Medical Policy: 10.01.15
Original Effective Date: May 2010
Reviewed: April 2016
Revised: April 2016
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.
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.
In 1994, the American Academy of Pediatrics Committee on Nutrition issued its recommendations on reimbursement for medical foods for inborn errors of metabolism. In-born errors of metabolism are a group of rare disorders resulting in the excessive accumulation of an amino acid or other product along the metabolic pathway for lack of a natural enzyme required to digest certain foods. Manifestations of these disorders generally include central nervous system dysfunction, developmental delay, seizures and liver dysfunction.
The clinical manifestations in many of these disorders can be prevented if diagnosis is achieved early and necessary treatment with dietary protein or amino acid restriction is instituted immediately. These disorders are named for the accumulating amino acid and include, but are not limited to, phenylketonuria (PKU), maple syrup urine disease, citrullinemia, cystinosis, homocystinuria, methylmalonic acidemia, propionic acidemia, isovaleric acidemia acidemia type I, tyrosinemia types I and II, and urea cycle disorders.
Treatment might include restriction of specific amino acids, restriction of total nitrogen intake, or supplementation of certain substances. For some of the inborn errors of metabolism, special formulas and medical foods have been developed which eliminate the amino acid that cannot be metabolized from the protein context of the food. As adults, they must avoid certain foods as well. Women with classic PKU desiring pregnancy need to alter their diet by using a special maternal dietary supplement low in phenylalanine. The use of this supplement reduces the risk of severe retardation in the infant of a mother with PKU.
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. There are three major categories of IEMs: protein disorders, fatty acid oxidation disorders, and carbohydrate disorders. 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. Inborne errors of metabolism are considered present at birth and permanent.
Examples of an inherited metabolic disorder (inborn errors of metabolism) include, but are not limited to:
- Phenylketonuria (PKU)
- Maple syrup urine disease (MSUD)
- Urea cycle disorders
- Organic acidemias
Individuals with inborn errors of metabolism require "exempt" specialized metabolic infant formulas. The FDA has requirements for the minimum and maximum amount of nutrients in infant formulas; if these requirements are not met, the infant formula may be "exempt" from these nutrient requirements. "An exempt infant formula is any infant formula which is represented and labeled for use by an infant who has an inborn error of metabolism (IEM), or low birth weight, or who otherwise has an unusual medical or dietary problem." Exempt amino acid-based metabolic formulas require a prescription. Examples include:
- OA 1 or 2
- PFD 2 or Toddler
- Phenyl-Free BCAD 1 or 2.
Food allergies are not considered an inborne error of metabolism.
- The product must be a medical food for oral feeding; and
- The product must be labeled and used for the dietary management of a specific medical disorder, disease, or condition for which there are distinctive nutritional requirements to avert the development of serious physical or mental disabilities or to promote normal development or function. The condition must be present at birth and considered a permanent condition.
Conditions associated with an inborn error of metabolism interfere with the metabolism of specific nutrients. Examples of an inherited metabolic disorder (inborn errors of metabolism) include, but are not limited to:
- Phenylketonuria (PKU)
- Maple syrup urine disease (MSUD)
- Urea cycle disorders
- Organic acidemias
The purchase of food and formula that is not prescription and food and formula that is not monitored by a physician is not considered a medical food. This includes low protein/low phe groceries and over the counter formula. The coverage of over the counter food are not considered a covered benefit.
Oral enteral nutrition is considered not a covered benefit when the criteria above have not been met.
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
- 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.
- American Academy of Pediatrics Committee on Nutrition. Reimbursement for medical foods for inborn errors of metabolism. Pediatrics. 1994;93(5):860. Available at: http://pediatrics.aappublications.org/content/93/5/860.full.pdf+html?sid=a217ddcd-3dc9-4300-8ad3-9b6ee8572792
- National Institutes of Health, Consensus Development Panel. Phenylketonuria: screening and management. Pediatrics. 2001;108:972–982. Available at: http://consensus.nih.gov/2000/2000phenylketonuria113html.htm
- National Institutes of Health. National Library of Medicine. Medical Encyclopedia. Inborn errors of metabolism. Jun 2013. Available at: http://www.nlm.nih.gov/medlineplus/ency/article/002438.htm
- U.S. Food and Drug Administration (FDA). Center for Food Safety and Applied Nutrition. Office of Nutritional Products, Labeling, and Dietary Supplements. Draft Guidance for Industry: Frequently Asked Questions about Medical Foods. Available at: http://www.fda.gov/Food/GuidanceRegulation/GuidanceDocumentsRegulatoryInformation/MedicalFoods/ucm054048.htm.
- National Institute for Health and Clinical Excellence (NICE). Quality Standard for Nutrition Support in Adults. NICE quality standards [QS24]. November 2012. Available at: http://www.nice.org.uk/guidance/QS24/chapter/introduction-and-overview.
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
June 2014 Interim Review Policy revised
June 2014 Annual Review Policy renewed
May 2015 Annual Review Policy revised
April 2016 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.