Version May 2024
Amyotrophic lateral sclerosis: Initial: Oral: One packet (sodium phenylbutyrate 3 g/taurursodiol 1 g) once daily for 3 weeks, then increase dose to 1 packet twice daily, if tolerated (Ref).
Dosage adjustment for concomitant therapy: Significant drug interactions exist, requiring dose/frequency adjustment or avoidance. Consult drug interactions database for more information.
Mild impairment: No dosage adjustment necessary.
Moderate to severe impairment: Avoid use (has not been studied).
Mild impairment: No dosage adjustment necessary.
Moderate to severe impairment: Avoid use (has not been studied).
Refer to adult dosing.
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified. Reported adverse reactions are for adults.
>10%:
Gastrointestinal: Abdominal pain (21%), diarrhea (25%), nausea (18%), sialorrhea (11%)
Nervous system: Fatigue (12%)
Respiratory: Upper respiratory tract infection (18%)
1% to 10%: Nervous system: Dizziness (10%)
There are no contraindications listed in the manufacturer's US labeling.
Canadian labeling: Hypersensitivity to sodium phenylbutyrate, taurursodiol, bile salts, or any component of the formulation; pregnancy; breastfeeding.
Disease-related concerns:
• Biliary disorders: Enterohepatic circulation disorders (eg, biliary infection, active cholecystitis) may increase risk for worsening diarrhea.
• Pancreatic or intestinal disease: Pancreatic insufficiency (eg, pancreatitis), intestinal malabsorption, or intestinal disorders (eg, ileal resection, regional ileitis) may alter the concentration of bile acids and lead to decreased absorption of sodium phenylbutyrate and taurursodiol.
• Renal impairment: Use with caution in patients with renal impairment due to risk of sodium retention and edema.
Dosage form specific issues:
• Sodium: Use caution in patients sensitive to salt intake (eg, congestive heart failure, hypertension, renal impairment); product contains 928 mg of sodium/2 packets.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Packet, Oral:
Relyvrio: Sodium phenylbutyrate 3 g and taurursodiol 1 g (7 ea, 56 ea)
No
Pack (Relyvrio Oral)
3-1 g (per each): $276.53
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Packet, Oral:
Albrioza: Sodium phenylbutyrate 3 g and taurursodiol 1 g (7 ea, 56 ea)
Oral: Vigorously stir the contents of 1 packet in a cup (250 mL or 8 oz) of room temperature water. Take before a snack or meal. Administer orally or via feeding tube within 1 hour of preparation; discard unused reconstituted suspension after 1 hour.
Amyotrophic lateral sclerosis: Treatment of adults with amyotrophic lateral sclerosis.
Refer to individual components.
Note: Interacting drugs may not be individually listed below if they are part of a group interaction (eg, individual drugs within “CYP3A4 Inducers [Strong]” are NOT listed). For a complete list of drug interactions by individual drug name and detailed management recommendations, use the Lexicomp drug interactions program by clicking on the “Launch drug interactions program” link above.
Almagate: May decrease the absorption of Taurursodiol. Risk X: Avoid combination
Aluminum Hydroxide: May decrease the absorption of Taurursodiol. Risk X: Avoid combination
BCRP/ABCG2 Substrates (Clinically Relevant with Inhibitors): Taurursodiol may increase the serum concentration of BCRP/ABCG2 Substrates (Clinically Relevant with Inhibitors). Risk X: Avoid combination
Benperidol: May diminish the therapeutic effect of Urea Cycle Disorder Agents. Risk C: Monitor therapy
Bile Acid Sequestrants: May decrease the absorption of Taurursodiol. Risk X: Avoid combination
BSEP/ABCB11 Inhibitors: May enhance the adverse/toxic effect of Taurursodiol. Specifically, the risk for liver dysfunction may be increased. Management: Avoid coadministration of sodium phenylbutyrate and taurursodiol with BSEP inhibitors when possible. If concomitant use is necessary, monitoring of serum transaminases and bilirubin is recommended. Risk D: Consider therapy modification
Corticosteroids (Systemic): May diminish the therapeutic effect of Urea Cycle Disorder Agents. More specifically, Corticosteroids (Systemic) may increase protein catabolism and plasma ammonia concentrations, thereby increasing the doses of Urea Cycle Disorder Agents needed to maintain these concentrations in the target range. Risk C: Monitor therapy
