Birth Prevalence of Sickle Cell Disease and County-Level Social Vulnerability - Sickle Cell Data Collection Program, 11 States, 2016-2020.

Sickle cell disease (SCD) remains a public health priority in the United States because of its association with complex health needs, reduced life expectancy, lifelong disabilities, and high cost of care. A cross-sectional analysis was conducted to calculate the crude and race-specific birth prevalence for SCD using state newborn screening program records during 2016-2020 from 11 Sickle Cell Data Collection program states. The percentage distribution of birth mother residence within Social Vulnerability Index quartiles was derived. Among 3,305 newborns with confirmed SCD (including 57% with homozygous hemoglobin S or sickle ß-null thalassemia across 11 states, 90% of whom were Black or African American [Black], and 4% of whom were Hispanic or Latino), the crude SCD birth prevalence was 4.83 per 10,000 (one in every 2,070) live births and 28.54 per 10,000 (one in every 350) non-Hispanic Black newborns. Approximately two thirds (67%) of mothers of newborns with SCD lived in counties with high or very high levels of social vulnerability; most mothers lived in counties with high or very high levels of vulnerability for racial and ethnic minority status (89%) and housing type and transportation (64%) themes. These findings can guide public health, health care systems, and community program planning and implementation that address social determinants of health for infants with SCD. Implementation of tailored interventions, including increasing access to transportation, improving housing, and advancing equity in high vulnerability areas, could facilitate care and improve health outcomes for children with SCD.

Detection of changes of functioning over time after asthma exacerbation in children with the use of PROMIS domains.

OBJECTIVES: Patient reported outcome measures, such as the Patient Reported Outcomes Measurement Information System (PROMIS) may be utilized to understand experiences of patients. The purpose of this study was to determine the ability of PROMIS domains to detect changes in pain, physical functioning, and asthma impact over time for children experiencing asthma exacerbation. METHODS: Our prospective cohort study included children presenting to the emergency department (ED) for asthma exacerbation. Children completed PROMIS surveys in the ED, 7-10 days, and 1-3 months post-discharge. We used linear mixed models adjusted for age, gender, acute care utilization, and child global health to determine changes in PROMIS T-scores. We used self-reported child health response (Much better now versus a little better now or worse) at discharge as an anchor to determine if change in PROMIS scores corresponded with changes in health. A change was statistically significant if the 95% CI did not include 0. RESULTS: Our study included 63 children who presented to the ED for acute asthma exacerbation. We identified that children improved significantly in all domains over time. There was improvement over time following discharge from ED for all pain and physical functioning domains, and asthma impact. Using the clinical anchor, those with considerable improvement in asthma symptoms had improved T scores from 4-17. CONCLUSIONS: PROMIS domains of pain, physical functioning, depression, fatigue, peer relationships, and asthma impact are responsive to changes in health states over time. These domains may be used to measure clinically significant change in children experiencing asthma exacerbation.

Measurement properties of Patient Reported Outcomes Measurement Information System domains for children with type 1 diabetes.

OBJECTIVE: Patient Reported Outcomes Measurement Information System (PROMIS) includes numerous domains to assess functioning among the pediatric population. These domains, however, have not been evaluated for use in children with type 1 diabetes (T1D). The objective of this study was to determine the measurement properties of PROMIS domains (pain behavior, pain quality, physical stress experience, physical activity, strength impact, and profile-25) in children with T1D. METHODS: This is a cross-sectional study of children with T1Drecruited from tertiary care facilities. To determine construct validity, we compared PROMIS T-scores between known-groups based on (a) glycemic control, hemoglobin A1c (HbA1c%) and (b) self-reported general health, using t test or analysis of variance. Reliability was determined using Cronbach's alpha and item response theory reliability. We also determined agreement between parent-proxy and child self-report PROMIS scores. RESULTS: Our study included 192 children, mean age 12.7 (SD = 2.9) years, eligible to self-report PROMIS surveys. There were significant differences in physical stress experience and pain intensity between children with HbA1c < 10% and those with HbA1c ≥ 10%. There also were significant differences in T-scores for all domains except physical function mobility and strength impact among children with poor/fair, good, very good/excellent general health. All valid domains had reliability >0.70. More than 40% of child-parent pairs were in agreement, with intraclass correlations coefficients (ICC) ranging between 0.41 and 0.63 for all domains, except pain behavior (%agreement = 23%; ICC = 0.29). CONCLUSIONS: Most of the PROMIS domains tested are valid, reliable, and able to differentiate children with T1D who report different general health states. There is moderate agreement between child-parent pairs for all domains except pain behavior.

