Living with Dysphagia: A Survey Exploring the Experiences of Adults Living with Neuromuscular Disease and their Caregivers in the United Kingdom.

Background: Dysphagia is common in adults living with neuromuscular disease (NMD). Increased life expectancy, secondary to improvements in standards of care, requires the recognition and treatment of dysphagia with an increased priority. Evidence to support the establishment of healthcare pathways is, however, lacking. The experiences of people living with NMD (pplwNMD) and their caregivers are valuable to guide targeted, value-based healthcare. Objective: To generate preliminary considerations for neuromuscular dysphagia care and future research in the United Kingdom, based on the experiences of those living with, or caring for, people with NMD. Methods: Two surveys (one for adults living with NMD and dysphagia, and a second for caregivers) were co-designed with an advisory group of people living with NMD. Surveys were electronically distributed to adults living with NMD and their caregivers between 18th May and 26th July 2020. Distribution was through UK disease registries, charity websites, newsletters, and social media. Results: Adults living with NMD receive little information or education that they are likely to develop swallowing difficulties. Most respondents report wanting this information prior to developing these difficulties. Difficulties with swallowing food and medication are common in this group, and instrumental assessment is considered a helpful assessment tool. Both adults living with NMD and caregivers want earlier access to neuromuscular swallowing specialists and training in how best to manage their difficulties. Conclusions: Improvement is needed in the dysphagia healthcare pathway for adults living with NMD to help mitigate any profound physical and psychological consequences that may be caused by dysphagia. Education about swallowing difficulties and early referral to a neuromuscular swallowing specialist are important to pplwNMD and their caregivers. Further research is required to better understand the experiences of pplwNMD and their caregivers to inform the development of dysphagia healthcare pathways.

Expiratory muscle strength training improves measures of pressure generation and cough strength in a patient with myotonic dystrophy type 1.

Expiratory muscle strength training (EMST) exercise programmes aim to improve respiratory function by increasing the force generating capability of expiratory muscles by resistance training. In neuromuscular conditions, in which cough flow generation is often decreased, there is increasing interest in EMST as a therapeutic intervention. We present data showing efficacy of EMST in a patient with adult onset Myotonic Dystrophy Type 1 (DM1). A domiciliary training programme (5 days per week over 32 weeks) resulted in increases in maximum expiratory mouth pressure (from 15 cmH2O to 38 cmH2O) and peak cough flow (300 L/min to 390 L/min). Improvements were also seen in maximum inspiratory mouth pressure (26 cmH2O to 52 cmH2O) and sniff nasal inspiratory pressure (40 cmH2O to 69 cmH2O). No changes were detected in speech or swallowing. This novel study demonstrates that cough flow generation in DM1 may be increased by a programme of expiratory muscle training. A clinical trial of EMST in DM1 is warranted.

Evaluation of the Bayer microalbumin/creatinine urinalysis dipstick.

BACKGROUND: The objective of this study was to evaluate the utility of the Bayer microalbumin/creatinine urine reagent strip compared to established laboratory methods. METHODS: Random urine specimens from low and high risk pregnancy clinics as well as a random cohort from the community were analyzed for microalbumin and creatinine using both the reagent strip and the Roche Integra 700, according to manufacturers' specifications. Sensitivity and specificity were then calculated. RESULTS: For the pregnant cohorts the sensitivities ranged from 19% to 59%, and the range of specificities was 45.4% to 84.2%. Using the microalbumin/creatinine data from the community, the sensitivity and specificity of the strip were 52.4% and 97.6%, respectively. CONCLUSION: The poor sensitivity of the microalbumin/creatinine urine reagent strip to detect significant microalbuminuria will likely limit its usefulness as a screening tool.