Subject:
Ketogenic Diet
Description:
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IMPORTANT NOTE:
The purpose of this policy is to provide general information applicable to the administration of health benefits that Horizon Blue Cross Blue Shield of New Jersey and Horizon Healthcare of New Jersey, Inc. (collectively “Horizon BCBSNJ”) insures or administers. If the member’s contract benefits differ from the medical policy, the contract prevails. Although a service, supply or procedure may be medically necessary, it may be subject to limitations and/or exclusions under a member’s benefit plan. If a service, supply or procedure is not covered and the member proceeds to obtain the service, supply or procedure, the member may be responsible for the cost. Decisions regarding treatment and treatment plans are the responsibility of the physician. This policy is not intended to direct the course of clinical care a physician provides to a member, and it does not replace a physician’s independent professional clinical judgment or duty to exercise special knowledge and skill in the treatment of Horizon BCBSNJ members. Horizon BCBSNJ is not responsible for, does not provide, and does not hold itself out as a provider of medical care. The physician remains responsible for the quality and type of health care services provided to a Horizon BCBSNJ member.
Horizon BCBSNJ medical policies do not constitute medical advice, authorization, certification, approval, explanation of benefits, offer of coverage, contract or guarantee of payment.
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The ketogenic diet is a special diet used in treating seizures. It was initially studied in the 1920's as a treatment option for children with intractable seizures. Although dietary therapy became less popular with the advent of effective antiepileptic drugs (AEDs), the ketogenic diet continued to be used over the years for patients, especially in children whose seizures were refractory to standard pharmacologic therapy. Increasing concern about side effects of AEDs, particularly their effect on cognitive function have prompted physicians to evaluate alternative therapies. With recent research publications and media interest, there is now resurgence of interest in the ketogenic diet.
The diet is high in fat (ketogenic), and low in carbohydrate and protein (antiketogenic), which results in ketosis. This ketotic state exerts an anti-epileptic effect, though its exact mechanism of action remains poorly understood. In order to produce ketonemia and ketonuria, the ratio of ketone-producing foods to those tending to produce an antiketogenic effect must be 3 or greater, thus giving rise to the current nomenclature of a 3:1 or 4:1 ketogenic diet. Modifications of these "classic" diets have been devised in recent years, and include the medium-chain triglyceride (MCT) diet, substitution of corn oil for MCT oil, and a "modified" MCT diet consisting of a mixture of long- and medium chain triglycerides. The MCT diet appears to be more ketogenic and allows a slightly greater proportion of calories and food containing carbohydrates and protein, but it frequently causes gastrointestinal problems. Regardless of the modification, protein and calorie intake is set at levels that will meet requirements for growth. Most of the calories are provided as fat. No sugar is allowed. Vitamins and minerals are supplemented. The diet is very rigid and requires strict nutritional supervision.
Policy:
(NOTE: For Medicare Advantage, Medicaid and FIDE-SNP, please refer to the Coverage Sections below for coverage guidance.)
1. The ketogenic diet must be prescribed by the treating physician.
2. The ketogenic diet is considered medically necessary as an alternative treatment for children with seizures:
A. refractory to antiepileptic drug therapy; or
B. who have unacceptable antiepileptic drug toxicity.
(NOTE: The ketogenic diet seems to have the greatest beneficial effects in younger children, who produce and utilize ketones more rapidly and whose dietary intake can be more closely supervised. The diet is often difficult to begin and maintain in children under 1 year old, who are also more resistant to developing ketosis. In teenagers and adults, it is more difficult (though not impossible) to prepare a palatable diet with the appropriate ratio of fat, carbohydrate and protein necessary to produce the desired level of ketosis; thus, dietary compliance may become a limiting factor. Furthermore, there appears to be a window of optimum age-responsiveness to the ketogenic diet, which is probably associated with age-related differences in the ability of the brain to utilize ketones as fuel.
The ketogenic diet appears to be most effective for control of myoclonic seizures, infantile spasms, atonic/akinetic seizures, and "mixed" seizures of Lennox-Gastaut syndrome, followed by varying degrees of success in all other refractory seizure types (partial or focal, and generalized tonic-clonic seizures). The diet appears to be least effective in controlling temporal lobe and in absence or petit mal types of seizures.)
3. Because of the potential for serious complications, the ketogenic diet should be initiated in the hospital under medical supervision for 4 to 5 days.
