Impacts of a Dysphagia Screening Questionnaire on Speech Pathology Input Using a Transdisciplinary Approach for Patients with Chronic Obstructive Pulmonary Disease in a Pulmonary Rehabilitation Program.

Patients with chronic obstructive pulmonary disease (COPD) in pulmonary rehabilitation programs (PRPs) are not routinely screened for dysphagia. An Australian regional health service audit revealed that patients with COPD are frequently referred to speech pathology during acute admissions, rather than proactively to mitigate the risk of dysphagia-related consequences. Referral patterns to speech pathology using a novel transdisciplinary approach for identifying at risk for dysphagia patients in a PRP were explored. The aim of this study was to investigate the impact of a transdisciplinary dysphagia screening questionnaire on speech pathology referrals within a cohort of patients with COPD enrolled in a PRP. This quasi-experimental study introduced a dysphagia screening questionnaire in a PRP using a transdisciplinary approach. A retrospective audit of PRP patients (n = 563) between 01/01/2014 and 31/12/2018 was conducted to identify the frequency of referrals to speech pathology for dysphagia. Data was compared to a cohort of patients (n = 50) enrolled in the PRP (from 01/02/21 to 30/11/21) after introduction of the questionnaire using Fisher's exact test. Less than 1% (n = 4/563) of PRP patients were referred to speech pathology prior to implementation of the questionnaire. Following the implementation, referrals to speech pathology significantly increased to 16% (8/50) (X2 = 7.72, P < 0.05; odds ratio = 7.89 95% CI [1.94, 32.1]). Introducing a dysphagia screening questionnaire increased referrals to speech pathology from a PRP. This study demonstrated the potential for a transdisciplinary approach in early screening for patients at risk of dysphagia for patients with COPD. Further research is encouraged to explore patient motivation towards speech pathology input with COPD-related dysphagia and clinicians' perceived self-efficacy in using the questionnaire.

Identifying New Factors Associated With Cognitive Decline and Delirium After Transcatheter Aortic Valve Implantation: A Study Protocol.

Background: Transcatheter aortic valve implantation (TAVI) has become the standard-of-care for treatment of severe symptomatic aortic stenosis and is also being increasingly recommended for low-risk patients. While TAVI boasts positive post-procedural outcomes, it is also associated with cognitive complications, namely delirium and cognitive decline. There is a pressing need for accurate risk tools which can identify TAVI patients at risk of delirium and cognitive decline, as risk scores designed for general cardiovascular surgery fall short. The present effect-finding exploratory study will assess the utility of various measures in the context of aging and frailty in predicting who will and who will not develop delirium or cognitive impairment following TAVI. The measures we propose include gait, visual symptoms, voice, swallowing, mood and sleep. Methods: This is an observational prospective cohort study focused on identifying pre-procedural risk factors for the development of delirium and cognitive decline following TAVI. Potential risk factors will be measured prior to TAVI. Primary outcomes will be post-procedure cognitive decline and delirium. Secondary outcomes include activities of daily living, quality of life, and mortality. Delirium presence will be measured on each of the first 2 days following TAVI. All other outcomes will be assessed at 3-, 6-, and 12-months post-operatively. A series of logistic regressions will be run to investigate the relationship between potential predictors and outcomes (presence vs. absence of either delirium or cognitive decline). Discussion: This study will assess the strengths of associations between a range of measures drawn from frailty and aging literature in terms of association with cognitive decline and delirium following TAVI. Identified measures can be used in future development of TAVI risk prediction models, which are essential for the accurate identification of cognitive at-risk patients and successful application of pre-procedural interventions. Clinical Trial Registration: This trial is registered with the Australian New Zealand Clinical Trials Registry. [https://bit.ly/2PAotP5], [ACTRN12618001114235].

Characterization of Dysphagia and Longitudinal Changes in Swallowing Function in Adults with Niemann-Pick Disease Type C Treated with Miglustat.

