Search

Objective: To evaluate neurodevelopmental status among children with inherited cholestatic liver diseases with native liver and variables predictive of impairment. Methods: Participants with Alagille syndrome (ALGS), progressive familial intrahepatic cholestasis (PFIC), and alpha 1 antitrypsin deficiency (A1AT) enrolled in a longitudinal, multicenter study and completed the Wechsler Preschool and Primary Scale of Intelligence-III or Intelligence Scale for Children-IV. Full Scale Intelligence Quotient (FSIQ) was analyzed continuously and categorically (>100, 85–99, 70–84, 1 standard deviation [SD] below average) was highest in ALGS (29%) versus 18.6% in PFIC and 12.8% in A1AT, and was greater than expected in ALGS based on normal distribution (29% vs 15.9%, P = 0.003). ALGS scored significantly lower than test norms in almost all Wechsler composites; A1AT scored lower on Working Memory and Processing Speed; PFIC was not different from test norms. Total bilirubin, alkaline phosphatase, albumin, hemoglobin, and parental education were significantly associated with FSIQ. Conclusions: Patients with ALGS are at increased risk of lower FSIQ, whereas our data suggest A1AT and PFIC are not. A1AT and ALGS appear vulnerable to working memory and processing speed deficits suggestive of attention/executive function impairment. Malnutrition, liver disease severity, and sociodemographic factors appear related to FSIQ deficits, potentially identifying targets for early interventions.

Advances in medical therapies and liver transplantation have resulted in a greater number of pediatric patients reaching young adulthood. However, there is an increased risk for medical complications and morbidity surrounding transfer from pediatric to adult hepatology and transplant services. Health care transition (HCT) is the process of moving from a child/family-centered model of care to an adult or patient-centered model of health care. Successful HCT requires a partnership between pediatric and adult providers across all disciplines resulting in a transition process that does not end at the time of transfer but continues throughout early adulthood. Joint consensus guidelines in collaboration with the American Society of Transplantation are presented to facilitate the adoption of a structured, multidisciplinary approach to transition planning utilizing The Six Core Elements of Health Care Transition TM for use by both pediatric and adult specialists. This paper provides guidance and seeks support for the implementation of an HCT program which spans across both pediatric and adult hepatology and transplant centers.

ImportanceDespite high disease morbidity and mortality and complex treatment decisions, little is known about the medical and end-of-life decision-making preferences of adolescents and young adults (AYA) with advanced heart disease. AYA decision-making involvement is associated with important outcomes in other chronic illness groups.ObjectiveTo characterize decision-making preferences of AYAs with advanced heart disease and their parents and determine factors associated with these preferences.Design, Setting, and ParticipantsCross-sectional survey between July 2018 and April 2021 at a single-center heart failure/transplant service at a Midwestern US children’s hospital. Participants were AYAs aged 12 to 24 years with heart failure, listed for heart transplantation, or posttransplant with life-limiting complications and a parent/caregiver. Data were analyzed from May 2021 to June 2022.Main Outcomes and MeasuresSingle-item measure of medical decision-making preferences, MyCHATT, and Lyon Family-Centered Advance Care Planning Survey.ResultsFifty-six of 63 eligible patients enrolled in the study (88.9%) with 53 AYA-parent dyads. Median (IQR) patient age was 17.8 (15.8-19.0) years; 34 (64.2%) patients were male, and 40 patients (75.5%) identified as White and 13 patients (24.5%) identified as members of a racial or ethnic minority group or multiracial. The greatest proportion of AYA participants (24 of 53 participants [45.3%]) indicated a preference for active, patient-led decision-making specific to heart disease management, while the greatest proportion of parents (18 of 51 participants [35.3%]) preferred they and physician(s) make shared medical decisions on behalf of their AYA, representing AYA-parent decision-making discordance (χ2 = 11.7; P = .01). Most AYA participants stated a preference to discuss adverse effects or risks of treatment (46 of 53 participants [86.8%]), procedural and/or surgical details (45 of 53 participants [84.9%]), impact of condition on daily activities (48 of 53 participants [90.6%]), and their prognosis (42 of 53 participants [79.2%]). More than half of AYAs preferred to be involved in end-of-life decisions if very ill (30 of 53 participants [56.6%]). Longer time since cardiac diagnosis (r = 0.32; P = .02) and worse functional status (mean [SD] 4.3 [1.4] in New York Heart Association class III or IV vs 2.8 [1.8] in New York Heart Association class I or II; t-value = 2.7; P = .01) were associated with a preference for more active, patient-led decision-making.Conclusions and RelevanceIn this survey study, most AYAs with advanced heart disease favored active roles in medical decision-making. Interventions and educational efforts targeting clinicians, AYAs with heart disease, and their caregivers are needed to ensure they are meeting the decision-making and communication preferences of this patient population with complex disease and treatment courses.

Objective The COVID-19 pandemic increased economic, social, and health stressors for families, yet its impacts on families of youth with chronic conditions, such as type 1 diabetes (T1D), are not well understood. Self-regulation (SR)—or the capacities to control emotions, cognition, and behavior in response to challenge—is known to support T1D management and coping in the face of stress. Strong SR may have protected youth with T1D from the impacts of pandemic-related stressors. This study compared youth and parent emotional functioning and T1D management before and after the pandemic’s onset in relation to family pandemic-related stress and youth SR. Methods Parents of youth with T1D (N = 88) and a subset of these youth (N = 43; Mean age 15.3 years [SD 2.2]) completed surveys regarding SR, stress, emotional functioning, and T1D-related functioning prior to and after March 2020. Outcomes were compared using mixed effects models adjusting for covariates. Family pandemic-related stress experiences and youth SR were tested as moderators of change. Results Parents’ responsibility for T1D management increased across pandemic onset and their diabetes-related distress decreased. Family pandemic-related stress was associated with decreased emotional functioning over time. Youth SR, particularly emotional and behavioral aspects, predicted better emotional and T1D-related functioning. Discussion While youth with T1D whose families experienced higher pandemic-related stress had poorer adjustment, strong emotional and behavioral SR appeared to protect against worsening youth mood and adherence across pandemic onset. Both social-contextual and individual factors are important to consider when working with families managing T1D.

