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Amino Acid-Based Elemental Formula and Medical Foods for the Treatment of Inborn Errors of Metabolism

» Summary» Procedure Codes
» Description» Selected References
» Prior Approval» Policy History
» Policy

Medical Policy: 10.01.15 
Original Effective Date: May 2010 
Reviewed: May 2015 
Revised: May 2015 

Benefit Application
Benefit determinations are based on the applicable contract language in effect at the time the services were rendered. Exclusions, limitations or exceptions may apply. Benefits may vary based on contract, and individual member benefits must be verified. Wellmark determines medical necessity only if the benefit exists and no contract exclusions are applicable. This medical policy may not apply to FEP. Benefits are determined by the Federal Employee Program.

This Medical Policy document describes the status of medical technology at the time the document was developed. Since that time, new technology may have emerged or new medical literature may have been published. This Medical Policy will be reviewed regularly and be updated as scientific and medical literature becomes available.


Amino acid-based elemental formulas are prepared from individual (single) non-allergenic amino acids different from regular dairy (milk or soy based) formulas and foods that include numerous complete proteins. Amino acid-based elemental formulas are made of proteins broken down to their "elemental level" (simplest and purest form) making them easily absorbed and digested by the body. Amino acid-based elemental formulas are the only thing an infant or child can appropriately digest and tolerate due to a variety of allergies or gastrointestinal conditions. Amino acid-based elemental formulas currently available are:

  • Neocate®
  • EleCare®
  • PurAmino ® (formerly  Nutramigen® AA™ )
  • Vivonex

The American Academy of Pediatrics has recommended extensively hydrolyzed formulas (not amino-acid based formula) for infants who are not breastfed and who cannot tolerate cow's milk formulas due to allergy. The U.S. Food and Drug Administration (FDA) defines a medical food in the Orphan Drug Amendment as a food which is formulated to be consumed or administered under the supervision of a physician and which is intended for the specific dietary management of a disease or condition for which distinctive nutritional requirements, based on recognized scientific principles, are established by medical evaluation. Inborn errors of metabolism (IEM) are inherited genetic disorders that interfere with specific metabolic pathways. These interferences may result in either a deficiency or excess of a particular product. Special food products are required to treat inborn errors of metabolism in order to avoid long-term complications which can result in mental retardation, seizures, coma or death.


Prior Approval: 


Not applicable



Amino Acid Based Elemental Formulas

100% Amino acid-based elemental oral formulas may be considered medically necessary when ordered by a physician for diagnosed cases of amino acid, organic acid and fatty acid metabolic and malabsorption disorders, cases of IgE mediated allergies to food proteins; food protein-induced enterocolitis syndrome; eosinophilic esophagitis; eosinophilic gastroenteritis; and eosinophilic colitis.


Coverage of 100% amino acid-based elemental formulas for patients diagnosed with an IgE mediated condition is limited to under 6 years of age.


The covered formula must contain 100% free amino acids as the protein source.  Hypoallergenic formulas are not always amino acid formulas.  Hydrolyzed or hypoallergenic formulas for allergies are not considered 100% amino acid based formulas necessary for inborn errors of metabolism.  Amino acid-based elemental formulas are considered not a covered benefit for all other diagnoses, including individuals with food allergies that will tolerate formulas that are not 100% amino acid-based.


Amino acid-based elemental formulas are considered not a covered benefit for patients diagnosed with an IgE mediated condition who are six years of age and older.


Medical Foods

Medical foods and formulas for individuals diagnosed with genetic (inherited) inborn errors of metabolism are considered medically necessary when prescribed and administered under the direction of a physician.


The purchase of food and formula that is not prescription and monitored by a physician is not considered medical food. This includes low protein/low phe groceries and over the counter formula.


Medical foods are not considered medically necessary for any other diagnosis.


