Telespirometry in Amyotrophic Lateral Sclerosis (ALS)

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

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

Study Overview

• Status: Enrolling by invitation
• Conditions: Amyotrophic Lateral Sclerosis
• Intervention / Treatment: Diagnostic test: 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.

• Study Type: Observational
• Enrollment (Estimated): 100

Contacts and Locations

Study Locations

• United States
  • New York
    • Syracuse, New York, United States, 13210
      • SUNY Upstate
  • North Carolina
    • Charlotte, North Carolina, United States, 28211
      • Atrium Health

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 14 years to 96 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No

• Sampling Method: Probability Sample

Study Population

Subjects are invited to volunteer and recruited from ALS specialty care outpatient clinics at SUNY Upstate and Atrium Health.

Description

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)

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
SUNY Upstate; One of 2 ALS certified treatment centers in USA expected to recruit 50 subjects.	Diagnostic test: Spirometry; 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.; Other Names: Pulmonary Function Test
Atrium Health; One of 2 ALS certified treatment centers in USA expected to recruit 50 subjects.	Diagnostic test: Spirometry; 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.; Other Names: Pulmonary Function Test

What is the study measuring?

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

Collaborators and Investigators

• Sponsor: State University of New York - Upstate Medical University
• Collaborators: Mitsubishi Tanabe Pharma America Inc.
• Investigators: Principal Investigator: Eufrosina Young, MD, SUNY Upstate

Publications and helpful links

General Publications

• Graham BL, Steenbruggen I, Miller MR, Barjaktarevic IZ, Cooper BG, Hall GL, Hallstrand TS, Kaminsky DA, McCarthy K, McCormack MC, Oropez CE, Rosenfeld M, Stanojevic S, Swanney MP, Thompson BR. Standardization of Spirometry 2019 Update. An Official American Thoracic Society and European Respiratory Society Technical Statement. Am J Respir Crit Care Med. 2019 Oct 15;200(8):e70-e88. doi: 10.1164/rccm.201908-1590ST.
• Geronimo A, Simmons Z. Evaluation of remote pulmonary function testing in motor neuron disease. Amyotroph Lateral Scler Frontotemporal Degener. 2019 Aug;20(5-6):348-355. doi: 10.1080/21678421.2019.1587633. Epub 2019 Apr 7.
• Hegewald MJ, Gallo HM, Wilson EL. Accuracy and Quality of Spirometry in Primary Care Offices. Ann Am Thorac Soc. 2016 Dec;13(12):2119-2124. doi: 10.1513/AnnalsATS.201605-418OC.
• Couratier P, Vincent F, Torny F, Lacoste M, Melloni B, Lemaire F, Antonini MT. Spirometer-dependence of vital capacity in ALS: validation of a portable device in 52 patients. Amyotroph Lateral Scler Other Motor Neuron Disord. 2005 Dec;6(4):239-45. doi: 10.1080/14660820510043244.
• Masa JF, Gonzalez MT, Pereira R, Mota M, Riesco JA, Corral J, Zamorano J, Rubio M, Teran J, Farre R. Validity of spirometry performed online. Eur Respir J. 2011 Apr;37(4):911-8. doi: 10.1183/09031936.00011510. Epub 2010 Jul 22.
• Rutkove SB, Qi K, Shelton K, Liss J, Berisha V, Shefner JM. ALS longitudinal studies with frequent data collection at home: study design and baseline data. Amyotroph Lateral Scler Frontotemporal Degener. 2019 Feb;20(1-2):61-67. doi: 10.1080/21678421.2018.1541095. Epub 2018 Nov 28.

Study record dates

Study Major Dates

• Study Start (Actual): April 28, 2021
• Primary Completion (Estimated): June 30, 2024
• Study Completion (Estimated): December 30, 2024

Study Registration Dates

• First Submitted: October 7, 2021
• First Submitted That Met QC Criteria: November 2, 2021
• First Posted (Actual): November 3, 2021

Study Record Updates

• Last Update Posted (Actual): June 13, 2023
• Last Update Submitted That Met QC Criteria: June 9, 2023
• Last Verified: June 1, 2023

More Information

Terms related to this study

• Keywords: Amyotrophic Lateral Sclerosis; Pulmonary Function Test; Slow Vital Capacity
• Additional Relevant MeSH Terms: Pathologic Processes; Metabolic Diseases; Central Nervous System Diseases; Nervous System Diseases; Neuromuscular Diseases; Neurodegenerative Diseases; Spinal Cord Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Sclerosis; Motor Neuron Disease; Amyotrophic Lateral Sclerosis
• Other Study ID Numbers: 1660190

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: YES
• IPD Plan Description: The plan is to share Individual Patient Data collected during the study to research team from both sites.
• IPD Sharing Time Frame: 2 years.
• IPD Sharing Access Criteria: Research team includes Principal investigators, sub-investigators, clinical research coordinators and respiratory therapists at each site, Pulmonologist Central Readers at SUNY Upstate. Deidentified data is shared with study sponsor MTPA.
• IPD Sharing Supporting Information Type: STUDY_PROTOCOL; SAP; ICF; ANALYTIC_CODE; CSR

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: Yes
• product manufactured in and exported from the U.S.: No