Frailty in Children with Liver Disease: A Prospective Multicenter Study.

OBJECTIVE: To assess frailty, a measure of physiologic declines in multiple organ systems, in children with chronic liver disease using a novel pediatric frailty tool. STUDY DESIGN: We performed a prospective cross-sectional multicenter study at 17 liver transplantation (LT) centers. 71 children (5-17 years of age), 36 with compensated chronic liver disease (CCLD) and 35 with end-stage liver disease (ESLD) and listed for LT, were assessed for frailty using validated pediatric tools to assess the 5 classic Fried Frailty Criteria-slowness, weakness, exhaustion, diminished physical activity, and shrinkage. Test scores were translated to age- and sex-dependent z scores, generating a maximum frailty score of 10. RESULTS: The median frailty score of the cohort was 4 (IQR 3, 5). Subjects with ESLD had significantly higher frailty scores (median 5; IQR 4, 7) than subjects with CCLD (median 3; IQR 2, 4); (P < .0001). Area under the curve receiver operating characteristic for frailty scores to discriminate between ESLD and CCLD was 0.83 (95% CI 0.73, 0.93). Forty-six percent of children with ESLD were frail and there was no correlation between pediatric frailty scores and physician's global assessments (r = -0.24, 95% CI -0.53, 0.10). CONCLUSIONS: A novel frailty tool assessed additional dimensions of health, not captured by standard laboratory measures and identified the sickest individuals among a cohort of children with chronic liver disease. This tool may have applicability to other children with chronic disease.

Pediatric solid organ transplant recipients: transition to home and chronic illness care.

Pediatric SOT recipients are medically fragile and present with complex care issues requiring high-level management at home. Parents of hospitalized children have reported inadequate preparation for discharge, resulting in problems transitioning from hospital to home and independently self-managing their child's complex care needs. The aim of this study was to investigate factors associated with the transition from hospital to home and chronic illness care for parents of heart, kidney, liver, lung, or multivisceral recipients. Fifty-one parents from five pediatric transplant centers completed questionnaires on the day of hospital discharge and telephone interviews at three wk, three months, and six months following discharge from the hospital. Care coordination (p = 0.02) and quality of discharge teaching (p < 0.01) was significantly associated with parent readiness for discharge. Readiness for hospital discharge was subsequently significantly associated with post-discharge coping difficulty (p = 0.02) at three wk, adherence with medication administration (p = 0.03) at three months, and post-discharge coping difficulty (p = 0.04) and family management (p = 0.02) at six months post-discharge. The results underscore the important aspect of education and care coordination in preparing patients and families to successfully self-manage after hospital discharge. Assessing parental readiness for hospital discharge is another critical component for identifying risk of difficulties in managing post-discharge care.

Transition from hospital to home following pediatric solid organ transplant: qualitative findings of parent experience.

Transplant providers are challenged to determine appropriate interventions for patients and families due to limited published research regarding the context of the post-discharge experience from the perspective of parents of transplanted children. The purpose of this study is to describe the parent perspective of the transition from hospital to home following their child's solid organ transplant. Within a mixed-methods design, 37 parents of pediatric heart, kidney, and liver transplant recipients from three pediatric hospitals responded to qualitative interview questions on the day of hospital discharge and three wk following hospital discharge. Insight to the discharge preparation process revealed necessary education components. Post-discharge themes were identified for coping, knowledge, and adherence. The parents' responses provide awareness as to specific stressors and concerns parents are faced with when their child is discharged from the hospital after solid organ transplant and opportunities for ways the transplant team can provide support.

Essential components of transition to adult transplant services: the transplant coordinators' perspective.

CONTEXT: Medical and surgical advancements have resulted in improved long-term survival of pediatric liver transplant recipients. As pediatric patients approach school age and adolescence, transplant centers are challenged to facilitate the process of transitioning from pediatric to adult centers. OBJECTIVE: To describe pediatric and adult liver transplant coordinators' perspective regarding practice for transitioning patients to adult-oriented transplant centers. DESIGN: Descriptive SETTING: Pediatric and adult liver transplant coordinators associated with Studies of Pediatric Liver Transplantation. PARTICIPANTS: A total of 35 pediatric liver transplant coordinators and 24 adult liver transplant coordinators completed the survey. METHOD: Investigator-developed survey to identify current practice for transition process and actual transfer of a patient from pediatric to adult care. RESULTS: Transplant coordinators play an integral role in the transition process, and study results highlight what experienced coordinators believe are important considerations for a successful transition process. Results also highlight the importance of communication and partnership between the pediatric and adult programs.

Validation of a Screening Tool to Identify Undernutrition in Ambulatory Patients With Liver Cirrhosis.

BACKGROUND: The prevalence of chronic liver disease is increasing in the United States. Malnutrition is common as liver disease progresses. However, an accepted method to screen these patients for malnutrition is lacking. The 6-question undernutrition screening tool was developed for professionals without nutrition training to identify a decline in the nutrition status of patients with liver cirrhosis. A 3-phase validation study was completed to assess face, content, and clinical validity of the screening tool in ambulatory patients with liver cirrhosis. METHODS: In phase I, face validity was determined by surveying 13 liver disease professionals. In phase II, content validity was assessed by surveying 12 registered dietitians who specialize in liver disease. In phase III, a cross-sectional investigation was completed to compare the agreement between the undernutrition screening tool and nutrition assessment by a registered dietitian (RD). RESULTS: Twenty-two patients with a diagnosis of liver cirrhosis participated in phase III of the investigation. The RD assessment identified undernutrition in 82% of patients (95% CI, 60%-95%). The κ statistic indicated a fair agreement between the screening tool and RD assessment. Sensitivity and specificity of the tool were 72% and 75%, respectively, and positive predictive value was 93%. CONCLUSIONS: Feedback from phase I, II, and III indicate that the undernutrition screening tool is simple, is easy to use, and measures the constructs that have the strongest link with undernutrition in liver disease. Additional minor adjustments to the screening tool and a multicenter investigation are indicated to confirm clinical effectiveness and cross-validity of the tool.

Successful liver transplant for unresectable hepatoblastoma.

BACKGROUND: Treatment of children with stage III and IV hepatoblastoma has shown little improvement with 5-year survival rates of 64% and 25%, respectively (J Clin Oncol 2000;18:2665-75). A timely and organized treatment program including preoperative chemotherapy combined with living donor liver transplantation and postoperative chemotherapy has been used seeking improved long-term survival in stage III and IV cases. METHODS: A retrospective review of 8 patients with stage III and IV hepatoblastoma unresectable by conventional resection were treated with complete hepatectomy and transplantation. Approval was obtained from our institutional review board. RESULTS: Since August of 2001, we have treated 6 patients with stage III hepatoblastoma and 2 patients with initial stage IV hepatoblastoma. These patients (age, 23 months-9 years) had all received extensive chemotherapy or prior resections. After chemotherapy, none had gross tumor documented outside of the liver at time of transplantation. All underwent hepatectomy including vena cava resection, in selected cases, with living donor orthotopic liver transplantation. All patients had at least 2 cycles of postoperative chemotherapy. Of 8 patients, 6 are alive and well with normalized alpha-fetoprotein levels. There were 2 late deaths from recurrent disease. Length of follow-up ranged from 7 to 53 months. CONCLUSION: Complete hepatectomy with living donor liver transplantation provides optimal surgical treatment in unresectable stage III and initial stage IV disease confined to the liver at resection. This series indicates that children tolerate complete hepatectomy, transplantation, and postoperative chemotherapy well. Referral to a transplant center during the first 3 cycles of chemotherapy appears to offers the best opportunity for long-term survival.