Coverage Policy Manual
Policy #: 2003034
Category: Medicine
Initiated: July 2003
Last Review: July 2023
  Nutritional Supplements

Description:
Classic phenylketonuria (PKU) is a rare metabolic disorder that usually results from a deficiency of a liver enzyme known as phenylalanine hydroxylase (PAH). This enzyme deficiency leads to elevated levels of the amino acid phenylalanine (Phe) in the blood and other tissues. Mental retardation, microcephaly, delayed speech, seizures, eczema, behavior abnormalities and other symptoms characterize the untreated state. Approximately 1 of every 15,000 infants in the United States is born with PKU.
 
The current treatment for this disorder involves strict metabolic control using a low-Phe diet that includes specialized medical foods. Metabolic control of PKU can be difficult to achieve, and poor control can result in significant decline of mental and behavioral performance. Women with PKU must also maintain strict metabolic control before and during pregnancy to prevent fetal damage.
 
Implementing a Phe-restricted diet early in life can significantly reduce mental deficiencies associated with PKU. Metabolic control via medical nutrition therapy involves the use of medical foods including medical protein sources and modified low-protein products in addition to the provision of required amounts of Phe through small amounts of natural protein. The response is monitored throughout periodic measurement of blood Phe levels in conjunction with analysis of nutritional intake and review of nutritional status. Most experts advocate lifelong dietary treatment for metabolic control of blood Phe levels. Data suggest that elevated Phe levels in adolescents and adults adversely affect cognitive function and case reports have documented deterioration of adult patients with PKU after diet discontinuation.
 
These medical food nutrients are not prescription drugs; they are, however, not products that one can walk into a drug store and purchase off the shelf. They must be special ordered through a pharmacy or pharmaceutical organization.
 
The available medical foods include but are not limited to, the following: Lofenalac, Phenyl Free 1, Phenyl Free 2, Phenyl Free HP, Milupa PKU-1, Milupa PKU-2, Milupa PKU-3, Crystalline amino acid mix plus Protein Free Diet Powder (MJ 80056), Periflex, X Phe Analog, X Phe Maxamaid, X Phe Maxamum, Phenex-1, Phenex-2,, Phlexy-10, Phenylade Blend, Lophlex, Phlexy-Vits, Add-Ins, Phenyl Ade, PKU Gel, PKU Express, PKU Cooler,and Camino Pro.

Policy/
Coverage:
Effective June 2014
Dietary and nutritional services are contract specific benefits. Subject to all terms, conditions, exclusions and limitations as set forth in the members benefit certificate, coverage is provided for Medical Foods and Low Protein Modified Food Products for the treatment of the following disorders:
 
    • Phenylketonuria (PKU) and other amino acid metabolism disorders (e.g., maple syrup urine disease, homocystinuria, tyrosinemia)
    • Galactosemia
    • Organic acidemias
    • Fatty acid and oxidative disorders (e.g, carnitine transporter deficiency)
 
Any services or supplies provided for dietary and nutritional services, other than supplements for those specific disorders listed above, including but not limited to medical nutrition therapy, unless such dietary supplies are the sole source of nutrition for the Covered Person, are not covered.  Baby formula or thickening agents, whether prescribed by a Physician or acquired over the counter, is not a covered benefit.
 

Rationale:
2017 Update
A literature search conducted through June 2017 did not reveal any new information that would prompt a change in the coverage statement.
 
2018 Update
A literature search was conducted through June 2018.  There was no new information identified that would prompt a change in the coverage statement.  
 
2019 Update
Annual policy review completed with a literature search using the MEDLINE database through June 2019. No new literature was identified that would prompt a change in the coverage statement.
 
2021 Update
Annual policy review completed with a literature search using the MEDLINE database through June 2021. No new literature was identified that would prompt a change in the coverage statement.
 
2022 Update
Annual policy review completed with a literature search using the MEDLINE database through June 2022. No new literature was identified that would prompt a change in the coverage statement.
 
2023 Update
Annual policy review completed with a literature search using the MEDLINE database through June 2023. No new literature was identified that would prompt a change in the coverage statement.

CPT/HCPCS:
S9435Medical foods for inborn errors of metabolism

References: Howell RR, et al.(2001) National institutes of health consensus development conference statement: Phenylketonuria: screening and management. Pediatrics 2001; 108:4; October.


Group specific policy will supersede this policy when applicable. This policy does not apply to the Wal-Mart Associates Group Health Plan participants or to the Tyson Group Health Plan participants.
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