Psychosocial functioning and determinants of the health-related quality of life in children with neurofibromatosis type 1 and cognitive impairments

Belinda Barton, Faculty of Health, Southern Cross University, Coffs Harbour, NSW, Australia. belinda.barton@scu.edu.au.
Pamela L. Wolters, Pediatric Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
Karin S. Walsh, Center for Neuroscience and Behavioral Medicine, Division of Neuropsychology, Children's National Hospital, Washington, DC, USA.
Nicole J. Ullrich, Department of Neurology, Boston Children's Hospital, Boston, MA, USA.
Tena Rosser, Department of Neurology, Children's Hospital of Los Angeles, Los Angeles, CA, USA.
James Tonsgard, Division of Neurology, University of Chicago Medicine Comer Children's Hospital, Chicago, IL, USA.
David Viskochil, Department of Genetics, University of Utah, Salt Lake City, USA.
Elizabeth Schorry, Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Laura J. Klesse, Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, USA.
Michael J. Fisher, Division of Oncology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
David H. Gutmann, Department of Neurology, Washington University School of Medicine, St Louis, MO, USA.
Roger J. Packer, Gilbert Family Neurofibromatosis Institute and Brain Tumor Institute, Children's National Hospital, Washington, DC, USA.
Bruce Korf, Department of Genetics, University of Alabama at Birmingham, Birmingham, USA.
Maria T. Acosta, Undiagnosed Disease Program, Office of the Clinical Director, National Human Genome Research Institute, National Institute of Health, Bethesda, MD, USA.
Kathryn N. North, Murdoch Children's Research Institute, The Royal Children's Hospital, Parkville, VIC, Australia.
Jonathan M. Payne, Murdoch Children's Research Institute, The Royal Children's Hospital, Parkville, VIC, Australia.

Document Type

Journal Article

Publication Date

8-1-2025

Journal

Journal of neuro-oncology

Volume

174

Issue

1

DOI

10.1007/s11060-025-05024-x

Keywords

Children; Depression; Neurofibromatosis type 1; Psychosocial; Quality of life; Social stress

Abstract

PURPOSE: Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic condition associated with cutaneous and neoplastic manifestations and other physical manifestations, as well as cognitive, psychosocial, and behavioural difficulties. NF1 negatively impacts the health-related quality of life (HRQoL) of children. There is limited evidence regarding the determinants of HRQoL of children with NF1. The aim of this study was to (i) compare the HRQoL of children with NF1 and cognitive impairments to published data of healthy children and children with cancer and (ii) identify specific determinants of child and parent-proxy reports of psychosocial HRQoL. METHODS: Children with NF1 and cognitive impairments (n = 135, 8-15 years 11 months) and their parents completed standardized measures assessing children's HRQoL, behavioral and emotional functioning. Children completed a brief intelligence test. Correlations and multiple linear regressions were conducted to identify determinants of psychosocial HRQoL. RESULTS: Children with NF1 had significantly poorer HRQoL for all domains than published data of healthy children and significantly poorer HRQoL for Psychosocial Health, School and Social Functioning than published data of children with cancer. For child self-report, attention problems and increased social stress predicted their psychosocial HRQoL. For parent-proxy reports, activities of daily living and depression were significant predictors of children's psychosocial HRQoL. Social stress and depression were the strongest predictors of Psychosocial HRQoL. CONCLUSION: Routine screening and early identification of depressive symptoms and interventions that promote social support, coping and resiliency may improve the HRQoL of children with NF1.

Department

Psychiatry and Behavioral Sciences

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