Odevixibat (Bylvay) is an ileal bile acid transporter (IBAT) inhibitor. It is indicated for the treatment of progressive familial intrahepatic cholestasis (PFIC)-related pruritus in patients aged 3 months and older, as well as cholestatic pruritus associated with Alagille syndrome (ALGS) in patients aged 12 months and older.

Precautions for Odevixibat (Bylvay) Administration

Contraindication Screening

Absolute Contraindications: It is contraindicated in patients with a history of or current decompensated liver function events (such as variceal bleeding, ascites, and hepatic encephalopathy).

Special Populations:

Patients with moderate to severe renal impairment require dosage adjustment.

Pregnant women need risk assessment (animal studies indicate a potential risk of cardiac malformations).

Dosage Forms and Administration Methods

Granules: Must be mixed with soft food or liquid; swallowing the intact shell directly is prohibited.

Capsules:Can be swallowed whole or opened, with the contents sprinkled on soft food.

Concomitant use with bile acid sequestrants should be avoided (a 4-hour interval is required).

Prevention and Control of Adverse Reactions

Hepatotoxicity: Frequent monitoring of alanine transaminase (ALT), aspartate transaminase (AST), bilirubin, and international normalized ratio (INR) is necessary within the first 6-8 months of treatment. If abnormalities occur, dosage reduction or treatment discontinuation is required.

Diarrhea: The incidence ranges from 21% to 39%. Dehydration should be monitored, and treatment interruption is necessary in severe cases.

Fat-Soluble Vitamin Deficiency: The levels of vitamins A, D, E, and K should be monitored at baseline and during treatment; supplementation is required if necessary.

Drug Interactions

Bile Acid Sequestrants (e.g., cholestyramine): Administration must be separated by a 4-hour interval; otherwise, the efficacy of odevixibat may be reduced.

Potent CYP3A4 Inhibitors (e.g., ketoconazole): May increase the plasma concentration of odevixibat, so caution is required when used in combination.

Management of Special Populations

Pediatric Patients:

For PFIC: Patients must be ≥ 3 months of age.

For ALGS: Patients must be ≥ 12 months of age.

Hepatic Impairment:

Patients with cirrhosis or portal hypertension require enhanced monitoring.

It is contraindicated in patients with decompensated hepatic function.

Monitoring for Odevixibat (Bylvay) Administration

Laboratory Tests

At baseline and every 3 months: Liver function, fat-soluble vitamin levels, and coagulation function (INR).

During diarrhea: Electrolytes and dehydration indicators.

Clinical Manifestations: Monthly assessment of impulsive behaviors (e.g., pathological gambling), bleeding tendency, and growth and development.

Management of Emergency Events

Liver Injury: If new-onset liver function abnormalities occur, treatment should be discontinued immediately; consideration of restarting at a lower dose may be made after the condition stabilizes.

Bleeding: Interrupt treatment and optimize vitamin K supplementation; assessment for restarting treatment should be conducted after the condition stabilizes.