Wolters Pamela L, Burns Katherine M, Martin Staci, Baldwin Andrea, Dombi Eva, Toledo-Tamula Mary Anne, Dudley William N, Gillespie Andrea, Widemann Brigitte C
Pediatric Oncology Branch, National Cancer Institute, Bethesda, Maryland.
Department of Psychology, The George Washington University, Washington, District of Columbia.
Am J Med Genet A. 2015 Sep;167A(9):2103-13. doi: 10.1002/ajmg.a.37123. Epub 2015 May 14.
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.
1型神经纤维瘤病（NF1）的身体表现可导致慢性疼痛。本研究在生物心理社会框架内，调查了NF1合并丛状神经纤维瘤（PNs）的青少年疼痛的影响及其与疾病因素、社会情感功能和生活质量（QOL）的关系。59名患有NF1和PNs的儿童及青少年（6 - 18岁）的照料者，以及其中41名青少年（10 - 18岁）完成了评估社会情感功能和生活质量的问卷，包括一项关于疼痛干扰的项目。疾病严重程度的测量指标包括按体重百分比计算的总PN体积和疾病并发症数量。尽管33%的人服用了止痛药，但照料者（73%）和自我报告（59%）的评分均表明疼痛会干扰孩子的日常功能。根据照料者的行为评分，更多的焦虑症状和更大的肿瘤体积预示着更大的疼痛干扰，而更大的疼痛干扰、更严重的抑郁症状和更多的疾病并发症预示着更差的生活质量。青少年的评分显示，更多的焦虑症状预示着更大的疼痛干扰，而更大的疼痛干扰和社会压力预示着更差的生活质量。此外，社会情感问题介导了疼痛干扰与生活质量之间的关系。因此，即使使用了止痛药，疼痛仍会干扰大多数患有NF1和PNs的青少年的日常功能。疼痛干扰、疾病严重程度，尤其是社会情感问题对生活质量的影响突出了NF1中身体和心理状态之间的相互作用。未来针对该人群疼痛的研究和治疗应采用生物心理社会方法，并涉及多学科治疗，包括针对社会情感功能的心理干预。
