Understanding caregiver burden with accessing sickle cell care in the Midwest and their perspective on telemedicine.

BACKGROUND: Survival for children with sickle cell disease (SCD) has improved significantly. However, patients with SCD still encounter several impediments to accessing adequate healthcare. Rural and medically underserved areas, such as parts of the Midwest, can exacerbate these barriers, separating children with SCD from subspecialists even further. Telemedicine has been a means to close these gaps in care for children with other special healthcare needs, but few studies have discussed how caregivers of children with SCD perceive its use. METHODS: The objective of this study is to understand the experiences of caregivers of pediatric SCD patients in a geographically diverse area in the Midwest in accessing care, and their perspectives of telemedicine. Caregivers of children with SCD completed an 88-item survey via a secured REDCap link either in-person or via secure text. Descriptive statistics (means, medians, ranges, frequencies) were performed for all responses. Univariate chi square tests were performed to analyze associations, particularly with telemedicine responses. RESULTS: The survey was completed by 101 caregivers. Nearly 20% of families traveled more than 1 hour to reach the comprehensive SCD center. Other than their SCD provider, caregivers reported their child having at least 2 other healthcare providers. Most barriers caregivers identified were financial or resource based. Almost a quarter of caregivers expressed feeling as though these barriers impacted their and/or their child's mental health. Ease of access to team members, as well as scheduling, were common facilitators of care cited by caregivers. The majority were willing to participate in telemedicine visits, regardless of how far they lived from the SCD center, though many noted aspects requiring adaptation. CONCLUSION: This cross-sectional study describes barriers to care experienced by caregivers of children with SCD, regardless of proximity to an SCD center, as well as caregiver perceptions of the usefulness and acceptability of telemedicine for SCD care.

Increasing optimal coagulation factor dosing in the paediatric emergency department: A quality improvement study.

INTRODUCTION: Haemophilia is a disorder complicated by bleeding episodes that require emergent medical evaluation. Factor replacement dosing can present challenges for emergency department (ED) care. AIMS: We aimed to reduce out-of-range factor dosing in the ED. Specifically, we sought to increase the number of haemophilia ED patient visits between encounters where sub-optimal factor dosing was administered from a baseline of 4-15 encounters. METHODS: A chart review was completed on all patients with haemophilia A (HA) or B (HB) seen in the ED for injuries requiring factor concentrate from September 2015 to August 2016. Injuries were classified as minor-requiring a 50% factor correction or major-requiring a 100% factor correction. Optimal dosing range was defined as 90%-120% of the institutional guideline goal for the degree of injury. The predicted optimal dose range for each patient was compared to the actual dose administered. RESULTS: Baseline data demonstrated optimal dosing range in 70% of encounters. There was no difference between patients with HA or HB in frequency of out-of-range dosing (P = 0.15). There was no difference in frequency of out-of-range dosing between types of clotting factor concentrate used. After initiation of quality improvement (QI) interventions, we achieved 16 encounters between out-of-range dosing, exceeding our goal of 15. However, this success was not sustained. CONCLUSION: Optimal coagulation factor dosing is important for patient care and resource management. QI interventions promoted increased accuracy of factor dosing for patients with haemophilia seen in the ED.

Optimizing patient flow in a multidisciplinary haemophilia clinic using quality improvement methodology.

