Practical problems and management of seating through the clinical stages of Duchenne’s muscular dystrophy11No commercial party having a direct financial interest in the results of the research supporting this article has or will confer a benefit upon the author(s) or upon any organization with which the author(s) is/are associated.

Liu, Meigen; Mineo, Kiyoshi; Hanayama, Kozo; Fujiwara, Toshiyuki; Chino, Naoichi

doi:10.1016/s0003-9993(02)04953-5

Cited by 34 publications

(7 citation statements)

References 12 publications

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“…Studies have reported a prevalence of WC related pain or discomfort between 31% and 41% in WC users with DMD and 86% in WC users with NMD and muscular diseases [9,11,13,38]. The topography of the pain is mainly the posterior thigh, the ischial areas and lateral thoracic areas [9,11], with a higher prevalence for worse disability stages. The WC users with SMA II and the HP for DMD used these keywords in relation to the long daily duration of sitting.…”

Section: Plos Onementioning

confidence: 99%

“…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][10][11][12][13]. Pain in DMD is mainly ischial or around spinal deformity sites [9,11].…”

Section: Introductionmentioning

confidence: 99%

“…Furthermore, prolonged WC use causes other comorbidities such as chronic pain and pressure injuries [9][10][11][12][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.…”

Section: Introductionmentioning

confidence: 99%

“…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].…”

Section: Introductionmentioning

confidence: 99%

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

Dupitier,

Voisin,

Stalens

et al. 2023

PLoS ONE

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Background 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.

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Section: Plos Onementioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

Dupitier,

Voisin,

Stalens

et al. 2023

PLoS ONE

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“…55 to 90% of the cases [4], [5], [6], [7]. Kyphoscoliotic deformities of the spinal column and chest wall [8], [9], [10], reduced mobility along with ongoing necrosis in the respiratory muscles contribute to the reduced vital capacity [11], [12], hypoxemia and hypercapnia [8] noted in DMD patients with respiratory insufficiency. While extremely important from a patho-physiological viewpoint, the possible contribution of chronic hypoxia (CH) to disease progression in DMD patients is not fully understood.…”

Section: Introductionmentioning

confidence: 99%

Chronic Hypoxia Impairs Muscle Function in the Drosophila Model of Duchenne's Muscular Dystrophy (DMD)

et al. 2010

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Duchenne's muscular dystrophy (DMD) is a severe progressive myopathy caused by mutations in the DMD gene leading to a deficiency of the dystrophin protein. Due to ongoing muscle necrosis in respiratory muscles late-stage DMD is associated with respiratory insufficiency and chronic hypoxia (CH). To understand the effects of CH on dystrophin-deficient muscle in vivo, we exposed the Drosophila model for DMD (dmDys) to CH during a 16-day ascent to the summit of Mount Denali/McKinley (6194 meters above sea level). Additionally, dmDys and wild type (WT) flies were also exposed to CH in laboratory simulations of high altitude hypoxia. Expression profiling was performed using Affymetrix GeneChips® and validated using qPCR. Hypoxic dmDys differentially expressed 1281 genes, whereas the hypoxic WT flies differentially expressed 56 genes. Interestingly, a number of genes (e.g. heat shock proteins) were discordantly regulated in response to CH between dmDys and WT. We tested the possibility that the disparate molecular responses of dystrophin-deficient tissues to CH could adversely affect muscle by performing functional assays in vivo. Normoxic and CH WT and dmDys flies were challenged with acute hypoxia and time-to-recover determined as well as subjected to climbing tests. Impaired performance was noted for CH-dmDys compared to normoxic dmDys or WT flies (rank order: Normoxic-WT ≈ CH-WT> Normoxic-dmDys> CH-dmDys). These data suggest that dystrophin-deficiency is associated with a disparate, pathological hypoxic stress response(s) and is more sensitive to hypoxia induced muscle dysfunction in vivo. We hypothesize that targeting/correcting the disparate molecular response(s) to hypoxia may offer a novel therapeutic strategy in DMD.

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