in further studies, in order to unravel this correlation.
Also, findings in this study showed that attitude towards prevention of sickle cell disorder improved as age increases, and this may be related to their knowledge regarding the disease, as it is expected that older persons would have had better knowledge about the condition which can in turn affect their perception concerning this phenomenon.
Another interesting finding regarding attitudes towards prevention of sickle cell disease was the influence of educational status. It emerged that people who were more educated seemed to have a more positive perception towards the disease. This was however more pronounced at the lower educational levels, since no statistical significant difference was observed between those with first degrees and postgraduate qualifications. Findings from this study further revealed that those who knew their genotypes reported higher attitude scores towards the disease prevention, and this could be as a result of the counselling received at the point of testing. Similarly, respondents who indicated that they had a relative or friend affected by the disease registered a more positive attitude towards the disease prevention.
More than a third of the study participants indicated that they do not associate with persons with sickle cell disorder, whilst close to a quarter indicated that they had discriminated against people with the condition in the past. These findings suggest a significant level of stigma towards the disease, and is in line with previous studies that reported discrimination and stigmatisation among persons affected with the disease [Citation38–Citation40]. Evidence exists which identifies stigma as a barrier to disease eradication [Citation41]. This perturbing revelation from this study therefore signals the need for a review of engagement policies to ensure that stigma prevention strategies are embedded in disease eradication programmes.
Whilst this study design focused on unmarried individuals, undertaking a comparison of attitudes towards sickle cell disease between married and single adults can be invaluable in providing further insights in this area. However, this was not part of the survey, which is a potential limitation. Further comparative study is therefore recommended.
Conclusion
This study reported considerable positivity in attitude and practice relating to sickle cell disease prevention. It was however revealed that significant gaps remained in certain aspects critical to the control and prevention of the disease, such as a disregard for sickle cell screening prior to relationships as well as a willingness to marry amongst individuals with genotype incompatibility. Similarly, the stigma associated with the disease, as well as the discrimination against affected persons emerged as barriers to efforts aimed at its control and prevention. These findings show that efforts should be intensified to educate the public about sickle cell disease. Adequate knowledge regarding the disease can promote a better attitude and practice towards prevention. Furthermore, findings from this study revealed that female participants reported a better attitude towards sickle cell disease prevention, and attitude was also significantly affected by age and level of education. A better understanding of these emergent correlations can underpin the design of more effective and efficient control mechanisms.
Given the high prevalence of sickle cell disease in Nigerian setting, it is critical to develop robust and comprehensive policies and legislation which can facilitate adequate contextual and effective knowledge-based campaigns. The study results indicate more effort is needed so as to promote sickle cell awareness in the Nigerian setting. Prioritisation of sickle cell disease awareness can underpin an effective strategy that would ensure an expeditious reduction of the burden of the disease. Further research in this area will be invaluable in developing contextual policies aimed at controlling and eradicating the disease.
Data availability
All data generated and analysed during this research are available on request.
Disclosure statement
No potential conflict of interest was reported by the author(s).
References
- Ugwu NI. Sickle cell disease: awareness, knowledge and attitude among undergraduate students of a Nigerian tertiary educational institution. Asian J Med Sci. 2016 Aug 31;7(5):87–92.
- Badawy SM, Barrera L, Cai S, et al. Association between participants’ characteristics, patient-reported outcomes, and clinical outcomes in youth with sickle cell disease. BioMed Res Int. 2018 Jul 18;2018; doi:10.1155/2018/8296139.
- Fogarty H, Gaul A, Syed S, et al. Adherence to hydroxyurea, health-related quality of life domains and attitudes towards a smartphone app among Irish adolescents and young adults with sickle cell disease. Ir J Med Sci (1971-). 2021 Mar 20: 1–8. doi:10.1007/s11845-021-02588-1.
- Makani J, Williams TN, Marsh K. Sickle cell disease in Africa: burden and research priorities. Ann Trop Med Parasitol. 2007 Jan 1;101(1):3–14.
- Akodu SO, Diaku-Akinwumi IN, Njokanma OF. Age at diagnosis of sickle cell anaemia in lagos, Nigeria. Mediterr J Hematol Infect Dis. 2013;5(1):e2013001, doi:10.4084/MJHID.2013.001.
- Piel FB, Patil AP, Howes RE, et al. Global epidemiology of sickle haemoglobin in neonates: a contemporary geostatistical model-based map and population estimates. The Lancet. 2013 Jan 12;381(9861):142–151. doi:10.1016/S0140-6736(12)61229-X.
- Fleming AF, Storey J, Molineaux L, et al. Abnormal haemoglobins in the Sudan savanna of Nigeria: I. Prevalence of haemoglobins and relationships between sickle cell trait, malaria and survival. Ann Trop Med Parasitol. 1979 Apr 1;73(2):161–172.
- Serjeant GR, Serjeant BE. The epidemiology of sickle cell disorder: a challenge for Africa. Arch Ibadan Med. 2001;2:4–52.
- Nwogoh B, Adewoyin AS, Iheanacho OE, et al. Prevalence of haemoglobin variants in Benin city, Nigeria. Ann Biomed Sci. 2012;11:60–64.
- Wood KC, Hsu LL, Gladwin MT. Sickle cell disease vasculopathy: a state of nitric oxide resistance. Free Radical Biol Med. 2008 Apr 15;44(8):1506–1528.
- Morris CR. Mechanisms of vasculopathy in sickle cell disease and thalassemia. Hematology Am Soc Hematol Educ Program. 2008;1:177–185.
- Gill FM, Sleeper LA, Weiner SJ, et al. Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease. Blood. 1995;86:776–783.
