Parental Reports of Health-related Quality of Life in Young Children with Neurofibromatosis Type 1: Influence of Condition Specific Determinants

Objective: To assess the health-related quality of life of 34 Dutch children 12 to 72 months of age with neurofibromatosis type 1 (NF1) using the Infant/Toddler Quality of Life Questionnaire (ITQOL) and to investigate the potential impact of clinical factors on parental reports of health-related quality of life.

Study Design: A parent-completed form including the ITQOL, NF1-specific questions, and sociodemographic questions was sent. ITQOL scale scores were compared for the study population against Dutch reference values. The influence of general and clinical characteristics on ITQOL scale scores was evaluated with multivariate analysis.

Results: A significant impact was observed on most aspects of quality of life, particularly for growth and development, general health perceptions and parental impact. The lowest scores were observed in children with complications because of NF1. ITQOL scale scores were affected by parental educational level, familial NF1, and parental reports of complications of NF1 and perceived disease severity.

Conclusions: Important aspects of health-related quality of life were observed to be negatively affected in children with NF1, as measured by the ITQOL. Family-related and disease-related variables appeared to influence the quality of life in children with NF1.