An Assessment of Knowledge, Awareness, and Attitude of Undergraduates toward Sickle Cell Disease in Lagos, Nigeria.

BACKGROUND: Sickle cell anaemia is an autosomal recessive disorder which occurs as a result of the substitution of glutamic acid with valine at the 6th position of the haemoglobin beta chain, resulting in the synthesis of abnormal haemoglobin and the consequent production of the characteristic sickled red blood cells. Sickle cell anaemia is the homozygous form where two HbS genes are inherited, whilst in Sickle cell disease, the HbS is inherited along with another abnormal haemoglobin eg HbE, HbC, etc. AIMS AND
OBJECTIVES: This study was aimed at assessing the awareness and knowledge of sickle cell disease among students in a tertiary institution, as well as their attitude to the disease.
MATERIALS AND METHODS: This was a cross-sectional quantitative interviewer administered questionnaire study, involving 200 undergraduates of the Lagos State University Ojo Campus, Lagos. Information collected include socio-demographic information, as well as questions to assess the awareness, knowledge and attitude to Sickle Cell Disease. Data obtained were analyzed using SPSS version 16.0.
RESULTS: The total knowledge score was 20 and depending on the total score of the respondents, knowledge levels were grouped as poor (0-6), fair (6-13) and good (14-20). Of the 200 respondents, 37.5% had good knowledge of SCD, despite high level of awareness (92.5%). The knowledge level of the respondents based on the score revealed a mean score 12.05 + 3.14 indicating fair general knowledge of the respondents. 67.5% of respondents were aware of their haemoglobin phenotypes. More than half (59%) of the respondents knew someone living with sickle cell disease and 154 (77%) agreed that haemoglobin phenotype would play a significant role in their choice of a life partnzer.
CONCLUSION: The awareness of SCD among the students is high but this awareness did not translate to good overall knowledge about the disease. This underscores the importance of increased public health education on SCD, in order to increase the knowledge about SCD.

INTRODUCTION

Sickle cell anemia is an autosomal recessive disease which occurs as a result of the substitution of glutamic acid with valine at the sixth position in the hemoglobin (Hb)-beta chain. This point mutation leads to the production of sickle Hb (HbS) which is less soluble than normal fetal Hb or adult Hb. In the sickle cell syndromes, the S mutation is co-inherited with a mutation at the other globin allele. These syndromes include sickle cell anemia (homozygous sickle mutation), sickle-beta thalassemia, and Hemoglobin SC disease.12

It is one of the most common monogenetic diseases worldwide, and the greatest burden lies mostly in Sub-Saharan Africa, the Middle East, and the Mediterranean regions.34

Within this region, Nigeria bears the highest burden of the disease where about 25% of the population possesses the sickle cell gene, with 2%–3% living with the disease.567

During conditions of low oxygen tension, the red blood cells in sickle cell disease (SCD) assume the characteristic sickle shape due to intracellular polymerization of Hb. This leads to the characteristic vaso-occlusive phenomena, culminating in pain, and tissue infarction. The sickled red cells are prematurely removed from the circulation, leading to chronic anemia.8

According to the World Health Organization (WHO), it is recommended that in areas where Hb disorders are common, special dedicated centers should be setup in appropriate numbers, appropriately situated, and with a high degree of autonomy.9 The WHO also advocates that carrier detection and genetic counseling should be done by multispecialist teams in these centers.910

Despite these recommendations by the WHO, there is a dearth of genetic counseling centers in Nigeria with seemingly low level of awareness about these centers among the populace. A study by Adewoyin et al. documented that among the respondents who were aware of SCD, none of them indicated genetic counseling centers as their source of knowledge.11

In addition, there are no newborn screening facilities for SCD and other genetic disorders in Nigeria, and often times, diagnosis is usually made during hospitalization for a childhood ailment or when serious life-threatening complications occur. The most cost-effective strategy to reduce the SCD burden in Nigeria would be to focus on primary prevention of the disease through public education and health awareness programs among others. Adequate knowledge is required among the populace in general and individuals with the sickle cell trait in particular. This will enable them make informed decisions about their reproductive lives.

