CurrentAvMedPolicy N/A

Livmarli (maralixibat) prior authorization/step-edit

Defines prior authorization requirements, dosing, and clinical criteria for coverage of Livmarli (maralixibat) for AvMed members with Alagille syndrome (ALGS) or progressive familial intrahepatic cholestasis (PFIC). Applies to prescribers submitting PA requests to AvMed.

Policy Summary

PayerAvMed

PolicyLivmarli (maralixibat) prior authorization/step-edit

Policy CodePolicy N/A

Change TypeNo material change

Effective DateN/A

Next Review DateN/A

Key ActionPrescribing physician must sign and print name on the prior authorization request and include member, prescriber, dosing, diagnosis, weight, and supporting documentation (labs, genetic test results, ItchRO scores, pharmacy claims) to avoid delays or denial.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 monthsInitial authorization duration

12 monthsReauthorization duration

>= 3 moMinimum age (ALGS)

>= 12 moMinimum age (PFIC)

>=100 µmol/LSerum bile acids threshold

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3 mLMax daily volume (9.5 mg/mL)

Coverage Criteria

Initial Therapy — Alagille syndrome

Covered for Alagille syndrome when ALL of the following are met:

ALGS Coverage Criteria: Member is 3 months of age or older; medication is prescribed by or in consultation with a hepatologist, gastroenterologist, cardiologist, or physician who specializes in Alagille syndrome; member has a diagnosis of Alagille syndrome.

Genetic confirmation: Provider has submitted results of genetic testing confirming a JAG1 or NOTCH2 deletion or mutation (submit results).

Clinical confirmation: Provider has submitted clinical confirmation including bile duct paucity on liver biopsy AND three or more major ALGS criteria (liver/cholestasis, dysmorphic facies, heart disease, axial skeleton/vertebral anomalies, eye/posterior embryotoxin).

Evidence of cholestasis confirmed by TWO of the following: total serum bile acid > 3 x ULN for age; conjugated bilirubin > 1 mg/dL; unexplained fat‑soluble vitamin deficiency; GGT > 3 x ULN for age; intractable pruritus attributable to liver disease.

Pruritus severity: Member has an average daily ItchRO score > 2 (submit ItchRO documentation).

Prior medication trial: Member has failed an adequate trial, is intolerant to, or has a contraindication to ONE of: ursodeoxycholic acid (ursodiol) or rifampin (verification via pharmacy paid claims and supporting clinical documentation required).

Exclusions absent: Member does NOT have surgical interruption of the enterohepatic circulation, prior liver transplantation, or decompensated liver cirrhosis.

Dosing limits: Prescribed dose must follow weight‑based dosing (start 190 mcg/kg once daily for 7 days then increase to 380 mcg/kg once daily as tolerated) and must not exceed maximum daily dose (28.5 mg/day for 9.5 mg/mL oral solution = 3 mL/day; 30 mg/day for tablets).

Initial Therapy — PFIC

Covered for PFIC when ALL of the following are met:

PFIC Coverage Criteria: Member has a diagnosis of progressive familial intrahepatic cholestasis (PFIC) and the prescriber selected dosing/formulation per weight and age guidance; medication is prescribed by or in consultation with a hepatologist, gastroenterologist, or physician who specializes in PFIC.

Dosing and formulation: Dosing titration follows recommended mcg/kg schedule (start 285 mcg/kg once daily, titrate to 285 mcg/kg twice daily then higher mcg/kg tiers as tolerated up to 570 mcg/kg twice daily); use the 19 mg/mL oral solution for PFIC when indicated and tablets per weight‑based tablet conversion guidance; maximum daily dose must not exceed 38 mg/day for 19 mg/mL solution (2 mL/day) or 40 mg/day for tablets.

Exclusions absent: Member does NOT have surgical interruption of the enterohepatic circulation, prior liver transplantation, or decompensated liver cirrhosis.

Reauthorization — Alagille syndrome

Reauthorization criteria (ALGS):

Reauthorization (ALGS): Provider submits documentation of ItchRO score decrease from baseline by at least 1 point and documentation of a decrease in serum bile acids; member does NOT have surgical interruption of the enterohepatic circulation, prior liver transplantation, or decompensated liver cirrhosis.

Initial and Reauthorization Criteria

Covered when ALL of the following are met

Eligibility and prescriber: Member is 12 months of age or older AND medication is prescribed by or in consultation with a hepatologist, gastroenterologist, or a physician who specializes in PFIC.

Symptoms and labs: Member is experiencing pruritus requiring at least medium scratching (≥2 on a 0–4 scale) per prescriber assessment AND member's total serum bile acids ≥ 100 µmol/L (submit labs).

