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 IssuesReduced environmental exposure & exploration for infants & toddlers

Limited social developmentHospitalizations & 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 yearsSignificant cognitive delays

Children > 5 yearsNo difference in overall IQSpecific 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 effectsHemorrhagingProgressive encephalopathyGrowth failure

Delays in cognitive developmentRelated to duration & severity of liver diseaseChildren dx’d < 1 y.o. at greater risk for global cognitive deficits

Psychosocial Effects of ESLD

Engage in fewer age-appropriate activitiesExperience peer teasing due to physical symptomsFear of death

No external life-sustaining proceduresDifficulty in locating liver25% 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

AdolescentsNeg. correlation for symptomatology & compliance

Pos. correlation for body dissatisfaction & noncompliance

Vicious cycle

Interventions aimed at increasing parental involvement & supervision are effective