Aldurazyme (laronidase) prior authorization for MPS I
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Requirements and clinical criteria for prior authorization and continuation of intravenous Aldurazyme (laronidase) for members with mucopolysaccharidosis type I (MPS I); applies to providers requesting medical benefit coverage from AvMed.
No material clinical or coverage changes in this revision.
Coverage Criteria for Aldurazyme (laronidase)
Initial Therapy
Covered when ALL of the following are met for initial authorization:
Definitive Diagnosis
- ONE of: Detection of biallelic pathogenic mutations in the IDUA gene by molecular genetic testing
- ONE of: Fibroblast or leukocyte alpha-L-iduronidase (IDUA) enzyme activity level < 10% of the lower limit of the normal range of the measuring laboratory
Phenotype and Cognitive Status
- ONE of: Diagnosis of Hurler (severe) or Hurler-Scheie (attenuated)
- ONE of: Diagnosis of Scheie (attenuated) with moderate to severe symptoms
Baseline Clinical Measures
- For members ≥ 6 years: Percent predicted FVC ≤ 77% and/or 6-minute walk test (must stand independently for 6 minutes and walk ≥ 5 m); joint range of motion; left ventricular hypertrophy; growth; quality of life (CHAQ/HAQ/MPS HAQ)
- For members 6 months to < 6 years: Cardiac status; upper airway obstruction during sleep; growth velocity; mental development; FVC; and/or 6-minute walk test (must stand independently for 6 minutes and walk ≥ 5 m)
Continuation Therapy
Covered when ALL of the following are met for continuation of therapy:
Clinical Benefit
- For members ≥ 6 years: Stability or improvement in percent predicted FVC and/or 6-minute walk test; increased joint range of motion; decreased left ventricular hypertrophy; improved growth; improved quality of life (clinically meaningful change in CHAQ/HAQ/MPS HAQ)
- For members 6 months to < 6 years: Stability or improvement in cardiac status; upper airway obstruction during sleep; growth velocity; mental development; FVC; and/or 6-minute walk test
Continuation of Aldurazyme (laronidase) is not permitted when the member experiences unacceptable toxicity attributable to the drug. Examples of unacceptable toxicities include anaphylaxis, severe hypersensitivity reactions, acute respiratory complications, acute cardiorespiratory failure, and severe infusion reactions. Such toxicities preclude continuation of therapy and should be documented in the medical record.
Covered Indications and Diagnostic Confirmation
Confirmation of MPS I to establish eligibility for laronidase therapy
Definitive diagnosis of MPS I can be established when ONE of the following is met:
Member Eligibility Requirements
To be eligible for initiation of Aldurazyme (laronidase) the member must meet all initial authorization criteria: be aged ≥ 6 months; have a definitive diagnosis of MPS I established by either detection of biallelic pathogenic IDUA mutations on molecular testing or fibroblast or leukocyte alpha-L-iduronidase enzyme activity <10% of the lower limit of normal; have a qualifying clinical diagnosis (Hurler or Hurler‑Scheie, or Scheie with moderate to severe symptoms); have absence of severe cognitive impairment; and have documented baseline laboratory and age‑appropriate clinical measures including urinary glycosaminoglycan (uGAG) and the specified functional assessments (for ≥6 years: percent predicted FVC ≤77% and/or 6‑minute walk test with the ability to stand independently and walk ≥5 m, joint ROM, LVH, growth, quality‑of‑life measures; for 6 months to <6 years: cardiac status, sleep‑related upper airway obstruction, growth velocity, mental development, FVC, and/or 6‑minute walk test).
Billing Codes and Quantity Limits
| J1931 | Aldurazyme (laronidase) IV solution |
Provider Actions, Documentation, and Prior Authorization
Prior Authorization Required
Prior authorization is required for Aldurazyme (laronidase) J1931 (medical benefit). Initial authorizations are for 6 months; continuation authorizations are for 12 months and require documentation of ongoing benefit and absence of unacceptable toxicity. Incomplete, incorrect, or illegible information on the request form may delay or deny authorization.
- J1931 (Aldurazyme) — Medical benefit
- Initial Authorization: 6 months
- Continuation Authorization: 12 months — requires documentation of benefit and absence of unacceptable toxicity
Samples and Prior Therapy Verification
Use of samples to initiate therapy does not meet preauthorization criteria. Previous therapies will be verified via pharmacy paid claims or submitted chart notes. For urgent reviews, contact AvMed Pre-Authorization Department per plan guidance.
- Samples do not meet step-edit/preauthorization criteria
- Previous therapies will be verified through pharmacy paid claims or submitted chart notes
- For urgent reviews, call AvMed Pre-Authorization Department
Required Clinical Documentation
Provide diagnostic and baseline data to support the clinical criteria. Requests lacking the required documentation may be delayed or denied.
- Definitive diagnosis of MPS I confirmed by one of: detection of biallelic pathogenic IDUA mutations OR fibroblast/leukocyte IDUA enzyme activity < 10% of the lab's lower limit of normal
- Documented diagnosis details (Hurler, Hurler-Scheie, or Scheie with moderate to severe symptoms) and absence of severe cognitive impairment
- Baseline urinary glycosaminoglycan (uGAG) value
- Baseline functional measures: For ≥6 years — percent predicted FVC, 6-minute walk test (must stand independently for 6 minutes and walk ≥5 meters), joint range of motion, left ventricular hypertrophy, growth, quality of life (CHAQ/HAQ/MPS HAQ); For 6 months to <6 years — cardiac status, upper airway obstruction during sleep, growth velocity, mental development, FVC, and/or 6-minute walk test
Form and Provider Identification
The prescribing physician must sign and clearly print their name on the request form (preprinted stamps are not valid). Include complete member and prescriber demographic and contact information to avoid delays.
- Prescriber signature and date (hand-signed; preprinted stamps not valid)
- Member name, AvMed ID, date of birth
- Prescriber name, NPI, office contact name, phone number, fax number
- Drug name/form/strength, dosing schedule, length of therapy, diagnosis and ICD code, weight and date obtained, quantity/units requested, and location/site of administration or specialty pharmacy
Missing Supporting Documentation
All clinical criteria boxes checked on the form must be supported by corresponding documentation (lab results, diagnostics, chart notes). Requests may be denied if supporting documentation is missing.
- To support each checked clinical criterion, provide corresponding documentation (e.g., genetic testing reports, enzyme assay results, uGAG, pulmonary function tests, 6-minute walk test, cardiac evaluations, growth records, QoL assessments)
- Incomplete or missing supporting documentation may result in denial of the request
Key Definitions
Background on Mucopolysaccharidosis Type I (MPS I)
Mucopolysaccharidosis type I (MPS I) is an inherited lysosomal storage disorder caused by pathogenic variants in the IDUA gene resulting in deficient alpha‑L‑iduronidase activity and accumulation of glycosaminoglycans. Clinical manifestations vary from severe (Hurler) to attenuated (Hurler‑Scheie, Scheie) phenotypes; non‑central nervous system manifestations (cardiac, respiratory, skeletal, growth, and functional limitations) are the primary targets for enzyme replacement therapy with laronidase (Aldurazyme).
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