Living with VACTERL association : From the perspectives of children, adolescents and their parents

Abstract: VACTERL association is a rare and complex congenital condition often requiring repeated surgery and entailing various physical sequelae. Knowledge is scarce regarding experiences of the health condition and health care, need of support at school, health-related quality of life (HRQoL), and psychological well-being in children, adolescents and their parents.This thesis aims to investigate various aspects of living with VACTERL association, from the perspectives of children, adolescents and their parents.Ten children aged five to eight years were interviewed using the computer-assisted interview technique In My Shoes (Study I). They expressed awareness of their health history and felt proud but also different due to physical dysfunction. While happy to meet familiar staff in the hospital they voiced worries about medical procedures.The nineteen parents interviewed described crisis reactions on the discovery of malformations in their child (Study II). Parental involvement in care was reported from the initial hospital admission until taking responsibility for treatments at home. Eventually the health condition became integrated in everyday life. Insufficient emotional and limited medical support were reported. Various levels of professionalism among healthcare professionals and discrepancies concerning knowledge and experience between the tertiary and local hospitals were described.In ten evaluated pre-school children (Study III), intelligence measured by Wechsler-scales was within the normal range. Eight children had attention difficulties and two were later diagnosed with attention deficit hyperactivity disorder (ADHD). All children had physical dysfunctions affecting their nutrition, bowel or bladder functions. All needed extra support and adjustments at school.Forty children and adolescents responded to validated questionnaires of DISABKIDS and Beck inventories (Study IV). The HRQoL was comparable to European children with chronic conditions. Their psychological well-being was similar to that of Swedish school children and significantly better than that of a clinical sample. Self-reported anxiety and depression in 38 mothers and 33 fathers were comparable to non-clinical samples.In conclusion, regular follow-up by multi-professional team with continuity is crucial to optimise the physical function in children with VACTERL, to identify those in need of extra support at school and to detect reduced psychological well-being in children and parents. Fear of medical procedures may be reduced by carefully providing information and individual care strategies. For the parents psychological processing, support from medical experts and peers is essential to achieve self-confidence and adaptation. Transfer of knowledge and information between multi-professional teams at the local and tertiary hospitals could be improved by the use of video sessions.