Feasibility of a clinic-based, multicomponent hydroxyurea adherence intervention for adolescents and young adults with sickle cell disease.

Despite the effectiveness of hydroxyurea, adherence remains low for adolescents and young adults (AYA) living with sickle cell disease (SCD). This study evaluated the feasibility, acceptability, and initial efficacy of a clinic-based, multicomponent (e.g., storytelling, problem solving) intervention with 20 AYA living with SCD. Results found that adherence significantly improved from intervention to follow-up 1 [t(19) = -2.213, p = .039]. AYA also were generally satisfied with the intervention. These findings, although promising, should be replicated on a larger scale.

The pervasive influence of systems of power on transition readiness for adult care in sickle cell disease: A qualitative study.

BACKGROUND: Adolescence and young adulthood are vulnerable developmental periods for individuals with sickle cell disease (SCD), particularly given the impact of social inequities, challenges with transitioning to adult healthcare services, and increased risk for morbidity and mortality. Systems of power, such as institutionalized and interpersonal manifestations of bias, could impact SCD transfer and engagement in adult care through their influence on healthcare transition readiness; yet research in this area is limited. OBJECTIVE: To characterize how systems of power impact transition readiness factors described in the Social-ecological Model of AYA Readiness for Transition to Promote Health Equity (SMART-E) framework at the patient, caregiver, and practitioner levels. METHODS: Pediatric adolescents and young adults (AYA), transferred AYA, caregivers, and practitioners participated in semi-structured focus groups and individual interviews examining health equity and systems of power during healthcare transition. Focus groups/interviews were transcribed and coded using a deductive approach via the updated SMART-E framework. RESULTS: Ten pediatric AYA with SCD, nine transferred AYA with SCD, eight caregivers, and nine practitioners participated in a focus group or interview. Qualitative findings across reporters emphasize the impact of systems of power (e.g., racial bias and disease stigma) on knowledge, skills and self-efficacy, beliefs and expectations, goals and motivation, and emotions and psychosocial functioning at the patient, caregiver, and practitioner levels. CONCLUSION: Systems of power are prevalent with respect to transition barriers for AYA with SCD and their supports. Structural, institutional, and individual factors with potential to reduce the influence of systems of power should be further identified and targeted for intervention.

Using the consolidated framework for implementation research to identify recruitment barriers and targeted strategies for a shared decision-making randomized clinical trial in pediatric sickle cell disease.

BACKGROUND/AIMS: Recruitment is often a barrier in clinical trials that include minoritized populations, such as individuals with sickle cell disease. In the United States, the majority of people with sickle cell disease identify as Black or African American. In sickle cell disease, 57% of the United States trials that ended early did so due to low enrollment. Thus, there is a need for interventions that improve trial enrollment in this population. After lower-than-expected recruitment during the first 6 months of the Engaging Parents of Children with Sickle Cell Anemia and their Providers in Shared-Decision-Making for Hydroxyurea trial, a multi-site study for young children with sickle cell disease, we collected data to understand barriers and used the Consolidated Framework for Implementation Research to categorize them and guide the development of targeted strategies. METHODS: Study staff used screening logs and coordinator and principal investigator calls to identify recruitment barriers that were then mapped onto Consolidated Framework for Implementation Research constructs. Targeted strategies were implemented during Months 7-13. Recruitment and enrollment data were summarized before (Months 1-6) and during the implementation period (Months 7-13). RESULTS: During the first 13 months, 60 caregivers (M = 30.65 years; SD = 6.35) enrolled in the trial. Most caregivers primarily self-identified as female (n = 54, 95%) and African American or Black (n = 51, 90%). Recruitment barriers mapped onto three Consolidated Framework for Implementation Research constructs: (1) Process barriers (i.e. no identified "site champion" and poor recruitment planning at several sites); (2) Inner setting barriers (i.e. limited communication, low relative study priority at several sites); and (3) Outer setting barriers (i.e. poor patient attendance at clinic appointments). Targeted strategies to improve recruitment included (1) principal investigator site visits and retraining on recruitment procedures to address process barriers; (2) increased frequency of communication through all coordinator, site principal investigator, and individual site calls to address inner setting barriers; and (3) development and implementation of no-show procedures for clinic appointments to address outer setting barriers. After implementation of the recruitment strategies, the number of caregivers identified for pre-screening increased from 54 to 164, and enrollment more than tripled from 14 to 46 caregiver participants. CONCLUSION: Consolidated Framework for Implementation Research constructs guided the development of targeted strategies that increased enrollment. This reflective process reframes recruitment challenges as the responsibility of the research team rather than characterizing minoritized populations as "difficult" or "hard to reach." Future trials including patients with sickle cell disease and minoritized populations may benefit from this approach.