INTRODUCTION: Multidisciplinary clinics in academic settings are often inefficient and can lead to lengthy clinic visits for patients and staff. AIM: We aimed to use quality improvement (QI) methodology and a multidisciplinary approach to optimize outpatient comprehensive haemophilia clinic flow. METHODS: At baseline, a multidisciplinary QI team created a key driver diagram to identify drivers of haemophilia clinic flow. Identified drivers included patient needs/scheduling, provider flow and laboratory/research requirements. From December 2016 to August 2017, value stream mapping (VSM) was used to identify barriers to clinic flow, and plan-do-study-act cycles were used to address these barriers. Interventions included (a) standardizing the order in which providers saw patients to enable time-sensitive laboratories, (b) improving HTC team meeting functionality, (c) optimizing a visual management board and implementing a flow coordinator, (d) initiating a team huddle prior to clinic start and (e) modifying the clinic appointment template. Timely laboratory draw was used as a surrogate marker of clinic flow, and VSM utilization percentage was used as an objective measure of efficiency. RESULTS: We did not demonstrate a statistically significant improvement in timed laboratory draws; however, clinic utilization percentage increased by 30%, which resulted in adding point-of-care musculoskeletal ultrasound services without lengthening clinic duration. CONCLUSION: Quality improvement methodology is an effective means of improving clinic utilization in a multidisciplinary clinic.

Inpatient Health Care Utilization in Children With Hemophilia Before and After the Joint Outcome Study Publication.

The "Joint Outcomes Study" (JOS) demonstrated improved joint outcomes for patients receiving primary prophylaxis versus on-demand therapy. The impact of primary prophylaxis on inpatient health care utilization is not well-defined. To evaluate changes in hospitalization care of children with hemophilia before and after the 2007 JOS publication, this study utilized the Pediatric Health Information System (PHIS) to evaluate admissions for patients with hemophilia A or B (age, 2 to 7) admitted between January 2002 and 2006 (pre-JOS) and January 2010 and 2014 (post-JOS). Discharge diagnosis codes and clinical transaction classifications were used to differentiate bleeding episodes, infections, bypass agent use, length of stay, and intensive care unit (ICU) management. Overall, admissions for bleeding episodes did not change (26.5% of admissions pre-JOS vs. 23.6% post-JOS, P=0.10). However, admissions for suspected infections increased (3.0% of admissions pre-JOS vs. 7.2% post-JOS, P<0.01) while confirmed infections remained stable. Meanwhile, ICU utilization decreased (7.8% of admissions pre-JOS vs. 4.9% post-JOS, P<0.01). The necessity for ICU care in children with hemophilia has decreased since publication of the JOS. However, expanded adoption of primary prophylaxis is associated with more hospitalizations for suspected systemic infections, likely due to utilization of central venous catheters to deliver clotting factor concentrates.

Experimenting with Governance: Alberta's Strategic Clinical Networks.

Alberta is undertaking a bold and somewhat risky step overhauling its health system governance to build higher performance in quality, safety and improved health outcomes for Albertans. On the heels of having re-established a single province-wide health authority (Alberta Health Services [AHS]), provincial health system decision makers have moved to establish province-wide Strategic Clinical Networks™ (SCNs). Sixteen SCNs have been implemented, and all are constituted as teams of healthcare professionals, researchers, government stakeholders, patients and families seeking to improve delivery of healthcare across the province. SCNs were developed in part as a strategy for strengthening clinical engagement to achieve a broad range of healthcare delivery benefits including improvement of clinical care processes and reduced variations in practice, better access to care and improved patient outcomes across the province. Here, we examine the rationale and potential of this governance intervention, while also considering some of the fundamental questions around their potential impact and the ultimate need for multidimensional assessment.

Local circulation of elites punctuated by transregional mobility enabled steppe political consolidation in the Xiongnu nomadic state.

