How people in Benin assess a couple’s risk of having a baby with sickle cell disease

Abstract

Sickle cell disease (SCD) is a genetic disease resulting from the inheritance from both parents of a mutant hemoglobin gene. Its occurrence can, at best, be prevented, and its daily life consequences can, at least, be limited. As the mutant gene is recessive, it should be useful for people living in countries where SCD is endemic to know their own genetic status and that of their actual or potential partner in order to assess the risk of having a baby with SCD. The present study aimed at examining how a convenience sample of 128 lay people and nine physicians in Benin judge the likelihood that a newborn will suffer from SCD as a function of the genetic status of the baby's mother and father. As expected, several qualitatively different clusters of participants were found. A minority (29 %) made judgments that were largely consistent with the correct rule for determining the likelihood of disease. A larger group (37 %) expressed, however, the view that (a) to know a child's likelihood of suffering from SCD, information is needed about the genetic statuses of both parents and (b) this likelihood depends additively on these genetic statuses. Finally, another group (34 %) thought that, if one parent is suffering from SCD or is a carrier of a sickle gene, the likelihood that the child will have SCD is high, irrespective of the other parent's status. Thus, even among a relatively well-educated group of people in Benin, only a minority used the correct judgment rule when assessing the risk of SCD. Work needs to be done to educate the population regarding the proper way to combine information.

Individual differences in information integration studies of children’s judgment/decision-making: Combining group with single-subject design via cluster analysis

“…Nevertheless, the individual difference groupings identified by cluster analysis are not automatically real, but must, in the end prove their lasting value in validation studies linking the clusters to variables outside the classification data. Throughout this chapter we emphasized that the clusters were internally coherent, with clear process interpretations and developmental trends as also found in other domains (Gamelin et al, 2006, Zounon et al, 2015Muñoz Sastre et al, 1998). Studies in other domains found meaningful learning effects, such that participants moved from less to more advanced clusters after feedback (Liegeois et al, 2003).…”

Commentary: Rehabilitation: a key service, yet highly underused, in the management of young patients with sickle cell disease after stroke in DR of Congo

“…In Benin, as in most sub-Saharan African countries, systematic screening for SCD is not a common practice as most sickle cell-trait carriers often are asymptomatic. This lack of systematic screening increases the rate of high-risk marriages and thus maintains the prevalence of the disease in the population (Alhamdan et al, 2007;Zounon et al, 2015). Therefore, the first challenge in the fight against SCD remains primary prevention through strategies that use systematic screening for SCD in the population and doing this in strict compliance with ethical rules.…”

“…Second, there is a need to improve health education programs for the population, more efforts are needed for the counseling of couples, and good management in timing screening could decrease high-risk marriages and ultimately reduce the prevalence of the disease. In this perspective, public authorities, as well as religious and traditional authorities, can play important roles (Alhamdan et al, 2007;Rahimy et al, 2009;Zounon et al, 2015;Ezugwu et al, 2019).…”

Ethical issues in denial of church wedding based on couple’s hemoglobin genotype in Enugu, south eastern Nigeria

“…Sickle cell anemia (SCA) is an autosomal recessive blood disorder [1]. Carriers who have one copy of the mutant hemoglobin S gene typically have no symptoms.…”