Sickle cell knowledge, premarital screening and marital decisions among local government workers in Ile-Ife, Nigeria

ABSTRACT Background In Nigeria, as in the rest of equatorial Africa, sickle cell disease (SCD) has its highest incidence and continues to cause high morbidity and early death. The condition is a major public health problem among the black race. The aim of this survey is to determine the level of knowledge about SCD and the factors associated with its prevention among local government workers in Ile-Ife. Method This is a cross-sectional descriptive study of the knowledge about SCD, attitude towards premarital sickle cell screening and marital decisions among local government workers in Ile-Ife, Nigeria, using a self-administered questionnaire. Results 69% of study subjects had poor knowledge of SCD, while attitude towards premarital screening was favourable in 95% of the study subjects. Knowledge and attitude were significantly better among subjects with tertiary education. There was a strong positive association between attitude towards sickle cell screening and a history of undergoing screening or partner screening. Most (86.7%) of the respondents and 74.0% of their partners have had sickle cell screening. One-quarter of married and engaged respondents did not know their partner's sickle cell status. One-third to two-thirds of study subjects will continue the relationship with their partner when either or both have haemoglobinopathy. Conclusion This study showed poor knowledge of SCD among the studied subjects. There is a need for more emphasis on health education through programmes promoting sickle cell education. In addition, the development of multifaceted patient and public health education programmes, the intensification of screening for the control of SCD by heterozygote detection, particularly during routine preplacement and premarital medical examinations, and the provision of genetic counselling to all SCD patients and carriers are vital to the identification and care of the couples at risk. These will enhance the capacity of the intending couples to make informed decisions and be aware of the consequences of such decisions. Policies are needed to ensure easily accessible community-wide sickle cell screening and premarital and genetic counselling to achieve the desired decline in new births of children with SCD.


INTRODUCTION
Non-communicable diseases, especially genetic diseases such as sickle cell disease (SCD), are a major cause of morbidity and mortality. The sickle cell gene is known to be widespread, reaching its highest incidence in equatorial Africa, with the proportion of carriers in the global human population increasing as a result of a relatively high birth rate in the affected populations.
SCD is one of the most common single-gene disorders. About 25% of adults in Nigeria have the sickle cell gene, south-western Nigeria, in whom it occurs in about 6%. 1,2 The prevalence of sickle cell anaemia is about 20 per 1 000 births. This means that in Nigeria alone, about 150 000 children are born annually with sickle cell anaemia. Despite recent advances in the management of SCD through improved care, re-induction of foetal haemoglobin synthesis and bone marrow transplantation, the condition continues to cause high morbidity and early death in Africa.
The chronic nature of SCD requiring life-long medical attention, expensive supportive symptomatic therapy, its specialised care, the associated high morbidity, reduction in life expectancy of the affected, poor school attendance, the potential risk of the development of drug addiction, especially to opiates, and its burden on the affected families all indicate that the condition is a major public health problem where ever its risk prevalence is high. 3 Methods of preventing new haemoglobinopathy births include premarital screening and genetic counselling, prenatal diagnosis, preconceptional diagnosis and implantation of normal embryos after in vitro fertilisation, and in utero therapy using stem cell transplantation. 3 Prevention of the disease through the disease and allows better use of available resources in the low-income countries where the condition is most prevalent. Programmes of population screening and genetic counselling can have a major impact on the birth rate of children with SCD and other genetic diseases. 4,5,6 The prospective control of SCD by at risk, is of utmost importance. The success of such a programme to a large extent depends on the SCD knowledge in the community, the understanding of the full consequences of having a sickler child, and people's attitude towards genetic screening and counselling. 7,8 group of people to target for the introduction of communitywide interventions such as sickle cell education, screening and counselling. This study therefore set out to assess the knowledge about SCD, attitudes towards premarital sickle cell screening and marital decisions among the local government workers in Ile-Ife.

AIM
The aim of this survey was to determine the level of knowledge about SCD and the factors associated with its prevention among local government workers in Ile-Ife.

METHOD
The study is a cross-sectional descriptive study of all members of staff present during the study period in two randomly selected local government areas (LGAs) in Ile-Ife. A total of 320 pre-tested self-administered, structured questionnaires were handed out and 300 were returned. The response rate was 91%. The knowledge and attitude responses were assigned weighted scores. Knowledge scores of at least 50% of the maximum were respectively.
The data were analysed with the use of the Statistical Package for Social Sciences (SPSS) for Windows, Version 11.0. The results are presented using simple frequencies, cross-tabulations and Table 1 shows that most of the local government workers were aged 21 to 30 years (65.7%), female (51%), married (61%), had tertiary education (69.7%) and engaged in non-health-related occupations (87.3%). Figure 1 shows that the majority (69%) of the study subjects had poor knowledge of SCD, while Figure 2 shows that attitude towards premarital screening was favourable among most (95%) study subjects.