Genetic confirmation: Diagnosis confirmed by genetic testing demonstrating a gene mutation affiliated with PFIC (e.g., ATP8B1, ABCB11 [BSEP 1 and BSEP 2]).

Prior therapy failure/intolerance/contraindication: Member has failed, is intolerant to, or has a contraindication to at least ONE of cholestyramine, rifampicin, or ursodiol; AND member has failed, is intolerant to, or has a contraindication to odevixibat (Bylvay) (Bylvay requires prior authorization). Documentation or pharmacy paid claims required.

Members are excluded from coverage if they have undergone surgical interruption of the enterohepatic circulation, have a history of liver transplantation, or have decompensated liver cirrhosis. These exclusionary conditions must be absent on both initial request and at reauthorization; documentation confirming their absence is required with the prior authorization submission.

Coverage is not approved for members with cirrhosis, portal hypertension, or a history of hepatic decompensation events (for example variceal hemorrhage, ascites, or hepatic encephalopathy). Members with pathologic ABCB11 gene variants predicting complete absence of BSEP protein (BSEP 3) are excluded, as are those with other specified liver diseases or prior liver transplant, CKD with GFR < 70 mL/min/1.73 m2, or a medical history of persistent diarrhea. These conditions must be evaluated and documented as part of the prior authorization review.

Initial Therapy and Dosing

Initial dosing (ALGS and PFIC)

Weight-based dosing and formulation guidance

ALGS dosing: Recommended initiation: 190 mcg/kg once daily for Days 1–7, then increase to 380 mcg/kg once daily (taken ~30 minutes before a meal) as tolerated; use 9.5 mg/mL oral solution for ALGS when indicated; tablet dosing by weight per table (tablets used for higher weight bands). Max daily dose: 28.5 mg/day (3 mL) for 9.5 mg/mL solution; 30 mg/day for tablets.

PFIC dosing: Recommended initiation for PFIC: start 285 mcg/kg once daily then titrate to 285 mcg/kg twice daily, then to 428 mcg/kg twice daily and up to 570 mcg/kg twice daily as tolerated; use 19 mg/mL oral solution for PFIC when indicated; tablet conversion per weight bands. Max daily dose: 38 mg/day (2 mL) for 19 mg/mL solution; 40 mg/day for tablets.

Formulation selection: Select product based on patient weight and ability to swallow tablets: use oral solution for lower weight bands and tablets for specified higher weight bands per dosing tables.

Reauthorization / Continuation Criteria

Reauthorization (ALGS)

Reauthorization requirements

Continuation Therapy: For reauthorization (ALGS), submit documentation showing improvement: ItchRO improvement (decrease from baseline by at least 1 point) and a decrease in serum bile acids; confirm absence of surgical interruption of enterohepatic circulation, prior liver transplantation, or decompensated liver cirrhosis.

Reauthorization / Continuation Therapy

Reauthorization requirements for continued coverage

Clinical response: Member has experienced a reduction in serum bile acids from baseline AND a decrease of at least 1 in the pruritus scratching score (ItchRO or prescriber assessment).

Safety for continuation: Member has NOT experienced treatment‑restricting adverse effects (e.g., persistent diarrhea; persistent fat‑soluble vitamin deficiency despite supplementation; elevated liver function tests or bilirubin) AND has NOT developed decompensated cirrhosis or significant portal hypertension.

Provider Actions & Submission Requirements

Prior Authorization

Authorization Duration & Prescriber

Prior authorization is required. Initial authorizations are for 6 months; reauthorizations are for 12 months. The medication must be prescribed by or in consultation with a specialist (hepatologist, gastroenterologist, or a physician who specializes in the relevant disease: Alagille syndrome for ALGS or progressive familial intrahepatic cholestasis [PFIC]).

Initial authorization: 6 months (ALGS and PFIC-specific criteria apply)
Reauthorization: 12 months (documentation of clinical response and absence of treatment-restricting adverse effects required)
Specialty prescriber required: hepatologist, gastroenterologist, or disease-specialist (ALGS or PFIC)

Documentation Required

Submission Requirements & Form Completeness

The prescribing physician must sign and clearly print their name on the PA request (preprinted stamps are not valid). Incomplete, incorrect, or illegible forms or missing required supporting documentation may delay processing or result in denial.

Step Therapy Requirements

Requirement	Details
Step prior therapy required before approval (ALGS)
Member must have failed, been intolerant to, or have a contraindication to ONE of ursodeoxycholic acid (ursodiol) or rifampin; failures must be verified by pharmacy paid claims and supporting documentation (labs/ItchRO) must be submitted.

