Case 3
A 31-year-old male with CF (F508del/F508del) status post living donor liver transplant April 2019 for CFLD with esophageal varices, ascites, and cirrhosis was interested in elx/tez/iva therapy. After discussing with his liver transplantation team, he was switched from full dose tez/iva to elx/tez/iva in early November 2019, seven months after his transplantation at full dose. Baseline ppFEV1 was 90% and baseline LFTs and total bilirubin were within normal limits (WNL) at baseline and were ordered for two weeks after initiation. Two days after initiating therapy, the patient presented to the emergency department with complaints of sudden onset abdominal pain with diffuse tenderness to palpation acute tenderness to palpation in the left upper quadrant with positive guarding. On presentation, LFTs and bilirubin were stable WNL. Full workup was performed with no remarkable imaging findings on abdominal imaging. At the time of presentation, he was also diagnosed with a CF pulmonary exacerbation and admitted for intravenous antibiotic therapy. With all other causes reasonably excluded, the patient’s pain was classified as a side effect of elx/tez/iva. Upon discharge five days later to complete his antibiotic course at home, the patient’s abdominal pain had resolved and LFTs and bilirubin remained WNL. Repeat LFTs and bilirubin at one-week post discharge remained WNL with plans to continue to monitor every two weeks. At approximately one month after therapy initiation, ppFEV1 was 95, but AST rose to 6 times the ULN, ALT increased to 2 times the ULN, and total bilirubin remained WNL. Repeat labs were ordered for one week later at which point the patient’s AST and ALT were 5.7 and 5 times the ULN, respectively with total bilirubin WNL. At this time, elx/tez/iva therapy was discontinued and patient underwent testing for hepatic graft rejection. Hepatic graft rejection was ruled out and pathological evidence of drug induced liver disease was identified. His transplant team ordered labs for every two weeks for close monitoring of hepatic markers, which gradually trended back to WNL over several months. In May 2020, the patient was re-challenged with elx/tez/iva at reduced dose of one triple combination pill in the morning and one iva tablet in the evening. Labs were ordered weekly to monitor. At one week post re-initiation, his ALT and AST rose to 3.4 and 5.7 times the upper limit of normal respectively with total bilirubin of 1.4 mg/dL, so therapy was discontinued. Again, liver function tests (LFTs) trended to WNL within about three months. In September 2020, he restarted full dose tez/iva by the transplant clinic. To date, LFTs have remained WNL.