Home-monitoring of vital capacity in people with a motor neuron disease

BACKGROUND: Home-monitoring of spirometry has the potential to improve care for patients with a motor neuron disease (MND) by enabling early detection of respiratory dysfunction and reducing travel burden. Our aim was to evaluate the validity and feasibility of home-monitoring vital capacity (VC) in patients with MND. METHODS: We included 33 patients with amyotrophic lateral sclerosis, progressive muscular atrophy or primary lateral sclerosis who completed a 12-week home-monitoring protocol, consisting of 4-weekly unsupervised home assessments of VC and a functional rating scale. At baseline, during a home visit, patients/caregivers were trained in performing a VC test, and the investigator performed a supervised VC test, which was repeated at final follow-up during a second home visit. Validity of the unsupervised VC tests was evaluated by the differences between supervised and unsupervised VC tests, and through Bland-Altman 95% limits-of-agreement. Feasibility was assessed by means of a survey of user-experiences. RESULTS: The 95% limits-of-agreement were [- 14.3; 11.7] %predicted VC, and 88% of unsupervised VC tests fell within 10%predicted of supervised VC. 88% of patients experienced VC testing as easy and not burdensome, however, 15% patients did not think their VC test was performed as well as in the clinic. 94% of patients would like home-monitoring of VC in MND care. DISCUSSION: Unsupervised VC testing at home, with prior face-to-face training, is a valid and time-efficient method for the remote monitoring of respiratory function, and well-accepted by patients with MND and their caregivers

Telehealth as part of specialized ALS care: feasibility and user experiences with "ALS home-monitoring and coaching"

Objective: To evaluate the use of telehealth as part of specialized care for patients with amyotrophic lateral sclerosis (ALS) and the user experiences of patients and healthcare professionals. Methods: Fifty patients with ALS were recruited from a single specialist center and used telehealth, consisting of an ALS-app for self-monitoring and messaging, alerts for symptom-worsening, and nurse practitioner follow-up. Patients self-monitored their well-being (daily report), body weight (weekly) and functional status (monthly). The use of the telehealth service was evaluated through adoption rate, dropout rate and adherence to self-monitoring. User-experiences were collected through online surveys among 23 patients and nine healthcare professionals, and interviews with 12 patients. Results: The adoption rate was 80%, dropout rate 4% and median follow-up was 11 months. Good adherence was seen in 49% of patients for well-being, 83% for body weight and 87% for functional assessment. For patients who discontinued using telehealth due to the end-of-life phase, median time between last measurement and death was 19 days. The majority of patients experienced using telehealth as easy, helpful, not burdensome, and reported satisfaction with flexible clinic visits and the continuity of care. Healthcare professionals reported that telehealth was of added value in ALS-care. Conclusions: ALS-care supplemented by home-monitoring and nurse practitioner follow-up was shown to be suitable and widely accepted by patients and healthcare professionals in our ALS clinic. Success factors were low self-monitoring burden, a user-friendly platform and the provision of personalized feedback. Further research is needed to replicate these findings in other ALS clinics

Using patient-reported symptoms of dyspnea for screening reduced respiratory function in patients with motor neuron diseases

