Emily M. Fredericks

Psychological Functioning, Nonadherence and Health Outcomes After Pediatric Liver Transplantation

The present study empirically assessed the relationships between adherence behaviors and HRQOL, parent and child psychological functioning and family functioning, and investigated the relationship between adherence behaviors and health outcomes in children who were within 5 years of their liver transplantation. Participants included 38 children (mean = 8.5 years, range 28 months to 16 years) and their parent/guardian(s). HRQOL and psychological functioning were examined using well‐validated assessment measures. Measures of adherence included the rate of clinic attendance and standard deviations (SDs) of consecutive tacrolimus blood levels, which were collected and evaluated retrospectively. Measures of child health status included the frequency of hospital admissions, liver biopsies, episodes of rejection and graft function for the year prior to study participation. Results indicated that nonadherence was related to lower physical HRQOL, more limitations in social and school activities related to emotional and behavioral problems, parental emotional distress and decreased family cohesion. Nonadherence was also related to frequency and duration of hospitalizations, liver biopsies and rejection episodes. These results suggest that empirically based assessment of HRQOL, parenting stress and family functioning may help identify patients at risk for nonadherence, and may allow for the need‐based delivery of appropriate clinical interventions.

Assessment of transition readiness skills and adherence in pediatric liver transplant recipients

Fredericks EM, Dore‐Stites D, Well A, Magee JC, Freed GL, Shieck V, Lopez MJ. Assessment of transition readiness skills and adherence in pediatric liver transplant recipients.
Pediatr Transplantation 2010: 14:944–953.

Adherence and health-related quality of life in adolescent liver transplant recipients

Abstract:  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.

Pediatric Health-Related Quality of Life: Feasibility, Reliability and Validity of the PedsQL™ Transplant Module

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 α= 0.93; total scale score for parent proxy‐report α= 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.

Continuous subcutaneous insulin infusion benefits quality of life in preschool‐age children with type 1 diabetes mellitus

Objective:  To compare medical, nutritional, and psychosocial outcomes of continuous subcutaneous insulin infusion (CSII) therapy and multiple daily insulin injections (MDI) in preschoolers with type 1 diabetes mellitus (T1DM) in a randomized controlled trial.

Nonadherence and the Transition to Adulthood

Key Points

Transition of pediatric liver transplant recipients to adult care: Patient and parent perspectives

Fredericks EM, Dore‐Stites D, Lopez MJ, Well A, Shieck V, Freed GL, Eder SJ, Magee JC. Transition of pediatric liver transplant recipients to adult care: Patient and parent perspectives.
Pediatr Transplantation 2011: 15: 414–424.

Adherence to immunosuppressants: how can it be improved in adolescent organ transplant recipients?

Purpose of reviewNonadherence 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. Recent findingsTo 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. SummaryStrategies 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.

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 ± 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” (&rgr; = 0.43, P = 0.003), “telling medical staff I do not like or am having trouble following a treatment” (&rgr; = 0.37, P = 0.003), and “naming my medications” (&rgr; = 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.

Health Related Quality of Life in Patients with Biliary Atresia Surviving with their Native Liver

OBJECTIVES To quantify health related quality of life (HRQOL) of patients with biliary atresia with their native livers and compare them with healthy children and patients with biliary atresia post-liver transplant (LT) and to examine the relationship between HRQOL and medical variables. STUDY DESIGN A cross-sectional HRQOL study of patients with biliary atresia with their native livers (ages 2-25 years) was conducted and compared with healthy and post-LT biliary atresia samples using Pediatric Quality of Life Inventory 4.0 child self and parent proxy reports, a validated measure of physical/psychosocial functioning. RESULTS 221 patients with biliary atresia with native livers (54% female, 67% white) were studied. Patient self and parent proxy reports showed significantly poorer HRQOL than healthy children across all domains (P < .001), particularly in emotional and psychosocial functioning. Child self and parent proxy HRQOL scores from patients with biliary atresia with their native livers and post-LT biliary atresia were similar across all domains (P = not significant). Child self and parent proxy reports showed moderate agreement across all scales, except social functioning (poor to fair agreement). On multivariate regression analysis, black race and elevated total bilirubin were associated with lower Total and Psychosocial HRQOL summary scores. CONCLUSIONS HRQOL in patients with biliary atresia with their native livers is significantly poorer than healthy children and similar to children with post-LT biliary atresia. These findings identify significant opportunities to optimize the overall health of patients with biliary atresia.