Search

Introduction: Routine comprehensive hematological care is critical for children with sickle cell disease (SCD) to prevent or mitigate complications and ensure optimal quality of life. Estimates of children with SCD with missed appointments or lost to care (no visit >1 year) range from 40-65%. Previously identified barriers to care include obstacles framed at the patient and provider levels including: forgetting appointments, competing demands, logistical challenges, health status, dissatisfaction with patient-provider relationships, and adverse clinic experiences. The Conceptual Framework of Access to Care Model proposes that access and engagement in health care involves a complex interaction of health care/systemic and patient/community factors. The purpose of this study was to apply this model to identify barriers and facilitators to SCD specialty care as part of the Dissemination and Implementation of Stroke Prevention Looking at the Care Environment (DISPLACE) project. Methods: Key informant interviews were conducted with a national sample of patient/caregivers as part of the DISPLACE study. DISPLACE is a large, multi-site study designed to examine stroke prevention practices and identify interventions to improve guideline-informed care. Due to the inclusion criteria for DISPLACE, all participants had sickle cell anemia (SCA) or a child with SCA. Participants were identified using purposive sampling with the goal of data saturation. Interviews included questions pertaining to barriers and facilitators to SCD specialty care, were audio recorded, and transcribed. Qualitative data were analyzed using a deductive-inductive approach with the Conceptual Framework of Access to Care Model as an initial coding framework. Themes were developed according to the theoretical model. Results: Themes and frequencies are in tables 1 and 2. Participants were 26 caregivers of children/adolescents with SCA (mean age = 39; 21 mothers, 4 fathers, 1 aunt) and one young adult (age 18, female). At the health care/systems level, participants reported a range of facilitators to care, with most factors focused on positive qualities of care provided by hematologists, clinic staff, and the overall health care system (appropriateness of care). The ability of the health care system to be available and accommodate patient needs (availability and accommodation) with supports to schedule, remember, and attend the visit in a convenient and timely way were also noted. At the patient/community level, family experiences with care impacted their ability to perceive a health care need and seek services (ability to perceive and seek). In addition, community supports, such as transportation and social support, helped families obtain care (ability to reach). Additional facilitators included insurance and work supports (ability to pay) and family engagement with SCD-specific education (ability to engage). Fewer barriers were reported by participants. At the health care/systems level, barriers included negative health care experiences (appropriateness) and complex health care processes and clinic location (availability and accommodation). At the patient/community level, participants reported difficulties reaching care due to transportation problems or competing demands (ability to reach) and insurance challenges (ability to pay). Conclusions: Health care access and engagement is complex and involves multi-level factors. As illustrated by patient perspectives in this study, a range of facilitators at the health care/systems level can promote access and engagement in care. Notably, participants predominantly focused on qualities of the provider and system that positively engaged them in care. At the patient/community level, clinics can provide support to mitigate complex health care processes and social determinants of health that impact care, such as through family navigators, psychosocial supports, transportation resources, and outreach clinics. Limitations included the focus on caregivers, the pediatric setting, and the lack of participants with other subtypes of SCD. In addition, while we attempted to reach participants with a range of health care experiences, our sample likely reflected families who were more adherent. Future work with families lost to care would contribute additional factors that can be targeted to promote consistent specialty care. Figure 1 Figure 1. Disclosures Adams: Global Blood Therapeutics: Consultancy, Honoraria. Kanter: Fulcrum Therapeutics, Inc.: Consultancy; Novartis: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees; Forma: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees; Agios: Honoraria, Membership on an entity's Board of Directors or advisory committees; Beam: Honoraria, Membership on an entity's Board of Directors or advisory committees; Sanofi: Honoraria, Membership on an entity's Board of Directors or advisory committees; Graphite Bio: Consultancy; GuidePoint Global: Honoraria; Fulcrum Tx: Consultancy.

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 (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 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.

