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Background It is unknown whether nurses' knowledge about pain among patients with sickle cell disease (SCD) reflects the current standard of care. The authors evaluated changes in nurses' knowledge and simulated practice behavior after a continuing education program. Method Inpatient nurses completed an e-learning program on SCD pain; a pretest and a posttest with the same 10 questions; and two patient cases with four pain intervention options at the posttest. Results On the pretest, the mean percentage of correct answers was 83% ( SD = 13%). The mean percentage of correct answers increased by 12% ( p < .0001) on the posttest. For the first simulated patient case, 100% ( n = 31) of the nurses selected an appropriate pain intervention option based on the patient-reported pain score. For the second simulated patient case, 84% ( n = 26) did so. Conclusion Increased knowledge does not always translate into simulated practice change. The reasons for this are unknown, but they could include implicit bias from exposure to patients who have high use of acute care, although the minority of patients with SCD fit this description. [ J Contin Educ Nurs . 2022;53(3):137–144.]

BACKGROUND The purpose of this article is to describe the research protocol for a randomized controlled trial of a self-management intervention for adults diagnosed with sickle cell disease (SCD). People living with SCD suffer with lifelong recurrent episodes of acute and chronic pain, both of which are exacerbated by stress. OBJECTIVE The aims of this study are to decrease stress and improve SCD pain control with reduced opioid use through an intervention with self-management relaxation/distraction exercises (RDE), named You Cope, We Support (YCWS). Building on our prior findings of formative studies, this study is designed to test the efficacy of YCWS on stress intensity, pain intensity, and opioid use in adults with SCD. METHODS A randomized controlled trial of the short-term (8 weeks) and long-term (6 months) effects of YCWS on stress, pain, and opioid use will be conducted with 170 adults with SCD. Patients will be randomized based on a 1:1 ratio (stratified on pain intensity [5]) to be either in the experimental (Self-monitoring of outcomes + alerts/reminders + use of YCWS [RDE + Support]) or control (Self-monitoring of outcomes + alerts/reminders) group. Patients will be asked to report outcomes daily. During weeks 1-8, patients in both groups will receive system-generated alerts/reminders via phone call, text, or email to facilitate data entry (both groups) and intervention use support (experimental). If the participant does not enter data after 24 hours, the study support staff will contact him/her for data entry troubleshooting (both groups) and YCWS use (experimental). We will time stamp and track patients' online activities to understand the study context and conduct exit interviews on the acceptability of system-generated and staff support. RESULTS The study was funded by the National Institute of Nursing Research of the National Institutes of Health in 2020. Our Institutional Review Board approved this study. Currently in the recruitment phase, the study began in March 2021 and will be concluded in June 2025. As of September 2021, we have enrolled 40 patients. We will analyze the data using the mixed-effects regression models (short-term, long-term) to account for the repeated measurements over time and utilize machine-learning to construct and evaluate prediction models. Due to the COVID-19 pandemic, the study was modified to allow for a mail-in consent process and a virtual consent process via email or Zoom video conference. CONCLUSIONS We expect that the intervention group will report reductions in pain intensity (primary outcome, 0-10 scale) and in stress intensity (0-10 scale) and opioid use, which are secondary outcomes. Our study will contribute to advancing the use of non-opioid therapy such as guided relaxation/distraction techniques for managing sickle cell pain. CLINICALTRIAL ClinicalTrials.gov NCT04484272

Background Delayed hemolytic transfusion reaction (DHTR) with hyperhemolysis is a potentially fatal complication resulting from alloimmunization that can cause severe hemolysis of both transfused and intrinsic red blood cells (RBCs). Patients with sickle cell disease often receive multiple RBC units during their lifetime and thus are likely to develop alloantibodies that increase the risk for DHTR. Treatment to decrease hemolysis includes intravenous immunoglobulin (IVIG), steroids, eculizumab, rituximab, and plasmapheresis in addition to erythropoietin (EPO), intravenous (IV) iron, vitamin B12, and folate to support erythropoiesis. RBC transfusion is preferably avoided in DHTR due to an increased risk of exacerbating the hemolysis. Case report We report a rare case of anti-N and anti-Doa immunoglobulin (Ig)G alloantibody-mediated life-threatening DHTR with hyperhemolysis in a patient with hemoglobin SS after RBC transfusion for acute chest syndrome who was successfully treated with eculizumab and HBOC-201 (Hemopure) in addition to steroids, IVIG, EPO, IV iron, and vitamin B12. HBOC-201 (Hemopure) was successfully used as a RBC alternative in this patient. Conclusion Anti-N and anti-Doa IgG alloantibodies can rarely cause severe life-threatening DHTR with hyperhemolysis. HBOC-201 (Hemopure) can be a lifesaving alternative in this scenario. Our report also supports the use of eculizumab in DHTR; however, prospective studies are needed to determine the appropriate dose and sequence of eculizumab administration.

