Fatigue in facioscapulohumeral muscular dystrophy: a qualitative study of people’s experiences

Schipper, Kerstin; Bakker, Minne; Abma, Tineke

doi:10.1080/09638288.2016.1212109

Cited by 18 publications

(22 citation statements)

References 39 publications

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“…In FSHD, fatigue occurs as an early leitmotif of the disease and as a disabling symptom in daily activities; in a recent qualitative study, patients described fatigue as “an overwhelming and unpredictable experience” without recognizing the underlying causes, making it difficult to cope with and thus massively affecting participation, social contacts, and quality of life (Schipper et al 2017 ). Furthermore, in a survey involving 328 participants with FSHD, one of the symptoms with the highest prevalence was fatigue (93.8%) (Hamel et al 2019 ).…”

Section: Introductionmentioning

confidence: 99%

Increased resistance towards fatigability in patients with facioscapulohumeral muscular dystrophy

et al. 2021

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Purpose In facioscapulohumeral muscular dystrophy (FSHD) fatigue is a major complaint. We aimed to investigate whether during isometric sustained elbow flexions, performance fatigability indexes differ in patients with FSHD with respect to healthy controls. Methods Seventeen patients with FSHD and seventeen healthy controls performed two isometric flexions of the dominant biceps brachii at 20% of their maximal voluntary contraction (MVC) for 2 min and then at 60% MVC until exhaustion. Muscle weakness was characterized as a percentage of predicted values. Maximal voluntary strength, endurance time and performance fatigability indices (mean frequency of the power spectrum (MNF), muscle fiber conduction velocity (CV) and fractal dimension (FD)), extracted from the surface electromyogram signal (sEMG) were compared between the two groups. Results In patients with FSHD, maximal voluntary strength was 68.7% of predicted value (p < 0.01). Compared to healthy controls, FSHD patients showed reduced MVC (p < 0.001; r = 0.62) and lower levels of performance fatigability, characterized by reduced rate of changes in MNF (p < 0.01; r = 0.56), CV (p < 0.05; 0.37) and FD (p < 0.001; r = 0.51) and increased endurance time (p < 0.001; r = 0.63), during the isometric contraction at 60% MVC. Conclusion A decreased reduction in the slopes of all the considered sEMG parameters during sustained isometric elbow flexions suggests that patients with FSHD experience lower levels of performance fatigability compared to healthy controls.

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Section: Introductionmentioning

confidence: 99%

Increased resistance towards fatigability in patients with facioscapulohumeral muscular dystrophy

et al. 2021

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“…Regarding the motor base of breathing, measured in the acoustic analysis by the maximum phonation time (MPT), patients with FSHD presented values well below normal and lower than the control group 18 . One hypothesis is that this reduction in maximum phonation time may be related to muscle fatigue and weakness 8 , 25 , 26 . FSHD patients have respiratory muscle weakness as an intrinsic characteristic of the disease, which can involve the diaphragm and expiratory abdominal muscles 27 .…”

Section: Discussionmentioning

confidence: 99%

Speech and swallowing characteristics in patients with facioscapulohumeral muscular dystrophy

Santos

Saute

Jacinto-Scudeiro

et al. 2022

Arq. Neuro-Psiquiatr.

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Background: Although facial muscle weakness is common in patients with Facioscapulohumeral Muscular Dystrophy (FSHD), the literature is scarce on the speech and swallowing aspects. Objective: To investigate speech and swallowing patterns in FSHD and assess the correlation with clinical data. Methods: A cross-sectional study was conducted. Patients with clinical confirmation of FSHD and aged above 18 years were included and paired with healthy control individuals by age and gender. Individuals who had neurological conditions that could interfere with test results were excluded. The following assessments were applied: speech tests (acoustic and auditory-perceptual analysis); swallowing tests with the Northwestern Dysphagia Patient Check Sheet (NDPCS), the Eat Assessment Tool (EAT-10), the Speech Therapy Protocol for Dysphagia Risk (PARD), and the Functional Oral Intake Scale (FOIS); disease staging using the modified Gardner-Medwin-Walton scale (GMWS); and quality of life with the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36). The correlation between test results and clinical data was verified by non-parametric statistics. Results: Thirteen individuals with FSHD and 10 healthy controls were evaluated. The groups presented significant differences in the motor bases of phonation and breathing. Regarding swallowing, two (15%) individuals presented mild dysphagia and seven (53.8%) showed reduced facial muscles strength. These results were not correlated with duration of the disease, age at symptoms onset, and quality of life. Dysphagia was related to worsening disease severity. Conclusions: FSHD patients presented mild dysarthria and dysphagia. Frequent monitoring of these symptoms could be an important way to provide early rehabilitation and better quality of life.

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“…Fatigue severity is quantified by self‐reported scales 23 designed to measure chronic fatigue burden over a proceeding period or acute fatigue responses to a task 24 . There are multiple domains of fatigue perception 25 linked to specific neural mechanisms.…”

Section: Fatigue Perceptionmentioning

confidence: 99%

Review article: The aetiology of fatigue in inflammatory bowel disease and potential therapeutic management strategies

McGing

Radford

Francis

et al. 2021

Aliment Pharmacol Ther

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Background: Fatigue is the inability to achieve or maintain an expected work output resulting from central or peripheral mechanisms. The prevalence of inflammatory bowel disease (IBD) fatigue can reach 86% in active disease, persisting in 50%-52% of patients with mild to inactive disease. Fatigue is the commonest reason for work absence in IBD, and patients often report fatigue burden to be greater than that of primary disease symptoms. Relatively few evidence-based treatment options exist, and the aetiology is poorly understood. Aim:To review the available data and suggest a possible aetiology of IBD fatigue and to consider the efficacy of existing management strategies and highlight potential future interventions. Methods:We reviewed fatigue-related literature in IBD using PubMed database. Results: Disease related factors such as inflammation and pharmacological treatments negatively impact skeletal muscle and brain physiology, likely contributing to fatigue symptoms. Secondary factors such as malnutrition, anaemia, sleep disturbance and psychological comorbidity are potential determinants. Immune profile, faecal microbiota composition and physical fitness differ significantly between fatigued and non-fatigued patients, suggesting these may be aetiological factors. Solution-focused therapy, high-dosage thiamine supplementation and biological therapy may reduce fatigue perception in IBD. The effect of physical activity interventions is inconclusive. Conclusions: A multimodal approach is likely required to treat IBD fatigue. Established reversible factors like anaemia, micronutrient deficiencies and active disease should initially be resolved. Psychosocial intervention shows potential efficacy in reducing fatigue perception in quiescent disease. Restoring physical deconditioning by exercise training intervention may further improve fatigue burden.| 369 MCGING et al.

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Fatigue in facioscapulohumeral muscular dystrophy: a qualitative study of people’s experiences

Cited by 18 publications

References 39 publications

Increased resistance towards fatigability in patients with facioscapulohumeral muscular dystrophy

Increased resistance towards fatigability in patients with facioscapulohumeral muscular dystrophy

Speech and swallowing characteristics in patients with facioscapulohumeral muscular dystrophy

Review article: The aetiology of fatigue in inflammatory bowel disease and potential therapeutic management strategies

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