An Open-label, Single Center Study Assessing Tenapanor as a Non-CFTR-mediated Treatment of CF-related Constipation in People With CF.
Tenapanor is the newest FDA-approved drug for IBS with constipation (IBS-C). This study seeks to understand tenapanor as a treatment for cystic fibrosis-related constipation (CFrC) in CF patients. Participants will ingest one 50 mg tablet of tenapanor, twice daily, for a 4-week treatment period. They will also complete three questionnaires, the PAC-SYM, PAC-QoL, and IBS-SSS, and daily diaries to characterize GI symptom burden and spontaneous bowel movement (SBM) frequency.
• Having confirmed cystic fibrosis (either by sweat chloride or genetic testing)
• Meeting criteria for CFrC
• Must include 2 of the following, with or without abdominal pain for at least 3 months, with symptom onset at least 6-months prior:
⁃ Straining in at least 25% of defecations
⁃ Bristol Stool Scale 1-2 more than 25% of defecations (change in stool form)
⁃ Sensation of incomplete evacuation more than 25% of defecations
⁃ Sensation of anorectal obstruction/blockage more than 25% of defecations
⁃ Manual disimpaction/manipulation of pelvic floor to facilitate more than 25% of defecations
⁃ Fewer than 3 spontaneous bowel movements weekly (change in stool frequency)
⁃ Loose stools rarely present without the use of laxatives
• Willingness to avoid major dietary or lifestyle changes during study.