Chee Y. Ooi

We have read with interest and express our agreement with the recently published “Addressing pain in people living with cystic fibrosis: Cystic fibrosis foundation evidence-informed guidelines” [2]. Dellon and colleagues have published a timely Cystic Fibrosis Foundation paper outlining recommendations and evidence-informed guidelines on addressing pain in people with cystic fibrosis (pwCF) [2]. These guidelines, derived from co-design methodologies, provide in-depth recommendations on managing pain in CF and intend to guide various stakeholders or healthcare practitioners at all stages of their patients’ development.

Paediatric home enteral nutrition (HEN) is becoming more common. Existing research and guidelines point to the importance of carer education. However, ongoing HEN care in the community is not well covered, and many of those caring for children with feeding tubes have insufficient training, contributing to safety incidents in paediatric HEN. This article presents a novel gold standard for home nasogastric tube feeding for infants and children. It is developed through interviews with clinicians (n = 13) and parents (n = 4), and revised in consultation with a wider group of clinicians (n = 22), parents (n = 4), researchers (n = 2) and other professionals involved in supporting families with children who tube feed (n = 2). The gold standard reflects the authors' consensus recommendations. Alignment of its features with peer-reviewed research is highlighted. The gold standard considers NG tube feeding at home as a mealtime (rather than medical procedure) that is safe, responsive to the child's cues, and family friendly. Where weaning is expected, it also prepares the child for this.

Cystic fibrosis (CF) patients experience higher risks of colorectal cancer but the pathogenesis is unclear. In the general population, polyketide synthase-positive (pks+) E. coli is implicated in intestinal carcinogenesis via the production of colibactin; however, the relevance in CF is unknown. In this study, we investigate pks+E. coli prevalence in CF and potential associations between pks+E. coli, gastrointestinal inflammation, and microbiome dynamics with fecal calprotectin and 16SrRNA gene taxonomic data. Cross-sectional analysis demonstrated no difference in pks+E. coli carriage between CF patients and healthy controls, 21/55 (38%) vs. 26/55 (47%), p = 0.32. Pks+E. coli was not associated with significant differences in mean (SD) calprotectin concentration (124 (154) vs. 158 (268) mg/kg; p = 0.60), microbial richness (159 (76.5) vs. 147 (70.4); p = 0.50) or Shannon diversity index (2.78 (0.77) vs. 2.65 (0.74); p = 0.50) in CF. Additionally, there was no association with exocrine pancreatic status (p = 0.2) or overall antibiotic use (p = 0.6). Longitudinally, CF subjects demonstrated intra-individual variation in pks+E. coli presence but no significant difference in overall prevalence. Future investigation into the effects of repeat exposure on risk profile and analysis of older CF cohorts is necessary to identify if associations with colorectal cancer exist.

Background: A previous Australia-wide pilot study identified pain as a significant burden in people with CF (pwCF). However, the prevalence, frequency and severity have not been evaluated using validated tools. Methods: Australian adults, pwCF and healthy controls (HC) were invited to complete an online questionnaire from July 2023 - February 2024, consisting of four validated tools: Brief Pain Inventory, Pain Catastrophising Scale, PAGI-SYM and PAC-SYM. The questionnaire, disseminated via Cystic Fibrosis Australia, CF Together and online social media groups, explored experiences surrounding pain and its management using closed and free text entries. Results: There were 206 respondents, consisting of 117 CF patients and 89 HC. Over 70 % (n = 69) of pwCF reported pain compared to 28 % (n = 21) of HC (p = <0.001). Further, significantly higher pain frequency per month was reported for pwCF than HC (40 % vs. 10 %; p < 0.001). Symptom clustering was also observed where at least three other locations of pain were reported, and pain was reported to trigger other physiological and psychological symptoms. Notably, there was no significant difference in the locations, occurrence, frequency or severity of pain between those on a CFTR modulator or not (p = 0.625). PwCF also reported significantly lower relief from over-the-counter therapies (p = 0.002) and expressed themes of unmet symptom and management needs. Conclusions: This study identified a high prevalence of pain affecting multiple body parts in pwCF compared to HC and suggests that pain is sub-optimally managed, impairing their quality of life. Increased awareness and early recognition within the CF clinics and the development of clinical pathways are critically needed to better manage and monitor pain in pwCF, leading to improved quality of life and health outcomes.

Highly effective modulator therapy (HEMT), particularly the triple combination elexacaftor-tezacaftor-ivacaftor (ETI), significantly improved clinical outcomes and quality of life in people with Cystic Fibrosis (pwCF). This review analyzes current knowledge on the impact of HEMTs on gastrointestinal (GI) symptoms and features in pwCF. A descriptive review of English literature until February 29, 2024, was conducted using medical databases. Observational studies and clinical trials addressing GI reflux disease (GERD), lower GI symptoms and pancreatic disease were considered. Studies report positive effects of HEMTs on pH levels and bicarbonate secretion as well as improvement on intestinal inflammation. HEMTs also demonstrated positive effects on GERD and lower GI symptoms or conditions CF related such as dysbiosis. Taking ETI during pregnancy could also allow resolution of meconium ileus in fetuses with CF. The best benefits were observed in pancreatic function, potentially delaying CF-related diabetes and recovering pancreatic function in some children on ETI. Larger trials, particularly in pediatric populations, need to confirm these findings and explore long-term effects.