Dysphagia in Duchenne muscular dystrophy: practical recommendations to guide management

Purpose: Duchenne muscular dystrophy (DMD) is a rapidly progressive neuromuscular disorder causing weakness of the skeletal, respiratory, cardiac and oropharyngeal muscles with up to one third of young men reporting difficulty swallowing (dysphagia). Recent studies on dysphagia in DMD clarify the pathophysiology of swallowing disorders and offer new tools for its assessment but little guidance is available for its management. This paper aims to provide a step-by-step algorithm to facilitate clinical decisions regarding dysphagia management in this patient population.

Methods: This algorithm is based on 30 years of clinical experience with DMD in a specialised Centre for Neuromuscular Disorders (Inkendaal Rehabilitation Hospital, Belgium) and is supported by literature where available.

Results: Dysphagia can worsen the condition of ageing patients with DMD. Apart from the difficulties of chewing and oral fragmentation of the food bolus, dysphagia is rather a consequence of an impairment in the pharyngeal phase of swallowing. By contrast with central neurologic disorders, dysphagia in DMD accompanies solid rather than liquid intake. Symptoms of dysphagia may not be clinically evident; however laryngeal food penetration, accumulation of food residue in the pharynx and/or true laryngeal food aspiration may occur. The prevalence of these issues in DMD is likely underestimated.

Conclusions: There is little guidance available for clinicians to manage dysphagia and improve feeding for young men with DMD. This report aims to provide a clinical algorithm to facilitate the diagnosis of dysphagia, to identify the symptoms and to propose practical recommendations to treat dysphagia in the adult DMD population.

• Implications for Rehabilitation

• Little guidance is available for the management of dysphagia in Duchenne dystrophy.

• Food can penetrate the vestibule, accumulate as residue or cause aspiration.

• We propose recommendations and an algorithm to guide management of dysphagia.

• Penetration/residue accumulation: prohibit solid food and promote intake of fluids.

• Aspiration: if cough augmentation techniques are ineffective, consider tracheostomy.

     

Quality of life of patients with Duchenne muscular dystrophy: from adolescence to young men

Purpose: This study investigated quality of life (QOL) in adolescent and young men with Duchenne muscular dystrophy (DMD).

Methods: Health-related QOL and global QOL were assessed with the Short Form 36 (SF-36) and World Health Organization Quality of Life-BREF (WHOQOL-BREF). Associations between functional status and QOL were assessed.

Results: All domains of the SF-36 were below Taiwan norms (effect size: ?14.2 to ?0.5), especially Physical Function, Role Physical, and Social Function. Three of the four domains of the WHOQOL-BREF were below Taiwan norms (effect size: ?2.0 to ?0.7). The Physical Function of the SF-36 was moderately correlated with functional status (mobility, basic activities of daily living, and arm function). The Social Function of the SF-36 and Social Relationships of the WHOQOL-BREF were also moderately correlated with functional status (impairment, basic activities of daily living, and arm function).

Conclusion: The adolescent and young men with DMD had poor health-related and global QOL. Poor QOL was related to both physical condition and social health. We suggest that rehabilitation programs focus on using assistive devices to facilitate arm function and encouraging participation in social activities to improve the QOL of patients with DMD.

• Implications for rehabilitation

• Duchenne muscular dystrophy (DMD) is a progressive muscle weakness disease that not only impacts physical health but also leads to poor quality of life in many domains.

• A valuable rehabilitation goal for patients with DMD is to encourage participation in social activities. Medical care and educational programs should plan a formal transition processes for patients with DMD from pediatric to adult care to maximum their quality of life.

• Arm function is associated with many domains of global quality of life, so a key element in improving quality of life may be to improve arm function.

     

Evaluation of hand orthoses in Duchenne muscular dystrophy

Abstract

Purpose: The purpose of this study was to evaluate whether treatment of boys with Duchenne muscular dystrophy using hand orthoses could benefit joint mobility, grip strength, or fine motor function.

Method: Eight boys with Duchenne muscular dystrophy were provided with individually customised rest orthoses. The results were analysed using single-subject design. The study included a baseline and an intervention phase. A follow-up examination was also performed.

