J2508 — Injection, pegunigalsidase alfa-iwxj, 1 mg
HCPCS/CPT
Prior Authorization
No Prior Auth Required
Code is covered without prior authorization (high confidence)
Medicare Pricing
Work RVU
0.00
Facility
N/A
Non-Facility
N/A
Documentation Required
- Objective laboratory confirmation of diagnosis for each therapy: as specified per drug — typically one of: (a) deficiency/absence of specific enzyme activity measured in fibroblasts, leukocytes, dried blood spots, lymphocytes, serum, or muscle (policy specifies thresholds where applicable: e.g., Aldurazyme alpha‑L‑iduronidase; Elfabrio/Fabrazyme α‑Gal A <5% of mean; Lamzede alpha‑Mannosidase <10% of mean; Lumizyme infantile <1% fibroblasts; Lumizyme/Nexviazyme/Pombiliti late‑onset <40% lab mean), OR (b) molecular genetic testing confirming pathogenic mutations in the disease‑specific gene (examples: IDUA for MPS I, IDS for MPS II, GLA for Fabry, LIPA for LAL‑D, MAN2B1 for alpha‑mannosidosis, GAA for Pompe, GUSB for MPS VII, ASB for MPS VI, MOCS1 for MoCD type A, ADA1 for ADA‑SCID, GALNS for MPS IVA, SMPD1 for ASMD).
- Presence of clinical signs and symptoms consistent with the disease at time of request (policy lists disease‑specific examples: e.g., hepatosplenomegaly, cardiac abnormalities, corneal clouding, dysostosis multiplex for MPS I; hepatosplenomegaly and skeletal deformities for MPS II; acroparesthesias, angiokeratomas, renal disease for Fabry; abdominal distention, hepatosplenomegaly, liver fibrosis for LAL‑D; cardiomyopathy, respiratory distress, skeletal muscle weakness for Pompe; seizures, exaggerated startle, feeding difficulties and specific biochemical markers within first 28 days for MoCD type A; etc.).
Key Coverage Criteria
- Aldurazyme (laronidase) for treatment of mucopolysaccharidosis I (MPS I): Hurler variant (MPS IH), Hurler-Scheie variant (MPS IHS), or Scheie variant (MPS IS) when diagnosis is confirmed by deficiency/absence of alpha-L-iduronidase enzyme activity in fibroblasts or leukocytes or by molecular genetic confirmation of mutations in the alpha-L-iduronidase gene, plus presence of clinical signs/symptoms.
- Elaprase (idursulfase) for treatment of mucopolysaccharidosis II (MPS II, Hunter syndrome) when diagnosis is confirmed by deficiency in iduronate 2-sulfatase activity in fibroblasts or leukocytes (with normal activity of another sulfatase) or by molecular genetic testing for iduronate 2-sulfatase gene mutations, plus presence of clinical signs/symptoms.
- Elfabrio (pegunigalsidase alfa-iwxj) for treatment of adults with confirmed Fabry disease when diagnosis is confirmed by absence/deficiency (<5% of mean) of alpha-galactosidase A activity in leukocytes, dried blood spots, or serum analysis, or by molecular genetic testing for GLA (galactosidase alpha) gene mutations, plus presence of clinical signs/symptoms, and when not given in combination with another disease-modifying Fabry therapy.
- Fabrazyme (agalsidase beta) for treatment of Fabry disease when diagnosis is confirmed by absence/deficiency (<5% of mean) of alpha-galactosidase A activity in leukocytes, dried blood spots, or serum analysis, or by molecular genetic testing for GLA gene mutations, plus presence of clinical signs/symptoms, and when not given in combination with another disease-modifying Fabry therapy.
- Kanuma (sebelipase alfa) for treatment of lysosomal acid lipase deficiency (LAL-D; including Wolman disease and cholesteryl ester storage disease) when diagnosis is confirmed by absence/deficiency of LAL activity by dried blood spot test or by molecular genetic testing for LIPA gene mutations, plus presence of clinical signs/symptoms.
- Lamzede (velmanase alfa-tycv) for treatment of alpha-mannosidosis when diagnosis is confirmed by absence/deficiency (<10% of lab-specific normal mean) of alpha-mannosidase enzyme activity or by molecular genetic testing for MAN2B1 gene mutations, plus presence of clinical signs/symptoms, and only for non–central nervous system (non‑CNS) manifestations.
9 Active Policies
UHC-POL-enzyme-replacement-therapy — Medical Therapies for Enzyme Deficiencies
UHC-POL-provider-administered-drugs-soc — Provider Administered Drugs – Site of Care
UMR-POL-UMR-enzyme-replacement-therapy — Medical Therapies for Enzyme Deficiencies
UMR-POL-UMR-provider-administered-drugs-soc — Provider Administered Drugs – Site of Care
Related Codes
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