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A grumbling concern: A survey of gastrointestinal symptoms in cystic fibrosis in the modulator era.

作者信息

Calthorpe Rebecca J, Goodchild Natalie, Gleetus Vigilius, Premakumar Vinishaa, Hayee Bu, Elliott Zoe, Evans Bethinn, Rowbotham Nicola J, Carr Siobhán B, Barr Helen, Horsley Alexander, Peckham Daniel, Smyth Alan R

机构信息

University of Nottingham School of Medicine & NIHR Nottingham Biomedical Research Centre, Nottingham, UK.

Patient and Public representative, N/A, UK.

出版信息

NIHR Open Res. 2024 Feb 5;3:18. doi: 10.3310/nihropenres.13384.1. eCollection 2023.


DOI:10.3310/nihropenres.13384.1
PMID:37881465
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10593346/
Abstract

BACKGROUND: Gastrointestinal symptoms in cystic fibrosis (CF) are common and intrusive to daily life. Relieving gastrointestinal symptoms was identified as an important research priority and previously explored in an international survey in 2018. However, following the widespread introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modulators in 2019, the landscape of CF treatment has changed. We repeated an online survey to further describe gastrointestinal symptoms and their effect on quality of life (QoL) in the CFTR modulator era. METHODS: An electronic survey consisting of closed questions and free text responses was distributed via social media and professional networks for a period of one month between March - April 2022. People with CF (pwCF), their family and friends, and healthcare professionals (HCPs) were invited to take part. RESULTS: There were 164 respondents: 88 pwCF (54%), 22 (13%) family, and 54 (33%) healthcare professionals (HCPs). A total of 89/110 (81%) pwCF or family members reported CFTR modulator treatment. The most commonly reported symptoms were wind / gas and rumbling stomach noises (borborygmi) in both the modulator and non-modulator groups in addition to loose motions (modulator group) and bloating (no modulator group). Abdominal pain and bloating had the greatest impact on QoL.For those on a CFTR modulator, the proportion of pwCF reporting "no change" or "worse" for all of the symptoms surveyed was greater than the proportion reporting an improvement. For some symptoms such as stomach pains and reduced appetite, improvements were perceived more commonly in HCPs than what was reported by pwCF. Following modulator introduction, dietary changes to manage GI symptoms were recommended by 28/35 (80%) of HCPs and reported by 38/76 (50%) lay respondents. Changes in medication were recommended by 19/35 (54%) HCPs and reported by 44/76 (58%) of patients and family members. CONCLUSION: This survey has shown that gastrointestinal symptoms remain prevalent in pwCF in the CFTR modulator era, though the nature of these symptoms may have changed. A better understanding of the underlying pathophysiology of these symptoms is essential. Future clinical studies should focus on improving symptoms and QoL.

摘要

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引用本文的文献

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本文引用的文献

[1]
A refresh of the top 10 research priorities in cystic fibrosis.

Thorax. 2023-8

[2]
Multicenter prospective study showing a high gastrointestinal symptom burden in cystic fibrosis.

J Cyst Fibros. 2023-3

[3]
Elexacaftor/tezacaftor/ivacaftor and gastrointestinal outcomes in cystic fibrosis: Report of promise-GI.

J Cyst Fibros. 2023-3

[4]
Elexacaftor-Tezacaftor-Ivacaftor Treatment Reduces Abdominal Symptoms in Cystic Fibrosis-Early results Obtained With the CF-Specific CFAbd-Score.

Front Pharmacol. 2022-6-3

[5]
The impact of FDA and EMA regulatory decision-making process on the access to CFTR modulators for the treatment of cystic fibrosis.

Orphanet J Rare Dis. 2022-5-7

[6]
Prevalence and Impact of Rheumatologic Pain in Cystic Fibrosis Adult Patients.

Front Med (Lausanne). 2022-2-8

[7]
Combined use of rheology and portable low-field NMR in cystic fibrosis patients.

Respir Med. 2021

[8]
Telehealth after the pandemic: Will the inverse care law apply? (Commentary).

J Cyst Fibros. 2021-12

[9]
Triple Therapy for Cystic Fibrosis -Gating and -Residual Function Genotypes.

N Engl J Med. 2021-8-26

[10]
A Phase 3 Open-Label Study of Elexacaftor/Tezacaftor/Ivacaftor in Children 6 through 11 Years of Age with Cystic Fibrosis and at Least One Allele.

Am J Respir Crit Care Med. 2021-6-15

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