- Barrett-Connor E. Bacterial infection and sickle cell anemia. An analysis of 250 infections in 166 patients and a review of the literature. Medicine (Baltimore). 1971;50:97–112.
- Khatib R, Rabah R, Sarnaik SA. The spleen in the sickling disorders: an update. Pediatr Radiol. 2009;39:17–22.
- Castro O, Brambilla DJ, Thorington B, et al. The acute chest syndrome in sickle cell disease: incidence and risk factors. The cooperative study of Sickle Cell Disease. Blood. 1994;84:643–649.
- Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Sickle cell disease TC. cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91:288–294.
- Oliveira CC, Ciasca SM, Moura-Ribeiro MV. Stroke in patients with sickle cell disease: clinical and neurological aspects. Arq Neuropsiquiatr. 2008;66:30–33.
- Powars DR. Sickle cell anemia and major organ failure. Hemoglobin. 1990;14:573–598.
- Galadanci N, Wudil BJ, Balogun TM, et al. Current sickle cell disease management practices in Nigeria. Int Health. 2014 Mar 1;6(1):23–28.
- Williams TN, Obaro SK. Sickle cell disease and malaria morbidity: a tale with two tails. Trends Parasitol. 2011;27:315–320.
- McAuley CF, Webb C, Makani J, et al. High mortality from plasmodium falciparum malaria in children living with sickle cell anemia on the coast of Kenya. Blood. 2010;116:1663–1668.
- Uche E, Olowoselu O, Augustine B, et al. An assessment of knowledge, awareness, and attitude of undergraduates toward sickle cell disease in Lagos, Nigeria. Nigerian Medical Journal: J Niger Med Assoc. 2017 Nov;58(6):167–172.
- Abioye-Kuteyi EA, Oyegbade O, Bello I, et al. Sickle cell knowledge, premarital screening and marital decisions among local government workers in Ile-Ife, Nigeria. Afr J Prm Health Care Fam Med. 2009;1(1):1–5. doi:10.4102/phcfm.v1i1.22.
- El-Hazmi MA. Ethics of genetic counseling – basic concepts and relevance to Islamic communities. Ann Saudi Med. 2004 Mar;24(2):84–92.
- Adewoyin AS, Alagbe AE, Adedokun BO, et al. Knowledge, attitude and control practices of sickle cell disease among youth corps members in Benin City, Nigeria. Ann Ib Postgrad Med. 2015;13(2):100–107.
- Daak AA, Elsamani E, Ali EH, et al. Sickle cell disease in western Sudan: genetic epidemiology and predictors of knowledge attitude and practices. Trop Med Int Health. 2016 May;21(5):642–653.
- Boadu I, Addoah T. Knowledge, beliefs and attitude towards sickle cell disease among university students. J Community Med Health Educ. 2018;8(1):1000593, doi:10.4172/2161-0711.1000593.
- Emerson RW. Convenience sampling, random sampling, and snowball sampling: how does sampling affect the validity of research? J Vis Impair Blind. 2015 Mar;109(2):164–168.
- Olakunle OS, Kenneth E, Olakekan AW, et al. Knowledge and attitude of secondary school students in Jos, Nigeria on sickle cell disease. Pan Afri Med J. 2013;15(1):1–9.
- Al-Farsi OA, Al-Farsi YM, Gupta I, et al. A study on knowledge, attitude, and practice towards premarital carrier screening among adults attending primary healthcare centers in a region in Oman. BMC Public Health. 2014 Dec;14(1):1–7.
- Wonkam A, De Vries J, Royal CD, et al. Would you terminate a pregnancy affected by sickle cell disease? Analysis of views of patients in Cameroon. J Med Ethics. 2014 Sep 1;40(9):615–620.
- Arlebrink J. The moral roots of prenatal diagnosis. Ethical aspects of the early introduction and presentation of prenatal diagnosis in Sweden. J Med Ethics. 1997 Aug;23(4):260–261.
- Asch A. Prenatal diagnosis and selective abortion: a challenge to practice and policy. Am J Public Health. 1999 Nov;89(11):1649–1657.
- Kauf TL, Coates TD, Huazhi L, et al. The cost of health care for children and adults with sickle cell disease. Am J Hematol. 2009 Jun;84(6):323–327.
- Olatunya OS, Ogundare EO, Fadare JO, et al. The financial burden of sickle cell disease on households in Ekiti, Southwest Nigeria. Clinico Economics and Outcomes Research: CEOR. 2015;7:545–553. doi:10.2147/CEOR.S86599.
- Kindi RA, Rujaibi SA, Kendi MA. Knowledge and attitude of university students towards premarital screening program. Oman Med J. 2012;27(4):291–296.
- Isah BA, Musa Y, Mohammed UK, et al. Knowledge and attitude regarding premarital screening for sickle cell disease among students of state school of nursing Sokoto. Annals of International Medical and Dental Research. 2016;2(3):29–34.
- Marsh V, Kamuya D, Molyneux S. ‘All her children are born that way’: gendered experiences of stigma in families affected by sickle cell disorder in rural Kenya. Ethn Health. 2011;16(4–5):343–359. doi:10.1080/13557858.2010.541903.
- Bediako S, Lanzkron S, Diener-West M, et al. The measure of sickle cell stigma: initial findings from the improving patient outcomes through respect and trust study. J Health Psychol. 2014;21(5):808–820.
- Adeyemo T, Ojewunmi O, Diaku-Akinwumi I, et al. Health related quality of life and perception of stigmatisation in adolescents living with sickle cell disease in Nigeria: a cross sectional study. Pediatr Blood Cancer. 2015;62(7):1245–1251.
- Blake A, Asnani V, Leger RR, et al. Stigma and illness uncertainty: adding to the burden of sickle cell disease. Hematology. 2018 Feb 7;23(2):122–230.