The Johns Hopkins University School of Medicine organized 2 multistakeholder symposia on February 2, 2018 and January 11, 2019 to address the problem of high graft failure in adolescent and young adult (AYA) solid organ transplant (SOT) recipients. Participants included international experts in transplantation, behavioral psychology, patient/parent advocacy, and technology. The objectives of the symposia were as follows: (1) to identify and discuss the barriers to and facilitators of effective transfer of care for AYA SOT recipients; (2) to actively explore strategies and digital solutions to promote their successful transfer of care; and (3) to develop meaningful partnerships for the successful development, evaluation, implementation, and dissemination of these digital solutions. Additionally, data were collected from 152 AYA SOT recipients demonstrating a substantial gap in how this population uses technologies for health-related activities, alongside an increased interest in an app to help them manage their transplant.

BACKGROUND: Adverse effects of clinician burnout has been studied across multiple specialties, however, there have been no studies examining rates of burnout among pediatric solid organ transplant teams. This study aimed to measure burnout, work exhaustion, professional fulfillment, and post-traumatic stress symptoms among clinicians and administrators practicing in this high stress field. METHODS: This cross-sectional study utilized a 50 item web-based survey that included the Personal Fulfillment Index and the Impact of Events Scale (Revised). This survey was distributed across four pediatric solid organ transplant centers in North America. Basic demographics, clinician characteristics, and information regarding wellness and self-care activities were collected. Descriptive and correlational analyses were performed. RESULTS: 135 participants completed the survey; 76% were female and 78% were Caucasian. One-third (34%) of participants endorsed burnout, while 43% reported professional fulfillment. Approximately 15% of respondents endorsed clinically significant levels of post-traumatic stress symptoms related to patient deaths, with female clinicians more likely to endorse symptoms (p=.01). Nearly 80% of participants reported engaging in self-care activities outside of work and only 10% of participants reported participation in hospital-sponsored wellness programs. CONCLUSIONS: Pediatric solid-organ transplant team members exhibited moderate levels of burnout, professional fulfillment, and post-traumatic stress. Female clinicians were the most likely to experience both work exhaustion and post-traumatic stress symptoms. Transplant centers are encouraged to consider interventions and programming to improve clinician wellness.

PURPOSE OF REVIEW: Youth with strong self-regulation (SR), or the ability to manage thoughts, emotions, and behaviors, engage in more effective type 1 diabetes (T1D) management. However, while parent support and engagement are critical to ensuring positive youth T1D outcomes, it is rarely considered that parents’ SR may also influence youth T1D management. If this is the case, novel interventions to improve parents’ SR or ensure adequate support for parents with SR challenges offer great potential to improve family functioning and youth T1D management. RECENT FINDINGS: Theoretical and preliminary empirical evidence suggests that parental SR impacts family processes that support youth T1D treatment regimen adherence. Further, parent and youth SR likely interact, with high parent SR enhancing the positive effects of high youth SR or compensating for low youth SR. SUMMARY: Continued research is needed to better understand the ways in which parent SR matters to youth T1D management, and identify how to support improvements in T1D management among families of parents with low SR.

Adolescents have the poorest blood sugar control compared to other age groups (Foster et al., 2019). Adolescence is also a period of developing self-regulation (SR) abilities, defined as the ability to manage thoughts, emotions, and actions to achieve a desired outcome (Eisenberg, 2019). The attention, planning, and decision making required for optimal T1D management is both cognitively and emotionally draining on the developing system of adolescents, which may be contributing to difficultly with diabetes management and glycemic control (Duke & Harris, 2014). This project examines the relationship between specific aspects of SR, including executive functions and affect; and T1D outcomes, including diabetes management behaviors and HbA1c. As part of an ongoing clinical trial (NCT03688919), adolescents (13-17 yo) diagnosed with T1D for ≥ 6 months with HbA1c ≥ 7.0 were recruited from diabetes clinics (n = 56). They completed baselines measures of self-regulation including the Behavior Rating Inventory of Executive Functioning, Positive and Negative Affect Schedule for Children, and self-reported diabetes management behaviors. HbA1c was also collected. Parent reported cognitive aspects of SR were negatively related to number of insulin boluses per day, specifically attentional shift, task completion and planning, (r(47)= -.36, -.31, -.33 respectively, all p < .05); however, no association was found between inhibition, self-monitoring, and working memory and diabetes outcomes (p >.05). With regard to emotional aspects of SR, positive affect, including excitement, enthusiasm, pride, determination, and activation (r(47)= .34, .33, .36, .31, .29 respectively, all p < .05) were positively related to number of insulin boluses per day. No aspects of SR were related to blood glucose testing behaviors or HbA1c. SR in adolescents may be an important factor in successful T1D management. With evidence-based approaches, SR may be an optimal target for intervention. Disclosure D. Albright: None. H.O. Riley: None. E. Fredericks: None. J. Sturza: None. S. Lo: None. K.M. Clark: None. J.M. Lee: Advisory Panel; Self; Goodrx. Consultant; Self; T1D Exchange. Research Support; Self; Lenovo. A.L. Miller: None. Funding National Institutes of Health (NCT03688919)

