Livmarli
Livmarli (maralixibat) is FDA approved to help with itching caused by liver problems (cholestatic pruritis) from certain genetic conditions. It's for adults and children with Alagille syndrome (ALGS). This medication is also for people 1 year and older with progressive familial intrahepatic cholestasis (PFIC). It works as an ileal bile acid transporter (IBAT) inhibitor. Livmarli (maralixibat) comes as a tablet and a liquid. You take each dose by mouth on an empty stomach. Common side effects include diarrhea and stomach pain.
What is Livmarli (maralixibat)?
What is Livmarli (maralixibat) used for?
- Itching caused by liver problems (cholestatic pruritis) from:
- Alagille syndrome (ALGS) in people 3 months of age and older
- Progressive familial intrahepatic cholestasis (PFIC) in people 1 year of age and older
How Livmarli (maralixibat) works
Livmarli (maralixibat) is an ileal bile acid transporter (IBAT) inhibitor. It blocks IBAT, a protein that normally helps keep bile acid in the body.
People with Alagille syndrome and progressive familial intrahepatic cholestasis have too much bile acid. Bile acids help the body digest and absorb fat. But when there's too much bile acid, it can damage the liver and it's thought to lead to symptoms like itchiness.
By blocking the IBAT protein, Livmarli (maralixibat) works to lower the amount of bile acids in the body. Researchers think this helps relieve itchiness.
Drug facts
Side effects of Livmarli (maralixibat)
The following side effects may get better over time as your body gets used to the medication. Let your healthcare provider know immediately if you continue to experience these symptoms or if they worsen over time.
Common Side Effects
ALGS:
- Diarrhea (56%)
- Stomach pain (54%)
- Vomiting (41%)
- Low vitamin A, D, E, and K levels (26%)
- Higher liver enzyme levels (19%)
PFIC:
- Diarrhea (57%)
- Stomach pain (28%)
- Higher liver enzyme levels (17%)
Less Common Side Effects
- Nausea
- Bone fracture
Livmarli (maralixibat) serious side effects
Contact your healthcare provider immediately if you experience any of the following.
- Severe liver damage: swollen stomach, yellowing of the skin or whites of the eyes, black- or clay-colored stool, nausea, vomiting blood, confusion
- Blood in stool
- Dehydration: dry mouth, very thirsty, lightheadedness, dizziness, not urinating as much
The following Livmarli (maralixibat) side effects have also been reported
Side effects that you should report to your care team as soon as possible:
- Allergic reactions—skin rash, itching, hives, swelling of the face, lips, tongue, or throat
- Bleeding—bloody or black, tar-like stools, vomiting blood or brown material that looks like coffee grounds, red or dark brown urine, small red or purple spots on skin, unusual bruising or bleeding
- Dehydration—increased thirst, dry mouth, feeling faint or lightheaded, headache, dark yellow or brown urine
- Liver injury—right upper belly pain, loss of appetite, nausea, light-colored stool, dark yellow or brown urine, yellowing skin or eyes, unusual weakness or fatigue
Side effects that usually do not require medical attention (report these to your care team if they continue or are bothersome):
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Pros and cons of Livmarli (maralixibat)
Pros
- Works well to help improve itchiness from ALGS and PFIC
- Approved for both adults and children with ALGS or PFIC
- Available as a tablet and a grape-flavored liquid
Cons
- Need to take 30 minutes before a meal
- Might take several weeks to start working
- Can cause stomach upset and poor absorption of certain vitamins (vitamins A, D, E, and K)
Pharmacist tips for Livmarli (maralixibat)
- Take Livmarli (maralixibat) 30 minutes before a meal.
- Swallow Livmarli (maralixibat) tablets whole. If you have trouble swallowing them whole, talk to your prescriber. They can switch you to the liquid form of the medication.
- If you take Livmarli (maralixibat) once a day and you miss a dose: Take the medication as soon as you remember. But if your next dose is less than 12 hours away, skip the missed dose. Just wait to take the medication at your usual dose at your next scheduled time. After that, you can continue with your normal schedule.
- If you take Livmarli (maralixibat) twice a day and you miss a dose: Take the missed dose as soon as possible if it's within 6 hours of the time you usually take it. But if your next dose is less than 6 hours away, skip the missed dose. Then, take the medication at your usual dose at the next regularly scheduled time. Afterwards, follow your usual dosing schedule.
- Livmarli (maralixibat) can often cause diarrhea. If this happens, drink plenty of fluids to stay hydrated. Call your care team if you have diarrhea that won't go away. Also call them if you have bloody diarrhea, diarrhea with vomiting, diarrhea with a fever. Get medical help right away if you're severely dehydrated.
- Take Livmarli (maralixibat) at least 4 hours before or 4 hours after any bile acid sequestrants. Examples include cholestyramine (Prevalite) and colesevelam (Welchol). These medications interfere with Livmarli (maralixibat). So spacing them out helps the body absorb Livmarli (maralixibat) better. If you need help timing your medications, ask your pharmacist.
Tips for the Livmarli (maralixibat) liquid:
- There are two different strengths of the liquid. Each strength is for people with different genetic conditions. The 9.5 mg/mL liquid is for ALGS, and the 19 mg/mL liquid is for PFIC. If you're not sure which one you need, ask your prescriber
- Follow the instructions for taking the Livmarli (maralixibat) liquid. If you have questions about measuring it or taking it the right way, ask your care team.
- To measure the Livmarli (maralixibat) liquid, use the dosing dispensers (oral syringes) that come in the package. Make sure you use the right size of the dosing dispenser for the prescribed dose. Don't use household spoons because they aren't accurate. Ask your care team if you're not sure which dosing dispenser to use and how much medication to draw up.
- To take Livmarli (maralixibat), place the tip of the dosing dispenser against the inside of the cheek. Slowly push the plunger all the way down to give the full dose. Then, swallow the medication.
- You can reuse each dosing dispenser for up to 100 days (or a little more than 3 months). Make sure to follow the directions to rinse it with water and let it air dry after each time you use it.
- Store the Livmarli (maralixibat) liquid at room temperature. Once opened, the liquid inside the bottle is good for 100 days (a little more than 3 months). After 100 days or after it expires, throw away the medication, even if there's still some left.
Common questions about Livmarli (maralixibat)
Yes, Livmarli (maralixibat) does come as a tablet. The tablets are for people with ALGS or PFIC who weigh at least 55 pounds and can swallow pills. For people who have trouble swallowing the tablets, there's a liquid version of the medication for them. Talk to your prescriber about which form of the medication is right for you.
Studies showed that children with ALGS who took Livmarli (maralixibat) overall had large improvements in how itchy they felt. In other studies, many had less itchiness after a year or more of treatment compared to when they started the medication. Be sure to follow up with your prescriber about how well Livmarli (maralixibat) is working for you.
In studies, people with PFIC who took Livmarli (maralixibat) had a lot less itchiness than those who took a placebo. In general, Livmarli (maralixibat) started to work within 6 weeks. But it took about 3 months for people to get the full benefits of the medication. Check in with your prescriber about how well Livmarli (maralixibat) is working for you.
Diarrhea and vomiting are common side effects of Livmarli (maralixibat). There are a few things you can do to help manage these side effects if they happen. The most important thing is to make sure to stay well hydrated. This helps prevent dehydration, which can be serious if it's severe. Also call your care team if you have these side effects. They might recommend other medications to relieve the specific stomach issue you have. Your prescriber can also consider lowering your Livmarli (maralixibat) dose or stopping treatment until these side effects go away.
It's probably a good idea to avoid alcohol while taking Livmarli (maralixibat). This can help prevent further damage to the liver. ALGS and PFIC are genetic conditions that can affect the liver. And, this medication has a risk of harming the liver. What's more, drinking large amounts of alcohol can also damage the liver. Talk to your prescriber about your drinking habits and ask them if you have questions.
It's not clear if Livmarli (maralixibat) is safe to take during pregnancy or while breastfeeding. Usually, only a small amount of the medication gets absorbed. But it could still have a risk of lowering vitamin levels. To be safe, talk to your prescriber about the risks and benefits of taking Livmarli (maralixibat) during pregnancy. If you're breastfeeding, also check with your baby's pediatrician or primary care provider.
Bylvay (odevixibat) and Livmarli (maralixibat) are both used to treat itching from ALGS and PFIC. But the approved ages are different. For ALGS, Livmarli is for people ages 3 months and older and Bylvay is for people ages 1 year and older. It's the opposite for PFIC: Livmarli is used for people ages 1 year and older and Bylvay is for people ages 3 months and older. The medications also come in different forms for people who can't swallow pills. There's a grape-flavored liquid for Livmarli. And for Bylvay, there are oral pellets that you can open and then mix the contents inside with soft food or liquid. Talk to your prescriber to help you compare the medications.
