Premarital screening for genetic blood disorders — an integrated review on the knowledge and attitudes of Middle Eastern university students

Abstract

Background

Genetic blood disorders are prevalent in the Middle Eastern countries causing physical, mental, and economic handicap. Premarital screening (PMS) is actively implemented to reduce the burgeoning load of these disorders. Literature shows a low response rate for PMS in this region. This integrated review aims to identify the knowledge and attitude of university students on PMS, as they are at the verge of entering into marriage relationships.

Main body of the abstract

We systematically reviewed the electronic databases like MEDLINE, PubMed, CINAHL, and Scopus. Studies reporting about the knowledge and attitudes of Middle Eastern university students between 2010 and 2020 are included in the review. Twelve cross-sectional descriptive studies conducted in six countries met the inclusion criteria. Majority of the studies reported good level of knowledge on PMS among the university students, whereas not all of them agreed to undergo PMS due to various religious and cultural beliefs.

Short conclusion

We recommend massive health education programs at the higher secondary and university levels to create better awareness on PMS. To improve the attitude towards PMS, life cycle approach and support of the religious leaders could be sought.

Background

Autosomal recessive disorders like sickle cell disease (SCD), beta thalassemia, and other hemoglobinopathies are considered to be the most common genetic blood disorders in the world as well as in the Middle Eastern countries [1]. The incidence of children born with sickle cell disease is expected to increase globally by 30% by 2050 [2]. Available evidence suggests that congenital and genetic disorders are responsible for a large portion of mortality and handicap leading to poor quality of life of younger adults in this region. The incidence of hereditary blood disorders is intertwined with social, cultural, and religious practices in Middle Eastern countries [3], which makes the management of the disorders more complicated. This creates a huge burden on the individuals and families as well as and the health care systems in these countries.

The Centre for Arab Genomic Studies based in United Arab Emirates has released a list of 906 genetic pathologies, which are endemic to Arab states. Some of the commonly prevalent genetic disorders are SCD, thalassemia, Tourette’s syndrome, Wilson’s disease, Charcot-Marie-Tooth disease, mitochondrial encephalomyopathies, and Niemann-Pick disease [4]. Few studies have estimated that the highest prevalence rates of G6PD deficiency is ranging from 1% among Egyptians to 11.55% among Iranians [5]. On the other hand, the prevalence of thalassemia among adult patients in the Middle East is reported to be 43.3% [6]. Regarding SCD, sickle cell hemoglobin (Hbs) is commonly seen in all Middle Eastern countries ranging from approximately 0.04 to 2.1% [7].

Premarital screening (PMS) and genetic counseling (GC) programs can identify and modify the health risk factors known to impact genetic disorders. PMS could be the most important way to prevent the abovementioned genetic blood disorders and many medical, psychological, and social marital problems [8]. PMS and GC programs are declared mandatory in eight Middle Eastern countries. They are free of cost to reduce the at-risk marriages and the prevalence of genetic disorders [9].

The worldwide epidemiologic burden of inherited erythrocyte disorders remains high according to the recent worldwide epidemiological report [10]. In the Arab region, a greater proportion of people (25–60%) prefer consanguineous marriages [11]. Abortion is considered illegal in a majority of Arab countries, which necessitates the efforts to prevent the marriage of the disease carrier couples [12]. PMS can help the couple prepare themselves for marriage with proper premarital counseling giving them a better chance for a stable and satisfying marriage. According to the National Society of Genetic Counselors, “Genetic counseling is the process of helping people understand and adapt to the medical, psychological and familial implications of genetic contributions to disease” [13]. Though the PMS program has been widely implemented in these countries for approximately two decades, the success of the program lies in the awareness and attitudes towards PMS among the younger generation. Though Saudi Arabia has mandated the PMS program before marriage, 48% of the genetically incompatible couple have gone ahead with their marriage decision [14]. The population in Arab countries is characterized by large families, high maternal and paternal ages, and high levels of inbreeding [15]. A review conducted on the effectiveness of PMS and GC programs for beta thalassemia in Middle Eastern countries has revealed that a cancelation rate of 65% of at-risk marriage could not be achieved except in Iran, Turkey, and Iraq [9]. As an effort to identify the reason for the low response to this PMS program, we decided to review the studies conducted in this region on the aspects of knowledge and attitudes on PMS among the university students. Understanding the knowledge and attitude of this age group is crucial in developing strategies to create awareness and build positive attitudes towards PMS. University students are the target group as they are on the verge of making decisions about marriage.

Main text

Aims and methods

Whittemore and Knalf’s integrated review process with five steps was used. The steps involved are identification of the problem to be studied, thorough literature search, appraisal of the quality of data, examination of the data, and presentation of conclusions [16]. A systematic literature search was conducted to identify the studies exploring the knowledge and attitudes on PMS among university students. The search included databases like MEDLINE, PubMed, Cumulative Index to Nursing and Allied Health Literature (CINAHL), and Scopus. Key words including knowledge, attitude, premarital screening, university students, and genetic blood disorders were used in the search. Inclusion criteria included quantitative studies that (a) assessed the knowledge of university students on PMS, (b) explored the attitude of university students towards PMS, (c) were published in peer-reviewed articles in English, (d) were published between 2010 and 2020, and (e) were from Middle Eastern countries. The final criterion was included due to international variation in culture and health policies. The search yielded 328 articles, and twelve of them met the inclusion criteria for review as depicted in Fig. 1.

Fig. 1

Inclusion criteria for review

Measures

Each article was evaluated based on the revised criteria of the American Association of Critical-Care Nurses (AACN), which has six rating levels [17, 18]. All included articles in our review were quantitative descriptive studies scoring “C” for level of evidence.

