INTRODUCTION:National guidelines for the acute management of sickle cell disease vaso-occlusive episodes recommend the use of a patient-specific or a weight-based protocol. The authors compared patient satisfaction with pain management between those randomized to receive either a patient-specific or weight-based pain protocol in the COMPARE-VOE randomized control trial. METHODS:Participants with sickle cell disease were pre-enrolled and patient satisfaction with pain management was assessed at the time of discharge from the 6 participating emergency departments. Patients were randomized to receive a patient-specific or weight-based pain protocol. The authors compared continuous variables between the patient-specific and weight-based protocols with the 2-sample t test and categorical variables by the chi-square test. RESULTS:The authors enrolled 104 participants. Compared with satisfaction with pain management on previous ED visits, more participants in the patient-specific protocol group than the weight-based group (57.1% vs 31.8%; P = .02) were satisfied with pain management. Most who were discharged home (91.2%) felt their pain was sufficiently relieved to be discharged home. DISCUSSION:These findings support evidence-based guidelines to manage vaso-occlusive episodes in emergency departments. Patient-specific protocols can be implemented by partnering with local sickle cell disease providers to make protocols available in the emergency department.

Sickle cell disease (SCD) is a life-limiting multisystem disease primarily affecting individuals of African and Latinx descent. Its most common complication is painful vaso-occlusive episodes (VOEs), which is also the most common reason individuals with SCD seek care in the emergency department (ED). National guidelines recommend the use of standardized approaches to pain management in the ED, preferably using pain management plans tailored to each patient. However, no standard approach to developing these plans exists. This article describes the development of an opioid calculator to help SCD clinicians create individualized plans to better manage acute painful VOE in the ED setting.

People with sickle cell disease (SCD) often have emergency department (ED) revisits. The characteristics of people with SCD with ED revisits were assessed in this study using Medicaid administrative claims data from California and Georgia, representing 2794 and 3641 individuals with SCD, respectively. In both states, those with 6+ primary care provider (PCP) encounters had the highest percentage of ED revisits. In California, those with 6+ hematology encounters had the lowest percentage of individuals with an ED revisit; in Georgia, those with 1-2 hematology encounters. Increasing access to hematologic care may reduce ED revisits among people with SCD.

INTRODUCTION:Sickle cell disease (SCD) is the most common genetic disorder among children. As the most common type of SCD, sickle cell anemia (SCA) is associated with severe complications across the lifespan. As parents/caregivers hold primary disease management responsibility during childhood, their involvement in disease management activities, including medical decision-making, is critical to successful and timely management of pediatric SCD. However, the processes through which caregivers make SCD-related decisions remain unknown. The current paper examined caregivers' decision-making processes and priorities when managing their child's SCD. METHODS:Parents and primary caregivers (N = 27) of children with SCA (ages 0-12) completed individual semi-structured qualitative interviews exploring links between caregivers' decision-making and both daily and ongoing SCA management practices. Data were transcribed verbatim, cleaned, systematically coded, and analyzed using applied thematic analysis. RESULTS:Participating caregivers were primarily Black or African American (88.9%), mothers (81.5%), publicly insured (55.6%), and single (51.9%). Caregivers described medical decision-making across acute symptom response, preventive disease management, and treatment initiation and/or discontinuation. Across these contexts of disease management, caregivers overarchingly prioritized protecting and improving their child's quality of life. Caregivers' medical decision-making processes were influenced by their SCA management experience, acquisition of SCA knowledge, and trust in medical providers. The extent to which these influences impacted caregivers' decision-making varied based on disease severity, disease management experience, and time since diagnosis. DISCUSSION:Findings highlight how processes underlying caregivers' decision-making are directly influenced and informed by caregivers' lived experiences. Future work should develop provider-initiated collaborative interventions to support medical decision-making.

We aimed to determine the minimal clinically important difference (MCID) in pain severity and agreement between the visual analog scale (VAS) and the verbal numeric rating scale (NRS) in people with sickle cell disease experiencing an acute vaso-occlusive episode in the emergency department (ED). In the COMPARE-VOE trial (NCT03933397), participants were administered the VAS (0-100), NRS (0-100), and descriptor scale (a lot better, a little better, same, a little worse, much worse) every 30 minutes while in the ED. We analyzed data from 100 participants (mean age 30.2 years; 61% female). We calculated the mean differences and 95% confidence intervals (CI) between current and preceding scores when the participant reported a little worse or a little better pain for each scale (255 VAS and 150 NRS observations) to assess the MCID for the VAS & NRS. Pearson correlation and the Bland Altmann method were used to assess the agreement among 411 paired VAS & NRS observations. Our results indicated that the MCID for the VAS was 8.77 mm (95% CI: 7.43 mm, 10.83 mm) and the NRS was 8.29 (95% CI: 6.47, 11.60). The VAS & NRS scales had a correlation of 0.88 (p < 0.001). The Bland Altmann indicated a mean difference of -4.6 ±1.96 and the 95% limits of agreement ranged from 20 to -29. Despite high correlation, there was considerable variability of agreement between the VAS and NRS scales, indicating that these scales are not interchangeable to assess pain during a vaso-occlusive event. Perspective The average MCID in pain severity for individuals with sickle cell disease experiencing a VOE using the VAS (8.77 mm) is lower than previously reported, and the MCID for NRS was (8.29). We assessed the agreement between the VAS and NRS and determined that the scales cannot be used interchangeably to measure SCD pain intensity.

BACKGROUND:Vaso-occlusive crises (VOCs) cause debilitating pain and are a common cause of emergency department (ED) visits, for people with sickle cell disease (SCD). Strategies for achieving optimal pain control vary widely despite evidence-based guidelines. We tested existing guidelines and hypothesized that a patient-specific pain protocol (PSP) written by their SCD provider may be more effective than weight-based (WB) dosing of parenteral opiate medication, in relieving pain.METHODS:This study was a prospective, randomized controlled trial comparing a PSP versus WB protocol for patients presenting with VOCs to six EDs. Patients were randomized to a PSP or WB protocol prior to an ED visit. The SCD provider wrote their protocol and placed it in the electronic health record for future ED visits with VOC exclusion criteria that included preexisting PSP excluding parenteral opioid analgesia or outpatient use of buprenorphine or methadone or highly suspected for COVID-19. Pain intensity scores, side effects, and safety were obtained every 30 min for up to 6 h post-ED bed placement. The primary outcome was change in pain intensity score from placement in an ED space to disposition or 6 h.RESULTS:A total of 328 subjects were randomized; 104 participants enrolled (ED visit, target n = 230) with complete data for 96 visits. The study was unable to reach the target sample size and stopped early due to the impact of COVID-19. We found no significant differences between groups in the primary outcome; patients randomized to a PSP had a shorter ED length of stay (p = 0.008), and the prevalence of side effects was low in both groups. Subjects in both groups experienced both a clinically meaningful and a statistically significant decrease in pain (27 mm on a 0- to 100-mm scale).CONCLUSIONS:We found a shorter ED length of stay for patients assigned to a PSP. Patients in both groups experienced good pain relief without significant side effects.

BACKGROUND:Children with sickle cell anemia (SCA) have substantial medical needs and more unmet basic needs than children with other medical conditions. Despite a recent focus on social determinants of health (SDoH), there remains an incomplete understanding of the processes linking SDoH and disease management, particularly for youth with SCA. This study elucidated these processes and identified ways to mitigate deleterious effects of adverse SDoH on SCA management. METHODS:Parents/primary caregivers (N = 27) of children with SCA (≤12 years old) participated in semi-structured interviews regarding SCA management and SDoH and completed quantitative measures of basic needs. Qualitative data were systematically coded and analyzed using applied thematic analysis. Quantitative data were presented descriptively. RESULTS:Three qualitative themes were identified. First, SCA management is bidirectionally linked with the social environment, whereby challenges of SCA management can hinder basic needs from being met, and unmet basic needs and financial hardship hinder SCA management. Second, due to limited resources, parents/caregivers are faced with difficult choices between prioritizing basic needs versus SCA management. Third, addressing material, emotional, and informational needs may improve SCA management. Quantitatively, 73% of families endorsed ≥1 basic need, including food insecurity (42%), housing instability (62%), and/or energy insecurity 19% (vs. 20%). CONCLUSION:Despite documented associations, there remains a poor understanding of the processes linking SDoH and health. Findings underscore how day-to-day conditions undermine the management of SCA treatments, symptoms, and complications, limiting treatment effectiveness. Understanding these processes may inform family-centered, health equity interventions and policies to improve living conditions, disease management, and health outcomes.