Results: Boys with less than 50° passive wrist extension mobility were included. Wrist extension of the dominant hand increased in four and was maintained in four. Wrist extension in the non-dominant hand increased in five, was maintained in two and decreased in one. Thumb abduction in the dominant hand increased in six and two remained stable. In the non-dominant hand five increased and three remained stable. Grip strength and fine motor function showed also positive results.

Conclusions: This study indicates that the use of hand orthoses in Duchenne muscular dystrophy can delay development of contractures and improve passive wrist extension and thumb abduction. Hand orthoses can therefore be recommended for boys who start to develop contractures in the long finger flexors. Due to small sample size further studies are needed to confirm this result.

• Implications for rehabilitation

• Evaluation of hand orthoses in Duchenne muscular dystrophy.

• Preserved hand function is of uttermost importance for performance of activities in the late stages of Duchenne muscular dystrophy.

• Contractures of long finger flexors affect hand function and limit performance of daily activities.

• Hand orthoses can delay development of contractures and preserve hand function and give prerequisites for independence.

• The occupational therapists should measure wrist joint mobility regularly to be able to find the right time for intervention with hand orthoses in this progressive disorder.

     

Transitioning to adulthood with a progressive condition: best practice assumptions and individual experiences of young men with Duchenne muscular dystrophy

Abstract

Purpose: Youth with progressive conditions are living longer, and there is increased health care focus on assisting them with “transitioning” to adult services and adult life. The purpose of this investigation was to examine key discourses and normative assumptions underpinning transitions best practices and how they are reflected in the experiences of young men with Duchenne muscular dystrophy (DMD). Methods: Using a critical perspective, we qualitatively analyzed influential transitions best practice documents to identify their underpinning discursive assumptions. We compared these to the analysis of qualitative interviews and diary data from a study of 11 young men with DMD. Results: Transitions best practices are underpinned by discourses of developmental progression. They reproduce notions that associate successful transitions with becoming as independent as possible, approximating normal life trajectories, and planning for future adulthood. The accounts of youth with DMD both reflected and resisted these future-oriented discourses in creative ways that maintained positive personal identities. Conclusions: Normal developmental progression towards typical adult roles constitutes the generally accepted aims of transitions practices. Such aims may not be appropriate for all youth with disabilities. We suggest that alternative understandings of the life course and approaches to care need to be considered alongside dominant practices.

• Implications for Rehabilitation

• Children and youth with progressive conditions, such as DMD, are living longer and there is increased interest in designing programs that will assist them with “transitioning” to adulthood.

• Transitions best practices reflect dominant social values and assumptions about what constitutes a successful adulthood, embedded in goals such as independent living, self-management and obtaining work.

• Rehabilitation professionals should be aware of both positive (e.g. feelings of achievement) and negative (e.g. anxiety about the future) consequences of transitions practices that emphasize normal social developmental trajectories and milestones.

• Discussions with youth should offer multiple possibilities for living a good life in the present and provide support to address negative feelings and the progressive effects of DMD.

     

Participation in daily life activities and its relationship to strength and functional measures in boys with Duchenne muscular dystrophy