Recommendation to reality: Closing the transcranial Doppler screening gap for children with sickle cell anemia.

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.

Coronavirus Disease among Persons with Sickle Cell Disease, United States, March 20-May 21, 2020.

Sickle cell disease (SCD) disproportionately affects Black or African American persons in the United States and can cause multisystem organ damage and reduced lifespan. Among 178 persons with SCD in the United States who were reported to an SCD-coronavirus disease case registry, 122 (69%) were hospitalized and 13 (7%) died.

Can PROMIS domains of pain and physical functioning detect changes in health over time for children with sickle cell disease?

BACKGROUND: The Patient-Reported Outcomes Measurement Information System (PROMIS) includes multiple domains that measure pain and physical functioning which are valid and reliable for use in children with sickle cell disease. The responsiveness of these measures to detect changes in health status over time among children with sickle cell disease is unknown. PROCEDURE: We conducted a prospective cohort study of children presenting to emergency department (ED) with vaso-occlusive crises. Children completed PROMIS surveys in the ED and at two follow-up time points (7-10 days and 1-3 months) after their acute care visit. Linear mixed models were used to determine if there were significant changes in PROMIS T scores over time. We used a patient's global assessment of change in pain question to anchor the changes in PROMIS scores (mean and 95% confidence interval). A change was considered statistically significant if the 95% CI did not include 0. RESULTS: We found that patients improved significantly in all domains 1 to 3 months after discharge from an acute care visit for pain. In addition, the pain and physical stress experience domains were responsive to change 7 to 10 days after discharge. Using the anchor of change in pain, for children who had considerable improvement in pain, there were significant changes in PROMIS T scores ranging from 6 to 15. CONCLUSIONS: Relevant PROMIS domains detect changes in children experiencing acute vaso-occlusive crises. These domains can be used in research and clinic settings to measure clinically relevant change in children with sickle cell disease.

Use of the new pediatric PROMIS measures of pain and physical experiences for children with sickle cell disease.

BACKGROUND: There are new pediatric domains to measure patients' pain and physical experiences in the Patient-Reported Outcomes Measurement Information System (PROMIS). The objective of this study was to establish the psychometric properties of these domains for children with sickle cell disease (SCD). PROCEDURE: We conducted a cross-sectional analysis of PROMIS assessments of children with SCD recruited from a pediatric tertiary care clinic. Validity of the new PROMIS domains was determined by comparing scores between known groups and describing their correlations with previously validated PROMIS measures. Cronbach's alpha and item response theory (IRT) reliability were used to assess internal consistency reliability. Agreement between parent-proxy and child self-report was determined for all domains. RESULTS: Our study included 164 subjects, of whom 117 were eligible to self-report. The mean T-scores for physical stress experience, strength impact, pain behavior, and pain quality sensory scores were significantly different between children who used pain medications in the prior week and those who did not. There were also differences in T-scores across children reporting mild, moderate, and severe pain on the pain intensity scale. All measures had Cronbach's alpha and IRT reliability > 0.80. The percentage of agreement between child and parent-proxy PROMIS domains ranged from 36% to 60% depending on the domain. CONCLUSIONS: The new PROMIS domains of physical stress experience, strength impact, pain behavior, and pain quality sensory domains are valid and reliable for children with SCD. The low-moderate agreement between parent-proxy and child self-report scores support the complementary information provided by the two perspectives.

Longitudinal Trend in Emergency Department Reliance for Pain Among Sickle Cell Disease Patients in Wisconsin.

