Longitudinal Assessment of Strength, Functional Capacity, Oropharyngeal Function, and Quality of Life in Oculopharyngeal Muscular Dystrophy.

ObjectiveOculopharyngeal muscular dystrophy (OPMD) is a late-onset, progressive muscle disease. Disease progression is known to be slow, but details on the natural history remain unknown. We aimed to examine the natural history of OPMD in a large nationwide cohort to determine clinical outcome measures that capture disease progression and can be used in future clinical trials.MethodsPatients, invited by their treating physicians or from the national neuromuscular database, and invited family members, were examined twice, 20 months apart, using fixed dynamometry, MRC grading, maximum bite force and isometric tongue strength, Motor Function Measure (MFM), 10-step stair test, maximum swallowing-, chewing-, and speech-tasks and quality of life assessments.ResultsDisease progression was captured by 8 out of 18 measures over 20 months in forty-three genetically confirmed OPMD patients. The largest deterioration was seen in deltoid muscle strength (-27% (range -17 - -37%)), followed by the quadriceps (-14% (range -6 - -23%)), iliopsoas (-12.2%), tongue (-9.9%) and MRC sum-score (-2.5%). The 10-step stair test (-12.5%), MFM part D1 (-7.1%), and maximum repetition rate of /pa/ (-5.3%) showed a significant decrease as well (all p<0.05). Domain 'Physical functioning' of the SF-36 Health Survey significantly deteriorated (p=0.044). No relationship was found between disease progression and genotype or disease duration (p>0.05).ConclusionsDespite the slow disease progression of OPMD, this study showed that several outcome measures detected progression within 20 months. The deltoid muscle strength, measured by fixed dynamometry, showed the greatest decline. This longitudinal data provides clinical outcome measures that can be used as biomarkers in future clinical trials.

Factors influencing the implementation of Home-Based Stroke Rehabilitation: Professionals' perspective.

BACKGROUND: Stroke has a major impact on survivors and their social environment. Care delivery is advocated to become more client-centered and home-based because of their positive impact on client outcomes. The objective of this study was to explore professionals' perspectives on the provision of Home-Based Stroke Rehabilitation (HBSR) in the Netherlands and on the barriers and facilitators influencing the implementation of HBSR in daily practice. METHODS: Semi-structured focus groups were conducted to explore the perspectives of health and social care professionals involved in stroke rehabilitation. Directed content analysis was performed to analyze the transcripts of recorded conversations. RESULTS: Fourteen professionals participated in focus groups (n = 12) or, if unable to attend, an interview (n = 2). Participants varied in professional backgrounds and roles in treating Dutch clients post stroke. Barriers and facilitators influencing the implementation of HBSR in daily practice were identified in relation to: the innovation, the user, the organization and the socio-political context. Participants reported that HBSR can be efficient and effective to most clients because it facilitates client- and caregiver-centered rehabilitation within the clients' own environment. However, barriers in implementing HBSR were perceived in a lack of (structured) inter-professional collaboration and the transparency of expertise of primary care professionals. Also, the current financial structures for HBSR in the Netherlands are viewed as inappropriate. DISCUSSION: In line with previous studies, we found that HBSR is recognized by professionals as a promising alternative to institution-based rehabilitation for clients with sufficient capabilities (e.g. their own health and informal support). CONCLUSION: Multiple factors influencing the implementation of HBSR were identified. Our study suggests that, in order to implement HBSR in daily practice, region specific implementation strategies need to be developed. We recommend developing strategies concerning: organized and coordinated inter-professional collaboration, transparency of the expertise of primary care professionals, and the financial structures of HBSR.

"Everyone sees you sitting there struggling with your food": experiences of adolescents and young adults with cerebral palsy.

Objective: The impact of difficulties with eating and drinking in adolescents and young adults with cerebral palsy is unknown. The purpose of this study is to find out which difficulties adolescents and young adults with cerebral palsy experience with eating and drinking in daily life and how they deal with these difficulties. The study also explores how they think about themselves with respect to eating and what does or does not help regarding social participation. Method: We collected the data from ten participants with spastic cerebral palsy (aged 15-23 years) living in the Netherlands. We used a qualitative study design with a conventional content analysis. Semi-structured in-depth interviews were used to identify meaningful factors related to eating and drinking difficulties. We coded relevant phrases from each interview and clustered and synthesized them into categories. Result: We derived four categories from the transcripts: (I) perceived eating and drinking difficulties (e.g., not managing to eat all food textures and/or choking); (II) challenges in physical and social context (e.g., accessibility of restaurants, menu supply, and/or needing assistance or not); (III) dealing with eating and drinking difficulties (e.g., adaptation, food avoidance, and/or giving up); (IV) Negative feelings about their eating and drinking (e.g., shame, frustration, fear for choking, and/or concerns about the future). One striking finding was that most participants had not recently received either monitoring or intervention for their feeding skills. Conclusion: This study shows that adolescents and young adults with cerebral palsy experience many restrictions in eating and drinking situations leading to negative feelings and lower participation levels, while little attention is directed towards these difficulties. Regular multidisciplinary rehabilitation programs should include evaluation, advice, and intervention regarding eating and drinking ability in order to increase social participation and self-management. Implications for Rehabilitation Adolescents and young adults with cerebral palsy experience difficulties with eating, drinking, and swallowing, and they encounter difficulties in participating in mealtimes with family and friends. Although adolescents and young adults with cerebral palsy rated their eating and drinking abilities as reasonable, they reported feelings of shame, frustration, fear, distress, and concerns for the future or unknown environments. Regular multidisciplinary involvement with eating and drinking is needed for purposes of evaluation, advice, and intervention throughout the life course, adjusted to living conditions and the latest evidence. Adolescents and young adults with cerebral palsy showed limited initiative in asking for personal assistance in eating and drinking activities.