Abstract

Purpose: While most studies of Duchenne muscular dystrophy (DMD) have focused on physical impairment, there is a need to explore how impairment impacts real-life experiences in order to provide intervention strategies focused on participation. Objectives were: (1) to investigate the domains of participation in a sample of boys with DMD; (2) to compare a younger (<10 years) and older (≥10 years) group of boys with DMD with regard to participation; (3) to investigate strength and timed functional tests in a sample of boys with DMD; (4) to compare a younger (<10 years) and older (≥10 years) group of boys with DMD with regard to strength and timed functional tests; and (5) to explore associations between participation and strength and timed functional tests for our DMD cohorts. Methods: This cross-sectional study included 60 boys with DMD (mean 9.3 years?±?0.3). Boys completed strength testing, timed functional tests, the Children’s Assessment of Participation and Enjoyment and the ACTIVLIM. Independent samples t-tests were used to test for differences in all measures between our younger and older cohorts; Spearman’s (rank) correlation was used to assess relationships between participation and strength and time functional tests. Results: Significant differences were found between our younger and older boys with DMD in the areas of recreational (p?<?0.01), social (p?<?0.001), and skill-based activities (p?<?0.05), as well as with whom and where the activities were performed (p?<?0.05 and 0.001, respectively). Older boys with DMD report lower levels of participation in these areas, as well as less engagement in activities with individuals other than family members and less participation outside of the home. Lower levels of strength and slower rates of functional performance correlate with participation in fewer physical activities for our younger cohort and fewer physical and social activities for our older cohort. Conclusions: Strength and function relate to the variability and type of activities in which boys with DMD participate. A key finding is the significant decline in social activities and community-based engagement as the boys with DMD age. The ultimate goal of an intervention is for our children to be as actively engaged in life as they desire. This requires addressing participation when measuring outcomes in order to more fully understand limitations and provide appropriate strategies for continued participation for boys and their families.

• Implications for Rehabilitation

• Duchenne muscular dystrophy is a devastating progressive neuromuscular disorder that leads to significant strength and functional limitations, which affect physical and social participation for these boys.

• The ability to move beyond clinically-based outcomes and assess and monitor a child’s daily activities through participation measures may provide information for therapeutic interventions.

• Rehabilitation specialists have a role as advocates for social and community engagement for children with physical limitations.

• Providing families with information on community-based opportunities, and the strategies and environmental modifications available may increase social participation for our youth growing up with a neuromuscular disorder.

     

杜氏肌营养不良与贝氏肌营养不良常规MRI的对比研究

目的 探讨常规MRI鉴别诊断杜氏肌营养不良（DMD）和贝氏肌营养不良（BMD）的价值。方法 回顾性分析经临床确诊为抗肌萎缩蛋白病697例患者的臀部及大腿肌肉影像学资料,比较DMD与BMD影像学征象的异同。结果 DMD与BMD患者肌肉脂肪浸润的分布规律一致,DMD总体脂肪浸润程度高于BMD（P=0.034）,且以臀大肌、股直肌、缝匠肌为著;肌肉总体水肿程度差异无统计学意义（P=0.065）,但DMD大腿后群肌肉及缝匠肌的水肿明显高于BMD。DMD与BMD患者肌肉萎缩及肥大的选择性受累规律一致,BMD股外侧肌、股中间肌、股内侧肌、半膜肌及股二头肌长头萎缩频率明显高于DMD（P<0.05）;DMD与BMD患者肌肉的肥大改变差异无统计学意义（P>0.05）。结论 常规MRI可鉴别诊断DMD与BMD。     

Towards a short questionnaire for stepwise assessment of upper limb function,pain and stiffness in Duchenne muscular dystrophy

Purpose: Duchenne muscular dystrophy can lead to upper extremity limitations, pain and stiffness. In a previous study, these domains have been investigated using extensive questionnaires, which are too time-consuming for clinical practice. This study aimed at gaining insight into the underlying dimensions of these questionnaires, and to construct a short questionnaire that can be used for clinical assessment.

Methods: Exploratory factor analysis was performed on the responses of 213 participants to a web-based survey to find the underlying dimensions in the Capabilities of Upper Extremity questionnaire, the ABILHAND questionnaire, and questionnaires regarding pain and stiffness. Based on these underlying dimensions, a stepwise approach was formulated. In addition, construct validity of the factors was investigated.

Results: In total, 14 factors were identified. All had high internal consistency (Cronbach's alpha >0.89) and explained 80–88% of the variance of the original questionnaires. Construct validity was supported, because participants in the early ambulatory stage performed significantly better (pConclusion: The factors identified from the set of questionnaires provide a valid representation of upper extremity function, pain and stiffness in Duchenne muscular dystrophy. Based on the factor commonalities, the Upper Limb Short Questionnaire was formulated.

• Implications for Rehabilitation

• New insights into the underlying dimensions of upper extremity function, pain and stiffness in Duchenne muscular dystrophy are gained.

