Amyotrophic Lateral Sclerosis
Conditions
Keywords
telemonitoring, digital health, mobile health, dysphagia, dysarthria
Brief summary
The investigators propose a longitudinal home study of ALS patients to measure the severity of speech and swallowing (bulbar) impairment via a smartphone-based, remote speech and swallow assessment (rSSA). The study is designed to assess the feasibility and validity of such a monitoring intervention. Furthermore, it is proposed that regular monitoring of these two bulbar processes may shed light on their co-evolution over the course of ALS.
Detailed description
Most individuals with ALS experience bulbar function deterioration over the course of the disease which impacts their quality of life significantly. Furthermore, in some patients, bulbar symptoms are an initial presenting symptom of ALS. Identifying these changes, which may be rapid in some individuals, is complicated by the recent acceleration of virtual care delivery. The investigators propose a longitudinal home study of ALS patients to assess bulbar progression via a smartphone-based, self-administered remote speech and swallow assessment (rSSA). The overall hypothesis is that this monitoring protocol can be used in a way that, 1) is satisfactory to the patient, 2) performs at least as well as standard clinical measures of dysarthria and dysphagia, and 3) resolves the development of emergence of speech and swallowing pathologies in ALS.
Interventions
DEVICEDigital recording of speech and swallow sounds using the Beiwe Digital Health App on a personal smartphone
\[Patients only\] Weekly completion of audio recordings of speech and swallowing performed at home by the subject using the study smartphone application on their personal device.
RADIATIONModified Barium Swallow Study (MBSS)
\[Patients only\] MBSS is an instrumented evaluation of oropharyngeal swallowing function that is performed using videofluoroscopy.
BEHAVIORALSpeech Intelligibility Test (SIT)
\[Patients only\] The SIT is a standardized test for measuring speech intelligibility.
BEHAVIORALSIT Scoring
\[Listeners only\] Listeners will judge the intelligibility of speech recordings made by patients in the study.
Sponsors
Milton S. Hershey Medical Center
Penn State University
Congressionally Directed Medical Research Programs
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
Yes
Inclusion criteria
Patients 1. At least 18 years of age 2. Possess a diagnosis of ALS (definite, probable, probable laboratory-supported, or possible by El Escorial criteria \[35\]) 3. Have symptom onset within the last 3 years 4. Demonstrate a score of 2 or 3 in either the speech (item #1) or swallowing (item #3) components of the ALSFRS-R at time of enrollment 5. Demonstrate decline in bulbar function prior to enrollment, measured by a reduction of at least 1 point on either the speech or swallowing components of the ALSFRS-R in the 6 months prior to enrollment. If 6 months of data is not available, an estimate of the change in ALSFRS-R will be calculated from the time of symptom onset. 6. Have a life expectancy ≥ 6 months as determined by the attending neurologist 7. Be fluent in written and spoken English 8. Possess a smartphone capable of running the study application 9. Follow up in the Penn State Hershey multidisciplinary ALS clinic over next 6 months 10. Be able to visit the study site for in-person procedures at weeks 0 and 24 Listeners 1. Between 18 and 40 years of age 2. Native speakers of American English 2.3. Currently residing within the United States. 3.4. Hearing within normal limits, as defined by self report.
Exclusion criteria
Patients 1. Possess a co-existing neurological or psychiatric illness that, in the opinion of the study team, exclude the subject from participation 2. Demonstrate clinically significant dementia, as determined by the ALS study neurologist 3. Possess abnormal speech or swallowing processes due to a condition independent of their ALS diagnosis Listeners 1. Have an identified speech, language, learning, or neurological disorders per self-report 2. Experience communicating with people with motor speech disorders
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Digital recording of speech and swallow | weekly for 24 weeks | Audio recordings of speech tasks and spontaneous swallowing task |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Speech Intelligibility Task (SIT) Intelligibility listener scoring | Weeks 0, 12, and 24 | Listeners will score the intelligibility of patient SIT recordings |
| ALS Functional Rating Scale - Revised (ALSFRS-R) | Weeks 0, 12, and 24 | ALS Specific Assessment of physical function ranging from 0-48, with 48 being normal function. |
| Center for Neurological Study - Bulbar Function Scale (CNS-BFS) | Weeks 0, 4, 8, 12, 16, 20, and 24 | Self-administered rating scale for speech (7-49) and salivation (7-35) functions, with high scores indicating impaired function. |
| MBS Impairment Profile (MBSImp) | Weeks 0 and 24 | Standardized scoring for the MBSS in 17 domains. Domains are aggregated in to scores for Oral Impairment (0-22), Pharyngeal Impairment (0-29), and Esophageal Impairment (0-4), with higher scores indicating impaired function. |
| EAT-10 Questionnaire | Weeks 0, 12, and 24 | A self-reported assessment of eating and swallowing function. Scores range from 0-40, with higher scores indicating higher impairment. |
| Forced Vital Capacity (FVC) | Weeks 0, 12, and 24 | A standard clinical respiratory assessment of vital capacity |
| Maximal Inspiratory Pressure (MIP) | Weeks 0, 12, and 24 | A standard clinical respiratory assessment of inspiratory strength |
| Mann Assessment of Swallowing Ability (MASA) | Weeks 0, 12, and 24 | A standard clinical swallowing assessment performed by the speech-language pathologist. Scores range from 38-200, with lower scores indicating higher impairment. |
Countries
United States
Contacts
PRINCIPAL_INVESTIGATORAndrew Geronimo, PhD
Penn State College of Medicine
Outcome results
None listed