Introduction: Without intervention, children with sickle cell anemia (SCA) have an 11% chance of overt ischemic stroke. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) proved that children with SCA at increased risk of stroke can be identified using transcranial Doppler ultrasound (TCD) and, for those at risk, chronic red cell transfusion (CRCT) therapy is effective at preventing stroke. The 2014 National Heart, Lung, and Blood Institute (NHLBI) guidelines include annual TCD screening for children with SCA ages 2 - 16, and CRCT for primary and secondary ischemic stroke prevention. The NHLBI-funded Dissemination and Implementation of Stroke Prevention Looking at the Care Environment (DISPLACE) project was designed to 1.) evaluate current stroke screening and prevention practices in a 28-site consortium through intensive medical record review and an electronic data capture system, 2.) determine barriers and facilitators to stroke prevention practices through qualitative methods, and 3.) design and deliver interventions to increase rates of 'sickle stroke screening' using implementation strategies. The purpose of this abstract is to describe initial (pre-study) consortium actions following abnormal TCD results including whether all children had a repeat TCD within the next 4 weeks, whether they initiated CRCT, characteristics of which children received CRCT, and what alternate treatments were used. Methods: DISPLACE data collection years were 2012 - 2016. 5247 children with SCA who met criteria for annual TCD screening were entered into the electronic data capture system with clinical and demographic information. 163 children with abnormal TCD results were identified from the cohort database. Additional data were collected and subsequently adjudicated including timing of follow-up testing (repeat TCD or MRI) after abnormal TCD, implementation of CRCT following abnormal TCD, alternate treatment following abnormal TCD, and whether the STOP protocol was implemented. Data were analyzed using descriptive statistics. Results: In response to abnormal TCD, 75% of children had a repeat TCD ordered (Table 1). Of these, most repeat TCDs were completed within 3 months, with Conclusions: In this nationally representative sample of children with SCA at risk for stroke, nearly ¾ of children had a repeat TCDs, suggesting confirmation of abnormal results was common prior to beginning CRCT. Notably, there were a large number of ischemic strokes in children when CRCT was delayed futher confirming the utility of the screening and stroke prevention protocol. An additional key concern identified in this study were the barriers to STOP protocol implementation. Common reasons for lack of implementation included the parent's decision for HU instead of CRCT, delays in CRCT initiation, and delays in obtaining repeat TCDs. Difficulties implementing the STOP protocol suggest the need for interventions for more rapid initiation of CRCT and stroke prevention. Disclosures Kanter: AGIOS: Membership on an entity's Board of Directors or advisory committees; Novartis: Consultancy; Sanofi: Consultancy; bluebird bio, inc: Consultancy, Honoraria; 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; SCDAA Medical and Research 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.

Introduction: The NHLBI funded Dissemination and Implementation of Stroke Prevention Looking at the Care Environment (DISPLACE) study was designed to improve implementation of stroke prevention guidelines in children with sickle cell anemia (SCA), particularly implementation of transcranial Doppler (TCD) ultrasound for identifying individuals at risk of stroke. The study consists of 3 phases: 1) evaluating current stroke risk screening practices, 2) exploring barriers and facilitators to guideline implementation (needs assessment), and 3) designing and implementing interventions to improve stroke risk screening. A key barrier identified through qualitative methods during the needs assessment was a gap in education, including an overall lack of understanding among patients and caregivers of the purpose of TCD screening. This abstract describes the process of developing one of the interventions for phase 3, a rebranding and educational initiative. Methods: During the needs