Reliable Monitoring of Respiratory Function with Home Spirometry in People Living with Amyotrophic Lateral Sclerosis

Julian Peller^1*Marcos Alberto Trevisan^2,3Gaston Bujia¹Felipe Aguirre¹Diego Edgar Shalom^2,3Alan Taitz⁴Stephanie Henze¹Silviya Bastola¹Jason Osik⁵Ryan Shewcraft⁵Peng Jiang⁵Joel Schwartz⁵Terry Heiman-Patterson⁶Michael Sherman⁷Matthew F Wipperman⁸Oren Levy⁸Guofa Shou⁹Karl Sillay¹Lyle Ostrow⁶Ernest Fraenkel¹⁰James Berry¹¹Indu Navar Bingham¹Esteban Roitberg^1,12

• ¹EverythingALS, Seattle, California, United States
• ²Universidad de Buenos Aires, Facultad de Ciencias Exactas y Naturales, Departamento de Física., Buenos Aires, Argentina
• ³CONICET - Universidad de Buenos Aires, Instituto de Física Interdisciplinaria y Aplicada (INFINA), Buenos Aires, Argentina
• ⁴SRI International, Menlo Park, California, United States
• ⁵Bristol Myers Squibb, Cambridge, United States
• ⁶Department of Neurology, Lewis Katz School of Medicine, Temple University, Philadelphia, Pennsylvania, United States
• ⁷Department of Medicine, MCG-Hearst Health, Drexel University College of Medicine, Philadelphia, United States
• ⁸Regeneron Pharmaceuticals, Inc., Tarrytown, New York, United States
• ⁹Eli Lilly and Company, Boston, United States
• ¹⁰Department of Biological Engineering, School of Engineering, Massachusetts Institute of Technology, Cambridge, Massachusetts, United States
• ¹¹Sean M. Healey and AMG Center for ALS, Massachusetts General Hospital & Harvard Medical Schoo, Boston, United States
• ¹²Escuela de Ciencia y Tecnología, Universidad Nacional de General San Martín, Buenos Aires, Buenos Aires, Argentina

Monitoring respiratory function is essential for assessing the progression of Amyotrophic Lateral Sclerosis (ALS) and planning interventions. Using spirometry data from the Radcliff Study (a fully remote, longitudinal, exploratory study with a cohort of 67 pALS) we demonstrate that flexible coaching, combined with a quality control analysis that excludes values of `0' and timepoints with failed measurement trials, produces consistent remote spirometry results. Our findings indicate that home-measured Slow Vital Capacity (SVC) and Forced Vital Capacity (FVC) evolve similarly and progress linearly over the study period (7.7 ± months). This remains true in both slow and fast progressor subpopulations. This observed linearity in respiratory trajectories supports the potential for early, accurate estimation of progression, reinforcing the feasibility of less frequent monitoring without compromising assessment precision, and reducing the burden on both pALS and the healthcare system. Furthermore, our results align with reported in-clinic pulmonary tests, validating remote monitoring as a means to promote more equitable and accessible clinical trial designs.