Objective Non-adherence to pediatric regimens is a common concern. Low health literacy is correlated with poor outcomes in adults but is understudied in pediatrics. The current project aimed to determine the relationship between health literacy, adherence, and outcomes in pediatric liver transplant recipients. Hypotheses included a) parent and patient health literacy would be positively correlated; and b) low patient and/or parent health literacy would be negatively correlated with adherence and health outcomes. Patients and methods Eligible participants were recruited during routine follow-up visits in a pediatric liver transplant clinic. Parents and patients (>13 years old) completed 2 measures of health literacy. Patients ≥18 years completed health literacy measures without corresponding parent surveys. Adherence variables and health outcomes were obtained from medical records. Results Seventy-nine patients across two sites completed the study. Variance in classification of health literacy between measures was observed; however, most parents (82%-100%) scored within an "adequate literacy" range. More adolescents scored in lower health literacy ranges relative to the parents. Markers of SES were positively correlated with health literacy. Parent health literacy was negatively associated with biopsy-proven rejection episodes and the number of hospitalizations; however, it was not associated with measures of tacrolimus adherence. There were no relationships observed between parent and adolescent health literacy. Conclusions Health literacy is an important consideration in managing patient care; however, available measures demonstrate variability in capturing the skills of patients. Effective communication strategies may ameliorate admittedly small, but negative, impacts of limited health literacy on outcomes.

Self-regulation (SR), or the ability to manage thoughts, emotions, and behaviours in order to achieve a desired goal, is seen as underlying positive health behaviours. In adults, behaviour change techniques (BCTs) are recommended to promote SR across health domains; although establishing healthy habits early in life is important, studies of SR and health in children are rare. This conceptual review provides guidance on developmental considerations for applying BCTs to enhance SR capacity in children and youth with the goal of fostering positive behavioural health trajectories early in the lifespan. Key considerations include the nature of developmental changes in SR and interaction among SR processes; temporal associations between SR and health behaviours; and relevance of health goals for children and youth. Building on a meta-review of BCT's used to promote SR in adults and youth, this conceptual review highlights key SR milestones to consider in behaviour change-focused interventions from early childhood through adolescence and provides an overview of social-ecologic influences on SR development and associations between SR and health behaviours across these age periods. Implications for and examples of using developmentally-informed BCTs in interventions to enhance SR in children and youth are noted and suggestions for future research are discussed.

OBJECTIVES The aim of the study was to assess neurodevelopmental outcomes among children with biliary atresia (BA) surviving with their native liver at ages 3 to 12 years and evaluate variables that associate with neurodevelopment. METHODS Participants (ages 3-12 years) in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with Weschler Preschool and Primary Scale of Intelligence, 3rd edition (WPPSI-III, ages 3-5 years) and Weschler Intelligence Scale for Children, 4th edition (WISC-IV, ages 6-12 years). Continuous scores were analyzed using Kolmogorov-Smironov tests compared with a normal distribution (mean = 100 ± 15). Effect of covariates on Full-Scale Intelligence Quotient (FSIQ) was analyzed using linear regression. RESULTS Ninety-three participants completed 164 WPPSI-III (mean age 3.9) and 51 WISC-IV (mean age 6.9) tests. WPPSI-III FSIQ (104 ± 14, P

For children with various forms of end-stage organ diseases, solid organ transplantation offers improved long-term survival. Transplantation of the major organs has become routine in many countries around the world, and some of the best outcomes following transplantation are being achieved in pediatric recipients. Solid organ transplantation provides a treatment option for youth with life-threatening and/or life-limiting diseases, allowing for improved physical, emotional, and social functioning. Yet, organ transplant is a treatment and not an ultimate cure. Children who receive a solid organ transplant must implement and sustain a complex medical regimen to increase the likelihood of optimal graft function and long-term survival. Nonadherence to the medical regimen after solid organ transplantation can contribute to poor clinical outcomes, including rejection, graft loss, and mortality. This chapter describes the prevalence of regimen nonadherence among pediatric solid organ transplant recipients and presents an overview of assessment and intervention strategies. Emerging areas of research, including technology-based interventions, are also discussed.

Due to advancements in treatment and survival, solid organ transplantation is a common intervention for pediatric patients with end-stage organ disease. Despite improved medical outcomes overall, pediatric solid organ transplantation is accompanied by risks of morbidity and mortality, along with daily medication and treatment demands. As such, a subset of patients and families experience significant psychological stressors throughout the transplant process, from transplant evaluation and listing to years post surgery. Pediatric transplant psychologists play a critical role in the care of these young people and their families. This chapter provides a review of psychological consultation in pediatric solid organ transplantation and includes both medical and psychosocial overviews, a review of evidence-based assessments and interventions for common referrals, and tips and tools, including handouts and resources, for working in pediatric solid organ transplantation.

Self-regulation, known as the ability to harness cognitive, emotional, and motivational resources to achieve goals, is hypothesized to contribute to health behaviors across the lifespan. Enhancing self-regulation early in life may increase positive health outcomes. During pre-adolescence, children assume increased autonomy in health behaviors (e.g., eating; physical activity), many of which involve self-regulation. This article presents results from a clinical trial (NCT03060863) that used a factorial design to test behavioral interventions designed to enhance self-regulation, specifically targeting executive functioning, emotion regulation, future-oriented thinking, and approach biases. Participants were 118 children (9–12 years of age, M = 10.2 years) who had a history of living in poverty. They were randomized to receive up to four interventions that were delivered via home visits. Self-regulation was assayed using behavioral tasks, observations, interviews, and parent- and child-report surveys. Results were that self-regulation targets were reliably assessed and that interventions were delivered with high fidelity. Intervention effect sizes were very small to moderate (d range = .02–.65, median = .14), and most were not statistically significant. Intercorrelation analyses indicated that associations between measures within each target varied based on the self-regulation target evaluated. Results are discussed with regard to the role of self-regulation-focused interventions in child health promotion. Implications of findings are reviewed for informing next steps in behavioral self-regulation interventions among children from low-income backgrounds. Published version