(NOTE: Because young children could easily become hypoglycemic or severely ketonemic during fasting and diet initiation, they are observed in the hospital for 4-5 days. This time is also used to teach dietary calculation and planning to the family and to possibly begin tapering of antiepileptic drugs. Typically, the child is fasted in the hospital for 24-72 hours until 4+ ketonuria is produced. After ketosis is established, caloric intake is increased by thirds until a full 4:1 ketogenic diet is reached. Fluids are restricted to maintenance levels of 800-1,200 ml/day. All sugar-containing medications are avoided and sufficient fat-soluble vitamins and calcium supplements are added. After discharge, patients are followed-up every month for the first few months becoming less frequent as the family and child adjust to the diet.
Phone consultations may also be frequent at the onset of the diet.
In some cases, improvement in seizure control is immediately evident as soon as the child becomes ketotic, while it may take 1-2 weeks in other cases. When seizure control is not improved after 6 months, the ketogenic diet is most likely to be discontinued, according to one study. If the diet proves to be a worthwhile form of therapy, it is usually maintained for a 2-year period and weaned in the 3rd year, similar to what might be tried with antiepileptic drugs. Many children continue to go without seizures or other medications when the diet has been discontinued.
4. Use of a ketogenic diet for control of epilepsy in adults is investigational.
Medicare Coverage:
There is no National Coverage Determination (NCD). In the absence of an NCD, coverage decisions are left to the discretion of Local Medicare Carriers. Novitas Solutions, Inc, the Local Medicare Carrier for jurisdiction JL, has not issued a determination for this service. Therefore, Medicare Advantage Products will follow the Horizon BCBSNJ Medical Policy.
Medicaid Coverage:
For members enrolled in Medicaid and NJ FamilyCare plans, Horizon BCBSNJ applies the above medical policy
FIDE-SNP Coverage:
For members enrolled in a Fully Integrated Dual Eligible Special Needs Plan (FIDE-SNP): (1) to the extent the service is covered under the Medicare portion of the member’s benefit package, the above Medicare Coverage statement applies; and (2) to the extent the service is not covered under the Medicare portion of the member’s benefit package, the above Medicaid Coverage statement applies.
Note regarding the various diets used from UpToDate :
Diet selection — The four major KDTs for the treatment of epilepsy are the classic (long-chain) ketogenic diet, the medium-chain triglyceride diet, the modified Atkins diet, and the low glycemic index treatment. The choice of a particular diet is individualized and often at the discretion of the parent. Most centers agree, however, that infants and children under two years of age should use the classic ketogenic diet, whereas adolescents and adults may have improved compliance if the modified Atkins diet or low glycemic index treatment is chosen.
Composition
Classic ketogenic diet — In the classic ketogenic diet, fat comes primarily from long-chain triglycerides obtained through standard foods; protein intake is based on that required for growth; and carbohydrates are restricted. The classic ketogenic diet consists of four parts fat to one part protein and carbohydrate (ie, 4:1 lipid to nonlipid ratio) Fat provides 90 percent of the calories. At most centers, dietitians match calories to the pre-ketogenic diet baseline. In the past, total calories were restricted to 80 to 90 percent of recommended values for age, but this did not result in clear benefit Modification of the diet to a somewhat lower fat content (3:1 lipid to nonlipid ratio from the classic 4:1 ratio) improves tolerability of the diet, but may reduce its efficacy during the initial three months.
Alternatively, the diet can be given as a liquid-based formula to bottle-fed infants and enterally fed patients. There are a number of commercially available products. Because the diet is easy to administer in this form, compliance and efficacy are generally excellent in this population.
Medium-chain triglyceride diet — In an alternative form of the classic ketogenic diet, medium-chain triglycerides (MCTs) provided in an oil supplement are utilized as a major fat source. These MCTs yield more ketones, are more efficiently absorbed, and are carried directly to the liver. As a result, less total fat is needed in the diet and more protein and carbohydrates can be allowed. The MCT diet is widely used in England, Canada, and other countries because the highly ketogenic nature of MCT oils allows for additional carbohydrates. Many centers will also add MCT oils to the classic ketogenic diet as a supplement.
The traditional MCT diet derived 60 percent of the energy from MCT . In some children, this needs to be reduced to 30 to 50 percent in order to minimize gastrointestinal symptoms, with the resulting decrement in energy source to be made up from long-chain fats.
MCTs should be given with each meal or evenly divided up between several smaller meals in order to improve tolerance.
Modified Atkins diet — The modified Atkins diet is an alternative KDT designed to mimic some aspects of the classic ketogenic diet but allow more ad lib protein, fluids, and calories.