assessment, 27 key informant interviews and 173 complete surveys were conducted with individuals with SCA and their caregivers. Transcripts from the interviews and survey responses were reviewed to better understand the extent of educational gaps described by families as well as to guide initial rebranding prototypes. Prototypes were developed by the study team, including a new name and logo for TCD as well as an infographic. An interview guide was then created to obtain feedback on the prototypes from individuals with SCA and/or the parent or primary caregiver from two sites in the consortium. Cue cards with prototypes were included with prompts for the "think aloud" method to be applied during interviews. Cue cards were presented first with prototypes for the new name in black font on a white background to solicit feedback on the wording alone. Then, cue cards included various layouts, fonts, and graphics with the prototype names for in-depth feedback on the logo appearance. Finally, participants were asked questions pertaining to the infographic. Results: Twenty interviews were conducted with individuals with SCA and/or the parent/caregiver at two DISPLACE sites. Almost all participants (95%) made the connection between the wording prototypes and TCD without prompting. Many participants expressed that the word "stroke" in both options was "scary," and sometimes chose the option that was "less scary to them." However, many participants also felt that the word "stroke" was necessary to explain the reason for the procedure and would prompt families to ask about the screening as opposed to making them more fearful. The majority of participants (60%) chose "Sickle Stroke Screen" over "Stroke Risk Screen." Participants reported preferring this wording because it is specific to SCA, was easier to remember and represented a less "scary" option. The most commonly preferred logo is presented in Figure 1. Participant reasons for selecting this option were: it is easier to read; they preferred the stacked layout; it is less spread out; they liked the bold letters; it is more eye catching; and it includes the words "sickle cell" in the logo. When asked about preferences for an infographic, the majority described including a picture of a brain. Nearly all participants believed a reassuring message was needed to balance out the fear of the word "stroke." The message, "knowledge is power" provided this balance and resonated with nearly all participants (95%). Figure 2 presents the infographic developed based on participant feedback. Conclusions: Results from this educational rebranding effort highlight the importance of understanding patient and family educational gaps and incorporating their perspective and feedback into educational campaigns. The new logo and infographic were integrated into an educational pamphlet, informative posters and other material designed by the DISPLACE site principal investigators. Part 3 of the study is underway including implementation of the educational initiative at the DISPLACE sites. The new terminology and logo have also been broadly distributed throughout the US through community-based organizations to other patients, families, and stakeholders. Disclosures Kanter: AGIOS: Membership on an entity's Board of Directors or advisory committees; NHLBI Sickle Cell Advisory Board: Membership on an entity's Board of Directors or advisory committees; SCDAA Medical and Research Advisory Board: Membership on an entity's Board of Directors or advisory committees; Wells Fargo: Honoraria; Jeffries: Honoraria; Cowen: Honoraria; bluebird bio, inc: Consultancy, Honoraria; Novartis: Consultancy; Sanofi: Consultancy; Medscape: Honoraria; Guidepoint Global: Honoraria; GLG: Honoraria; BEAM: Membership on an entity's Board of Directors or advisory committees.

