The Egyptian Journal of Haematology

ORIGINAL ARTICLE
Year
: 2020  |  Volume : 45  |  Issue : 1  |  Page : 16--22

Perceived stigma among adolescents with sickle cell anemia in a tertiary health facility


Chioma Okechukwu1, Chukwuma U Okeafor2, Innocent O George3,  
1 Departments of Paediatrics, University of Port Harcourt Teaching Hospital, Port Harcourt, Nigeria
2 Departments of Mental Health/Neuropsychiatry, University of Port Harcourt Teaching Hospital, Port Harcourt, Nigeria
3 Departments of Paediatric Haematology, University of Port Harcourt Teaching Hospital, Port Harcourt, Nigeria

Correspondence Address:
Chukwuma U Okeafor
Clinical Neurology, Department of Mental Health/Neuropsychiatry, University of Port Harcourt Teaching Hospital, Port Harcourt, 500102, Nigeria
Nigeria

Abstract

Background Adolescents with sickle cell anemia (SCA) are challenged by a myriad of complex psychosocial issues, which may be triggered or exacerbated by stigmatization. The magnitude of childhood stigma is scarcely being assessed, as more focus is often given to the physical symptoms and signs of SCA. This study sought to assess the prevalence of perceived stigma and its associated factors among adolescents with SCA in steady state. Patients and methods Using a cross-sectional design, 200 adolescents with SCA (patients) were recruited consecutively from the Paediatric Haematology Clinic. Controls matched for age, sex, and socioeconomic class, with hemoglobin genotype AA or AS, were recruited from the Children Outpatient Clinic. A self-administered questionnaire was used to obtain the clinicodemographic data. A validated perceived stigma scale was used in this study. Bivariate and multivariate analyses were performed at 0.05 significant level. Results There were 97 (48.5%) males and 103 (51.5%) females in each group. Perceived stigma was present in 159 (79.5%) patients and 16 (8.0%) controls (P=0.001). Perceived stigma was significantly higher among patients who were in their early and mid-adolescence, those who had sickle cell pain crises frequency of greater than thrice a year, and those with poor self-perception. Patients in early and mid-adolescence were about three times more likely to experience perceived stigma than those in late adolescence (odds ratio=2.9; 95% confidence interval=1.1–7.7; P=0.031). Conclusion Stigma is a major problem among adolescents with SCA. Regular screening for stigma is recommended for patients with SCA, especially those in early and mid-adolescence.



How to cite this article:
Okechukwu C, Okeafor CU, George IO. Perceived stigma among adolescents with sickle cell anemia in a tertiary health facility.Egypt J Haematol 2020;45:16-22


How to cite this URL:
Okechukwu C, Okeafor CU, George IO. Perceived stigma among adolescents with sickle cell anemia in a tertiary health facility. Egypt J Haematol [serial online] 2020 [cited 2020 Nov 30 ];45:16-22
Available from: http://www.ehj.eg.net/text.asp?2020/45/1/16/294786


Full Text



 Introduction



Sickle cell anemia (SCA) is one of the most common genetic diseases worldwide [1] and in Nigeria [2]. The WHO reported in the 59th World Health Assembly that nearly 300 000 infants each year are born with major hemoglobin disorders in Africa, with more than 200 000 cases of SCA [3]. Nigeria has the highest burden of SCA, with 20 of every 1000 births [3]. Studies have shown that childhood mortality from SCA in Africa is as high as 50–90%, with deaths occurring more in children 5 years and younger [4],[5],[6].

The clinical course in SCA is variable [7]. However, a significant proportion of children with SCA require frequent medical attention and hospitalizations for symptoms that may begin as early as 6 months of age. The frequent clinic appointments may disrupt schooling, employment, and other social activities, hence posing both medical and psychosocial challenges to the affected individual and their families [7].

Stigma has been identified by the WHO as a significant burden of disease in people with chronic diseases like SCA [8]. The potential contribution of stigma to psychosocial difficulties in SCA is increasingly becoming recognized [9]. The few comparable studies assessing the prevalence of perceived stigma in adolescents with SCA in the USA, UK, and Nigeria have shown perceived stigma to be much higher in Nigeria (60–90%) than in these Western countries (5–15%) [10],[11],[12],[13],[14]. Stigma in patients with SCA leads to denial of social support delayed diagnosis of complications, treatment, and comprehensive health care [8]. Furthermore, their optimal mental health is vital as they advance into adult phase. This study, therefore, sought to assess the prevalence of perceived stigma, and its associated factors among adolescents with SCA in steady state.