The Xiongnu polity (ca. 200 BC- 150 AD) emerged out of indigenous community-centered socio-political structures to forge a powerful state that commanded the Mongolian steppe and beyond. Underpinned by a highly mobile pastoralist population, accustomed to seasonally rhythmic moves and embedded in an equestrian culture that facilitated rapid transport over long-distances, it remains unclear precisely how the movement of commoners, local aristocrats and regional elites abetted the formation and organization of Xiongnu state structures. Here, we evaluate Xiongnu movement and dietary intake through multi-stable isotopic analyses of tooth enamel from directly dated Xiongnu intermediate elites recovered from the mortuary center of Baga Gazaryn Chuluu-a prominent granite outcrop set in the Gobi Desert. Carbon isotope (δ13C) analysis indicates millet was consumed by some individuals, but whether or not this C4 cultivar contributed to the diets of most elites remains ambiguous in this C3/C4 desert-steppe environment. The effectiveness of oxygen isotopes (δ18O) to establish mobility appears much reduced in steppe environments, where geospatially sensitive information appears disrupted by extraordinary seasonality in meteoric water oxygen isotopes, pronounced oxygen isotopic variation in potential drinking water sources, and culturally mediated drinking practices. Most revealing, strontium isotopes (87Sr/86Sr) indicate circulation of local elites around this central place and beyond, a mobility format that helped leaders cement their own position through political consolidation of spatially dispersed mobile pastoralist communities. The consistent presence at Baga Gazaryn Chuluu of extra-local intermediate elites also points toward the importance of transregional mobility in binding together the Xiongnu polity over the vast distances of the eastern steppe.

Implementation of an Anterior Mediastinal Mass Pathway to Improve Time to Biopsy and Multidisciplinary Communication.

Background: Mediastinal masses in children with cancer present unique challenges, including the risk of respiratory and hemodynamic compromise due to the complex anatomy of the mediastinum. Multidisciplinary communication is often a challenge in the management of these patients. After a series of patients with mediastinal masses were admitted to Riley Hospital for Children Pediatric Intensive Care Unit, the time from presentation to biopsy and pathology was greater than expected. We aimed to reduce the time to biopsy by 25% and demonstrate improved multidisciplinary communication within 6 months of protocol implementation for patients presenting to Riley Hospital for Children Emergency Department with an anterior mediastinal mass. Methods: Quality improvement methodology created a pathway that included early multidisciplinary communication. The pathway includes communication between the emergency department and multiple surgical and medical teams via a HIPPA-compliant texting platform. Based on patient stability, imaging findings, and sedation risks, the approach and timing of the biopsy were determined. Results: The pathway has been used 20 times to date. We successfully reduced the time to biopsy by 38%, from 25.1 hours to 15.4 hours. There was no statistically significant reduction in time to pathology. The multidisciplinary team reported improved communication from a baseline Likert score of 3.24 to 4. Conclusions: By initiating early multidisciplinary communication, we reduced the time to biopsy and pathology results, improving care for our patients presenting with anterior mediastinal masses.

Right on Schedule: Improving the Rate of Clinic Appointments Scheduled Prior to Hospital Hospital Discharge.

INTRODUCTION: Children with cancer and blood disorders have many healthcare needs that often require inpatient and outpatient management. There is potential for a lapse in care when patients frequently transition between these settings. We aimed to improve the process and increase the rate of scheduled outpatient follow-up appointments at the time of inpatient discharge for all pediatric hematology-oncology patients from a baseline of 68-80%. METHODS: A multidisciplinary team developed several Plan-Do-Study-Act cycles to standardize and improve the process of scheduling follow-up appointments, communication to schedulers, and discussion of discharge planning. QI Macros for Excel Version 2019.06 was used for statistical analysis. Our primary outcome was displayed over time with a p-chart. RESULTS: Plan-Do-Study-Act interventions had a statistically significant impact in increasing the percentage of patients with follow-up outpatient appointments scheduled at the time of inpatient discharge from a baseline of 68% to consistently over 80%. CONCLUSIONS: This study demonstrates that standardization of care processes and reminders and education of healthcare providers about the new approaches can improve the rates of outpatient follow-up appointments scheduled at the time of hospital discharge from inpatient care.

Perfect storm along the neuro-cardiac axis: stroke complicated by acute biventricular dysfunction-Takotsubo syndrome or neurogenic stress cardiomyopathy?

Acute biventricular dysfunction complicating acute ischaemic stroke poses diagnostic and therapeutic challenges with respect to anticoagulation and short-term and long-term prognosis. We present the uncommon case of an elderly patient presenting with this clinical scenario, who made a full recovery on conservative therapy. Management strategies are discussed and the emerging topic of neuro-cardiac syndromes, namely neurogenic stress cardiomyopathy as distinct from Takotsubo syndrome, is explored.