• Fourteen factors, with good internal consistency and construct validity, are identified regarding upper extremity function, pain and stiffness in Duchenne muscular dystrophy. Based on these factors, the Upper Limb Short Questionnaire is presented.

• The Upper Limb Short Questionnaire can be used as an identifier of arm-hand limitations and the start of more thorough clinical investigation.

     

Community-based study of health-related quality of life in spinal cord injury,muscular dystrophy,multiple sclerosis,and cerebral palsy

Purpose: To assess health-related quality of life (HRQOL) in adults with spinal cord injury (SCI), muscular dystrophy (MD), multiple sclerosis (MS), and cerebral palsy (CP). Methods: This is a multicenter, community-based, cross-sectional study of adults diagnosed with CP (94), MD (99), MS (98), SCI (99), and healthy adults (105). The WHOQOL-BREF and WHOQOL-DIS questionnaire were used. Results: Significant differences in physical functioning between adults with CP and SCI and adults with MS (p?=?0.003 and p < 0.001, respectively), as well as between adults with SCI and MD (p?=?0.001) were found. Univariate tests revealed significant psychological functioning differences between adults with SCI and MD (p?=?0.02) and SCI and MS (p?=?0.001). There was a significant difference in physical functioning between controls and adults with SCI (p?=?0.049) and a significant difference in psychological functioning between controls and adults with MS (p?=?0.039). No statistically significant differences were found between the groups in social and environmental domains. Conclusions: Physical and physiological functioning were affected to various degrees in the studied neurodisabilities, while all groups reported similar levels of functioning and well-being in social and environmental domains.

Implications for Rehabilitation

• Health-related quality of life (HRQOL) in adults with neurodisabilities living in a community differs from HRQOL in these individuals sampled in clinical and/or rehabilitation settings.

• Psychosocial aspects of HRQOL relevant to physical disability were similarly affected across those groups.

• Physical and physiological domains in HRQOL were affected to various degrees among adults with neurodisabilities living in a community.

• Levels of functioning and well-being in social and environment domains of HRQOL are similar among adults with neurodisabilities living in a community.

• Community-based study of health-related quality of life in spinal cord injury, muscular dystrophy, multiple sclerosis, and cerebral palsy.

     

Dysphagia in patients with Duchenne muscular dystrophy evaluated with a questionnaire and videofluorography

Purpose. To investigate swallowing problems in patients with Duchenne muscular dystrophy (DMD) using a questionnaire and videofluorography (VF).

Method. A questionnaire survey was performed of swallowing-related symptoms and VF in 31 male patients with DMD (mean age 19.9 years, range 9 – 26 years). The relationships among age, frequency of symptoms and VF abnormalities were analysed using Spearman's rank correlation. The differences in VF abnormalities among different food textures were analysed with the Kruskal – Wallis test.

Results. Symptoms related to pharyngeal phase dysfunction were more frequent than those related to oral and oesophageal phases. Coughing while eating was seen in 71% of the patients, choking while eating in 32% and the need to clear the throat in 26%. VF abnormalities were observed in 30 patients (96.8%). Common VF abnormalities included pooling in the valleculae (90.3%) and in the pyriform sinus (90.3%). Pharyngo-oral regurgitation was seen in 35.5% of the patients. Pooling in the pyriform sinus after repeated swallowing seen in VF correlated significantly with symptoms related to the pharyngeal phase (Spearman's rho 0.356 – 0.544).

Conclusion. Because oropharyngeal dysphagia in DMD was evident in teenage patients as well as those without clinical symptoms, VF is recommended in patients with DMD.     