 Patients and methods



Study design

This was a hospital-based cross-sectional study done over a period of 9 months (July 2017–March 2018).

Study population

The study population consisted of two groups: 200 patients and 200 controls. The patients were adolescents aged 10–18 years with SCA in steady state who came for follow-up at the Haematology Clinic of the Department of Paediatrics. Steady state was defined as that period when the patient with SCA is free from infection, pain, or other disease processes [5]. It includes the following criteria: no history of fever in the preceding 4 weeks, no history of painful crises that required treatment in the hospital in the preceding four consecutive weeks, no history of blood transfusion during the previous 4 months, and no history of intercurrent illness such as infection and inflammation in the previous 4 weeks.

The controls were adolescents aged 10–18 years with HbAA or HbAS genotype matched for age, sex, and socioeconomic class with the study patients recruited from the Children Outpatient Clinic of the Department of Paediatrics.

Ethical considerations

Ethical approval for the study was obtained from the Ethical committee of the University Teaching Hospital. Consent was obtained from the adolescents as well as their parents/guardian. The principles of ethics were upheld in carrying out the research.

Sample size calculation and sampling

Sample size formula for cross-sectional studies was employed based on the 0.05 significant level, prevalence of SCA of 2.9% [13], precision of 0.025, and nonresponse of 10% to attain a sample size of 200. Hence, 200 patients with SCA and 200 controls were recruited into the study. Patients who met the selection criteria were enrolled consecutively until the desired sample size was obtained. The control group was then selected after matching for age, sex, and socioeconomic class. For each case, at least two matched controls were listed and assigned numbers at random. Using the simple random sampling method via a computer-generated table of random numbers, one of the controls was selected. This process was continued until an equal number of controls, matched in a 1 : 1 ratio with the patients, was obtained.

Data collection

A self-administered questionnaire comprising of a validated perceived stigma scale [15], sociodemographic variables of interest, past medical history, and psychosocial data was used for data collection. The questionnaire was pretested among 10% of the sample size (20 patients and 20 controls). No major changes were made to questionnaire after the pretest. The results from the pretest were not included in the main study. The ages of respondents were stratified into 10–13, 14–16, and 17–18 years based on the WHO classification of adolescents into early, mid, and late adolescents [1].

Statistical analysis

The Statistical Package for Social Sciences (IBM Armon, New York, United States), version 20 was used to analyze the data. Comparison of subgroups was carried out using Pearson’s χ2 or Fisher’s exact test as appropriate. Fisher’s exact test was used when the expected cell value was less than five in at least 20% of the cross-tabulation cells. The dependent variable was perceived stigma, categorized as present/absent, whereas the sociodemographic factors, clinical characteristics, and self-perception comprised the independent variables. Bivariate analysis was performed using c2 statistics at the 0.05 significant level. Statistically significant variables on bivariate analysis which were not collinear were entered into a multivariate analysis model to adjust for confounders and identify predictors. Multivariate analysis employed binary logistic regression. Odds ratios were used to estimate the magnitude of associations with a confidence interval set at 95%. The level of significance was taken to be P value less than 0.05.

 Results



Sociodemographic characteristics

A total of 400 adolescents, comprising 200 adolescents with SCA (patients) and 200 controls matched for age, sex, and socioeconomic class were involved in the study. There were 97 (48.5%) males and 103 (51.5%) females in both groups, with a male to female ratio of 1 : 1.1. A total of 196 (49.0%) participants were of high social class, 140 (35.0%) were middle class, and 64 (16.0%) of low social class ([Table 1]).{Table 1}

Prevalence of perceived stigma

One hundred and fifty-nine (79.5%) patients and 16 (8.0%) controls had perceived stigma. The difference was statistically significant (P=0.001) ([Table 2]).{Table 2}

Sociodemographic factors and perceived stigma among adolescents with sickle cell anemia