The National Sickle Cell Centre was established in 1994 by the sickle cell foundation and is the first comprehensive sickle cell center in Africa. The center was established with the vision “to alleviate the burden of sickle cell disorder on the country and to ensure that all affected persons can live normal pain-free lives.”12 Services currently being offered include the provision of free routine drugs such as folic acid and Paludrine as well as equipment such as automated full blood count machines in some states in Nigeria. They also offer genetic counseling and prenatal diagnosis, with a proposed program to commence newborn screening in the future so as to diagnose affected children early in life. This early diagnosis will aid in the introduction of interventions aimed at reducing SCD morbidities and mortalities. It is also involved in advocacy and information dissemination through public lectures and seminars, radio/television presentations, as well as through the print media.12

It is important that students in tertiary institutions are educated about SCD since this cohort of young adults is in the reproductive age, and several would go into relationships which may culminate in marriage.

This study, therefore, aimed to assess the awareness and the level of knowledge of SCD among undergraduates in the Lagos State University Ojo Campus. It also assessed their attitudes toward the disease.

MATERIALS AND METHODS

Study design

This was a descriptive cross-sectional study carried out among university undergraduates.

Study area and population

This study was carried out at the Lagos State University Ojo Campus, Lagos, Nigeria. Respondents were recruited from various departments in the University.

The study participants were selected using a multistage sampling technique where three faculties were selected using a balloting system. The three faculties selected were arts, sciences, and law. Next, the same method (simple random balloting method) was used to select two departments from each of the faculties chosen. The departments of theater arts, music, accounting, physics, and law were selected.

The number of respondents was allotted to each department, and systematic sampling was then used to select respondents. The sampling frame was obtained, and respondents who were not available at the time of questionnaire distribution were replaced by the next student in the sampling frame.

Lagos State University was established in 1983 with a population of over 35,000 students enrolled in various faculties and departments. It offers diploma, degree, and postgraduate programs. There are three campuses located at Ojo, Epe, and Ikeja; the latter is where the College of Medicine is situated, while Epe houses the Engineering and Agricultural faculties; Ojo which is the main campus has the highest students' population and the highest number of faculties. It is also the campus where the Senate building, Vice Chancellor's, Registrar's, and Bursary offices are located.

Only respondents who gave informed consent and who were identified by a valid student identification card were co-opted into the study. The students were selected during lecture periods and filled the questionnaires during the break period.

Sample size determination

The sample size is calculated using Daniel formula.13

Where n = sample size

z = z statistics for a level of confidence of 95%, which is conventional, z value is 1.96

p = proportion of people with good knowledge of SCD.11

q = 1 – p

d = precision

z = 1.96

P = 17% (0.17)

d = 0.05

Two hundred respondents were recruited into the study.

Data collection and analyses

Two hundred valid questionnaires were subjected to statistical analysis. Information collected from the questionnaire included sociodemographic parameters, awareness, knowledge, and attitude toward SCD.

The section on knowledge included questions on the etiology, clinical manifestations, and treatment of SCD. Each of the 20 questions in the knowledge section was scored as 1 for “Yes” response or 0 for “No/I don't know” response to give a total knowledge score of 20. Scores between 0 and 6 were considered poor knowledge, 7 and 13 fair knowledge, and 14 and 20 good knowledge. All the “Yes” responses were the correct answers to the questions asked.

Data were analyzed using the Statistical Package for the Social Sciences Inc., Chicago IL 2002. Descriptive analysis was performed and presented as frequency counts and percentages in tables. The mean knowledge scores were compared between various groups (age range of respondents, sex, religion, knowing someone with SCD, and Hb phenotype), and P < 0.05 was considered statistically significant.

Ethical consideration and clearance

Ethical approval was obtained from the Health Research and Ethics Committee of Lagos State University Teaching Hospital.

Students' informed consent was also obtained.

RESULTS

Sociodemographic characteristics of the respondents

A total of 200 participants were involved in the study with 57% males and 43% females [Table 1]. The mean age of the participants was 20.2 ± 3.38 years with the majority (43.5%) within the age range of 19–22 years. Almost all the participants (95.5%) were single, and majority of them (56.5%) were Christians [Table 1].

