Diet and the gut-lung axis in cystic fibrosis – direct & indirect links

Abstract

Cystic fibrosis (CF) is a multisystem, autosomal, recessive disease primarily affecting the lungs, pancreas, gastrointestinal tract, and liver. Whilst there is increasing evidence of a microbial ‘gut-lung axis’ in chronic respiratory conditions, there has been limited analysis of such a concept in CF. We performed a comprehensive dietary and microbiota analysis to explore the interactions between diet, gastrointestinal microbiota, respiratory microbiota, and clinical outcomes in children with CF. Our results demonstrate significant alterations in intestinal inflammation and respiratory and gastrointestinal microbiota when compared to age and gender matched children without CF. We identified correlations between the gastrointestinal and respiratory microbiota, lung function, CF pulmonary exacerbations and anthropometrics, supporting the concept of an altered gut-lung axis in children with CF. We also identified significant differences in dietary quality with CF children consuming greater relative proportions of total, saturated and trans fats, and less relative proportions of carbohydrates, wholegrains, fiber, insoluble fiber, starch, and resistant starch. Our findings position the CF diet as a potential modulator in gastrointestinal inflammation and the proposed gut-lung axial relationship in CF. The dietary intake of wholegrains, fiber and resistant starch may be protective against intestinal inflammation and should be explored as potential therapeutic adjuvants for children with CF.

Nutritional management of people living with cystic fibrosis throughout life and disease continuum: Changing times, new challenges

“…Correlations were also established between the intake of specific components such as fibres and fatty acids and the intestinal microbiota composition in pwCF. 157 Although the findings of the study should be interpreted with caution given its small sample and crosssectional design; they provide evidence highlighting the importance of the quality of the CF diet for intestinal health.…”

“…A recent study investigating the dietary intake of young pwCF showed that their relative intake of resistant starch, whole grains and dietary fibres (total, insoluble and soluble) was lower than that of healthy controls. 157 Participants with CF also reported a greater proportion of energy intake derived from non-core foods (e.g., sweetened drinks, packaged snacks) than non-CF participants. Such higher intakes were correlated with faecal calprotectin levels (a marker of intestinal inflammation).…”

“…Dietary components with prebiotic properties, including resistant starch and dietary fibres, are known to influence the composition of the intestinal microbiota, which in turn may promote or alleviate intestinal inflammation. A recent study investigating the dietary intake of young pwCF showed that their relative intake of resistant starch, whole grains and dietary fibres (total, insoluble and soluble) was lower than that of healthy controls 157 . Participants with CF also reported a greater proportion of energy intake derived from non‐core foods (e.g., sweetened drinks, packaged snacks) than non‐CF participants.…”

Impact of extended Elexacaftor/Tezacaftor/Ivacaftor therapy on the gut microbiome in cystic fibrosis

“…This encompasses pulmonary antibiotic usage, which was a key explanator across our multivariate analyses and of high prevalence across pwCF undertaking extended ETI. Though not investigated in the current study, additional research into the ability of specific dietary intake and probiotic usage to shape the microbiota and patient outcomes is desirable [62–64], especially since resilience of the gut microbiota has been recently suggested following diet and exercise intervention in pwCF [65]. It could be that long-term substantial changes to such factors are necessary for subsequent beneficial changes to the intestinal microbiome in an era of modulator therapy.…”

Evolution of nutritional management in children with cystic fibrosis – a narrative review

“…There is increasing evidence suggesting an altered gut flora in pwCF with more frequent dysbiosis 125 . Many suffer from GI symptoms, 126 and diet is understandably in focus with its links to short‐chain fatty acids (SCFA), inflammation and modulator role in the GI tract 127 …”