Methadone-based Multimodal Analgesia Provides the Best-in-class Acute Surgical Pain Control and Functional Outcomes With Lower Opioid Use Following Major Posterior Fusion Surgery in Adolescents With Idiopathic Scoliosis.

INTRODUCTION: Posterior spinal fusion for idiopathic scoliosis is extremely painful, with no superior single analgesic modality. We introduced a methadone-based multimodal analgesia protocol, aiming to decrease the length of hospital stay (LOS), improve pain control, and decrease the need for additional opioids. METHODS: We analyzed 122 idiopathic scoliosis patients with posterior instrumented spinal fusion. They were matched by age, sex, surgeon, and the number of levels fused before and after the implementation of the new protocol. This analysis included 61 controls (intrathecal morphine, gabapentin, intravenous opioids, and adjuncts) and 61 patients on the new protocol (scheduled methadone, methocarbamol, ketorolac/ibuprofen, acetaminophen, and oxycodone with intravenous opioids as needed). The primary outcome was LOS. Secondary outcomes included pain scores, total opioid use (morphine milligram equivalents), time to a first bowel movement, and postdischarge phone calls. RESULTS: New protocol patients were discharged earlier (median LOS, 2 days) compared with control patients (3 days; P < 0.001). Total inpatient morphine consumption was lower in the protocol group (P < 0.001). Pain scores were higher in the protocol group on the day of surgery, similar on postoperative day (POD) 1, and lower by POD 2 (P = 0.01). The new protocol also reduced the median time to first bowel movement (P < 0.001), and the number of postdischarge pain-related phone calls (P < 0.006). CONCLUSION: Methadone-based multimodal analgesia resulted in significantly lower LOS compared with the conventional regimen. It also provided improved pain control, reduced total opioid consumption, and early bowel movement compared with the control group.

Functions of the Triceps Brachii in Humans: A Review.

The triceps brachii (TB) is the only muscle that lies along the posterior humerus, and its role in elbow extension has been the topic of investigation for many decades. The TB research has also included subjects such as strength training, effects of aging, training adaptions, etc. The purpose of this paper was to review some of the more unique investigations regarding the functions of the human TB. Specifically, this paper discusses articles that have explored the actions of the TB when manipulating the angles of the joints it crosses, the elbow and shoulder.

Actions of the Biceps Brachii at the Shoulder: A Review.

Bi-articular muscles cross more than one joint and contribute to motion at both joints, and the extremities of the human body contain several such muscles. Actions produced by all muscles are determined, to a large extent, by joint moment arms and muscle length. These are transient factors which change as joint angles are altered. Measuring muscle moments while manipulating both joints will produce a better understanding of the actions of bi-articular muscles. This review summarizes investigations which have explored the actions of the biceps brachii as shoulder and elbow joints are moved into various angle combinations. Clinical implications of the findings are discussed.

Y-family polymerase conformation is a major determinant of fidelity and translesion specificity.

Y-family polymerases help cells tolerate DNA damage by performing translesion synthesis opposite damaged DNA bases, yet they also have a high intrinsic error rate. We constructed chimeras of two closely related Y-family polymerases that display distinctly different activity profiles and found that the polypeptide linker that tethers the catalytic polymerase domain to the C-terminal DNA-binding domain is a major determinant of overall polymerase activity, nucleotide incorporation fidelity, and abasic site-bypass ability. Exchanging just 3 out of the 15 linker residues is sufficient to interconvert the polymerase activities tested. Crystal structures of four chimeras show that the conformation of the protein correlates with the identity of the interdomain linker sequence. Thus, residues that are more than 15 Å away from the active site are able to influence many aspects of polymerase activity by altering the relative orientations of the catalytic and DNA-binding domains.