For Black children with sickle cell disease (SCD) and their families, high disease stigmatization and pervasive racism increase susceptibility to discrimination in healthcare settings. Childhood experiences of discrimination can result in medical nonadherence, mistrust of healthcare providers, and poorer health outcomes across the lifespan. Caregivers and medical providers are essential to childhood SCD management and are therefore well-positioned to provide insight into discrimination in the context of pediatric SCD. This mixed-methods study sought caregivers’ and providers’ perspectives on processes underlying discrimination and potential solutions to mitigate the negative effects of perceived discrimination among children with SCD. Caregivers (N = 27) of children with SCD (≤ 12 years old) and providers from their hematology clinics (N = 11) participated in individual semi-structured interviews exploring experiences of discrimination and daily SCD management and completed a quantitative measure of discrimination. Qualitative data were collected until themes reached saturation and subsequently transcribed verbatim, coded, and analyzed using applied thematic analysis. Quantitative and qualitative data converged to suggest the pervasiveness of discrimination in healthcare settings. Three qualitative themes emerged: (1) healthcare system factors underlie discrimination, (2) families’ challenging interactions with providers lead to perceptions of discrimination, and (3) experiences of discrimination impact caregiver-provider interactions. Both caregivers and providers highlighted building trusting patient-provider relationships and encouraging patients’ self-advocacy as means to reduce experiences and impacts of discrimination. These findings offer potential approaches to tangibly mitigate occurrences of discrimination in pediatric healthcare settings by trust building, accountability keeping, and fostering rapport to improve quality of care and pediatric SCD health outcomes.

<h3>Importance</h3> Sickle cell disease (SCD) is an inherited disorder of hemoglobin, characterized by formation of long chains of hemoglobin when deoxygenated within capillary beds, resulting in sickle-shaped red blood cells, progressive multiorgan damage, and increased mortality. An estimated 300 000 infants are born annually worldwide with SCD. Most individuals with SCD live in sub-Saharan Africa, India, the Mediterranean, and Middle East; approximately 100 000 individuals with SCD live in the US. <h3>Observations</h3> SCD is diagnosed through newborn screening programs, where available, or when patients present with unexplained severe atraumatic pain or normocytic anemia. In SCD, sickling and hemolysis of red blood cells result in vaso-occlusion with associated ischemia. SCD is characterized by repeated episodes of severe acute pain and acute chest syndrome, and by other complications including stroke, chronic pain, nephropathy, retinopathy, avascular necrosis, priapism, and leg ulcers. In the US, nearly all children with SCD survive to adulthood, but average life expectancy remains 20 years less than the general population, with higher mortality as individuals transition from pediatric to adult-focused health care systems. Until 2017, hydroxyurea, which increases fetal hemoglobin and reduces red blood cell sickling, was the only disease-modifying therapy available for SCD and remains first-line therapy for most individuals with SCD. Three additional therapies, L-glutamine, crizanlizumab, and voxelotor, have been approved as adjunctive or second-line agents. In clinical trials, L-glutamine reduced hospitalization rates by 33% and mean length of stay from 11 to 7 days compared with placebo. Crizanlizumab reduced pain crises from 2.98 to 1.63 per year compared with placebo. Voxelotor increased hemoglobin by at least 1 g/dL, significantly more than placebo (51% vs 7%). Hematopoietic stem cell transplant is the only curative therapy, but it is limited by donor availability, with best results seen in children with a matched sibling donor. While SCD is characterized by acute and chronic pain, patients are not more likely to develop addiction to pain medications than the general population. <h3>Conclusions and Relevance</h3> In the US, approximately 100 000 people have SCD, which is characterized by hemolytic anemia, acute and chronic pain, acute chest syndrome; increased incidence of stroke, nephropathy, and retinopathy; and a life span that is 20 years shorter than the general population. While hydroxyurea is first-line therapy for SCD, L-glutamine, crizanlizumab, and voxelotor have been approved in the US since 2017 as adjunctive or second-line treatments, and hematopoietic stem cell transplant with a matched sibling donor is now standard care for severe disease.