The overall objective of this paper was to systematically review and synthesize the emerging literature investigating the role of father involvement in pediatric outcomes among chronic illness populations. This review sought to answer the following questions: (1) what measures are used to assess father involvement in pediatric chronic illness populations, and who is the respondent, and (2) how is father involvement associated with child psychosocial and health related outcomes in pediatric chronic illness populations? Databases were searched using a key word search strategy. Articles were screened according to exclusion criteria, resulting in 15 identified articles that included a pediatric illness population, and assessed both father involvement and a child outcome variable. Qualitative analysis revealed that several measures have been used to assess father involvement in pediatric chronic illness populations. As a whole, the majority of findings indicate that better outcomes are associated with more father involvement in illness and non-illness related activities, and higher father-child relationship quality. Contradictory findings may be due to the quality of the involvement being assessed, or the possibility that father's become more involved with illness tasks in response to their child's poorer health outcomes. Future research should include the development and use of psychometrically sound measures of father involvement and employ more diverse samples with rigorous methodology.

The Johns Hopkins University School of Medicine organized 2 multistakeholder symposia on February 2, 2018 and January 11, 2019 to address the problem of high graft failure in adolescent and young adult (AYA) solid organ transplant (SOT) recipients. Participants included international experts in transplantation, behavioral psychology, patient/parent advocacy, and technology. The objectives of the symposia were as follows: (1) to identify and discuss the barriers to and facilitators of effective transfer of care for AYA SOT recipients; (2) to actively explore strategies and digital solutions to promote their successful transfer of care; and (3) to develop meaningful partnerships for the successful development, evaluation, implementation, and dissemination of these digital solutions. Additionally, data were collected from 152 AYA SOT recipients demonstrating a substantial gap in how this population uses technologies for health-related activities, alongside an increased interest in an app to help them manage their transplant.

Background Despite advancements in treatment and survival, pediatric organ failure and transplant populations continue to face significant risks of morbidity and mortality. Little scientific attention has been given to addressing the end-of-life care needs of this growing population of young people. This study characterized current practices, beliefs, and challenges specific to the disclosure of prognosis and end-of-life care topics among providers caring for pediatric organ failure and transplant populations. Methods This cross-sectional study included 144 healthcare providers actively caring for children, adolescents, and young adults with organ failure or solid organ transplant history. Participants completed an electronic survey measuring frequency and comfort in discussing the following topics with patients and parents: prognosis/survival statistics, re-transplantation, advance care planning (ACP), and death/dying. Descriptive statistics, two-sample t tests, and analysis of variance were used. Results Fewer than half of respondents regularly discuss prognosis/survival statistics and potential need for re-transplantation with their pediatric and young adult patients. Less than 20% of providers engage their pediatric patients in ACP discussions, and approximately 30% facilitate such discussions with young adult patients. Pediatric organ failure and transplant providers endorse a number of barriers specific to discussing these topics. Conclusion Pediatric organ failure and transplant providers do not regularly discuss prognosis or end-of-life care topics with this patient population. Communication-focused intervention research is needed to improve honest and compassionate discussion of these topics that is aligned with both patients' and parents' needs and preferences.

Transition readiness assessment is defined as the measurement of indicators that an adolescent or young adult with special health-care needs (AYASHCN) can successfully initiate, continue, and finish the process of transferring from pediatric-centered to adult-centered medical care. Despite national research and clinical initiatives focused on the promotion of transition readiness, there is evidence that routine transition readiness assessment is not in place for many AYASHCN and their families, ultimately posing risks for inadequate health-care utilization, morbidity, and mortality. Challenges to transition readiness assessment include the lack of well-validated measures, inadequate resources and training, and difficulties with care coordination across providers. In this chapter, we define transition readiness using an evidenced-based, social-ecological framework—the Social-ecological Model of AYA Readiness to Transition (SMART)—and provide an updated review of the available transition readiness assessment measures. We describe ways in which emerging transition readiness assessment research may be incorporated into routine clinical practice through quality improvement science. Finally, we discuss limitations of the current literature and provide opportunities for future research, including the need for rigorous validation studies with attention to multiple transition stakeholders, to develop psychometrically robust measures with strong clinical utility.

Background Understanding the impact of effective paediatric adherence promotion interventions on patients, families and the healthcare system is necessary to inform efforts to improve healthcare quality and control costs. Building on previous research suggesting that improving adherence may have far-reaching benefits, the objective of this study was to quantify the impact of effective adherence promotion interventions for children and adolescents with a chronic medical condition on patients, families and the healthcare system. Methods Authors systematically reviewed articles indexed in PubMed, PsycINFO and CINAHL to identify randomized controlled trials of paediatric adherence promotion interventions. Interventions that improved paediatric adherence and examined patient-level, family-level or healthcare system-level outcomes in children and adolescents (M age ≤ 18 years) with a chronic medical condition were included. Two authors independently extracted and classified outcome variables as patient-level (quality of life and disease-related activity restrictions), micro-level (family functioning, family conflict, caregiver quality of life, caregiver sleep interruption, caregiver days away from work and patient missed school days) or macro-level variables (emergency department visits, hospitalizations, outpatient visits and urgent care visits). Outcome variables detailed in previously published reviews (i.e. disease severity) were excluded. Results Twenty studies representing 19 unique samples met inclusion criteria. An additional eight articles representing trials that did not significantly improve adherence were included in post hoc analyses. Compared with control interventions, effective paediatric adherence promotion interventions improved patient quality of life and family-level outcomes and decreased healthcare utilization among children and adolescents with a chronic medical condition. Conclusions Interdisciplinary efforts to improve healthcare quality and reduce spending among children and adolescents with a chronic medical condition may be enhanced by incorporating effective paediatric adherence promotion interventions. As relatively few chronic medical conditions were represented in included studies, future research should examine the impact of paediatric adherence promotion interventions in other populations.