Niemann-Pick disease type C (NPC) is a rare, autosomal recessive neurodegenerative disease, characterized by progressive psychiatric and neurological deficits. Neurological symptoms include cognitive decline and dysphagia. Aspiration pneumonia secondary to dysphagia is a leading cause of death in NPC. Miglustat is currently the only approved disease-specific treatment shown to be effective in stabilizing neurological symptoms. Miglustat has previously been reported to halt or improve early dysphagia and cognitive symptoms. Here we examine the characteristics of dysphagia, the relationship between dysphagia and the presence of cognitive impairment, and longitudinal changes in swallowing function during miglustat treatment in adult-and-adolescent-onset NPC. Retrospective analysis of videofluoroscopic swallow studies (VFSS) was completed for ten adults with NPC (mean age 28.44 years ± 9.34 years). Participants were recruited through the Royal Melbourne Hospital in Australia between 2008 and 2015. The Bethlehem Swallowing Scale and the Penetration-Aspiration Scale were used to quantify VFSS data. Dysphagia was present in 90% of participants at baseline with reduced lingual function and a delayed swallowing reflex as the most common symptoms. Swallow impairment appeared to stabilize during miglustat therapy for periods up to 66 months, with no significant changes in scores (p > 0.05). Data were in accordance with the literature and support the use of miglustat as an efficacious treatment for reducing swallowing impairment and stabilizing cognitive function. Findings provide detailed information on the impairments experienced by patients, give context to events leading to aspiration in NPC and, importantly, inform how management of dysphagia can complement pharmaceutical treatment.

Imaging and Clinical Data on Swallowing Function of Individuals with Huntington's Disease and Dysphagia.

BACKGROUND: Dysphagia is common in Huntington's disease (HD) affecting all phases of swallowing. Correlations exist between non-instrumental measures of dysphagia and clinical features of HD, including age, disease duration and degree of motor impairment. Lack of instrumental data limits our ability to wholly characterize HD-related dysphagia and prognosticate swallowing changes over time. OBJECTIVE: To retrospectively describe a relatively large database of videofluoroscopic studies (VFSSs) and determine the relationships between dysphagia and HD clinical parameters, including disease duration and burden of pathology score. METHODS: Medical and swallowing data of 49 individuals with HD and dysphagia were examined. VFSS data were interpreted using the Bethlehem Assessment Scale and Penetration-Aspiration Scale. Data from clinical bedside examination and social information were collated to describe the impact of dysphagia in HD. Repeated VFSS data were available for seven individuals. RESULTS: Swallowing was characterized by lingual dysfunction, reduced soft palate elevation, delayed pharyngeal swallow initiation, and inability to clear matter from the pharynx. Two-thirds of cases presented with compromised airway protection with both liquid and solid consistencies. Tachyphagia and difficulty self-feeding were common. Dysphagia correlated with disease severity and duration. Longitudinal analysis revealed a mixed pattern of progression with some individuals presenting with worsening dysphagia whilst others appeared to remain stable or improved in function. CONCLUSIONS: Dysphagia in HD is exacerbated by difficulties with self-feeding and monitoring feeding rate. Burden of pathology relates to pharyngeal swallow initiation and penetration and aspiration of fluid. Dysphagia did not appear to worsen in a systematic way in a subset of participants.

Changes detected in swallowing function in Friedreich ataxia over 12 months.

Friedreich ataxia (FRDA) is a multisystem neurodegenerative disorder and the most common hereditary ataxia. Dysphagia (swallowing impairment) is present in 98% of individuals with FRDA and is characterized by lingual and pharyngeal dysfunction (manifesting in impaired bolus preparation and transfer, and post-swallow residue in the mouth and pharynx), delayed swallow initiation, and entry of material into the airway (penetration/aspiration). Dysphagia severity correlates with disease severity and duration however no longitudinal studies describe changes in function in FRDA. The aim of this study was to investigate the progression of dysphagia in FRDA over one year. Fifty-nine individuals with FRDA and confirmed dysphagia were recruited and 23 of them underwent a second assessment 12 months later. Assessments of swallowing related quality of life, oral motor function (Frenchay Dysarthria Assessment 2nd Ed [FDA-2]) and functional swallowing via videofluoroscopy were conducted. Trials of thin liquid, puree and biscuit were interpreted using the Bethlehem Assessment Scale and the Penetration-Aspiration Scale by two blinded raters. Data from the videofluoroscopy revealed a decline in tongue function, pharyngeal clearance and cricopharyngeal function on solid food. However, severity of penetration/aspiration did not increase. Swallowing-related quality of life and oral-motor function remained stable. A decline in function was observed at three anatomical sites considered important for safe and effective swallowing (tongue, pharyngeal, and cricopharyngeal). However, these deficits did not translate into any meaningful functional decline in swallowing related health over 12 months for individuals with FRDA.

Dysphagia in Friedreich Ataxia.