Introduction: Sickle cell anemia (SCA) is the most common form of sickle cell disease and is associated with more severe complications in children. Disease management activities including adherence to medication regimens and health-promoting behaviors improve quality of life and minimize serious complications for children with SCA (e.g., stroke, premature morbidity or mortality). Parents’ and primary caregivers’ involvement in medical decision making is also critical to successful and timely management of pediatric SCA, as parents/caregivers hold primary disease-management responsibility during formative childhood years. Available research documents parental involvement in medical decision-making as integral to cultivating children's long term self-efficacy in SCA disease management, yet the processes through which caregivers make SCA-related decisions remain unknown. As such, the current study examined caregivers’ decision-making processes and related priorities when managing their child's SCA. Methods: Parents and primary caregivers (N=28), hereafter referred to as caregivers, of children with SCA (ages 0-12 years old) were recruited from four outpatient pediatric hematology clinics in New England. The exploratory nature of this research and focus on uncovering processes was best suited to a qualitative approach. As such, participants completed individual semi-structured qualitative interviews exploring links between caregivers’ decision-making and both daily and ongoing disease management practices of their child's SCA. Data were transcribed verbatim, cleaned, systematically coded, and analyzed using applied thematic analysis. Data collection continued until themes reached saturation. Results: Participating caregivers were primarily mothers (84%), single (56%), publicly insured (60%) and identified as Black or African American (88%). Decision-making processes were driven by caregivers’ SCA knowledge, their disease management experience, and the child's disease severity. Caregivers described their decision-making processes across three contexts: acute symptom management (e.g., hospitalizations, at-home management of pain crises), preventative disease management (e.g., adhering to medication regimens, being mindful of extreme temperatures) and treatment initiation or discontinuation (e.g., agreeing to chronic transfusions or starting hydroxyurea). Across all three contexts, caregivers described themselves as the primary decision-maker but noted conferring with their child's healthcare providers, their partners, and other family members. The well-being of the child with SCA was prioritized in decision-making processes, as caregivers repeatedly reported making decisions based on their child's SCA severity level (e.g., having recurring severe symptoms) and not wanting to worsen the child's condition. Consequently, the prioritization of the child with SCA's well-being often superseded household needs (e.g., prioritizing child's medication over paying other bills). Caregivers reported relying on their SCA disease-knowledge and trust, or lack thereof, in their child's SCA providers when making SCA-related decisions. Further, the weight placed on SCA-disease knowledge and trust in providers often depended on the child's age and the caregiver's experience to date with managing the child's SCA needs. Caregivers reported that decision making became easier with more SCA knowledge and/or experience and with time, as did their trust in providers. Discussion: Understanding how caregivers make SCA-related decisions is critical to identifying specific areas for development of targeted interventions and support. In this study, we found that caregivers’ decision making is often driven by their SCA disease knowledge, disease management experience and overall prioritized desire to protect and improve their child's quality of life. Findings of this research should inform the development of various interventions (e.g., pamphlets, web-based platforms) to determine best approaches for providers to implement collaborative decision-making supports throughout childhood for children with SCA.

OBJECTIVEOur aim was to develop an efficient search strategy for prognostic studies and clinical prediction guides (CPGs), optimally balancing sensitivity and precision while independent of MeSH terms, as relying on them may miss the most current literature.MATERIALS AND METHODSWe combined 2 Hedges-based search strategies, modified to remove MeSH terms for overall prognostic studies and CPGs, and ran the search on 269 journals. We read abstracts from a random subset of retrieved references until ≥ 20 per journal were reviewed and classified them as positive when fulfilling standardized quality criteria, thereby assembling a standard dataset used to calibrate the search strategy. We determined performance characteristics of our new search strategy against the Hedges standard and performance characteristics of published search strategies against the standard dataset.RESULTSOur search strategy retrieved 16 089 references from 269 journals during our study period. One hundred fifty-four journals yielded ≥ 20 references and ≥ 1 prognostic study or CPG. Against the Hedges standard, the new search strategy had sensitivity/specificity/precision/accuracy of 84%/80%/2%/80%, respectively. Existing published strategies tested against our standard dataset had sensitivities of 36%-94% and precision of 5%-10%.DISCUSSIONWe developed a new search strategy to identify overall prognosis studies and CPGs independent of MeSH terms. These studies are important for medical decision-making, as they identify specific populations and individuals who may benefit from interventions.CONCLUSIONOur results may benefit literature surveillance and clinical guideline efforts, as our search strategy performs as well as published search strategies while capturing literature at the time of publication.

