Nutritional Treatment of Inborn Errors of Metabolism
DESCRIPTION
Nutritional treatment of inborn errors of metabolism refers to genetic disorders in which the body is unable to normally metabolize certain foods. If these disorders are left untreated, the individual (most always an infant or young child) will sustain severe health complications or die. Only a qualified physician and a clinical nutritionist familiar with inborn errors of metabolism should monitor this treatment process. This nutritional treatment may include: 1) specially formulated products based on appropriate medical evaluation, 2) nutritional prescription products that can only be obtained from specialized vendors, & 3) pharmacies.
POLICY
Nutritional treatment for inborn errors of metabolism is considered medically necessary if the medical appropriateness criteria are met. (See Medical Appropriateness criteria below.)
MEDICAL APPROPRIATENESS
Nutritional treatment for inborn errors of metabolism is considered medically appropriate when ALL of the following criteria are met:
Nutritional treatment is prescribed by a physician with a clinical nutritionist's determination of the individual's caloric/nutritional requirements (note: caloric/nutritional requirements should be specific for the individual being treated)
Appropriate diagnosis is confirmed by testing. Examples of inborn errors of metabolism may include but are not limited to the following:
Phenylketonuria (PKU)
Maple Syrup Urine Disease
Homocystinuria
Methylmalonic acidemia
Propionic Acidemia
Isovaleric Acidemia
Disorders of Leucine Metabolism
Glutaric Acidemia Type I
Tyrosinemia Types I and II
Urea Cycle Disorders
The individual will sustain severe health complications or not survive without this nutritional treatment
IMPORTANT REMINDER
We develop Medical Policies to provide guidance to Members and Providers. This Medical Policy relates only to the services or supplies described in it. The existence of a Medical Policy is not an authorization, certification, explanation of benefits or a contract for the service (or supply) that is referenced in the Medical Policy. For a determination of the benefits that a Member is entitled to receive under his or her health plan, the Member's health plan must be reviewed. If there is a conflict between the Medical Policy and a health plan, the express terms of the health plan will govern.
SOURCES
Agency for Healthcare Research and Quality. (2008 March) Screening for Phenylketonuria. Reaffirmation Recommendation Statement. Retrieved May 6, 2009 from http://www.ahrq.gov.
Bijarnia, S., Wiley, V., Carpenter, K., Christodoulou, J., Ellaway, C., & Wilcken, B. (2008) Glutaric aciduria type I: outcome following detection by newborn screening. Journal of Inherited Metabolic Diseases, 31 (4), 503 - 507. (Level 5 Evidence)
Hayes. Medical Technology Directory. (2007 September). Tandem mass spectrometry for metabolic disease screening of newborns. Retrieved April 7, 2009 from http://www.hayesinc.com/hayes/. (76 articles and/ or guidelines reviewed)
Kamboj, M. (2008) Clinical approach to the diagnoses of inborn errors of metabolism. Pediatric Clinics of North America, 55 (5), 1113 - 1127. (Level 5 Evidence)
Levy, P. (2009).Inborn errors of metabolism: part 1: overview. Pediatric Review, 30 (4), 131 - 138. (Level 5 Evidence)
Rezvani, I. (2004). Tyrosine. In Behrman (Ed.). Nelson textbook of pediatrics (17th ed., pp. 402 - 405). Philadelphia: W. B. Saunders Company.
Rutherford P, Poustie VJ. Protein substitute for children and adults with phenylketonuria. The Cochrane Database of Systematic Reviews 2005, Issue 4. Art. No.: CD004731.pub2. DOI: 10.1002/14651858.CD004731.pub2. (Level 5 Evidence)
U. S. Food and Drug Administration. Center for Food Safety and Applied Nutrition. Food Compliance Program. (2006). Medical foods - import and domestic. Retrieved May 4, 2009 from http://www.cfsan.fda.gov/~comm/cp21002.html.
ORIGINAL EFFECTIVE DATE: 5/1/2000
MOST RECENT REVIEW DATE: 6/11/2009