Upper limb function and activity in people with facioscapulohumeral muscular dystrophy: a web-based survey

Purpose To investigate the upper extremity (UE) at the level of impairments and related activity limitations and participation restrictions in people with facioscapulohumeral muscular dystrophy (FSHD). Methods The study was conducted using web-based questionnaires that were distributed amongst people with FSHD in the Netherlands. Eighty-eight respondents started the survey, and 71 completed it. The questionnaires covered the following dimensions: Function, Activity and Participation of the International Classification of Functioning Disability and Health. Results More than 40% of the respondents experienced pain in one arm or both the arms. Increased pain and stiffness scores and longer disease duration were associated with increased limitation scores. For basic activities, lifting the arm above shoulder-level was most frequently reported as most limited, coherent with the clinical picture of FSHD. Among the respondents, 50% indicated restrictions at school, 78% indicated restrictions at work and more than 80% indicated restrictions whilst participating in sports, hobbies, household activities and romantic relationships. Conclusions This study has shown that alongside the well-known problem of lifting the arms above shoulder-level, UE activities below shoulder height during vocational and occupational activities are also problematic in patients with FSHD. Alongside disease duration, pain and stiffness are associated with UE activity limitations.

• Implications for Rehabilitation

• Attention is needed for pain and experienced stiffness in the upper extremity as it is frequently present in patients with FSHD.

• Rehabilitation professionals need to be aware that patients with FSHD not only experience problems with activities above shoulder height, but also with activities below shoulder height.

• At least 50% of the patients with FSHD experience restrictions in participation as a result of limitations in their UE.

     

Upper extremity function and activity in facioscapulohumeral dystrophy and limb-girdle muscular dystrophies: a systematic review

Abstract

Purpose: The aims of this review were (1) to provide insight into the natural course of upper-extremity (UE) impairments and UE activity limitations associated with facioscapulohumeral dystrophy (FSHD) and limb-girdle muscular dystrophies (LGMD), and (2) to provide an overview of outcome measures used to evaluate UE function and activity in patients with FSHD and LGMD. Methods: Scientific literature databases (PubMed, MEDLINE, EMBASE, CINAHL and Cochrane) were searched for relevant publications. Inclusion criteria: (1) studies that included persons with a diagnosis of FSHD or LGMD; and (2) studies that reported the natural course of the UE functions and/or activity with outcome measures at these levels. Results: 247 publications were screened, of which 16 fulfilled the selection criteria. Most studies used manual muscle testing (MMT) to evaluate UE function and the Brooke Scale to evaluate UE mobility activities. The clinical picture of UE impairments and limitations of UE activities in FSHD and LGMD patients was highly variable. In general, FSHD and LGMD patients experience difficulty elevating their upper extremities and the execution of tasks takes considerably longer time. Conclusions: The clinical course of UE impairments and activity limitations associated with FSHD and LGMD is difficult to predict due to its high variability. Although measures like MMT and the Brooke Scale are often used, there is a lack of more specific outcome measures to assess UE function and UE capacity and performance in daily life. Measures such as 3D motion analysis and electromyography (EMG) recordings are recommended to provide additional insight in UE function. Questionnaires like the Abilhand are recommended to assess UE capacity and accelerometry to assess UE performance in daily life.

• Implications for Rehabilitation

• There is a need for specific outcome measures on the level of UE activity.

• Both the level of capacity and performance should be assessed.

• Possible outcome measures include 3D motion analysis to assess UE function, questionnaires like the Abilhand to assess UE capacity and accelerometry to assess performance of UE activities in daily life.

     

The Pediatric Version of the Eating Assessment Tool: a caregiver administered dyphagia-specific outcome instrument for children

Purpose: To develop and evaluate the psychometric properties of the Pediatric version of the caregiver administered Eating Assessment Tool.

Methods: The study included developmental phase and reported content, criterion validity, internal consistency and test–retest reliability of the Pediatric Eating Assessment Tool. Literature review and the original Eating Assessment Tool were used for line-item generation. Expert consensus assessed the items for content validity over two Delphi rounds. Fifty-one healthy children to obtain normative data and 138 children with cerebral palsy to evaluate test–retest reliability, internal consistency, and criterion validity were included. The Penetration-Aspiration Scale was used to assess criterion validity.