Background The experiences of African American adult patients before, during, and after acute care utilization are not well characterized for individuals with sickle cell disease (SCD) or cancer. Objective To describe the experiences of African Americans with SCD or cancer before, during, and after hospitalization for pain control. Methods We conducted a qualitative study among African American participants with SCD (n = 15; 11 male; mean age, 32.7 ± 10.9 years; mean pain intensity, 7.8 ± 2.6) or cancer (n = 15; 7 male; mean age, 53.7 ± 15.2 years; mean pain intensity, 4.9 ± 3.7). Participants completed demographic questions and pain intensity using PAINReportIt and responded to a 7-item open-ended interview, which was recorded and transcribed verbatim. We used content analysis to identify themes in the participants' responses. Results Themes identified included reason for admission, hospital experiences, and discharge expectations. Pain was the primary reason for admission for participants with SCD (n = 15) and for most participants with cancer (n = 10). Participants of both groups indicated that they experienced delayed treatment and a lack of communication. Participants with SCD also reported accusations of drug-seeking behavior, perceived mistreatment, and feeling of not being heard or believed. Participants from both groups verbalized concerns about well-being after discharge and hopeful expectations. Conclusions Race-concordant participants with SCD but not with cancer communicated perceived bias from healthcare providers. Implications for practice Practice change interventions are needed to improve patient-provider interactions, reduce implicit bias, and increase mutual trust, as well as facilitate more effective pain control, especially for those who with SCD.

Little is known about the effects of self-managed relaxation interventions on pain, stress, and autonomic responses in patients with sickle cell disease (SCD). This pre-post randomized controlled pilot study was conducted to determine the feasibility of using computer tablets for relaxation intervention delivery; acceptability of study procedures; and intervention effects on pain, stress, and indicators of relaxation. The 30 research participants ranged in age from 22 years to 59 years. All were African American; 53% were male. They were randomized to an experimental group that watched a relaxation video or a control group that discussed their disease. All participants completed the study, indicating feasibility. Acceptability rates were also high. Data were obtained for the intervention’s immediate effect on pain, stress, respiration, pulse, finger skin temperature, and self-reported relaxation. These preliminary findings will guide future, higher-powered studies to determine the intervention’s efficacy and mechanism in SCD. The ClinicalTrials.gov Identifier: NCT02729363

Human malaria has arguably affected more of human history than any other pathogen. Pregnant women have a higher risk of developing severe malaria as well as the risk of severe complications. We present a case of severe malaria in a pregnant patient from sub-Saharan Africa who was treated successfully with artesunate. A 28-year-old Nigerian woman with a 20-week intrauterine pregnancy presented with a five-day history of fever and diffuse joint pains. Evaluation of peripheral thin blood smear demonstrated a parasitemia of 9.8%. The patient was admitted to the intensive care unit, and oral clindamycin/quinine was initiated until intravenous artesunate was obtained. The patient completed four doses of IV artesunate, and after the 4th dose of artesunate, no blood parasites were seen on peripheral smear. The patient was discharged home and, upon clinic follow-up, did not have any further complications associated with either her disease or therapy. A review on the treatment of severe malaria in all trimesters of pregnancy supports the WHO recommendation for intravenous artesunate as the drug of choice. This case illustrates the importance of recognizing malaria in pregnant women from endemic countries and shows that artesunate compounds can be used safely in pregnancy, particularly with high parasitemia.

