Kormelink, Lauren N (LN);Jacobs, HollyAnn R (HR);

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Pediatr Pulmonol.2024 Dec 02;60(1):e27431.doi:10.1002/ppul.27431

Abstract

INTRODUCTION: Cystic Fibrosis (CF) is a multisystem autosomal recessive disease characterized by thick, sticky mucus which causes lung disease, pancreatic insufficiency, and many other manifestations. Constipation is a common complication in CF and few advances in treatment have been made until recent years. Linaclotide is a treatment approved for chronic idiopathic constipation and irritable bowel syndrome with constipation. This case series investigates the potential for use of this agent in adult people with CF (pwCF).

METHODS: A retrospective case series of 39 pwCF who are currently or previously prescribed linaclotide was completed at two adult Cystic Fibrosis Foundation (CFF) Accredited Care Centers. All patients 18 years and older were included with no exclusions. We reported results using descriptive statistics.

RESULTS: A total of 39 patients were identified for inclusion. The daily starting dose of linaclotide did vary with 18%, 72%, and 10% started on 72 mcg, 145 mcg, and 290 mcg respectively. Most patients (n = 30, 77%) reported experiencing no side effects. Of the total patients, 15% (n = 6) reported the medicine was not effective and 10% (n = 4) had to stop therapy due to adverse reactions. More than half of patients started on therapy were able to reduce baseline treatments for constipation.

CONCLUSION: While this case series shows compelling data that may indicate benefit and tolerability of linaclotide use in pw CF, more research is needed to fully evaluate safety and efficacy of linaclotide for treating constipation in pwCF.