Awareness of Sickle Cell Disease Among Adolescents and Young Adults

Abstract

Background: Sickle hemoglobinopathy is the most common genetic disorder identified through the state mandated newborn screening program, occurring in 1:2,647 births and may pose an enormous health burden. While sickle cell disease is associated with significant morbidity, sickle cell trait is a benign condition. Due to this reason, knowledge of their sickle cell trait for an individual may be lacking while the condition may have been identified at birth. There were 2904 cases of sickle cell traits identified through the NJ newborn screening program in 2020. While other countries have programs to increase awareness of sickle hemoglobinopathy status to include premarital genetic screening, the implication of sickle cell trait on procreation and family planning are not highly emphasized in the US. Assessing awareness of sickle cell disease among adolescents is important given high rates of teenage pregnancies among this population. Through this study, we set out to assess awareness of sickle cell disease among adolescents. Methods: We performed a cross sectional study of 180 adolescents who sought medical care at one of the outpatient clinics or emergency room at Children's Hospital of New Jersey in Newark, NJ and administered an anonymous questionnaire to volunteers 12-21 years of age from January 2020-June 2022. The study was approved by our local IRB and an informed consent and/or assent was obtained from each enrolled subject. Questionnaires included a variety of questions including demographics and several questions to assess their knowledge of sickle cell disease. Questionnaires that were incomplete were not included in analysis. Results: Of the 180 adolescents, most were teenagers ages 14-18 years old (72%), majority identified as females (59%) and 92% were African Americans or Hispanic. More than 30% of subjects' parents were high school graduates, 25% were college graduates and the remainder had some other lower level of education. Out of 180,136 children (76%) had heard of sickle cell disease and only 78 children (44%) reported that they knew about sickle cell disease. However, when asked questions regarding sickle cell disease, only 12% were able to correctly identify that it is a genetic disease that may cause pain crises. One hundred and eighteen patients (66%) were unaware of their sickle cell status. Of those with sexual partners, 53% did not think that knowing their partner's sickle cell status would influence their choice and yet, 59% wouldn't consider having a child with someone who has sickle cell disease. Only 5 individuals (2.78%) were able to correctly identify the inheritance pattern of sickle cell disease. Of the 4 individuals with children, only 1 (25%) reported being aware of their child's sickle cell status. Ninety-two percent were interested in learning about sickle cell disease, and the majority identified their doctor, internet and school as primary forms of obtaining health information. Conclusion: Sickle cell disease is associated with significant health burden. While we cannot prevent sickle cell disease, as health care providers, we should make every effort to educate youth about this important condition. One of the ways is to review newborn screening hemoglobinopathy results during adolescent well visits, especially for those with sickle cell trait. Appropriate education and genetic counseling must be provided to these young individuals especially since many may engage in sexual activity at a young age.