Quality of life in spina bifida: importance of parental hope.

BACKGROUND AND AIMS: Prognosis in spina bifida (SB) is often based only on neurological deficits present at birth. We hypothesised that both parental hope and the neurophysical examination predict quality of life in children and adolescents with SB. METHODS: A previously validated disease and age specific health related quality of life (HRQL) instrument was posted to families of children (aged 5-12 years) and adolescents (aged 13-20 years) with SB. We measured parental hope, determined the child's current physical function, and obtained retrospective data on the neonatal neurophysical examination (NPE). Regression analysis modelled HRQL firstly as a dependent variable on parental hope and NPE ("birth status"); and secondly on parental hope and current physical function ("current function"). RESULTS: Response rates were 71% (137 of 194) for families of children, and 54% (74 of 138) for families of adolescents. NPE data were available for 121 children and 60 adolescents. In children, the birth status model predicted 26% of the variability (R(2) hope 21%) compared with 23% of the variability (R(2) hope 23%)in the adolescents. The current function model explained 47% of the variability (R(2) hope 19%) in children compared with 31% of the variability (R(2) hope 24%) in the adolescents. CONCLUSIONS: In both age groups, parental hope was more strongly associated with the HRQL than neonatal or current physical deficits. A prospective study is required to determine whether a causal relation exists between parental hope and HRQL of children and adolescents with SB.

Development of a health-related quality of life instrument for use in children with spina bifida.

The objective of this study was to develop a spina bifida health-related quality of life (HRQOL) instrument. Items were generated through semistructured interviews, and reduced by frequency-importance product ranking. Validity was assessed by correlating the HRQOL score with a global question concerning the child's well-being using the Spearman's rank coefficient, and the Piers-Harris Children's Self-Concept Scale (P-H) using the Pearson correlation coefficient. Reproducibility was assessed at 2-week intervals using the intra-class correlation coefficient (ICC). Field testing was undertaken in a larger sample to evaluate item-total correlation, internal consistency and construct validity. Patients taking part in the study were 329 children and adolescents with spina bifida attending two treatment centres. Over 600 items were generated. These were reduced to 47 questions and 50 questions, for children and adolescents respectively. The correlation between the HRQOL score and the global question was r = 0.57, and with the P-H was 0.26 (children). These values for adolescents were 0.63, and 0.89, respectively. Reproducibility was ICC = 0.78 (children) and 0.96 (adolescents). Following field testing, the questionnaire was further reduced to 44 questions (children) and 47 questions (adolescents) by eliminating questions with an item- total correlation less than 0.20. Cronbach's alphas for the final instrument were 0.93 (children) and 0.94 (adolescents), and construct validity correlations were 0.63 (children) and 0.37 (adolescents). The spina bifida HRQOL instrument has good measurement properties and may be used as a discriminative instrument. Assessment of responsiveness is necessary before using it to evaluate therapy in clinical trials.