Poor self-regulation (i.e., inability to harness cognitive, emotional, motivational resources to achieve goals) is hypothesized to contribute to unhealthy behaviors across the lifespan. Enhancing early self-regulation may increase positive health outcomes. Obesity is a major public health concern with early-emerging precursors related to self-regulation; it is therefore a good model for understanding self-regulation and health behavior. Preadolescence is a transition when children increase autonomy in health behaviors (e.g., eating, exercise habits), many of which involve self-regulation. This paper presents the scientific rationale for examining self-regulation mechanisms that are hypothesized to relate to health behaviors, specifically obesogenic eating, that have not been examined in children. We describe novel intervention protocols designed to enhance self-regulation skills, specifically executive functioning, emotion regulation, future-oriented thinking, and approach bias. Interventions are delivered via home visits. Assays of self-regulation and obesogenic eating behaviors using behavioral tasks and self-reports are implemented and evaluated to determine feasibility and psychometrics and to test intervention effects. Participants are low-income 9-12 year-old children who have been phenotyped for self-regulation, stress, eating behavior and adiposity through early childhood. Study goals are to examine intervention effects on self-regulation and whether change in self-regulation improves obesogenic eating.

To assess neurodevelopmental outcomes among participants with biliary atresia with their native liver at ages 12 months (group 1) and 24 months (group 2), and to evaluate variables predictive of neurodevelopmental impairment.Participants enrolled in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with either the Bayley Scales of Infant Development, 2nd edition, or Bayley Scales of Infant and Toddler Development, 3rd edition. Scores (normative mean = 100 ± 15) were categorized as ≥100, 85-99, and85 for χThere were 148 children who completed 217 Bayley Scales of Infant and Toddler Development, 3rd edition, examinations (group 1, n = 132; group 2, n = 85). Neurodevelopmental score distributions significantly shifted downward compared with test norms at 1 and 2 years of age. Multivariate analysis identified ascites (OR, 3.17; P = .01) and low length z-scores at time of testing (OR, 0.70; P .04) as risk factors for physical/motor impairment; low weight z-score (OR, 0.57; P = .001) and ascites (OR, 2.89; P = .01) for mental/cognitive/language impairment at 1 year of age. An unsuccessful hepatoportoenterostomy was predictive of both physical/motor (OR, 4.88; P .02) and mental/cognitive/language impairment (OR, 4.76; P = .02) at 2 years of age.Participants with biliary atresia surviving with native livers after hepatoportoenterostomy are at increased risk for neurodevelopmental delays at 12 and 24 months of age. Those with unsuccessful hepatoportoenterostomy are4 times more likely to have neurodevelopmental impairment compared with those with successful hepatoportoenterostomy. Growth delays and/or complications indicating advanced liver disease should alert clinicians to the risk for neurodevelopmental delays, and expedite appropriate interventions.Clinicaltrials.gov: NCT00061828 and NCT00294684.

As survival rates for pediatric solid organ transplantation have continued to improve, researchers and health care providers have increasingly focused on understanding and enhancing the health related quality of life (HRQOL) and psychosocial functioning of their patients. This manuscript reviews the psychosocial functioning of pediatric transplant recipients during the “later years”, defined as more than 3 years post-transplant, and focuses on the day-to-day impact of living with a transplant after the immediate period of adjustment and early years after surgery. Key topics reviewed include health-related quality of life, cognitive functioning, impact on the family, regimen adherence, and transition of responsibility for self-management tasks. Overall, pediatric transplant recipients evidence impairment in HRQOL, neuropsychological outcomes, and family functioning as compared to non-transplant recipients. However, the degree of impairment is influenced by a variety of factors including, disease severity, age, solid organ type, and study methodologies. Studies are limited by small samples, cross-sectional design, and the lack of universal assessment battery to allow for comparisons across solid organ populations. Areas for future research are discussed.

Parents of pediatric liver and kidney transplant recipients were surveyed regarding their current education plans (eg, Individualized Education Program, 504), satisfaction with these plans, and interest in educational support from the psychosocial transplant team. Survey results indicate high rates of IEP and 504 plans, academic and related services, and accommodations among this population. The majority of parents/guardians reported satisfaction with their child's current school plan and did not report need for additional transplant team support specific to school services on the survey measure. However, other information highlights the importance for pediatric transplant teams to consider other ways to support this population's educational needs.

