BACKGROUND:Adverse childhood experiences (ACEs) are stressful or traumatic events prior to age 18 that are known to have a lasting impact on individuals' health and well-being. There is a gap in understanding the relationship between ACEs and Other Life Stressors and health status for adults with sickle cell disease (SCD). We examined the impact of adversities within clinical and behavioral health domains that have been associated with ACEs for other populations. PROCEDURE:Sociodemographics; medical history; patient-reported outcomes, including pain and emotional distress; and reports of de-identified ACEs and Other Life Stressors, were collected from n = 553 participants enrolled in the Sickle Cell Disease Implementation Consortium Registry. RESULTS:Participants were a median age of 28 years; 57% female; 95% Black/African American; 55% annual household income ≤$25,000; primarily diagnosed with sickle cell anemia (SS or Sβ0 thalassemia: 69.6%). Adults with SCD evidenced a high prevalence of exposure to adversity (28.3% reporting four or more ACEs), significantly higher than general or Black/African American populations (p < 0.001). Adjusting for age, gender, SCD genotype, annual household income, and disease-modifying therapy, we found a graded effect for categories of original ACEs (one to three, and four or more), with progressively greater odds of being diagnosed with asthma with increasing ACEs (p <0.0001), as well as for "ever been treated for depression" (p < 0.05). CONCLUSIONS:Our findings support the need for consistent screening for ACEs in adults with SCD and highlight the importance of trauma-informed care approaches to improve clinical and behavioral outcomes and quality of life.

To address acute vaso-occlusive episodes (VOEs), the leading cause of Emergency Department (ED) visits among individuals with sickle cell disease (SCD), we conducted the clinical study, An Individualized Pain Plan with Patient and Provider Access for Emergency Department care of SCD (ALIGN), across eight sites. We hypothesized an improvement of 0.5 standard deviations in perceived quality of ED pain treatment of a VOE after implementing individualized pain plans (IPPs) accessible to both patients and providers. Patients with SCD were 18-45, owned a cell phone, and had an ED VOE visit within 90 days prior. Patients completed perceived quality of care surveys at baseline and within 96 hours after a VOE ED visit. Providers completed surveys regarding comfort managing VOEs at baseline and after managing an enrolled patient. Most of the 153 patients were African American (95.4%), female (64.7%) and had Hb SS/Sβ0 genotype (71.9%). The perceived quality of ED pain treatment was high at both baseline and post implementation of IPPs; our primary outcome hypothesis was not met, as no statistically significant change in patient perceived quality ED treatment ocurred. A total of 135 providers completed baseline and follow-up surveys. On a scale of 1-7, with 7 being extremely comfortable managing VOEs, 60.5% reported a score ≥6 post IPP implementation vs. 57.8% at baseline. Almost all (97.6%) ordered the recommended medication, and 94.7% intend to use IPPs. In this implementation protocol, all sites successfully implemented IPPs . Patients and ED providers both endorsed the use of IPPs.

Background: HIV/AIDS remains a highly stigmatizing disease worldwide, preventing people with risk or infection from testing to learn their HIV status, accessing supportive services, or taking antiretroviral therapy. Despite many studies of HIV in blood donors, no studies have evaluated the factors that contribute to stigma surrounding this illness following notification process and counseling provided by blood centers. Methods: A cross-sectional questionnaire-based survey was conducted between 2016 and 2017. Persons with HIV were invited to return to the blood center for an audio computer-assisted interview after participation in an HIV risk factor assessment study conducted from 2007 to 2016. The questionnaire was based on HIV risk interviews developed by the US CDC, with modifications appropriate to the Brazilian setting which aimed to evaluate their follow-up activities, perceptions of HIV stigma and discrimination, and the quality of counseling and notification after the donation that tested positive for HIV. Response frequencies and adjusted odds ratios from multivariable logistic regression analyses are reported. Results: 268 HIV-positive blood donors agreed to participate in the study. Almost all participants, 262 (97 %), rated as very important or important the blood center counseling experience in their decision to seek health care. One-hundred-five (39 %) participants reported none to low levels of stigma, and 163 (61 %) participants moderate stigma. Individuals reporting heterosexual orientation (OR=2.13, 95 % CI [1.08‒4.22]) and healthcare-seeking behavior (OR=2.46, 95 % CI [1.10‒5.48]) had significantly increased odds of reporting moderate levels of stigma. Conclusions: Our study provides information about perceived stigma and discrimination in the Brazilian blood donor population and reinforces the importance of the counseling process in linkage to care and reducing HIV-related stigma.

