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.
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.
Ileal bile acid transporter (IBAT) inhibitor.
Administer bile acid binding resins (eg, cholestyramine, colesevelam, colestipol) at least 4hrs before or 4hrs after odevixibat.
Liver test abnormalities, diarrhea, abdominal pain, vomiting, fat-soluble vitamin deficiency.
Generic Drug Availability: