Miscellaneous gastrohepatic disorders:
Indications for: LIVMARLI
Treatment of cholestatic pruritus in Alagille syndrome (ALGS).
Adults and Children:
<1yr: not established. Use a calibrated measuring device. Take at 30mins before the 1st meal of the day. ≥1yr: initially 190mcg/kg once daily; after 1 week, increase to 380mcg/kg once daily, as tolerated. Max daily dose (patients >70kg): 3mL or 28.5mg/day. For individual dose volume by weight, dose modification for adverse events: see full labeling.
Obtain baseline liver tests (eg, ALT, AST, total/direct bilirubin, INR) and monitor during therapy; consider dose reduction or interruption if abnormalities occur in the absence of other causes. Consider discontinuing if persistent or recurrent liver test abnormalities occur. ALGS with cirrhosis: monitor for liver elevations, liver-related adverse reactions; permanently discontinue if progresses to portal hypertension or a hepatic decompensation event (eg, variceal hemorrhage, ascites, hepatic encephalopathy). Consider dose reduction or interruption if GI reactions occur (diarrhea, abdominal pain, vomiting) and no alternate etiology is identified. Monitor for dehydration if diarrhea or vomiting occur. Consider dose interruption if persistent diarrhea or diarrhea accompanied with signs/symptoms occur; restart and adjust dose appropriately when resolves. Obtain serum fat-soluble vitamin (FSV) levels at baseline and monitor during therapy; consider discontinuing if FSV deficiency persists or worsens despite supplementation. Elderly (≥65yrs), ALGS with clinically significant portal hypertension or with decompensated cirrhosis: not established. 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. Concomitant OATP2B1 substrates (eg, statins); monitor as needed.
Diarrhea, abdominal pain, vomiting, fat-soluble vitamin deficiency, liver test abnormalities, GI bleeding, bone fractures.
Generic Drug Availability: