The Adolescent Transplant Recipient: An Overview of Neurocognitive Functioning and Implications for Long-Term Outcomes.

BACKGROUND: Solid organ transplantation (SOT) offers improved long-term survival for youth with end-stage organ disease. From a neurodevelopmental, cognitive, and academic perspective, children with solid organ transplant have a number of unique risk factors. While cognitive functioning may improve post-transplantation, it is important to understand the trajectory of neurocognitive development starting in transplant candidacy to evaluate the implications of early deficits. AIM: The aim of this paper is to describe the neurocognitive risks and long-term implications for adolescent transplant recipients. METHOD: This paper provides an overview of neurocognitive functioning in youth with end-stage organ dysfunction with discussion of implications for adolescent transplant recipients. RESULTS: Post-transplant, adolescent, and young adult solid organ transplant recipients exhibit significant levels of executive dysfunction, with implications for decision-making, regimen adherence, and transition to adult transplant care. CONCLUSION: Transplantation may reduce the risk for poor long-term neurocognitive effects, yet adolescent transplant recipients remain at increased risk, particularly in executive functioning, which has implications for adherence and transition to adulthood. Baseline and follow-up assessments for youth with end-stage organ disease and transplant are important for the monitoring of neurocognitive development and may be used to mitigate risk for low adherence to post-transplantation treatment regimens and reduce barriers to transitioning to adult transplant care.

Changes over time in self-efficacy and the allocation of responsibility for health management tasks in pediatric liver transplant recipients: Targets to improve the transition process.

BACKGROUND: The process of transition to adult-based care encompasses a critical period in the life of an adolescent and young adult living with a chronic illness and one that comes with an increase in the risk of poor health outcomes. As yet, there is a dearth of empirical data to help optimize this process to ensure the best long-term outcome. METHODS: This study used a principal components analysis to determine specific constructs measured by a revised version of the transition readiness survey used in our clinic. We investigated changes in these constructs over time. We further investigated the relationship between the change in these constructs over time spent in a focused transition program with adherence. RESULTS: The primary component underlying our transition readiness survey for patients and parents represented self-efficacy. Time spent in the transition program was an independent predictor of change in self-efficacy (rho 0.299, p = .015); however, the magnitude of that change had no relationship to adherence. Change in parent-proxy self-efficacy was found to have a statistically significant relationship with tacrolimus standard deviation (rho -0.301, p = .026). There was disagreement identified between patient and parent responses on the survey. Neither change in patient nor parent reports of self-efficacy was found to have a relationship with post-transfer adherence. CONCLUSIONS: This study reaches the novel conclusion that self-efficacy and parent-proxy self-efficacy are dynamic concepts that change over time spent in a focused transition program. The patient-parent disagreement and the relationship between parent-proxy self-efficacy and adherence stress the importance of involving parents/guardians in the transition process as well.

Health Care Transition for Adolescents and Young Adults With Pediatric-Onset Liver Disease and Transplantation: A Position Paper by the North American Society of Pediatric Gastroenterology, Hepatology, and Nutrition.

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.

Use of Behavior Change Techniques and Quality of Commercially Available Inflammatory Bowel Disease Apps.

BACKGROUND: Inclusion of evidence-based behavior change techniques (e.g., self-monitoring) in mobile health apps has the potential to promote adherence to inflammatory bowel disease treatment. While inflammatory bowel disease management apps exist, the extent to which they incorporate behavior change techniques remains unknown. AIMS: The present study systematically evaluated the content and quality of free, commercially available inflammatory bowel disease management apps. METHODS: Apps were identified using a systematic search of the Apple App and Google Play stores. Apps were evaluated using Abraham and Michie's taxonomy of 26 behavior change techniques. A literature search was conducted to identify behavior change techniques specific and relevant for people with inflammatory bowel disease. App quality was assessed using the Mobile App Rating Scale with scores ranging from 1 (Inadequate) to 5 (Excellent). RESULTS: A total of 51 inflammatory bowel disease management apps were evaluated. Apps included 0-16 behavior change techniques (Mean = 4.55) and 0-10 inflammatory bowel disease management behavior change techniques (Mean = 3.43). App quality ranged from 2.03 to 4.62 (Mean = 3.39) out of 5.00. Two apps, My IBD Care: Crohn's & Colitis and MyGiHealth GI Symptom Tracker, included the highest number of overall and inflammatory bowel disease management behavior change techniques along with high-quality scores. Bezzy IBD was the only app with a high number of overall and inflammatory bowel disease management behavior change techniques with a primary focus on social support/change. CONCLUSION: Most inflammatory bowel disease management apps reviewed included evidence-based inflammatory bowel disease management behavior change techniques.

Neurodevelopmental Outcomes in Children With Inherited Liver Disease and Native Liver.

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, <70). Univariate linear regression was performed to study association between FSIQ and risk factors, stratified by disease. RESULTS: Two hundred and fifteen completed testing (ALGS n = 70, PFIC n = 43, A1AT n = 102); median age was 7.6 years (3.0-16.9). Mean FSIQ in ALGS was lower than A1AT (94 vs 101, P = 0.01). Frequency of FSIQ < 85 (>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.

Self-Regulation as a Protective Factor for Diabetes Distress and Adherence in Youth with Type 1 Diabetes During the COVID-19 Pandemic.

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.

Burnout, professional fulfillment, and post-traumatic stress among pediatric solid organ transplant teams.

BACKGROUND: Adverse effects of clinician burnout have 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 IES-R. 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: One hundred and thirty five 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.

Targeted self-regulation interventions in low-income children: Clinical trial results and implications for health behavior change.

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.

The Role of Parent Self-Regulation in Youth Type 1 Diabetes Management.

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. Furthermore, parent and youth SR likely interact, with high parent SR enhancing the positive effects of high youth SR or compensating for low youth SR. 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.

Health literacy and its association with adherence in pediatric liver transplant recipients and their parents.

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.

Neurodevelopmental Outcomes in Preschool and School Aged Children With Biliary Atresia and Their Native Liver.

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 ±â€Š15). 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 ±â€Š14, P < 0.02), Verbal IQ (106 ±â€Š14, P < 0.001), and General Language Composite (107 ±â€Š16, P < 0.001) distributions were shifted higher compared with test norms. WISC-IV FSIQ (105 ±â€Š12, P < 0.01), Perceptual Reasoning Index (107 ±â€Š12, P < 0.01), and Processing Speed Index (105 ±â€Š10, P < 0.02) also shifted upwards. In univariate and multivariable analysis, parent education (P < 0.01) was a significant predictor of FSIQ on WPPSI-III and positively associated with WISC-IV FSIQ. Male sex and higher total bilirubin and gamma glutamyl transferase (GGT) predicted lower WPPSI-III FSIQ. Portal hypertension was predictive of lower WISC-IV FSIQ. CONCLUSIONS: This cohort of children with BA and native liver did not demonstrate higher prevalence of neurodevelopmental delays. Markers of advanced liver disease (higher total bilirubin and GGT for age ≤5 years; portal hypertension for age ≥6) correlate with lower FSIQ and may identify a vulnerable subset of patients who would benefit from intervention.

Adolescent Interventions to Manage Self-Regulation in Type 1 Diabetes (AIMS-T1D): randomized control trial study protocol.

BACKGROUND: Self-regulation (SR), or the capacity to control one's thoughts, emotions, and behaviors in order to achieve a desired goal, shapes health outcomes through many pathways, including supporting adherence to medical treatment regimens. Type 1 Diabetes (T1D) is one specific condition that requires SR to ensure adherence to daily treatment regimens that can be arduous and effortful (e.g., monitoring blood glucose). Adolescents, in particular, have poor adherence to T1D treatment regimens, yet it is essential that they assume increased responsibility for managing their T1D as they approach young adulthood. Adolescence is also a time of rapid changes in SR capacity and thus a compelling period for intervention. Promoting SR among adolescents with T1D may thus be a novel method to improve treatment regimen adherence. The current study tests a behavioral intervention to enhance SR among adolescents with T1D. SR and T1D medical regimen adherence will be examined as primary and secondary outcomes, respectively. METHODS: We will use a randomized control trial design to test the impact of a behavioral intervention on three SR targets: Executive Functioning (EF), Emotion Regulation (ER), and Future Orientation (FO); and T1D medical regimen adherence. Adolescents with T1D (n = 94) will be recruited from pediatric endocrinology clinics and randomly assigned to treatment or control group. The behavioral intervention consists of working memory training (to enhance EF), biofeedback and relaxation training (to enhance ER), and episodic future thinking training (to enhance FO) across an 8-week period. SR and treatment regimen adherence will be assessed at pre- and post-test using multiple methods (behavioral tasks, diabetes device downloads, self- and parent-report). We will use an intent-to-treat framework using generalized linear mixed models to test our hypotheses that: 1) the treatment group will demonstrate greater improvements in SR than the control group, and 2) the treatment group will demonstrate better treatment regimen adherence outcomes than the control group. DISCUSSION: If successful, SR-focused behavioral interventions could improve health outcomes among adolescents with T1D and have transdiagnostic implications across multiple chronic conditions requiring treatment regimen adherence. TRIAL REGISTRATION: ClinicalTrials.gov: NCT03688919; registered September 28, 2018.

Systematic Review of Father Involvement and Child Outcomes in Pediatric Chronic Illness Populations.

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.

Educational needs in families of pediatric liver and kidney transplant recipients: A quality improvement project.

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.

Communication about prognosis and end-of-life in pediatric organ failure and transplantation.

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.

Neurodevelopmental Outcome of Young Children with Biliary Atresia and Native Liver: Results from the ChiLDReN Study.

OBJECTIVES: 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. STUDY DESIGN: 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, and <85 for χ2 analysis. Risk for neurodevelopmental impairment (defined as ≥1 score of <85 on the Bayley Scales of Infant Development, 2nd edition, or Bayley Scales of Infant and Toddler Development, 3rd edition, scales) was analyzed using logistic regression. RESULTS: There 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. CONCLUSION: 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 are >4 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. TRIAL REGISTRATION: Clinicaltrials.gov: NCT00061828 and NCT00294684.

Psychosocial functioning in pediatric heart transplant recipients and their families.

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.

A systematic review of parent and family functioning in pediatric solid organ transplant populations.

The process of pediatric solid organ transplantation (SOT) places new and increased stressors on patients and family members. Measures of family functioning may predict psychological and health outcomes for pediatric patients and their families, and provide opportunity for targeted intervention. This systematic review investigated parent and family functioning and factors associated with poorer functioning in the pediatric SOT population. Thirty-seven studies were identified and reviewed. Studies featured a range of organ populations (eg, heart, liver, kidney, lung, intestine) at various stages in the transplant process. Findings highlighted that parents of pediatric SOT populations commonly report increased stress and mental health symptoms, including posttraumatic stress disorder. Pediatric SOT is also associated with increased family stress and burden throughout the transplant process. Measures of parent and family functioning were associated with several important health-related factors, such as medication adherence, readiness for discharge, and number of hospitalizations. Overall, findings suggest that family stress and burden persists post-transplant, and parent and family functioning is associated with health-related factors in SOT, highlighting family-level functioning as an important target for future intervention.

Quality of Life and Its Determinants in a Multicenter Cohort of Children with Alagille Syndrome.

OBJECTIVES: To assess health-related quality of life (HRQOL) in children with Alagille syndrome (ALGS) in comparison with healthy and other liver disease cohorts, and to identify determinants of HRQOL in patients with ALGS. STUDY DESIGN: Within the Childhood Liver Disease Research Network prospective study of cholestasis, Pediatric Quality of Life Inventory (PedsQL) questionnaires were administered to 70 children with ALGS, 95 children with alpha-1-antitrypsin deficiency (A1ATD), and 49 children with other causes of chronic intrahepatic cholestasis (IHC) aged 5-18 years. Parent proxy PedsQL scores were recorded for children aged 2-18 years (98 ALGS, 123 A1ATD, and 68 IHC). RESULTS: Mean ages and total bilirubin (mg/dL) were ALGS 9.4 years; 4.4, A1ATD 9.5 years; 0.7, and IHC 10.3 years; 2.9. ALGS child PedsQL scores were lower than in healthy children and children with A1ATD (mean 73 vs 83; P = .001). Children with ALGS and IHC were similar, except in physical scores (73 vs 79; P = .05). Parents of children with ALGS perceived their children to have worse HRQOL than A1ATD (P ≤ .001) and marginally lower compared with IHC. Univariate analysis revealed ALGS child-reported scores were positively associated with better growth and inversely with total bilirubin. Growth failure, elevated international normalized ratio, and an intracardiac defect were predictive of poor parental scores (P ≤ .05). In multivariate analysis, only weight z-score remained significant for child- and parent-reported scores. CONCLUSIONS: HRQOL is impaired in children with ALGS compared with healthy and children with A1ATD, similar to children with IHC and is associated with growth failure, which is a potentially treatable cause of impaired HRQOL.

Transition readiness in pediatric patients with inflammatory bowel disease: patient survey of self-management skills.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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 ±â€Š2.5 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. CONCLUSIONS: 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.