Home Letters to the Editor Current state of quality of care for sickle cell trait carrier awareness

Current state of quality of care for sickle cell trait carrier awareness

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According to the Centers for Disease Control and Prevention, “Sickle cell trait affects approximately 100,000 individuals in the United States. Sickle cell trait and sickle cell disease are particularly common among those with ancestors from sub-Saharan Africa; Spanish-speaking regions in the Western Hemisphere (South America, Caribbean, and Central America); Saudi Arabia; India; and Mediterranean countries such as Turkey, Greece, and Italy.” The importance of knowing one’s sickle cell trait status involves reproductive health decisions and self-care health initiatives to improve health outcomes. Sickle cell trait is a recessive autosomal gene that may not cause problems on its own, when two people who are sickle cell trait carriers reproduce, the child may have sickle cell disease, which leads to substantial morbidity and premature mortality for the child. Sickle cell trait carriers may be asymptomatic and lead normal lives, but it has been found to cause long-term health complications of renal and venous disease in some cases. In extreme conditions such as dehydration and high-intensity physical activity, sickle cell trait carriers can experience serious health issues, including sudden death. As described by the Texas Children’s Hospital, “The only cure for sickle cell disease is bone marrow transplants, but they are only effective in children who have a full brother or sister without sickle cell disease and closely matching bone marrow.”

Necessity of sickle cell trait status awareness policy

Currently, no policy exists to address people born before 2006 to identify the at-risk population for sickle cell trait status. In 2006, the United States began screening all newborns for sickle cell trait and sickle cell disease. People born before 2006 are a vulnerable population for unknown sickle cell trait status. Possible sickle cell trait carriers will need genetic testing and, if positive, genetic counseling for reproductive health decisions. Also, positive sickle cell trait carriers will need education for self-care initiatives to avoid possible life-long complications. A policy for identifying sickle cell trait carriers born before 2006 could improve long-term health outcomes and decrease sickle cell disease occurrence in this population.

Improving the quality of care for sickle cell trait carriers

A policy for the population at risk for sickle cell trait born before 2006 is needed to raise awareness of the need for individual’s knowledge of sickle cell trait status and provide resources for testing, genetic counseling, and self-care education to reduce long-term health complications and make informed reproductive decisions. The policy should include healthcare providers, educators, and community leaders to reach this diverse at-risk population. Sickle cell trait and sickle cell disease education should be provided to interprofessional team members to effectively work with the diverse population. Informational and educational opportunities for this at-risk population should be offered in different venues due to the age span and cultural diversity of the population.

Suggested venues include the individuals’ healthcare providers, school nurses, public healthcare, community outreach groups in culturally diverse communities, and readily available pamphlet distributions. Resources for genetic testing, genetic counseling, and self-care education should be made available to the at-risk population.

People who know their sickle cell trait status can be proactive in making informed decisions for reproductive health and self-care measures to decrease complications. The study by Creary and colleagues found individuals with low knowledge of sickle cell demonstrated improved knowledge after in-person counseling for caregivers of individuals with sickle cell trait and sickle cell disease.

How the policy will support achievement of desired outcomes

A sickle cell trait status awareness policy will increase healthcare provider, educational institution, and community awareness and help them identify at-risk individuals born before 2006 to improve well-being and long-term health. The goals of the policy are to decrease health complications and healthcare costs and increase informed reproductive decisions of those with sickle cell trait. Knowledge of sickle cell trait status allows incorporating preventive into everyday life to prevent long-term renal and venous complications, which are costly and decrease quality of life. A positive sickle cell trait carrier can seek genetic counseling to make informed decisions for reproductive health in light of possibly passing sickle cell disease to their children. Quality of life will be improved for this at-risk population.

Policy development advocacy

Sickle cell trait status awareness policy will provide at-risk populations born before 2006 with information about the implications of sickle cell trait on their health and reproductive decisions so they can seek testing and if positive seek education for managing the condition to decrease health and reproductive complications. For athletes and physically active individuals, this knowledge could prevent sudden death. For young adults dreaming of marriage and parenthood, this knowledge can direct them to seek genetic counseling to make informed decisions for the future.

References

American Society of Hematology. Sickle cell disease. hematology.org/education/patients/anemia/sickle-cell-disease

Centers for Disease Control and Prevention. cdc.gov/ncbddd/hemoglobinopathies/features/keyfinding-state-based.html

Creary S, Adan I, Stanek J, et al. Sickle cell trait knowledge and health literacy in caregivers who receive in-person sickle cell trait education. Mol Genet Genomic Med. 2017;5(6): 692-9. doi:10.1002/mgg3.327

Pecker L, Naik R. The current state of sickle cell trait: Implications for reproductive and genetic counseling. Blood. 2018;132(22):2331-8. doi:10.1182/blood-2018-06-848705

Texas Children’s Hospital. Bone marrow transplantation in children with sickle cell disease. texaschildrens.org/health/bone-marrow-transplantation-children-sickle-cell-disease

Thoreson CK, O’Conner MY, Ricks M, Chung ST, Sumner AE. Sickle cell trait from a metabolic, renal, and vascular perspective: Linking history, knowledge, and health. J Racial Ethn Health Disparities. 20152(3):330-5. doi:10.1007/s40615-014-0077-4

Virginia K. Street, RN
Hilham, TN

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