Familial Chylomicronemia Syndrome Therapy
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Defines medical necessity, site-of-care, and prior authorization requirements for use of olezarsen (Tryngolza) and plozasiran (Redemplo) as adjuncts to diet to reduce triglycerides in adults with familial chylomicronemia syndrome (FCS) for Blue Cross NC members.
Added olezarsen (Tryngolza) to policy for the treatment of adults with familial chylomicronemia syndrome, as an adjunct to diet to reduce triglycerides.
For Tryngolza, added requirement for trial and failure of Redemplo (or intolerance/contraindication) and requirement for use of self-administered formulation unless criteria for supervised administration are met.
Added associated dosing, maximum units, and HCPCS codes C9399, J3490, and J3590 to the FDA label reference table for Tryngolza and Redemplo.
Removed requirement for medical record documentation for specialist requirement, concomitant use statement, and verification that secondary causes have been ruled out.
Changed policy name from 'Plozasiran (Redemplo ® )' to 'Familial Chylomicronemia Syndrome Therapy'.
Coverage and Medical Necessity Criteria
Site of Care
Site-of-Care Medical Necessity Criteria for inpatient/outpatient administration:
The requested agent must not be received in combination with olezarsen (Tryngolza) or plozasiran (Redemplo). This is a required exclusion in the initial and continuation coverage criteria and medical record documentation should demonstrate that the member will not be co‑administered either restricted product.
Injection or infusion administration in an inpatient setting solely for the purpose of administering the injection/infusion is not permitted. Inpatient administration is only allowed when the admission is for clinical reasons beyond drug administration and the member meets the policy's medical necessity criteria. If inpatient or outpatient hospital administration is requested, the applicable Site‑of‑Care Medical Necessity Criteria must be met; otherwise administration should occur in a home‑based infusion or physician office setting.
Coding, Dosing, and Clinical Thresholds
Prior Authorization, Documentation, and Provider Requirements
Prior Authorization Required
Prior authorization is required for olezarsen (Tryngolza) and plozasiran (Redemplo). Requests must meet the applicable initial or continuation criteria and dosing/maximum units must correspond to the HCPCS entries and maximum units listed in the policy.
Step-Therapy Requirement for Olezarsen
For olezarsen (Tryngolza) only: a documented trial and inadequate response to plozasiran (Redemplo) OR documented intolerance, FDA-labeled contraindication, or hypersensitivity to plozasiran is required before approval.
- Must document trial and inadequate response to plozasiran OR intolerance/contraindication/hypersensitivity to plozasiran
Medical Record Documentation Required
Medical record documentation is required to support the diagnosis of familial chylomicronemia syndrome (FCS), confirmation method (genetic testing, NAFCS ≥45, or ≥3 fasting TG measurements ≥880 mg/dL), prior use and inadequate response to conventional lipid-lowering therapies, adherence to a low-fat diet, inability to self-administer (and lack of caregiver), specialist involvement or consultation, and baseline and follow-up triglyceride measurements for continuation.
- Documentation of FCS diagnostic criteria (genetic confirmation OR NAFCS ≥45 OR ≥3 fasting TG ≥880 mg/dL)
- Documentation of trial and inadequate response (<20% TG decrease) to conventional lipid-lowering therapies (fibrates, omega-3s, statins, niacin, ezetimibe, PCSK9 inhibitors)
- Documentation of secondary causes of hypertriglyceridemia being ruled out
- Documentation of adherence to a low-fat diet (≤20 g fat/day)
- Documentation of inability to self-administer and lack of caregiver/support if requesting facility administration
- Specialist clinician involvement or consult note
- Baseline fasting TG ≥880 mg/dL and follow-up TG demonstrating clinical response for continuation
Requests Missing Required Documentation May Be Denied
Requests that lack the required medical record documentation for diagnosis confirmation, trial/failure of conventional therapy, step-therapy for olezarsen, diet adherence, administration limitations, specialist involvement, or baseline/follow-up triglyceride measurements will be subject to denial.
- Missing genetic test results, NAFCS score, or serial fasting TG records (≥3 values)
- No documentation of trial and inadequate response to conventional lipid-lowering therapies
- No documentation of trial or intolerance/contraindication to plozasiran when requesting olezarsen
- No documentation of low-fat diet adherence (≤20 g/day) or lack of self-administration capability and caregiver availability
- Absence of specialist involvement or consult
Background and Condition Overview
Familial chylomicronemia syndrome (FCS) is a rare genetic disorder that results in severe hypertriglyceridemia and an increased risk of recurrent acute pancreatitis. Management centers on strict dietary fat restriction and therapies that lower triglycerides. Targeted agents such as olezarsen (Tryngolza) and plozasiran (Redemplo) are approved as adjuncts to a low‑fat diet to reduce triglyceride levels in adults with confirmed FCS. Policy criteria require objective confirmation of FCS (genetic testing, NAFCS, or repeated fasting triglyceride measurements), documentation of baseline fasting triglycerides, and evidence of pancreatitis history or other qualifying features prior to initiating these agents.
Definitions and Diagnostic Criteria
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