Background: Continuous glucose monitoring (CGM) systems are widely and increasingly used in diabetes self-management and in the context of clinic visits. However, access to CGM data during visits can be challenging. Clinic inefficiencies can restrict the time available for patient education, and the inability to integrate CGM data into electronic health record (EHR) systems can result in data being lost. In this study, we describe our institution's approach to integrating CGM data directly into the EHR through a partnership with a CGM device manufacturer and without a third-party data aggregation/data visualization platform. Methods: We interviewed key stakeholders with the hospital Information Technology Department, the Division of Pediatric Endocrinology, and a CGM device manufacturer. A collaborative, human-centered design approach was used to define the workflow. Health Level 7 (HL7) standards were used to build all data exchanges. Results: In collaboration with all parties, we created a simple network architecture design for both account linkage and data acquisition. The system uses the standard, computerized, physician order entry interface available in the EHR for both processes. Data acquisition occurs in real time, and customized reports are displayed within the results section of the EHR. The entire process is Health Insurance Portability and Accountability Act (HIPAA) compliant and meets all security requirements. Conclusions: Building scalable data integration using HL7 standards is possible and allows real-time access to CGM data within the diabetes provider's existing workflow and can occur with or without the patient present. This may lead to improved clinical outcomes, increased efficiency, and new revenue opportunities by documenting CGM data capture and review.

Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by polymerization of hemoglobin S upon deoxygenation that results in the formation of rigid sickled-shaped red blood cells that can occlude the microvasculature, which leads to sudden onsets of pain. The severity of vaso-occlusive crises (VOC) is quite variable among patients, which is not fully explained by their genetic and biological profiles. The mechanism that initiates the transition from steady state to VOC remains unknown, as is the role of clinically reported triggers such as stress, cold and pain. The rate of hemoglobin S polymerization after deoxygenation is an important determinant of vaso-occlusion. Similarly, the microvascular blood flow rate plays a critical role as fast-moving red blood cells are better able to escape the microvasculature before polymerization of deoxy-hemoglobin S causes the red cells to become rigid and lodge in small vessels. The role of the autonomic nervous system (ANS) activity in VOC initiation and propagation has been underestimated considering that the ANS is the major regulator of microvascular blood flow and that most triggers of VOC can alter the autonomic balance. Here, we will briefly review the evidence supporting the presence of ANS dysfunction in SCD, its implications in the onset of VOC, and how differences in autonomic vasoreactivity might potentially contribute to variability in VOC severity.

Persons with sickle cell disease (SCD) exhibit subjective hypersensitivity to cold and heat perception in experimental settings, and triggers such as cold exposure are known to precipitate vaso-occlusive crises by still unclear mechanisms. Decreased microvascular blood flow (MBF) increases the likelihood of vaso-occlusion by increasing entrapment of sickled red blood cells in the microvasculature. Because those with SCD have dysautonomia, we anticipated that thermal exposure would induce autonomic hypersensitivity of their microvasculature with an increased propensity toward vasoconstriction. We exposed 17 patients with SCD and 16 control participants to a sequence of predetermined threshold temperatures for cold and heat detection and cold and heat pain via a thermode placed on the right hand. MBF was measured on the contralateral hand by photoplethysmography, and cardiac autonomic balance was assessed by determining heart rate variability. Thermal stimuli at both detection and pain thresholds caused a significant decrease in MBF in the contralateral hand within seconds of stimulus application, with patients with SCD showing significantly stronger vasoconstriction (P = .019). Furthermore, patients with SCD showed a greater progressive decrease in blood flow than did the controls, with poor recovery between episodes of thermal stimulation (P = .042). They had faster vasoconstriction than the controls (P = .033), especially with cold detection stimulus. Individuals with higher anxiety also experienced more rapid vasoconstriction (P = .007). Augmented vasoconstriction responses and progressive decreases in perfusion with repeated thermal stimulation in SCD are indicative of autonomic hypersensitivity in the microvasculature. These effects are likely to increase red cell entrapment in response to clinical triggers such as cold or stress, which have been associated with vaso-occlusive crises in SCD.