Odevixibat (Bylvay) is an ileal bile acid transporter (IBAT) inhibitor used for the treatment of pruritus associated with progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome (ALGS). Its efficacy and safety require individualized adjustment based on the patient’s age, body weight, and disease type.

How to Use Odevixibat (Bylvay)

1. Indications and Administration Timing

(1) For PFIC patients: Indicated for the treatment of pruritus in patients aged 3 months and older; administer with breakfast in the morning.(2) For ALGS patients: Indicated for the treatment of cholestatic pruritus in patients aged 12 months and older; also administer with breakfast in the morning.

2. Dosage and Dosage Form Selection

(1) Initial dosage for PFIC: 40 mcg/kg once daily. If pruritus does not improve after 3 months, the dosage may be increased by 40 mcg/kg increments up to a maximum of 120 mcg/kg (not exceeding 6 mg per day).

(2) Fixed dosage for ALGS: 120 mcg/kg once daily.

Dosage Forms

(1) Oral granules: Suitable for patients with a body weight < 19.5 kg (available in 200 mcg / 600 mcg strengths).

(2) Capsules: Suitable for patients with a body weight ≥ 19.5 kg (available in 400 mcg / 1200 mcg strengths).

3. Administration Precautions

(1) Swallow whole: Capsules must not be chewed or crushed.

(2) Administration of oral granules: Mix with soft food (e.g., applesauce) or liquid, and administer within 5 minutes.

(3) Drug interactions: An interval of 4 hours is required between administration of odevixibat and bile acid sequestrants (e.g., cholestyramine).

Dosage Adjustment of Odevixibat (Bylvay)

1. Management of Adverse Reactions

(1) Diarrhea: If persistent diarrhea occurs, discontinue administration temporarily. After symptoms resolve, restart at 40 mcg/kg and gradually increase the dosage.

(2) Hepatotoxicity: If abnormal liver enzymes or symptoms of hepatitis occur, suspend administration. After recovery, consider dosage reduction or permanent discontinuation.

2. Adjustment for Hepatic Impairment

(1) Baseline monitoring: Before treatment, test indicators such as ALT, AST, and bilirubin.

(2) Patients with cirrhosis: Contraindicated or require close monitoring; permanent discontinuation is necessary if decompensating events (e.g., ascites) occur.

3. Adjustment for Renal Impairment

(1) Mild to moderate impairment: No dosage adjustment is required.

(2) Severe impairment or dialysis: Safety data is insufficient; use with caution.

Odevixibat (Bylvay) Use in Special Populations

1. Pregnancy and Lactation

(1) Pregnancy: Animal studies have shown potential fetal cardiac malformations; human data is limited, and risks must be weighed against benefits.

(2) Lactation: Drug absorption is low, but it may affect the absorption of fat-soluble vitamins; monitoring and supplementation are required.

2. Patients with Hepatic Impairment

(1) Compensated cirrhosis: Enhanced monitoring is required.

(2) Decompensated cirrhosis: Contraindicated.