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BACKGROUND: Sickle cell disease (SCD), an inherited red blood cell disorder, primarily affects African Americans in the United States. Adolescents and young adults with SCD (AYA-SCD) are at risk of high morbidity and mortality when transitioning from pediatric to adult care. The goal of this qualitative study was to understand factors associated with optimal implementation of the AYA-SCD transition. METHODS: Participants were recruited from a large hospital system and the community. Interview guides included topics on access to primary and specialized care, beliefs and practices related to pain control, transition from pediatric to adult care, and patient experiences in the emergency department (ED). Data were coded and analyzed using an inductive thematic coding approach in combination with a deductive coding approach using domains from the Consolidated Framework for Implementation Research (CFIR). RESULTS: Fifty-nine participants, including twenty-one AYA-SCD from both the pediatric and adult clinics, seventeen caregivers, nine pediatric SCD providers, six adult SCD providers, and six ED providers, completed 11 focus groups and 5 semi-structured interviews. Results identified multiple factors within the domains of CFIR including the outer setting, inner setting, individual characteristics, and intervention characteristics. Results were incorporated into a transition framework to inform local practice improvement. CONCLUSION: Our study highlights the importance of multi-level barriers and facilitators for AYA-SCD transition from pediatric to adult care. Future studies could use implementation science frameworks to understand local context and identify strategies and intervention characteristics to improve transition programming. These efforts will ultimately reduce health disparities and ensure health equity.

Background: Children with sickle cell disease (SCD), a genetic blood disorder, are at increased risk of developmental delays but are not routinely referred to Early Intervention (EI). The study aimed to understand the need for and acceptability and feasibility of screening and providing EI services to children with SCD. Methods: Eleven EI partners and eight caregivers of children aged 3 to 5 years with SCD completed semi-structured interviews and short surveys. Results: Three main themes emerged from interviews and surveys: (a) awareness of disparities and moderate caregiver knowledge of child development, (b) high acceptability of screening for EI, and (c) the need for partner education to increase buy-in and feasibility of potential programming. Conclusion: Partners identified a clear need for EI screening and referral for children with SCD and indicated that a future program would be acceptable and feasible. However, education and incentives are needed to encourage providers to change current practice patterns.

AIM To summarize developmental delay among infants and toddlers with sickle cell disease (SCD). METHOD This systematic review included studies that reported developmental outcomes of children with SCD between 0 months and 48 months of age and followed standards set forth by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. RESULTS Ten studies were included, describing 596 unique developmental assessments. The rate of developmental delay ranged from 17.5% to 50% and increased with age. Cognition was the only domain included in all studies and the most frequently identified delay. One study reported that more severe SCD genotypes predicted worse development, while five studies reported no difference in rates of developmental delay across genotypes. INTERPRETATION These findings emphasize the need for standardized screening to identify children with SCD at risk of delay at a young age to facilitate appropriate referrals for therapeutic intervention. Frequent and comprehensive developmental screening is necessary among all SCD genotypes.

Recently, the design of biomimetic enzymes based on ultrathin two-dimension (2D) metal-organic framework (MOF) nanosheets have attracted great attention due to their large surface area and abundant accessible active sites. In this work, ultrathin 2D MOF nanosheets and multi-walled carbon nanotubes (MWCNTs) are simply mixed to prepare the composite (2D MOF/MWCNT) films though a Langmuir–Schafer method. The 2D MOF/MWCNT films are easily transferred on electrodes as a novel electrochemical platform for the detection of hydrogen peroxide (H2O2), with a detection limit of 0.70 μM (S/N = 3), a wide linear range from 0.001 to 8.159 mM, a high sensitivity of 157 μA cm−2 mM−1, a short response time of

Date Presented 04/01/2022 Around half of children with sickle cell disease (SCD) have a developmental delay by the age of 3, but developmental screening and intervention are not part of standard care. Through interviews with caregivers and early intervention providers, we identified three key themes to support successful programing: acceptability, recognition of systemic disparities, and buy-in. Findings will support the implementation of a screening program to enhance the health and well-being of children with SCD. Primary Author and Speaker: Catherine R. Hoyt Contributing Authors: Jeni Erickson, Lingzi Luo, Ashley Housten, Allison King