The prevalence of perceived stigma was significantly higher in early and mid-adolescence (82.6 and 82.4%, respectively) in comparison with late adolescence (62.1%) (P=0.042). There was no significant association between the prevalence of perceived stigma and sex or social class of patients ([Table 3]).{Table 3}

Past medical history and perceived stigma among adolescents with sickle cell anemia

Perceived stigma was significantly higher in patients with three to four sickle cell pain crises and three or more hospital admissions in the preceding 1 year (P=0.035 and 0.001, respectively). There was no significant relationship between perceived stigma and the number of blood transfusions in the preceding 1 year (P=0.286) ([Table 4]).{Table 4}

Clinical characteristics and perceived stigma among adolescents with sickle cell anemia

Perceived stigma was higher among adolescents with SCA who had frontal bossing (81.1%) and thin long limbs (79.8%). Bivariate analysis showed that perceived stigma occurred among adolescents with SCA irrespective of the clinical characteristics, as shown in [Table 5].{Table 5}

Self-perception and perceived stigma among adolescents with sickle cell anemia

There was a significant relationship between self-perception and occurrence of perceived stigma among the adolescents with SCA, as shown in [Table 6].{Table 6}

Multivariate analysis of factors associated with perceived stigma

Early and middle aged adolescents with SCA were approximately three times more likely to experience perceived stigma than late-aged adolescents (odds ratio, 2.91; 95% confidence interval, 1.10–7.70; P=0.031). Perception of adolescents that people look at them in different way because of the way they look than other children was significantly associated with perceived stigma (odds ratio, 10.71; 95% confidence interval, 4.75–24.17; P=0.0001) ([Table 7]).{Table 7}

 Discussion



This study showed a higher prevalence of perceived stigma in the adolescents with SCA (79.5%), in comparison with the controls (8.0%). This finding is consistent with another study involving 80 adolescents with SCA and 80 controls (with genotype AA and AS) by Adeyemo et al. [13] in Lagos, Nigeria. The study by Adeyemo and colleagues reported prevalence of more than 70% among adolescents with SCA, thus, along with index study, exposing the fact that perceived stigma is not uncommon among adolescents with SCA. Noteworthy, the prevalence of perceived stigma in this present study is higher than that reported by Ani et al. [11] in the United Kingdom who reported the prevalence of 15%. In the UK study, the study population included persons with all other disorders under the SCA spectrum, for example, HbSC and HbSβThal. These are known to be less severe SCA variants, and so the effect of the disease on their daily lives may be less compared with those with SCA. Moreover, the difference in geographical region between this present study and that of the UK study [11] may contribute to the observed disparity in terms of varying sociocultural influences on human behavior. The implication of the finding of higher prevalence among adolescents with SCA in comparison with age-matched, sex-matched, and socioeconomic status-matched controls in the index study is the need for regular/routine screening for perceived stigma among adolescents with SCA. This will not only lead to early detection of those with perceived stigma, it could also curtail any psychosocial complications stirring up as a result of perceived stigma.

In this present study, perceived stigma was significantly higher in early and mid-adolescence compared with late adolescence. This is similar to the reports of Ola et al. [12], and Moronkola and Fadairo [16], both in Nigeria, but in contrast with the reports of Ani et al. [11], in the UK. These studies in Nigeria [12],[16] both noted that age and stigmatization against persons with SCA were inversely related. The comparability with this present study in terms of the age group they studied and location of study may explain this similarity. Other factors that may contribute to this similarity in the studies done in Nigeria include poverty and poor funding of the health care sector. This is apparent in the scope of health care delivery which is largely deficient especially in the area of adequate health education and information dissemination.

Furthermore, during early and mid-adolescence, the bodily changes of puberty and sexual maturation occur. The delayed sexual maturation common in SCA adolescents may contribute to feelings of body dissatisfaction, negative body image, and hence the perception of stigmatization in these adolescents. Although the effect of delayed sexual maturation was not assessed in this present study, earlier researchers [17],[18],[19] have documented the central role pubertal delay plays in the etiology of psychosocial complications in SCA.