Summary The transition from pediatric transplant care to adult-centered transplant care has received increased attentionbecause more pediatric liver transplant recipients are surviv-ing into adulthood. Routine and standardized assessmentsof transition readiness may help in identifying a high-riskpopulation for interventions both before and after the trans-fer to adult care. There remains a need for the developmentof evidence-based practices to guide the transition processand to identify predictors of successful outcomes. CORRESPONDENCEEmily M. Fredericks, Ph.D., Department of Pediatrics and CommunicableDiseases, University of Michigan, 300 North Ingalls Street, Room 6E07, AnnArbor, MI 48109-5456. E-mail: emfred@med.umich.edu. References 1. Ng VL, Alonso EM, Bucuvalas JC, Cohen G, Limbers CA, Varni JW, et al.; forStudies of Pediatric Liver Transplantation (SPLIT) Research Group. Healthstatus of children alive 10 years after pediatric liver transplantation per-formed in the US and Canada: report of the Studies of Pediatric Liver Trans-plantation experience. J Pediatr 2012;160:820-826.2. LaRosa C, Glah C, Baluarte HJ, Meyers KE. Solid-organ transplantationin childhood: transitioning to adult health care. Pediatrics 2011;127:742-753.3. Kennedy A, Sawyer S. Transition from pediatric to adult services: are we get-ting it right? Curr Opin Pediatr 2008;20:403-409.4. Fredericks EM, Dore-Stites D, Well A, Magee JC, Freed GL, Shieck V, et al.Assessment of transition readiness skills and adherence in pediatric livertransplant recipients. Pediatr Transplant 2010;14:944-953.5. Kieckhefer GM, Trahms CM. Supporting development of children withchronic conditions: from compliance toward shared management. PediatrNurs 2000;26:354-363.6. Annunziato RA, Emre S, Shneider B, Barton C, Dugan CA, Shemesh E.Adherence and medical outcomes in pediatric liver transplant recipients whotransition to adult services. Pediatr Transplant 2007;11:608-614.7. Magee JC, Thomas SE, Fredericks EM, Guidinger MK, Port FK,Kalbfleisch JD, et al. Effect of recipient age and ‘‘transition’’ on graft lossin pediatric transplant recipients [abstract]. Am J Transplant 2006;6(suppl2):214.8. Dobbels F, Van Damme-Lombaert R, Vanhaecke J, De Geest S. Growing pains:non-adherence with the immunosuppressive regimen in adolescent trans-plant recipients. Pediatr Transplant 2005;9:381-390.9. Shemesh E, Shneider BL, Savitzky JK, Arnott L, Gondolesi GE, Krieger NR,et al. Medication adherence in pediatric and adolescent liver transplant recip-ients. Pediatrics 2004;113:825-832.10. Suris JC, Michaud PA, Viner R. The adolescent with a chronic condition. PartI: developmental issues. Arch Dis Child 2004;89:938-942.11. Bell LE, Bartosh SM, Davis CL, Dobbels F, Al-Uzri A, Lotstein D, et al. Adoles-cent transition to adult care in solid organ transplantation: a consensus con-ference report. Am J Transplant 2008;8:2230-2242.

This review focuses on active clinical research in pediatric liver transplantation with special emphasis on areas that could benefit from studies utilizing the SPLIT infrastructure and data repository. Ideas were solicited by members of the SPLIT Research Committee and sections were drafted by members of the committee with expertise in those given areas. This review is intended to highlight priorities for clinical research that could successfully be conducted through the SPLIT collaborative and would have significant impact in pediatric liver transplantation.

Objective To document current clinical practices for medical regimen adherence assessment and intervention in the field of pediatric psychology. Methods 113 members of the Society of Pediatric Psychology completed an anonymous online survey that assessed use of adherence assessments and interventions in clinical practice, barriers and facilitators to their use, and preferred resources for obtaining information on adherence assessments and interventions. Results Respondents reported using a range of adherence assessment and intervention strategies, some of which are evidence-based. Barriers to implementing these clinical strategies included time constraints and lack of familiarity with available clinical tools. Respondents reported that education about effective clinical tools would facilitate their use of adherence assessments and interventions. Conclusions Future research and clinical efforts in adherence should consider developing practical tools for clinical practice, making accessible resources to promote dissemination of these tools, and increase understanding of clinician implementation of adherence assessments and interventions.

The need to prepare pediatric transplant recipients for the transfer to adult-centered transplant care has received increased attention. This study aimed to determine adolescent and young adult LTR and parent perceptions and attitudes about the transition process. LTR and their parents completed a survey assessing level of prior thought and interest in learning about transferring care, knowledge of the transition process, perceived importance of self-management skills, concerns about moving to the adult clinic, and responsibility for health management tasks. Responses were analyzed by age, gender, and time since transplantation. Participants included 46 LTR (mean age = 16.6 yr; range 12-21), and 31 parents. Recipients and parents reported moderate concern about transition, with leaving pediatric providers being a primary worry. LTR ≥16 yr reported greater health care responsibility and increased thought, interest, and knowledge about transition. There were significant differences between parent and LTR perceptions of health care responsibility, indicating that LTR perceive having more independence than what their parents report. Overall, results suggest that adolescent and young adult LTR and their parents perceive the importance of transitional care, but demonstrate poor knowledge of the process. There remains a need for improved transition planning for both adolescents and parents.

To examine transition readiness, adherence, and health out- comes in pediatric liver transplant recipients using a clinically admin- istered screening measure. Seventy-one pediatric liver transplant recipients (11-20 yr) and 58 parents completed a clinic-based TRS measuring perceived and demonstrated self-management skills, AoR for health-related tasks, regimen knowledge, and psychosocial adjustment. Adherence was measured using s.d. of immunosuppressants, proportion of immunosuppressant blood levels out of target range, and clinic attendance. Health outcomes included liver test panels, biopsies, rejec- tion episodes, and hospitalizations. Results indicate that all domains of transition readiness, with the exception of demonstrated skills, and non- adherence were positively correlated with age. Proportion of immuno- suppressant blood levels below target range was positively correlated with self-management skills and increased responsibility for medication tasks. Parent regimen knowledge was associated with clinic attendance. Health outcomes were significantly related to medication non-adher- ence, but not to transition readiness domains. Medication adherence is considered to be a key factor in the transition from pediatric to adult- centered transplant care. Non-adherence is associated with an increased risk for medical complications and is potentially modifiable. Interven- tions to promote self-management skills and adherence should be an essential component of transition planning.