Background: Stroke is one of the most devastating complications of sickle cell anemia (SCA). In 1998, the Stroke Prevention Trial in Sickle Cell Anemia (STOP), demonstrated that a high-risk group of children with SCA could be identified using Transcranial Doppler ultrasound(TCD) and that chronic red cell transfusion therapy (CRCT) could reduce the risk of first ischemic stroke in this group by over 90% (Adams, et al NEJM 1998).At STOP studyenrollment, 9.7% of children with SCA were identified as having an abnormal TCD. Baby HUG, (NCT00006400), an NHLBI supported phase III trial showed that severe anemia in SCA was associated with elevated white blood cell (WBC) and higher TCD velocities (Lebensburger, et al Blood 2010 ). It is unclear if lower hemoglobin (Hb) and/or higher WBC are causative of elevated TCD velocities or correlative biomarkers. As new disease therapies become available, it is important to know the current rate of abnormal TCD and characteristics of those patients. The DISPLACE (Dissemination and Implementation Looking at the Care Environment) project is a multicenter, NHLBI-funded study whose primary purpose is to identify barriers to implementation of stroke screening in SCA and test novel methods for improving outcomes. DISPLACE is a 3-part study: retrospective assessment of current practice, qualitative review of barriers and facilitators to screening and a cluster-randomized intervention implementation project to improve stroke screening. Part 1 of the study showedthat TCD screening rates varied widely among institutions ranging from 30-75.2% (mean 48.4%, median 47%). We are now reporting on the rate of abnormal TCD and the characteristics and outcomes of patients with abnormal TCD. Methods: DISPLACE is a consortium of 28 US centers. Each site performed a rigorous retrospective chart review of children with SCA aged 2-16 years from 2012-2016. To be eligible for inclusion, children must have been seen at their institution at least 2x during the study period and have confirmation of SCA. A custom electronic data capture (EDC) system facilitated entry of de-identified data including demographics, TCD and MRI results, medications, transfusions, and laboratory values. For children with SCA who had TCD or central nervous system imaging prior to 2012, these results were also entered into the EDC. TCD results were recorded in the EDC as normal, conditional or abnormal based on their institutional interpretation. Labs and vitals were entered for each patient in closest proximity to each TCD. Confirmation and adjudication of each abnormal TCD and associated outcomes were performed. Stroke status was also recorded as well as presence or absence of CRCT. Results: In total, 5247 children with SCA are included in the database of whom 5225 should have received a TCD. Of this cohort, 4210 children (80.6%) had at least one TCD recorded in the database. Within this group, 207 (4.9%) of children had an abnormal TCD and 816 (19.4%) had a conditional TCD. For those children who underwent TCD during the study (2012-2016) period, there were 105 (2.9%) abnormal TCD and 501 (13.6%) conditional TCD (Table 1). The mean age of children at the time of abnormal TCD was 6.6 years (range 2-16 yr). The majority of children were Of the total 5247 patients in the database, 3093 (59%) had been prescribed hydroxyurea (HU) and 999 (19%) were prescribed CRCT. CRCT was prescribed most often for abnormal TCD (37%) or secondary stroke prevention (31%). Discussion: DISPLACE is the largest contemporary cohort of children with SCA. The incidence of abnormal TCD in the DISPLACE cohort is significantly lower than at randomization in the STOP study. The number of children receiving CRCT is higher than expected which may partly account for the decrease in frequency of abnormal TCD. Many patients with abnormal TCD were receiving HU when their TCD was abnormal and were started on CRCT. Additionally, while the outcomes of children with conditional TCD are still being evaluated, many of those children reverted to normal TCD without intervention. These data may also help us redefine the use and interventions needed for abnormal TCD. Disclosures Kanter: NHLBI: Membership on an entity's Board of Directors or advisory committees; bluebird bio, Inc.: Consultancy; SCDAA: Membership on an entity's Board of Directors or advisory committees; Guidepoint Global: Consultancy; GLG: Consultancy; Sangamo: Consultancy, Honoraria; Modus: Consultancy, Honoraria; Novartis: Consultancy, Honoraria; Imara: Consultancy; Cowen: Consultancy; Jeffries: Consultancy; Medscape: Honoraria; Rockpointe: Honoraria; Peerview: Honoraria. Adams:GBT: Consultancy, Other: consultancy to companies GBT and Blueburd Bio; Bluebird: Consultancy.

