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

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.

CFTR Modulator Therapy and Its Impact on Lung Transplantation in Cystic Fibrosis

“…Hayes et al very recently published a case series of five lung transplant recipients with CF who were started on CFTR modulator therapy [ 94 ]. All patients received induction therapy with basiliximab and standard triple immunosuppression including tacrolimus, mycophenolate, and steroids.…”

“…All patients received induction therapy with basiliximab and standard triple immunosuppression including tacrolimus, mycophenolate, and steroids. Three patients were started on tezacaftor/ivacaftor pre-transplant and continued on dual CFTR modulator treatment after transplantation; the other two patients were placed on elexacaftor/tezacaftor/ivacaftor post transplant [ 94 ]. The authors of the case series describe the extrapulmonary benefits in each individual patient, including predominantly improvement in nutritional status and reduced symptoms of CF-related chronic sinus disease [ 94 ].…”

“…Three patients were started on tezacaftor/ivacaftor pre-transplant and continued on dual CFTR modulator treatment after transplantation; the other two patients were placed on elexacaftor/tezacaftor/ivacaftor post transplant [ 94 ]. The authors of the case series describe the extrapulmonary benefits in each individual patient, including predominantly improvement in nutritional status and reduced symptoms of CF-related chronic sinus disease [ 94 ]. Only one of the five lung transplant recipients demonstrated mildly elevated live enzymes within 1 week of starting triple CFTR modulator therapy (elexacaftor/tezacaftor/ivacaftor) [ 94 ].…”

“…The authors of the case series describe the extrapulmonary benefits in each individual patient, including predominantly improvement in nutritional status and reduced symptoms of CF-related chronic sinus disease [ 94 ]. Only one of the five lung transplant recipients demonstrated mildly elevated live enzymes within 1 week of starting triple CFTR modulator therapy (elexacaftor/tezacaftor/ivacaftor) [ 94 ]. The authors point out the safe usage of CFTR modulators in their small patient cohort and the extrapulmonary benefits seen; however, Hayes et al also highlight disadvantages of the CFTR modulator usage post lung transplantation including drug–drug interactions, the need for drug dosage adjustments, and treatment costs [ 94 ].…”

“…Only one of the five lung transplant recipients demonstrated mildly elevated live enzymes within 1 week of starting triple CFTR modulator therapy (elexacaftor/tezacaftor/ivacaftor) [ 94 ]. The authors point out the safe usage of CFTR modulators in their small patient cohort and the extrapulmonary benefits seen; however, Hayes et al also highlight disadvantages of the CFTR modulator usage post lung transplantation including drug–drug interactions, the need for drug dosage adjustments, and treatment costs [ 94 ]. Ramos et al, in a recently published review article, discuss additional potential benefits of CFTR modulator therapy following lung transplantation [ 95 ].…”