Parental Counseling on Sickle Cell Trait: A Scoping Review and Plan for Implementation
Authors: Vorri Stamatia C , Rivlin Kenneth 
Published: June 2024

Presentation Date: 6/8/2024

Presentation Start Time: 6:00:00 PM

Background

Newborn screening (NBS) for sickle cell disease (SCD) has resulted in a significant reduction in morbidity and mortality. All states have established protocols to ensure all infants identified with SCD receive appropriate care. A consequence of this screening is the identification of individuals with sickle cell trait (SCT). There is consensus among healthcare professionals and the sickle cell community on the need to establish a coherent counseling program for parents and adolescents with SCT found on NBS. However, there is no consensus on how this should be done. Currently, most NBS programs leave this to the primary care physician (PCP). Communication of these results and the counseling provided varies greatly by the PCPs. A recent NYC law tasked the NYC DOHMH with guidance for the NYC public hospital system to develop provider education to improve trait counseling. This study aims to review the literature to identify common themes in SCT counseling and inform an SCT educational and implementation strategy.

Methods

Using a combination of keywords, we searched PubMed for literature on parental counseling of newborn carriers for SCT. We identified 297 publications, which we filtered to exclude literature before 1975 (when NBS was first implemented in the US) and/or not available in English. We also searched relevant records’ reference lists. We removed duplicates and screened the remaining 234 titles and abstracts to exclude anything focusing on sickle cell disease, epidemiology of SCT, or high-risk population screening. We screened 170 full-text articles and excluded 92 of them based on pre-selected inclusion criteria.

Results

Common themes that emerged in the literature involve 1) The lack of counseling standardization, 2) Pros and cons of informing parents, 3) Parental experiences, preferences, and attitudes, 4) Counselor training (clinician, community members), and 5) Rare complications of SCT. There is evidence that the observed lack of counseling protocols leads to most families being unaware of the child’s SCT status. Non-standardized counseling can have negative effects, such as parental anxiety, vulnerable child syndrome, and discrimination. Carriers themselves have inadequate status knowledge and are not routinely offered reproductive counseling in adolescence or young adulthood. Proper counseling and follow-up on the other hand can decrease anxiety, provide a space for questions, initiate family planning discussions, and early recognition and prevention of rare SCT complications important for public health.

Conclusions

Current practices on SCT counseling highlight the critical need for standardization of what is said, when, and how counseling is done. The historical, racial, and cultural context of SCT counseling must be taken into consideration. An implementation strategy should involve primary care provider education and standardization of counseling methodology. The content, timing, and setting of the counseling need to be optimized to minimize parental anxiety and increase carrier status awareness. Also, counseling should ideally be repeated to involve SCT carries themselves when they reach early adolescence. This will ensure the overall higher status knowledge and prevention of health complications.

PRISMA diagram for scoping review

Research strategy for record identification and screening of included papers