Introduction: Chronic red cell transfusion therapy (CRCT) is one of the only disease-modifying therapies for individuals with sickle cell anemia (SCA). CRCT plays a particularly critical role in stroke prevention for children with SCA who are at high risk (primary prevention) or who have had a previous stroke (secondary prevention). CRCT is also used to prevent other non-neurologic severe complications from SCA, such as recurrent splenic sequestration or severe acute chest syndrome. Only a few previous studies have focused on patient and provider experiences related to CRCT. This previous work has predominantly focused on patient/caregiver perceptions of children's quality of life while receiving CRCT and provider decision-making around CRCT for SCA. The present study sought to build on this previous work by examining barriers and facilitators to CRCT for stroke prevention as part of the aims of the Dissemination and Implementation Looking at the Care Environment (DISPLACE) study. DISPLACE is a multi-site study funded to evaluate and improve implementation of stroke prevention practices for SCA. The goal of this particular aim was to inform optimal strategies to enhance CRCT initiation for stroke prevention in SCA. Methods: A qualitative descriptive approach was used to obtain provider and patient/caregiver perspectives through key informant interviews with open-ended questions. Purposive sampling was used to identify patient/caregiver participants and purposive and snowball sampling were used to identify provider participants with the goal of enrolling participants until data saturation was reached. Interviews were digitally audio recorded and transcribed for analysis. Data were analyzed using inductive and deductive approaches. Deductive analysis followed the directed content analysis approach with the Multilevel Ecological Model of Health as an initial coding framework. Themes were developed around the barriers and facilitators to CRCT from an ecological perspective. Results: Fifty-two interviews were completed across patients/caregivers and providers (27 patient/caregivers and 25 providers) in order to reach data saturation. For patient/caregivers, 26 caregivers of children and adolescents with SCA (mean age = 39; 21 mothers, 4 fathers, and 1 aunt) and one young adult (age 18, female) completed interviews. For providers, 19 physicians, 2 nurse practitioners, 1 physician assistant, and 3 ancillary providers completed interviews (mean age = 50; 14 female; mean years of practice in SCA = 18.8). Patient/caregiver and provider themes were consistent with the multi-ecological framework and reflected barriers and facilitators at the patient, provider, organizational, and social-environmental/policy levels (Figure 1). Predominant (or high-frequency) themes with definitions and illustrative quotes are presented in Table 1. Predominant barriers reported by patients/caregivers and providers included: logistical challenges (patient level), obtaining and maintaining venous access (patient level), and alloantibodies/alloimmunization and transfusion reactions (patient level). A predominant barrier reported by providers was iron overload and adherence to chelation therapy (patient level). Predominant facilitators reported by patient/caregivers and providers included: nursing and non-nursing staff support (provider level), positive child/family experience (organizational level), and logistical help and social resources (social-environmental and policy level). Predominant facilitators reported by providers included transfusion-specific resources (organizational level) and proximity to a blood bank and access to blood (organizational level). Conclusions: Patient/caregivers and providers reported a range of barriers and facilitators to CRCT, though most occurred at the patient and organizational levels. Many of the barriers and facilitators also involved interactions across ecological levels. Results suggest interventions should address predominant and remediable barriers (e.g., logistical challenges and obtaining and maintaining venous access) and incorporate predominant facilitators (e.g., nursing and non-nursing staff support and positive child/family experience). Findings from this study may be used to guide interventions for improving CRCT initiation among children with SCA. Disclosures Adams: GBT: Consultancy, Other: consultancy to companies GBT and Blueburd Bio; Bluebird: Consultancy.

