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.
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.
Authors: Mariska van Lier; M Hadi Saiepour; Koen Kole; Juliette E Cheyne; Nawal Zabouri; Thomas Blok; Yi Qin; Emma Ruimschotel; J Alexander Heimel; Christian Lohmann; Christiaan N Levelt Journal: J Neurosci Date: 2020-06-11 Impact factor: 6.167
Authors: J B Lane; H-S Lee; L W Smith; P Cheng; A K Percy; D G Glaze; J L Neul; K J Motil; J O Barrish; S A Skinner; F Annese; L McNair; J Graham; O Khwaja; K Barnes; J P Krischer Journal: Neurology Date: 2011-10-19 Impact factor: 9.910
Authors: Pamela L Wolters; Katherine M Burns; Staci Martin; Andrea Baldwin; Eva Dombi; Mary Anne Toledo-Tamula; William N Dudley; Andrea Gillespie; Brigitte C Widemann Journal: Am J Med Genet A Date: 2015-05-14 Impact factor: 2.802
Authors: Thijs van der Vaart; André B Rietman; Ellen Plasschaert; Eric Legius; Ype Elgersma; Henriëtte A Moll Journal: Neurology Date: 2015-10-30 Impact factor: 9.910
Authors: Melissa K Cousino; Rebecca Hazen; Katherine Leigh Josie; Kelly Laschinger; Peter de Blank; H Gerry Taylor Journal: J Clin Psychol Med Settings Date: 2017-12