Diversity, Equity, and Inclusion within Pediatric Adherence Science.

Given the long-standing history of systemic racism in psychological science, diversity, equity, and inclusion (DEI) efforts are increasingly vital to the advancement and improvement of the field. This commentary extends the seminal work of the article Upending Racism in Psychological Science: Strategies to Change How Our Science is Conducted, Reported, Reviewed, and Disseminated (Buchanan et al., Am Psychol, https://doi.org/10.31234/osf.io/6nk4x , 2020) by providing tangible applications and recommendations to improve DEI integration into pediatric adherence science. Real-world adherence examples are discussed regarding the challenges faced in systematically integrating DEI principles, potential solutions to overcoming barriers, and the implications of these efforts on scientific advancement in an effort to address and dismantle research practices that perpetuate inequity and White supremacy. Specifically, we provide discourse and practical guidance related to the conduct, reporting, reviewing, and dissemination of pediatric adherence science to promote dialog and produce actionable change toward the promotion of health equity and social justice.

Family-centered communication in pediatric sickle cell disease.

BACKGROUND: Individuals with sickle cell disease (SCD) experience systemic barriers in accessing high-quality care. Research suggests that patient/family-provider relationships are an important indicator of healthcare quality and can influence disease self-management and outcomes. The Patient Centered Communication (PCC) framework holds that patient/family-centered communication (e.g., eliciting, understanding, and validating patients' perspectives within their unique psychosocial contexts) contributes to improved family-provider relationships, as well as self-efficacy for disease management, adherence, and health outcomes. While the PCC framework has been useful in guiding the evaluation of patient/family-provider communication in other pediatric populations, it has not yet been applied in the context of pediatric SCD. This study aimed to use this framework to examine patient and family perceptions of communication with pediatric SCD healthcare providers. PROCEDURE: Total 17 caregivers (82% mothers, 94% Black/African American) and eight patients (62% female, aged 13-19 years, M = 16.50) completed semi-structured interviews. The PCC framework informed the development of a preliminary codebook. Thematic content analysis summarized family perspectives regarding communication with providers. RESULTS: For youth with SCD and their caregivers, specific themes related to family-centered communication included: reducing patient/family distress, supporting disease self-management efforts, facilitating information exchange and decision-making, and fostering positive and trusting relationships with providers. CONCLUSIONS: This study helps to address gaps in the literature related to patient/family-provider communication within pediatric SCD. Results underscore the importance of patient- and family-centered communication across pediatric SCD care. These findings can inform future research and clinical care initiatives to improve patient/family-provider interactions and health outcomes for this underserved population.

An Immersive Virtual Reality Curriculum for Pediatric Hematology Clinicians on Shared Decision-making for Hydroxyurea in Sickle Cell Anemia.

Although hydroxyurea (HU) is an effective treatment for sickle cell anemia, uptake remains low. Shared decision-making (SDM) is a recommended strategy for HU initiation to elicit family preferences; however, clinicians lack SDM training. We implemented an immersive virtual reality (VR) curriculum at 8 pediatric institutions to train clinicians on SDM that included counseling virtual patients. Clinicians' self-reported confidence significantly improved following the VR simulations on all communication skills assessed, including asking open-ended questions, eliciting specific concerns, and confirming understanding (Ps≤0.01 for all). VR may be an effective method for educating clinicians to engage in SDM for HU.

The influence of perceived racial bias and health-related stigma on quality of life among children with sickle cell disease.

