Digestive symptoms in CF fall into 4 distinct patterns, study finds
Patterns were associated with different complications and medication use
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A new study identified four distinct patterns of digestive symptoms in adults with cystic fibrosis (CF), ranging from mild to severe and widespread symptoms.
These patterns of symptoms showed “associations with [gastrointestinal] complications and medication requirements, suggesting distinct underlying [mechanisms] and a varying degree of involvement of complex gastrointestinal abnormalities,” the researchers wrote.
Further analysis will assess the mechanisms behind these patterns and may help researchers develop more personalized approaches to treatment, according to the study authors.
The study, “Distinct gastrointestinal symptom phenotypes in adults with cystic fibrosis are linked to complications and medication use: the GRAMPUS-CF study,” was published in the Journal of Cystic Fibrosis.
Researchers identify 4 patterns of digestive symptoms in CF
CF is caused by genetic mutations that affect how the CFTR protein works. This protein helps control the movement of salt and water in and out of cells. When it doesn’t work properly, thick, sticky mucus builds up in organs, including the lungs and digestive system.
Although CFTR modulators have improved lung health in people with CF, many still experience digestive symptoms, such as bloating, abdominal pain, constipation, and diarrhea. The underlying causes of these digestive problems remain poorly understood.
To learn more, researchers from three specialist CF centers in the U.K. conducted the GRAMPUS-CF study to investigate whether adults with CF could be grouped based on their digestive symptoms. They also looked for links between these symptom patterns, health complications and medication use.
The study included 300 adults with CF, with a median age of 31 years. Most had at least one copy of the F508del mutation (92.3%). Most also had exocrine pancreatic insufficiency (EPI, 88.3%), a condition in which the pancreas cannot release enough enzymes to digest food. In addition, 84% were prescribed pancreatic enzyme replacement therapy.
Most participants (84.3%) were prescribed CFTR modulators. The most commonly prescribed was Kaftrio (elexacaftor, tezacaftor, and ivacaftor), sold as Trikafta in the U.S., which was prescribed to 50.7% of participants.
Participants completed standardized, scientifically tested questionnaires about digestive symptoms. All reported at least one symptom. The most common were bloating (73%), gas (65%), and abdominal pain (55%).
The researchers then used a statistical method called latent class analysis to examine 36 symptom indicators and group people with similar symptoms. They identified four patterns: mild (36%), moderate constipation-predominant (33%), moderate diarrhea-predominant (19%), and severe (12%). There were no statistically significant differences among the groups in sex, ethnicity, CFTR gene mutations, or EPI status.
Severe digestive symptoms linked to reflux and bowel obstruction
Researchers also assessed diet quality and the potential of foods to promote inflammation in 221 participants. Neither measure differed significantly among the four groups. However, the severe-symptom group had the highest score on the dietary inflammatory index, while the diarrhea-predominant group had the lowest.
Compared with the mild-symptom group, the severe-symptom pattern was significantly associated with gastroesophageal reflux disease, in which stomach contents flow back into the esophagus, and distal intestinal obstruction syndrome, a condition in which thick stool can block the intestines. The severe-symptom pattern was also associated with a greater likelihood of proton pump inhibitor and laxative use. Proton pump inhibitors reduce stomach acid, while laxatives help treat or prevent constipation.
Both moderate symptom patterns were associated with CF-related liver disease and lower use of long-term oral antibiotics, also relative to the mild-symptom group.
Overall, the study found that digestive symptoms in adults with CF vary widely but follow distinct patterns linked to different clinical features, “suggesting different underlying mechanisms,” the researchers noted. This approach “provides a foundation for precision-based [gastrointestinal] symptom management strategies,” they added.
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