Background Individuals living with sickle cell disease often require aggressive treatment of pain associated with vaso-occlusive episodes in the emergency department. Frequently, pain relief is poor. The 2014 National Heart, Lung, and Blood Institute evidence-based guidelines recommended an individualized treatment and monitoring protocol to improve pain management of vaso-occlusive episodes. Objective This study will implement an electronic health record–embedded individualized pain plan with provider and patient access in the emergency departments of 8 US academic centers to improve pain treatment for adult patients with sickle cell disease. This study will assess the overall effects of electronic health record–embedded individualized pain plans on improving patient and provider outcomes associated with pain treatment in the emergency department setting and explore barriers and facilitators to the implementation process. Methods A preimplementation and postimplementation study is being conducted by all 8 sites that are members of the National Heart, Lung, and Blood Institute–funded Sickle Cell Disease Implementation Consortium. Adults with sickle cell disease aged 18 to 45 years who had a visit to a participating emergency department for vaso-occlusive episodes within 90 days prior to enrollment will be eligible for inclusion. Patients will be enrolled in the clinic or remotely. The target analytical sample size of this study is 160 patient participants (20 per site) who have had an emergency department visit for vaso-occlusive episode treatment at participating emergency departments during the study period. Each site is expected to enroll approximately 40 participants to reach the analytical sample size. The electronic health record–embedded individualized pain plans will be written by the patient’s sickle cell disease provider, and sites will work with the local informatics team to identify the best method to build the electronic health record–embedded individualized pain plan with patient and provider access. Each site will adopt required patient and provider implementation strategies and can choose to adopt optional strategies to improve the uptake and sustainability of the intervention. The study is informed by the Technology Acceptance Model 2 and the Reach, Effectiveness, Adoption, Implementation, and Maintenance framework. Provider and patient baseline survey, follow-up survey within 96 hours of an emergency department vaso-occlusive episode visit, and selected qualitative interviews within 2 weeks of an emergency department visit will be performed to assess the primary outcome, patient-perceived quality of emergency department pain treatment, and additional implementation and intervention outcomes. Electronic health record data will be used to analyze individualized pain plan adherence and additional secondary outcomes, such as hospital admission and readmission rates. Results The study is currently enrolling study participants. The active implementation period is 18 months. Conclusions This study proposes a structured, framework-informed approach to implement electronic health record–embedded individualized pain plans with both patient and provider access in routine emergency department practice. The results of the study will inform the implementation of electronic health record–embedded individualized pain plans at a larger scale outside of Sickle Cell Disease Implementation Consortium centers. Trial Registration ClinicalTrials.gov NCT04584528; https://clinicaltrials.gov/ct2/show/NCT04584528. International Registered Report Identifier (IRRID) DERR1-10.2196/24818

BACKGROUND Individuals living with sickle cell disease often require aggressive treatment of pain associated with vaso-occlusive episodes in the emergency department. Frequently, pain relief is poor. The 2014 National Heart, Lung, and Blood Institute evidence-based guidelines recommended an individualized treatment and monitoring protocol to improve pain management of vaso-occlusive episodes. OBJECTIVE This study will implement an electronic health record–embedded individualized pain plan with provider and patient access in the emergency departments of 8 US academic centers to improve pain treatment for adult patients with sickle cell disease. This study will assess the overall effects of electronic health record–embedded individualized pain plans on improving patient and provider outcomes associated with pain treatment in the emergency department setting and explore barriers and facilitators to the implementation process. METHODS A preimplementation and postimplementation study is being conducted by all 8 sites that are members of the National Heart, Lung, and Blood Institute–funded Sickle Cell Disease Implementation Consortium. Adults with sickle cell disease aged 18 to 45 years who had a visit to a participating emergency department for vaso-occlusive episodes within 90 days prior to enrollment will be eligible for inclusion. Patients will be enrolled in the clinic or remotely. The target analytical sample size of this study is 160 patient participants (20 per site) who have had an emergency department visit for vaso-occlusive episode treatment at participating emergency departments during the study period. Each site is expected to enroll approximately 40 participants to reach the analytical sample size. The electronic health record–embedded individualized pain plans will be written by the patient’s sickle cell disease provider, and sites will work with the local informatics team to identify the best method to build the electronic health record–embedded individualized pain plan with patient and provider access. Each site will adopt required patient and provider implementation strategies and can choose to adopt optional strategies to improve the uptake and sustainability of the intervention. The study is informed by the Technology Acceptance Model 2 and the Reach, Effectiveness, Adoption, Implementation, and Maintenance framework. Provider and patient baseline survey, follow-up survey within 96 hours of an emergency department vaso-occlusive episode visit, and selected qualitative interviews within 2 weeks of an emergency department visit will be performed to assess the primary outcome, patient-perceived quality of emergency department pain treatment, and additional implementation and intervention outcomes. Electronic health record data will be used to analyze individualized pain plan adherence and additional secondary outcomes, such as hospital admission and readmission rates. RESULTS The study is currently enrolling study participants. The active implementation period is 18 months. CONCLUSIONS This study proposes a structured, framework-informed approach to implement electronic health record–embedded individualized pain plans with both patient and provider access in routine emergency department practice. The results of the study will inform the implementation of electronic health record–embedded individualized pain plans at a larger scale outside of Sickle Cell Disease Implementation Consortium centers. CLINICALTRIAL ClinicalTrials.gov NCT04584528; https://clinicaltrials.gov/ct2/show/NCT04584528. INTERNATIONAL REGISTERED REPORT DERR1-10.2196/24818

