AI Summary Bylvay (odevixibat) and Livmarli (maralixibat) are prescription IBAT inhibitors that lower bile acid levels and help relieve severe itching in progressive familial intrahepatic cholestasis (PFIC).
Medically reviewed.
Progressive familial intrahepatic cholestasis (PFIC) is a group of rare inherited liver disorders that start in childhood. Treatment focuses on lowering bile acid levels and easing the severe itching (pruritus) that PFIC can cause. Two prescription medicines in a class called ileal bile acid transporter (IBAT) inhibitors are used: Bylvay (odevixibat) and Livmarli (maralixibat).
This page explains how these therapies work, who may be prescribed them, what lifestyle steps support treatment, and how your care team monitors you. You can also connect with a liver specialist to discuss whether one fits your care plan.
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PFIC THERAPY OVERVIEW
Progressive familial intrahepatic cholestasis (PFIC) is a group of rare inherited liver disorders that start in childhood. Treatment aims to lower bile acid levels in the body and relieve severe itching (pruritus). Care usually combines medicine with good nutrition, vitamin support, and regular follow-up with a liver specialist.
Two prescription medicines are used for PFIC: Bylvay (odevixibat) and Livmarli (maralixibat). Both are IBAT inhibitors. Because the right approach depends on your diagnosis and health history, a liver specialist can help you understand which option fits your care plan.
Bylvay is an ileal bile acid transporter (IBAT) inhibitor used to treat PFIC. It reduces bile acid reabsorption in the intestine, which lowers bile acid levels and helps relieve severe itching. It comes as oral capsules or oral pellets, taken once daily in the morning.
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Livmarli is also an IBAT inhibitor. It treats cholestatic pruritus (severe itching from cholestatic liver disease) in eligible people with PFIC or Alagille syndrome. It comes as an oral solution, taken once daily about 30 minutes before the first meal of the day.
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Providers may consider these medicines for people with PFIC who have severe itching or need treatment to reduce bile acid buildup. For Bylvay, this may include people whose symptoms conservative treatments have not adequately controlled. Only a qualified healthcare provider can decide if one of them is right for you.
NUTRITION & LIFESTYLE SUPPORT
PFIC medicine works best as part of a broader care plan. That plan includes attention to nutrition, vitamins, itch comfort, and regular specialist follow-up.
Keep up adequate nutrition and take or supplement fat-soluble vitamins as your care team directs.
Skin care measures may help ease itching. Avoid overheating, which can worsen it. Supportive sleep and hydration strategies may also help.
Avoid substances that can harm the liver, including alcohol in older patients, and keep regular follow-up with a liver specialist.
Ask your care team which nutrition products fit your plan. A nutrition bar designed for liver health can be one simple way to support daily eating habits.
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PFIC TYPES AND TREATMENT
PFIC includes several inherited types that start in childhood. Three major categories exist, each linked to a different gene. Your specialist can explain which type applies to you.
Bylvay and Livmarli both act in the intestine rather than in the liver. The entries below summarize the types and the shared way these medicines work.
Type 1 involves low yGT and mutations in the ATP8B1 gene. It can also cause features outside the liver, such as diarrhea and pancreatitis.
Type 2 also involves low yGT. It is linked to ABCB11 mutations (BSEP deficiency) and carries a high risk of developing hepatocellular carcinoma (HCC), a type of liver cancer.
Type 3 involves markedly elevated yGT and mutations in the ABCB4 gene (MDR3 deficiency).
Bylvay and Livmarli block the ileal bile acid transporter (IBAT) in the small intestine. This reduces how much bile acid the body reabsorbs, increases bile acid removal in the stool, and lowers bile acid levels in the blood. That helps relieve pruritus.
TREATMENT MONITORING
Both Bylvay and Livmarli are long-term therapies. Regular monitoring shows how well treatment works and helps catch problems early.
Blood tests track liver enzymes and other markers. Elevated liver enzymes are a reported side effect of both medicines.
Vitamins A, D, E, and K are checked regularly, because fat-soluble vitamin deficiency is a reported side effect.
Because PFIC starts in childhood, your care team tracks growth in pediatric patients during treatment.
Your provider reviews whether itching and bile acid levels are improving. Treatment usually continues as long as it helps and is well tolerated.
Only a qualified healthcare provider can adjust your treatment plan based on your test results and health history.
COMMON QUESTIONS
Progressive Familial Intrahepatic Cholestasis (PFIC) is a group of rare inherited liver disorders that start in childhood. In PFIC, bile acids build up in the body, which is called cholestasis, and this often causes severe itching (pruritus). A liver specialist can confirm which type applies and explain what it means for care. Talk with your healthcare provider about your own situation.
There are three major types of Progressive Familial Intrahepatic Cholestasis (PFIC), each linked to a different gene. Type 1 involves ATP8B1 and can cause diarrhea and pancreatitis. Type 2 involves ABCB11 (BSEP deficiency) and carries a high risk of hepatocellular carcinoma (HCC), a liver cancer. Type 3 involves ABCB4 (MDR3 deficiency). A liver specialist can explain which type applies to you.
Two prescription medicines called ileal bile acid transporter (IBAT) inhibitors are used for Progressive Familial Intrahepatic Cholestasis (PFIC): Bylvay (odevixibat) and Livmarli (maralixibat). Both lower bile acid levels and help relieve severe itching. Livmarli is also used for cholestatic pruritus in eligible people with Alagille syndrome. Only a healthcare provider can decide whether one fits your care plan.
IBAT inhibitors block a bile acid transporter in the small intestine, so the body reabsorbs less bile acid. More bile acid leaves in the stool, and bile acid levels in the blood fall. This helps relieve the severe itching (pruritus) linked to Progressive Familial Intrahepatic Cholestasis (PFIC). Results vary from person to person, so ask your healthcare provider what to expect.
Common side effects of PFIC medicines include diarrhea, abdominal pain, vomiting, fat-soluble vitamin deficiency, and elevated liver enzymes. Your care team checks liver tests, vitamin levels, and growth in children during treatment to catch problems early. Your provider can help manage side effects. Tell your healthcare provider about any new or worsening symptoms, and do not stop a medicine on your own.
Your care team monitors Progressive Familial Intrahepatic Cholestasis (PFIC) treatment with regular checks. These include liver function tests, fat-soluble vitamin levels, growth in children, and how you respond to treatment. Treatment is generally continued as long as it helps and is well tolerated. Ask your healthcare provider how often you need follow-up visits and which tests apply to you.
Key steps include adequate nutrition, fat-soluble vitamins as directed, skin care, and avoiding overheating, which can worsen itching. Avoid substances that harm the liver, such as alcohol in older patients, and keep regular follow-up with a liver specialist. These steps work alongside medicine for Progressive Familial Intrahepatic Cholestasis (PFIC). Ask your care team which steps fit your plan.
Medical disclaimer: LiverDirectory provides educational information and a directory of providers. It does not provide medical advice, diagnosis, or treatment. Always consult a licensed physician regarding any medical condition.