Objectives: Individuals with sickle cell disease (SCD) experience significant health problems that may result in unpredictable pain episodes and frequent healthcare utilization. Disparities in clinical care may contribute to health-related stigma and racial bias for this majority African-American/Black population. There is less known about the influence of health-related stigma and racial bias on the health-related quality of life (HRQOL) of children with SCD. In the present study, we assessed these relationships and identified differences across demographic factors (i.e. age, gender).Design: Data was collected from African American children with SCD aged 8-16 years (57% male, 63% HbSS). Children completed the Childhood Stigma Scale (adapted for SCD), the Child Perceptions of Racism in Children and Youth scale, and the Pediatric Quality of Life Inventory Sickle Cell Disease Module. Caregivers provided demographic information.Results: In the first regression model, health-related stigma (p = .007) predicted HRQOL, but neither age nor gender were significant predictors. In the second regression model, age (p = .03) predicted HRQOL, but neither gender nor racial bias were significant predictors. Of interest, there was a significant interaction between age, gender, and racial bias (p = .02). Specifically, older girls who reported high levels of perceived racial bias had poorer HRQOL.Conclusions: Our study highlights the need for increased awareness about the effects of health-related stigma and racial bias on HRQOL for children with SCD, particularly for older girls who endorse racial bias. Our findings will guide future stigma and bias reduction interventions that may meet the needs of older girls with SCD.

Factors Associated With Participation in Clinical Trials Among Patients With Lupus.

BACKGROUND/OBJECTIVE: Participation rates for clinical trials, including lupus trials, in the United States are low, but are even lower for underrepresented minorities. The impact of underrepresentation in trials can be far-reaching and is problematic because female subjects of color with lupus experience greater morbidity and mortality. As such, the overarching goal of this study was to characterize the factors that influence participation in lupus clinical trials. METHODS: The Lupus and Allied Diseases Association, the Lupus Foundation of America, and the Lupus Research Alliance collected data for their externally led Patient-Focused Drug Development Initiative-for the purpose of understanding and improving the rates of participation in lupus-related clinical trials. Participants completed a 46-question survey (in English or Spanish) electronically or on paper, which was distributed online or at lupus events. Logistic regression was used to test whether demographic and disease characteristics were associated with participation in past lupus trials. RESULTS: Data were available for 2220 respondents. Black respondents with lupus were more likely, than their White and Hispanic counterparts, to have participated in past clinical trials (p < 0.05). Although not statistically significant, Hispanic respondents were also more likely to have participated than their White counterparts (odds ratio, 1.40; 95% confidence interval, 0.96-2.11). Both demographic (ie, race/ethnicity) and medical (ie, disease severity defined as more organ involvement) factors seem to be important determinants of participation in clinical trials (p < 0.05). CONCLUSIONS: Combining the results from this study and prior research provides insight into recruitment strategies to increase participation rates of historically underrepresented minorities.

Pain-Related Injustice Appraisals in Youth with Sickle Cell Disease: A Preliminary Investigation.

OBJECTIVES: Sickle cell disease (SCD) is a genetic disorder that affects approximately 100,000 Americans, the majority of whom are African American. SCD-related pain often has deleterious effects on functioning and quality of life. The inherited nature of SCD, SCD-related stigma, and serious physical and functional impact of SCD-related pain create a situation ripe for individuals to appraise their SCD-related pain as unfair or unjust. The aim of this preliminary investigation is to explore the extent to which pediatric patients with SCD appraise their pain as unjust and how these appraisals relate to functioning. METHODS: Participants were youth with SCD (N = 30, mean age = 11.3, 57% boys) who attended a hematology clinic visit. Patients were invited to complete paper-based questionnaires assessing pain-related injustice appraisals, pain catastrophizing, pain and hurt, functional disability, depression, anxiety, and peer relationships. RESULTS: Results of hierarchical regressions indicate that pain-related injustice significantly predicted functional disability, depression, and anxiety after controlling for patient pain and catastrophizing. CONCLUSIONS: These findings suggest that pain-related injustice appraisals are an important contributor to the pain experience of youth with SCD. Early identification and remediation of pain-related injustice appraisals could have long-term functional benefits for youth with SCD.

