Multicenter prospective study showing a high gastrointestinal symptom burden in cystic fibrosis

GALAXY Study Group

Research output: Contribution to journalArticlepeer-review

14 Scopus citations


BACKGROUND AND AIMS: People with cystic fibrosis (PwCF) suffer from gastrointestinal (GI) symptoms affecting their quality of life (QOL). Despite the relevance of GI symptoms to the overall health of PwCF, a paucity of studies only have comprehensively assessed the prevalence, severity and QOL of GI symptoms in both children and adults with Cystic Fibrosis (CF).

METHODS: Eligible participants ≥2 years of age across 26 US CF centers were followed for 4 weeks. Three validated GI electronic patient-reported outcome measures (ePROMs) with a recall period of 2 weeks and a stool-specific questionnaire were administered weekly over four weeks. Total and domain scores of ePROMs were evaluated overall and in subgroups using linear mixed-effect models.

RESULTS: Of 402 enrolled, 58% were ≥ 18 years of age (52% male). The mean (SD) of the total score for PAC-SYM was 0.52 (0.55), for PAGI-SYM was 0.63 (0.67), and for PAC-QOL was 0.67 (0.55). For specific ePROM questions, prevalence of moderate to very severe symptoms were as follows: straining (20.3%), fullness (18.3%), incomplete bowel movements (17.1%), bloating (16.4%), distension (16.4%), abdominal pain (upper-5.1%, lower-7.5%). Comparing participants ≥18 versus <18, a higher prevalence of bloating (63.7% versus 27.3%), lower abdominal pain (39.8% vs 26.2%), stomach fullness (75.6% versus 56.2%), and abdominal distension (60.2% versus 34.9%) was found. Both age groups reported high treatment dissatisfaction as measured with PAC-QOL, mean 1.39 (95% CI: 1.30, 1.47).

CONCLUSION: GI symptoms were reported in all age ranges irrespective of gender, with higher prevalence observed amongst older and female subgroups. Dissatisfaction with GI targeted treatments were reported in a large proportion of participants despite therapy, highlighting an unmet need for clinical interventions.


Original languageEnglish (US)
Pages (from-to)266-274
Number of pages9
JournalJournal of Cystic Fibrosis
Issue number2
StatePublished - Mar 2023

Bibliographical note

Funding Information:
SJS COI: Grant funding NIH, CFF, and Gilead. Advisory work for Abbvie, Nestle, and UpToDate

Funding Information:
This study was done with grant funding from the NIH , Cystic Fibrosis Foundation (CFF) grant # MOSHIR19K01

Publisher Copyright:
© 2022 European Cystic Fibrosis Society


  • Constipation
  • Cystic fibrosis
  • Gastrointestinal questionnaires
  • Gastrointestinal symptoms
  • Patient-reported outcome measures

PubMed: MeSH publication types

  • Multicenter Study
  • Journal Article
  • Research Support, Non-U.S. Gov't
  • Research Support, N.I.H., Extramural


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