To treat or not to treat: CFTR modulators after lung transplantation

Pediatr Transplant. 2021 Jun;25(4):e14007. doi: 10.1111/petr.14007. Epub 2021 Mar 20.

Abstract

Evidence for the extrapulmonary benefits of (CFTR) modulators is rapidly expanding. The use of CFTR modulators in CF patients who have undergone lung transplantation is not clear without guidance published in the medical literature to assist clinicians in the care of these patients. We discuss the potential benefits of CFTR modulators and provide insight into their use based on our experience in a small cohort of CF LTx recipients. We present pros and cons of CFTR modulator therapy for LTx recipients with CF. CFTR modulators should be considered in CF patients after lung transplantation for the time being until further research defines how to best use these therapies in transplant recipients.

Keywords: CF; CFTR; LTx; modulator.

MeSH terms

  • Adolescent
  • Aminophenols / therapeutic use*
  • Benzodioxoles / therapeutic use*
  • Biomarkers / metabolism
  • Combined Modality Therapy
  • Cystic Fibrosis / drug therapy*
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis / metabolism
  • Cystic Fibrosis / surgery*
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Cystic Fibrosis Transmembrane Conductance Regulator / metabolism*
  • Drug Combinations
  • Humans
  • Indoles / therapeutic use*
  • Lung Transplantation*
  • Postoperative Care / methods*
  • Pyrazoles / therapeutic use*
  • Pyridines / therapeutic use*
  • Quinolines / therapeutic use*
  • Quinolones / therapeutic use*
  • Treatment Outcome
  • Young Adult

Substances

  • Aminophenols
  • Benzodioxoles
  • Biomarkers
  • Drug Combinations
  • Indoles
  • Pyrazoles
  • Pyridines
  • Quinolines
  • Quinolones
  • elexacaftor, ivacaftor, tezacaftor drug combination
  • tezacaftor, ivacaftor drug combination
  • Cystic Fibrosis Transmembrane Conductance Regulator