Background: Sodium phenylbutyrate and ursodoxicoltaurine/taurursodiol (PB&TURSO) is indicated for the treatment of amyotrophic lateral sclerosis (ALS) in Canada and the U.S. In the CENTAUR trial, a phase 2 U.S. multicenter trial in ALS, PB&TURSO was generally well-tolerated. The most common adverse reactions were gastrointestinal (GI) and occurred most often during the first three weeks of therapy. Although the occurrence of GI events in people living with ALS (PLWALS) and treated with PB&TURSO are recognisable and generally manageable, many of the symptoms are often not managed proactively. We sought to develop an evidence-based tool to help guide clinicians on managing diarrhea and abdominal pain in PLWALS and treated with PB&TURSO. Methods: Three ALS specialized neurologists and one gastroenterologist combined their clinical experience, research, and each Medications respective product monograph, to develop a patient-centric GI tool. Results: A guide to monitoring and managing potential GI events with PB&TURSO was developed. The tool provides clinicians a proactive, step-by-step guide to help manage diarrhea and abdominal pain in PLWALS treated with PB&TURSO. Conclusions: The GI tool has the potential to improve monitoring, recognition, and treatment of GI events in PLWALS treated with PB&TURSO. Proactively managing GI events may aid medication adherence and improve patient quality of life.