Liver transplantation has saved the lives of many young children in the last decade and has been welcomed by families and the medical community alike; but it is a highly technical medical intervention requiring lengthy hospitalizations and frequent procedures. Because of this, many parents have questions about the long term developmental effects of liver disease and transplantation. To answer these questions, the developmental specialist on the liver transplant team at Stanford University Medical Center is conducting a study that examines the developmental outcome of children with biliary atresia.

This study has focused on children transplanted before the age of two. This age group was selected because this is a time when many new skills emerge that could potentially be affected by liver disease and transplantation. Forty children were assessed with the Bayley Scales of Infant Development prior to transplantation and then 3, 12, and 24 months following transplantation. The scores from these assessments were then averaged to see if there were any general developmental trends. We found that prior to transplant, the children showed moderate delays in cognitive development and significant delays in gross motor development.

Three months after transplant the effects of surgery were evident and the developmental level of functioning dropped significantly for both mental and motor skill. However, the first few months following transplantation is a time of rapid recovery so that by the one year anniversary date, developmental functioning had returned to pre-transplant levels.

At 1 year post transplant, cognitive skills showed little change but gross motor skills tended to "catch up" to the level of cognitive functioning.

At 2 years post transplant, children continue to demonstrate some delays in both the cognitive and gross motor areas of development. Information for children 3 and 4 years post transplant is sketchy but it appears that the recovery process continues and the children continue to close the gap between developmental scores and their chronological age. Given the seriousness of the disease prior to transplant and invasiveness of the transplant surgery itself, these finds suggest that these children have a remarkable resiliency and have the potential to recover many developmental skills.

These findings raised another question: What specific developmental behaviors or skills are most affected by liver disease and transplantation?

It appears that major motor milestones and communication skills tend to lag behind other areas of development. For many children, the emergence of sitting, crawling, and walking was delayed by 3-6 months. One factor which may account for this is limitation of movement prior to transplant. This is a time when the liver can be enlarged or ascites may be present, therefore, these young children are reluctant to spend time on their tummy, sit upright or rotate their trunk—all positions or patterns of movement that are important prerequisites for strengthening the trunk muscles that support the child in walking. Furthermore, the transplant surgery severs all abdominal muscles which exacerbates the child’s already weakened trunk muscles. Following transplant, the children bounce back and begin to regain lost gross motor skills. However, we have found that intensive intervention prior to transplant and in the 3 months immediately following transplantation helps to support the child in the recovery of gross motor skill. This therapeutic intervention assures that as gross motor skills reemerge they do so with good quality of movements and normal patterns of development.

The other skill that tends to lag behind is expressive language. Expressive language is the child’s ability to verbalize his or her thoughts, needs and request. The children in our group appeared to understand the communicative attempts of adults and other children, but verbalization such as first words and 2 and 3 word phrases tended to emerge 4-6 months later than other developmental skills, after transplantation, most children seem to naturally "catch up" while others needed a little help with speech and language therapy. Children who had not caught up at approximately 1 year post transplant were referred to language therapy with good results.

The developmental picture for those children transplanted at age 3 and older appears to be somewhat different since language and gross motor skills are already in place at the time of transplant. Similar to the infants and toddlers, transplantation interrupts the developmental process, but the older children tend to recover their developmental skills more quickly with less impact on future skill development.

Many developmental questions remain, and I am sure this pioneering group of young children will continue to give us answers. My work with these children and their families has been intensely rewarding, and I want to tank them for their willingness to share information with me and other families so that together we can determine the best information and therapeutic interventions for helping future transplant patients.

Ms. Wayman is a Developmental Specialist on the liver transplant team at Lucile Packard Children’s Hospital at Stanford University Medical Center