Examples of an inherited metabolic disorder (inborn errors of metabolism) include, but are not limited to:

  • Phenylketonuria (PKU)
  • Maple syrup urine disease (MSUD)
  • Homocystinuria
  • Urea cycle disorders
  • Organic acidemias
  • Histidinemia
  • Tyrosinemia


Procedure Codes and Billing Guidelines: 

  • To report provider services, use appropriate CPT* codes, Modifiers, Alpha Numeric (HCPCS level 2) codes, Revenue codes, and/or diagnosis codes.
  • S9433  Medical food nutritionally complete, administered orally, providing 100% of nutritional intake
  • S9434  Modified solid food supplements for inborn errors of metabolism
  • S9435  Medical foods for inborn errors of metabolism


Selected References: 

  • Nowak-Wegrzyn A, Sampson HA, Wood RA, Sicherer SH. Food protein-induced enterocolitis syndrome caused by solid food proteins. Pediatrics. 2003 Apr;111(4 Pt 1):829-35.
  • Crittenden RG, Bennett LE. Cow's milk allergy: a complex disorder. J Am Coll Nutr. 2005 Dec;24(6 Suppl):582S-91S.
  • Kemp AS, Hill DJ, Allen KJ, Anderson K, Davidson GP et al. Guidelines for the use of infant formulas to treat cows milk protein allergy: an Australian consensus panel opinion. Med J Aust. 2008 Jan 21;188(2):109-12.
  • Vanderhoof JA. Hypoallergenicity and effects on growth and tolerance of a new amino acid-based formula with DHA and ARA. J Pediatr Gastroenterol Nutr. 2008 Nov;47 Suppl 2:S60-1.
  • Bone J, Claver a, Guallar I, Plaza AM. Allergic proctocolitis, food-induced enterocolitis: immune mechanisms, diagnosis and treatment. Allergol Immunopathol (Madr). 2009 Jan-Feb;37(1):36-42.
  • Benhamou AH, Schappi Tempia MG, Belie DC, Eigenmann PA. An overview of cow's milk allergy in children. Swiss Med Wkly. 2009 May 30;139(21-22):300-7.
  • Putnam PE. Evaluation of the child who has eosinophilic esophagitis. Immunol Allergy Clin North Am. 2009 Feb;29(1):1-10, vii.
  • Potter BK, Chakraborty P, Kronick JB, Wilson K, et al. Achieving the "triple aim" for inborn errors of metabolism: a review of challenges to outcomes research and presentation of a new practice-based evidence framework. Genet Med. 2012 Dec 6. [Epub ahead of print]
  • Camp KM, Lloyd-Puryear MA, Huntington KL. Nutritional treatment for inborn errors of metabolism: indications, regulations, and availability of medical foods and dietary supplements using phenylketonuria as an example. Mol Genet Metabl 2012 Sep;107(1-2):3-9. 
  • Dellon E., Gonsalves N., et al. ACG Clinical Guideline: Evidenced based approach to the diagnosis and management of esophageal eosinophilia and eosinophilic esophagitis. Am J Gastro. 2013; 108:679-692.


Policy History: 



Date                                        Reason                              Action

September 2011                     Annual review                    Policy renewed

September 2012                     Annual review                    Policy renewed

February 2013                        Interim review                    Policy revised

August 2013                           Annual review                   Policy renewed

February 2015                                                               Policy revised

May 2015                              Annual review                    Policy revised


Wellmark medical policies address the complex issue of technology assessment of new and emerging treatments, devices, drugs, etc.   They are developed to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. Wellmark medical policies contain only a partial, general description of plan or program benefits and do not constitute a contract. Wellmark does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Wellmark or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. Our medical policies may be updated and therefore are subject to change without notice.

*Current Procedural Terminology © 2012 American Medical Association. All Rights Reserved.

Contact Information
New information or technology that would be relevant for Wellmark to consider when this policy is next reviewed may be submitted to:
  Wellmark Blue Cross and Blue Shield
  Medical Policy Analyst
  P.O. Box 9232
  Des Moines, IA 50306-9232
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