Results: All items were found to be necessary. Content validity index was 0.91. The mean score of Pediatric Eating Assessment Tool for healthy children and children with cerebral palsy was 0.26?±?1.83 and 19.5?±?11, respectively. The internal consistency was high with Cronbach’s alpha =0.87 for test and retest. An excellent correlation between the Pediatric Eating Assessment Tool and Penetration-Aspiration score for liquid and pudding swallowing was found (p?r?=?0.77; p?r?=?0.83, respectively). A score >4 demonstrated a sensitivity of 91.3% and specificity of 98.8% to predict penetration/aspiration.

Conclusions: The Pediatric Eating Assessment Tool was shown to be a valid and reliable tool to determine penetration/aspiration risk in children.

• Implications for rehabilitation

• The pediatric eating assessment tool: a new dyphagia-specific outcome survey for children.

• The Pediatric Version of the Eating Assessment Tool is a dysphagia specific, parent report outcome instrument to determine penetration/aspiration risk in children.

• The Pediatric Version of the Eating Assessment Tool has good internal consistency, test–retest reliability and criterion-based validity.

• The Pediatric Version of the Eating Assessment Tool may be utilized as a clinical instrument to assess the need for further instrumental evaluation of swallowing function in children.

     

The applicability of four clinical methods to evaluate arm and hand function in all stages of spinal muscular atrophy type II

Abstract

Aim: To evaluate the ability of four clinical methods to reflect arm and hand function at impairment and activity level and to determine their ability to discriminate among SMA II patients of all ages and in all stages of the disease. Methods: Fifty-two patients with SMA II (age range: 8–73 years) were assessed by means of the Egen Klassifikation 2 (EK2 scale), the Motor Function Measure Scale (MFM D3), the Manual Muscle Test (MMT) and Hand-Held Dynamometry (HHD) in full fist grip and lateral pinch grip. Patients were classified into six levels of upper limb function by means of the Brooke Upper Limb Scale, and the four methods’ ability to differentiate among patients within these levels was calculated. Modified versions of the EK2 scale (EK Upper Limb) and the MFM D3 (MFM D3 Upper Limb) were assessed in the same manner. Results: The patients’ physical abilities were best described by the MMT and EK2 while the “EK Upper Limb”, MFM D3 and MMT were best at discriminating among patients across the range of upper limb function. Quantitative muscle tests as measured by Citec? HHD were less applicable to weak patients; full fist grip could discriminate among patients at Brooke levels 3–5, and lateral pinch grip among the strongest patients. Conclusion: At the impairment level, MMT is the superior measure of muscle function in very weak patients in whom HHD cannot reflect capacity. At the activity level, the EK 2 represents daily activities whereas the MFM D3 measures motor functions. In differentiating among SMA II patients of all ages and in all stages of the disease, the ability of abbreviated versions of scales targeting upper limb function is superior to unabridged versions of these scales.

• Implications for Rehabilitation

• Evaluation of upper limb function in spinal muscular atrophy II

• Even very weak patients with SMA II have some residual upper limb function that is measurable if the right method is chosen.

• The Manual muscle test is applicable to all patients with SMA II and is useful to determine possible interventions – such as methods to drive a wheelchair or operate a computer.

• Abbreviated versions of the EK2 scale and the MFM are useful as methods to evaluate subtle changes in upper limb function resulting from disease progression or interventions.

     

A head-to-head comparison of the de Morton Mobility Index (DEMMI) and Elderly Mobility Scale (EMS) in an older acute medical population

Abstract

Purpose: To compare the clinimetric properties of the de Morton Mobility Index (DEMMI®) and the Elderly Mobility Scale (EMS). Method: A head-to-head comparison of the EMS and DEMMI® with 120 consecutive older acute medical patients. The DEMMI® and EMS were administered within 48?h of hospital admission and discharge. Results: At admission, 6% and 15% of participants scored the lowest scale score for the DEMMI® and EMS, respectively. For the DEMMI®, 17% of participants scored within the minimal detectable change of the lowest scale score compared to 20% for the EMS at admission. At hospital discharge, DEMMI® scores were normally distributed and the EMS had a ceiling effect. Similar evidence of convergent, discriminant and known groups validity were obtained for the DEMMI® and EMS. There was no significant difference in responsiveness to change between the DEMMI® and EMS. The EMS was significantly quicker to administer compared to the DEMMI®. Conclusion: The DEMMI® and EMS are both valid measures of mobility for older acute medical patients. The DEMMI® has a broader scale width than the EMS with interval level measurement and therefore provides a more accurate method for measuring and monitoring changes in mobility for older acute medical patients.

