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

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 p...

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Bibliographic Details
Published in:Pediatric transplantation 2021-06, Vol.25 (4), p.e14007-n/a
Main Authors: Hayes, Don, Darland, Leanna K., Hjelm, Michelle A., Mansour, Heidi M., Wikenheiser‐Brokamp, Kathryn A.
Format: Article
Language:English
Subjects:
Adolescent
Aminophenols - therapeutic use
Benzodioxoles - therapeutic use
Biomarkers - metabolism
CFTR
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
LTx
Lung Transplantation
modulator
Postoperative Care - methods
Pyrazoles - therapeutic use
Pyridines - therapeutic use
Quinolines - therapeutic use
Quinolones - therapeutic use
Transplants & implants
Treatment Outcome
Young Adult
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Summary: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.
ISSN:1397-3142
1399-3046
DOI:10.1111/petr.14007