Department of Rehabilitation, Physical Therapy Science & Sports, University Medical Center Utrecht, Wilhelmina Children's Hospital, Utrecht, The Netherlands.
Center of Excellence for Rehabilitation Medicine, UMC Utrecht Brain Center, University Medical Center Utrecht, and De Hoogstraat Rehabilitation, Utrecht, The Netherlands.
Disabil Rehabil. 2024 Jun;46(12):2593-2599. doi: 10.1080/09638288.2023.2227953. Epub 2023 Jul 3.
Exploring the impact of contractures in adolescents and young adults (AYA) with spinal muscular atrophy (SMA) on daily functioning and participation and the impact of received contracture management.
We included 14, non-ambulant AYA with SMA types 2/3 (10 females and 4 males), aged 16-30 years. Interviews focused on two topics: perceived impact of contractures on daily functioning and of previous contracture management. We used inductive thematic analysis for interview analysis.
In general, participants experienced muscle weakness to be more of a hindrance than contractures; they had adapted to their contractures over time. Participants considered contracture treatment useful when goals were meaningful and realistic. Participants mentioned that their perspective on contracture management would change in light of a promise of improved motor function due to disease-modifying treatment.
Despite the relatively low impact of contractures in comparison to the loss of muscle strength, non-ambulant AYA with SMA should be informed on the potential impact of contractures and benefits and potential adverse effects of their management. This information can support the shared decision-making process. While respecting individual choices, allows for incorporating interventions into daily life and the promotion of daily functioning and participation when children with SMA are growing up.
探讨脊柱肌萎缩症（SMA）青少年和年轻成人（AYA）的挛缩对日常功能和参与的影响，以及接受挛缩管理的影响。
我们纳入了 14 名非卧床 SMA 型 2/3 的 AYA（10 名女性和 4 名男性），年龄 16-30 岁。访谈集中在两个主题：挛缩对日常功能的感知影响和以前的挛缩管理。我们使用归纳主题分析进行访谈分析。
一般来说，参与者认为肌肉无力比挛缩更碍事；他们随着时间的推移已经适应了挛缩。当目标有意义且现实时，参与者认为挛缩治疗有用。参与者提到，由于疾病修正治疗有望改善运动功能，他们对挛缩管理的看法会发生变化。
尽管与肌肉力量丧失相比，挛缩的影响相对较小，但应该向非卧床 SMA AYA 告知挛缩的潜在影响以及管理的益处和潜在的不良反应。这一信息可以支持共同决策过程。在尊重个人选择的同时，允许将干预措施纳入日常生活，并在 SMA 患儿成长过程中促进日常功能和参与。
