Miscellaneous gastrohepatic disorders:

Indications for: BYLVAY

Treatment of pruritus in progressive familial intrahepatic cholestasis (PFIC).

Limitations of Use:

May not be effective in PFIC type 2 patients with ABCB11 variants resulting in non-functional or complete absence of bile salt export pump protein (BSEP-3).

Adults and Children:

<3months: not established. See full labeling. Take once daily in the AM with food. ≥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. 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.

BYLVAY Warnings/Precautions:

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 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.

BYLVAY Classification:

Ileal bile acid transporter (IBAT) inhibitor.

BYLVAY Interactions:

Administer bile acid binding resins (eg, cholestyramine, colesevelam, colestipol) at least 4hrs before or 4hrs after odevixibat.

Adverse Reactions:

Liver test abnormalities, diarrhea, abdominal pain, vomiting, fat-soluble vitamin deficiency.

Generic Drug Availability:


How Supplied:

Caps, pellets—30