Pediatric Organ Transplantation: Renal & Liver Disease December 4, 2007

Background Information Kidney & liver disease are rare in children & adolescents Medications, dietary changes, & dialysis often are successful treatments End Stage Renal Disease (ESRD) and End Stage Liver Disease (ESLD)

ESRD Due to congenital & acquired conditions Usually requires lifelong dialysis Hemodialysis Peritoneal dialysis

ESRD 1/3 – 1/2 of new ESRD patients are potential candidates for transplantation Less complex & more common than any other organ transplant Survival rates far exceed 90%

ESLD May result from infections, structural, metabolic, obstructive, & toxic causes Chronic hepatitis; biliary atresia, tyrosinemia; ingestion of poisons ¾ of liver cells can be destroyed before it stops functioning No cure exists for liver failure; transplantation is the only treatment

Liver Transplant Procedures Technically difficult medical procedure Healthy organ obtained from a brain- dead patient Survival rates are lower than for renal transplants, but are improving dramatically

Post-Transplant Medical Management Post-transplant morbidity Infection & Rejection Medication side effects Repeated blood testing Potential additional surgeries

Developmental Issues Reduced environmental exposure & exploration for infants & toddlers Limited social development Hospitalizations & clinic appointments Lengthy treatments Reduced physical stamina Restricted social system

Developmental Issues Fears & understanding of medical procedures Parent-adolescent relations Dependence Overprotective parents (e.g., compliance) Privacy Perceived lack of control

Neurocognitive Effects of ESRD Children < 5 years Significant cognitive delays Children > 5 years No difference in overall IQ Specific differences Patients w/ earlier onset & longer duration of renal failure performed at lower levels than healthy peers

Psychosocial Effects of ESRD Dialysis pts  more severe difficulties Fatigue, school absenteeism, health concerns, and physical side effects  adjustment difficulties Short stature Cushingoid facial features

Neurocognitive Effects of ESLD Physical effects Hemorrhaging Progressive encephalopathy Growth failure Delays in cognitive development Related to duration & severity of liver disease Children dx’d < 1 y.o. at greater risk for global cognitive deficits

Psychosocial Effects of ESLD Engage in fewer age-appropriate activities Experience peer teasing due to physical symptoms Fear of death No external life-sustaining procedures Difficulty in locating liver 25% of pts. qualified for transplant die before the donor is located

Pre-Transplant Evaluation Intellectual & developmental functioning Academic achievement Memory & learning

Pre-Transplant Evaluation Attention Visual-motor integration Personality & psychological functioning

Pre-Transplant Evaluation Family functioning Evaluation of the donor Assessment of compliance

Ethical concerns

Medication Issues Steroids (e.g., prednisone) Benefits Side-effects Immunosuppressants (e.g., cyclosporine) Benefits Side-effects Other medications

Effects of Transplantation Neurocognitive effects Psychosocial effects

Post-Transplant Compliance Adolescents Neg. correlation for symptomatology & compliance Pos. correlation for body dissatisfaction & noncompliance Vicious cycle Interventions aimed at increasing parental involvement & supervision are effective