Case 1
A 14-year-old male with CF (F508del/F508del) who required a liver
transplant in February 2019 for CFLD. He has not had any issues with
rejection or other post-transplant complications. There was one
hospitalization for transaminitis, and the etiology was presumed to be a
viral infection with resolution by a week without any interventions. He
was started on elx/tez/iva at the end of November 2019, nine months
post-transplant. One tablet of elx/tez/iva was initiated in the morning
and increased to two tablets in the morning after three weeks. LFTs and
bilirubin levels were scheduled bimonthly along with bimonthly
tacrolimus troughs. LFTs and bilirubin were mildly elevated after
initiation and stabilized after two months of therapy. The tacrolimus
concentration slightly fluctuated after elx/tez/iva initiation from 4.2
to 6 ng/mL (goal: 4-7 ng/mL). No other adverse events were reported.
Before elx/tez/iva, gastrostomy tube feedings were required and after
one month of therapy, his body mass index (BMI) increased from the
12th percentile to the 48thpercentile. His baseline FEV1 percent predicted
(ppFEV1) was 90% in early 2019, trended downward to
58% before elx/tez/iva initiation, and increased to 82%. The patient
reports overall improvement in quality of life and can take on more
independent tasks.