The diet is initiated as an outpatient without a fast. Carbohydrates are initially restricted to 10 grams per day (15 to 20 grams per day in adolescents and adults) with patients counseled to increase their use of high fat foods (at the expense of protein). When implemented, most parents will provide an approximately 1:1 to 2:1 lipid to nonlipid ratio (although there is no weighing and measuring of foods recommended in this regimen). A ketogenic liquid supplement (KetoCal shake) can be used during the first month to increase the ketogenic ratio. All children are started on a multivitamin and calcium, and lab studies are obtained identically to the classic ketogenic diet. After one to three months, the carbohydrate restrictions can be loosened and/or antiseizure drugs reduced as clinically indicated.
This less restrictive diet has been used successfully in adults who are not typically offered the classic ketogenic diet , in countries with limited financial and dietitian resources, in children on the classic ketogenic diet for many years who desire fewer restrictions, and as an early treatment for epilepsy before intractability is established. One center reported that the modified Atkins diet could be successfully maintained in selected adults using electronic communication (email) rather than in-person clinic visits. This may be particularly useful in resource-poor regions of the world.
Ketosis will occur with the modified Atkins diet and may correlate with seizure control, but only initially, during the first month.
Low glycemic index treatment — An even less restrictive KDT, the low glycemic index diet restricts carbohydrates to 40 to 60 grams per day, does not restrict fluids or protein, and loosely monitors fat and calories. It is started as an outpatient without a fasting period. As with the other KDTs, most children eat significantly more fat on this regimen than they did before. Unlike the modified Atkins diet, the type of carbohydrates is important in the low glycemic index diet, with only carbohydrates with low glycemic indices (<50) allowed. These carbohydrates include strawberries and whole grain breads as opposed to potatoes, white bread, and most citrus fruits. In patients on this diet, there is nearly no serum ketosis noted.
Studies using ketogenic diets in adults for epilepsy control are either small or case studies. There are insufficient peer reviewed studies showing efficacy in adults.)
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Horizon BCBSNJ Medical Policy Development Process:
This Horizon BCBSNJ Medical Policy (the “Medical Policy”) has been developed by Horizon BCBSNJ’s Medical Policy Committee (the “Committee”) consistent with generally accepted standards of medical practice, and reflects Horizon BCBSNJ’s view of the subject health care services, supplies or procedures, and in what circumstances they are deemed to be medically necessary or experimental/ investigational in nature. This Medical Policy also considers whether and to what degree the subject health care services, supplies or procedures are clinically appropriate, in terms of type, frequency, extent, site and duration and if they are considered effective for the illnesses, injuries or diseases discussed. Where relevant, this Medical Policy considers whether the subject health care services, supplies or procedures are being requested primarily for the convenience of the covered person or the health care provider. It may also consider whether the services, supplies or procedures are more costly than an alternative service or sequence of services, supplies or procedures that are at least as likely to produce equivalent therapeutic or diagnostic results as to the diagnosis or treatment of the relevant illness, injury or disease. In reaching its conclusion regarding what it considers to be the generally accepted standards of medical practice, the Committee reviews and considers the following: all credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, physician and health care provider specialty society recommendations, the views of physicians and health care providers practicing in relevant clinical areas (including, but not limited to, the prevailing opinion within the appropriate specialty) and any other relevant factor as determined by applicable State and Federal laws and regulations.
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Index:
Ketogenic Diet
Diet, Ketogenic
Seizure Control Diet
References:
1. Lefevre F, Aronson N. Ketogenic Diet for the Treatment of Refractory Epilepsy in Children: A Systematic Review of Efficacy. Pediatrics. April 2000;105(4):E46.
2. Prasad AN, Stafstrom CF, Holmes GL. Alternative Epilepsy Therapies: The Ketogenic Diet, Immunoglobulins, and Steroids. Epilepsia. 1996;37(Suppl I):S81-S95.
3. Kinsman SL, Vining EP, et al. Efficacy of the Ketogenic Diet for Intractable Seizure Disorders: Review of 58 Cases. Epilepsia. February 1992;33(6):1132-36.
4. The Johns Hopkins Ketogenic Factsheet.
5. Ketogenic Diet, Packard Children's Hospital, Stanford University Medical Center on the Internet @ http://www-leland.stanford.edu/group/ketodiet.
6. ECRI. TARGET Report #202: Ketogenic diets for prevention of epileptic seizures in children. Content current as of: February 1999. (current as of 08/31/07)
7. Nordli DR Jr, Kuroda MM, Carroll J et al. Experience with the ketogenic diet in infants. Pediatrics. 2001 Jul;108(1):129-33.
8. Hemingway C, Freeman JM, Pillas DJ, Pyzik PL. The ketogenic diet: a 3- to 6-year follow-up of 150 children enrolled prospectively. Pediatrics. 2001 Oct;108(4):898-905.