Objectives: Contraception use reduces teen pregnancy, and long-acting reversible contraception is recommended as first-line treatment. Since many adolescents use the emergency department (ED) as a primary source of health care, it is a potential site of contraceptive counseling and provision. We surveyed female adolescents to assess desire for contraceptive counseling and initiation/change during an ED visit. Materials and Methods: This was a cross sectional study of a convenience sample of female ED patients aged 16-21 years in an urban pediatric ED. Participants completed an anonymous questionnaire about sexual health, contraceptive use, and interest in contraceptive counseling. The primary outcome was adolescent interest in starting/changing contraception during an ED visit. Results: Three hundred eighty-one patients (mean age 19.1 ± 1.6 years) completed the survey. Most (80.5%) had been sexually active with a male partner, and 110 (28.2%) had previously been pregnant. Two-thirds were interested in discussing contraception and 22.5% were likely to start or change contraception during the ED visit. Those who wanted to start or change contraception were more likely to be sexually active with a male partner (93% vs. 82%, p = 0.02) and to report that they were not satisfied with their current contraception (44% vs. 21%, p = 0.0003). Fifteen (17%) of the adolescents likely to start or change contraception were interested in progestin implant initiation in the ED. Conclusions: Adolescents were interested in initiating or changing contraception during the ED visit, providing an important opportunity to discuss and initiate effective contraception.

OBJECTIVES:Coordinated measurement strategies are needed to inform collaborative approaches to improve access to and quality of care for persons with sickle cell disease (SCD). The objective of our study was to develop a multilevel measurement strategy to assess improvements in access to and quality of care for persons with SCD in 4 US regions.METHODS:From 2014 through 2017, regional grantees in the Sickle Cell Disease Treatment Demonstration Program collected administrative and patient-level electronic health record (EHR) data to assess quality improvement initiatives. Four grantees-covering 29 US states and territories and an SCD population of 56 720-used a collective impact model to organize their work. The grantees collected administrative data from state Medicaid and Medicaid managed care organizations (MCOs) at multiple points during 2014-2017 to assess improvements at the population level, and local patient-level data were abstracted from site-level EHRs at regular intervals to track improvements over time.RESULTS:Administrative data were an important source of understanding population-level improvements but were delayed, whereas patient-level data were more sensitive to small-scale quality improvements.CONCLUSIONS:We established a shared measurement approach in partnership with Medicaid and Medicaid MCO stakeholders that can be leveraged to effectively support quality improvement initiatives for persons with SCD in the United States.

OBJECTIVES:Painful vaso-occlusive episodes (VOE) are the most common reason for emergency department (ED) visits experienced by patients with sickle cell disease (SCD). The National Heart, Lung and Blood Institute (NHLBI) evidence-based recommendations for VOE treatment are based primarily on expert opinion. In this randomized controlled trial (RCT), we will compare changes in pain scores between patients randomized to a patient-specific analgesic protocol versus those randomized to a weight-based analgesic protocol, as recommended by the NHLBI guidelines. METHODS:We report the rationale and design of a multi-site, phase III, single-blinded, RCT to be conducted in six EDs in the United States. Eligible participants will be randomized after providing consent, anticipating 50% of those randomized would have an ED visit during the enrollment period. A total of 230 participants with one VOE ED visit provides sufficient power to detect a clinically significant difference in pain score reductions of 14 between groups with 0.05 type I error. Uniquely, this trial randomizes participants in a larger population than the study population, given the impossibility of consenting and randomizing participants during emergencies. The primary endpoint is the change in pain scores in the ED from time of placement in treatment area to time of disposition (hospitalization, discharged home, or assigned to observation status) or a maximum treatment duration of 6 hours. Additional outcomes include hospitalizations and ED visits seven days post enrollment, side effects, and safety assessments. CONCLUSIONS:The COMPARE-VOE study design will provide high-level evidence to support the NHLBI VOE treatment guidelines.