BACKGROUND: Poor monitoring of respiratory function may lead to late initiation of non-invasive ventilation (NIV) in patients with motor neuron diseases (MND). Monitoring could be improved by remotely assessing hypoventilation symptoms between clinic visits. We aimed to determine which patient-reported hypoventilation symptoms are best for screening reduced respiratory function in patients with MND, and compared them to the respiratory domain of the amyotrophic lateral sclerosis functional rating scale (ALSFRS-R). METHODS: This prospective multi-center study included 100 patients with MND, who were able to perform a supine vital capacity test. Reduced respiratory function was defined as a predicted supine vital capacity ≤ 80%. We developed a 14-item hypoventilation symptom questionnaire (HYSQ) based on guidelines, expert opinion and think-aloud interviews with patients. Symptoms of the HYSQ were related to dyspnea, sleep quality, sleepiness/fatigue and pneumonia. The diagnostic performances of these symptoms and the ALSFRS-R respiratory domain were determined from the receiver operating characteristic (ROC) curves, area under the curve (AUC), sensitivity, specificity, predictive values and accuracy. RESULTS: Dyspnea-related symptoms (dyspnea while eating/talking, while lying flat and during light activity) were combined into the MND Dyspnea Scale (MND-DS). ROC curves showed that the MND-DS had the best diagnostic performance, with the highest AUC = 0.72, sensitivity = 75% and accuracy = 71%. Sleep-quality symptoms, sleepiness/fatigue-related symptoms and the ALSFRS-R respiratory domain showed weak diagnostic performance. CONCLUSION: The diagnostic performance of the MND-DS was better than the respiratory domain of the ALSFRS-R for screening reduced respiratory function in patients with MND, and is, therefore, the preferred method for (remotely) monitoring respiratory function

Using telehealth to optimize care for people with a motor neuron disease: The digital road to personalized care

Motor neuron disease (MND) is a rapidly progressive neurodegenerative disease that leads to loss of motor neurons and eventually leads to paralysis of most voluntary muscles. The initial symptoms of MND may occur in the arms or legs, known as a spinal onset, or show in difficulties with speech and swallowing, known as a bulbar onset. The average disease duration is 2 to 4 years, and the main cause of death in patients with MND is respiratory failure, due to severe respiratory muscle weakness. Patients with respiratory muscle weakness, can develop hypoventilation, which leads to shortness of breath, fatigue and sleepiness during the day, and restless sleep during the night. Patients with MND require multidisciplinary care, which is aimed at optimizing participation and quality of life and includes 3 to 4 monthly visits to a multidisciplinary clinic. Besides improving quality of life, multidisciplinary MND care has shown to also prolong survival, compared to non-specialized care. One of the most effective treatments for improving survival in patients with MND is non-invasive ventilation (NIV), as it provides ventilatory support when patients are respiratory insufficient. A drawback of multidisciplinary MND care, is that accessing and receiving care at a multidisciplinary clinic can be timeconsuming and physically challenging for patients with MND, due to their physical disability. This results in considerable burden of care, and may hinder the continuity of care, which can increase distress and negatively affect patients’ psychological well-being. Telehealth, which is the remote provision of care, holds the potential to improve accessibility and personalization, and reduce burden of multidisciplinary MND care, by enabling frequent monitoring of patients from home. Despite these promising benefits of telehealth and the wide availability of digital technologies, the use of telehealth in care for patients with MND is limited. For these reasons it would be of great benefit to know what factors determine the success of the implementation of telehealth in MND care, and what methods can best be used for remote monitoring of patients with MND. In particular, the remote monitoring of respiratory function is important, as it can help healthcare professionals to detect respiratory decline earlier and optimize the timing of NIV. In order to determine which respiratory measures are appropriate for remote monitoring in a home setting, their validity and feasibility should be investigated. This thesis aims to expand the knowledge on the use and implementation of telehealth in multidisciplinary MND care, and identify adequate methods for the remote monitoring of patients with MND

The current use of telehealth in ALS care and the barriers to and facilitators of implementation: a systematic review

