Sally J. Eder

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.

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.

Relationship Between Sleep Problems and Health-Related Quality of Life Among Pediatric Liver Transplant Recipients

Among adult liver transplant recipients (LTRs), sleep disturbances and fatigue are common. Sleep problems following pediatric liver transplantation may contribute to daytime fatigue and lower health‐related quality of life (HRQOL). The aim of this cross‐sectional study was to determine the impact of sleep problems on the HRQOL of pediatric LTRs using validated measures. Participants included 47 LTRs. Mean age of the LTRs was 10.9 ± 4.6 years, and mean time since transplantation was 6.2 ± 3.9 years. The primary indication for transplantation was biliary atresia (51%). According to parent reports, pediatric transplant recipients had symptoms of sleep‐disordered breathing, excessive daytime sleepiness, daytime behavior problems, and restless legs; 40.4% of parents and 43.8% of children reported significantly lower total HRQOL for the recipients. Age, time since transplantation, and health status were not significantly related to the quality of life. Hierarchical regression analyses revealed that the sleep‐disordered breathing subscale of the Pediatric Sleep Questionnaire accounted for significant variance in parent‐proxy reports on the Pediatric Quality of Life (PedsQL) summary scales measuring childrens psychosocial health (R2 = 0.36, P < 0.001), physical health (R2 = 0.19, P = 0.004), and total HRQOL (R2 = 0.35, P < 0.001). Also, the sleep‐disordered breathing subscale accounted for significant variance in the child self‐reported school functioning scale (R2 = 0.18, P = 0.03). Clinically significant sleep problems were more common among children with low total HRQOL. In conclusion, sleep problems were common in this cohort of pediatric LTRs and predicted significant variance in HRQOL. Prospective larger scale studies are needed to assess factors that contribute to sleep difficulties and low HRQOL in this population. The detection and treatment of significant sleep problems may benefit the HRQOL of pediatric LTRs. Liver Transpl 18:707–715, 2012.

Assessing allocation of responsibility for health management in pediatric liver transplant recipients

Given the increased risk for non‐adherence 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 AoR for health management in pediatric liver transplant patients. A total of 48 patients and 37 parents completed a 13‐item measure of AoR. We performed an exploratory PCA on survey results and used component scores to assess the relationship between AoR and age, age at transplant, adherence, and health outcomes. Two primary components were identified: communication with the healthcare 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 non‐adherence while responsibility for tasks related to self‐management was correlated, in older patients, with decreased non‐adherence. These results support AoR as a two‐domain construct, and they provide targets for monitoring and intervention as adolescent patients advance toward transfer.

Use of continuous glucose monitoring in young children with type 1 diabetes: implications for behavioral research

Patton SR, Williams LB, Eder SJ, Crawford MJ, Dolan L, Powers SW. Use of continuous glucose monitoring in young children with type 1 diabetes: implications for behavioral research.

Variation in Infliximab Administration Practices in the Treatment of Pediatric Inflammatory Bowel Disease

Objectives: Infliximab is used increasingly to treat inflammatory bowel disease (IBD). Infliximab is supplied in 100-mg vials. Doses that are typically calculated as 5 mg · kg−1 · dose−1 are commonly rounded up or down to the nearest 100 mg. Variation in dosing practices is unknown. Underdosing based on weight may increase the risk for disease exacerbation, whereas overimmune suppression could increase the risk of infection. Children may be at greater risk from dosage rounding. We aimed to characterize infliximab dosing practices, the use of corticosteroid premedication, and duration of infusions among pediatric practitioners participating in the ImproveCareNow Network, a national collaboration to improve IBD care and outcomes. Methods: A national survey of infliximab dosing practices was sent to 279 pediatric IBD practitioners from March to December 2011. Double data reconciliation, t test, and &khgr;2 analyses were performed. Results: The response rate was 74% (N = 207). Thirty-eight percent (78/207) indicated that their practice has no uniform approach to the rounding of doses. Of 114 respondents indicating a uniform approach to rounding doses, 43% always round up to the nearest 100 mg, 33% always round up or down to the nearest 100 mg, and 14% never round doses. In addition, 28% of respondents always premedicate with corticosteroids and 12% never premedicate. Of respondents indicating “it depends,” 95% premedicate if there has been a previous infusion reaction, 46% if there has been a prolonged lapse between treatment doses, 40% if antibodies to infliximab are present, and 11% if giving infliximab monotherapy. The duration of infusions is most often 2 hours, but varies between 1 and 4 hours. Conclusions: Wide variation exists in the practice of infliximab administration in pediatric IBD. The effect of these variations on outcomes is unknown.

Perianal Crohn Disease in a Large Multicenter Pediatric Collaborative.

Background: Although perianal complications of Crohn disease (CD) are commonly encountered in clinical practice, the epidemiology of perianal CD among populations of children is poorly understood. We sought to characterize the prevalence of perianal disease in a large and diverse population of pediatric patients with CD. Methods: We conducted retrospective analyses from a prospective observational cohort, the ImproveCareNow Network (May 2006–October 2014), a multicenter pediatric inflammatory bowel disease quality improvement collaborative. Clinicians prospectively documented physical examination and phenotype classification at outpatient visits. Perianal examination findings and concomitant phenotype change were used to corroborate time of new-onset perianal disease. Results were stratified by age, sex, and race and compared across groups with logistic regression. Cumulative incidence was estimated using Kaplan-Meier analyses and compared between groups with Cox proportional hazard regression models. Results: The registry included 7076 patients with CD (41% girls). Missing/conflicting entries resulted in 397 (6%) patient exclusions. Among the remaining 6679 cases, 1399 (21%) developed perianal disease. Perianal disease was more common among boys (22%) than girls (20%; P = 0.013) and developed sooner after diagnosis among those with later rather than early onset disease (P < 0.001). Perianal disease was also more common among blacks (26%) compared with whites (20%; P = 0.017). Asians with later onset CD developed perianal disease earlier in their disease course (P = 0.01). There was no association between disease location or nutritional status at diagnosis and later development of perianal disease. Conclusions: In this large multicenter collaborative, we found that perianal disease is more common among children with CD than previously recognized. Differences in the development of perianal disease were found across racial and other subgroups. Treatment strategies are needed to prevent perianal disease development.

Fistulizing Crohn's Disease Presenting After Surgery on a Perianal Lesion.

Perianal skin lesions, such as skin tags, can be an early presenting sign of Crohn’s disease. Surgical intervention on these lesions may increase the risk of fistula development and lead to worse outcomes. This case series examined 8 patients who underwent surgical intervention on what appeared to be benign perianal skin lesions, only to reveal fistulas leading to the diagnosis of Crohn’s disease. This patient population comprised 20% of all pediatric patients with Crohn’s disease who had perianal fistula present at diagnosis. The initial type of perianal lesion varied from case to case and included skin tags, hemorrhoids, and perianal abscesses. All of the patients had other presenting features that, in retrospect, may have been attributed to Crohn’s disease. None presented solely with a perianal lesion. Four patients had weight loss or growth failure. Most of the remainder had abnormal laboratory test results. These findings should raise the awareness of primary care providers that perianal lesions can be the first presenting sign of possible Crohn’s disease in otherwise healthy appearing children. Such children should undergo a thorough evaluation for Crohn’s disease before surgical intervention on perianal lesions because surgical procedures may be associated with worse outcomes.

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.