Impact of contractures on daily functioning in adolescents with spinal muscular atrophy: a qualitative study

I L B Oude Lansink; J W Gorter; W L van der Pol; B Bartels; A Beelen

doi:10.1080/09638288.2023.2227953

Impact of contractures on daily functioning in adolescents with spinal muscular atrophy: a qualitative study

Disabil Rehabil. 2023 Jul 3:1-7. doi: 10.1080/09638288.2023.2227953. Online ahead of print.

Authors

I L B Oude Lansink^{1

2}, J W Gorter^{1

2

3}, W L van der Pol⁴, B Bartels⁵, A Beelen²

Affiliations

¹ Department of Rehabilitation, Physical Therapy Science & Sports, University Medical Center Utrecht, Wilhelmina Children's Hospital, Utrecht, The Netherlands.
² Center of Excellence for Rehabilitation Medicine, UMC Utrecht Brain Center, University Medical Center Utrecht, and De Hoogstraat Rehabilitation, Utrecht, The Netherlands.
³ CanChild, Department of Pediatrics, McMaster University, Hamilton, Canada.
⁴ Department of Neurology and Neurosurgery, UMC Utrecht Brain Center, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
⁵ Child Development and Exercise Center, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.

PMID: 37401093
DOI: 10.1080/09638288.2023.2227953

Abstract

Purpose: 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.

Methods: 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.

Results: 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.

Conclusion: 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.

Keywords: Spinal muscular atrophy; adolescents and young adults; contractures; daily functioning; treatment.

Plain language summary

The variability of the perceived impact of contractures in non-ambulant adolescents and young adults with spinal muscular atrophy (SMA) underscores the need for individualized assessment and contracture management based on discussions about challenges, benefits and burden.In the treatment plan for contracture management in SMA it is important to take into account: 1) that adolescents often experienced physiotherapy as additional comfort, with less feeling of stiffness, and 2) that most perceived orthosis and standers as unnecessary and burdensome as contracture management.Clinicians should consider postural management in an early stage given the improved prognosis on motor development of children with SMA.Clinicians should monitor and revisit treatment goals, benefits and adverse effects on a regular basis as part of the shared goal-setting and decision-making in contracture management for children with SMA.