Background Electronic health record-linked portals may improve health-care quality for patients with cancer. Barriers to portal access and use undermine interventions that rely on portals to reduce cancer care disparities. This study examined portal access and persistence of portal use and associations with patient and structural factors before the implementation of 3 portal-based interventions within the Improving the Management of symPtoms during And following Cancer Treatment (IMPACT) Consortium.Methods Portal use data were extracted from electronic health records for the 12 months preceding intervention implementation. Sociodemographic factors, mode of accessing portals (web vs mobile), and number of clinical encounters before intervention implementation were also extracted. Rurality was derived using rural-urban commuting area codes. Broadband access was estimated using the 2015-2019 American Community Survey. Multiple logistic regression models tested the associations of these factors with portal access (ever accessed or never accessed) and persistence of portal use (accessed the portal <= 20 weeks vs >= 21 weeks in the 35-week study period).Results Of 28 942 eligible patients, 10 061 (35%) never accessed the portal. Male sex, membership in a racial and ethnic minority group, rural dwelling, not working, and limited broadband access were associated with lower odds of portal access. Younger age and more clinical encounters were associated with higher odds of portal access. Of those with portal access, 25% were persistent users. Using multiple modalities for portal access, being middle-aged, and having more clinical encounters were associated with persistent portal use.Conclusion Patient and structural factors affect portal access and use and may exacerbate disparities in electronic health record-based cancer symptom surveillance and management.

ObjectiveSickle cell disease (SCD) is associated with complications during pregnancy and can negatively influence maternal outcomes. Our study aimed to determine the prevalence and predictors of maternal morbidity among participants enrolled in an eight-site SCD Implementation Consortium (SCDIC) registry.MethodsWe conducted a cross-sectional analysis of female registry participants, aged 15-45 years, with a confirmed diagnosis of SCD. Participants completed a survey of self-reported pregnancies and outcomes.ResultsSeven hundred and thirty-eight individuals had at least one pregnancy event, with 1076 live births. Twenty percent reported a pregnancy loss or fetal demise. Of the 1076 live births, 75% involved at least one complication. The most prevalent complications were pain crises (61.1%) and pregnancy requiring blood transfusion(s) (33.0%). Multiparous individuals with a prior occurrence of a complication in a previous pregnancy had higher odds of recurrence of the same complication in subsequent pregnancies (i.e., previous acute crisis was associated with subsequent acute pain events odds ratio [OR]: 3.13; 95% confidence interval [CI]: 2.06-4.76) and prior transfusion requiring another transfusion (OR: 3.22; 95% CI: 2.01-5.16).ResultsSeven hundred and thirty-eight individuals had at least one pregnancy event, with 1076 live births. Twenty percent reported a pregnancy loss or fetal demise. Of the 1076 live births, 75% involved at least one complication. The most prevalent complications were pain crises (61.1%) and pregnancy requiring blood transfusion(s) (33.0%). Multiparous individuals with a prior occurrence of a complication in a previous pregnancy had higher odds of recurrence of the same complication in subsequent pregnancies (i.e., previous acute crisis was associated with subsequent acute pain events odds ratio [OR]: 3.13; 95% confidence interval [CI]: 2.06-4.76) and prior transfusion requiring another transfusion (OR: 3.22; 95% CI: 2.01-5.16).ConclusionIndividuals reported a high prevalence of pregnancy loss and maternal complications. Our findings underscore the importance of preconception counseling and early initiation of perinatal care in SCD.

