Bylvay Generic Name & Formulations
Mechanism of Action
Odevixibat decreases the reabsorption of bile acids (primarily the salt forms) from the terminal ileum. The complete mechanism by which odevixibat improves pruritus in PFIC and ALGS patients is unknown, but it may involve inhibition of the IBAT, which results in decreased reuptake of bile salts, as observed by a decrease in serum bile acids.
Treatment of pruritus in progressive familial intrahepatic cholestasis (PFIC). Treatment of cholestatic pruritus in Alagille syndrome (ALGS).
Limitations of Use
Bylvay Dosage and Administration
Adults and Children
PFIC (<3months) or ALGS (<12months): not established. See full labeling. Take once daily in the AM with food. PFIS: ≥3months (≤7.4kg): 200mcg; (7.5–12.4kg): 400mcg; (12.5–17.4kg): 600mcg; (17.5–25.4kg): 800mcg; (25.5–35.4kg): 1200mcg; (35.5–45.4kg): 1600mcg; (45.5–55.4kg): 2000mcg; (≥55.5kg): 2400mcg. If no response after 3months, may increase in 40mcg/kg increments up to 120mcg/kg once daily; max 6mg/day. ALGS: ≥12months (≤7.4kg): 600mcg; (7.5–12.4kg): 1200mcg; (12.5–17.4kg): 1800mcg; (17.5–25.4kg): 2400mcg; (25.5–35.4kg): 3600mcg; (35.5–45.4kg): 4800mcg; (45.5–55.4kg): 6000mcg; (≥55.5kg): 7200mcg. Patients weighing <19.5kg: use oral pellets; mix contents of the shell containing oral pellet(s) into soft food (eg, applesauce, oatmeal, banana or carrot puree, chocolate or rice pudding) or age-appropriate liquid (eg, breast milk, infant formula, or water); do not swallow the shells whole. Patients weighing ≥19.5kg: use capsules; swallow whole or mix contents of capsule with soft food or age-appropriate liquid. For ALGS: may consider reducing dose to 40mcg/kg/day if tolerability issues occur in the absence of other causes.
Bylvay Boxed Warnings
Obtain baseline liver tests (eg, ALT, AST, total/direct bilirubin, INR) and monitor during therapy; reduce or interrupt dose if abnormalities occur. Consider discontinuing if persistent or recurrent liver test abnormalities occur. PFIC or ALGS with cirrhosis: monitor closely for liver test abnormalities; permanently discontinue if progresses to portal hypertension or a hepatic decompensation event (eg, variceal hemorrhage, ascites, hepatic encephalopathy). Monitor for dehydration if diarrhea occurs. Interrupt therapy if persistent diarrhea; restart and adjust dose appropriately when resolves. Discontinue if diarrhea persists and no alternate etiology is identified. Obtain serum fat-soluble vitamin (FSV) levels at baseline and monitor during therapy. Discontinue if FSV deficiency persists or worsens despite supplementation. PFIC with clinically significant portal hypertension or with decompensated cirrhosis: not established. Elderly. Pregnancy. Nursing mothers.
After a single administration of odevixibat 7.2 mg, Cmax is reached between 1 to 5 hours.
The effect of food and sprinkling on soft food on systemic exposure of odevixibat is not clinically significant.
>99% plasma protein binding.
Fecal (82.9%). Half-life: 2.36 hours.
Administer bile acid binding resins (eg, cholestyramine, colesevelam, colestipol) at least 4hrs before or 4hrs after odevixibat.
Bylvay Adverse Reactions
For PFIC: liver test abnormalities, diarrhea, abdominal pain, vomiting, fat-soluble vitamin deficiency. For ALGS: diarrhea, abdominal pain, hematoma, decreased weight.
Bylvay Clinical Trials
Bylvay Patient Counseling