Amyotrophic Lateral Sclerosis
Conditions
Keywords
Pulmonary Function Test, Amyotrophic Lateral Sclerosis, Slow Vital Capacity
Brief summary
The investigators seek to validate Slow Vital Capacity (SVC) measurement in seated and supine positions using conventional and portable spirometry.
Detailed description
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.
Interventions
DIAGNOSTIC_TESTSpirometry
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.
Sponsors
Tanabe Pharma America, Inc.
State University of New York - Upstate Medical University
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 100 Years
Healthy volunteers
No
Inclusion criteria
1. Diagnosis of ALS as Clinically Possible, Clinically Probable, Laboratory-supported Probable and Clinically Definite ALS 2. 18 years old to 100 years old, English-speaking ALS subjects, male and female
Exclusion criteria
1. Use of non-invasive ventilation more than 16 hours daily 2. Non-English Speaker 3. Psychosis or severe mental illness 4. Use of high-dose sedating psychotropic medications determined to potentially interfere with task performance 5. Infection Control issues and specific Pulmonary, Cardiac, Vascular contraindications as listed in the Standardization of Spirometry 2019 Update (Graham 2019)
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Change from baseline in ALS Functional Rating Scale, Revised (ALS FRSR) | 2 week intervals for 6 months | Minimum 0, Maximum 48; Lower scores worse |
| Change from baseline SVC percent predicted at clinic | 3 months | SVC measured at clinic in seated and supine positions using conventional and portable spirometer |
| Change from baseline SVC percent predicted at home | 2 week intervals for 6 months | SVC measured from home in seated and supine positions using portable spirometer |
| Change from baseline Dyspnea in Amyotrophic Lateral Sclerosis 15 (DALS-15) | 2 week intervals for 6 months | Minimum 0, Maximum 30; Higher scores worse |
| Change from baseline Hospital Anxiety and Depression Scale (HADS) | 6 months | Minimum 0, Maximum 42; Higher scores worse |
| Change from baseline Amyotrophic Lateral Sclerosis Assessment Questionnaire-5 (ALSAQ-5) at 6 months | 6 months | Minimum 0, Maximum 20; Higher scores worse |
| Change from baseline Amyotrophic Lateral Sclerosis Cognitive Behavioral Screen (ALS-CBCS) | 6 months | Minimum 0, Maximum 20; Lower scores worse |
| Change from baseline Amyotrophic Lateral Sclerosis Cognitive Behavioral Screen (ALS-CBCS) ALS Caregiver Behavioral Questionnaire at 6 months | 6 months | Minimum 0, Maximum 45; Lower scores worse |
| Change from baseline Amyotrophic Lateral Sclerosis Treatment Questionnaire | 2 week intervals for 6 months | Reports usage of non-invasive ventilation, gastrostomy tube, ALS medications and ALS devices |
Countries
United States
Outcome results
None listed