What does shared decision making look like in natural settings? A mixed methods study of patient–provider conversations

Objective: To understand the variability and nature of shared decision making (SDM) regarding a uniform type of serious medical decision, and to make normative judgments about how these conversations might be improved. Methods: This was a mixed-methods sub-analysis of the Improving Patient Outcomes with Respect and Trust (IMPORT) study. We used the Braddock framework to identify and describe seven elements of SDM in audio-recorded encounters regarding initiation of hydroxyurea, and used data from medical records and patient questionnaires to understand whether and how these tasks were achieved. Results: Physicians covered a spectrum of SDM behaviors: all dialogues contained discussion regarding the clinical issue and the pros and cons of treatment; the patient's understanding and role were not explicitly assessed or stated in any encounter. Yet no patient agreed to start hydroxyurea who did not already prefer it. There was no uniform approach to how physicians presented risk; many concerns expressed by patients in a pre-visit questionnaire were not discussed. Conclusion: In this analysis, patients seemed to understand their role in the decision-making process, suggesting that a patient's role may not always need to be explicitly stated. However, shared decision making might be improved with more routine assessment of patient understanding and concerns. Standardized decision aids might help fully inform patients of risks and benefits

Association of Sickle Cell Trait With Chronic Kidney Disease and Albuminuria in African Americans

The association between sickle cell trait (SCT) and chronic kidney disease (CKD) is uncertain

Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor

Objective. We present the case of a 73-year-old female, with no family or personal history of a bleeding disorder, who had a classic presentation for acquired hemophilia A. Factor VIII activity was low but detectable and a factor VIII inhibitor was undetectable. Methods. The patient’s plasma was comprehensively studied to determine the cause of the acquired coagulopathy. Using the Nijmegen modification of the Bethesda assay, no factor VIII autoantibody was measureable despite varying the incubation time from 1 to 3 hours. Results. The aPTT was prolonged at 46.8 seconds, which did not correct in the 4 : 1 mix but did with 1 : 1 mix. Using a one stage factor VIII activity assay, the FVIII activity was 16% and chromogenic FVIII activity was also 16%. The patient was treated with recombinant FVII and transfusion, significantly reducing bleeding. Long-term therapy was initiated with cyclophosphamide and prednisone with normalization of FVIII activity. Conclusions. Physicians can be presented with the challenging clinical picture of an acquired factor VIII inhibitor without a detectable inhibitor by the Bethesda assay. Standard therapy for an acquired hemophilia A should be considered

Case Report Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor

Objective. We present the case of a 73-year-old female, with no family or personal history of a bleeding disorder, who had a classic presentation for acquired hemophilia A. Factor VIII activity was low but detectable and a factor VIII inhibitor was undetectable. Methods. The patient's plasma was comprehensively studied to determine the cause of the acquired coagulopathy. Using the Nijmegen modification of the Bethesda assay, no factor VIII autoantibody was measureable despite varying the incubation time from 1 to 3 hours. Results. The aPTT was prolonged at 46.8 seconds, which did not correct in the 4 : 1 mix but did with 1 : 1 mix. Using a one stage factor VIII activity assay, the FVIII activity was 16% and chromogenic FVIII activity was also 16%. The patient was treated with recombinant FVII and transfusion, significantly reducing bleeding. Long-term therapy was initiated with cyclophosphamide and prednisone with normalization of FVIII activity. Conclusions. Physicians can be presented with the challenging clinical picture of an acquired factor VIII inhibitor without a detectable inhibitor by the Bethesda assay. Standard therapy for an acquired hemophilia A should be considered

Marijuana use and health behaviors in a US clinic sample of patients with sickle cell disease.

IntroductionAs marijuana use becomes more common, it is essential clinicians understand the relationship between marijuana use and health behaviors.MethodsUsing a retrospective cohort of adolescents and adults with sickle cell disease (SCD) stratified into a young ( = 25 years), we conducted multiple linear regression examining relationship of marijuana use (independent variable) on each dependent variable (SCD self-management score and pain management).ResultsAmong young cohort, 16.9% used marijuana compared to 21.8% of older cohort. The younger cohort reporting marijuana use had lower mean self-care scores (β = -2.74;p = 0.009) and were more likely to have admissions to the hospital for pain (β = 0.87;p = 0.047) compared to non-users. In contrast, the older cohort reporting marijuana use had more days treating pain at home (β = 0.44;p = 0.035).ConclusionsOnly a minority of patients with SCD reported lifetime marijuana use. Among those reporting marijuana use, there were different associations with self-care and health-related behaviors by age. The older cohort who endorsed marijuana use reported more days of treating pain at home, although this did not translate into increased acute care visits for pain crisis. Among youth, endorsing marijuana use was associated with worse SCD self-care

Patent Foramen Ovale in Patients with Sickle Cell Disease and Stroke: Case Presentations and Review of the Literature

Although individuals with sickle cell disease (SCD) are at increased risk for stroke, the underlying pathophysiology is incompletely understood. Intracardiac shunting via a patent foramen ovale (PFO) is associated with cryptogenic stroke in individuals without SCD. Recent evidence suggests that PFOs are associated with stroke in children with SCD, although the role of PFOs in adults with stroke and SCD is unknown. Here, we report 2 young adults with SCD, stroke, and PFOs. The first patient had hemoglobin SC and presented with a transient ischemic attack and a subsequent ischemic stroke. There was no evidence of cerebral vascular disease on imaging studies and the PFO was closed. The second patient had hemoglobin SS and two acute ischemic strokes. She had cerebral vascular disease with moyamoya in addition to a peripheral deep venous thrombosis (DVT). Chronic transfusion therapy was recommended, and the DVT was managed with warfarin. The PFO was not closed, and the patients' neurologic symptoms were stabilized. We review the literature on PFOs and stroke in SCD. Our cases and the literature review illustrate the dire need for further research to evaluate PFO as a potential risk factor for stroke in adults with SCD

Preliminary evidence that hydroxyurea is associated with attenuated peripheral sensitization in adults with sickle cell disease

Abstract. Introduction:. Hydroxyurea (HU) is a drug that targets the underlying pathophysiology of sickle cell disease (SCD); however, it continues to be an underutilized treatment for adults. Previous research suggests that HU treatment can result in fewer hospital contacts for acute vaso-occlusive pain crises (VOC). Hydroxyurea's impact on non-VOC pain, however, is not well established. Objectives:. This study examined whether HU moderated patterns of static and dynamic pain processing and clinical pain in SCD individuals. Methods:. Fifty-eight patients with SCD (N taking HU = 17) underwent quantitative sensory testing (QST) and completed twice daily symptom diaries for 12 weeks. Quantitative sensory testing established thermal threshold and tolerance, mechanical thresholds, and thermal and mechanical temporal summation of pain. Results:. Groups did not differ in age, sex, or opioid use. After controlling for morphine use, QST results showed that participants taking HU had higher heat and mechanical pain thresholds (static QST measures) but not thermal and mechanical temporal summation (dynamic QST measures). Participants taking HU also reported lower VOC pain compared with SCD participants not taking HU; however, HU did not moderate non-VOC clinical pain ratings. Conclusion:. Findings cautiously suggest that HU acts on pain hypersensitivity and VOC pain, rather than inhibiting pain facilitation and non-VOC pain. These differences may reflect HU's influence on peripheral rather than central sensitization. Future research is warranted to replicate these findings in a larger sample and determine whether early HU administration can prevent peripheral sensitization in SCD individuals