Lung transplantation surgery often relies on the use of intraoperative extracorporeal membrane oxygenation (ECMO) and necessitates the need for high dose anticoagulation. Heparin induced thrombocytopenia complicates intraoperative anticoagulation management during lung transplant surgery requiring ECMO. Though other anticoagulants such as argatroban and bivalrudin are utilized for the treatment of Heparin Induced Thrombocytopenia (HIT), the lack of reversal agents makes it difficult to use these agents intraoperatively in cases with high bleeding risk. This is especially true in patients with end stage fibrotic lung disease with calcified mediastinal lymphadenopathy and pulmonary hypertension undergoing lung transplantation. Here we describe a case of HIT in a patient with Sarcoidosis listed for lung transplant who was treated with Therapeutic Plasma Exchange and Intravenous Immune globulin preoperatively and successfully underwent lung transplantation with the use of intraoperative venoarterial ECMO and heparin anticoagulation.

Acquired von Willebrand syndrome is a rare bleeding disorder characterised by a later age of onset without a personal or family history of bleeding diathesis. It is vital to discern acquired von Willebrand syndrome from inherited von Willebrand disease and other acquired bleeding disorders as management differs significantly. Acquired von Willebrand syndrome is usually secondary to an underlying disorder such as lymphoproliferative disorder, myeloproliferative neoplasm, solid tumour, cardiovascular disorder, autoimmune disorders or hypothyroidism. Diagnosis is often delayed with a significant risk of morbidity and even mortality. Here we present a case of a 74-year-old man with an acquired bleeding disorder and work up suggestive of acquired von Willebrand syndrome secondary to immunoglobulin G kappa multiple myeloma. He was treated successfully with intravenous immunoglobulin, von Willebrand Factor/Coagulation Factor VIII Complex (human), myeloma directed chemotherapy and autologous stem cell transplantation. We also discuss the management strategies that are largely based on retrospective studies and case reports.

Delayed hemolytic transfusion reaction (DHTR) with hyperhemolysis is a potentially fatal complication resulting from alloimmunization that can cause severe hemolysis of both transfused and intrinsic red blood cells (RBCs). Patients with sickle cell disease often receive multiple RBC units during their lifetime and thus are likely to develop alloantibodies that increase the risk for DHTR. Treatment to decrease hemolysis includes intravenous immunoglobulin (IVIG), steroids, eculizumab, rituximab, and plasmapheresis in addition to erythropoietin (EPO), intravenous (IV) iron, vitamin B12, and folate to support erythropoiesis. RBC transfusion is preferably avoided in DHTR due to an increased risk of exacerbating the hemolysis.We report a rare case of anti-N and anti-DoAnti-N and anti-Do

A 46-year-old man presented with splenomegaly, abdominal adenopathy and profoundly elevated prothrombin time and partial thromboplastin time. He was diagnosed with marginal zone lymphoma (MZL) and small lymphocytic lymphoma, and the abnormal coagulation studies were secondary to the presence of a lupus anticoagulant. Optimal upfront therapy for MZL has not been established, and the incidence of antiphospholipid antibodies (APLA) in this patient population is rare. Following treatment with six cycles of bendamustine and rituximab with 2 years of rituximab maintenance, our patient remained in remission and his coagulation studies normalised. This report describes a case of successful treatment of APLA associated with MZL that resolved after treatment of the lymphoma.

To evaluate the pain coping strategies of patients with sickle cell disease (SCD) who experience healthcare injustice from either physicians or nurses during medical visits for pain management. It is unknown how patients' coping with pain relates to their experiences of healthcare injustice from physicians or nurses. This descriptive comparative study included adult outpatients with SCD who completed the PAINReportIt®, Healthcare Justice Questionnaire©, and Coping Strategies Questionnaire-SCD. Data were analyzed using independent t tests. Frequent coping strategies of patients who experienced healthcare justice from physicians were praying-hoping and from nurses were praying-hoping, calming self-statements, diverting attention, and increasing behavioral activity. In contrast, frequent coping strategies of patients who experienced healthcare injustice from physicians were catastrophizing and isolation and from nurses were isolation. Patients who experienced healthcare justice used different sets of pain coping strategies than those who experienced healthcare injustice during medical visits for pain management.