Introduction The leading cause of Emergency Department (ED) visits among individuals with sickle cell disease (SCD) is acute pain vaso-occlusive episodes (VOE's). NHLBI guidelines recommend treatment with individualized pain plans (IPP's). To facilitate the uptake of IPP's, the National Heart Lung and Blood Institute funded eight centers across the US to participate in the Sickle Cell Disease Implementation Consortium (SCDIC). We report findings from one of the consortium's implementation projects: An Individualized Pain Plan with Patient and Provider Access for Emergency Department Care of Sickle Cell Disease (ALIGN). In ALIGN, the SCD specialist developed individualized pain plans (IPPs) for ED providers via the electronic health record (EHR) and patients via their patient portal in a phone app. We assessed changes in patient satisfaction with ED care and ED provider comfort in managing VOE after a recent visit between baseline and after IPP implementation for ED providers and patients. We hypothesized an improvement of 0.5 standard deviations in patient satisfaction from baseline to after the implementation of IPPs. We also assessed patient and provider willingness to use IPPs in the EHR and patient portal. Methods Patients and providers were enrolled from the eight SCDIC sites; six sites had IPPs already available in the EHR for ED providers, and none had IPPs available in the patient portal. Patients were eligible if they were 18-45 years old, had SCD, had access to a cell phone, had an ED VOE visit 90 days before enrollment, and had an ED VOE visit to the study site's ED within one year. Provider inclusion criteria were ED physicians, nurse practitioners, and physician's assistants with access to the EHR. The SCD specialist wrote the IPP after patient enrollment. Patients completed surveys at baseline about an ED visit within the last 90 days and again after enrollment, within 96 hours after an ED visit for VOE. The surveys collected data on patient-perceived quality of care and satisfaction with the ED. Providers completed surveys at baseline and after managing an enrolled patient in the ED. The surveys collected data on provider comfort in managing VOE. Both patients and providers answered questions about their likelihood of using IPPs. Descriptive statistics were utilized to examine sociodemographic characteristics and summarize patient and provider satisfaction. Results One hundred fifty-nine patients completed the baseline and follow-up survey. Almost all were African American (95.4%), female (64.7%), and had Hb SS/Sβ 0 genotype (71.9%). Majority (57.6%) reported they were quite a bit or very much satisfied with the care they received at baseline. There was no difference in satisfaction at baseline vs post IPP implementation (P=0.3). About 77.2% of patients responded either very much or quite a bit when asked how much the ED doctors and nurses believed they had very bad SCD pain after implementing IPPs compared with 65.4% at baseline (p=0.02). Most (64.1%) patients were more or extremely likely to show the doctor their pain plan when they returned to the ED. One hundred thirty-five providers completed the baseline and at least one follow-up survey. There was no difference is provider comfortability in their ability to manage the acute VOE post IPP implementation compared to baseline (p=0.6). More than half (62.2%) could see the pain plan in the EHR and 52.38% found the IPPs extremely easy to use. Almost all (97.6%) ED providers ordered the opioids as suggested in the IPPs. Almost all (94.7%) reported they intended to use the IPPs most or all the time. Conclusion In this implementation science protocol, all eight sites successfully implemented IPPs in the EHR for ED providers and patients in their patient portal. Both patients and ED providers enthusiastically endorsed the use of IPP's, which can help guide management of VOE in any ED, regardless of the center.

IMPORTANCE Pain related to sickle cell disease (SCD) is complex and associated with social determinants of health. Emotional and stress-related effects of SCD impact daily quality of life and the frequency and severity of pain. OBJECTIVE To explore the association of educational attainment, employment status, and mental health with pain episode frequency and severity among individuals with SCD. DESIGN, SETTING, AND PARTICIPANTS This is a cross-sectional analysis of patient registry data collected at baseline (2017-2018) from patients treated at 8 sites of the US Sickle Cell Disease Implementation Consortium. Data analysis was performed from September 2020 to March 2022. MAIN OUTCOMES AND MEASURES Electronic medical record abstraction and a participant survey provided demographic data, mental health diagnosis, and Adult Sickle Cell Quality of Life Measurement Information System pain scores. Multivariable regression was used to examine the associations of education, employment, and mental health with the main outcomes (pain frequency and pain severity). RESULTS The study enrolled a total of 2264 participants aged 15 to 45 years (mean [SD] age, 27.9 [7.9] years; 1272 female participants [56.2%]) with SCD. Nearly one-half of the participant sample reported taking daily pain medication (1057 participants [47.0%]) and/or hydroxyurea use (1091 participants [49.2%]), 627 participants (28.0%) received regular blood transfusion, 457 (20.0%) had a depression diagnosis confirmed by medical record abstraction, 1789 (79.8%) reported severe pain (rated most recent pain crises as similar to 7 out of 10), and 1078 (47.8%) reported more than 4 pain episodes in the prior 12 months. The mean (SD) pain frequency and severity t scores for the sample were 48.6 (11.4) and 50.3 (10.1), respectively. Educational attainment and income were not associated with increased pain frequency or severity. Unemployment (beta, 2.13; 95% CI, 0.99 to 3.23; P <.001) and female sex (beta, 1.78; 95% CI, 0.80 to 2.76; P <.001) were associated with increased pain frequency. Age younger than 18 years was inversely associated with pain frequency (beta, -5.72; 95% CI, -7.72 to -3.72; P <.001) and pain severity (beta, 5.10; 95% CI, -6.70 to -3.51; P <.001). Depression was associated with increased pain frequency (beta, 2.18; 95% CI, 1.04 to 3.31; P <.001) but not pain severity. Hydroxyurea use was associated with increased pain severity (beta, 1.36; 95% CI, 0.47 to 2.24; P =.003), and daily use of pain medication was associated with both increased pain frequency (beta, 6.29; 95% CI, 5.28 to 7.31; P <.001) and pain severity (beta, 2.87; 95% CI, 1.95 to 3.80; P <.001). CONCLUSIONS AND RELEVANCE These findings suggest that employment status, sex, age, and depression are associated with pain frequency among patients with SCD. Depression screening for these patients is warranted, especially among those experiencing higher pain frequency and severity.

