AMERICAN ACADEMY OF PEDIATRICS POLICY STATEMENT Organizational Principles to Guide and Define the Child Health Care System and/or Improve the Health of All Children Committee on Nutrition Reimbursement for Foods for Special Dietary Use ABSTRACT. Foods for special dietary use are recom- DEFINITION OF FOODS FOR SPECIAL mended by physicians for chronic diseases or conditions DIETARY USE of childhood, including inherited metabolic diseases. Al- The US Food and Drug Administration, in the though many states have created legislation requiring Code of Federal Regulations,2 defines special dietary reimbursement for foods for special dietary use, legisla- use of foods as the following: tion is now needed to mandate consistent coverage and reimbursement for foods for special dietary use and re- a. Uses for supplying particular dietary needs that lated support services with accepted medical benefit for exist by reason of a physical, physiologic, patho- children with designated medical conditions. logic, or other condition, including but not limited to the conditions of diseases, convalescence, preg- ABBREVIATION. AAP, American Academy of Pediatrics. nancy, lactation, allergic hypersensitivity to food, [and being] underweight and overweight; b. Uses for supplying particular dietary needs which BACKGROUND exist by reason of age, including but not limited to pecial foods are recommended by physicians to the ages of infancy and childhood; foster normal growth and development in some c. Uses for supplementing or fortifying the ordinary or usual diet with any vitamin, mineral, or other children and to prevent serious disability and S dietary property. Any such particular use of a even death in others. Many of these special foods are food is a special dietary use, regardless of whether technically specialized formulas for which there may such food also purports to be or is represented for be a relatively small market, which makes them more general use. expensive than standard formula. Since publication of the American Academy of Pediatrics (AAP) policy MEDICAL CONDITIONS COVERED 1 statement in 1994, many states have created legisla- Diseases covered would include all chronic dis- tion for mandating reimbursement for foods for chil- eases or conditions of childhood requiring special dren with inborn errors of metabolism. Third-party dietary intervention, including inherited metabolic payment for foods for special dietary use is inconsis- diseases (Table 1). tent, however, and state statutes regarding reim- Many childhood chronic diseases (eg, inflamma- bursement vary widely. Some states require cover- tory bowel disease, cystic fibrosis, celiac disease, can- age only for inherited metabolic diseases, such as cer, congenital heart disease, renal failure, hepatic phenylketonuria, and others include a range of met- diseases) are associated with increased nutritional abolic conditions. Legislation is now needed to man- requirements and metabolic demands or with de- date consistent coverage and reimbursement for all creased nutrient intakes, limitations of digestion and aspects of foods for special dietary use and related absorption, and/or increased nutrient losses. An op- supplies and services for children with designated timal state of nutrition is especially important in medical conditions. these children, and poor nutrition is associated with There is a great need for pediatricians and the AAP increased risk of infections, inadequate growth, and poor response to treatment modalities, including sur- to take a leadership role in this area of child health 3,4 affecting infants, children, and adolescents by build- gery. The goals of nutritional support for children ing on the legislative advancements that have been with these chronic illnesses include normal growth and development, promotion of catch-up growth, made as a result of the 1994 policy.1 A model bill and improved clinical outcome. Specialized nutri- (Pediatric Medical Nutrition Support Act) for pro- tional support is often required, including enteral posed legislation is available on the AAP Web site tube feedings or parenteral nutrition. (http://www.aap.org/policy/m972.html). Special nutritional supplements and feeding ap- proaches often are required for infants and children with devastating neurologic diseases or impair- PEDIATRICS (ISSN 0031 4005). Copyright © 2003 by the American Acad- ments, such as severe cerebral palsy, and progressive emy of Pediatrics. neurodevelopmental diseases.5 Downloaded from www.aappublications.org/news by guestPEDIATRICS on September 28, Vol. 2021 111 No. 5 May 2003 1117 TABLE 1. Medical Conditions for Which Foods for Special TABLE 1. Continued. Dietary Use May Be Reimbursed* Metabolic disorders Conditions requiring specific dietary components