Case 2
An 18-year-old female with CF (F508del/W1282X) who required a liver transplant in April 2015 for CFLD. Her post-transplant course was complicated by recurrent ascites and alloimmune hepatitis, requiring an inferior vena cava stent placement and hepatic vein dilation in December 2016 and revision in April 2017. Prior to therapy initiation, the patient intermittently had lower extremity edema. She was started on elx/tez/iva at the end of December 2019, 4.7 years post-transplant, at a reduced dose of one tablet of elx/tez/iva in the morning. After 3.6 weeks of therapy, the dose was titrated up to two tablets in the morning. Laboratory monitoring, including LFTs, bilirubin and tacrolimus levels, was conducted one day after therapy initiation, weekly for the first month and every two weeks thereafter. LFTs and bilirubin fluctuated, but peaked at 4 times the upper limit of normal (ULN) based on the laboratory reference range after one month at the increased dose. Concurrently, the patient had worsening lower extremity edema and new onset ascites prompting evaluation by her transplant center. The patient underwent a ballooning of her existing stent and had two additional stents placed. Elx/tez/iva was felt to not be a contributing factor in the patient’s transaminitis as LFT values decreased following her procedure. The tacrolimus concentration increased to 22.3 ng/mL at one week after elx/tez/iva initiation prompting a dose reduction in tacrolimus. Following her elx/tez/iva dose increase, tacrolimus levels were between 3.6 to 7.3 ng/mL (goal: 3 to 8 ng/mL). The patient is tolerating elx/tez/iva without any reported adverse events. After eight months of therapy, the patient reported improved quality of life with minimal respiratory symptoms at baseline. The patient has not required any oral or systemic antibiotic therapy for a pulmonary exacerbation since initiating elx/tez/iva. Additionally, pulmonary function testing showed an improvement in ppFEV1 from 61% to 83%.