Victoria Shieck

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.

Corticosteroid withdrawal after liver transplantation

BACKGROUND Long-term side effects of corticosteroids (CSs) result in > major morbidity for recipients of orthotopic liver transplants (OLT). We instituted a program of CS withdrawal among OLT recipients to quantify the contribution of CS to adverse clinical sequelae and to determine whether long-term CS administration is necessary to avoid rejection. METHODS Recipients who had normal allograft function on CS, cyclosporine, and azathioprine more than 1 year after OLT were offered CS withdrawal during 12 to 22 weeks. Patients underwent routine clinical monitoring and laboratory studies. Continuous variables were compared by paired t test analysis. RESULTS CSs were discontinued in 51 recipients; 45 (88%) of 51 patients remain steroid-free after a mean follow-up of 13.8 months (range, 4 to 36). CS therapy was reinstituted in 6 patients who had abnormal transaminase levels during routine follow-up. Among the patients who remain off CS, there were no significant changes in blood pressure, transaminase, alkaline phosphatase, bilirubin, or glucose levels during the study period. Mean number of blood pressure medications decreased from 0.7 +/- 0.1 to 0.4 +/- 0.1 (p = 0.007). Cholesterol decreased from 217 +/- 8 mg/dl on CS to 204 +/- 9 mg/dl at 1 month (p = 0.0001), 183 +/- 10 mg/dl at 3 months (p = 0.0001), 198 +/- 8 mg/dl at 6 months (p = 0.04), 213 +/- 11 mg/dl at 12 months (p = 0.01), 209 mg/dl +/- 16 at 18 months (p = 0.02), and 183 +/- 19 mg/dl at 24 months (p = 0.2) off CS. Weight loss occurred in 88% of patients and averaged 9.5 pounds. CONCLUSIONS CS therapy can be successfully withdrawn without precipitating rejection in liver transplant recipients who have stable graft function 1 year after OLT. The incidence and severity of hypertension and hypercholesterolemia are reduced in patients whose CSs have been withdrawn.

Long-term follow-up of percutaneous transhepatic balloon cholangioplasty in the management of biliary strictures after liver transplantation.

Background. This study evaluated the efficacy of a protocol of initial balloon dilation for biliary strictures after liver transplantation. Methods. Complete records from 96 patients with biliary strictures were retrospectively reviewed. Seventy-six patients received percutaneous transhepatic balloon cholangioplasty (PTBC) after initial placement of biliary drainage (percutaneous transluminal cholangiography [PTC]) tube. In most cases, three dilations were performed with a 4 to 8 week interval between procedures. Follow-up ranged from 6 months to 10 years. Results. PTBC successfully treated strictures in 39 of 76 (51.3%) cases. Factors favoring successful PTBC included older age at transplant, shorter cold ischemic time, and single strictures. There were nine recurrent strictures after PTBC, all of which were successfully treated by nonoperative measures. The number of dilations performed affected both the likelihood of success and the long-term risk of stricture recurrence. Of the 37 PTBC failures, 14 underwent subsequent surgical revision. When both angiographic and surgical modalities were considered, treatment success was associated with first transplants, shorter cold ischemic time and operative time, and less intraoperative transfusion requirements. Factors associated with treatment failure included multiple, central hepatic duct, and intrahepatic strictures. PTC-tube independence was achieved in 51 of 76 (67%) patients using the combined approach of PTBC and surgery for PTBC failures. Conclusions. PTBC is an effective initial modality for treating posttransplant biliary strictures. Prolonged cold ischemic and operative times and multiple or peripheral strictures predispose to treatment failure. Solitary extrahepatic strictures that fail PTBC are salvageable with surgical revision with excellent results.

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.

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.

Immune response to rabies vaccination in pediatric transplant patients

Abstract:  Children have become engaged in a wider variety of activities as the success of solid organ transplantation has improved. These activities can result in exposure to infectious agents for which there are no data documenting the efficacy of standard treatment in children on immunosuppressive therapy. This is a retrospective review of five OLT patients and three RT patients who were potentially exposed to rabies during camp. They completed the immunoprophylaxis treatment for rabies exposure outlined by the CDC in the 2003 Red Book. Rabies titers were followed for six to 12 months post‐immunization. All five OLT patients were on tacrolimus. All three RT patients were on tacrolimus, mycophenolate mofetil, and prednisone. At the time of exposure median age was 10.0 yr (8.4–17.3). None of the subjects developed rabies. A positive rabies titer, indicative of successful immunization, was present by one month in seven subjects and all subjects by six months. Rabies vaccination in pediatric transplant patients is safe and associated with the successful production of antirabies titers.

Looking Outside: Summer Camp for Children Who Have Undergone a Solid-Organ Transplant.

A high-quality critical care team is an essential component of any successful organ transplant program. From pretransplant care to the crucial postoperative period, its importance cannot be discounted. However, because of the focused nature of work in an intensive care unit (ICU), all too often members of the ICU team are not able to see and appreciate the ultimate fruits of their labor. These are factors that can contribute to the high rates of burnout and turnover among ICU teams. This article presents the concept of a summer camp for children who have received a solid-organ transplant. We discuss a vehicle by which ICU staff as well as other members of a patients care team can gain a better appreciation of the full nature of both medical and, perhaps more importantly in this situation, nonmedical goals and outcomes in pediatric transplantation. We review our institutional experience running a summer camp for these children, discuss some of the important points in making such a camp successful, and discuss potential benefits to the campers as well as those taking care of them.

Pediatric liver transplantation unique concerns for the critical care team

Liver transplantation originated in children more than 50 years ago, and these youngest patients, while comprising the minority of liver transplant recipients nationwide, can have some of the best and most rewarding outcomes. The indications for liver transplantation in children are generally more diverse than those seen in adult patients. This diversity in underlying cause of disease brings with it increased complexity for all who care for these patients. Children, still being completely dependent on others for survival, also require a care team that is able and ready to work with parents and family in addition to the patient at the center of the process. In this review, we aim to discuss diagnoses of particular uniqueness or importance to pediatric liver transplantation. We also discuss the evaluation of a pediatric patient for liver transplant, the system for allocating them a new liver, and also touch on postoperative concerns that are unique to the pediatric population.