Health-Related Quality of Life in Children with Neurofibromatosis Type 1: Contribution of Demographic Factors, Disease-Related Factors, and Behavior
Objective
To investigate health-related quality of life (HR-QOL) in children with neurofibromatosis type 1 (NF1) with parental reports and children's self-reports, and to investigate the potential contribution of demographic factors, disease-specific factors, and problems in school performance or behavior.
Study design
In a prospective observational study, parents of 58 children with NF1 (32 boys, 26 girls, age 12.2 ± 2.5 years) visiting a university clinic, and their 43 children 10 years or older were assessed with the Child Health Questionnaire (CHQ). Potential determinants of domain scores were assessed in 3 explorative regression models.
Results
Parents reported a significant impact of NF1 on 9/13 CHQ scales, with moderate effect sizes on 8 (general health perceptions, physical functioning, general behavior, mental health, self esteem, family activities, role functioning emotional/behavioral, and parent emotional impact). Children report an impact on bodily pain, and an above average general behavior. Multiple CHQ scales were sensitive to demographic factors and behavioral problems, and 1 to NF1 severity. NF1 visibility and school problems did not influence HR-QOL.
Conclusions
Parents, but not the children with NF1, report a profound impact of NF1 on physical, social, behavioral, and emotional aspects of HR-QOL. Multiple HR-QOL domains were most sensitive to behavioral problems, which points to an exciting potential opportunity to improve HR-QOL in children with NF1 by addressing these behavioral problems.
Abbreviations: ADHD, Attention deficit hyperactivity disorder, CF, Child form, CHQ, Child health questionnaire, HR-QOL, Health related quality of life, ICC, Intraclass correlation coefficient, NF1, Neurofibromatosis type 1, PF, Parent form, QOL, Quality of life, SES, Socioeconomic status, TACQOL, TNO-AZL Child Quality of Life Questionnaire, TRF, Teacher's Report Form
To access this article, please choose from the options below
Supported by a grant from the Hersenstichting Nederland to L.C.K. and Y.E., by a grant from the Sophia Foundation for Medical Research to L.C.K., H.A.M and Y.E., and by a donation from the Dutch Neurofibromatosis Foundation (NFVN). The study sponsors had no role in the study design, the collection, analysis, and interpretation of data, the writing of the report, or the decision to submit the manuscript for publication. The authors declare no potential, real, or perceived conflicts of interest.
PII: S0022-3476(08)00760-9
doi:10.1016/j.jpeds.2008.08.045
© 2009 Mosby, Inc. All rights reserved.