Requirement	Details
Document prior therapies
Prior therapies must be documented via pharmacy paid claims or submitted chart notes; member must have failed, been intolerant to, or have a contraindication to at least one of cholestyramine, rifampicin, or ursodiol, and must have failed/been intolerant to or have a contraindication to odevixibat (Bylvay) — Bylvay requires prior authorization.

Quantity Limits and Formulations

inv-29: Livmarli oral solution 9.5 mg/mL — 3 mL

Drug / FormulationLivmarli (maralixibat) oral solution 9.5 mg/mL

Quantity limit3 mL per day

ContextQuantity limit listed under 'Quantity Limits' for Livmarli formulations

inv-30: Livmarli oral solution 19 mg/mL — 2 mL

Drug / FormulationLivmarli (maralixibat) oral solution 19 mg/mL

Quantity limit2 mL per day

ContextQuantity limit listed under 'Quantity Limits' for Livmarli formulations

Coding & Clinical Thresholds

inv-07: Total serum bile acids — >= 100 µmol/L

TestTotal serum bile acids

Threshold>= 100 µmol/L

UseRequired laboratory criterion for PFIC initial authorization (submit labs)

Site of Care / Dispensing

Billing Rule

Medication dispensed via specialty pharmacy (Proprium Rx)

Medication will be provided by the designated specialty pharmacy (Proprium Rx); use of samples to initiate therapy does not meet step-edit/prior authorization criteria.

Specialty pharmacy: Proprium Rx
Samples do not satisfy step-edit or PA requirements

Definitions

inv-21: Itch-Reported Outcome score used to quantify pruritus severity and response — ItchRO™

MeasureItch-Reported Outcome score (ItchRO™)

PurposeUsed to quantify pruritus severity and response for ALGS (average daily score >2 for initial criteria)

DocumentationItchRO scores must be submitted for authorization and reauthorization decisions

inv-22: Pruritus assessment — Pruritus scoring: medium scratching defined as ≥2 on a 0–4 scale per prescriber assessment

AssessmentPruritus scoring: medium scratching defined as ≥2 on a 0–4 scale per prescriber assessment

UseRequired clinical symptom threshold for PFIC initial authorization (submit pruritus assessment)

Background

Alagille syndrome (ALGS) and progressive familial intrahepatic cholestasis (PFIC) are cholestatic liver disorders characterized by elevated bile acids and troublesome pruritus. Maralixibat (Livmarli), an ileal bile acid transporter (IBAT) inhibitor, is used to reduce cholestatic pruritus in these conditions. For ALGS, coverage requires genetic confirmation (JAG1 or NOTCH2) and clinical evidence including bile duct paucity and multiple major ALGS features, laboratory evidence of cholestasis, and an elevated ItchRO™ score. For PFIC, coverage requires genetic confirmation of a PFIC-associated mutation, pruritus of at least moderate severity, and elevated total serum bile acids, with dosing and formulation determined by weight and age.

Revision History

RevisionLatest

Policy created/updated detailing prior authorization criteria, dosing, exclusions, and documentation requirements for Livmarli (maralixibat) for ALGS and PFIC; initial authorization 6 months and reauthorization 12 months; quantity limits set for formulations; specialty pharmacy designated (Proprium Rx).

Policy Summary

PayerAvMed

PolicyLivmarli (maralixibat) prior authorization/step-edit

Policy CodePolicy N/A

Change TypeNo material change

Effective DateN/A

Next Review DateN/A

SourceLink

On This Page

Exclusion conditions absent: Member does NOT have cirrhosis, portal hypertension, history of hepatic decompensation, pathologic ABCB11 variants predicting complete absence of BSEP (BSEP 3), other specified liver diseases or prior liver transplant, CKD with GFR <70 mL/min/1.73 m2, or medical history of persistent diarrhea.

Dose and safety: Prescribed dose must NOT exceed FDA‑approved labeling; for reauthorization, member must have reduction in serum bile acids from baseline and a decrease of at least 1 in pruritus scratching score and must not have treatment‑restricting adverse effects or developed decompensated cirrhosis/portal hypertension.

Initial Therapy — Dosing guidance and initial authorization

Dosing guidance and initial authorization

Weight-based dosing: Tablet and oral solution dosing must follow the weight‑based mcg/kg schedules and conversion tables; use oral solution for patients below tablet weight thresholds and tablets where indicated by table guidance.

Authorization period: Initial authorization duration: 6 months.

Dose limit: Prescribed dose must not exceed FDA‑approved labeling.