Neurodevelopmental and psychosocial interventions for individuals with CHD: a research agenda and recommendations from the Cardiac Neurodevelopmental Outcome Collaborative.

In 2018, the Neurodevelopmental and Psychosocial Interventions Working Group of the Cardiac Neurodevelopmental Outcome Collaborative convened through support from an R13 grant from the National Heart, Lung, and Blood Institute to survey the state of neurodevelopmental and psychosocial intervention research in CHD and to propose a slate of critical questions and investigations required to improve outcomes for this growing population of survivors and their families. Prior research, although limited, suggests that individualised developmental care interventions delivered early in life are beneficial for improving a range of outcomes including feeding, motor and cognitive development, and physiological regulation. Interventions to address self-regulatory, cognitive, and social-emotional challenges have shown promise in other medical populations, yet their applicability and effectiveness for use in individuals with CHD have not been examined. To move this field of research forward, we must strive to better understand the impact of neurodevelopmental and psychosocial intervention within the CHD population including adapting existing interventions for individuals with CHD. We must examine the ways in which dedicated cardiac neurodevelopmental follow-up programmes bolster resilience and support children and families through the myriad transitions inherent to the experience of living with CHD. And, we must ensure that interventions are person-/family-centred, inclusive of individuals from diverse cultural backgrounds as well as those with genetic/medical comorbidities, and proactive in their efforts to include individuals who are at highest risk but who may be traditionally less likely to participate in intervention trials.

Weight status and health behaviors of adolescents and young adults with sickle cell disease: The emerging risk for obesity.

Historically, youth with sickle cell disease (SCD) were at risk for being underweight, but recent data suggests this population is replicating obesity trends of youth in the United States. The current observational study assessed the weight status and health behaviors of 44 adolescents and young adults with SCD via a self-report survey and chart review. Using height and weight data closest to survey completion date, 27% of participants were either overweight or obese. With respect to obesogenic risk behaviors, 77% ate fast food 1-3 times per week, 25% had no fruits/vegetables with any of their meals, 11% drank no water, and 57% watched 4 or more hours of television per day. Though more research is needed, this preliminary study adds to the SCD literature suggesting an emerging shift toward obesity in this population. As such, adolescents with SCD may benefit from interventions to decrease obesity risk factors as being overweight or obese has the potential to worsen SCD-related symptoms and complications.

Improving self-management in adolescents with sickle cell disease.

BACKGROUND: Sickle cell disease (SCD) is associated with significant medical challenges that often worsen in adolescence when caregivers are beginning to transfer responsibility for disease management. Behavioral activation (BA) is an important precedent to improvements in self-management and ultimately health outcomes; however, few interventions targeting BA have been developed for the SCD population. The goal of the present study was to evaluate a technology-enhanced self-management intervention for adolescents and young adults (AYA) with SCD targeting BA domains (ie, disease knowledge, self-efficacy, motivation, and self-management skills). DESIGN/METHODS: Participants were randomized to one of two study arms. SCThrive participants (N = 26) completed six weekly group sessions, an in-person booster session, and used a companion app (iManage) to record symptoms, progress on goals, and connect with other group members. Each SCHealthEd participant (N = 27) received six weekly phone calls on SCD-related and general health education topics. All AYA completed questionnaires assessing BA at baseline and posttreatment. RESULTS: Separate mixed ANOVA analyses to assess for the effects of group (SCThrive/SCHealthEd), time (baseline/posttreatment), and group × time interaction indicated that there was a clinically meaningful improvement (8-point change) in self-efficacy, with a medium effect size, P = .09, η2  = .06, and there was statistically significant improvement in one self-management skill (tracking health), P = .001, d = .71, among SCThrive participants. CONCLUSIONS: The results support the potential for a self-management intervention to improve self-efficacy in AYA with SCD. Health care providers are encouraged to target BA skills to support self-management of AYA with SCD.

Disease Self-Efficacy and Health-Related Quality of Life in Adolescents With Sickle Cell Disease.

