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Drug-induced dyspnea versus cystic fibrosis exacerbation: a diagnostic dilemma

 

Authors Walayat S, Hussain N, Patel J, Hussain F, Patel P, Dhillon S, Aulakh B, Chittivelu S

Received 6 April 2017

Accepted for publication 28 June 2017

Published 19 July 2017 Volume 2017:10 Pages 243—246

DOI https://doi.org/10.2147/IMCRJ.S139022

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Lucy Goodman

Peer reviewer comments 3

Editor who approved publication: Dr Ronald Prineas

Abstract: Cystic fibrosis (CF) is a disease caused by a mutation in the cystic fibrosis transmembrane conductance regulator protein in the epithelial membrane, and affects at least 30,000 people in the USA. There are between 900 and 1000 new cases diagnosed every year. Traditionally, CF has been treated symptomatically with pancreatic enzymes, bronchodilators, hypertonic saline, and pulmozyme. In July 2015, the US Food and Drug Administration approved Orkambi (lumacaftor/ivacaftor), a combination drug that works on reversing the effects of the defective cystic fibrosis transmembrane conductance regulator protein. Orkambi and mucolytics decrease the viscosity of mucous secretions, leading to an accumulation of hypoviscous fluid in the alveoli, resulting in dyspnea. This presentation can be mistaken for an infective exacerbation. We present a case in which a young female with CF recently started on Orkambi therapy presented to her primary care physician with dyspnea and increased respiratory secretions and was admitted to the hospital for 2 weeks of intravenous and inhaled antibiotic therapy for a presumed CF exacerbation. We highlight this case to bring awareness and educate patients and clinicians of the side-effect profile of Orkambi therapy with an intent to avoid unnecessary hospitalizations, inpatient antibiotics, and other costly medical services.
Keywords: cystic fibrosis, lumacaftor/ivacaftor, dyspnea, CFTR protein, pulmozyme, hypertonic saline, pancrelipase


摘要视频链接Effects of Orkambi on CF