DESCRIPTION
The ketogenic diet is a high fat, low carbohydrate, low protein diet. This is a very restrictive diet, requiring strict maintenance of the fat to carbohydrate ratio. The precise contents of each food item must be known and exactly measured. It is intended to control the occurrence of seizures by inducing a state of ketosis. The diet is used for children with refractory epilepsy. Refractory epilepsy occurs when seizures are inadequately controlled despite optimal treatment with conventional medications.
Initiation of this diet may be done as an outpatient, with one to two days of fasting required. This necessitates monitoring for dehydration and metabolic derangements, such as hypocalcemia and acidosis. The diet is introduced gradually over a number of days with the full diet attained by approximately day five.
POLICY
Ketogenic diet counseling for the treatment of children with refractory seizures is considered medically necessary if the medical appropriateness criteria are met. (See Medical Appropriateness below.)
Ketogenic diet counseling for the treatment of other conditions / diseases, including, but not limited to the following: partial seizures, refractory seizure control in adults, metabolic, hepatic, cerebrovascular, renal or degenerative disease is considered investigational.
MEDICAL APPROPRIATENESS
Ketogenic diet counseling for the treatment of children with refractory seizures is considered medically appropriate if ALL of the following criteria are met:
The child is unresponsive to conventional anti-epileptic drug (AED) treatment
The neurologist has documented unacceptable AED toxicity and adverse reactions
The neurologist has documented a supportive and involved family environment
The neurologist has documented the child and parent have high motivation to adhere to the diet
The neurologist has documented that parents are able to understand and implement the diet and health care needs related to the diet
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.
ADDITIONAL INFORMATION
Ketogenic diet counseling should be used with caution in children with, but not limited to, any of the following comorbid conditions:
Diabetes
Cerebrovascular disease
Kidney stones
Gallbladder stones
Gastrointestinal disease
Heart disease
Lipid disease
Urolithiasis
Nutritional deficiency
Cystic fibrosis
There is a lack of evidence in the published literature to validate the use of the ketogenic diet for other conditions/diseases.
SOURCES
Agency for Healthcare Research and Quality. (2003, April). Evidence Report/Technology Assessment, Number 77. Management of treatment-resistant epilepsy. Retrieved May 27, 2009 from http://www.ahrq.gov/clinic/epcsums/epilsum.htm.
American Epilepsy Society. (2004, November). Dietary approaches to epilepsy treatment: Old and new options on the menu. Retrieved May 16, 2006 from PubMed database.
BlueCross BlueShield Association. Medical Policy Reference Manual. (1:2005). Ketogenic diet as a treatment of refractory epilepsy (2.01.32). Retrieved May 27, 2009 from BlueWeb.
Klepper, J., Scheffer, H., Leiendecker, B., Gertsen, E., Binder, S., Leferink, M., et al. (2005). Seizure control and acceptance of the ketogenic diet in GLUT1 deficiency syndrome: a 2- to 5-year follow-up of 15 children enrolled prospectively. Neuropediatrics, 36-(5), 302-308. Abstract retrieved May 16, 2006 from PubMed database.
Kossoff, E. H., McGrogan, J. R., Bluml, R. M., Pillas, D. J., Rubenstein, J. E., & Vining, E. P. (2006). A modified atkins diet is effective for the treatment of intractable pediatric epilepsy. Epilepsia, 47- (2), 421-424. Abstract retrieved May 16, 2006 from PubMed database.
Kossoff, E. H., Pyzik, P. L., McGrogan, J. R., Vining, P. G., & Freeman, J. M. (2002). Efficacy of the ketogenic diet for infantile spasms. Pediatrics, 109 (5), 780-783.
Marsh, E. B., Freeman, J. M., Kossoff, E. H., Vining, E. P., Rubenstein, J. E., Pyzik, P. L., et al. (2006). The outcome of children with intractable seizures: a 3- to 6- year follow-up of 67 children who remained on the ketogenic diet less than one year. Epilepsia, 47 (2), 425-430. Abstract retrieved May 16, 2006 from PubMed database.
National Guideline Clearinghouse. American Academy of Neurology and Child Neurology Society. (2004, May). Medical treatment of infantile spasms. Retrieved May 16, 2005 from http://www.guidelines.gov.
National Institute of Neurological Disorders and Stroke. (2009, May). Seizure and epilepsy: Hope through research. Retrieved May 27, 2009.from http://www.ninds.nih.gov/disorders/epilepsy/detail_epilepsy.htm.
ORIGINAL EFFECTIVE DATE: 4/1/2002
MOST RECENT REVIEW DATE: 7/9/2009
Policies included in the Medical Policy Manual are not intended to certify coverage availability. They are medical determinations about a particular technology, service, drug, etc. While a policy or technology may be medically necessary, it could be excluded in a member's benefit plan. Please check with the appropriate claims department to determine if the service in question is a covered service under a particular benefit plan. Use of the Medical Policy Manual is not intended to replace independent medical judgment for treatment of individuals. The content on this Web site is not intended to be a substitute for professional medical advice in any way. Always seek the advice of your physician or other qualified health care provider if you have questions regarding a medical condition or treatment.
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