Hydroxyurea is an efficacious treatment for sickle cell disease (SCD), but adoption is low among individuals with SCD. The objective of this study was to examine barriers to patients’ adherence to hydroxyurea use regimens by using the intentional and unintentional medication nonadherence framework. We interviewed individuals with SCD age 15 to 49.9 years who were participants in the Sickle Cell Disease Implementation Consortium (SCDIC) Needs Assessment. The intentional and unintentional medication nonadherence framework explains barriers to using hydroxyurea and adds granularity to the understanding of medication adherence barriers unique to the SCD population. In total, 90 semi-structured interviews were completed across 5 of the 8 SCDIC sites. Among interviewed participants, 57.8% (n = 52) were currently taking hydroxyurea, 28.9% (n = 26) were former hydroxyurea users at the time of the interview, and 13.3% (n = 12) had never used hydroxyurea but were familiar with the medication. Using a constructivist grounded theory approach, we discovered important themes that contributed to nonadherence to hydroxyurea, which were categorized under unintentional (eg, Forgetfulness, External Influencers) and intentional (Negative Perceptions of Hydroxyurea, Aversion to Taking Any Medications) nonadherence types. Participants more frequently endorsed adherence barriers that fell into the unintentional nonadherence type (70%) vs intentional nonadherence type (30%). Results from this study will help SCD health care providers understand patient choices and decisions as being either unintentional or intentional, guide tailored clinical discussions regarding hydroxyurea therapy, and develop specific, more nuanced interventions to address nonadherence factors.

Background Sickle cell disease (SCD) is one of the most common genetic conditions worldwide. In the United States it affects over 100,000 people, primarily African Americans, with increasing prevalence. With the widespread implementation of prophylactic penicillin, pneumococcal vaccinations and the use of disease modifying therapies such as Hydroxyurea, survival amongst children with SCD has improved greatly. Nonetheless, the life expectancy for adults with SCD remains 20 years lower than African Americans without SCD. The adolescent and young adult period is well described as time of increased healthcare utilization, high morbidity and mortality for patients with SCD. As a result, increased efforts to understand and improve the transition from pediatric to adult care are ongoing. Previous literature has eluded to the importance of patients' disease specific knowledge in improving this transition. However, there is limited literature that examines knowledge gaps across multiple domains. The goal of this study was to use qualitative methods to understand the role of health knowledge in the successful transition from pediatric to adult care for patients with SCD. Methods Participants were recruited using a purposeful recruitment approach from a large hospital system and local community. Stratified qualitative focus groups with patients (ages 15-25 years with any genotype of SCD) and their parents; and providers who cared for SCD patients were held. Semi-structured interviews with emergency department (ED) providers were also conducted. An interview guide was developed that specifically addressed faciltators and barriers to essential healthcare and educational attainment for young adults with SCD. Using an inductive and emergent coding approach, field notes and transcripts were analyzed for themes/subthemes. A focused sub-analysis looking at health knowledge was then conducted. Transcripts were double-coded by trained coders, discrepancies were resolved by consensus. Results 62 participants (23 patients, 20 caregivers, 20 providers) completed a total of 11 focus groups (8 patient-caregiver dyad focus groups, 3 provider focus groups) and 5 semi-structured interviews with ED providers. Analysis showed knowledge gaps across four major domains: patients, caregivers, educators and in the occupational setting. Patients expressed the desire to know more about treatment options for SCD. They noted specific challenges with self-management as new symptoms of SCD developed in adolescence and disease management increased in complexity. Patients noted that increased disease education and earlier transition planning may be helpful to improve this gap. Caregivers noted challenges with supporting their loved ones during the transition period due to limited knowledge about disease complications and management as they age. Patients and caregivers noted that this was mediated by patient-provider communication. Patients expressed educators' lack of knowledge about SCD as a barrier in the school setting. Participants noted that the implementation of formalized educational plans as well as educating teachers improved their academic experiences. Similarly, participants expressed that the lack of understanding about SCD related disease complications was a barrier to success in the occupational setting. Conclusions Multi-level knowledge gaps exist when examining the transition from pediatric to adult care. This study supports previous literature that highlights the need for patient education to improve disease specific knowledge, health literacy and self-management for young adults transitioning to adult care. This data also suggests that educational interventions across multiple settings may optimize this process. Focused interventions designed to increase SCD specific knowledge amongst caregivers, educators and in the occupational setting represent a feasible and readily implementable point of impact to improve patient-provider communication and overall outcomes in adolescents and young adults with SCD. The successful transition from pediatric to adult care requires a multidisciplinary approach in which the onus is not simply on the patient, but all involved in the care of the patient. Closing knowledge gaps across multiple domains is essential in improving care, quality of life and educational attainment in this vulnerable population. Disclosures No relevant conflicts of interest to declare.