Disorders of carbohydrate metabolism Chronic pulmonary insufficiency Glycogen storage disease Congenital or acquired chronic cardiac insufficiency Glucose transport protein deficiency Movement disorder (neuromuscular) Galactosemia Catch-up growth in children attributable to undernutrition Hereditary fructose intolerance Rett syndrome Pyruvate dehydrogenase complex deficiency Inflammatory bowel disease Phosphoenolpyruvate carboxykinase deficiency Conditions requiring the alteration of specific dietary Disorders of lipid metabolism components Fasting chylomicronemia Short bowel syndrome (long-segment Hirschsprung disease, Mitochondrial fatty acid oxidation defects (eg, ␣- small intestinal atresia, necrotizing enterocolitis, volvulus, ketoadipicaciduria, methylmalonicadipicaciduria, long-chain gastroschisis) acyl-CoA dehydrogenase deficiency, and medium-chain Intestinal pseudo-obstruction syndromes acyl-CoA dehydrogenase deficiency) Nonspecific malabsorption syndromes (eg, postviral ␤-ketothiolase deficiency gastroenteropathy, small intestinal bacterial overgrowth) Succinyl-CoA ketoacid transferase Intestinal lymphangiectasia Other organic acidemias Abetalipoproteinemia Disorders of vitamin metabolism Microvillus inclusion disease Biotinidase deficiency Eosinophilic gastroenteropathy Holocarboxylase synthetase deficiency Partial villus atrophy attributable to food protein sensitivity Methylmalonicacidemia Intestinal transplantation Methylcrotonyl-CoA carboxylase deficiency Exocrine pancreatic insufficiency (eg, cystic fibrosis, Thiamine-responsive maple syrup urine disease Schwachman syndrome, Johanson-Blizzard syndrome, Pyridoxine responsive seizures Pearson syndrome, pancreatic hypoplasia, congenital Disorders of mineral metabolism trypsinogen deficiency) Hypercalcemia Chronic liver disease with cholestasis Williams syndrome Impaired bile acid synthesis Disorders of amino acid or nitrogen metabolism Bile duct atresia Phenylketonuria Interrupted enterohepatic circulation (eg, ileal resection, Cystinosis congenital malabsorption of bile acids, blind loop Homocystinuria syndrome) Glutaric acidemia (types I and II) Enterokinase deficiency Disorders of branched-chain amino acid metabolism (eg, Immunodeficiency states (eg, villus atrophy associated disorders of leucine metabolism, isovaleric acidemia, maple with immunodeficiency conditions, autoimmune syrup urine disease, 3-hydroxy-3-methylglutaricaciduria, enteropathy, or enterocolitis associated with immune 3-methylcrotonylglycinemia, 3-methylglutaconicaciduria) deficiency) Tyrosinemia (types I and II) Chylothorax Lysinuric protein intolerance Conditions impairing adequate oral intake Urea cycle defects (eg, ornithine transcarbamylase deficiency, Cystic fibrosis hyperornithine-hyperammonemia-homocitrullinuria Acquired immunodeficiency syndrome syndrome, argininemia, argininosuccinicaciduria, carbamyl Transplant patients: bone marrow, liver, kidney, heart, lung, phosphate synthetase deficiency, citrullinemia) or multiple organs Methylmalonicacidemia Cerebral palsy Propionicacidemia Aspiration syndrome Gyrate atrophy of the choroid and retina Oral dysfunction Adrenoleukodystrophy Pharyngeal dysfunction Miscellaneous mitochondrial disorders and mitochondrial Esophageal dysfunction electron transport defects Neurologic disorders Oral feeding aversion (resulting from chronic use of enteral * List is not intended to be all inclusive. tube feedings or parenteral nutrition) CoA indicates coenzyme A. Pancreatitis Treatment of childhood cancer Chronic renal failure, hemodialysis, and peritoneal dialysis Anatomic, congenital, or postsurgical defects precluding oral cial barriers to acquiring modified low-protein foods nutritional intake (eg, craniofacial defects) and outlined support services required to maintain appropriate phenylalanine concentrations.7 Children with other metabolic disorders, including those of Metabolic diseases include inborn errors of amino carbohydrate metabolism, lipid metabolism, vitamin acid metabolism, such as phenylketonuria, maternal or cofactor metabolism, or mineral metabolism, may phenylketonuria, maple syrup urine disease, homo- also benefit from dietary interventions. cystinuria, methylmalonicacidemia, propionicaci- demia, isovalericacidemia, and other disorders of DEFINITION OF REIMBURSABLE EXPENSES leucine metabolism; glutaricaciduria type I and ty- Any health insurance policy that is delivered, is- rosinemia types I and II; and urea cycle disorders. sued for delivery, renewed, extended, or modified in These are all disorders treatable by dietary modifi- a particular state by any health care insurer and that cations, which can prevent complications like severe provides coverage for a child should optimally pro- mental retardation and death.6