Promoting Sickle Cell Trait Awareness and Education: A Typology of Interventions in the United States to Inform Ongoing Efforts to Patients and Providers

Research Background: Sickle cell trait has no treatment or cure and predominantly affects people who are Black, but can affect anyone of any race or ethnicity. While commonly incorrectly considered benign by providers and the public, people with a sickle cell trait experience life-threatening outcomes that are exacerbated by extreme conditions. There is a severe lack of awareness and understanding of sickle cell trait and the associated health complications among sickle cell trait carriers and healthcare providers. Purpose/Aim: Interventions that aim to improve awareness of sickle cell trait differ in approaches and are not well documented in the literature. This typology aims to highlight current efforts to inform targeted interventions that raise awareness through consistent messaging, educate people and providers on sickle cell trait and the related health complications, and support the design and implementation of comprehensive sickle cell trait awareness initiatives. Methods: We conducted a scoping review of United States-based sickle cell trait interventions and performed a content analysis to identify the categories and characteristics of these efforts. We then organized the results into a typology according to established protocols. Results: Among 164 interventions, twenty-five (15%) met the typology inclusion criteria described above and were grouped into categories: Seven of twenty-five interventions were Educational Interventions (28%), three of twenty-five interventions (12%) were Combined Screening and Educational-Based Interventions, eight of twenty-five interventions (32%) were Policy and Guideline-Based Intervention, and six of twenty-five interventions (24%) were Sickle Cell Trait Organization-Led Interventions. Conclusions: There is a lack of consistency in messaging across interventions whether delivered by credible healthcare institutions or national organizations, which can result in lack of education and awareness and confusion around sickle cell trait. Categorizing interventions through a typology allows clarity and informs consistency in messaging, which should be at the forefront of future sickle cell trait efforts.

Choroidal vascular occlusion in a young male patient with sickle cell trait

Dear Editor,Choroidal vascular occlusion is a rare finding. Choroidal perfusion disorders may range from focal infarction of the choriocapillaris to fibrinoid arteriolar necrosis. Vascular occlusion due to microembolism is more common in choroidal than in retinal vasculature. Due to its unique hemodynamic characteristics, which involve one of the highest rates of blood flow in the body and the fact that it is functionally an end artery system, the choroidal circulation is prone to platelet emboli associated with cardiovascular disease and other pathologies with increased risk of microembolismt.

Sickle Cell Trait, Hemoglobin Levels and Anemia among Black Patients with Predialysis Chronic Kidney Disease: A Post Hoc Analysis

Objective: To assess the relationship between SCT, hemoglobin levels and anemia in CKD black patients. Method: A post-hoc analysis of data from 188 patients, enrolled in a cross-sectional study of sickle cell trait (SCT) and chronic kidney disease (CKD), was performed to assess the relationship between SCT, hemoglobin (Hb) levels and anemia defined as Hb < 12 g/dl in men and <11 g/dl in women. Student t test, Mann Whitney and Chi square test were used as appropriate for different comparisons. P < 0.05 defined the level of statistical significance. Results: SCT (HbAS) and normal hemoglobin (HbAA) were present in 39 (21%) and 149 (79%) CKD patients, respectively. Despite similar estimated GFR (eGFR) and age, HbAS patients had significantly lower Hb levels (8.8 ± 1.8 vs 10 ± 2.2 g/dl;p = 0.001) and a higher proportion of anemia (95% vs 72%, p = 0.001). In multiple linear regression analysis, eGFR, BMI, SBP and SCT emerged as independent determinants of Hb levels. The presence of SCT was associated with 1.185 g/dl decrease in Hb levels. Conclusion: In the present case series, SCT was associated with lower Hb levels suggesting its potential contribution to the pathogenesis of CKD-associated anemia.

Stiffening of sickle cell trait red blood cells under simulated strenuous exercise conditions

The higher risk of vaso-occlusion events and sudden death for sickle-cell trait(SCT)athletes has been speculatively ascribed to SCT red blood cell(RBC)stiffening during strenuous exercise.However,the microenvironmental changes that could induce the stiffening of SCT RBCs are unknown.To address this question,we measured the mechanical properties of and changes in SCT RBCs under deoxygenated and acidic environments,which are two typical conditions present in the circulation of athletes undertaking strenuous exercise.The results reveal that SCT RBCs are inherently stiffer than RBCs from non-SCT healthy subjects,and a lower pH further stiffens the SCT cells.Furthermore,at both normal and low pH levels,deoxygenation was found to not be the cause of the stiffness of SCT RBCs.This study confirms that the stiffening of SCT RBCs occurs at a low pH and implies that SCT RBC stiffening could be responsible for vaso-occlusion in SCT athletes during strenuous exercise.

RMC （Renal Medullary Cancer）： A Case Report and Literature Review

RMC （renal medullary carcinoma） is considered a rare entity, corresponding to approximately 2% of primary kidney tumors, usually occurring in the second decade of the patient＇s life and it is closely related with previous history of sickle cell disease. Studies have reported RMC as highly aggressive, poorly responsive to chemotherapy, surgery, radiotherapy or targeted therapy. This disease＇s median overall survival is less than 12 months. We are reporting a case of a 21-year-old woman with sickle cell trait, whose initial clinical presentation was RMC already metastatic to the liver. She underwent total right nephrectomy, and her clinical picture worsened during hospitalization with pneumonia, bowel obstruction, oliguria and death in less than two months after diagnosis, with no performance status to attempt any treatment options to her cancer at any time. Although some cases in the literature had reported good response to neoadjuvant therapy, this disease has early recurrence and little response to treatment. Our objective is to call the attention for this aggressive desease to estimulate new perspectives and further studies with chemotherapy and other different kinds of treatment, mainly because only surgical intervention is an incomplete aproach.