Analysis of findings

All the selected papers were reviewed carefully by two authors (JRN and MAJ) independently and finalized based on the inclusion criteria. JRN and MAJ extracted data from the literature, and discrepancies were resolved by discussion. Data was analyzed and synthesized using the matrix table suggested by [19], and the details of the studies (author, year of publication, country, purpose, methods, and findings) with level of evidence are provided in Table 1.

Table 1 Summary of the articles with author, country, year, participants, methods, instruments, study purpose, and main findings

Results

A total of twelve studies conducted in six countries, including Kuwait, 1; Yemen, 1; Jordan, 1; Oman, 1; Egypt, 1; and KSA, 7, met the inclusion criteria for this review. All of the 12 studies were cross-sectional descriptive studies and have used researcher-developed self-reported questionnaires, as there are no valid standardized questionnaires available to assess the knowledge and attitude of PMS. The majority of the studies are conducted in the Middle Eastern countries where these hereditary disorders are commonly prevalent. The findings are discussed as narrative analysis under knowledge and attitudes on premarital screening of the university students in Table 2.

Table 2 Themes from the review on PMS

Discussion

The study has reviewed the knowledge and attitudes of Middle Eastern country university students on premarital screening. Regarding knowledge of the university students about PMS, eight of the studies reported good and adequate knowledge [14, 20,21,22,23,24,25,26]. One study reported fair level of knowledge [27], and two studies reported low level of knowledge [28,29,30]. One study even reported that female students had a good level of knowledge whereas male students had insufficient knowledge. Findings cannot be conclusive about the knowledge levels of the university students, as none of the studies used valid and reliable standardized questionnaires to assess the knowledge level on PMS. Even the educated Arab population has an extremely low level of genetic literacy, which poses a hindrance for genetic counseling [31]. Many Middle Eastern countries have realized the importance of PMS as a cost-effective measure to reduce the economic burden on society. Middle Eastern countries in general have good existing programs in reproductive and other primary health services where community genetic services could easily be integrated [32]. Massive health education starting from the higher secondary curriculum advancing to the college curriculum can increase knowledge levels. Education and examinations could be targeted towards unmarried male university students, which can facilitate them in making informed choices about unmarried females and consanguineous marriages.

Ten studies explored university students’ attitudes towards PMS while two studies did not. Though the majority of the students from the reviewed studies were reported to have good and adequate knowledge, it is discouraging to note that not all of them agreed to undergo PMS due to various religious and cultural beliefs. Three of the reviewed studies reported negative or fair attitudes of university students related to PMS [21, 25, 27]. Participants of seven reviewed studies had positive attitudes towards PMS, and even held beliefs about making policies about it stronger [14, 20, 22,23,24, 29, 30]. One third of the participants in a study reported they would proceed with their marriage even if both partners tested positive for thalassemia, and they were unwilling to undergo PMS as it would affect their future marriage opportunities [22]. A higher level of female literacy, fear of hereditary blood disorders, urbanization, and better economic status of the families have caused a decrease in consanguineous marriages in Arab countries in recent years [33]. In a study conducted in Oman on South Batinah Governorate’s adults, unwillingness to undergo PMS was associated with the following characteristics: female gender, lower education levels, younger age groups, and single populations [32]. Life cycle approaches are believed to attract a better holistic public response as a remedy to the barriers to PMS program success in the Middle East [9]. India and Canada successfully incorporated PMS into school health packages [34, 35]. Middle Eastern countries could follow this model of PMS incorporation at the higher secondary education level or make PMS mandatory for admission to the universities. Emphasis on structured health education on hereditary diseases and consanguineous marriages are needed in higher education levels.

Some participants believed that their fate is determined by God and therefore willing to accept the risk of bearing a sick child without undergoing PMS. This is similar to a study done in Oman among higher secondary school students who reported that they would accept the birth of children with genetic blood disorders as God’s will [36]. Though Islam promotes healthy marriage and the role of counseling, 90% of the couples detected as carriers in KSA proceeded with their marriages. A Nigerian study conducted among religious leaders had reported good knowledge and positive attitudes towards PMS for sickle cell disease [37], and a similar study can be conducted in Middle Eastern countries to understand the viewpoints of religious leaders. Individuals with incompatible genes expressed a fear of stigma associated with the carrier status. They frequently face familial pressures with consanguineous marriages. These problems could be reduced with community health education with the support of religious authorities [38]. There is a need for intensification of health education campaigns among youth, and religious leaders could be involved to clarify and correct misconceptions about PMS [22].

Limitations

All of the 12 studies reviewed relied upon the researcher-developed self-reported questionnaires. The questionnaires were developed and reviewed by experts in most of the cases. Future studies could observe the practice of PMS tests among university students along with their knowledge and attitudes. Only studies published in English were included, which might have resulted in the omission of valuable studies in other languages.

Conclusion

Various factors have contributed to the inadequate information on the extent to which these genetic blood disorders in the Middle Eastern region pose health and economic burdens; this exposes the importance of sustainable research activities in this area. The findings of this review have opened up new areas of health education needs among the yet-to-be-married couples (university students) regarding genetic blood disorders. Health education strategies involving family members, lawmakers, and religious leaders could enhance the younger Middle Eastern population’s compliance to PMS. Mass outreach programs through various social media platforms is the need of the hour to create awareness among the younger generation and to alleviate the burden of these genetic blood disorders on the individuals and society in the Middle East. A very exciting phase for reducing the genetic burden in the human population in this region of the world has begun, and it is hoped that this mission will succeed.