Patients with sickle cell disease frequently visit the emergency department for pain. The metric of emergency department reliance (EDR) describes emergency department utilization in relation to all ambulatory visits and serves as a quality of care indicator. This study uses Wisconsin Medicaid data from 2011 to 2015 to examine trend of EDR for pain over the period of 5 years. We stratified our cohort (N=750) by patient ages into 4 groups: (1) children; (2) transition group; (3) young adults; and (4) adults. Using a linear mixed model, we estimated longitudinal trends adjusting for age group and hydroxyurea possession calculated as medication possession ratio. Results show that EDR for pain has distinct temporal patterns for each group. EDR for pediatrics continually remained less than the established threshold of 0.33. The EDR for transition group significantly increased over time; however, the EDR for young adults has significantly decreased since 2011. There were no significant differences in EDR over time for adults older than 30 years. Overall, increase in medication possession ratio was associated with lower EDR. The low EDR for pain among children and the improvements among adults indicate the success of efforts for sickle cell disease patients. However, further interventions are needed for the transition age group.

Deciphering the role of charge, hydration, and hydrophobicity for cytotoxic activities and membrane interactions of bile acid based facial amphiphiles.

We synthesized four cationic bile acid based facial amphiphiles featuring trimethyl ammonium head groups. We evaluated the role of these amphiphiles for cytotoxic activities against colon cancer cells and their membrane interactions by varying charge, hydration and hydrophobicity. The singly charged cationic Lithocholic acid based amphiphile (LCA-TMA1) is most cytotoxic, whereas the triply charged cationic Cholic acid based amphiphile (CA-TMA3) is least cytotoxic. Light microscopy and Annexin-FITC assay revealed that these facial amphiphiles caused late apoptosis. In addition, we studied the interactions of these amphiphiles with model membrane systems by Prodan-based hydration, DPH-based anisotropy, and differential scanning calorimetry. LCA-TMA1 is most hydrophobic with a hard charge causing efficient dehydration and maximum perturbations of membranes thereby facilitating translocation and high cytotoxicity against colon cancer cells. In contrast, the highly hydrated and multiple charged CA-TMA3 caused least membrane perturbations leading to low translocation and less cytotoxicity. As expected, Chenodeoxycholic acid and Deoxycholic acid based amphiphiles (CDCA-TMA2, DCA-TMA2) featuring two charged head groups showed intermediate behavior. Thus, we deciphered that charge, hydration, and hydrophobicity of these amphiphiles govern membrane interactions, translocation, and resulting cytoxicity against colon cancer cells.

Acute care utilization among individuals with sickle cell disease and related cardiopulmonary and renal complications.

Cardiopulmonary and renal end organ (CPR) complications are associated with early mortality among individuals with sickle cell disease (SCD). However, there is limited knowledge regarding acute care utilization for individuals with SCD and CPR complications. Our objective was to determine the prevalence of CPR complications in a state specific SCD population and compare acute care utilization among individuals with and without CPR complications. We leveraged 2017-2020 data for individuals with SCD identified by the Sickle Cell Data Collection program in Wisconsin. The prevalence of CPR complications is determined for distinct age groups. Generalized linear models adjusted for age compared the rate of acute care visits/person/year among individuals who had cardiopulmonary only, renal only, both cardiopulmonary and renal, or no CPR complications. There were 1378 individuals with SCD, 52% females, mean (SD) age 28.3 (18.5) years; 48% had at least one CPR complication during the study period. The prevalence of CPR complications was higher in adults (69%) compared to pediatric (15%) and transition (51%) groups. Individuals with SCD and cardiopulmonary complications had higher acute visit rates than those without CPR complications (5.4 (IQR 5.0-5.8) vs 2.4 (IQR 2.1-2.5), p <0.001)). Acute care visit rates were similar between individuals with SCD who had renal only complications and no CPR complications (2.7 (IQR 2.5-3.0) vs 2.4 (2.1-2.5), p = 0.24). The high acute care visit rates, especially for those with cardiopulmonary complications, warrant further investigation to understand risk factors for CPR complications, the underlying reasons and identify effective disease management strategies.

Timing and co-occurrence of symptoms prior to a diagnosis of light chain (AL) amyloidosis.