BACKGROUND: Arterial tortuosity is associated with adverse events in Marfan and Loeys-Dietz syndromes but remains understudied in Vascular Ehlers-Danlos syndrome. METHODS AND RESULTS: Subjects with a pathogenic COL3A1 variant diagnosed at age <50 years were included from 2 institutions and the GenTAC Registry (National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions). Height-adjusted vertebral artery tortuosity index (VTI-h) using magnetic resonance or computed tomography angiography was calculated. Associations between VTI-h and outcomes of (1) cardiovascular events (arterial dissection/rupture, aneurysm requiring intervention, stroke), or (2) hollow organ collapse/rupture at age <50 years were evaluated using receiver operator curve analysis (using outcome by age 30 years) and mixed-effects Poisson regression for incidence rate ratios. Of 65 subjects (54% male), median VTI-h was 12 (interquartile range, 8-16). Variants were missense in 46%, splice site in 31%, and null/gene deletion in 14%. Thirty-two subjects (49%) had 59 events, including 28 dissections, 5 arterial ruptures, 4 aneurysms requiring intervention, 4 strokes, 11 hollow organ ruptures, and 7 pneumothoraces. Receiver operator curve analysis suggested optimal discrimination at VTI-h >= 15.5 for cardiovascular events (sensitivity 70%, specificity 76%) and no association with noncardiovascular events (area under the curve, 0.49 [95% CI, 0.22-0.78]). By multivariable analysis, older age was associated with increased cardiovascular event rate while VTI-h >= 15.5 was not (incidence rate ratios, 1.79 [95% CI, 0.76-4.24], P=0.185). However, VTI-h >= 15.5 was associated with events among those with high-risk variants <40 years (incidence rate ratios, 4.14 [95% CI, 1.13-15.10], P= 0.032), suggesting effect modification by genotype and age. CONCLUSIONS: Increased arterial tortuosity is associated with a higher incidence rate of cardiovascular events in Vascular Ehlers-Danlos syndrome. Vertebral tortuosity index may be a useful biomarker for prognosis when evaluated in conjunction with genotype and age.

419 Background: The patient portal is part of an electronic health record (EHR) that allows patients to communicate with their healthcare team. The portal also provides a platform for patients to receive and complete symptom surveys that can be directly integrated into the EHR, allowing clinical care teams to monitor symptoms and provide cancer symptom management. The Improving Management of symPtoms during And following Cancer Treatment (IMPACT) consortium, supported by the National Cancer Institute’s Cancer MoonshotSM, aims to improve symptom control for cancer patients through assessment and symptom management interventions deployed via the EHR. This initiative presents an opportunity to examine portal enrollment and variation in use, factors critical to successful implementation, especially among groups that have high cancer symptom burden. To this end, we examine: 1) relationships between portal enrollment prior to the launch of IMPACT interventions and neighborhood broadband access, demographic, and social characteristics; and 2) frequency of pre-intervention portal use for any purpose among enrollees. Methods: Data are derived from two of three IMPACT research centers. Enrollment in and frequency of portal use, mode of accessing the portal (web vs. phone), social, demographic, and cultural factors were extracted from the EHR. Rural Urban Commuting Area (RUCA) codes were used to classify population density and degree of rurality. Broadband access was estimated using 2015-2019 American Community Survey estimates matched to zip codes from enrolled IMPACT patients and classified as a patient’s residence being in a community with high (≥85% of households) or low (< 85% of households) access. Bivariate comparisons and adjusted odds ratios were used to describe all associations. Results: Forty-seven percent of patients (22,596/48,034) were enrolled in the portal prior to the intervention. Patients in zip codes with low broadband access and those who were men, > 65 years old, not White, of Hispanic ethnicity, or disabled or not employed had significantly lower odds of being enrolled in the portal. If enrolled, 21% (n = 4825) used the portal at least once a week. Less variation was found in the average frequency of portal use, but patients younger than 40, and those who were Black, disabled, unemployed, or those who used a mobile device to access the portal had the lowest odds of accessing it at least once a week. Conclusions: Significant disparities in portal enrollment exist across demographic groups and among those with limited broadband access. Among those enrolled, most used the portal less than once a week. Fewer differences in frequency of use were observed by sociodemographic factors. Improving portal enrollment and frequency of use may be critical for symptom management interventions deployed via patient portals.

