CurrentAvMedPolicy N/A

Ctexli (chenodiol) prior authorization — Pharmacy Prior Authorization / Coverage Criteria

Defines pharmacy prior authorization, initial and reauthorization clinical criteria, documentation and quantity limits for Ctexli (chenodiol) for members; applies to prescribers and pharmacy prior authorization process.

Policy Summary

PayerAvMed

PolicyCtexli (chenodiol) prior authorization — Coverage criteria

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateN/A

Key ActionObtain prior authorization with required documentation including genetic test results and baseline labs within 30 days.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

3/dayquantity limit

6 moinitial auth

12 moreauth

≥18min age

30 dayslabs window

Coverage Criteria for Ctexli (chenodiol)

Initial Therapy — Covered when ALL of the following are met for initial authorization

Covered when ALL of the following are met for initial authorization:

Initial Authorization Criteria: Member is 18 years of age or older; prescribed by or in consultation with a physician experienced in treatment of cerebrotendinous xanthomatosis (CTX) (e.g., neurologist, endocrinologist, hepatologist, gastroenterologist, or other metabolic specialist); diagnosis of CTX confirmed by genetic testing with submission of test results documenting a pathogenic variant in the CYP27A1 gene; member has symptoms of CTX with at least ONE of the following: neonatal cholestasis, infantile-onset diarrhea, juvenile cataracts, adolescent- to young adult-onset tendon xanthomas, or adult-onset progressive neurologic dysfunction (e.g., dementia, psychiatric disturbances, pyramidal and/or cerebellar signs, or seizures); baseline laboratory results within the last 30 days documenting ALT, AST, total bilirubin, urine 23S-pentol (must be above normal limits per reference range), and plasma cholestanol (must be above normal limits per reference range); provider attests member will receive liver function monitoring at least annually due to potential hepatotoxicity during treatment with Ctexli.

Initial authorization duration: 6 months

Continuation Therapy / Reauthorization — Reauthorization covered when ALL of the following are met

Reauthorization covered when ALL of the following are met:

Policy Summary

PayerAvMed

PolicyCtexli (chenodiol) prior authorization — Coverage criteria

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateN/A

Key ActionObtain prior authorization with required documentation including genetic test results and baseline labs within 30 days.

SourceLink

On This Page

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

Reauthorization Criteria: Member continues to meet ALL initial authorization criteria; member must have reduction in urine 23S-pentol and plasma cholestanol from baseline after initial approval, and reduction or stabilization in urine 23S-pentol and plasma cholestanol after subsequent approvals (current lab test results must be submitted); member has experienced an absence of unacceptable toxicity from the medication (e.g., hepatotoxicity).

Reauthorization duration: 12 months

Concomitant Medication Exclusion — Drug interaction restriction

Drug interaction restriction

Concomitant medication prohibition: Member will NOT use Ctexli (chenodiol) with medications that impact bile acid absorption or synthesis (examples: cholestyramine, colestipol, colesevelam, aluminum-based antacids, Cholbam (cholic acid), Iqirvo (elafibranor), Livdelzi (seladelpar), Ocaliva (obeticholic acid), Bylvay (odevixibat), Livmarli (maralixibat)).

Concurrent use of Ctexli (chenodiol) with agents that affect bile acid absorption or synthesis is prohibited. Examples include cholestyramine, colestipol, colesevelam, aluminum-based antacids, Cholbam (cholic acid), Iqirvo (elafibranor), Livdelzi (seladelpar), Ocaliva (obeticholic acid), Bylvay (odevixibat), and Livmarli (maralixibat).

Therapy does not meet criteria if the member lacks genetic confirmation of CTX, required baseline laboratory testing, specialist involvement, or is under age 18. Specifically, approval requires documentation of a pathogenic CYP27A1 variant by genetic testing, submission of baseline labs within the last 30 days including ALT, AST, total bilirubin, urine 23S-pentol (above reference range), and plasma cholestanol (above reference range), and that the drug is prescribed by or in consultation with a physician experienced in CTX care (e.g., neurologist, endocrinologist, hepatologist, gastroenterologist, or metabolic specialist).

