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.

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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. 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 21 AYA-SCD from both the pediatric and adult clinics, 17 caregivers, 9 pediatric SCD providers, 6 adult SCD providers, and 6 emergency department providers, completed 11 focus groups and 5 semistructured 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 multilevel 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 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

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.

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BACKGROUND Background: The implementation of COVID-19 lockdown measures had immediate and delayed psychological effects. From March 27 to June 1, 2022, the Shanghai government enforced a city-wide lockdown that affected 25 million residents. During this period, mental health services were predominantly provided through digital platforms. However, limited knowledge exists regarding the general population's intention to use mental health services during this time. OBJECTIVE Objective: This study aimed to assess the intention of Shanghai residents to use mental health services during the 2022 Shanghai lockdown and identify factors associated with the intention to utilize mobile mental health services. METHODS Methods: An online survey was distributed from April 29 to June 1, 2022, using a purposive sampling approach across 16 districts in Shanghai. Eligible participants were adults over 18 years of age who were physically present in Shanghai during the lockdown. Multivariable logistic regression was used to estimate the associations between demographic factors, lockdown-related stressors and experiences, physical and mental health status, and study outcomes –mobile mental health service use intention (mobile applications and/or WeChat Mini Programs). RESULTS Results: The analytical sample comprised 3230 respondents, among whom 29.7% (weighted percentage, 1030) screened positive for depression and/or anxiety based on the nine-item Patient Health Questionnaire or the seven-item Generalized Anxiety Disorder Scale. Less than one fourth of the respondents (24.4%, 914) expressed an intention to use any form of mental health services, with mobile mental health service being the most considered option (19.3%, 728). Only 10.9% (440) used digital mental health services during the lockdown. Factors associated with increased odds of mobile mental health service use intention included being female, being employed, being a permeant resident, experiencing COVID-related stressors (such as loss of income, food insecurity, and traumatic experiences), and having social and financial support. Individuals with moderate or severe anxiety, as well as those with comorbid anxiety and depression, demonstrated a higher intention to use mobile mental health services. However, individuals with depression alone did not exhibit a significantly higher intention compared to those without common mental disorders. CONCLUSIONS Conclusions: Despite a high prevalence of common mental disorders among Shanghai residents, less than one-fourth of the study respondents expressed an intention to use any form of mental health services during the lockdown. Mobile applications and/or WeChat Mini Programs were the most considered mental health service formats. The study provided insights for developing more person-centered mobile mental health services to meet the diverse needs of different populations. CLINICALTRIAL N/A

Background The adolescent and young adult (AYA) period is a time of high morbidity and mortality for persons with sickle cell disease (SCD). The goal of this study was to use implementation science combined with qualitative methods to understand the determinants of an optimal transition to adult care for AYA with SCD. Methods Sixty participants were purposefully recruited from a large hospital system and the community for 16 focus groups or semi-structured interviews. Patient-caregiver dyads and health care providers shared experiences in the health care and academic settings. Data were coded and analyzed by paired coders using an inductive coding approach in combination with an implementation research framework, the Consolidated Framework for Implementation Research (CFIR). Results At the Individual Characteristics level, AYA patients expressed challenges with adult life experiences, disease self-management, and preparation for the adult health care model. Caregivers and health care providers reported independence and autonomy as facilitators of a successful transition. At the Inner and Outer Setting levels, differences between pediatric and adult health care models were noted barriers, highlighting the gap in patientcentered care. Patient and provider communication was identified as both a barrier and facilitator. A standardized approach to transition planning and coordination was noted as a facilitator. At the Intervention Characteristics level, adaptability, incentive, and perceived attractiveness of the intervention were important components. Conclusion This study highlights which determinants of transition are most amenable to evidence-based interventions. This is imperative for rapidly improving this process for AYA with SCD and, thus, improving overall outcomes.