Introduction: Current stroke prevention guidelines strongly recommend the use of Transcranial Doppler (TCD) screening to detect high risk for ischemic stroke among children with sickle cell anemia (SCA). Despite these guidelines, even recent assessments at sites that participated in the pivotal studies clearly indicate that many children still are not getting screened. DISPLACE (Dissemination and Implementation Looking at the Care Environment) is a multi-center study funded to evaluate and improve optimal implementation of stroke prevention guidelines. Results from Part 1 of DISPLACE (intensive retrospective chart review) determined sub-optimal rates of implementation of TCD screening across sites. To inform effective interventions to improve implementation rates, a multi-level qualitative investigation of barriers and facilitators to obtaining TCD for children with SCA was conducted with patients/caregivers and medical providers. The purpose of this assessment was to identify barriers and facilitators to TCD screening to inform effective interventions in an upcoming phase IV implementation study. Methods: A qualitative descriptive approach was used to obtain provider and patient/caregiver perspectives through key informant interviews with open-ended questions. Purposive sampling was used to identify patient/caregiver participants and purposive and snowball sampling were used to identify provider participants with the goal of enrolling participants until data saturation was reached. Interviews were digitally audio recorded and transcribed for analysis. Inductive and deductive approaches were used to analyze interview data. Deductive analysis followed the directed content analysis approach with the Multilevel Ecological Model of Health as an initial coding framework. Themes were developed around the barriers and facilitators to TCD screening from an ecological perspective. Results: Fifty-two interviews were completed among patients/caregivers (n = 27) and providers (n = 25) to reach data saturation. For patients/caregivers, 26 caregivers of children and adolescents with SCA (mean age = 39; 21 mothers, 4 fathers, and 1 aunt) and one young adult (age 18, female) completed interviews. For providers, 19 physicians, 2 nurse practitioners, 1 physician assistant, and 3 ancillary providers completed interviews (mean age = 50; 14 female; mean years of practice in SCA = 18.8). Patient/caregiver and provider themes were consistent with the multi-ecological framework and reflected barriers and facilitators at the patient, provider, organizational, and social-environmental/policy levels (Figure 1); however, there were some notable differences between patients/caregiver and provider findings. Providers easily identified a much larger range of barriers and facilitators to stroke screening than patients/caregivers whereas the latter group often had to be prompted to consider what might be a barrier or facilitator to TCD screening. Providers, but not patients also reported barriers and facilitators at the social-environmental/policy level. Across patient/caregiver and provider interviews, high frequency or predominant barriers (Table 1) included logistical difficulties and competing life demands (patient level) and gaps in scheduling and coordination (organizational level). High frequency or predominant facilitators included education and information (patient level), provider and staff investment and assistance (provider level), scheduling, coordination, and reminders (organizational level), positive patient experience (organizational level), and convenient location (organizational level). Conclusions: Patient/caregivers and providers reported a wide range of barriers and facilitators to TCD screening for children with SCA, consistent with the ecological framework. Many of these themes involved interactions across multiple levels (e.g., patient-provider, provider-organizational). This analysis suggests that multi-level interventions involving patients, providers, administrators, and policy makers will be instrumental to improving implementation rates of TCD screening. Specific attention will be given to enhanced coordination and provider-specific interventions in the interventional phase IV study of DISPLACE. Disclosures Adams: GBT: Consultancy, Other: consultancy to companies GBT and Blueburd Bio; Bluebird: Consultancy. Kanter:bluebird bio, Inc.: Consultancy; NHLBI: Membership on an entity's Board of Directors or advisory committees; SCDAA: Membership on an entity's Board of Directors or advisory committees; Peerview: Honoraria; Rockpointe: Honoraria; Medscape: Honoraria; Jeffries: Consultancy; Novartis: Consultancy, Honoraria; Imara: Consultancy; Sangamo: Consultancy, Honoraria; Modus: Consultancy, Honoraria; Guidepoint Global: Consultancy; GLG: Consultancy; Cowen: Consultancy.