The objective of the study was to comprehensively characterise dysphagia in Friedreich ataxia (FRDA) and identify predictors of penetration/aspiration during swallowing. We also investigated the psychosocial impact of dysphagia on individuals with FRDA. Sixty participants with FRDA were screened for dysphagia using a swallowing quality of life questionnaire (Swal-QOL) and case history. Individuals reporting dysphagia underwent a standardised oromotor assessment (Frenchay Dysarthria Assessment, 2, FDA-2) and videofluoroscopic study of swallowing (VFSS). Data were correlated with disease parameters (age at symptom onset, age at assessment, disease duration, FXN intron 1 GAA repeat sizes, and Friedreich Ataxia Rating Scale (FARS) score). Predictors of airway penetration/aspiration were explored using logistic regression analysis. Ninety-eight percent (59/60) of participants reported dysphagia, of whom 35 (58.3%) underwent FDA-2 assessment, and 38 (63.3%) underwent VFSS. Laryngeal, respiratory, and tongue dysfunction was observed on the FDA-2. A Penetration-Aspiration Scale score above 3 (deemed significant airway compromise based on non-clinical groups) was observed on at least one consistency in 13/38 (34.2%) participants. All of those who aspirated (10/38, 26.3%) did so silently, with no overt signs of airway entry such as reflexive cough. Significant correlations were observed between dysphagic symptoms and disease duration and severity. No reliable predictors of penetration or aspiration were identified. Oropharyngeal dysphagia is commonly present in individuals with FRDA and worsens with disease duration and severity. Individuals with FRDA are at risk of aspiration at any stage of the disease and should be reviewed regularly. Instrumental analysis remains the only reliable method to detect aspiration in this population. Dysphagia significantly affects the quality of life of individuals with FRDA.

Treatment for dysphagia (swallowing difficulties) in hereditary ataxia.

BACKGROUND: Hereditary ataxias are a heterogeneous group of disorders resulting in progressive inco-ordination. Swallowing impairment, also known as dysphagia, is a common and potentially life threatening sequel of disease progression. The incidence and nature of dysphagia in these conditions is largely unknown. The loss of an effective and safe swallow can dramatically affect the health and well-being of an individual. Remediation of difficulties of eating and drinking is an important goal in the clinical care of people with hereditary ataxia. OBJECTIVES: To assess the effects of interventions for swallowing impairment (dysphagia) in people with hereditary ataxias. SEARCH METHODS: We searched the Cochrane Neuromuscular Disease Group Specialized Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, CINAHL Plus, PsycINFO, and the Education Resources Information Center (ERIC) on 14 September 2015. We also searched Linguistics and Language Behavior Abstracts (LLBA), Dissertation Abstracts, and Trials Registries on 24 September 2015. SELECTION CRITERIA: We considered all randomised controlled trials (RCTs) and quasi-RCTs that compared treatments for hereditary ataxia with placebo or no treatment. We only included studies measuring dysphagia. DATA COLLECTION AND ANALYSIS: Three review authors (ES, KJ, MK) independently screened all titles and abstracts. In the event of any disagreement or uncertainty over the inclusion of a particular paper, the review authors planned to meet and reach consensus. MAIN RESULTS: We identified no RCTs from the 519 titles and abstracts screened. We excluded papers primarily for not including participants with a hereditary ataxia (that is, being focused on other neurological conditions), being theoretical reviews rather than intervention studies, or being neither randomised nor quasi-randomised trials.We identified five papers of various design that described treatment for dysphagia, or improvement to swallow as a by-product of treatment, in people with hereditary ataxia. None of these studies were RCTs or quasi-RCTs. AUTHORS' CONCLUSIONS: There is an absence of any significant evidence supporting the use of any dysphagia intervention in hereditary ataxia. The lack of evidence highlights the critical need for well-controlled treatment trials in the field.

A systematic review of self-reported swallowing assessments in progressive neurological disorders.

Dysphagia experienced as a consequence of neurodegenerative disease can have severe consequences on a patient's health and well-being. Regular assessment of swallowing function can assist to achieve adequate nutrition and hydration. Here we review subjective swallowing assessments currently available are suitable for use in people with neurodegenerative disease. Measurement properties were reviewed for each tool and coverage of the World Health Organization's International Classification of Functioning, Disability and Health (WHO ICF) was considered. Assessments were identified following a review of the published literature Instruments were reviewed on the basis of reliability and validity, as well as administrative properties, such an interpretability, acceptability, and feasibility. Tools were also evaluated according to the WHO ICF framework. In total, 19 studies were identified for full-text review from 13,315 abstracts. Nine self-reported dysphagia assessment tools suitable for use in progressive neurological disorders were identified. The Swallowing Quality of Life Questionnaire (SWAL-QOL) yields the strongest combination of reliability (including internal consistency and test-retest reliability) and convergent validity while simultaneously covering all WHO ICF domains. Lengthy administration time was identified as a limitation of the SWAL-QOL. The review highlights a relative lack of well-validated self-report questionnaires in dysphagia for people with progressive neurological disease. Additional validation and evaluation of the clinical utility of the tools currently available is required to further promote an informed selection of available assessments.