RESULTS
subjects with tertiary education, as shown in Tables 2 to 5 attitude towards premarital screening. Knowledge was also not associated with other behaviours in relation to SCD prevention. About 70% of the subjects with favourable attitude compared to 33.3% of those with unfavourable attitude were aware of their partner's sickle cell status (p = 0.007), while 59.3% of those with favourable attitude discussed their sickle cell status with their partners compared to 6.7% of those with unfavourable attitude (p = 0.001), as shown in Table 6. There was therefore a strong positive association between attitude towards sickle cell screening and a history of undergoing screening or partner screening. 40 (13.3%) study subjects did not know their sickle cell status. Figure 3 shows that about 20% of respondents were aware that they have haemoglobinopathy. It also shows that an approximately similar proportion of respondents knew their partners had haemoglobinopathy. Of the 271 (90.3%) respondents who were either married or have already decided on who to marry, 202 (74.5%) knew their partner's sickle cell status. 46 (25.1%) of the 183 married respondents did not know their spouse's sickle cell status while 23 (26.1%) of the 88 respondents engaged to a marital partner (committed to a marital relationship) did not know their future partner's sickle cell status. Among those who knew both their sickle cell status and that of their partner (Table 7), high proportions (34.4-64.5%) of subjects with haemoglobinopathy stated that they will continue with conjugal relationships. Even when both partners have haemoglobinopathy, as many as 50% did not decide to discontinue the relationship. Figure 4 shows the distribution of the decisions of both partners with haemoglobinopathy according to conjugal status. Table 7 also shows that variable proportions (20-60%) took the decision to screen the other partner when one had haemoglobinopathy. Of 382 offspring of respondents whose sickle cell status was reported, 52 (13.6%) in 40 families had haemoglobinopathy (50 HBAS; 1 HBSS and 1 HBSC respectively).

DISCUSSION
In this study, the majority of the 300 respondents (86.7%) and their partners (74.0%) have had sickle cell screening, as shown in attitude towards screening in 95% of the respondents. There is a strong positive association between attitude towards sickle cell majority (65.7%) of subjects were 30 years of age or younger, more than half of whom were yet to marry. Even though the majority (69.7%) had tertiary education, only a small proportion of subjects (31.0%) had good knowledge of SCD. About a quarter of the married respondents and those engaged to a partner did not necessity of functional education, early life sickle cell education, screening and counselling. Population screening and genetic counselling have been very successful in places like Cyprus, with almost no new births of affected children. 9 Similarly, the affected child birth rate has fallen to about 20% of the expectation in the whole of mainland Italy and Greece. 10 In Bahrain the establishment of genetic clinics, premarital counselling, screening of all pregnant women, newborn testing, student screening and a multifaceted population education programme have resulted in an increase in premarital counselling attendance, premarital mandatory counselling (PMC), PMC law and a reduction in the incidence of SCD from 2.1% to 0.9% -a 60% decline in incidence rate over two decades. 4 Similarly, in Cyprus the incidence of b-thalassaemia was reduced through health education, carrier screening, premarital counselling and prenatal diagnosis. This success has been reproduced in the control of other genetic diseases using carrier screening and premarital counselling, either voluntarily or by legal enforcement. 5,6 One-third to two-thirds of the subjects in this study will continue the relationship with their partner when either or both have haemoglobinopathy, emphasising the urgent need for focused health education as the foundation for genetic counselling before marriage. As expected, both knowledge and attitude compared to the group with less education. A favourable disposition towards screening was associated with awareness of the partner's sickle cell status and discussion of haemoglobin and marital decisions, the universal provision of and emphasis on sickle cell screening and genetic counseling especially long before marriage as recommended by WHO 11 will facilitate informed rational marital decisions and reproductive behaviour that will lead to a sharp decline in new births of children with SCD. 12 C This study showed poor knowledge of SCD among the studied subjects. There is a need for more emphasis on health education through programmes promoting sickle cell education using the mass media, the school health services and by including the subject in the physical and health education curriculum of primary and secondary schools in the country. In addition, the development of multifaceted patient and public health education preplacement and premarital medical examinations, and the provision of genetic counselling to all sickle cell patients and risk. These will enhance the capacity of the intending couples to make informed decisions, to improve their communication about their sickle cell status and to be aware of the risks of