Introduction: Stroke is one of the most devastating complications of sickle cell anemia (SCA). The STOP (Stroke Prevention Trial in Sickle Cell Anemia) protocol has been adopted by National Heart Lung and Blood Institute (NHLBI) as the guideline for stroke screening using transcranial Doppler ultrasound (TCD) and prevention with chronic red cell transfusion therapy (CRCT). Evidence from the STOP I/II studies indicates the protocol is highly effective, yet wide scale implementation has not been achieved. The DISPLACE (Dissemination and Implementation Looking at the Care Environment) project is a multicenter, NHLBI funded consortium of 28 sites across the United States whose purpose is to identify barriers to the implementation of the STOP protocol and test novel methods for overcoming barriers. The purpose of this study was to use data on practice patterns to evaluate current measurement and practice standards at DISPLACE consortia sites. This abstract presents reported TCD screening and CRCT practices. Methods: A practice patterns survey was sent to the principal investigator (PI) for each DISPLACE site via RedCap ©. PIs were hematology/oncology specialty care providers for children with SCA. Sites represent urban and rural regions, large and small academic institutions, and community-based institutions. The survey was developed by the study investigators; questions were predominantly in a multiple-choice format. Items pertaining to TCD screening included: screening technique (including type of TCD); screening frequency; follow-up for abnormal, conditional, and inadequate results; standard value ranges. Results: All 28 PIs completed the survey. About half of the respondents were female (53.5%). Most identified as White (77.8%), followed by Asian (11.1%) and Black or African American (7.4%). Two identified as Hispanic or Latino (7.4%). Slightly more sites reported using standard TCD (57.1%) versus imaging TCD (TCDi) (42.9%). To calculate the time-averaged mean of the maximum (TAMM) velocities and characterize TCD results, nearly all sites use the middle cerebral artery (96.4%); a majority also use the anterior cerebral artery and/or the terminal internal cerebral artery or distal internal cerebral artery (71.4%). Fewer sites use the posterior cerebral artery (35.7%) or the basilar artery (14.3%). In 7.1% of sites, the radiologist determines which vessels to use during the exam. TCD screening is ordered for children with SCA annually in 92.9% of sites and every 6 months in 7.1% of sites. When TCD screening indicates abnormal TAMM velocities, 85.7% of sites initiate CRCT, 7.1% initiate hydroxyurea (HU) therapy, and 3.6% initiate both HU and CRCT. For additional evaluation, an MRI/MRA is obtained at 64.3% of sites. For high conditional results, the most common action is to initiate HU (67.9%). Other responses include obtaining an MRI/MRA (46.4%) and/or repeating the TCD in 2-4 weeks (25.0%), 6-8 weeks (35.7%), or 12-16 weeks (7.1%). Results deemed inadequate led to repeating the TCD in 2-12 weeks (57.1%) or in one year (3.6%), obtaining an MRI/MRA (57.1%), beginning HU therapy (7.1%), or no repeat TCD or change in management (3.6%). Actions for low conditional results include repeating the TCD (71.4%), obtaining an MRI/MRA (32.3%), and/or initiating HU therapy (57.1%). Surprisingly, sites use different TAMM ranges to characterize the normal/abnormal findings (Table 1). Conclusions: Nearly all DISPLACE sites order TCD screening annually, as recommended in the guidelines, with some ordering screening more frequently. A few sites did not report initiation of CRCT per STOP protocol for abnormal TCD results; however, over half of the sites reported following up with an MRI/MRA, which may suggest evaluating for vasculopathy prior to CRCT. Some sites reported beginning HU therapy for abnormal results; this may reflect consideration of patients for whom CRCT is not possible, but data were not collected for confirmation. Interestingly, results suggest a reliance on MRI/MRA since sites commonly reported ordering neuroradiology studies for abnormal, conditional, and inadequate TCD results. This may suggest an unclear pathway for children with borderline TCD results, and an area for future study. While reported value ranges closely approximated those in the STOP protocol, results indicate sites conducting screening with TCDi may use more conservative values than the validated protocol. Disclosures Phillips: NHLBI: Research Funding. Mueller:NHLBI: Research Funding. Schlenz:NHLBI: Research Funding. Melvin:NHLBI: Research Funding. Adams:NHLBI: Research Funding. Kanter:AstraZeneca: Membership on an entity's Board of Directors or advisory committees; bluebird bio: Membership on an entity's Board of Directors or advisory committees, Research Funding; Global Blood Therapeutics: Research Funding; Novartis: Membership on an entity's Board of Directors or advisory committees, Research Funding; Sancilio: Research Funding; NHLBI: Membership on an entity's Board of Directors or advisory committees, Research Funding; Pfizer: Research Funding; Apopharma: Research Funding; ASH: Membership on an entity's Board of Directors or advisory committees.