Abstract Background Although annual transcranial Doppler (TCD) screening is recommended for children with sickle cell anemia (SCA), compliance is low and variable. Our objective was to utilize an electronic health record (EHR)‐based registry to improve TCD adherence among children with SCA, 2‐16 years of age, at our institution. Methods We developed an in‐EPIC real time registry for children with sickle cell disease in year 2016. Since end of year 2016, we have been extracting data quarterly to examine TCD rates and share the list of children who have not received a TCD screen in the past 18 months with the clinical team. The registry also includes a TCD risk score to enhance point of care. We also added Child Life support to increase TCD compliance among children <7 years. Control charts are used to examine TCD rates. Results At baseline, prior to and start of quarterly data audit and feedback, 63% of children received the recommended annual TCD screen. TCD rates steadily increased to 80% by the third quarter of 2017. We observed a dip in TCD rates, driven by failure of screening young children. Since the initiation of Child Life support for children <7 years, we have sustained TCD screen rates >70%. Overall, our data meet criteria for special cause variation, indicating improvement in TCD rates since 2015. Conclusions Regular tracking and identification of patients overdue for a TCD screen using an EHR‐based registry resulted in sustained improvement in TCD screening rates. Involvement of Child Life support further improved TCD rates.
We describe the challenging perioperative course of a 55-year-old patient with hepatic failure requiring liver transplantation (LT). Different modalities of the extracorporeal device were successfully used, ranging from veno-veno bypass to partial and full veno-veno extracorporeal membrane oxygenation (ECMO) in order to optimize preload, reduce bleeding from the collateral circulation, optimize acid base balance and/or improve oxygenation. The case highlights the potential use of the device as a rescue method in challenging cases. Furthermore this is the first documented case that extracorporeal CO 2 removal (ECCO 2 R) is used to optimize the biochemistry profile intraoperatively during a LT. The patient was weaned off the device at the end of the case and has been discharged home.
Objective: Sickle cell disease (SCD) is associated with neurocognitive and academic impairment. Routine screening is recommended to evaluate for possible concerns. This paper describes results from a neurocognitive screening battery and evaluates relationships with psychosocial and medical variables. Methods: Participants included 61 patients stratified among three age groups, including ages 6-7, ages 11-12, and ages 15-16. Patients completed a screening assessing cognitive performance, academic functioning, and attention (older subjects completed a task of executive function) and a measure assessing HRQL. Caregivers completed a clinical interview and forms evaluating HRQL, executive functioning, and attention. The impact of relevant medical variables and socio-economic status (SES) was also considered. Results: Between 20% and 33% of participants scored ≤ 1 SD below age-based means on intellectual subtests, and between 31 and 48% scored ≤ 1 SD below grade-based means on academic subtests. Attention concerns were noted in 25% of patients, while 20-31% of parents reported executive function concerns. Neither disease subtype nor hemoglobin were associated with cognitive or academic functioning. There was an association between mean corpuscular volume (MCV) and both cognitive abilities and psychosocial health. Cognitive scores were correlated with SES, suggesting an impact of socioeconomic disparities on performance. Conclusions: Sickle Cell Disease negatively impacts cognitive and academic functioning both directly and indirectly due to pain, missed school, and through socioeconomic and racial disparities. These concerns may increase over time as disease processes and medication compliance often worsen. Longitudinal research is needed to further explore these trends.
Spin-dependent optical transitions are attractive for a plethora of applications in quantum technologies. Here we report on utilization of high quality ring resonators fabricated from TiO2 to enhance the emission from negatively charged boron vacancies in hexagonal Boron Nitride. We show that the emission from these defects can efficiently couple into the whispering gallery modes of the ring resonators. Optically coupled boron vacancy showed photoluminescence contrast in optically detected magnetic resonance signals from the hybrid coupled devices. Our results demonstrate a practical method for integration of spin defects in 2D materials with dielectric resonators which is a promising platform for quantum technologies.
