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%.