Objective: We aimed to provide an overview of telehealth used in the care for patients with amyotrophic lateral sclerosis (ALS), and identify the barriers to and facilitators of its implementation. Methods: We searched Pubmed and Embase to identify relevant articles. Full-text articles with original research reporting on the use of telehealth in ALS care, were included. Data were synthesized using the Consolidation Framework for Implementation Research. Two authors independently screened articles based on the inclusion criteria. Results: Sixteen articles were included that investigated three types of telehealth: Videoconferencing, home-based self-monitoring and remote NIV monitoring. Telehealth was mainly used by patients with respiratory impairment and focused on monitoring respiratory function. Facilitators for telehealth implementation were a positive attitude of patients (and caregivers) toward telehealth and the provision of training and ongoing support. Healthcare professionals were more likely to have a negative attitude toward telehealth, due to the lack of personal evaluation/contact and technical issues; this was a known barrier. Other important barriers to telehealth were lack of reimbursement and cost-effectiveness analyses. Barriers and facilitators identified in this review correspond to known determinants found in other healthcare settings. Conclusions: Our findings show that telehealth in ALS care is well-received by patients and their caregivers. Healthcare professionals, however, show mixed experiences and perceive barriers to telehealth use. Challenges related to finance and legislation may hinder telehealth implementation in ALS care. Future research should report the barriers and facilitators of implementation and determine the cost-effectiveness of telehealth

Sensitivity to change of the field-based Wheelchair Mobility Performance Test in wheelchair basketball

In this study we measured the performance times on the Wheelchair Mobility Performance (WMP) test during different test conditions to see if the performance times changed when wheelchair settings were changed. The overall performance time on the WMP test increased when the tire pressure was reduced and also when extra mass was attached to the wheelchair. It can be concluded that the WMP test is sensitive to changes in wheelchair settings. It is recommended to use this field-based test in further research to investigate the effect of wheelchair settings on mobility performance time. Objective: The Wheelchair Mobility Performance (WMP) test is a reliable and valid measure to assess mobility performance in wheelchair basketball. The aim of this study was to examine the sensitivity to change of the WMP test by manipulating wheelchair configurations. Methods: Sixteen wheelchair basketball players performed the WMP test 3 times in their own wheelchair: (i) without adjustments (“control condition”); (ii) with 10 kg additional mass (“weighted condition”); and (iii) with 50% reduced tyre pressure (“tyre condition”). The outcome measure was time (s). If paired t-tests were significant (p < 0.05) and differences between conditions were larger than the standard error of measurement, the effect sizes (ES) were used to evaluate the sensitivity to change. ES values ≥0.2 were regarded as sensitive to change. Results: The overall performance times for the manipulations were significantly higher than the control condition, with mean differences of 4.40 s (weight – control, ES = 0.44) and 2.81 s (tyre – control, ES = 0.27). The overall performance time on the WMP test was judged as sensitive to change. For 8 of the 15 separate tasks on the WMP test, the tasks were judged as sensitive to change for at least one of the manipulations. Conclusion: The WMP test can detect change in mobility performance when wheelchair configurations are manipulated. https://www.medicaljournals.se/jrm/content/html/10.2340/16501977-234

Sensitivity to change of the field-based Wheelchair Mobility Performance Test in wheelchair basketball

OBJECTIVE: The Wheelchair Mobility Performance (WMP) test is a reliable and valid measure to assess mobility performance in wheelchair basketball. The aim of this study was to examine the sensitivity to change of the WMP test by manipulating wheelchair configurations. METHODS: Sixteen wheelchair basketball players performed the WMP test 3 times in their own wheelchair: (i) without adjustments ("control condition"); (ii) with 10 kg additional mass ("weighted condition"); and (iii) with 50% reduced tyre pressure ("tyre condition"). The outcome measure was time (s). If paired t-tests were significant (p < 0.05) and differences between conditions were larger than the standard error of measurement, the effect sizes (ES) were used to evaluate the sensitivity to change. ES values ≥0.2 were regarded as sensitive to change. RESULTS: The overall performance times for the manipulations were significantly higher than the control condition, with mean differences of 4.40 s (weight - control, ES = 0.44) and 2.81 s (tyre - control, ES = 0.27). The overall performance time on the WMP test was judged as sensitive to change. For 8 of the 15 separate tasks on the WMP test, the tasks were judged as sensitive to change for at least one of the manipulations. CONCLUSION: The WMP test can detect change in mobility performance when wheelchair configurations are manipulated