Adolescents and young adults (AYA) with sickle cell disease (SCD) are at risk for poor health-related quality of life (HRQOL). Research suggests that vulnerability factors (eg, disease severity) and self-management resources (eg, disease self-efficacy) jointly impact health outcomes, including HRQOL; however, this has not been studied among AYA with SCD. This study examined the relationship between disease self-efficacy, HRQOL, and disease severity in AYA with SCD. HRQOL was positively correlated with disease self-efficacy and negatively correlated with disease severity. Disease self-efficacy and severity accounted for 35% of variance in HRQOL. Findings support the impact of disease self-efficacy on HRQOL.

Commentary: Reflections on the COVID-19 Pandemic and Health Disparities in Pediatric Psychology.

The COVID-19 (2019 novel coronavirus) pandemic has had a significant economic, social, emotional, and public health impact in the United States. A disturbing trend is that Black, Indigenous, and/or People of Color (BIPOC) are disproportionately contracting coronavirus, as well as dying from COVID-19. Objective/Methods The pandemic has the potential to entrench and magnify existing health disparities and families marginalized across multiple demographic intersections such as race/ethnicity, class, immigration status, are especially vulnerable. These inequities have been further underscored by the recent murders of Black Americans by police and a resulting spotlight on racial injustice in the United States. Results Efforts to lessen the spread of the virus, have resulted in changes in pediatric primary and subspecialty service delivery which may affect access for BIPOC communities. BIPOC trainees including those with debt or caregiving responsibilities may be faced with new barriers resulting in delays in completion of their training. Further, clinical, community-based, and translational research has been disrupted by heightened safety precautions and social distancing which may affect BIPOC representation in research downstream. Conclusion In our roles as clinicians, supervisors, trainees, and researchers in primary and subspecialty care as well as in academia, pediatric psychologists have an ethical responsibility to address the disproportionate burden of this pandemic on vulnerable communities and to allocate our time and resources to ensuring health equity now and in the aftermath of COVID-19.

Patient-Reported Outcomes for Pediatric Adherence and Self-Management: A Systematic Review.

OBJECTIVE: Treatment adherence is approximately 50% across pediatric conditions. Patient-reported outcomes (PROs) are the most common method of measuring adherence and self-management across research and clinical contexts. The aim of this systematic review is to evaluate adherence and self-management PROs, including measures of adherence behaviors, adherence barriers, disease management skills, and treatment responsibility. METHODS: Following PRISMA guidelines for systematic reviews, literature searches were performed. Measures meeting inclusion/exclusion criteria were evaluated using Hunsley and Mash's (2018) criteria for evidence-based assessment across several domains (e.g., internal consistency, interrater reliability, test-retest reliability, content validity, construct validity, validity generalization, treatment sensitivity, and clinical utility). Rating categories were adapted for the present study to include the original categories of adequate, good, and excellent, as well as an additional category of below adequate. RESULTS: After screening 172 articles, 50 PROs across a variety of pediatric conditions were reviewed and evaluated. Most measures demonstrated at least adequate content validity (n = 44), internal consistency (n = 34), and validity generalization (n = 45). Findings were mixed regarding interrater reliability, test-retest reliability, and treatment sensitivity. Less than half of the measures (n = 22) exhibited adequate, good, or excellent construct validity. CONCLUSIONS: Although use of adherence and self-management PROs is widespread across several pediatric conditions, few PROs achieved good or excellent ratings based on rigorous psychometric standards. Validation and replication studies with larger, more diverse samples are needed. Future research should consider the use of emerging technologies to enhance the feasibility of broad implementation.

Acceptability and Feasibility of a Disease-specific Patient Portal in Adolescents With Sickle Cell Disease.

OBJECTIVE: To examine the acceptability, feasibility, and efficacy of a health care portal. OBSERVATIONS: Adolescents and young adults with sickle cell disease were taught how to use sickle cell disease conditions page in MyChart and completed questionnaires at baseline, postintervention (T2, 6 wk after baseline) and after 3 months (T3). In total, 44 participants (M age=18.82, SD=2.72) viewed an average of 58.07 pages from T1 to T2. The portal was highly accepted (90.32%). Efficacy data indicated that portal use was associated with improved patient-provider communication. CONCLUSIONS: Electronic portals are promising tools for improving medical self-management.