Purpose To examine the relations between patient-reported outcomes (PROs) within a conceptual model for adults with sickle cell disease (SCD) ages 18 – 45 years enrolled in the multi-site Sickle Cell Disease Implementation Consortium (SCDIC) registry. We hypothesized that patient and SCD-related factors, particularly pain, and barriers to care would independently contribute to functioning as measured using PRO domains. Methods Participants ( N = 2054) completed a 48-item survey including socio-demographics and PRO measures, e.g., social functioning, pain impact, emotional distress, and cognitive functioning. Participants reported on lifetime SCD complications, pain episode frequency and severity, and barriers to healthcare. Results Higher pain frequency was associated with higher odds of worse outcomes in all PRO domains, controlling for age, gender and site (OR range 1.02–1.10, 95% CI range [1.004–1.12]). Reported history of treatment for depression was associated with 5 of 7 PRO measures (OR range 1.58–3.28 95% CI range [1.18–4.32]). Fewer individual barriers to care and fewer SCD complications were associated with better outcomes in the emotion domain (OR range 0.46–0.64, 95% CI range [0.34–0.86]). Conclusions Study results highlight the importance of the biopsychosocial model to enhance understanding of the needs of this complex population, and to design multi-dimensional approaches for providing more effective interventions to improve outcomes.

SummaryIndividuals with sickle cell disease (SCD) have historically been considered underweight. Despite increasing body mass index (BMI) in the general population, the prevalence of overweight and obese status remains unclear in the adult SCD population. Our primary aim was to determine the prevalence of overweight and obese status and to identify associations between BMI, demographic, and clinical characteristics. We conducted an analysis of abstracted electronic health record data and patient‐reported outcomes from the Sickle Cell Disease Implementation Consortium registry; individuals aged 20–45 years were included. The median (interquartile range) BMI for the 1664 adults in this analysis was 23.9 (21.1–28) kg/m2. In this cohort, 42.9% had a BMI of >25 kg/m2 (Centers for Disease Control and Prevention definition of overweight/obese). In multivariable analysis, higher odds of being overweight or obese were associated with female gender, older age, college education, private insurance, and hypertension diagnosis. Higher odds of a BMI of >25 kg/m2 were observed in individuals with HbSC or HbSβ+ thalassaemia regardless of hydroxycarbamide (hydroxyurea) exposure (odds ratio [OR] 3.4, p < 0.0001) and HbSS or HbSβ0 thalassaemia exposed to hydroxycarbamide (OR 1.6, p = 0.0003) compared to those with HbSS or HbSβ0 thalassaemia with no hydroxycarbamide exposure. These data highlight the importance of early identification, prevention, and intervention for increasing BMI to reduce obesity‐related complications that may impact SCD‐related complications.

Sickle cell disease (SCD) is a genetic disorder predominantly affecting people of African descent and is associated with significant morbidity and mortality. To improve SCD outcomes, the National Heart Lung and Blood Institute funded eight centers to participate in the SCD Implementation Consortium. Sites were required to each recruit 300 individuals with SCD, over 20 months. We aim to describe recruitment strategies and challenges encountered. Participants aged 15–45 years with confirmed diagnosis of SCD were eligible. Descriptive statistics were used to analyze the effectiveness of each recruitment strategy. A total of 2432 participants were recruited. Majority (95.3%) were African American. Successful strategies were recruitment from clinics (68.1%) and affiliated sites (15.6%). Recruitment at community events, emergency departments and pain centers had the lowest yield. Challenges included saturation of strategies and time constraints. Effective recruitment of participants in multi-site studies requires multiple strategies to achieve adequate sample sizes.

OBJECTIVES:Sickle cell disease (SCD) leads to chronic and acute complications that require specialised care to manage symptoms and optimise clinical results. The National Heart Lung and Blood Institute (NHLBI) evidence-based guidelines assist providers in caring for individuals with SCD, but adoption of these guidelines by providers has not been optimal. The objective of this study was to identify barriers to treating individuals with SCD.METHODS:The SCD Implementation Consortium aimed to investigate the perception and level of comfort of providers regarding evidence-based care by surveying providers in the regions of six clinical centres across the USA, focusing on non-emergency care from the providers' perspective.RESULTS:Respondents included 105 providers delivering clinical care for individuals with SCD. Areas of practice were most frequently paediatrics (24%) or haematology/SCD specialist (24%). The majority (77%) reported that they were comfortable managing acute pain episodes while 63% expressed comfort with managing chronic pain. Haematologists and SCD specialists showed higher comfort levels prescribing opioids (100% vs 67%, p=0.004) and managing care with hydroxyurea (90% vs 51%, p=0.005) compared with non-haematology providers. Approximately 33% of providers were unaware of the 2014 NHLBI guidelines. Nearly 63% of providers felt patients' medical needs were addressed while only 22% felt their mental health needs were met.CONCLUSIONS:A substantial number of providers did not know about NHLBI's SCD care guidelines. Barriers to providing care for patients with SCD were influenced by providers' specialty, training and practice setting. Increasing provider knowledge could improve hydroxyurea utilisation, pain management and mental health support.