• Implications for Rehabilitation

• Mobility of older acute medical patients.

• Hospitalised older acute medical patients are at “high risk” of mobility decline.

• Accurate measurement of mobility is essential for preventing and treating mobility decline.

• Many existing mobility measures have significant measurement limitations.

• The DEMMI® is a more accurate measure of mobility than the EMS in an older acute medical population.

     

Regaining water swallowing function in the rehabilitation of critically ill patients with intensive-care-unit acquired muscle weakness

Purpose: Treatment in intensive care units (ICUs) often results in swallowing dysfunction. Recent longitudinal studies have described the recovery of critically ill people, but we are not aware of studies of the recovery of swallowing function in patients with ICU-acquired muscle weakness. This paper aims to describe the time course of regaining water swallowing function in patients with ICU-acquired weakness in the post-acute phase and to describe the risks of regaining water swallowing function and the risk factors involved.

Methods: This cohort study included patients with ICU-acquired muscle weakness in our post-acute department, who were unable to swallow. We monitored the process of regaining water swallowing function using the 3-ounce water swallowing test.

Results: We included 108 patients with ICU-acquired muscle weakness. Water swallowing function was regained after a median of 12?days (interquartile range =17) from inclusion in the study and after a median of 59?days (interquartile range=?36) from the onset of the primary illness. Our multivariate Cox Proportional Hazard model yielded two main risk factors for regaining water swallowing function: the number of medical tubes such as catheters at admission to the post-acute department (adjusted hazard ratio [HR]?=?1.282; 95% confidence interval [CI]: 1.099–1.495) and the time until weaning from the respirator in days (adjusted HR =1.02 per day; 95%CI: 0.998 to 1.008).

Conclusion: We describe a time course for regaining water swallowing function based on daily tests in the post-acute phase of critically ill patients. Risk factors associated with regaining water swallowing function in rehabilitation are the number of medical tubes and the duration of weaning from the respirator.

• Implications for rehabilitation

• Little guidance is available for the management of swallowing dysfunction in the rehabilitation of critically ill patients with intensive-care-units acquired muscle weakness.

• There is a time dependent pattern of recovery from swallowing dysfunction with daily water swallowing tests in the post-acute phase of critically ill patients.

• Daily water swallowing tests can be used to test swallowing dysfunction in the post-acute phase of critically ill patients

• The amount of medical tubes and the duration of weaning from respirator are associated risk factors for recovery of swallowing dysfunction.

     

Quality of life for post-polio syndrome: a patient derived,Rasch standard scale

Objective: To design a disease-specific quality of life (QoL) questionnaire for people with post-polio syndrome (PPS).

Methods: Qualitative interviews were conducted with 45 people with PPS to identify themes and derive potential items reflecting impact upon QoL. After cognitive debriefing, these were made into a questionnaire pack along with comparative questionnaires and posted to 319 patients. The 271 (85%) returned questionnaires were subjected to exploratory factor analysis (EFA) and Rasch analysis.

Results: A 25 item scale, the post-polio quality of life scale (PP-QoL), showed good fit to the Rasch model (conditional chi-square p?=?0.156), unidimensionality (% t-tests 2.0: CI 0.7–3.8), and Cronbach’s alpha of 0.87. With the latent estimate transformed to a 0–100 scale, the mean score was 56.9 (SD 18.5) with only 3.3% of respondents at the floor or ceiling of the scale. Test–retest reliability showed an intraclass correlation coefficient (ICC) (2.1) of 0.916, and correlation of 0.85.

Conclusion: The disease-specific PP-QoL demonstrated excellent reliability, appropriate concurrent validity, and satisfied the standards of the Rasch model. It enables examination of the impact of health status upon perceived QoL, and the impact of rehabilitation interventions. The scale is freely available for academic or not-for-profit users to improve research in this neglected, disabling condition.