9. Maydell BV, Wyllie E, Akhtar N et al. Efficacy of the ketogenic diet in focal versus generalized seizures. Pediatr Neurol. 2001 Sep;25(3):208-12.
10. Lightstone L, Shinnar S, Callahan CM et al. Reasons for failure of the ketogenic diet. J Neurosci Nurs. 2001 Dec;33(6):292-5.
11. Mandel A, Ballew M, Pina-Garza et al. Medical costs are reduced when children with intractable epilepsy are successfully treated with the ketogenic diet. J Am Diet Assoc. 2002 Mar;102(3):396-8.
12. Kossoff EH. More fat and fewer seizures: dietary therapies for epilepsy. Lancet Neurol. 2004 Jul;3(7):415-20.
13. Vaisleib II, Buchhalter JR, Zupanc ML. Ketogenic diet: Outpatient initiation, without fluid, or caloric restrictions. Pediatr Neurol. 2004 Sep;31(3):198-202.
14. Kang HC, Chung da E, Kim DW, Kim HD. Early- and late-onset complications of the ketogenic diet for intractable epilepsy. Epilepsia. 2004 Sep;45(9):1116-23.
15. Vaisleib II, Buchhalter JR, Zupanc ML. Ketogenic diet: outpatient initiation, without fluid, or caloric restrictions. Pediatr Neurol. 2004 Sep;31(3):198-202.
16. Gasior M, Rogawski MA, Hartman AL. Neuroprotective and disease-modifying efects of the ketogenic diet. Behav Pharmacol 2006 Sep;17(5-6):431-9.
17. Henderson CB, Filloux FM, Alder SC, et al. Efficacy of the ketogenic diet as a treatment option for epilepsy: meta-analysis. J Child Neurol 2006 Mar;21(3):193-8.
18. Huffman J, Kossoff EH. State of the ketogenic diet(s) in epilepsy. Curr Neurol Neurosci Rep 2006 Jul;6(4):332-40.
19. Keene DL. A systematic review of the use of the ketogenic diet in childhood epilepsy. Pediatr Neurol 2006 Jul;35(1):1-5.
20. Levy RG, Cooper PN, Giri P. Ketogenic diet and other dietary treatments for epilepsy. Cochrane Database Syst Rev. 2012, (3):CD001903.
21. National Institute for Health and Clinical Excellence. Clinical guidelines CG137. The epilepsies: the diagnosis and management of the epilepsies in adults and children in primary and secondary care. January 2012. Available at: http://publications.nice.org.uk/the-epilepsies-the-diagnosis-and-management-of-the-epilepsies-in-adults-and-children-in-primary-and-cg137.
22. Burakgazi E, French JA. Treatment of epilepsy in adults. Epileptic Disord 2016 Jul. [Epub ahead of print]
23. Appavu B, Vanatta L, Condie J, et al. Ketogenic diet treatment for pediatric super-refractory status epilepticus. Seizure 2016 Jul 21;41:62-65.
24. UpToDate. The ketogenic diet and other dietary therapies for the treatment of epilepsy. Literature review current through June 2016. Topic last updated May 4, 2016.
25. UpToDate. Seizures and epilepsy in children: Refractory seizures and prognosis. Literature review current through June 2016. Topic last updated March 14, 2016.
26. Wilfong A. Seizures and epilepsy in children: Refractory seizures and prognosis. In: UpToDate, Nordli DR, Eichler AF (Eds), UpToDate, Waltham, MA. (Accessed on June 9, 2017.)
27. Paleologou E, Ismayilova N, Kinali M. Use of the Ketogenic Diet to Treat Intractable Epilepsy in Mitochondrial Disorders. Journal of Clinical Medicine. 2017; 6(6):56.
28. Kossoff EHW. The ketogenic diet and other dietary therapies for the treatment of epilepsy. In: UpToDate, Dashe JF (Eds), UpToDate, Waltham, MA. (Accessed on May 2, 2018.)
29. Kossoff EHW. The ketogenic diet and other dietary therapies for the treatment of epilepsy. In: UpToDate, Nordi DR, Dashe JF (Eds), UpToDate, Waltham, MA. (Accessed on May 2, 2019.)
30. Kossoff EHW. Ketogenic dietary therapies for the treatment of epilepsy. In: UpToDate, Nordi DR, Dashe JF (Eds), UpToDate, Waltham, MA. (Accessed on October 7, 2019.)
31. Kossoff EHW. Ketogenic dietary therapies for the treatment of epilepsy. In: UpToDate, Nordi DR, Dashe JF (Eds), UpToDate, Waltham, MA. (Accessed on August 19, 2020.)
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