Genetic Education and Sickle Cell Disease

Abstract

Sickle cell disease (SCD) genetic knowledge is important when individuals make reproductive decisions. This study assessed feasibility and efficacy of delivering basic genetic information to 101 adolescents with SCD. Participants completed a questionnaire to test SCD genetic knowledge at 3 timepoints: before genetic education session (pretest), after the session (posttest), and 6 months later (follow-up). Scores at 3 timepoints were compared by Wilcoxon signed-rank tests, and group differences were compared by Wilcoxon-Mann-Whitney and Kruskal-Wallis tests. Participants' median scores significantly increased from pretest to posttest and from pretest to follow-up. Males had a greater change in scores than females. Scores decreased slightly from posttest to follow-up. Participants with HbSS/HbSβ⁰-thal genotype and participants with more prior pain episodes exhibited a smaller increase in median scores than those with HbSC/HbSβ⁺-thal genotype and no prior pain history; however, all groups had substantial gains from pretest to posttest and follow-up tests demonstrating that adolescents with SCD can learn basic genetics. This study established that genetic education can successfully be incorporated in transition to adult care programs for adolescents with SCD. Genetic education should be included in the standard plan of care for adolescents with SCD to assist them in making informed reproductive choices.

Sickle Cell Disease: A Review of Nonpharmacological Approaches for Pain

“…In our broad literature search, there were nine studies that tested educational interventions for persons with SCD (56–64), but none included pain as an outcome and thus were excluded in this review.…”

Sickle cell carriers' unmet information needs: Beyond knowing trait status

“…For example, it was apparent that when information on the genetic risk for SCD was received, carriers did not accurately understand these risks. Therefore, implementing strategies such as the "teach-back" method may be helpful to confirm that this information has been accurately received (Nutbeam et al, 2017;Porter et al, 2014). This method is shown to be effective in genetic education (Porter et al, 2014;Tluczek et al, 2011).…”

“…Therefore, implementing strategies such as the "teach-back" method may be helpful to confirm that this information has been accurately received (Nutbeam et al, 2017;Porter et al, 2014). This method is shown to be effective in genetic education (Porter et al, 2014;Tluczek et al, 2011). Teach-back techniques involve asking participants to use their own words to illustrate their understanding of material discussed (Kripilani, Bengtzen, Henderson, & Jacobsen, 2008).…”

Development of an Online Reproductive Health Intervention for Individuals with Sickle Cell Disease or Trait

“… 4 We identified one study that investigated the efficacy of a clinically delivered, genetic education program for adolescents with SCD. 5 Porter et al used a genetic counseling framework with a candy model strategy to convey genetic risks and found that adolescents were able to grasp the basic genetic inheritance concepts of SCD. Other quantitative studies explored the attitudes and beliefs and desire for reproductive health knowledge on SCD or SCT.…”

“…Other quantitative studies explored the attitudes and beliefs and desire for reproductive health knowledge on SCD or SCT. 5–8 Each of these studies was conducted in the United States, except for Melaibari et al's study, which investigated views of young adults in Saudi Arabia on the national premarital screening program of the country. 9 Findings were similar across the board, with studies indicating a general lack of knowledge about reproductive options and sickle cell inheritance, and confusion about adequacy of resources for this information.…”