It is well-established that light chain (AL) amyloidosis patients have multi-organ involvement and are often diagnosed after a lag period of increasing symptoms. We leverage electronic health record (EHR) data from the TriNetX research network to describe the incidence, timing, and co-occurrence of precursor conditions of interests in a cohort of AL amyloidosis patients identified between October 2015-December 2020. Nineteen precursor diagnoses of interest representing features of AL amyloidosis were identified using ICD codes up to 36 months prior to AL amyloidosis diagnosis. Among 1,401 patients with at least 36 months of EHR data prior to AL amyloidosis diagnosis, 46% were females, 16% were non-Hispanic Black, and 6% were Hispanic. The median age was 71 (range, 21-91) years. The median number of precursor diagnoses was 5 with dyspnea and fatigue being the most prevalent. The time from the first occurrence of a precursor to AL diagnosis ranged from 3.2 to 21.4 months. Analyses of pairwise co-occurrence of specific diagnoses indicated a high association (Cole's coefficient > 0.6) among the examined precursor diagnoses. These findings provide novel information about the timing and co-occurrence of key precursor conditions and could be used to develop algorithms for early identification of AL amyloidosis.

Timing and co-occurrence of symptoms prior to a diagnosis of light chain (AL) amyloidosis.

It is well-established that most patients with systemic light chain (AL) amyloidosis have multi-organ involvement and are often diagnosed after a lag period of increasing symptoms. We leverage electronic health record (EHR) data from the TriNetX research network to describe the incidence, timing, and co-occurrence of precursor conditions of interests in a cohort of AL amyloidosis patients identified between October 2015-December 2020. Nineteen precursor diagnoses of interest representing features of AL amyloidosis were identified using ICD codes up to 36 months prior to AL amyloidosis diagnosis. Among 1,401 patients with at least 36 months of EHR data prior to AL amyloidosis diagnosis, 46% were females, 16% were non-Hispanic Black, and 6% were Hispanic. The median age was 71 (range, 21-91) years. The median number of precursor diagnoses was 5 with dyspnea and fatigue being the most prevalent. The time from the first occurrence of a precursor to AL diagnosis ranged from 3.2 to 21.4 months. Analyses of pairwise co-occurrence of specific diagnoses indicated a high association (Cole's coefficient >0.6) among the examined precursor diagnoses. These findings provide novel information about the timing and co-occurrence of key precursor conditions and could be used to develop algorithms for early identification of AL amyloidosis.

The importance of nursing homes in the spread of methicillin-resistant Staphylococcus aureus (MRSA) among hospitals.

BACKGROUND: Hospital infection control strategies and programs may not consider control of methicillin-resistant Staphylococcus aureus (MRSA) in nursing homes in a county. METHODS: Using our Regional Healthcare Ecosystem Analyst, we augmented our existing agent-based model of all hospitals in Orange County (OC), California, by adding all nursing homes and then simulated MRSA outbreaks in various health care facilities. RESULTS: The addition of nursing homes substantially changed MRSA transmission dynamics throughout the county. The presence of nursing homes substantially potentiated the effects of hospital outbreaks on other hospitals, leading to an average 46.2% (range, 3.3%-156.1%) relative increase above and beyond the impact when only hospitals are included for an outbreak in OC's largest hospital. An outbreak in the largest hospital affected all other hospitals (average 2.1% relative prevalence increase) and the majority (~90%) of nursing homes (average 3.2% relative increase) after 6 months. An outbreak in the largest nursing home had effects on multiple OC hospitals, increasing MRSA prevalence in directly connected hospitals by an average 0.3% and in hospitals not directly connected through patient transfers by an average 0.1% after 6 months. A nursing home outbreak also had some effect on MRSA prevalence in other nursing homes. CONCLUSIONS: Nursing homes, even those not connected by direct patient transfers, may be a vital component of a hospital's infection control strategy. To achieve effective control, a hospital may want to better understand how regional nursing homes and hospitals are connected through both direct and indirect (with intervening stays at home) patient sharing.

Impacts of changing atmospheric deposition chemistry on nitrogen and phosphorus loading to Ganga River (India).

Investigations on atmospheric deposition (AD) and water chemistry along a 35 km stretch of Ganga River indicated that although N:P stoichiometry of AD did not change, there were over 1.4-2.0 fold increase in AD-NO3⁻, AD-NH4⁺ and AD-PO4³â» overtime. Concentration of dissolved inorganic-N (DIN) in river showed significant positive correlations with AD-NO3⁻ and runoff DIN. Similarly, dissolved reactive-P (DRP) in river showed significant positive correlation with AD-PO4³â» and runoff DRP. The study shows that AD has become an important source of N and P input to Ganga River.

OmicPredict: a framework for omics data prediction using ANOVA-Firefly algorithm for feature selection.