Sickle cell disease (SCD) is a devastating disease that affects approximately 100,000 individuals in the United States, most commonly blacks.1Hassell K.L. Population estimates of sickle cell disease in the US.Am J Prev Med. 2010; 38: S512-S521Abstract Full Text Full Text PDF PubMed Scopus (751) Google Scholar,2Brousseau D.C. Panepinto J. Nimmer M. et al.The number of people with sickle-cell disease in the United States: national and state estimates.Am J Hematol. 2010; 85: 77-78Crossref PubMed Scopus (252) Google Scholar Individuals with SCD often experience multiple life-threatening complications during their lifetimes.3Kato G.J. Piel F.B. Reid C.D. et al.Sickle cell disease.Nat Rev Dis Primers. 2018; 4: 18010Crossref PubMed Scopus (270) Google Scholar Given the progressive nature of SCD, individuals increasingly rely on emergency department (ED) care as they age.4Paulukonis S.T. Feuchtbaum L.B. Coates T.D. et al.Emergency department utilization by Californians with sickle cell disease, 2005-2014.Pediatr Blood Cancer. 2017; 64e26390Crossref Scopus (33) Google Scholar The most common reason for ED visits is acute painful vaso-occlusive episodes, which require treatment with parenteral opioids; however, individuals with SCD may develop other serious complications, including acute chest syndrome and stroke.3Kato G.J. Piel F.B. Reid C.D. et al.Sickle cell disease.Nat Rev Dis Primers. 2018; 4: 18010Crossref PubMed Scopus (270) Google Scholar In 2014, the National Heart, Lung, and Blood Institute published guidelines for the management of SCD, including rapid and aggressive treatment of vaso-occlusive episodes,5Yawn B.P. Buchanan G.R. Afenyi-Annan A.N. et al.Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members.JAMA. 2014; 312: 1033-1048Crossref PubMed Scopus (911) Google Scholar but these recommendations have not been widely adopted.6Masese R.V. Bulgin D. Douglas C. et al.Barriers and facilitators to care for individuals with sickle cell disease in central North Carolina: the emergency department providers' perspective.PLoS One. 2019; 14: e0216414Crossref PubMed Google Scholar Unfortunately, patients often relate poor experiences in the ED, many spending hours in waiting rooms before being assessed and adequately treated.7Tanabe P. Myers R. Zosel A. et al.Emergency department management of acute pain episodes in sickle cell disease.Acad Emerg Med. 2007; 14: 419-425Crossref PubMed Google Scholar,8Zempsky W.T. Treatment of sickle cell pain: fostering trust and justice.JAMA. 2009; 302: 2479-2480Crossref PubMed Scopus (57) Google Scholar Considering the multiple challenges faced by both individuals with SCD and clinicians, a summit on ED care for SCD was held in 2015 and led to the formation of a national coalition to improve the emergency care, the Emergency Department Sickle Cell Care Coalition (EDSC3). EDSC3 is composed of stakeholder groups from emergency medicine, pediatrics, hematology, federal agencies, and patient advocacy, including individuals with SCD, and receives staff support from the American College of Emergency Physicians (ACEP) (Figure 1). The purpose of EDSC3 is to provide a national forum dedicated to disseminating evidence-based emergency care best practices and optimizing patient-family-provider communication in the ED setting. The overarching goals of EDSC3 are to bring awareness and recognition to the national challenges surrounding SCD care in the ED, as well as to work collaboratively across specialties to drive improvement. To date, EDSC3 has organized its activities around 4 key areas: research, education, advocacy, and community outreach. Members of the coalition conducted surveys in 2017 to 2018 through the Emergency Medicine Practice Research Network, which is organized by ACEP and surveys approximately 1,000 practicing emergency physicians every 3 to 6 months. The survey evaluated physician attitudes toward patients with SCD and attitudes toward those with acute chronic pancreatitis to serve as a comparison. Publication of results is expected shortly. EDSC3 members have been actively educating emergency providers about the 2014 National Heart, Lung, and Blood Institute guidelines and innovative approaches to SCD management, such as the use of intranasal fentanyl as the first dose for children with SCD presenting to the ED with severe acute vaso-occlusive episodes.9Kavanagh P.L. Sprinz P.G. Wolfgang T.L. et al.Improving the management of vaso-occlusive episodes in the pediatric emergency department.Pediatrics. 2015; 136: e1016-e1023Crossref PubMed Scopus (42) Google Scholar,10Akinsola B, Hagbom R, Zmitrovich A, et al. Impact of intranasal fentanyl in nurse-initiated protocols for sickle cell vaso-occlusive pain episodes in the pediatric emergency department. Am J Hematol. 