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. 2015 Sep;167A(9):2103-13.
doi: 10.1002/ajmg.a.37123. Epub 2015 May 14.

Pain interference in youth with neurofibromatosis type 1 and plexiform neurofibromas and relation to disease severity, social-emotional functioning, and quality of life

Pamela L Wolters  1 , Katherine M Burns  1   2 , Staci Martin  1 , Andrea Baldwin  1 , Eva Dombi  1 , Mary Anne Toledo-Tamula  3 , William N Dudley  4 , Andrea Gillespie  1 , Brigitte C Widemann  1
Affiliations

Pain interference in youth with neurofibromatosis type 1 and plexiform neurofibromas and relation to disease severity, social-emotional functioning, and quality of life

Pamela L Wolters et al. Am J Med Genet A. 2015 Sep.

Abstract

The physical manifestations of neurofibromatosis type 1 (NF1) can cause chronic pain. This study investigated the impact of pain in youth with NF1 and plexiform neurofibromas (PNs) and its relationship to disease factors, social-emotional functioning, and quality of life (QOL) within a biopsychosocial framework. Caregivers of 59 children and adolescents with NF1 and PNs (6-18 years), and 41 of these youth (10-18 years), completed questionnaires assessing social-emotional functioning and QOL, including an item on pain interference. Measures of disease severity included total PN volume by percent body weight and number of disease complications. Both caregiver (73%) and self-report (59%) ratings indicated that pain interferes with the child's daily functioning despite 33% taking pain medication. Based on caregivers' behavior ratings, more symptoms of anxiety and larger tumor volumes predicted greater pain interference, while greater pain interference, worse depressive symptoms, and more disease complications predicted poorer QOL. As rated by adolescents, more symptoms of anxiety predicted greater pain interference, while greater pain interference and social stress predicted poorer QOL. Further, social-emotional problems mediate the relationship between pain interference and QOL. Thus, pain interferes with daily functioning in the majority of youth with NF1 and PNs even when using pain medication. The impact of pain interference, disease severity, and particularly social-emotional problems on QOL highlights the interaction between physical and psychological states in NF1. Future research and treatment of pain in this population should utilize a biopsychosocial approach and involve multidisciplinary therapies including psychological interventions that target social-emotional functioning.

Keywords: children; neurofibromatosis type 1; pain; plexiform neurofibromas; quality of life.

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Conflict of interest statement

The authors have no conflicts of interest to disclose.

Figures

FIG. 1.
FIG. 1.
Mediation model of the indirect effects of pain interference on overall QOL via depressive symptoms as rated by caregivers.
FIG. 2.
FIG. 2.
Mediation model of the indirect effects of pain interference on overall QOL via social stress as rated by adolescents.
See this image and copyright information in PMC

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