Department of Health and Human Performance, The University of Scranton, Edward Leahy Hall Rm. 824, 800 Linden St, Scranton, PA, 18510, USA.
Department of Biomedical Sciences, Midwestern University, Glendale, AZ, USA.
Qual Life Res. 2022 Dec;31(12):3365-3375. doi: 10.1007/s11136-022-03192-1. Epub 2022 Jul 22.
Marfan syndrome (MFS) is a connective tissue disorder that affects skeletal, ocular, pulmonary, cardiovascular, and central nervous systems. Symptoms may lead to diminished quality of life (QoL) in individuals with MFS compared with healthy individuals. Currently, there is little evidence regarding the impact of MFS on swallowing and QoL. This study examined perceptions of swallowing difficulties and QoL among persons with MFS.
A total of 356 participants (1% response rate) with a self-reported diagnosis of MFS provided medication regime and completed a Quality-of-Life Index and a modified version of the SWAL-QOL used to assess 8 QOL concepts related to swallowing: burden, duration, desire, food choice, fear, mental health, social concerns, and fatigue.
Dysphagia symptoms were reported by 62% (N = 356) of the participants. Analyses assessing correlations between responses to SWAL-QOL questions, QoL scores, and reported medications were conducted. Further, responses on the SWAL-QOL predicted QoL satisfaction, even after controlling for medications. Findings revealed that greater swallowing difficulty affects QoL satisfaction and overall QoL, but not QoL importance. Further, specific medications were associated with differences in swallowing difficulty as well as QoL satisfaction.
The findings of the current study suggest that individuals with MFS may experience specific swallowing difficulties which impact QoL. Specific classes of drugs may also be associated with reported swallowing QoL and QoL satisfaction in MFS. These findings have implications for clinicians who work with individuals with MFS.
马凡综合征（MFS）是一种影响骨骼、眼睛、肺部、心血管和中枢神经系统的结缔组织疾病。与健康个体相比，MFS 患者的症状可能导致生活质量（QoL）下降。目前，关于 MFS 对吞咽和 QoL 的影响的证据很少。本研究调查了 MFS 患者对吞咽困难和 QoL 的看法。
共有 356 名（1%的回复率）自我报告患有 MFS 的参与者提供了药物治疗方案，并完成了生活质量指数和 SWAL-QOL 的修订版，用于评估与吞咽相关的 8 个 QOL 概念：负担、持续时间、欲望、食物选择、恐惧、心理健康、社会关注和疲劳。
62%（N=356）的参与者报告有吞咽困难症状。分析了评估 SWAL-QOL 问题答案、QoL 评分和报告药物之间相关性的结果。此外，即使在控制药物的情况下，SWAL-QOL 的回答也可以预测 QoL 满意度。研究结果表明，更大的吞咽困难程度会影响 QoL 满意度和整体 QoL，但不会影响 QoL 的重要性。此外，特定的药物与吞咽困难以及 QoL 满意度的差异有关。
本研究的结果表明，MFS 患者可能会经历特定的吞咽困难，从而影响 QoL。特定类别的药物也可能与 MFS 患者报告的吞咽 QoL 和 QoL 满意度有关。这些发现对与 MFS 患者合作的临床医生具有重要意义。