2018; https://doi.org/10.1002/ajh.25144.Google Scholar To date, in-person presentations, often with patient copresenters, have been given to emergency physicians and others at multiple state, regional, and national meetings. The coalition has also contacted the broader emergency medicine community by recording podcasts (eg, ACEP Frontline); participating in the Webinar "Sickle Cell Disease Care in the Emergency Department: Improvement Initiatives and Ongoing Research" at the request of the Office of Minority Health, US Department of Health and Human Services; and serving as content experts on a Twitter chat (#SCDintheED) with the American Society of Health-System Pharmacists. Currently, EDSC3 is collaborating with the American Society of Hematology to develop a guideline-based point-of-care tool for ED providers to disseminate guidelines and best practices to manage SCD-related complications. Many of these efforts were supported by grant funding secured by ACEP; efforts continue to procure additional funds to increase the reach of these activities. EDSC3 has lent its support to a number of initiatives, including the Sickle Cell Disease and Other Heritable Blood Disorders Research, Surveillance, Prevention, and Treatment Act of 2018 (S.2465), which was signed into law on December 18, 2018. Additionally, the coalition also partnered with ACEP, the American Society of Hematology, and the Sickle Cell Disease Association of America in sending a letter to the leaders of the US Department of Health and Human Services and the Centers for Disease Control and Prevention in June 2019. The letter highlighted the critical and ongoing challenges that individuals with SCD continue to experience when trying to access opioids to manage their acute and chronic pain, and asked them to "promote cautious, thoughtful consideration in order to avoid unintended consequences for patients with chronic diseases, such as SCD." Finally, the coalition fully supported the Emergency Nurses Association resolution "Management of Vaso-occlusive Episodes in Persons With Sickle Cell Disease in the Emergency Department," which passed at their national General Assembly in 2019. It calls on the Emergency Nurses Association to disseminate the National Heart, Lung, and Blood Institute recommendations for the treatment of vaso-occlusive episodes and investigate the need for further educational materials. These efforts highlight the engagement of EDSC3 with key federal and organizational stakeholders to promote improvement in SCD care in the ED. EDSC3 hosted national summits on SCD ED care in 2018 and 2019 to bring together coalition members and leadership from key government, emergency medicine, and patient advocacy groups to find ways to collaborate on improving SCD care. The goals of both summits were 2-fold: develop specific activities and objectives within each organization's scope of influence, directed at improving the delivery of ED care for patients living with SCD; and collaborate with other organizations to identify and commit to concrete steps for improving care coordination and communication among providers across care settings who are caring for individuals with SCD. These summits have fostered relationships between organizations and led to ongoing activities to improve SCD care, including the development and dissemination of patient videos on SCD pain by the National Institute on Drug Abuse and the aforementioned educational activities. The work of EDSC3 has just begun. Given the nature of SCD, individuals with the disease will continue to require ED care until improved alternate care models or definitive treatments are realized and widely accessible. Therefore, EDSC3 will continue to collaboratively develop and disseminate educational offerings on SCD for all providers working in the ED setting, including physicians, nurse practitioners, physician assistants, nurses, and pharmacists, and work closely with patient advocacy groups to fully embrace the patient voice into the national conversation. EDSC3 is committed to this work until all ED clinicians are proficient in delivering guideline-based care for individuals living with SCD, and patients receive the consistent, compassionate, and timely care they deserve in all EDs.

Brousseau, David C. MD, MS*,†; Muslu, Cagla S. BS†; Visotcky, Alexis MS‡; Fraser, Raphael PhD‡; Kavanagh, Patricia L. MD§; Chelius, Thomas H. MS∥; Schuh, Abigail M. MD, MMHPE*,†Author Information

•Adolescents with sickle cell disease (SCD) are at risk of poor outcomes.•Better self-management skills may improve transition outcomes.•Problem solving strategies are promising as self-management support.•Input from teens with SCD and their parents is important for intervention development.