Venous thromboembolism (VTE) results in significant morbidity and mortality. The prevention and treatment of VTE is managed with anticoagulant therapy, historically parenteral anticoagulants such as unfractionated heparin, low molecular weight heparin, and fondaparinux, and oral vitamin K antagonists such as warfarin. In the last few years, several target-specific oral anticoagulants have been developed, including the direct thrombin inhibitor dabigatran and anti-Xa inhibitors rivaroxaban, apixaban, and edoxaban. The target-specific oral anticoagulants have proven to be noninferior to vitamin K antagonists and heparins in the prevention and treatment of VTE. This review will focus on the pharmacology, clinical trial data, and laboratory assessment of apixaban. Moreover, perioperative management, use in special populations, and management of bleeding complications in patients taking apixaban for the prevention and treatment of VTE will also be discussed.

Abstract 4248 Background: In our tertiary academic medical center, standardized pre-printed physician order sets were implemented in 2008 for inpatient venous thromboembolism (VTE) risk stratification and prophylaxis on the day of admission. Electronic medical records were not utilized during the study period. Recommendations for pharmacologic prophylaxis were derived from the 2008 American College of Chest Physicians (ACCP) guidelines. Patients undergoing abdominal or pelvic surgery for cancer were specifically identified in the highest risk category with enoxaparin, fondaparinux, or warfarin (goal INR 2–3) provided as options for pharmacologic VTE prophylaxis. Sequential compression devices (SCDs) were also recommended. This retrospective analysis evaluates adherence to risk stratification and recommendations for pharmacologic VTE prophylaxis in patients undergoing abdominal or pelvic surgery for cancer. Methods: Using the hospital administrative database, all patients ≥ 18 years hospitalized between January 1, 2009 and December 31, 2010 were identified by ICD-9 codes for cancer and surgery performed. Patients undergoing an abdominal or pelvic surgery for cancer lasting >45 minutes were included. Exclusion criteria were age >90 years, patients requiring therapeutic anticoagulation, length of hospitalization 30 days, transplant recipients, patients requiring multiple visits to the operating room during hospitalization, laparoscopic or transurethral procedures, and esophagectomy. Primary outcomes included adherence to order set risk stratification and pharmacologic VTE prophylaxis recommendations, and timing, type, and dose of pharmacologic VTE prophylaxis prescribed. Chart review of physician orders for mechanical and pharmacologic prophylaxis for each day of hospitalization was completed on all patients. Operative reports, medication administration records, and progress notes were reviewed if clarification of physician orders was required. Administration of the prescribed medication during hospitalization was not verified. Results: A total of 773 hospital admissions representing 767 patients met inclusion criteria. The mean length of hospitalization was 7 +/− 5 days during the study period. The mean age was 62 +/− 12 years and 51% were female. Pharmacologic prophylaxis was prescribed 5645 of 6147 patient days (91.8%). Enoxaparin was prescribed 630 (9.8%) patient days, and fondaparinux in 15 patient days (0.002%). Although not indicated as an option for this patient population in our order set, low dose unfractionated heparin (LDUH) was prescribed 4991 (81.2%) of the patient days and was to be dosed every 8 hours (80%) or every 12 hours (19.8%). Warfarin was not prescribed. Mechanical thromboprophylaxis with SCDs was ordered 93.7% of the patient encounters on the day of surgery. The standardized pre-printed physician order set was utilized for prescribing prophylaxis at the time of admission for 630 (82.1%) patient encounters. Clinicians risk stratified only 350 (45.3%) of these patient encounters per the order set guidelines. Of those, 131 (37.4%) were appropriately deemed high risk based upon “abdominal/pelvic cancer undergoing operative procedure”. In the high risk group, LDUH was prescribed in 90 (68.7%) patient encounters, and enoxaparin as recommended in 30 (22.9%). Eleven (8.4%) were not prescribed pharmacologic prophylaxis on the day of admission. Conclusion: Completion of standardized pre-printed physician order sets with appropriate risk stratification in patients undergoing major, open abdominal or pelvic surgery for cancer is underutilized in our institution. Although pharmacologic prophylaxis is prescribed for the majority of inpatient days, LDUH is primarily ordered despite preferred recommendations in favor of enoxaparin or fondaparinux. Thus, an opportunity exists to implement an educational intervention to improve adherence to evidence based order set risk stratification and pharmacologic VTE prophylaxis recommendations. Disclosures: No relevant conflicts of interest to declare.