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Life balance is a new and important concept in occupational therapy. New measurements are needed to assess and evaluate life balance and interventions aimed to achieve this concept. This article describes the test-retest reliability of three life balance measures: the Activity Calculator (AC), Activity Card Sort (ACS-NL(18-64)) and Occupational Balance Questionnaire (OBQ11-NL). Data collection took place among 50 participants with neuromuscular diseases: facioscapulohumeral dystrophy (FSHD, n = 25) or mitochondrial myopathy (MM, n = 25). The AC, the ACS-NL(18–64) and the OBQ11-NL were assessed twice with an interval of one week. Intraclass correlation coefficients (ICC-agreement) were applied to examine test-retest reliability. The ICC of the AC-average total day score was.95 (95% CI.91-.97), whereas the ICC of the weights allocated to each activity was 0.80 (95% CI.77–0.82). The ICC of the ACS-NL(18–64) percentage retained activities was 0.92 (95% CI 0.86 − 0.96) and the ICC of the importance score per activity was-.76 (95% CI. 0.68–0.89). The ICC of the OBQ11-NL total score was.76 (95% CI 0.62–0.86). All three tools showed good to excellent test-retest reliability in a sample of patients with FSHD or MM, which is promising for its use in clinical practice and research. The AC, ACS-NL(18–64) and the OBQ11-NL are promising, reliable measures of life balance in patients with neuromuscular diseases. The development of three new instruments for life balance enlarges the possibility for health professionals to measure life balance in clinical practice and research. [ABSTRACT FROM AUTHOR]

Background: Chronic fatigue is present in more than 60% of the patients with a neuromuscular disease and can be their most disabling symptom. Chronic fatigue often coincides with low levels of physical activity and decreased social participation. We have developed a self-management outpatient rehabilitation intervention for patients with a neuromuscular disease and chronic fatigue, called Energetic. This multidisciplinary group programme aims to alleviate fatigue, improve physical endurance and social participation. Objective: The aim of this study was to evaluate the effectiveness of Energetic. Methods: A multicentre, assessor-blinded, two-armed randomized controlled trial was conducted. Assessments were made at inclusion, directly after intervention, and three and eleven months followup. We included patients with a neuromuscular disease and chronic fatigue, and their caregivers. The participants were randomized (1:1) to either an intervention (‘Energetic’) or control (‘usual care’) group. Energetic covered four months and included four modules: (1) individually tailored aerobic exercise training; (2) education about aerobic exercise; (3) self-management training in applying energy-conservation strategies; and (4) implementation and relapse prevention in daily life. The primary outcome was the perceived performance score of the Canadian Occupational Performance Measure (COPM). Secondary outcomes included the COPM satisfaction score, and measures of fatigue, physical endurance, activity engagement, mood, self-efficacy and caregiver-burden. Results: Fifty-three patients were included. There was significantly more improvement on COPM-performance, COPM-satisfaction, Checklist Individual Strength (CIS), CIS subscale-fatigue and 6-Minute Walk Test for the intervention group than the control group after intervention, adjusted for baseline COPM scores, gender, work and diagnosis. Conclusion: These results warrant further implementation of this intervention in different settings. [ABSTRACT FROM AUTHOR]

Myotonic dystrophy type 1 is a neuromuscular disorder affecting multiple organ systems and is characterized by a variety of clinical presentations. Anticipation leads to an earlier and more severe phenotype in subsequent generations. Early-onset cataract is a common initial manifestation of the late or adult-onset type of myotonic dystrophy 1. Due to its multicausal nature, early-onset cataract is often not recognized as a feature of this disease, leading to diagnostic delay resulting in consequences for successive generations, treatment and counseling. A qualitative study with semi-structured interviews was performed with purposive sampling of eight participants with myotonic dystrophy type 1 and early-onset cataract to investigate the physical and psychosocial consequences experienced due to diagnostic delay. Prior to the early-onset cataract, all participants experienced other multisystem symptoms that could have been explained by myotonic dystrophy. The diagnostic delay had severe hereditary consequences: a subsequent generation with more severely affected (grand)children was born resulting in large emotional burden for the patients. To conclude, early-onset cataract is a warning sign and ophthalmologists play a crucial role in the early detection of myotonic dystrophy type 1 by recognizing this symptom and preventing the birth of severely affected children leading to emotional and psychosocial consequences., Competing Interests: Declaration of Competing interests The authors report no competing interests., (Copyright © 2022. Published by Elsevier B.V.)

Background: To retard shortening of finger flexors in patients with Duchenne muscular dystrophy (DMD), hand orthoses are prescribed. However, many patients do not wear the orthoses regularly. To optimize orthotic interventions, we need insight into the factors influencing compliance., Objective: To evaluate the compliance regarding hand orthoses in an adult DMD population and to explore experiences and perceptions of DMD patients wearing orthoses, and of their caregivers., Methods: Mixed methods observational study, combining quantitative and qualitative data from medical charts combined with qualitative semi-structured interviews using a constant comparative method and a short validated questionnaire (D-QUEST)., Results: 65 medical charts were analyzed. 48 patients were assessed as needing hand orthoses, of whom 37.5 % were compliant. Qualitative data analyses revealed (1) motivation: preservation of hand function; (2) barriers: discomfort and impediments; (3) facilitators: good fit and personalized wearing schedule; (4) fitting process: satisfactory, but patients do not readily seek help when barriers appear., Conclusions: Patients are motivated to wear hand orthoses, but often discontinue use because of orthosis-and disease-specific barriers. The identification of these barriers leads to practical and feasible recommendations concerning the orthoses and the fitting process, such as less rigid material, preservation of some function while wearing the orthoses, and fixed evaluation points. The findings were confirmed by the D-QUEST.

Purpose: A systematic review was conducted to summarize and evaluate the literature on the effectiveness of speech pathology interventions in adults with neuromuscular diseases., Method: Databases searched included the Cochrane Database of Systematic Reviews, the Cochrane Central Register of Controlled Trials, MEDLINE, CINAHL, EMBASE, PsycINFO and PubMed. A total of 1,772 articles were independently screened on title and abstract by 2 reviewers., Results: No randomized controlled trials or clinical controlled trials were found. Four other designs were included. Only one study on oculopharyngeal muscle dystrophy (OPMD) appeared to have sufficient methodological quality. There is evidence indicating that correction of head position in patients with OPMD improves swallowing efficiency (level III evidence)., Conclusion: Despite 1,772 studies, there is only evidence of level III regarding the effectiveness of speech pathology interventions in patients with OPMD. Recommendations for future research are given., (Copyright © 2010 S. Karger AG, Basel.)