Introduction Sex-based clinical outcome differences in sickle cell disease (SCD) remain largely unknown despite evidence that female sex is associated with an increased lifespan. To better characterize sex-based differences in SCD, we assessed pain, treatment characteristics, laboratory measures and complications among males and females currently enrolled in the Sickle Cell Disease Implementation Consortium (SCDIC) registry. Methods The SCDIC consists of eight comprehensive SCD centers and one data coordinating center that received funding from the National Heart Lung and Blood Institute to improve outcomes for individuals with SCD. Eligibility criteria included: 15 to 45 years of age and a confirmed diagnosis of SCD. Self-report surveys were completed and data were also abstracted from the participants’ medical records. Results A total of 2,124 participants were included (mean age: 27.8 years; 56% female). The majority had hemoglobin SS SCD genotype. Females had worse reports of pain severity (mean (SD) T-score 51.6 (9.6) vs 49.3 (10), p<0.001), more vaso-occlusive episodes (p = 0.01) and a higher occurrence of 3 or more hospital admissions in the past year (30.9% vs. 25.5, p = 0.03). On multivariable analysis, males had higher odds of acute chest syndrome (odds ratio (OR) 1.4, p = 0.002), cardiovascular (OR 1.70, p<0.001) and musculoskeletal (OR 1.33, p = 0.0034) complications and lower odds of depression (OR 0.77, p = 0.0381). Females had higher fetal hemoglobin levels with and without hydroxyurea use (9.6% vs 8.5%, p = 0.03 and 3% vs 2.2%, p = 0.0005, respectively). Conclusion Our data suggests that sex differences in clinical outcomes do occur among individuals with SCD. Future research needs to explore the mechanisms underlying these differences.

Incidence in first‐time and repeat blood donors is an important measure of transfusion‐transmitted HIV infection (TT‐HIV) risk. This study assessed HIV incidence over time at four large blood centres in Brazil.

Background Acquisition of HIV primary drug resistant (PDR) infection can lead to poor virologic and clinical outcomes in individuals and hampers public health efforts in epidemic control. Monitoring PDR in HIV-positive blood donors can be used to inform nationwide trends in the spread of drug-resistant HIV strains. Methods We conducted a cross-sectional study using genetic sequence analysis to assess HIV pol sequences, PDR, and risk factors for infection using audio computer-assisted structured interviews in four large blood centers in Brazil from 2007 to 2017. Results Of 716 HIV-positive blood donors, 504 (70.4%) were successfully sequenced. HIV clade B (73.2%) was the most prevalent subtype, followed by a mix of non-B (21.2%) sub-types. A twofold increase (from 4% to 8%) in recombinants prevalence was observed during the study period. Sixty-four (12.7%) presented PDR. Overall, HIV PDR prevalence remained stable during the study period. Drug resistance mutations for non-nucleoside reverse transcriptase inhibitors were found in 39 (7.7%) donors, while for nucleoside reverse transcriptase inhibitors were found in 26 (5.1%), and for protease inhibitors in 24 (4.8%) of HIV-infected donors. We did not find statistically significant differences in demographics, behavioural risk factors, or HIV genotypes when comparing volunteers with and without PDR. Conclusion The HIV PDR rate among donors remained stable during the study period. HIV-positive blood donors can be an informative population to monitor primary HIV resistance and ultimately may help to increase the knowledge and awareness of HIV risk factors and PDR.