Nonadherence among pediatric transplant recipients is prevalent, with rates as high as 75% among adolescents. These rates are alarming given that adherence behavior has the potential to be modified. This review examines evidence from recent studies and position statements to determine the current issues related to improving medication adherence in adolescent transplant recipients.To date, there are no empirically validated treatments to promote adherence in pediatric transplant recipients. Yet, interventions in other chronic illness populations suggest that combined education and behavioral strategies are the most effective in improving medication adherence. The use of technology, such as the Internet and cellphones, provides a promising mechanism for delivering adherence-promoting interventions to adolescents. Preliminary studies in pediatric solid organ transplant suggest that interventions focused on targeted education and behavioral cues using cellphone text messages are promising strategies for promoting medication adherence.Strategies for promoting adherence in adolescent transplant recipients should include developmentally appropriate models and should incorporate health-related education, motivational strategies, and behavioral skills. Interventions should be tailored to meet individual needs, and should be a collaborative effort between the adolescent, family and healthcare providers.

The measurement properties of the newly developed Pediatric Quality of Life Inventory (PedsQL) 3.0 Transplant Module in pediatric solid organ transplant recipients were evaluated. Participants included pediatric recipients of liver, kidney, heart and small bowel transplantation who were cared for at seven medical centers across the United States and their parents. Three hundred and thirty-eight parents of children ages 2-18 and 274 children ages 5-18 completed both the PedsQL 4.0 Generic Core Scales and the Transplant Module. Findings suggest that child self-report and parent proxy-report scales on the Transplant Module demonstrated excellent reliability (total scale score for child self-report alpha= 0.93; total scale score for parent proxy-report alpha= 0.94). Transplant-specific symptoms or problems were significantly correlated with lower generic HRQOL, supporting construct validity. Children with solid organ transplants and their parents reported statistically significant lower generic HRQOL than healthy children. Parent and child reports showed moderate to good agreement across the scales. In conclusion, the PedsQL Transplant Module demonstrated excellent initial feasibility, reliability and construct validity in pediatric patients with solid organ transplants.

Background: Pediatric admissions for pain have been increasing, and pediatric consultation-liaison (CL) psychologists are frequently consulted to address pain coping for hospitalized youth. Many apps have been developed to target pain coping strategies, but no specific intervention has been developed to target pain coping using mobile health (mHealth) at the bedside. We describe an innovative, pilot, proof of concept mHealth intervention program using clinical apps to target pain coping strategies in youth referred for a pediatric psychology consult during their hospitalization. Methods: Parent-child dyads (n=18, youth ages 7–19) completed measures assessing the child’s pain history, pain catastrophizing, and pain interference during their child’s inpatient admission. Participants were loaned a tablet and prescribed specific clinical apps as part of their psychology consult to target pain coping. Youth tracked the frequency and duration of clinical app use. At about 2 weeks post-discharge families (n=10) completed a satisfaction survey about their experience with the intervention and again completed the pain outcome measures administered at baseline. Results: The clinical apps were used frequently during the hospitalization, primarily for relaxation. Youth who tracked their app use in the hospital were more likely to participate at Time 2. There was significant attrition from enrollment to Time 2. There were no changes in pain level from Time 1 to Time 2. Pain catastrophizing scores for youth decreased significantly at the follow-up interval. Most participants felt that the clinical apps helped with inpatient pain management and 70% continued to use the clinical apps after discharge. Parents universally would recommend the mHealth intervention to other families. Conclusions: The feasibility of this proof of concept intervention is mixed. The intervention was acceptable to our CL team, we had no implementation issues, and there were high levels of acceptability and accessibility for patients. However, attrition from across time points presents a challenge to interpretation of results and highlights the difficulty in implementing such an intervention with a high acuity population. Further research is needed to better describe the impact of clinical apps use on post-discharge outcomes, but this mHealth pilot showed promise as an adjunct to standard inpatient CL interventions for pediatric pain.

Across pediatric organ transplant populations, patient and family psychosocial functioning is associated with important health-related outcomes. Research has suggested that pediatric heart transplant recipients and their families are at increased risk for adverse psychosocial outcomes; however, recent investigation of psychosocial functioning in this population is lacking. This study aimed to provide a contemporary characterization of psychosocial functioning in pediatric heart transplant recipients and their families. Associations between psychosocial function, demographic variables, and transplant-related variables were investigated. Fifty-six parents/guardians of pediatric heart transplant recipients completed a comprehensive psychosocial screening measure during transplant follow-up clinic visits. Descriptive statistics, correlational analyses, and independent samples t tests were performed. Forty percent of pediatric heart transplant recipients and their families endorsed clinically meaningful levels of total psychosocial risk. One-third of patients presented with clinically significant psychological problems per parent report. Psychosocial risk was unassociated with demographic or transplant-related factors. Despite notable improvements in the survival of pediatric heart transplant recipients over the past decade, patients and families present with sustained psychosocial risks well beyond the immediate post-transplant period, necessitating mental health intervention to mitigate adverse impact on health-related outcomes.

Adolescence is a particularly high-risk period for non-adherence with post-transplant medical regimens. There remains a lack of research investigating factors related to non-adherence in adolescent LT recipients. The present study empirically assessed the relationship between adherence and HRQOL in adolescent LT recipients. Participants included 25 adolescents (mean = 15.1 yr, range 12-17.9) and their parent/guardian(s). Adherence was assessed using multiple indices including clinician-conducted interviews, rate of clinic attendance, and s.d. of consecutive tacrolimus blood levels. HRQOL was examined using self-report and parent-proxy report on well-validated assessment measures. Results indicated that 76% of participants were non-adherent on at least one measure of adherence, and HRQOL was significantly lower than normative data for healthy children. Tacrolimus s.d. were significant related to poor HRQOL across domains of physical, school, and social functioning. Non-adherent adolescents reported poorer health perceptions, self-esteem, mental health, family cohesion, and more limitations in social and school activities related to physical, emotional, and behavioral problems. These results suggest that empirically based assessment of HRQOL may help identify those at highest risk for behavior, emotional and school difficulties, as well as non-adherence. The examination of tacrolimus s.d. may also help identify patients who may benefit from intervention to promote adherence and HRQOL. Prospective investigations are necessary to further identify the impact of HRQOL on adherence and long-term health outcomes to further guide clinical intervention.

