Objective Current guidelines recommend high‐priority treatment of severe sickle cell disease (SCD) pain with opioids; however, patients with SCD have historically been undertreated. We used mixed methods to assess pediatric residents’ perceptions toward opioid use in SCD pain management. Methods We distributed a survey to 88 residents at an urban pediatric medical center in a cross‐sectional study. Participants responded to questions about perceived barriers to acute SCD pain management and attitudes toward patients with SCD. Responses were examined using bivariate analyses. Five pediatric residents were interviewed, to provide more in‐depth understanding of barriers to SCD pain management. Results Fifty‐three residents (60%) completed the survey. Participants were divided into “more experienced” (had seen ≥ 21 patients with SCD; 45.3%) or “less experienced.” Both groups reported potential for tolerance and dependence as major barriers to opioid use in SCD. Less experienced residents reported a greater need for additional training in SCD pain management (P < 0.05), more concern about addiction, and greater empathy for patients with SCD (P < 0.05). Both groups agreed that patients with SCD were “frustrating to care for.” Thematic analysis revealed that increased patient and provider barriers led to distrust, ultimately leading to undertreatment of pain and inadequate care. Conclusion Although more experienced residents reported feeling more comfortable treating acute SCD pain and were less concerned with addiction compared with less experienced residents, certain negative views of patients with SCD were prevalent among all residents. Findings suggest that residency training must address provider attitudes as well as knowledge about SCD.
Protein S (PS) deficiency is associated with a well documented risk of venous thromboembolism. However, the relation between PS deficiency itself to arterial thrombotic events (ATEs) is not clearly established. In our case, we report an ATE in a patient with a documented novel PROS1 mutation and a family history of PS deficiency. Other etiologies for arterial thrombosis were excluded. The role of precise diagnosis with levels of PS and documentation for mutational analysis are discussed. We highlight the problems with diagnosis in previously reported cases with arterial thrombotic events and discuss the potential for treatment with antiplatelet therapy in a subset of patients with PS deficiency.
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