Abstract Introduction Emerging literature suggests body mass index (BMI) may be increasing in individuals with sickle cell disease (SCD), a condition historically associated with underweight status. However, the current prevalence of overweight and obese adults with SCD remains unclear in a country where high BMI is prevalent in the general population. We present an epidemiological analysis of the prevalence of overweight and obese individuals in a large representative sample of adult SCD patients, identifying associations between BMI, sociodemographic and clinical characteristics. Methods Using the Sickle Cell Disease Consortium (SCDIC) registry, we compiled a detailed clinical and demographical from a non-random sample of adults, aged 20-45 years. The SCDIC collects data from eight academic centers providing comprehensive care throughout the U.S. Adult participants were excluded from the analysis if they were under 20 years of age, pregnant at the time of enrollment, or were without medical records or patient enrollment surveys. Sociodemographic information and patient-reported outcomes, including pain frequency and severity, SCD complications, and hydroxyurea use, were collected at the time of enrollment. Non-SCD medical conditions and anthropometric measurements were abstracted from medical records. We stratified BMI, measured in kilograms per meters squared (kg/m2) following CDC criteria: underweight (<18.5 kg/m2), normal weight (18.5-24.9 kg/m2), overweight (25.0-29.9 kg/m2), and obese (>30 kg/m2). An epidemiological analysis was performed of BMI in those with SCD. Bivariate analyses were conducted using Chi-square test, t-test, and ANOVA; non-parametric tests were used when appropriate. Data were analyzed using SAS 9.4 (SAS Institute; Cary, NC). Results In total, 1,664 adults met the inclusion criteria for this cross-sectional analysis of SCDIC registry data. The median BMI for the entire sample was 23.9 kg/m² (IQR: 21.1-28). The majority of participants were African American (99.1%), female (56.6%), and had an HbSS genotype (69.6%). The prevalence of an obese BMI (17.5%) was greater than underweight BMI (6.4%) among the entire cohort (Table 1). When compared to under/normal weight participants, those who were overweight/obese were older (median age 31.2 versus 29.3 years; p<0.0001) and had a higher prevalence of hypertension (45.1% versus 28.3%; p<0.0001). Most participants in the overweight/obese BMI categories had genotype HbSS (59.0%), however, genotype HbSS accounted for 77% of the under/normal weight category. Most participants with an overweight/obese BMI also had some college or vocational training (39.9%) and had Medicare, Medicaid, or military insurance (71.9%). Median BMI did not differ on reported use or non-use of hydroxyurea (23.9 [IQR: 21.1-27.6] vs 23.9 [IQR: 21.0-28.4] p=0.1), mean number of patient-reported SCD complications (2.6 versus 2.6; p=0.6), nor mean pain frequency (49.6 versus 50.6; p=0.06). However, overweight/obese participants reported significantly higher mean pain intensity than their under/normal weight counterparts (51.8 versus 50.8; p=0.03). Conclusion To date, this is the largest analysis of adult BMI among individuals with SCD in the U.S. Among the eight SCDIC sites spanning from the Northeast to Southwest U.S., the prevalence of underweight BMI was less than that of overweight or obese BMI status, challenging previous understandings of weight status and further aligning with the growing rates of overweight and obesity in the general U.S. population. Significant associations between high BMI and hypertension, age, and pain intensity highlight an opportunity for further research to understand the impact of increasing BMI on SCD outcomes and non-SCD comorbidities. Figure 1 Figure 1. Disclosures Ibemere: bluebird bio Insights Council: Consultancy, Honoraria; Ugali Youth: Consultancy, Membership on an entity's Board of Directors or advisory committees. King: Health Resources and Services Administration: Research Funding; National Cancer Institute: Research Funding; Global Blood Therapeutics: Research Funding; National Heart, Lung, and Blood Institute: Research Funding. Hankins: Global Blood Therapeutics: Consultancy; UpToDate: Consultancy; Bluebird Bio: Consultancy; Vindico Medical Education: Consultancy. Tanabe: CSL Behring: Consultancy. Shah: Emmaus: Consultancy; GLG: Consultancy; Alexion: Speakers Bureau; Guidepoint Global: Consultancy; Bluebird Bio: Consultancy; CSL Behring: Consultancy; Novartis: Research Funding, Speakers Bureau; GBT: Consultancy, Research Funding, Speakers Bureau.

STUDY OBJECTIVE:Guided by an implementation science framework, this needs assessment identifies institutional-, provider-, and patient-level barriers to care of sickle cell disease (SCD) in the emergency department (ED) to inform future interventions conducted by the multicenter Sickle Cell Disease Implementation Consortium.METHODS:The consortium developed and implemented a validated needs assessment survey administered to a cross-sectional convenience sample of patients with SCD and ED providers caring for them. In total, 516 adolescents and adults with SCD and 243 ED providers from 7 and 5 regions of the United States, respectively, responded to the ED care delivery for SCD survey.RESULTS:Survey results demonstrated that 84.5% of respondents with SCD have an outpatient provider who treats many patients with SCD. In the ED, 54.3% reported not receiving care fast enough and 46.0% believed physicians did not care about them and believed similarly of nurses (34.9%). Consequently, 48.6% of respondents were "never" or "sometimes" satisfied with their ED care. Of surveyed ED providers, 75.1% were unaware of the National Heart, Lung, and Blood Institute recommendations for vaso-occlusive crises, yet 98.1% were confident in their knowledge about caring for patients with SCD. ED providers identified the following factors as barriers to care administration: opioid epidemic (62.1%), patient behavior (60.9%), crowding (58.0%), concern about addiction (47.3%), and implicit bias (37.0%).CONCLUSION:The results underscore that many patients with SCD are dissatisfied with their ED care and highlight challenges to optimal care on the practice, provider, and patient levels. Exploring these differences may facilitate improvements in ED care.