The transition from pediatric to adult transplant care is a high risk period for non-adherence and poor health outcomes. This article describes a quality improvement initiative integrated into a pediatric liver transplant program that focused on improving outcomes following the transfer from pediatric to adult liver transplant care. Using improvement science methodology, we evaluated the impact of our center’s transition readiness skills (TRS) program by conducting a chart review of 45 pediatric liver transplant recipients who transferred to adult transplant care. Medication adherence, clinic attendance, and health status variables were examined for the year pre-transfer and first year post-transfer. 19 recipients transferred without participating in the TRS program (control group) and 26 recipients participated in the program prior to transferring to the adult clinic (TRS group). The TRS group was significantly older at the time of transfer, more adherent with medications, and more likely to attend their first adult clinic visit compared to the control group. Among the TRS group, better adolescent and parent regimen knowledge were associated with greater adherence to post-transfer clinic appointments. Transition planning should focus on the gradual shift in responsibility for health management tasks, including clinic attendance, from parent to adolescent. There may be support for extending transition support for at least 1 year post-transfer to promote adherence.

Given the increased risk for nonadherence and poor health outcomes in late adolescence, there is a need for better methods to evaluate and improve the transition process as adolescent patients are prepared to be independent adults. This study assessed the psychometrics and concurrent validity of a newly developed measure of the allocation of responsibility for health management in pediatric liver transplant patients. 48 patients and 37 parents completed a 13-item measure of allocation of responsibility. We performed an exploratory principal components analysis on survey results and used component scores to assess the relationship between allocation of responsibility and age, age at transplant, adherence, and health outcomes. Two primary components were identified: Communication with the Health Care System, and Self-Management Tasks. Parent perception of adolescent responsibility for tasks related to communicating with the healthcare system was correlated, in younger patients, with increased nonadherence while responsibility for tasks related to self-management was correlated, in older patients, with decreased nonadherence. These results support allocation of responsibility as a two-domain construct, and they provide targets for monitoring and intervention as adolescent patients advance towards transfer.

Transition may be associated with poor health outcomes, but limited data exist regarding inflammatory bowel disease (IBD). Acquisition of self-management skills is believed to be important to this process. IBD-specific checklists of such skills have been developed to aid in transition, but none has been well studied or validated. This study aimed to describe self-assessment ability to perform tasks on one of these checklists and to explore the relation between patient age and disease duration.Patients ages 10 to 21 years with IBD were recruited. An iPad survey queried the patients for self-assessment of ability to perform specific self-management tasks. Task categories included basic knowledge of IBD, doctor visits, medications and other treatments, and disease management. Associations with age and disease duration were tested with Spearman rank correlation.A total of 67 patients (31 boys) with Crohn disease (n = 40), ulcerative colitis (n = 25), and indeterminate colitis (n = 2) participated in the study. Mean patient age was 15.8 ± 2.5 years, with median disease duration of 5 years (2 months-14 years). The proportion of patients who self-reported ability to complete a task without help increased with age for most tasks, including "telling others my diagnosis" (ρ = 0.43, P = 0.003), "telling medical staff I do not like or am having trouble following a treatment" (ρ = 0.37, P = 0.003), and "naming my medications" (ρ = 0.28, P = 0.02). No task significantly improved with disease duration.Self-assessment of ability to perform some key tasks of transition appears to improve with age, but not with disease duration. More important, communication with the medical team did not improve with age, despite being of critical importance to functioning within an adult care model.

With advances in medical care, youth with chronic illness have the potential for higher quality of life; however, these treatments often come with cost (i.e., burden, financial) that can result in non-adherence. Pediatric non-adherence, on average, is approximately 50% across chronic health conditions. Research has identified effective, evidence-based assessment measures and intervention strategies to promote regimen adherence in youth. Yet, these measures and strategies typically are designed for clinical trials and thus may not be feasible or practical in typical clinic settings. As the field of adherence assessment and intervention expands, it will be important to devise evidence-based tools that are pragmatic and can be translated easily into practice. To guide this future direction, the goals of this paper are to review evidence-based adherence assessment and intervention strategies that can be used with youth and families in clinical practice, to illustrate the complexities of addressing adherence concerns in routine practice, and to discuss the challenges of disseminating and implementing evidence-based strategies in the real world.

The use of methylphenidate (MPH) in the treatment of attention-deficit/hyperactivity disorder (ADHD) is widely accepted; however, there is increased concern regarding its abuse potential. Few studies have examined the reinforcing effects of drugs in individuals receiving them for clinical purposes. This study attempts to assess MPH preference in children with ADHD using a choice procedure in order to explore the relationship among drug preference, clinical efficacy, and abuse potential.Participants were 5 children (10-14 years of age) receiving MPH for the treatment of ADHD. Reinforcing effects were assessed using a double-blind choice procedure, with six sampling sessions and six choice sessions. Participant-rated effects were measured using self-report questionnaires. Clinical effects were measured using direct observations and behavior ratings.Differences between the number of MPH, Placebo, and Neither choices across participants were significant (chi2 = 9.6; p0.01). Three of five participants reliably chose MPH more often than placebo. MPH produced idiosyncratic patterns of participant-rated effects but failed to produce significant clinical effects.These findings add to the literature on the reinforcing effects of MPH and are the first reported in a clinical sample of children. Further research exploring the role of clinical efficacy in MPH preference is warranted.