• Implications for Rehabilitation

• In post-polio syndrome (PPS), existing work examines aspects of health-related quality of life (HRQoL), such as activity limitations.

• A disease-specific QoL measure would enable researchers to model the impact of health status, such as fatigue or mobility restrictions, upon QoL in PPS.

• The post-polio quality of life scale (PP-QoL) is based on the patients’ lived experience, meets Rasch standards and is free for use for academic and not-for-profit researchers.

• The raw score is reliable for individual use in clinical settings, and interval scale transformation is available for parametric applications and the calculation of change scores.

     

Dysarthria and dysphagia are highly prevalent among various types of neuromuscular diseases

Abstract

Purpose: Patients with a neuromuscular disease (NMD) can present with dysarthria and/or dysphagia. Literature regarding prevalence rates of dysarthria and dysphagia is scarce. The purpose of this study was to determine prevalence rates, severity and co-presence of dysarthria and dysphagia in adult patients with NMD. Method: Two groups of adult patients with NMD were included: 102 consecutive outpatients (the “unselected cohort”) and 118 consecutive patients who were referred for multidisciplinary assessment (the “selected cohort”). An experienced speech-language pathologist examined each patient in detail. Results: The pooled prevalence of dysarthria was 46% (95% CI: 36.5–55.9) and 62% (95% CI: 53.3–70.8) in the unselected and selected cohorts, respectively. The pooled prevalence of dysphagia was 36% (95% CI: 27.1–45.7) and 58% (95% CI: 49.4–67.2) in the unselected and selected cohorts, respectively. There was a modest but significant association between the presence of dysarthria and dysphagia (r_s?=?0.40; p?<?0.01). Although the dysphagia was generally mild, dysarthria was moderate to severe in 15% of the dysarthric patients. Conclusion: The prevalence rates of dysarthria and dysphagia among patients with various types of NMD are high. Physicians should therefore be aware of this prevalence and consider referring NMD patients to a speech-language pathologist.

• Implicatons of Rehabilitation

• Both dysarthria and dysphagia are highly prevalent among patients with neuromuscular diseases; moreover, although often mild, these disorders can occur relatively early in the course of the disease.

• Clinicians should routinely check for signs and symptoms related to dysarthria and/or dysphagia in patients who present with a neuromuscular disease, preferably using standardised instruments.

     

Carbonation as a sensory enhancement strategy: a narrative synthesis of existing evidence

Purpose: Sensory enhancement techniques, like other compensatory strategies, aim to reduce dysphagia symptoms. The use of carbonated liquids has been proposed as a possible sensory technique, however to date, there is limited information of its efficacy or guidance for clinical implementation. A narrative synthesis was completed to determine the quality and strength of the evidence base for use of carbonation as a compensatory strategy in dysphagia rehabilitation.

Methods: From 101 articles initially identified, 14 articles met the study criteria. Eleven papers described the effects of carbonation on swallowing in healthy participants whereas three described the impact of carbonation in dysphagic populations. A narrative synthesis of papers was undertaken given the diversity of identified studies.

Results: Synthesis of findings was challenging given the exploratory phase of most research activity with diverse populations described and extensive differences in research methodologies. There is currently weak, but potentially positive evidence to support using carbonation as a compensatory technique in dysphagia rehabilitation.

Conclusion: Despite future potential, existing evidence fails to provide clear direction for the clinical implementation of carbonation. Validation of carbonation use with the dysphagic population requires further research with consistent, controlled methodologies, and larger cohorts of participants to inform potential for dysphagia rehabilitation.

• Implications for Rehabilitation

• The use of carbonated liquids has been proposed as a possible sensory enhancement technique which may facilitate changes to swallow physiology.

• However to date, there is limited information to direct clinical implementation.

• This paper provides a narrative synthesis of existing knowledge and highlights possible limitations of findings reported.

• Research to date has used disparate research methodologies in varied populations making synthesis of current findings challenging.