The Radboud Dysarthria Assessment: Development and Clinimetric Evaluation.

OBJECTIVE: In the absence of an adequate dysarthria assessment in the Netherlands, we developed the Radboud Dysarthria Assessment (RDA). This article describes its development and clinimetric evaluation. PATIENTS AND METHODS: Forty-three patients were assessed with the RDA. The recording forms were subjected to exploratory factor analysis and estimation of internal consistency. The self-evaluation questionnaire was tested for internal consistency and the severity scale for intra- and inter-rater reliability. Construct validity of the severity scale and questionnaire was determined by relating them to the Speech Handicap Index (SHI), Dutch sentence intelligibility assessment (NSVO-Z), and category fluency task. RESULTS: Exploratory factor analysis extracted 4 factors (articulation, resonance, phonation, respiration/prosody) yielding an explained variance of 70.3%. Each factor showed good internal consistency (Cronbach's α: 0.89-0.91). The self-evaluation questionnaire showed excellent internal consistency (Cronbach's α: 0.90). Intra-class correlation coefficients of the severity scale (0.85-0.86) showed good reliability. The severity scores and self-evaluation questionnaire correlated substantially to strongly with the SHI (rs = 0.40 and 0.80) and substantially with the NSVO-Z (rs = -0.65 and -0.52). CONCLUSIONS: The RDA is a valid and reliable tool, but further investigation is needed to demonstrate whether this instrument can successfully support speech-language therapists in correctly diagnosing the type of dysarthria.

How is self-management perceived by community living people after a stroke? A focus group study.

PURPOSE: Self-management has become an important concept in stroke rehabilitation; however, the way that people post-stroke reflect on the concept of self-management has not yet been studied. This qualitative study explored the reflections of persons post-stroke on self-management, readiness and needs in self-management support. METHODS: Focus group interviews were conducted with 16 community living stroke survivors (53-84 years of age). Both verbal questions and photo elicitations were used to collect data. A constant comparative framework was used for the analysis. RESULT: Participants described their self-management as a complex, long-term, personal learning process. Post-discharge, participants were not ready to self-manage. Aside from individual self-management, participants also mentioned co-management with relatives. Relatives could provide support, but they also limited the development of participants' self-management skills. Participants missed having professional support post-discharge and would have appreciated additional psychological and emotional support in their process of self-management. CONCLUSION: Self-management post-stroke is complex. Stroke self-management programmes may be optimised when integrating role and emotional management in addition to medical management. Although readiness to self-manage differs among individuals, support should start as soon as possible and continue post-discharge in people's personal environments. Self-management programmes should not only focus on self-management of stroke survivors but also on co-management with relatives.

Barriers to and facilitators for the use of an evidence-based occupational therapy guideline for older people with dementia and their carers.

OBJECTIVE: Implementing evidence-based guidelines is not a simple task. This study aimed to define barriers to and facilitators for implementing the proven and effective Community Occupational Therapy in Dementia (COTiD) guideline for older people with dementia and their carers. METHODS: The qualitative method we used was the grounded theory. We collected data from focus group interviews with 17 occupational therapists (OTs) and telephone interviews with 10 physicians and 4 managers. We analysed the data with the constant comparative method by identifying codes, categories, and main themes. RESULTS: The main themes in barriers that OTs encountered were that they did not feel competent in treating older people with dementia at home according to the guideline; they had difficulties prioritising in this complex treatment, both for the client and themselves; and they were unsure about the minimal criteria for guideline adherence. The intensity of this programme was the main barrier. The barriers that confronted physicians and managers were lack of knowledge about occupational therapy and its reimbursement and lack of available trained OTs. For the OTs, the guideline's content and focus, evidence, and external support facilitated the use of the guideline. For physicians and managers, the guideline's evidence base and its benefits for clients and carers were the main facilitators. CONCLUSIONS: This study generates knowledge for applying innovations in health care settings. Improvement of the OTs' knowledge and self-confidence is needed for using the guideline. We expect that more competent OTs will motivate their physicians and managers to use this successful COTiD intervention.

Neonatal swallowing assessment and practical recommendations for oral feeding in a girl with a severe congenital myopathy.

The investigators report the case of a 6-week-old girl with a cap-like congenital myopathy and frequent coughing during feeding. Neonatal swallowing assessments were performed with surface electromyography of the submental muscle group and nasal airflow during sucking and swallowing. A videofluoroscopic swallow study was performed separately. The registration of swallowing and breathing revealed a highly coordinated sequence of sucking, swallowing, and breathing. However, after a series of sucking and swallowing there was residue in the hypopharyngeal area, causing laryngeal penetration and coughing. Based on the findings, specific recommendations such as the use of a soft nipple, diminished suck-swallow sequences, and limited feeding time were made for this girl. These proved to be appropriate to prevent coughing as a signal of penetration or aspiration of milk during swallowing. This case highlights the profit of neonatal swallowing assessment, which can provide recommendations for safe oral feeding.