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AetnaCommercial · Prior Auth · Medical Policy · Genetics, EndocrinologyMedium impact

Pegvaliase-pqpz (Palynziq) (CPB 0933, reviewed 2026-06-09)

Effective date
Not stated in the notice
Identified
Aug 13, 2026

Summary

Aetna considers pegvaliase-pqpz (Palynziq) medically necessary for phenylketonuria in members 12 years of age or older when PKU is confirmed by blood phenylalanine above 600 micromol/L or by genetic testing, and baseline blood phenylalanine on existing management is above 600 micromol/L. Continuation is medically necessary when blood phenylalanine is 600 micromol/L or less, or when the member has not been titrated to the maximum dose of 60 mg once daily or has received less than 16 weeks of continuous treatment at that dose. The drug must be prescribed by or in consultation with a physician who specializes in metabolic disease or PKU, and it should not be used concomitantly with Kuvan.

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What to do

Action · 5 steps
01Billing team: Before billing Palynziq, confirm the prescriber is a physician who specializes in metabolic disease or PKU, or is working in consultation with one.
02Confirm the member is 12 years of age or older, and document the PKU diagnosis (blood phenylalanine above 600 micromol/L or genetic testing) and the baseline blood phenylalanine above 600 micromol/L on existing management.
03For continuation, document blood phenylalanine of 600 micromol/L or less, or document that the member has not been titrated to 60 mg once daily or has had less than 16 weeks of continuous treatment at that dose.
04Use ICD-10 E70.0 for classical phenylketonuria.
05The policy lists CPT 84030 (blood phenylalanine test) and CPT 96372 (subcutaneous or intramuscular injection) as related codes, and Palynziq has no specific HCPCS code.

Codes mentioned

8403096372E70.0

Original notice

Pegvaliase-pqpz (Palynziq) (CPB 0933, reviewed 2026-06-09)
aetna.com · Always check the source before changing claims.
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Related policy hubs

Aetna policiesGeneticsEndocrinologyCode 84030Code 96372Code E70.0

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