High-throughput technologies and machine learning (ML), when applied to a huge pool of medical data such as omics data, result in efficient analysis. Recent research aims to apply and develop ML models to predict a disease well in time using available omics datasets. The present work proposed a framework, 'OmicPredict', deploying a hybrid feature selection method and deep neural network (DNN) model to predict multiple diseases using omics data. The hybrid feature selection method is developed using the Analysis of Variance (ANOVA) technique and firefly algorithm. The OmicPredict framework is applied to three case studies, Alzheimer's disease, Breast cancer, and Coronavirus disease 2019 (COVID-19). In the case study of Alzheimer's disease, the framework predicts patients using GSE33000 and GSE44770 dataset. In the case study of Breast cancer, the framework predicts human epidermal growth factor receptor 2 (HER2) subtype status using Molecular Taxonomy of Breast Cancer International Consortium (METABRIC) dataset. In the case study of COVID-19, the framework performs patients' classification using GSE157103 dataset. The experimental results show that DNN model achieved an Area Under Curve (AUC) score of 0.949 for the Alzheimer's (GSE33000 and GSE44770) dataset. Furthermore, it achieved an AUC score of 0.987 and 0.989 for breast cancer (METABRIC) and COVID-19 (GSE157103) datasets, respectively, outperforming Random Forest, Naïve Bayes models, and the existing research.

Comparison of CAT and short forms for PROMIS pain and physical health domains in children with sickle cell disease.

BACKGROUND: Pain and physical health domains included in Patient-Reported Outcomes Measurement Information System® (PROMIS®) can be administered as short forms (SF) or as computer adaptive tests (CAT). CAT is ideal in many settings but cannot be administered without specialized technology. We compared SF and CAT to identify items for customized SFs to improve the SF performance for children with sickle cell disease (SCD). METHODS: Eligible children 8-17 years old were administered CATs for 5 domains of physical health and 2 domains of pain, followed by any items on the corresponding SF that were not included in the CAT assessments. We describe the range of scores on the CAT and SFs, including the percentage of participants with floor or ceiling effects using the SF. The agreement and correlation between CAT and SF scores were assessed using Bland-Altman plots. Items frequently offered on CAT that had variable responses and were not already present on SF are recommended as additional items for customized SFs. RESULTS: Among 90 children with SCD, there were strong correlations between CAT and SF scores (Concordance Correlation Coefficient > 0.8) however, the SFs for fatigue, mobility, strength impact, pain behavior, and pain interference had substantial floor/ceiling effects. Fatigue, mobility, physical stress experience, and pain behavior domains had items that were frequently offered on CAT, variable responses, and were not present on the SF. CONCLUSIONS: Adding items to the SFs for the fatigue, mobility, physical stress experience, and pain behavior domains may improve these domains' SFs performance for children with SCD.

Preliminary construct validity of patient-reported outcomes to assess chronic pain in adults with sickle cell disease.

Chronic pain affects 30% to 40% of individuals with sickle cell disease (SCD) and impairs patient functioning. Clinically meaningful, practical, and valid assessment tools for investigation, evaluation, and management of chronic pain are limited, representing a barrier for advancing SCD care. We sought to determine whether patient-reported outcomes (PROs) show preliminary construct validity in identifying individuals with SCD who were a priori defined as suggestive of having chronic pain based on previously published criteria. All individuals completed the Patient-Reported Outcomes Measurement Information System (PROMIS) domains: pain interference, pain behavior, pain quality (nociceptive, neuropathic), fatigue, sleep disturbance, depression, and anxiety; the Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me) domains: pain impact and emotional impact; and the painDETECT questionnaire. Thirty-three adults living with SCD were enrolled, and 42.4% had chronic pain. Pain-related PROs scores distinctly differentiated individuals with chronic pain from those without. Individuals with chronic pain had significantly worse pain-related PROs scores: PROMIS pain interference (64.2 vs 54.3), PROMIS pain behavior (63.2 vs 50), and ASCQ-Me pain impact (42.9 vs 53.2). According to published PROMIS clinical cut scores for the pain-related domains, individuals with chronic pain were categorized as having moderate impairment, whereas those without chronic pain had mild or no impairment. Individuals with chronic pain had PRO pain features consistent with neuropathic pain and worse scores in fatigue, depression, sleep disturbance, and emotional impact. Pain-related PROs show preliminary construct validity in differentiating individuals with and without chronic SCD pain and could be used as valuable tools for research and clinical monitoring of chronic pain.