Table 1

Sociodemographic characteristics and sickle cell information of respondents

Variable	Frequency (%)
Age (years)
15-18	68 (34)
19-22	87 (43.5)
23-26	36 (18)
27-30	7 (3.5)
31-35	2 (1.0)
Sex
Male	114 (57)
Female	86 (43)
Religion
Christian	113 (56.5)
Islam	86 (43)
Traditional	1 (0.5)
Awareness of SCD
Yes	185 (92.5)
No	15 (7.5)
Source of sickle cell awareness
TV/radio	42 (21)
Friends and colleagues	41 (20.5)
Health workers	22 (11)
Lectures/seminars	50 (25)
Family members	26 (13)
Posters/handbills	4 (2)
Unaware	15 (7.5)
Test for SCD
Yes	165 (82.5)
No	35 (17.5)
Knows genotype
Yes	135 (67.5)
No	65 (32.5)

SCD – Sickle cell disease

Awareness of sickle cell disease

Most (92.5%) of the respondents were aware of SCD. The various sources of information about SCD are also shown in Table 1.

Knowledge of sickle cell disease

Of the 165 (82.5%) of respondents who had been tested, only 135 of them knew their Hb phenotypes [Table 1]. The distribution of different Hb phenotypes of the participants is seen in Figure 1.

Figure 1

Distribution of respondents' hemoglobin phenotypes

The total knowledge score was 20, and depending on the total score of the respondents, knowledge levels were grouped as poor (0–6), fair (6–13), and good (14–20).

The mean knowledge score was 12.05 ± 3.14, indicating a fair general knowledge of participants. Of the 200 respondents, 11.5%, 51%, and 37.5% had poor, fair, and good knowledge, respectively [Table 2].

Table 2

Respondents’ level of knowledge

Variable	Frequency (%)
Level of knowledge
0-6 (poor)	23 (11.5)
7-13 (fair)	102 (51)
14-20 (good)	75 (37.5)
Mean knowledge score	12.05±3.14

Respondents' attitudes to sickle cell disease

More than half (59%) of the respondents knew someone living with SCD, and 154 (77%) agreed that Hb phenotype would play a significant role in their choice of a life partner.

While 79% of them agreed that they would end their relationships if they discover that their Hb phenotype predisposes them to have children with SCD, 5% were undecided, whereas 16% said that they would continue the relationship [Table 3]. Responses of participants if both partners are found to have the carrier state and following a prenatal diagnosis of SCD are shown in Table 3.

Table 3

Respondents’ attitude to sickle cell disease

Question on attitude	Frequency (%)
Willing to do premarital genetic counseling and screening
Yes	154 (77)
No	46 (23)
Willingness to continue with a relationship despite the risk of having offspring with SCD
Yes	32 (16)
No	158 (79)
Undecided	10 (5)
Response if both partners are found to be carriers after marriage
Opt for prenatal screening	139 (69.5)
Accept not to have biological children	24 (12)
Accept to have children with SCD	25 (12.5)
Undecided	12 (6)
Response to unborn child following prenatal diagnosis of SCD
Abortion	0
Allow pregnancy	0
Undecided	139 (100)

SCD – Sickle cell disease

Comparison of level of knowledge within different groups

The knowledge score was not significantly associated with age, sex, or religion. Even though the group who knew a person living with SCD had a higher mean knowledge score, the difference was not statistically significant (P = 0.05) [Table 4].

Table 4

Comparing level of knowledge in different groups

Variable	Frequency	Mean±SD
Age (years)
15-18	68	11.75±3.18
19-22	87	11.97±3.06
23-26	36	12.86±2.30
27-30	7	13.29±3.25
31-35	2	7.50±3.54
P	0.064
Sex
Male	114	11.97±3.21
Female	86	12.16±3.01
P	0.628
Religion
Christianity	113	12.37±2.90
Islam	86	11.70±3.38
Traditional	1	7.0
P	0.764
Knowing someone with SCD
Yes	118	12.76±2.73
No	82	11.04±3.41
P	0.05
Hb phenotype
AA	102	12.75±2.78
AS	30	11.70±3.19
AC	2	8.50±3.54
SC	1	15.00±0.00
P	0.29

SCD – Sickle cell disease; SD – Standard deviation

DISCUSSION

There was a high level of awareness about SCD among the participants as 92.5% of them were aware of the disease. Awareness of SCD is important especially among undergraduates to enable them to make informed decisions in selecting their life partners. This high level of awareness has also been reported in similar studies.111415

The major sources of information about SCD were the mass media, lectures, and seminars. This highlights the important role the mass media can play in information dissemination.

