- •Moderate to severe gastrointestinal (GI) symptoms are highly prevalent in people with cystic fibrosis (PwCF).
- •In particular, bloating, early satiety, fullness and abdominal pain are highly prevalent in people with PwCF and are more common in females ≥ 18.
- •The CF-specific definition of constipation is different than in other populations and constipation in CF patients is defined by characteristic abdominal symptoms in addition to stool and rectal symptoms.
- •Treatment dissatisfaction scores and GI symptoms remain high despite use of PERT in >88% and other GI medications in >98% of participants.
- •Sex differences in symptom severity follow trends for many functional bowel diseases.
Background and aims
Abbreviations:BM (Bowel Movement), BSS (Bristol Stool Form Scale), CF (Cystic Fibrosis), CFTR (Cystic fibrosis transmembrane regulator), CFF (Cystic Fibrosis Foundation), CF-TDN (Cystic Fibrosis Therapeutics Development Network), ePROMs (Electronic Patient Reported Outcome Measures), GI (Gastrointestinal), GERD (Gastroesophageal reflux disease), PERT (Longitudinal Multicenter Study to Determine the Patient-reported Prevalence of GI Symptoms in Pancreatic Enzyme Replacement Therapy), GALAXY (Persons with Cystic Fibrosis), PAC-QOL (Patient Assessment of Constipation-Quality of Life), PAC-SYM (Patient Assessment of Constipation- Symptom Severity Index), PAGI-SYM (Patient Assessment of Upper Gastrointestinal Disorders-Symptom Severity Index), PROMs (Patient Reported Outcomes, Patient Reported Outcome Measures), PROMIS (Patient-reported Outcomes Measurement Information System), PedsQLGI (Pediatric Quality of Life Inventory, Gastrointestinal Symptoms Scales and Module), pwCF (People with Cystic Fibrosis), SIBO (Small Intestinal Bacterial Overgrowth Syndrome), SSQ (Stool Specific Questionnaire), QOL (Quality of Life)
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