Sodium phenylbutyrate/taurursodiol

What is Sodium phenylbutyrate/taurursodiol?

• Sodium phenylbutyrate/taurursodiol (Relyvrio) is a fixed-dose combination medication used for the treatment of amyotrophic lateral sclerosis (ALS).
• Sodium phenylbutyrate is a histone deacetylase inhibitor.
• Taurursodiol, also known as tauroursdeoxycholic acid, is an ambiphilic bile acid and is the taurine conjugate of ursodiol, also known as ursodeoxycholic acid.

Sodium phenylbutyrate

Tauroursodeoxycholic acid

What are the uses of this medicine?

Sodium phenylbutyrate/taurursodiol (Relyvrio) is a prescription medicine used to treat adults with amyotrophic lateral sclerosis (ALS).

How does this medicine work?

• Sodium phenylbutyrate/taurursodiol is a combination drug being studied for the treatment of amyotrophic lateral sclerosis (ALS), Alzheimer's disease, and Wolfram syndrome. It is a fixed-dose combination of two drugs, sodium phenylbutyrate and taurursodiol (tauroursodeoxycholic acid, TUDCA).
• The mechanism by which Relyvrio exerts its therapeutic effects in patients with ALS is unknown.
• It may reduce the breakdown and death of nerve cells associated with the worsening of ALS symptoms.

Who Should Not Use this medicine ?

• This medicine have no usage limitations.

What drug interactions can this medicine cause?

• Tell your healthcare provider about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements.

Be sure to mention any of the following:

• any taurursodiol products, such as tauroursodeoxycholic acid (TUDCA)
• cholestyramine, colestipol, colesevelam
• bile salt export pump (BSEP) (eg: cyclosporine)

• Avoid use of aluminum-based antacids with Relyvrio and consider other acid lowering agents.
• Avoid use of probenecid with Relyvrio as it may affect renal excretion of sodium phenylbutyrate metabolites.
• Avoid use of other HDAC inhibitors with Relyvrio.
• Avoid use of inhibitors of OATP1B3 with Relyvrio.

Is this medicine FDA approved?

• The combination was approved for medical use in the United States, as Relyvrio, in September 2022.

How should this medicine be used?

Recommended dosage:

• The recommended initial dosage of Relyvrio for oral suspension is 1 packet (3 g sodium phenylbutyrate and 1 g taurursodiol) daily for the first 3 weeks.
• After 3 weeks, increase to the maintenance dosage of 1 packet twice daily.

Administration:

• Your healthcare provider will tell you how much Relyvrio to take and when to take it.
• Empty 1 packet of Relyvrio in a cup (8 ounces) of room temperature water and stir well.
• Relyvrio can be taken by mouth or through a feeding tube within 1 hour of preparing. Throw away any unused Relyvrio after 1 hour.
• Relyvrio should be taken before a snack or meal.
• Avoid taking antacids that contain aluminum while you are receiving treatment with Relyvrio. If you need an antacid, talk to your healthcare provider about the best one for you to take.

What are the dosage forms and brand names of this medicine?

This medicine is available in fallowing doasage form:

• As oral suspension: 3 g sodium phenylbutyrate and 1 g taurursodiol in single-dose packets

This medicine is available in fallowing brand namesː

• Relyvrio

What side effects can this medication cause?

The most common side effects of this medicine include:

• diarrhea
• abdominal pain
• nausea
• upper respiratory tract infection

Less common, but serious side effects may include:

• Changes in bile acid levels
• Salt (sodium) retention

What special precautions should I follow?

• Relyvrio contains taurursodiol, which is a bile acid. In patients with disorders that interfere with bile acid circulation, there may be an increased risk for worsening diarrhea, and patients should be monitored appropriately for this adverse reaction.
• Relyvrio has a high sodium content. In patients sensitive to salt intake, consider the amount of daily sodium intake in each dose of Relyvrio and monitor appropriately.
• Instruct patients or caregivers to empty the contents of one packet in a cup containing 8 ounces of room temperature water and stir vigorously. Advise them that Relyvrio can be taken orally or administered via feeding tube, and to use or discard within 1 hour of preparation. Instruct to administer Relyvrio before a snack or meal.
• Inform patients that 2 packets of Relyvrio contain 928 mg sodium (46% of WHO daily recommended intake) and patients who are sensitive to sodium (e.g., those with congestive heart failure, severe renal insufficiency, or other conditions associated with sodium retention) should limit their sodium intake.
• Inform patients that aluminum-based antacids may interfere with the absorption of Relyvrio, and therefore should not be taken during treatment with Relyvrio.
• It is not known if Relyvrio passes into your breast milk. You and your healthcare provider should decide the best way to feed your baby.

What to do in case of emergency/overdose?

• In case of overdose, call the poison control helpline of your country. In the United States, call 1-800-222-1222.

• Overdose related information is also available online at poisonhelp.org/help.
• In the event that the victim has collapsed, had a seizure, has trouble breathing, or can't be awakened, immediately call emergency services. In the United States, call 911.
• In the event of overdose, general supportive measures should be instituted as necessary.
• Monitor the patient for adverse reactions.

Can this medicine be used in pregnancy?

• There are no available data on Relyvrio use in pregnant women to evaluate for a drug-associated risk of major birth defects, miscarriage, or other adverse maternal or fetal outcomes.
• Advise patients to notify their healthcare provider if they become pregnant or intend to become pregnant during Relyvrio therapy.

Can this medicine be used in children?

• Safety and effectiveness of Relyvrio in pediatric patients have not been established.

What are the active and inactive ingredients in this medicine?

• Active ingredients: sodium phenylbutyrate, taurursodiol
• Inactive ingredients: acacia, dibasic sodium phosphate, maltodextrin, medium-chain triglycerides, mixed berry flavoring, other flavoring ingredients, silicon dioxide, sodium stearyl fumarate, sorbitol, and sucralose.

Who manufactures and distributes this medicine?

Marketed and distributed by: Amylyx Pharmaceuticals, Inc., 43 Thorndike St., Cambridge, MA

What should I know about storage and disposal of this medication?

• Store Relyvrio at room temperature between 68⁰F to 77⁰F (20⁰C to 25⁰C).
• Keep Relyvrio packets away from moisture.

• Dailymed label info on Sodium phenylbutyrate/taurursodiol
• FDA Sodium phenylbutyrate/taurursodiol

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