Introduction Sickle cell disease (SCD) is the most common inherited blood disorders in the United States. The disease predominantly affects African Americans with 1 out of every 365 individuals born with SCD. The disease is characterized by vascular inflammation and vaso-occlusion leading to numerous complications and multi-organ dysfunction. Previous studies have shown women with SCD tend to outlive their male counterparts. Other than the increased life expectancy, sex-based clinical outcome differences in SCD remain largely unknown. To better characterize sex-based differences in SCD, we assessed sociodemographic characteristics, pain, treatment characteristics, laboratory measures and complications among males and females currently enrolled in the Sickle Cell Disease Implementation Consortium (SCDIC) registry. Methods The SCDIC consists of eight academic and comprehensive SCD centers, and one data-coordinating center that received funding from the National Heart Lung and Blood Institute to improve outcomes for individuals with SCD. Participants were eligible for the enrollment in the SCDIC registry if they were 15 to 45 years of age and had a confirmed diagnosis of SCD. Participants were excluded if they had sickle cell trait or had a successful bone marrow transplant. Enrolled participants completed surveys. Data were also abstracted from the participants' medical records. Data were entered into a REDCap database and analyzed using SAS version 9.4 (SAS Institute; Cary, NC). Categorical variables were presented as frequencies and percentages, continuous variables were presented as medians and interquartile ranges (IQR) or means and standard deviations. Categorical variables were analyzed using Chi-Square or Fisher exact tests when appropriate. Continuous variables were compared using the Mann-Whitney U test or independent sample t-tests depending on the distribution. A two-sided p-value less than 0.05 was deemed significant. Results A total of 2,124 participants were included in the study. The mean (SD) age of our participants was 27.8 (7.9) years. Almost all (95.6%) were Africa American, female (56%) and had hemoglobin SS (68.2%) SCD genotype. More males (55.4 % vs. 44.6%, p &lt;0.0001) were taking hydroxyurea. Females had significantly worse reports of pain frequency and severity (p=0.0002 and &lt;0.0001 respectively), more vaso-occlusive episodes (p=0.01) and a higher occurrence of 3 or more hospital admissions in the past year (30.9 % vs. 25.5, p= 0.03). Males had significantly more skin ulcers and respiratory, musculoskeletal, genitourinary and cardiovascular complications while females had more anxiety, depression and autoimmune conditions. Males also had significantly higher creatinine, blood urea nitrogen, albumin and liver enzymes (alkaline phosphatase, aspartate and alanine aminotransferases). Females had higher fetal hemoglobin levels with and without hydroxyurea use. There were no statistical differences in ethnicity, marital and employment status. Conclusion Key differences in SCD presentation and occurrence of complications exist among males and females. Females had higher rates of depression and anxiety while males had more chronic end-organ complications that are life threatening. Our findings emphasize the need for stratification of data analysis by sex in future SCD studies. Disclosures Hankins: Global Blood Therapeutics: Consultancy, Research Funding; National Heart, Lung, and Blood Institute: Honoraria, Research Funding; LINKS Incorporate Foundation: Research Funding; American Society of Pediatric Hematology/Oncology: Honoraria; MJH Life Sciences: Consultancy, Patents & Royalties; UptoDate: Consultancy; Novartis: Research Funding. Treadwell:UpToDate: Honoraria; Global Blood Therapeutics: Consultancy. King:Tioma Therapuetics: Consultancy; WUGEN: Current equity holder in private company; RiverVest: Consultancy; Celgene: Consultancy; Cell Works: Consultancy; Incyte: Consultancy; Magenta Therapeutics: Membership on an entity's Board of Directors or advisory committees; Bioline: Consultancy; Novimmune: Research Funding; Amphivena Therapeutics: Research Funding. Gordeuk:CSL Behring: Consultancy, Research Funding; Global Blood Therapeutics: Consultancy, Research Funding; Imara: Research Funding; Ironwood: Research Funding; Novartis: Consultancy. Kanter:bluebird bio, inc: Consultancy, Honoraria; SCDAA Medical and Research Advisory Board: Membership on an entity's Board of Directors or advisory committees; AGIOS: Membership on an entity's Board of Directors or advisory committees; Novartis: Consultancy; Sanofi: Consultancy; Medscape: Honoraria; Guidepoint Global: Honoraria; GLG: Honoraria; Jeffries: Honoraria; Cowen: Honoraria; Wells Fargo: Honoraria; NHLBI Sickle Cell Advisory Board: Membership on an entity's Board of Directors or advisory committees; BEAM: Membership on an entity's Board of Directors or advisory committees. Glassberg:Eli Lilly and Company: Research Funding; Pfizer: Research Funding; Global Blood Therapeutics: Consultancy. Shah:Bluebird Bio: Consultancy; Novartis: Consultancy, Research Funding, Speakers Bureau; Global Blood Therapeutics: Consultancy, Research Funding, Speakers Bureau; CSL Behring: Consultancy; Alexion: Speakers Bureau.