Current status and influencing factor analysis of decision-making conflicts regarding disease-modifying therapies in adult-type spinal muscular atrophy patients_West China Medical Journal

Authors：

HU Linxue , YI Xiaojiang , GAO Xia , LIANG Yan ,  CHEN Dezhi

Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding author：

CHEN Dezhi, Email: 737238683@qq.com

Keywords：

Adult-type; spinal muscular atrophy; disease-modifying therapy; decision conflict; influencing factors

DOI：

10.7507/1002-0179.202511348

Video：

Export PDF Favorites Scan Get Citation

Abstract Full text Figures/Tables Video References Cited by

Objective To investigate the current status and influencing factors of decision conflict regarding disease-modifying therapy in patients with spinal muscular atrophy (SMA), aiming to provide references for future interventions to improve the quality of patient decision-making. Methods Using convenience sampling, SMA patients admitted to the Department of Neurology, West China Hospital of Sichuan University between January 2023 and October 2025 were selected as study participants. Data were collected through questionnaires assessing general demographic characteristics, the Control Preferences Scale, and the Decisional Conflict Scale (DCS). Univariate analysis and multiple linear regression were employed to explore factors influencing patients’ decisional conflict. Results A total of 70 questionnaires were distributed, with 66 valid responses returned, resulting in an effective response rate of 94.3%. The standardized DCS score of SMA patients was 35.61±21.28, indicating moderate decisional conflict. Multiple linear regression analysis revealed that age [unstandardized partial regression coefficient (b)=−0.818, 95% confidence interval (−1.383, −0.252), P=0.005] and residential location [b=−12.660, 95% confidence interval (−19.822, −5.498), P=0.001] were independent influencing factors for decisional conflict, collectively explaining 35.0% of the variance (adjusted R²=0.318). Conclusions SMA patients exhibit moderate levels of disease-modifying therapy-related decisional conflict, with younger age and rural residence associated with more pronounced conflict. Clinical practice should focus on individual patient characteristics while enhancing decision aids and informational support to help reduce decisional uncertainty.

Citation： HU Linxue, YI Xiaojiang, GAO Xia, LIANG Yan, CHEN Dezhi. Current status and influencing factor analysis of decision-making conflicts regarding disease-modifying therapies in adult-type spinal muscular atrophy patients. West China Medical Journal, 2026, 41(1): 33-37. doi: 10.7507/1002-0179.202511348 Copy

1.	北京医学会罕见病分会, 北京医学会医学遗传学分会, 北京医学会神经病学分会神经肌肉病学组, 等. 脊髓性肌萎缩症多学科管理专家共识. 中华医学杂志, 2019, 99(19): 1460-1467.
2.	Mercuri E, Finkel RS, Muntoni F, et al. Diagnosis and management of spinal muscular atrophy: Part 1: recommendations for diagnosis, rehabilitation, orthopedic and nutritional care. Neuromuscul Disord, 2018, 28(2): 103-115.
3.	中华医学会医学遗传学分会遗传病临床实践指南撰写组. 脊髓性肌萎缩症的临床实践指南. 中华医学遗传学杂志, 2020, 37(3): 263-268.
4.	中国研究型医院学会罕见病分会, 中国罕见病联盟, 北京罕见病诊疗与保障学会, 等. 青少年成人脊髓性肌萎缩症临床诊疗指南. 罕见病研究, 2023, 2(1): 70-84.
5.	刘珊, 熊晖. 脊髓性肌萎缩症的疾病修正治疗. 中华实用儿科临床杂志, 2023, 38(6): 465-468.
6.	Janis IL, Mann L. Decision making: a psychological analysis of conflict, choice, and commitment. New York: Free Press, 1977.
7.	廖宗峰, 金迪, 梅翠红, 等. 患者决策冲突的研究进展. 护理学杂志, 2018, 33(12): 106-109.
8.	Heirman AN, Arends CR, de Jel DVC, et al. Decisional conflict and decision regret in head and neck oncology: a systematic review and meta-analysis. JAMA Otolaryngol Head Neck Surg, 2024, 150(5): 393-404.
9.	O’Connor AM. Validation of a decisional conflict scale. Med Decis Making, 1995, 15(1): 25-30.
10.	王露, 陈英, 崔金锐, 等. 中文版决策冲突量表在直肠癌患者术式决策中应用的信效度检验. 护理学杂志, 2019, 34(3): 31-34.
11.	Degner LF, Sloan JA, Venkatesh P. The control preferences scale. Can J Nurs Res, 1997, 29(3): 21-43.
12.	徐小琳. 患者对医疗决策参与的满意度量表的编制及信效度考评. 长沙: 中南大学, 2010.
13.	Heirman AN, de Kort DP, Petersen JF, et al. Decisional conflict in patients with advanced laryngeal carcinoma: a multicenter study. Laryngoscope, 2024, 134(8): 3604-3610.
14.	Sweeney J, Tichnell C, Christian S, et al. Characterizing decision-making surrounding exercise in ARVC: analysis of decisional conflict, decisional regret, and shared decision-making. Circ Genom Precis Med, 2023, 16(6): e004133.
15.	毛姗姗, 冯艺杰, 徐璐, 等. 诺西那生钠修正治疗儿童脊髓性肌萎缩症随访分析. 中华儿科杂志, 2022, 60(7): 688-693.
16.	诸丹蕙, 颜悦, 毛姗姗. 儿童脊髓性肌萎缩症的疾病修正治疗进展. 临床药物治疗杂志, 2025, 23(9): 8-14.
17.	张青月, 张秀丽, 王燕, 等. 即刻乳房再造乳腺癌患者决策冲突与人格特质的调查. 中国实用护理杂志, 2024, 40(19): 1484-1490.
18.	Liao YL, Wang TJ, Su CW, et al. Efficacy of a decision support intervention on decisional conflict related to hepatocellular cancer treatment: a randomized controlled trial. Clin Nurs Res, 2023, 32(1): 233-243.
19.	郭红涛, 张静, 李颖, 等. 共享决策辅助方案对白血病患儿父母决策冲突与疾病不确定感的影响. 护理研究, 2023, 37(12): 2227-2232.
20.	张涵, 吴志新, 朱雪萍. 脊髓性肌萎缩症的临床特征及基因治疗研究进展. 中华神经医学杂志, 2025, 24(3): 293-298.
21.	Yuan X, Yang Y, Wang C, et al. Decision-making conflicts regarding hematopoietic stem cell transplantation in patients with hematological neoplasms: a descriptive qualitative study. Eur J Oncol Nurs, 2024, 72: 102684.

