Telespirometry in Amyotrophic Lateral Sclerosis (ALS)

Study Overview
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Brief Summary
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The investigators seek to validate Slow Vital Capacity (SVC) measurement in seated and supine positions using conventional and portable spirometry.

Detailed Description
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SVC is obtained with in-clinic conventional spirometry, in-clinic portable spirometry, and in-home portable spirometry with respiratory therapist-supervised remote pulmonary function testing every two weeks in a six month prospective study of participants with a diagnosis of Amyotrophic Lateral Sclerosis. SVC decline over time, seated and supine, will be tracked with assessments for treatment changes.

Official Title
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Home-Based Spirometry Through Telemedicine in Amyotrophic Lateral Sclerosis (ALS)

Conditions
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Amyotrophic Lateral Sclerosis

Intervention / Treatment
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* Diagnostic Test: Spirometry


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Diagnosis of ALS as Clinically Possible, Clinically Probable, Laboratory-supported Probable and Clinically Definite ALS 18 years old to 100 years old, English-speaking ALS subjects, male and female Exclusion Criteria: Use of non-invasive ventilation more than 16 hours daily Non-English Speaker Psychosis or severe mental illness Use of high-dose sedating psychotropic medications determined to potentially interfere with task performance Infection Control issues and specific Pulmonary, Cardiac, Vascular contraindications as listed in the Standardization of Spirometry 2019 Update (Graham 2019)

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 100 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| SUNY Upstate<br>One of 2 ALS certified treatment centers in USA expected to recruit 50 subjects. | Diagnostic Test: Spirometry<br>* All study participants will undergo pulmonary function testing using conventional spirometry in clinic and portable spirometry in clinic and at home. In-clinic conventional laboratory spirometry is compared with portable spirometry and Slow vital capacity is obtained in upright and supine positions.<br>* Other names: Pulmonary Function Test;|
| Atrium Health<br>One of 2 ALS certified treatment centers in USA expected to recruit 50 subjects. | Diagnostic Test: Spirometry<br>* All study participants will undergo pulmonary function testing using conventional spirometry in clinic and portable spirometry in clinic and at home. In-clinic conventional laboratory spirometry is compared with portable spirometry and Slow vital capacity is obtained in upright and supine positions.<br>* Other names: Pulmonary Function Test;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change from baseline in ALS Functional Rating Scale, Revised (ALS FRSR) | Minimum 0, Maximum 48; Lower scores worse | 2 week intervals for 6 months | 
| Change from baseline SVC percent predicted at clinic | SVC measured at clinic in seated and supine positions using conventional and portable spirometer | 3 months | 
| Change from baseline SVC percent predicted at clinic | SVC measured at clinic in seated and supine positions using conventional and portable spirometer | 6 months | 
| Change from baseline SVC percent predicted at home | SVC measured from home in seated and supine positions using portable spirometer | 2 week intervals for 6 months | 
| Change from baseline Dyspnea in Amyotrophic Lateral Sclerosis 15 (DALS-15) | Minimum 0, Maximum 30; Higher scores worse | 2 week intervals for 6 months | 
| Change from baseline Hospital Anxiety and Depression Scale (HADS) | Minimum 0, Maximum 42; Higher scores worse | 6 months | 
| Change from baseline Amyotrophic Lateral Sclerosis Assessment Questionnaire-5 (ALSAQ-5) at 6 months | Minimum 0, Maximum 20; Higher scores worse | 6 months | 
| Change from baseline Amyotrophic Lateral Sclerosis Cognitive Behavioral Screen (ALS-CBCS) | Minimum 0, Maximum 20; Lower scores worse | 6 months | 
| Change from baseline Amyotrophic Lateral Sclerosis Cognitive Behavioral Screen (ALS-CBCS) ALS Caregiver Behavioral Questionnaire at 6 months | Minimum 0, Maximum 45; Lower scores worse | 6 months | 
| Change from baseline Amyotrophic Lateral Sclerosis Treatment Questionnaire | Reports usage of non-invasive ventilation, gastrostomy tube, ALS medications and ALS devices | 2 week intervals for 6 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Pulmonary Function Test, Amyotrophic Lateral Sclerosis, Slow Vital Capacity