Social determinants of health and treatment center affiliation: analysis from the sickle cell disease implementation consortium registry.

BACKGROUND: Adults with sickle cell disease (SCD) suffer early mortality and high morbidity. Many are not affiliated with SCD centers, defined as no ambulatory visit with a SCD specialist in 2 years. Negative social determinants of health (SDOH) can impair access to care. HYPOTHESIS: Negative SDOH are more likely to be experienced by unaffiliated adults than adults who regularly receive expert SCD care. METHODS: Cross-sectional analysis of the SCD Implementation Consortium (SCDIC) Registry, a convenience sample at 8 academic SCD centers in 2017-2019. A Distressed Communities Index (DCI) score was assigned to each registry member's zip code. Insurance status and other barriers to care were self-reported. Most patients were enrolled in the clinic or hospital setting. RESULTS: The SCDIC Registry enrolled 288 Unaffiliated and 2110 Affiliated SCD patients, ages 15-45y. The highest DCI quintile accounted for 39% of both Unaffiliated and Affiliated patients. Lack of health insurance was reported by 19% of Unaffiliated versus 7% of Affiliated patients. The most frequently selected barriers to care for both groups were "previous bad experience with the healthcare system" (40%) and "Worry about Cost" (17%). SCD co-morbidities had no straightforward trend of association with Unaffiliated status. The 8 sites' results varied. CONCLUSION: The DCI economic measure of SDOH was not associated with Unaffiliated status of patients recruited in the health care delivery setting. SCDIC Registrants reside in more distressed communities than other Americans. Other SDOH themes of affordability and negative experiences might contribute to Unaffiliated status. Recruiting Unaffiliated SCD patients to care might benefit from systems adopting value-based patient-centered solutions.

Janus kinase inhibition in the treatment and prevention of graft-versus-host disease.

Graft-versus-host disease (GVHD) is a significant cause of morbidity and mortality after allogeneic hematopoietic stem cell transplantation (HSCT). For many years, corticosteroids have been the mainstay treatment for GVHD, but cases of steroid-refractory GVHD and the severe adverse effects of high-dose corticosteroids have increased the need for preventative and therapeutic strategies for GVHD. Due to the nature of alloreactive T cells, GVHD is inherently linked to the graft-versus-leukemia (GVL) effect, the therapeutic driving force behind stem cell transplantation. A considerable clinical challenge is to preserve GVL while suppressing GVHD. The field of GVHD research has greatly expanded over the past decades, including advancements in T cell modulation and depletion, antibody therapies, chemotherapeutics, cellular therapies, and Janus kinase inhibition. In this review, we discuss current approaches and advances in the prophylaxis and treatment of GVHD with a focus on new emerging advancements in Janus kinase inhibitor therapy.

Thrombotic Thrombocytopenic Purpura Masquerading as Acute Ischemic Stroke.

Thrombotic thrombocytopenic purpura (TTP) is a hematologic disorder that results in widespread clotting due to a deficiency of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) protease. This leads to excessive von Willebrand factor (VWF) protein-platelet multimers. Due to platelet consumption, platelet levels fall, resulting in thrombocytopenia. We examined a case of a 31-year-old female with no significant medical history who presented with expressive aphasia and paresthesias and was sent to the Emergency Department for a stroke workup. Imaging was negative for ischemic or hemorrhagic stroke; however, a complete blood count (CBC) was consistent with anemia and thrombocytopenia, resulting in a high suspicion for TTP. She was admitted to the intensive care unit and given fresh frozen plasma and packed red blood cells. Plasma exchange therapy was initiated, and her aphasia and paresthesias began to improve. The ADAMTS13 level returned at less than 5%, which prompted the initiation of rituximab therapy. During her 36-day hospital stay, she continued to receive daily steroids and plasma exchange and a total of four courses of rituximab. Her platelets steadily climbed, and she was discharged with instructions to follow up with outpatient hematology. TTP is a thrombotic microangiopathy that results in microscopic blood clots anywhere in the body, including the cerebral arteries. This results in the neurologic abnormalities that are often seen in TTP. Because TTP is a rare disease, treatment modalities are still scarce but include steroids, plasma exchange therapy, and rituximab. Novel therapies are on their way, one being caplacizumab, a monoclonal antibody that inhibits VWF from interaction with glycoprotein 1b. A concern highlighted by this case is the exclusion criteria for the administration of tissue plasminogen activator (TPA). As this patient presented with stroke symptoms and a negative head CT, TPA would have been administered had a CBC not returned showing evidence of TTP. This highlights the importance of strict adherence to the American Heart Association/American Stroke Association guidelines that include ensuring that the platelet count is >100,000 prior to the initiation of TPA.

Understanding protein synthesis: a role-play approach in large undergraduate human anatomy and physiology classes.

This study investigated the effectiveness of role play in a large undergraduate science class. The targeted population consisted of 298 students enrolled in 2 sections of an undergraduate Human Anatomy and Physiology course taught by the same instructor. The section engaged in the role-play activity served as the study group, whereas the section presented with a traditional lecture served as the control group. A pretest/posttest assessment and a survey were administered to both sections and used in data analysis. In addition, overall test scores and item analysis were examined. The analysis revealed that participants in both groups improved significantly from pretest to posttest, but there were no significant differences between the groups in posttest scores. Neither group showed a significant change from posttest to the exam. However, there was a moderate positive effect on engagement and satisfaction survey questions from being in the study group (based on 255 total surveys returned by both groups). The role-play activity was at least as effective as the lecture in terms of student performance on the above-mentioned assessments. In addition, it proved successful in engaging students in the learning process and increasing their satisfaction.