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Risks and warnings for Livmarli (maralixibat)
Livmarli (maralixibat) can cause some serious health issues. This risk may be even higher for certain groups. If this worries you, talk to your doctor or pharmacist about other options.
Liver damage
Livmarli (maralixibat) has the potential to harm the liver. It can raise your liver enzyme levels, which can be a sign of liver damage.
Before starting Livmarli (maralixibat), you'll need to do a blood test. This helps your prescriber see what your liver health is like before treatment. Then, you'll need regular blood tests during treatment to keep checking your liver.
Get medical help right away if you have symptoms of severe liver damage. Look out for severe nausea, bloody vomit, swelling of your stomach, yellow skin or eyes, and confusion.
Stomach upset
Livmarli (maralixibat) often causes diarrhea, stomach pain, and vomiting. If you have diarrhea or vomit, make sure to drink plenty of fluids. These side effects can make you lose too much water from your body and lead to dehydration.
Reach out to your care team if you have diarrhea or stomach pain that won't go away. Also call them if you have bloody diarrhea, diarrhea with vomiting, diarrhea with a fever. And get medical help right away if you have symptoms of severe dehydration. Watch for things like thirst, exhaustion, dizziness, dark urine, and not urinating as much.
The medical team can help manage or treat these side effects. Your prescriber might lower your dose of Livmarli (maralixibat) to help. They could also decide to stop the medication and then restart it at a lower dose once you feel better.
Low vitamin A, D, E, and K
Livmarli (maralixibat) can make your body have a hard time absorbing certain vitamins (A, D, E, and K). Low vitamin levels can sometimes make you more likely to have bleeds or bone fractures.
Before starting Livmarli (maralixibat), your prescriber will check your vitamin levels with a blood test. They'll also check every so often while you're taking the medication. If your levels are low, your prescriber will give you supplements to help bring them back up.
Call your care team if you have any unusual bleeding or if you break a bone while you're taking Livmarli (maralixibat). They can check if it's from low vitamin levels.
Risk of propylene glycol toxicity in young children
- Risk factors: Children younger than 5 years old
The Livmarli (maralixibat) oral solution contains propylene glycol. It's a common additive in some medications.
Although the propylene glycol additive is generally safe, children younger than 5 are at higher risk of toxicity from it. For young children, it's not clear if it's safe to take medications with this additive for a long time. Propylene glycol toxicity can cause serious side effects. This can include seizures or kidney damage.
Your child's prescriber will likely order labs during treatment. This helps them monitor your child's safety with Livmarli (maralixibat). If you're worried about your child's safety, talk to their prescriber.
Livmarli (maralixibat) dosage
Bottle of oral solution| Dosage | Quantity | Price as low as | Price per unit |
|---|---|---|---|
| 30ml of 19mg/ml | 1 bottle | $111,946.33 | $111,946.33 |
Typical dosage for Livmarli (maralixibat)
Your prescriber will find the dose for you based on your body weight.
-
Alagille syndrome (ALGS) — tablets or 9.5 mg/mL oral solution
- Take Livmarli (maralixibat) 30 minutes before the first meal in the morning.
- The typical starting dose is 190 mcg/kg of weight by mouth once daily in the morning. After 1 week, your prescriber might raise the dose to 380 mcg/kg by mouth once daily in the morning.
-
Progressive familial intrahepatic cholestasis (PFIC) — tablets or 19 mg/mL oral solution
- The typical starting dose is 285 mcg/kg of weight by mouth once daily, 30 minutes before the first meal of the day. Then, your prescriber might raise your dose slowly. The recommended maintenance dose is 570 mcg/kg by mouth twice daily, 30 minutes before a meal.
How much does Livmarli (maralixibat) cost?
$111,946.33
Livmarli (maralixibat) contraindications
Contraindications are specific health conditions or situations in which a person should not take a medication due to safety concerns. If you have any of the following conditions or if any of the following apply to you, let your healthcare provider know because Livmarli (maralixibat) will not be safe for you to take.
- Currently have or have had complications from liver damage (also called hepatic decompensation)
What are alternatives to Livmarli (maralixibat)?
Livmarli (maralixibat) images