Although the WHO recommends that special dedicated centers should be setup in regions where Hb disorders are common to provide carrier detection tests and genetic counseling facilities.910 Even though such facilities exist in Nigeria, there is still a shortfall and the knowledge and awareness about the existence of these centers is quite poor. This reflects in this study where none of the respondents indicated screening/counseling facilities as their source of knowledge about SCD. This finding was corroborated by Adedoyin et al.11

A total of 165 participants had been screened for SCD, reflecting a good awareness toward the disease. Of this number, 135 correctly indicated their phenotypes. This study recorded a 24% carrier status for SCD. This figure is consistent with previous studies that have documented a carrier state of 20%–30% in Nigeria.567

Despite this high level of awareness, only 37.5% of them had good knowledge about SCD. Questions on the assessment of knowledge about SCD included diagnosis, mode of inheritance, symptoms, and management. This low level of adequate knowledge is consistent with reports from other researchers.1415 It was opined that because undergraduates are involved in numerous dating relationships, increased education and SCD screening among them are key to reducing the prevalence of SCD.16 However, researchers in Nigeria, the United Kingdom, and the United States of America have reported good knowledge of SCD.17181920 This difference in the level of knowledge seen among different researches could be attributed to indices used in knowledge assessment, the sample size used in the study, as well as the study population.

The level of knowledge was not significantly affected by age, gender, religion, or Hb phenotype.

Those respondents who knew persons living with SCD had higher mean knowledge scores when compared with respondents who did not know, but this difference did not reach statistical significance (P = 0.05).

In spite of the low level of knowledge seen among the respondents, most of them (77%) agreed that Hb phenotype will play a significant role in their choice of marriage partners and would consent to premarital genetic counseling. This is a positive attitude and indicates an awareness of the importance of genetic testing as a preventive tool in the control of SCD. Those who declined premarital genetic counseling and screening (23% of respondents) may have this negative attitude as a result of fear of losing potential life partners. Again, this brings to the fore the importance of proper health education in the primary prevention of SCD.

The positive attitude was seen in a similar study by Boadu and Addoah who documented that 85.4% of their study respondents agreed to consider genetic counseling before marriage.14

Majority of the respondents (79%) admitted to being unwilling to continue a relationship that carries the risk of having offspring with SCD. Again, this shows the positive attitude toward SCD among the respondents. Similar positive attitude has been reported by other researchers.142122

In evaluating their reactions if both partners are found to have the sickle cell carrier trait after marriage, 139 (69.5%) of the respondents admitted that they would opt for prenatal screening; however, all 139 of them were undecided about their response to an unborn child following prenatal diagnosis of SCD.

Overall, the students displayed a positive attitude towards SCD as shown by their willingness to undergo premarital genetic counselling and testing, as well as their reluctance to continue a relationship if both partners are found to be carriers of the sickle cell gene. This positive attitude may be due to the fact that many of them are aware of the physical, psychological and social trauma experienced by patients living with SCD as well as the financial and emotional toll it takes on the family. Thus they would most likely want to avoid such experiences.

Despite the fact that Nigeria currently lacks any legislation on premarital counseling and screening, health institutions, the mass media, and religious bodies should all be involved to aid in raising the level of awareness of SCD. At present, premarital genetic counseling in Nigeria is voluntary even though most religious bodies usually make genetic testing mandatory for intending couples.

Adewoyin et al.11 observed that school entry requirement, doctors' request, as well as premarital screening were the most common indications for carrier status check. This information should be leveraged on by the authorities of various bodies to improve awareness of SCD. Intending couples should be mandated to be screened before marriage, and children at school entry should also be tested. Pregnant women should also be tested and when possible offered prenatal diagnosis in cases where both partners are found to be carriers. If prenatal diagnosis results reveal that a couple is carrying a foetus with SCD, they can be then be counselled in order to enable them make informed decisions concerning the pregnancy.

The government should be encouraged to establish zonal more sickle cell centers as mandated by the WHO in every geopolitical zone of Nigeria. Furthermore, awareness about the center and the services offered should be created to enable the general public avail themselves of the facilities.

CONCLUSION

Most respondents demonstrated a fair level of knowledge of SCD despite having a good awareness of the disease. Information dissemination about the disease should be intensified both by the government and private sectors as well as religious bodies. In this way, intending couples will be encouraged to go for premarital genetic testing and thus make better-informed decisions based on test results.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.