Abstract：
OBJECTIVE: Neurofibromatosis (NF) is an incurable genetic neurological condition. Psychosocial interventions that promote resiliency are a promising approach to address the high emotional distress and low quality of life (QoL) associated with NF. However, no studies have examined the psychosocial needs of treatment-seeking adults with NF. Our goal was to explore, using data from the largest efficacy trial of a psychosocial intervention for NF, differences in QoL, emotional distress, resiliency, and pain-related outcomes compared to other chronic medical populations and within subtypes (NF1, NF2, schwannomatosis; SCHW).
METHODS: Enrolled participants (N = 228) were geographically diverse adults with NF and elevated stress. We performed secondary analysis on baseline measures of QoL, emotional distress, resiliency, and pain-related outcomes. We reported descriptive statistics and normative comparisons to understand the psychosocial characteristics of the overall sample and performed between-group analyses to explore differences within NF type.
RESULTS: Our sample endorsed worse QoL, emotional distress, resilience, and pain-related outcomes than similar chronic illness populations. Within NF types, participants with NF1 reported lower QoL and resilience compared to those with NF2. Participants with SCHW reported higher pain intensity than those with NF1. Participants with SCHW reported higher pain interference and lower physical QoL compared to those with NF1 and NF2.
CONCLUSIONS: Our findings support the urgent need for psychosocial interventions targeting deficits in QoL, emotional distress, resilience, and pain-related outcomes in adults with NF. We recommend efforts to enhance sample diversity, prepare clinicians to provide high-levels of support, and attune skills training to each NF type.
BACKGROUND: ClinicalTrials.gov NCT03406208; https://clinicaltrials.gov/ct2/show/NCT03406208 (Archived by WebCite at http://www.webcitation.org/72ZoTDQ6h ).
METHODS: Enrolled participants (N = 228) were geographically diverse adults with NF and elevated stress. We performed secondary analysis on baseline measures of QoL, emotional distress, resiliency, and pain-related outcomes. We reported descriptive statistics and normative comparisons to understand the psychosocial characteristics of the overall sample and performed between-group analyses to explore differences within NF type.
RESULTS: Our sample endorsed worse QoL, emotional distress, resilience, and pain-related outcomes than similar chronic illness populations. Within NF types, participants with NF1 reported lower QoL and resilience compared to those with NF2. Participants with SCHW reported higher pain intensity than those with NF1. Participants with SCHW reported higher pain interference and lower physical QoL compared to those with NF1 and NF2.
CONCLUSIONS: Our findings support the urgent need for psychosocial interventions targeting deficits in QoL, emotional distress, resilience, and pain-related outcomes in adults with NF. We recommend efforts to enhance sample diversity, prepare clinicians to provide high-levels of support, and attune skills training to each NF type.
BACKGROUND: ClinicalTrials.gov NCT03406208; https://clinicaltrials.gov/ct2/show/NCT03406208 (Archived by WebCite at http://www.webcitation.org/72ZoTDQ6h ).
摘要：
目的：神经纤维瘤病（NF）是一种无法治愈的遗传性神经系统疾病。促进弹性的心理社会干预是解决与NF相关的高情绪困扰和低生活质量（QoL）的有希望的方法。然而,尚无研究对寻求治疗的NF患者的心理社会需求进行了研究.我们的目标是探索，使用来自NF的心理社会干预的最大功效试验的数据，QoL的差异，情绪困扰,弹性，与其他慢性医学人群和亚型（NF1，NF2，神经鞘瘤病；SCHW）相比，疼痛相关结局。
方法：注册参与者（N=228）是具有NF和压力升高的不同地域的成年人。我们对QoL的基线测量进行了二次分析，情绪困扰,弹性，和疼痛相关的结果。我们报告了描述性统计和规范比较，以了解整个样本的社会心理特征，并进行了组间分析以探索NF类型内的差异。
结果：我们的样本认可了较差的QoL，情绪困扰,弹性，和疼痛相关的结局比类似的慢性病人群。在NF类型中，与NF2参与者相比，NF1参与者的QoL和弹性较低。SCHW患者的疼痛强度高于NF1患者。与NF1和NF2患者相比，SCHW患者的疼痛干扰更高，身体QoL更低。
结论：我们的研究结果支持迫切需要针对QoL缺陷的心理社会干预措施，情绪困扰,弹性，和成人NF患者的疼痛相关结局。我们建议努力提高样本多样性，准备临床医生提供高水平的支持，并对每种NF类型进行技能培训。
背景：ClinicalTrials.govNCT03406208;https://clinicaltrials.gov/ct2/show/NCT03406208（由WebCite在http://www上存档。webcitation.org/72ZoTDQ6h)。
方法：注册参与者（N=228）是具有NF和压力升高的不同地域的成年人。我们对QoL的基线测量进行了二次分析，情绪困扰,弹性，和疼痛相关的结果。我们报告了描述性统计和规范比较，以了解整个样本的社会心理特征，并进行了组间分析以探索NF类型内的差异。
结果：我们的样本认可了较差的QoL，情绪困扰,弹性，和疼痛相关的结局比类似的慢性病人群。在NF类型中，与NF2参与者相比，NF1参与者的QoL和弹性较低。SCHW患者的疼痛强度高于NF1患者。与NF1和NF2患者相比，SCHW患者的疼痛干扰更高，身体QoL更低。
结论：我们的研究结果支持迫切需要针对QoL缺陷的心理社会干预措施，情绪困扰,弹性，和成人NF患者的疼痛相关结局。我们建议努力提高样本多样性，准备临床医生提供高水平的支持，并对每种NF类型进行技能培训。
背景：ClinicalTrials.govNCT03406208;https://clinicaltrials.gov/ct2/show/NCT03406208（由WebCite在http://www上存档。webcitation.org/72ZoTDQ6h)。
