Identification of wheelchair seating criteria in adults with neuromuscular diseases: A Delphi study

PLOS ONE RESEARCH ARTICLE Identification of wheelchair seating criteria in adults with neuromuscular diseases: A Delphi study Elise Dupitier ID1,2,3*, Manon Voisin4, Caroline Stalens3, Pascal Laforêt2, Samuel Pouplin1 1 UR2020 ERPHAN, Paramedical Research Team in Neuromuscular Disability, Paris—Saclay University, University Hospital Site of Raymond Poincaré, Garches, France, 2 UFR Simone Veil-Santé, UMR 1179 EndiCap, Paris—Saclay University, University Hospital Site of Raymond Poincaré, Garches, France, 3 Medical Department, AFM-Téléthon, Evry, France, 4 National Invalid Institution, Paris, France a1111111111 a1111111111 a1111111111 a1111111111 a1111111111 * Abstract Background OPEN ACCESS Citation: Dupitier E, Voisin M, Stalens C, Laforêt P, Pouplin S (2023) Identification of wheelchair seating criteria in adults with neuromuscular diseases: A Delphi study. PLoS ONE 18(9): e0290627. https://doi.org/10.1371/journal. pone.0290627 Editor: Claudia Brogna, Fondazione Policlinico Universitario Gemelli IRCCS, ITALY Received: March 23, 2023 Accepted: August 13, 2023 Published: September 8, 2023 Copyright: © 2023 Dupitier et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Data Availability Statement: Data is accessible here: Dupitier, Elise et al. (2023), BPIP-MNM consensus by Delphi, Dryad, Dataset, https://doi. org/10.5061/dryad.h70rxwdq7. Funding: Funded studies: AFM-Téléthon: https:// www.afm-telethon.fr/fr Positi’F: https://www. positif-mobilite.fr/ NO - Include this sentence at the end of your statement: The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. Adults with neuromuscular diseases like spinal muscular atrophy or Duchenne muscular dystrophy require full-time use of a wheelchair (WC) and perform all activities of daily living in a sitting position. Optimal configuration of the WC and seating system is essential to maintain the health and quality of life of users. However, few recommendations for configuration exist. The aim of this study was to identify and select 10 WC seating criteria that ensure an optimal sitting posture for health and quality of life. Methods A four round Delphi method was used to collect the opinions of WC users and health professionals (HP), separately. First, the HP were asked if they believed that different criteria would apply to each disease. Then the HP and SMA II and DMD WC user experts responded to electronic surveys in 4 rounds. Results Overall, 74 experts took part: 31 HP, 21 WC users with SMA II and 22 WC users with DMD. In total, 52% of HP believed that different criteria would apply to each disease. Ten criteria were identified by the HP for SMA II and 10 for DMD. Of the 40 criteria selected, 30 (75%) were common to each panel. Six topics were similar across panels: comfort, access to the joystick, prevention of pain, stability, pressure management and power seat functions. However, power seat functions did not reach consensus between HP and WC users (30–33% of agreement for HP and 93–100% for the WC user panels, p < 0.001). Conclusion Adults with SMA II and DMD had similar WC seating needs. Therefore, the same recommendations can be applied to these groups. Further research is necessary to understand the impact of cost on the prescription of power seat functions by health professionals. PLOS ONE | https://doi.org/10.1371/journal.pone.0290627 September 8, 2023 1 / 19 PLOS ONE Competing interests: The authors have declared that no competing interests exist. Wheelchair seating in neuromuscular diseases Introduction Neuromuscular diseases (NMD), such as spinal muscular atrophy type II (SMA II) and Duchenne muscular dystrophy (DMD), are rare genetic diseases. SMA is an autosomal recessive neurodegenerative disorder caused by a defect in the survival motor neuron (SMN1). Type II is an intermediate form, with a prevalence of 2 per 100 000 [1]. DMD is an X-linked disorder that results from a mutation in the dystrophin gene, with a prevalence of 9.9 per 100 000 [1]. The fulltime use of a powered wheelchair (WC) is necessary for people with SMA II from the normal age of walking achievement [2] and for people with DMD from the age of 10 to 13 years [3]. Before the advent of new therapies for SMA, i.e., in 2017 in France, children with SMA II who could sit independently lost this ability, along with head control, in adulthood. Similarly, adults with DMD lose independent sitting and head control over time. In addition to loss of ambulation, these diseases cause scoliosis and muscle contractures [2,4], which impact on sitting posture, comfort in the WC [5] and performance of activities of daily living. Scoliosis is a common consequence of other neuromuscular disorders such as spinal cord injury, cerebral palsy and spina bifida [6]. However, the prevalence of scoliosis is particularly high in DMD and SMA: 60–90% in SMA and 90% in DMD, without glucocorticoid therapy [2,7]. Most affected individuals undergo instrumented spinal surgery to correct or prevent worsening of scoliosis. Pelvic alignment disorders, and trunk and head collapse are also frequent. Intrinsic myotendinous structural changes and extrinsic factors often cause joint contractures around the hips, knees, elbows and wrists in both SMA and DMD [8]. Furthermore, prolonged WC use causes other comorbidities such as chronic pain and pressure injuries [9– 13]. Pain in DMD is mainly ischial or around spinal deformity sites [9,11]. In SMA II, pain is mainly in the neck, back and legs. Poor sitting posture is considered as the largest pain exacerbating factor and changing position as the best pain relieving strategy [14,15]. The benefits of WC use on mobility are undeniable; however, the large number of hours spent in a sitting position each day has adverse effects on health and quality of life [16,17]. For example, in DMD, a high risk of pressure injuries, which increases with age, has been reported [9,11]. The type of cushion, backrest, seating components such as posterior or lateral supports or belts, as well as the WC size and parameters can either improve sitting tolerance and social participation [18,19] or can actually have a negative impact on the person’s life [20,21]. Three interventional studies have been published on the effects of wheelchair seating, 2 in DMD [22,23] and 1 in SMA II [24]. They report contradictory and sometimes controversial results for the influence of postural devices on pulmonary function in DMD, whereas a positive effect was found for SMA II. They also showed that postural support devices, like an individually selected cushion, backrest, upper limb and trunk support and headrest, improve upper limb function in both diseases and improve posture in DMD. International reco (...truncated)