Evaluating the Discriminatory Ability of the Sickle Cell Data Collection Program's Administrative Claims Case Definition in Identifying Adults With Sickle Cell Disease: Validation Study.

BACKGROUND: Sickle cell disease (SCD) was first recognized in 1910 and identified as a genetic condition in 1949. However, there is not a universal clinical registry that can be used currently to estimate its prevalence. The Sickle Cell Data Collection (SCDC) program, funded by the Centers for Disease Control and Prevention, funds state-level grantees to compile data within their states from various sources including administrative claims to identify individuals with SCD. The performance of the SCDC administrative claims case definition has been validated in a pediatric population with SCD, but it has not been tested in adults. OBJECTIVE: The objective of our study is to evaluate the discriminatory ability of the SCDC administrative claims case definition to accurately identify adults with SCD using Medicaid insurance claims data. METHODS: Our study used Medicaid claims data in combination with hospital-based medical record data from the Alabama, Georgia, and Wisconsin SCDC programs to identify individuals aged 18 years or older meeting the SCDC administrative claims case definition. In order to validate this definition, our study included only those individuals who were identified in both Medicaid's and the partnering clinical institution's records. We used clinical laboratory tests and diagnostic algorithms to determine the true SCD status of this subset of patients. Positive predictive values (PPV) are reported overall and by state under several scenarios. RESULTS: There were 1219 individuals (354 from Alabama and 865 from Georgia) who were identified through a 5-year time period. The 5-year time period yielded a PPV of 88.4% (91% for data from Alabama and 87% for data from Georgia), when only using data with laboratory-confirmed (gold standard) cases as true positives. With a narrower time period (3-year period) and data from 3 states (Alabama, Georgia, and Wisconsin), a total of 1432 individuals from these states were included in our study. The overall 3-year PPV was 89.4% (92%, 93%, and 81% for data from Alabama, Georgia, and Wisconsin, respectively) when only considering laboratory-confirmed cases as true cases. CONCLUSIONS: Adults identified as having SCD from administrative claims data based on the SCDC case definition have a high probability of truly having the disease, especially if those hospitals have active SCD programs. Administrative claims are thus a valuable data source to identify adults with SCD in a state and understand their epidemiology and health care service usage.

Perspectives on Virtual Care for Childhood Cancer Survivors in Non-Metropolitan Areas during the COVID-19 Pandemic.

The COVID-19 pandemic paved the way for the widespread use of virtual care for childhood cancer survivors (CCSs). CCSs were virtual recipients of diverse care, including long-term follow-up (LTFU), primary care, mental health care, and several others. Virtual care comes with well-documented benefits and challenges. These are further magnified for CCSs living in rural or non-metropolitan areas. Here, we describe the virtual care of CCSs from two Upper Midwest cities with well-established childhood cancer survivor programs within large comprehensive cancer centers in the United States. CCSs from non-metropolitan areas, especially CCSs with two or more late effects, used virtual care more often during the COVID-19 pandemic compared to CCSs from metropolitan areas. A review of the related literature is also included and the identified challenges in providing virtual care, such as privacy concerns, technology-connectivity constraints, and medical license restrictions. Despite these limitations, the care of CCSs has evolved to leverage virtual care and its ability to increase access for patients and promote continuity of care for CCSs living in rural areas.

Common data model for sickle cell disease surveillance: considerations and implications.

Objective: Population-level data on sickle cell disease (SCD) are sparse in the United States. The Centers for Disease Control and Prevention (CDC) is addressing the need for SCD surveillance through state-level Sickle Cell Data Collection Programs (SCDC). The SCDC developed a pilot common informatics infrastructure to standardize processes across states. Materials and Methods: We describe the process for establishing and maintaining the proposed common informatics infrastructure for a rare disease, starting with a common data model and identify key data elements for public health SCD reporting. Results: The proposed model is constructed to allow pooling of table shells across states for comparison. Core Surveillance Data reports are compiled based on aggregate data provided by states to CDC annually. Discussion and Conclusion: We successfully implemented a pilot SCDC common informatics infrastructure to strengthen our distributed data network and provide a blueprint for similar initiatives in other rare diseases.