Development and User Testing of a Dynamic Tool for Rheumatic Heart Disease Management.

OBJECTIVE: Most rheumatic heart disease (RHD) registries are static and centralized, collecting epidemiological and clinical data without providing tools to improve care. We developed a dynamic cloud-based RHD case management application with the goal of improving care for patients with RHD in Uganda. METHODS: The Active Community Case Management Tool (ACT) was designed to improve community-based case management for chronic disease, with RHD as the first test case. Global and local partner consultation informed selection of critical data fields and prioritization of application functionality. Multiple stages of review and revision culminated in user testing of the application at the Uganda Heart Institute. RESULTS: Global and local partners provided feedback of the application via survey and interview. The application was well received, and top considerations included avenues to import existing patient data, considering a minimum data entry form, and performing a situation assessment to tailor ACT to the health system setup for each new country. Test users completed a postuse survey. Responses were favorable regarding ease of use, desire to use the application in regular practice, and ability of the application to improve RHD care in Uganda. Concerns included appropriate technical skills and supports and potential disruption of workflow. CONCLUSION: Creating the ACT application was a dynamic process, incorporating iterative feedback from local and global partners. Results of the user testing will help refine and optimize the application. The ACT application showed potential for utility and integration into existing care models in Uganda.

Research Agenda for Implementation of Principles of Care for Children and Youth With Special Health Care Needs.

OBJECTIVE: Children and youth with special health care needs (CYSHCN) have a range of medical, educational, and support service needs to achieve optimal health and wellness. Principles of care for CYSHCN have been well described, but the literature is lacking particularly on implementation and integration of care across different settings and systems. The objective of this manuscript is to define a research agenda for principles of care for CYSHCN. METHODS: Literature review examined principles of care for CYSHCN. Existing research gaps and priorities for principles of care were drawn from the literature review, a recently developed national research agenda for CYSHCN, and stakeholder consensus. RESULTS: Specific implementation areas of inquiry include family partner roles within and across systems; life course approach for CYSHCN; roles and training of interdisciplinary team members; and implementation, spread, and sustainability studies. Proposed methods include implementation science-based and comparative effectiveness research. A common set of metrics including health care utilization, clinical outcomes, and family and provider needs should be considered to evaluate implementation of principles of care. CONCLUSIONS: Implementation science and comparative effectiveness methods are needed to further understanding about how to adopt and spread principles of care for CYSHCN. The evolving demographics of CYSHCN add relevance and urgency for research findings.

Tertiary Pediatric Academic Institution's Experience With Intraoperative Neuromonitoring for Nonspinal Surgery in Children With Mucopolysaccharidosis, Based on a Novel Evidence-Based Care Algorithm.

BACKGROUND: Musculoskeletal deformities in mucopolysaccharidoses (MPSs) patients pose unique challenges when patients present for surgery, especially nonspinal surgery. MPS patients have developed postsurgical neurological deficits after nonspinal surgery. While the incidence of neurological deficits after nonspinal surgery under anesthesia is unknown, accumulating evidence provides impetus to change current practice and increased neurological monitoring in these patients. Intraoperative neurophysiologic monitoring (IONM) with somatosensory evoked potentials (SSEPs) and transcranial motor evoked potentials (TcMEPs) has been implemented at select institutions with varying degree of success. This report describes our experience with IONM in the context of a multidisciplinary evidence-based care algorithm we developed at Cincinnati Children's Hospital Medical Center. METHODS: We conducted a retrospective chart review of the electronic medical record (EPIC), for data from all MPS patients at our institution undergoing nonspinal surgery between September 2016 and March 2018. Patients were identified from IONM logs, which include procedure and patient comorbidities. Data concerning demographics, morbidities, degree of kyphoscoliosis, intraoperative administered medications and vital signs, surgical procedure, the IONM data, duration of surgery, and blood loss were extracted. Descriptive analyses were generated for all variables in the data collected. In addition, any IONM changes noted during the surgeries were identified and factors contributing to the changes described. RESULTS: Thirty-eight patients with a diagnosis of MPS underwent nonspinal surgery, and of those 38, 21 received IONM based on preoperative decision-making according to our care algorithm. Of the 21 patients who received IONM, we were able to get reliable baseline potentials on all patients. Of the 21 patients, 3 had significant neurophysiologic changes necessitating surgical/anesthetic intervention. All of these changes lasted several minutes, and the real-time IONM monitoring was able to capture them as they arose. None of the patients sustained residual neurological deficits. Thus, children who did not fit the criteria for IONM (n = 13) based on our algorithm had 0% incidence of any untoward neurological deficits after surgery (97.5% confidence interval [CI], 00%-25.5%), while 14% (95% CI, 11.5%-30.1%) of children who did fit criteria for IONM and had IONM had significant IONM changes. CONCLUSIONS: Through this case series, we describe our experience with the use of IONM and a novel care algorithm for guiding the anesthetic management of MPS patients undergoing nonspinal surgery. We conclude that they can be useful tools for provision of safe anesthetic care in this high-risk cohort.