There was no sex difference in perceived stigma in this present study. This was also documented by Blake et al. [20] in Jamaica and Ani et al. [11] in the UK, but it was in contrast with the reports of Ani et al. [21] and Ola et al. [12] in Lagos. Nonetheless, the need for stigma prevention strategies targeted at both male and female adolescents is highlighted from the index study.

Perceived stigma was also independent of social class in this study. Although the prevalence of perceived stigma in the respondents of high socioeconomic class (79.6%) was slightly lower than that in respondents of the low socioeconomic class (81.2%), the difference was not statistically significant. This is in keeping with the reports of Ani et al. [11] in the UK, but at variance with the reports of Blake et al. [20] in Jamaica who reported lower stigma scores in people of high socioeconomic class in adults with SCA. The reason for the observed difference may be that the psychological effect of socioeconomic status may directly affect the adults more than the adolescents who are still dependent on their parents.

The study showed a positive relationship between stigma and increasing frequency of sickle cell pain crises and hospital admissions in the preceding year on bivariate analysis. This is in tandem with the reports of other researchers, which showed more perceived stigma in persons with SCA with frequent sickle cell pain crises and hospital admissions [9],[13]. There is clinical relevance to the relationship between stigma and the severity of sickle cell disease identified in this study. Frequent crises and hospital admissions will lead to school absenteeism and less participation in social activities with peers [11],[22]. This could promote the feeling of stigmatization. Nonetheless, the multivariate analysis showed that after adjusting for age of adolescents and self-perception, frequency of sickle cell crises was no longer significant. There was no association between perceived stigma and the frequency of blood transfusions in the preceding year. This is in contrast to the reports of Hasan et al. [23] who found psychosocial complications of SCA to be significantly higher in patients with multiple blood transfusions in the preceding 1 year.

There was no significant relationship between stigma and the presence of clinical characteristics related to SCA like jaundice, frontal bossing, or gnathopathy. This is similar to what was reported by Ani et al. [11], but in contrast with the stigma dimension of visibility, which proposes that disorders with peculiar body features have a higher potential for stigma [24]. These findings may, however, be a reflection of the poor knowledge of the clinical manifestations of sickle cell disease in the general public especially primary and secondary school students in Nigeria.There was also no association between the presence of perceived stigma and leg ulcers. This is consistent with the reports of Ani et al. [11] in the UK, but in contrast with the report of Umeh et al. [25] in the USA and Adeyemo et al. [13] in Lagos, which showed perceived stigma to be more in patients with leg ulcers. However, the occurrence of these ulcers in areas that could be easily concealed by clothing could be a possible explanation of the finding of no association between perceived stigma and leg ulcers in index study.

Despite the finding of no relationship between sickle cell habitus (gnathopathy, frontal bossing, etc.), it is interesting to note that the patients with a negative perception of their clinical characteristics had a significantly higher perceived stigma in index study. This is comparable to the reports of Ani et al. [11], Scott and Scott [17], and Patel and Pathan [26], who reported that most patients with SCA are less satisfied with their bodies and hence feel stigmatized, thus revealing that a negative self-perception influences psychosocial outcomes. Therefore, measures aimed at promoting positive self-perception among adolescents with SCA could forestall the occurrence of perceived stigma.

The identified factors associated with perceived stigma do not translate to causal factors owing to the limitation of the study design being cross-sectional. Although, the index study has highlighted the prevalence and factors associated with perceived stigma among adolescents with SCA, further research on the effect and causal factors of stigma are advocated to refine theories and institute evidence-based strategies for stigma reduction.

 Conclusion



Approximately eight in 10 adolescents with SCA experience perceive stigma in the study setting. Age and self-perception were predictors of perceived stigma among adolescents with SCA. Therefore, a regular assessment of stigma should be done for adolescents with SCA for early detection and prompt management.

Acknowledgements

The authors acknowledge the support of Dr Ibitein N. Okeafor and Eagles Watch Research Centre and Care, Port Harcourt in the preparation of the manuscript. Chioma Okechukwu, contributed to the concepts of the study, collected data, and prepared draft. Chukwuma U. Okeafor, conceptualized the study design, analyzed the data, and edited the draft. Innocent O. George, contributed to the concepts and reviewed the final draft.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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