Case 9
A 22-year old female with a history of CF (F508del/Q493X), short gut
syndrome due to bowel resection as an infant, status post liver
transplantation in 1998 (liver failure was likely secondary to TPN), and
autism spectrum disorder. Her immunosuppression regimen is tacrolimus
monotherapy. She was started on voriconazole late September 2019 for
allergic bronchopulmonary aspergillosis (ABPA), but therapy was
discontinued due to LFT elevations and plan to initiate elx/tez/iva.
Because of baseline LFT elevations and history of liver transplant, she
was initiated on elx/tez/iva at a reduced dose of 2 tablets daily in
November 2019. A week later, the CF team was notified by patient’s
mother that the patient had an unexpected seizure and was evaluated and
treated at an outside hospital’s emergency department (ED). Notably,
the patient has no history of seizures, but does have a significant
neuropsychiatric history of autism and developmental delay. Her workup,
including electroencephalogram (EEG) and computed tomography (CT) of the
head were normal. After this incident, elx/tez/iva was held for 1 week,
and restarted at same dose in early December, with no further issues
reported. After initiation of elx/tez/iva, LFTs were stable. The
patient’s tacrolimus concentration remained stable throughout treatment
with elx/tez/iva without the need for dose changes. The patient’s
baseline ppFEV1 was 67%, and after 3 months of therapy
with elx/tez/iva, remained stable at 68%, however the patient
experienced improvement in respiratory symptoms and improved quality of
life.