Health-related quality of life and disability in young patients with spina bifida

Objectives: To assess the health-related quality of life (QOL) and disability in young patients with spina bifida and to correlate them with the clinical examination findings. Design: Prospective multidimensional study by means of (1) clinical assessment, (2) self-administered questionnaire for general health, and (3) standardized disability measurements. Relationships between disability measurement, patient-oriented examination, and conventional clinical assessment were evaluated. Setting: Pediatric department at a university hospital in Italy. Patients: Twelve consecutive young patients with spina bifida (mean age, 15.2y; range, 14-18y). Interventions: Not applicable. Main Outcome Measures: The Medical Outcomes Study 36-Item Short-Form Health Survey, the FIM(TM) instrument, and the Barthel Index. Results: As expected, disability was inversely related (r=.72, P<.02) to the physical aspect of QOL. Unexpectedly, for the mental aspects of QOL, less disability was associated (r=-.70, P<.05) with higher psychologic distress and severe role disability because of emotional problems. The findings at clinical examination, especially proximal deficit of inferior limbs (r=-.70, P<.05), were usually related to higher disability and lower physical aspects of QOL. Conclusion: There was no linear inverse correlation between disability and QOL in patients with spina bifida. Patients with mild disability needed as much psychologic support as patients with severe whole disability.