Reauthorization / Continuation Criteria

Reauthorization — Reauthorization requirements

Reauthorization requirements

Reauthorization nodes: Member continues to meet initial criteria; documented reduction in urine 23S-pentol and plasma cholestanol from baseline after initial approval, and reduction or stabilization in these tests after subsequent approvals (current lab results must be submitted); absence of unacceptable toxicity from requested medication (e.g., hepatotoxicity).

Coding and Laboratory Thresholds

Diagnosis codes (form field)ICD-10

ICD code

ICD Code, if applicable (blank on form)

Laboratory abnormality thresholds — CTX monitoring

Urine 23S-pentol thresholdUrine 23S-pentol (ng/mL) must be above normal limits per the laboratory reference range

Plasma cholestanol thresholdPlasma cholestanol (μg/mL) must be above normal limits per the laboratory reference range

Timing of labsRequired laboratory results must be taken within the last 30 days and submitted with the request

Quantity Limits

Ctexli (chenodiol) — Quantity limit

Quantity limit (daily)3 tablets per day

Field on formQuantity Limit: 3 tablets per day appears on the drug information section of the prior authorization form

Authorization dependencyQuantity limit applies for initial and continuation authorizations per policy documentation

Provider Actions and Submission Requirements

Prior Authorization

Prior Authorization Required

Prior authorization required. Initial approval up to 6 months; reauthorization up to 12 months contingent on meeting criteria and submission of required documentation. Incomplete, incorrect, or illegible information on the request form can delay authorization or lead to denial.

Initial Authorization: up to 6 months
Reauthorization: up to 12 months
Fax completed form to 1-305-671-0200

Step Therapy

Samples and Prior Therapy Verification

Use of samples to initiate therapy does not satisfy step-edit or preauthorization criteria. Prior therapies will be verified via pharmacy paid claims or submitted chart notes; ensure any prior treatment documentation is provided.

Samples do not meet step-edit/preauthorization requirements
Previous therapies will be verified

Documentation Required

Required Submission Documents

Provide a fully completed request including prescriber signature (preprinted stamps are not valid), member name and AvMed ID, prescriber identifiers (NPI), dosing, diagnosis and ICD code if applicable, weight and date obtained if relevant, phone and fax numbers, and expected length of therapy.

Prescriber signature required — no preprinted stamps
Member AvMed # and date of birth
Prescriber name, NPI, office contact, phone and fax
Drug name/form/strength, dosing schedule, length of therapy, quantity limit

Denial Risk

Missing Clinical Documentation May Lead to Denial

Failure to provide required clinical documentation — for example genetic test results documenting a pathogenic variant in CYP27A1, recent lab results (ALT, AST, total bilirubin, urine 23S-pentol, plasma cholestanol), or supporting chart notes — may result in denial. Labs must be current as specified (e.g., lab results within last 30 days where indicated).

Submit genetic test results confirming pathogenic CYP27A1 variant when applicable
Submit liver function tests: ALT, AST, total bilirubin
Submit urine 23S-pentol and plasma cholestanol results (must be above normal limits)
Provide chart notes or diagnostics to support clinical criteria

Background

Cerebrotendinous xanthomatosis (CTX) is a rare metabolic disorder for which chenodiol (Ctexli) is indicated when disease is confirmed by genetic testing. Treatment requires involvement of a clinician experienced in CTX management (examples include neurologists, endocrinologists, hepatologists, gastroenterologists, or metabolic specialists) and mandated baseline and ongoing laboratory monitoring because of the potential for hepatotoxicity; initial authorization is for 6 months with reauthorization contingent on clinical and laboratory response.

Definitions

Confirmed CTX — Definition and documentation

DefinitionDiagnosis of cerebrotendinous xanthomatosis (CTX) confirmed by genetic testing documenting a pathogenic variant in the CYP27A1 gene

Documentation requiredMust submit genetic test results documenting the pathogenic CYP27A1 variant with the authorization request

Role in coverageGenetic confirmation is a required element of initial authorization criteria for Ctexli

Step Therapy / Prior Treatment Verification

Step	Requirement
1	Use of samples to initiate therapy does not meet step edit/preauthorization criteria; previous therapies will be verified through pharmacy paid claims or submitted chart notes.

Site of Care / Dispensing

Note

Specialty pharmacy (Proprium Rx)

This medication is dispensed through a specialty pharmacy: Proprium Rx.