1. 北京医学会罕见病分会, 北京医学会医学遗传学分会, 北京医学会神经病学分会神经肌肉病学组, 等. 脊髓性肌萎缩症多学科管理专家共识. 中华医学杂志, 2019, 99(19): 1460-1467.
2. Mercuri E, Finkel RS, Muntoni F, et al. Diagnosis and management of spinal muscular atrophy: Part 1: recommendations for diagnosis, rehabilitation, orthopedic and nutritional care. Neuromuscul Disord, 2018, 28(2): 103-115.
3. 中华医学会医学遗传学分会遗传病临床实践指南撰写组. 脊髓性肌萎缩症的临床实践指南. 中华医学遗传学杂志, 2020, 37(3): 263-268.
4. 中国研究型医院学会罕见病分会, 中国罕见病联盟, 北京罕见病诊疗与保障学会, 等. 青少年成人脊髓性肌萎缩症临床诊疗指南. 罕见病研究, 2023, 2(1): 70-84.
5. 刘珊, 熊晖. 脊髓性肌萎缩症的疾病修正治疗. 中华实用儿科临床杂志, 2023, 38(6): 465-468.
6. Janis IL, Mann L. Decision making: a psychological analysis of conflict, choice, and commitment. New York: Free Press, 1977.
7. 廖宗峰, 金迪, 梅翠红, 等. 患者决策冲突的研究进展. 护理学杂志, 2018, 33(12): 106-109.
8. Heirman AN, Arends CR, de Jel DVC, et al. Decisional conflict and decision regret in head and neck oncology: a systematic review and meta-analysis. JAMA Otolaryngol Head Neck Surg, 2024, 150(5): 393-404.
9. O’Connor AM. Validation of a decisional conflict scale. Med Decis Making, 1995, 15(1): 25-30.
10. 王露, 陈英, 崔金锐, 等. 中文版决策冲突量表在直肠癌患者术式决策中应用的信效度检验. 护理学杂志, 2019, 34(3): 31-34.
11. Degner LF, Sloan JA, Venkatesh P. The control preferences scale. Can J Nurs Res, 1997, 29(3): 21-43.
12. 徐小琳. 患者对医疗决策参与的满意度量表的编制及信效度考评. 长沙: 中南大学, 2010.
13. Heirman AN, de Kort DP, Petersen JF, et al. Decisional conflict in patients with advanced laryngeal carcinoma: a multicenter study. Laryngoscope, 2024, 134(8): 3604-3610.
14. Sweeney J, Tichnell C, Christian S, et al. Characterizing decision-making surrounding exercise in ARVC: analysis of decisional conflict, decisional regret, and shared decision-making. Circ Genom Precis Med, 2023, 16(6): e004133.
15. 毛姗姗, 冯艺杰, 徐璐, 等. 诺西那生钠修正治疗儿童脊髓性肌萎缩症随访分析. 中华儿科杂志, 2022, 60(7): 688-693.
16. 诸丹蕙, 颜悦, 毛姗姗. 儿童脊髓性肌萎缩症的疾病修正治疗进展. 临床药物治疗杂志, 2025, 23(9): 8-14.
17. 张青月, 张秀丽, 王燕, 等. 即刻乳房再造乳腺癌患者决策冲突与人格特质的调查. 中国实用护理杂志, 2024, 40(19): 1484-1490.
18. Liao YL, Wang TJ, Su CW, et al. Efficacy of a decision support intervention on decisional conflict related to hepatocellular cancer treatment: a randomized controlled trial. Clin Nurs Res, 2023, 32(1): 233-243.
19. 郭红涛, 张静, 李颖, 等. 共享决策辅助方案对白血病患儿父母决策冲突与疾病不确定感的影响. 护理研究, 2023, 37(12): 2227-2232.
20. 张涵, 吴志新, 朱雪萍. 脊髓性肌萎缩症的临床特征及基因治疗研究进展. 中华神经医学杂志, 2025, 24(3): 293-298.
21. Yuan X, Yang Y, Wang C, et al. Decision-making conflicts regarding hematopoietic stem cell transplantation in patients with hematological neoplasms: a descriptive qualitative study. Eur J Oncol Nurs, 2024, 72: 102684.

West China Medical Journal

Current status and influencing factor analysis of decision-making conflicts regarding disease-modifying therapies in adult-type spinal muscular atrophy patients

Abstract Full text Figures/Tables Video References Cited by

Previous Article

Next Article

Format

Content