Impact of Facility Dog and Certified Child Life Specialist Dyad on Children's Pain and Anxiety During Needlestick Procedures in a Pediatric Hematology Oncology Clinic Setting.

BACKGROUND: Pediatric Hematology Oncology patients undergo frequent needlestick procedures, often leading to negative outcomes including pain and anxiety. Animal-assisted therapy has been shown to minimize pediatric patient distress; however, its utilization by a Certified Child Life Specialist (CCLS) to reduce patient distress has not been widely studied. METHODS: Pediatric patients receiving needlesticks in the Hematology Oncology Clinic were enrolled between March 2018 and May 2021. Patients who had scheduled visits when the facility dog was present were assigned to the intervention group. Patients were assigned to the control group if the facility dog was not present. The primary objective was to use the Children's Anxiety and Pain Scale to determine whether the CCLS and facility dog dyad minimized patient pain and anxiety during procedures. RESULTS: A total of 285 patients, 5 to 17 years of age, were enrolled. One hundred forty-three patients were assigned the intervention and received procedural support from the CCLS and facility dog; 142 patients were assigned the control group and received support from the CCLS only. Patient-reported pain scores were significantly lower among patients who received the intervention ( P =0.033). CONCLUSIONS: Utilization of a CCLS and facility dog dyad during painful needlestick procedures decreases patient-reported pain compared with utilization of CCLS support alone.

Recruitment of MLL1 complex is essential for SETBP1 to induce myeloid transformation.

Abnormal activation of SETBP1 due to overexpression or missense mutations occurs frequently in various myeloid neoplasms and associates with poor prognosis. Direct activation of Hoxa9/Hoxa10/Myb transcription by SETBP1 and its missense mutants is essential for their transforming capability; however, the underlying epigenetic mechanisms remain elusive. We found that both SETBP1 and its missense mutant SETBP1(D/N) directly interact with histone methyltransferase MLL1. Using a combination of ChIP-seq and RNA-seq analysis in primary hematopoietic stem and progenitor cells, we uncovered extensive overlap in their genomic occupancy and their cooperation in activating many oncogenic transcription factor genes including Hoxa9/Hoxa10/Myb and a large group of ribosomal protein genes. Genetic ablation of Mll1 as well as treatment with an inhibitor of the MLL1 complex OICR-9429 abrogated Setbp1/Setbp1(D/N)-induced transcriptional activation and transformation. Thus, the MLL1 complex plays a critical role in Setbp1-induced transcriptional activation and transformation and represents a promising target for treating myeloid neoplasms with SETBP1 activation.

Clinical predictors of poor outcomes in patients with sickle cell disease and COVID-19 infection.

We aimed to identify predictors of outcomes and survival in patients living in 4 major metropolitan areas who had sickle cell disease (SCD) and COVID-19 to inform best approaches to prevention and care. Data were collected at baseline and during the clinical course in SCD patients diagnosed with COVID-19 in four COVID-19 epicenters. Patients were followed up posthospital discharge for up to 3 months. Of sixty-six SCD patients with COVID-19, fifty patients (75%) required hospitalization, and seven died (10.6%). Patients with preexisting kidney disease (chronic kidney disease) were more likely to be hospitalized. The most common presenting symptom was vaso-occlusive pain. Acute chest syndrome occurred in 30 (60%) of the 50 hospitalized patients and in all who died. Older age and histories of pulmonary hypertension, congestive heart failure, chronic kidney disease, and stroke were more prevalent in patients who died, as were higher creatinine, lactate dehydrogenase, and D-dimer levels. Anticoagulation use while inpatient was twice less common in patients who died. All deaths occurred in individuals not taking hydroxyurea or any other SCD-modifying therapy. Patients with SCD and COVID-19 exhibited a broad range of disease severity. We cannot definitively state that the overall mortality is higher in patients with SCD, although our case fatality rate was ∼10% compared with ∼3% in the general population, despite a median age of 34 years. Individuals with SCD aged >50 years, with preexisting cardiopulmonary, renal disease, and/or stroke not receiving hydroxyurea, who present with high serum creatinine, lactate dehydrogenase, and D-dimer levels, are at higher risk of death, irrespective of genotype or sex.

Evaluation of a Health Care Transition Improvement Process in Seven Large Health Care Systems.

