Real-life acute lung function changes after lumacaftor/ivacaftor first administration in pediatric patients with cystic fibrosis

J Cyst Fibros. 2017 Nov;16(6):709-712. doi: 10.1016/j.jcf.2017.05.002. Epub 2017 May 18.

Abstract

The combination of lumacaftor and ivacaftor (LUM/IVA) has been reported to induce a mean acute absolute drop of -4.1% predicted forced expiratory volume in 1s (FEV1) after a unique administration in healthy subjects. The aim of the present study was to assess acute FEV1 changes after the first dose of LUM/IVA in CF patients. A total of 32 pediatric patients were included. Respiratory manifestations occurred in only 3 patients (9.4%), but FEV1 consistently decreased (-10.4±4.6%, range: -1.5; -21.8%). FEV1 only partially resumed after salbutamol inhalation. Patients with previously known significant reversible airway obstruction and low FEV1 were more at risk of FEV1 decrease.

Keywords: Acute; Airway obstruction; Bronchoconstriction; Cystic fibrosis; Lumacaftor; Modulators; Pediatric.

MeSH terms

  • Adolescent
  • Aminophenols* / administration & dosage
  • Aminophenols* / adverse effects
  • Aminopyridines* / administration & dosage
  • Aminopyridines* / adverse effects
  • Benzodioxoles* / administration & dosage
  • Benzodioxoles* / adverse effects
  • Chloride Channel Agonists / administration & dosage
  • Chloride Channel Agonists / adverse effects
  • Cystic Fibrosis* / diagnosis
  • Cystic Fibrosis* / drug therapy
  • Cystic Fibrosis* / physiopathology
  • Drug Combinations
  • Drug Monitoring / methods
  • Female
  • Forced Expiratory Volume / drug effects*
  • France
  • Humans
  • Male
  • Quinolones* / administration & dosage
  • Quinolones* / adverse effects
  • Respiratory Function Tests / methods
  • Symptom Assessment / methods*
  • Treatment Outcome

Substances

  • Aminophenols
  • Aminopyridines
  • Benzodioxoles
  • Chloride Channel Agonists
  • Drug Combinations
  • Quinolones
  • lumacaftor, ivacaftor drug combination