CYP1A2 Substrates (Narrow Therapeutic Index/Sensitive with Inducers): Taurursodiol may decrease the serum concentration of CYP1A2 Substrates (Narrow Therapeutic Index/Sensitive with Inducers). Risk X: Avoid combination
CYP2B6 Substrates (Narrow Therapeutic Index/Sensitive with Inhibitors): Taurursodiol may increase the serum concentration of CYP2B6 Substrates (Narrow Therapeutic Index/Sensitive with Inhibitors). Risk X: Avoid combination
CYP2C8 Substrates (Narrow Therapeutic Index/Sensitive with Inhibitors): Taurursodiol may increase the serum concentration of CYP2C8 Substrates (Narrow Therapeutic Index/Sensitive with Inhibitors). Risk X: Avoid combination
CYP3A4 Inducers (Strong): May decrease the serum concentration of Taurursodiol. Specifically, the concentrations of phenylbutyrate may be decreased. Risk C: Monitor therapy
CYP3A4 Substrates (Narrow Therapeutic Index/Sensitive with Inducers): Taurursodiol may decrease the serum concentration of CYP3A4 Substrates (Narrow Therapeutic Index/Sensitive with Inducers). Risk X: Avoid combination
Haloperidol: May diminish the therapeutic effect of Urea Cycle Disorder Agents. More specifically, Haloperidol may increase plasma ammonia concentrations and thereby increase the doses of Urea Cycle Disorder Agents needed to maintain concentrations in the target range. Risk C: Monitor therapy
Histone Deacetylase Inhibitors: May enhance the adverse/toxic effect of Taurursodiol. Risk X: Avoid combination
OAT1/3 Substrates (Clinically Relevant): Taurursodiol may increase the serum concentration of OAT1/3 Substrates (Clinically Relevant). Risk X: Avoid combination
OATP1B1/1B3 (SLCO1B1/1B3) Inhibitors: May increase the serum concentration of Taurursodiol. Risk X: Avoid combination
P-glycoprotein/ABCB1 Substrates (Narrow Therapeutic Index/Sensitive with Inhibitors): Taurursodiol may increase the serum concentration of P-glycoprotein/ABCB1 Substrates (Narrow Therapeutic Index/Sensitive with Inhibitors). Risk X: Avoid combination
Probenecid: May increase serum concentrations of the active metabolite(s) of Urea Cycle Disorder Agents. Specifically, concentrations of phenylacetate and phenylacetylglutamine may be increased. Risk C: Monitor therapy
Taurursodiol: Histone Deacetylase Inhibitors may enhance the adverse/toxic effect of Taurursodiol. Risk X: Avoid combination
Adverse events were observed in some animal reproduction studies.
It is not known if sodium phenylbutyrate, taurursodiol, or their metabolites are present in breast milk.
According to the manufacturer, the decision to breastfeed during therapy should consider the risk of infant exposure, the benefits of breastfeeding to the infant, and the benefits of treatment to the mother.
Administer before a snack or meal. Product contains dextrates, sodium, sorbitol, and sucralose.
The mechanism by which the decline of physical function is decreased in patients with amyotrophic lateral sclerosis is unknown. The combination of sodium phenylbutyrate and taurursodiol reduces neuronal cell death in vitro.
Absorption: Sodium phenylbutyrate: Rapid; administration with a high-fat meal results in Cmax decreased by 76% and AUC decreased by 54%.
Protein binding: Sodium phenylbutyrate: 82%; taurursodiol: 98%.
Metabolism: Hepatic (saturable); sodium phenylbutyrate is metabolized by β-oxidation in the liver and kidney to phenylacetate, which is rapidly conjugated with glutamine via acetylation to form phenylacetylglutamine; taurursodiol undergoes enterohepatic recirculation and is deconjugated to ursodeoxycholic acid (UDCA) by intestinal microflora, which is reconjugated in the liver with glycine or taurine (glycoursodeoxycholic acid [GUDCA] and taurursodiol, respectively).
Half-life elimination: Phenylbutyrate: 0.46 hours; phenylacetate: 0.81 hours; taurursodiol: 4.34 hours.
Time to peak: Median: Phenylbutyrate: 0.5 hours; taurursodiol: 4.5 hours.
Excretion: Urine (~80% to 100% as phenylacetylglutamine [metabolite of sodium phenylbutyrate]).
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