Development of a Hydroxyurea Decision Aid for Parents of Children With Sickle Cell Anemia.

National evidence-based guidelines recommend offering hydroxyurea to patients with sickle cell anemia 9 months of age and older using shared decision making, but offer no strategies to aid implementation. We developed a hydroxyurea multicomponent decision aid via a needs assessment, clinic observations, and iterative feedback to address parent decision needs and promote a discussion between clinicians and parents. A total of 75 parents and 28 clinicians participated across all phases. The decision aid was rated as useful. Hydroxyurea knowledge improved and decisional conflict decreased supporting the potential for use to facilitate shared decision making in pediatric sickle cell anemia.

Allocation of Treatment Responsibility and Adherence to Hydroxyurea Among Adolescents With Sickle Cell Disease.

OBJECTIVE: Adolescents with sickle cell disease (SCD) are at increased risk for complications. Hydroxyurea is a medication that can ameliorate risk but to benefit, adolescents must adhere to treatment. Study aims were to describe how adolescents and their caregivers decided who was responsible for treatment tasks, to describe adolescents' and caregivers' responsibility for these tasks, and to examine if hydroxyurea adherence was associated with younger adolescent age, less discrepancy between adolescents' and caregivers' reports of adolescent responsibility, and higher caregiver involvement. METHODS: Twenty-nine dyads completed treatment responsibility measures. A combination of laboratory and electronic prescription data were used to determine hydroxyurea adherence and electronic medical records were used to determine appointment adherence. RESULTS: Few dyads agreed or planned how to complete treatment tasks. Adolescents shared responsibility with caregivers for medication-taking tasks. Adolescents perceived caregivers and caregivers perceived adolescents were overall responsible for treatment, especially for appointment tasks. Half of adolescents were adherent to hydroxyurea and half were adherent to appointments but medication adherence was not associated with age, discrepancy between adolescents' and caregivers' responses, or caregiver involvement. CONCLUSIONS: Despite frequent hydroxyurea and appointment nonadherence, few adolescents and caregivers plan how to manage adolescents' SCD treatment or perceive they are overall responsible. Future studies are needed to determine the factors that influence these perceptions and if increasing adolescent and caregiver treatment planning improves adherence and clinical outcomes.

Halo or horn? A qualitative study of mothers' experiences with feeding children during the first year following bariatric surgery.

BACKGROUND: Obesity is a chronic condition that has an intergenerational effect. The aims of the study were to better understand the impact of maternal bariatric surgery on obesogenic risks to child offspring in the home via documenting mothers' thoughts, behaviors, and experiences around child feeding, family meals, and the home food environment during her first year postsurgery. METHOD: Utilizing a mixed-method cross-sectional design, 20 mothers (Mage = 39.6 ±â€¯5.7 years, 75% White, MBMI = 33.6 ±â€¯4.3 kg/m2, Mtime = 7.7 ±â€¯3.1 months post-surgery) of children ages 6-12 years completed validated self-report measures and participated in a focus group. Mother and child heights/weights were measured. RESULTS: The majority of children (N = 20; Mage = 9.2 ±â€¯2.3 years, 65% White, 60% female) were overweight (N = 12; BMI≥85th percentile) and were not meeting the American Academy of Pediatrics healthy eating and activity recommendations to treat/reduce obesity risk. As child zBMI increased, mothers expressed significantly more weight concern (r = 0.59, p = 0.01) and lower obesity-specific quality of life (r = -0.56, p = 0.01), yet assumed less responsibility for child eating choices (r = -0.47, p = 0.04). Qualitative data demonstrated disconnects between mothers' changes to achieve her own healthier weight and applying this knowledge to feeding her child/family. CONCLUSIONS: While bariatric surgery and requisite lifestyle change are effective tools for weight loss at the individual level, there is a great need for innovative family-based solutions. Pediatric obesity is preventable or risk-diminished if addressed early. Maternal bariatric surgery may be a unique (yet missed) opportunity to intervene.