CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Medicaid_Chenodiol_Products_Chenodal_Ctexli_20250625

Defines clinical coverage and authorization criteria for chenodiol products (Chenodal for radiolucent gallstones; Ctexli for cerebrotendinous xanthomatosis) including initial and continuation requirements, dosing limits, contraindications (coadministration), and investigational exclusions.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyMedicaid_Chenodiol_Products_Chenodal_Ctexli_20250625

Policy CodePolicy N/A

Change TypeNo material change

Effective Date

Next Review Date

Key ActionSubmit prior authorization with required documentation (diagnostic testing, prior ursodiol trial/intolerance, dosing and weight, genetics and labs for CTX) to request a 6-month authorization.

SourceLink

POLICY UPDATE CHANGES

No material clinical/coverage changes

2Covered Indications

24 moMax Chenodal duration

6 moAuthorization interval

Coverage Summary

Coverage stance: covered_with_criteria. Scope: Defines clinical coverage and authorization criteria for chenodiol products (Chenodal for radiolucent gallstones; Ctexli for cerebrotendinous xanthomatosis) including initial and continuation requirements, dosing limits, contraindications, and investigational exclusions. FDA indications for Chenodal (dissolution of certain radiolucent gallstones in well-opacifying gallbladders when surgery is high risk) and Ctexli (treatment of CTX in adults) are conditionally covered when all specified criteria are met. Authorization interval is 6 months. Maximum duration for Chenodal therapy is 24 months.

Initial Therapy Criteria

Covered Indications Overview

The FDA-approved indications below are covered when ALL approval criteria are met and member has no exclusions.

Covered Indication (one of)

Radiolucent stones in well-opacifying gallbladders (Chenodal) - Initial authorization (6 months)
- Request is for Chenodal
- Member is 18 years of age or older
- Medication is prescribed by, or in consultation with, a gastroenterologist
- Documentation that the member has an increased surgical risk due to systemic disease or age
- Documentation that the member experienced an inadequate treatment response after a 6-month trial or intolerance to ursodiol
- Documentation that the member will not exceed a dose of 16 mg/kg/day16 mg/kg/day
- Documentation that the member has not received more than 24 months of therapy with Chenodal24 months
Cerebrotendinous xanthomatosis (CTX) (Ctexli) - Initial authorization (6 months)
- Member is 18 years of age or older
- Medication is prescribed by, or in consultation with, a physician experienced in CTX treatment (e.g., neurologist, geneticist, endocrinologist, gastroenterologist)
- Documentation of confirmed diagnosis of CTX by genetic testing indicating pathogenic variants in the CYP27A1 gene
- Documentation that the member has an elevated pretreatment plasma cholestanol level
- Documentation that the member has elevated levels of bile alcohol (i.e., 23s-pentol) in the urine
- Documentation that the member has signs and symptoms of CTX (e.g., bilateral cataracts, intractable diarrhea, progressive neurological signs and symptoms, tendon xanthomas)

Continuation Therapy Criteria

Continuation (Reauthorization) Criteria

Authorization of 6 months may be granted for continued treatment when ALL criteria are met.

Reauthorization - Radiolucent stones or CTX (select applicable)

Radiolucent stones - Reauthorization (6 months)
- Documentation that the member has experienced partial (or complete) dissolution of stones, or provider will discontinue therapy if response is not seen by 18 months of treatment
- There is no evidence of unacceptable toxicity
- Documentation that the member will not exceed a dose of 16 mg/kg/day16 mg/kg/day
- Documentation that the member has not received more than 24 months of therapy with Chenodal24 months
Cerebrotendinous xanthomatosis - Reauthorization (6 months)
- Member has not experienced signs and symptoms of hepatotoxicity (e.g., abdominal pain, bruising, dark-colored urine, jaundice)
- Documentation that the member has a confirmed liver transaminase (i.e., ALT, AST) level of less than or equal to 3 times the ULN<= 3 x ULN
- Documentation that the member has a confirmed bilirubin level of less than or equal to 2 times the ULN<= 2 x ULN
- The requested medication will not be used in combination with bile acid sequestering agents (e.g., cholestyramine, colestipol, aluminum-based antacid) or Cholbam
- Evidence of positive clinical response (one of)

Excluded / Not Medically Necessary

Other uses not listed above are not covered.

All other indications are considered experimental/investigational and not medically necessary
Chenodal will not dissolve calcified (radiopaque) or radiolucent bile pigment stones
Safety of use beyond 24 months is not established24 months

Provider Actions

Prior Authorization

Prior authorization required

Authorization of 6 months may be granted when specified initial criteria are met; provider must supply documentation requested (diagnostic testing, prior ursodiol trial/intolerance, dosing and weight, genetics and lab results for CTX).

Authorization interval: 6 months
Max Chenodal duration: 24 months

Documentation Required

Document genetic confirmation for CTX

For CTX, provider must submit genetic testing documentation showing pathogenic variants in CYP27A1 and supporting labs (elevated plasma cholestanol, elevated urine bile alcohol 23s-pentol) and baseline liver tests and bilirubin.

Documentation Required

Document prior ursodiol trial for gallstones

For radiolucent gallstones, provider must document inadequate response after a 6-month trial or intolerance to ursodiol and that patient is high surgical risk.

Denial Risk

Denial risk for contraindicated combinations

Requests will be denied/not approved if medication is to be used in combination with bile acid sequestering agents (e.g., cholestyramine, colestipol, aluminum-based antacid) or Cholbam.

Background

Background: Chenodiol (Chenodal) is FDA-indicated for dissolution of certain radiolucent gallstones in well-opacifying gallbladders for patients in whom selective surgery would otherwise be undertaken but who have increased surgical risk due to systemic disease or age. Safety of Chenodal use beyond 24 months is not established, and Chenodal will not dissolve calcified (radiopaque) or radiolucent bile pigment stones. Ctexli (chenodiol) is FDA-indicated for treatment of cerebrotendinous xanthomatosis (CTX) in adults.

Definitions: Radiolucent stones in well-opacifying gallbladders — Gallstones that are radiolucent on imaging in gallbladders that opacify well; such stones are candidates for chenodiol dissolution therapy when surgery poses increased risk. Cerebrotendinous xanthomatosis (CTX) — A genetic disorder caused by pathogenic variants in CYP27A1 characterized by elevated cholestanol, bile alcohols, cataracts, diarrhea, neurologic decline, and tendon xanthomas.