PURPOSE: Youth and young adults require systematic planning, transfer and integration into adult healthcare. A national health care transition (HCT) learning network (LN) shared strategies during monthly calls to improve HCTs using Got Transition™'s Six Core Elements. Among LN participants, we conducted a pre-post mixed-methods evaluation of this evidence-informed process improvement framework. DESIGN AND METHODS: Leaders from seven health systems in the LN recruited 55 participating practice sites (12 primary care, 43 specialty care, 47 pediatric care, and 8 adult care). Got Transition's Current Assessment (CA) of HCT Activities (possible score: 0-32) assessed implementation of HCT process improvements in all 55 sites at baseline (2015-2017) and again after 12-18 months. Pre-post results were compared overall and by type of practice (primary vs. specialty, pediatric vs. adult). In early 2018, health system leaders qualitatively described factors impacting HCT process implementation. RESULTS: Overall, baseline CA scores averaged 10.7, and increased to 17.9 after 12-18 months. Within each clinical setting, scores increased from: 10.8 to 16.5 among 12 primary care sites, 12.8 to 17.1 among 43 specialty sites, 12.4 to 17 among 47 pediatric sites, and 12 to 16.9 among 8 adult sites. All changes reached significance (p < 0.05). Qualitative feedback offered valuable feedback about motivators, facilitators and barriers to HCT process improvement. CONCLUSIONS: Participating systems made substantial progress in implementing a structured HCT process consistent with clinical recommendations using the Six Core Elements. PRACTICE IMPLICATIONS: The diverse perspectives of participating health systems provide a model for creating sustainable HCT process improvements.

Defining Medical Professionalism Across the Years of Training and Experience at the Uniformed Services University of the Health Sciences.

BACKGROUND: Many medical institutions have moved forward with curricular objectives aimed at teaching professionalism, but the question remains: are we teaching the most appropriate content at the most opportune times to maximize sustained learning? The students' point of view of professionalism is helpful in addressing this question. AIM: To describe the views of professionalism held by students and faculty at the Uniformed Services University of the Health Sciences. METHODS: In e-mailed surveys, students and faculty free-texted the three most important characteristics of a professional. Qualitative analysis was used to analyze the results. Data were compared on the basis of the percentage of each group affirming one of the characteristics. RESULTS: Fourteen characteristics of professionalism were found. There were significant differences across all participant groups in the characteristics that each indicated were most important. CONCLUSION: Differences emerge between definitions of professionalism that appear to relate to training and experience. Students' views of professionalism reflect the immediate context of their educational environment. Curricula targeted to the students' foci are relevant in teaching professionalism.

Reduction of Factor VIII Inhibitor Titers During Immune Tolerance Induction With Recombinant Factor VIII-Fc Fusion Protein.

The development of inhibitors toward factor VIII (FVIII) is a common and serious complication of hemophilia A (HA) therapy. Patients with hemophilia who develop inhibitors often undergo time- and resource-intensive immune tolerance induction (ITI) protocols. We report a 15-month-old male with severe HA and a high-titer inhibitor that occurred while receiving prophylactic treatment with recombinant FVIII (rFVIII), in whom significant inhibitor titer reduction was achieved with thrice weekly infusions of a new, prolonged half-life rFVIII-Fc fusion protein product (trade name Eloctate). Further studies are warranted to explore the potential of Eloctate in ITI protocols.

Venous thrombotic events in hospitalized children and adolescents with inflammatory bowel disease.

BACKGROUND: Adults with inflammatory bowel disease (IBD) have an increased risk of venous thrombotic events (TEs). We sought to evaluate the risk for TE in children and adolescents with IBD using a large population database. METHODS: The triennial Healthcare Cost and Utilization Project Kids' Inpatient Database was used in a retrospective cohort study of hospitalized children in the United States across 1997, 2000, 2003, 2006, and 2009. Billing codes were used to identify discharges with Crohn disease, ulcerative colitis, pulmonary embolism, deep vein thrombosis, thrombophlebitis, thrombosis of intracranial venous sinus, Budd-Chiari syndrome, and portal vein thrombosis. A logistic regression model was fitted to quantify the increased risk of TE in children with IBD, while adjusting for other risk factors of thrombosis. RESULTS: The total weighted number of pediatric discharges was 7,448,292, and 68,394 (0.92%) were identified with IBD. The incidence of any TE in a hospitalized child or adolescent with IBD was 117.9/10,000 with a relative risk (95% confidence interval) of 2.36 (2.15-2.58). The adjusted odds ratio for any TE in a patient with IBD without surgery was 1.22 (1.08-1.36). Risk factors for TE among patients with IBD include older age, central venous catheter, parenteral nutrition, and an identified hypercoagulable condition. There is an increasing trend of TE in both the IBD and non-IBD patients. CONCLUSIONS: Hospitalized children and adolescents with IBD are at increased risk for TE. Conservative methods of TE prevention including hydration, mobilization, or pneumatic devices should be considered in hospitalized patients with IBD.