Prescriber signature and printed name required on PA request (stamps not valid)
Provide complete member and prescriber information (including phone, fax, NPI)
Incomplete/illegible forms or missing information may delay or cause denial

Denial Risk

Required Clinical Documentation & Triggers for Denial

All required clinical documentation must be submitted to support the diagnosis and meet criteria for approval. Failure to provide genetic testing, laboratory results, pruritus assessments, ItchRO scores, pharmacy paid-claims, or chart notes as requested may trigger denial.

Genetic testing results: for ALGS submit JAG1 or NOTCH2 deletion/mutation results; for PFIC submit gene mutation results (e.g., ATP8B1, ABCB11)
Laboratory results: total serum bile acids, conjugated bilirubin, GGT, liver function tests as applicable
Pruritus assessment: ItchRO score at baseline and follow-up; submit documentation showing score change
Chart notes or liver biopsy reports: bile duct paucity for ALGS, other diagnostic confirmations
Pharmacy paid-claims or documentation verifying prior therapy trials or intolerance/contraindication

Documentation Required

Required Clinical Documentation for ALGS

For Alagille syndrome (ALGS) the provider must submit genetic testing confirming a JAG1 or NOTCH2 deletion or mutation and clinical confirmation of disease features. Documentation must include biopsy and/or chart notes demonstrating bile duct paucity and at least three major ALGS criteria, plus evidence of cholestasis and an ItchRO score >2 when applicable.

Genetic testing confirming JAG1 or NOTCH2 mutation (submit results)
Clinical confirmation: bile duct paucity on liver biopsy and >=3 major ALGS criteria (liver/cholestasis, dysmorphic facies, heart disease, axial skeleton/vertebral anomalies, eye/posterior embryotoxin)
Evidence of cholestasis: TWO of the following — total serum bile acids >3x ULN for age; conjugated bilirubin >1 mg/dL; unexplained fat-soluble vitamin deficiency; GGT >3x ULN for age; intractable pruritus attributable to liver disease
ItchRO: average daily score >2 at baseline (submit ItchRO documentation)

Documentation Required

Reauthorization Documentation

Reauthorization requires documentation of clinical benefit and safety: objective decrease in serum bile acids and improvement in pruritus scores, plus evidence of no treatment-restricting adverse effects or development of advanced liver disease.

Submit ItchRO score demonstrating at least a 1-point decrease in pruritus scratching score (PFIC) or documentation of ItchRO change for ALGS as specified
Submit labs showing reduction in total serum bile acids from baseline
Confirm absence of treatment-restricting adverse effects (e.g., persistent diarrhea, persistent fat-soluble vitamin deficiency despite supplementation, elevated LFTs)
Confirm no development of decompensated cirrhosis, liver transplantation, or significant portal hypertension

Step Therapy

Step Therapy / Prior Therapy Requirements & Documentation

Prior therapy trials are required and must be documented (pharmacy paid claims or chart notes). Requests should include evidence of treatment failure, intolerance, or documented contraindication to required agents before approval.

ALGS: Documentation of failure, intolerance, or contraindication to ONE of ursodeoxycholic acid (ursodiol) or rifampin (verified by pharmacy paid claims; labs/ItchRO evidence required)
PFIC: Documentation of failure, intolerance, or contraindication to at least ONE of cholestyramine, rifampicin, or ursodiol (verified by pharmacy paid claims)
For PFIC, documentation of prior trial and/or failure of Bylvay (odevixibat) is required if applicable (verified by pharmacy paid claims; labs/ItchRO evidence required)
Use of samples to initiate therapy does not meet step edit/prior authorization criteria

Documentation Required

Required Documentation & Specialty Pharmacy

Specialty pharmacy dispensation and claims verification: specialty pharmacy is used for medication provision; prior therapies will be verified through pharmacy paid claims. Missing supporting documentation (genetic tests, labs, ItchRO scores, pharmacy claims) may result in denial.

Medication provided by specialty pharmacy (Proprium Rx noted)
Previous therapies verified through pharmacy paid claims or submitted chart notes
Failure to submit required supporting documentation may delay or result in denial

inv-31: Livmarli tablets 10 mg, 15 mg, 20 mg — 2 tablets

Drug / FormulationLivmarli (maralixibat) oral tablets 10 mg, 15 mg, 20 mg

Quantity limit2 tablets per day

ContextQuantity limit applies to 10 mg, 15 mg, and 20 mg tablet strengths as listed

inv-32: Livmarli tablets 30 mg — 1 tablet

Drug / FormulationLivmarli (maralixibat) oral tablets 30 mg

Quantity limit1 tablet per day

ContextQuantity limit applies to 30 mg tablet strength as listed

DocumentationPrescriber must submit pruritus assessment with score as part of PA documentation