Refractory epilepsy can try a ketogenic diet

  What does a ketogenic diet mean?
  A ketogenic diet is a high-fat, low-carbohydrate, moderate-protein diet in which fat is broken down and metabolized in the body to produce ketone bodies to control seizures, called KD therapy. The therapy has biblical origins and has been used clinically since 1920, and has been in use for over 90 years.
  What are the main components of the ketogenic diet? How effective is it?   Several studies suggest that KD therapy has good efficacy: half of the seizures are reduced by >50%; seizures can be reduced by more than 90% in 30% of the children, and in 20% of them the seizures can be completely controlled, and the efficacy is maintained after stopping KD therapy in 79% of the cases; the cognitive function of many children is improved and enhanced after seizure reduction.
  What kind of patients with refractory epilepsy is the ketogenic diet suitable for?
  Children with epilepsy who have generally failed on 2 or 3 anticonvulsants may be considered for KD, regardless of age or gender, especially in those with symptomatic generalized seizures. The KD is the treatment of choice for 2 characteristic brain substitution ten disorders such as glucose carrier protein l (GLUT-1) deficiency and pyruvate dehydrogenase deficiency (PDHD). Certain epileptic encephalopathies or specific etiologies such as Dravet syndrome, infantile spasms, myoclonic-dystonic epilepsy and tuberous sclerosis, KD should be considered much earlier in patients.
  What is the difference between the ketogenic diet and the modified Atkins diet?
  The modified Atkins diet is a dietary therapy with a lower fat percentage similar to the ketogenic diet that has only been developed in recent years. It has similar efficacy to the ketogenic diet, but is more easily tolerated, making it a new treatment for epilepsy that is easier to implement.                                                                   Does the ketogenic diet require hospitalization?   Hospitalization for observation for 1 to 2 weeks during the initiation period is generally required, while the less restrictive modified Atkins diet and LGIT can be considered for outpatient implementation. The ratio of the starter diet is 2:1 to 4:1, and the transition period from the regular diet to KD is 1 to 2 weeks. The ratio of the diet can be adjusted according to the needs of the disease.
  What is the process of making a ketogenic diet?
  Patient expectations need to be discussed before initiation of the ketogenic diet, including expectations for seizure reduction, medication, and cognitive function; identification of possible psychological barriers for patients in adopting KD implementation: e.g., fear of difficulty in implementation, fear of adverse effects, etc.; knowledge of the sugar content of the antiepileptic drugs (AEDs) and other commonly used medications used; and recommended parental learning about KD. Assess baseline data on the child’s height and body mass, as well as ideal height and body mass. Dietary history: 3-d food history, preferences, poor eating habits, food fears and intolerances Determine the form and manner of dietary formulation: transoral or intestinal, or mixed, or partially parenteral. Decide which diet to start (medium chain triacylglycerol diet, classical KD, modified KD, LGIT) Calculate calories, fluid volume and KD ratios to supplement with appropriate nutrients according to the absorption of the diet. The preparation process should be strictly based on the calculated nutrient ratios. The dietitian designs the meal plan and milk quantity according to the child’s eating habits and weight calculation. The intake of other diets is stopped after the ketogenic diet treatment.
  How often should the recipes be adjusted?
  In the 1st year of starting KD, it is important to review at least every 3 months at the clinic to adjust the recipes to take the recommendations according to the child’s condition, which is important for the success of the treatment of children undergoing KD. For infants and children at high risk of nutritional deficiencies, more frequent outpatient review is needed.
  Can I borrow recipes directly from others?
  Every recipe is individualized, as children have different nutritional status, body tolerance levels, and eating habits, so you cannot imitate someone else’s recipe step by step.
  What foods should I strictly follow and not let go of?
  During the ketogenic diet, the family and the child should strictly follow the recipes, especially limiting the intake of sugary substances; even some medications can cause changes in the ketone bodies of the child because of the additives containing sugar.
  Does it matter if I eat an extra strawberry or an extra lychee once in a while?
  Try not to take food outside the recipe at will, which can easily affect the stability of the ketone body and even aggravate the seizure in serious cases.
  How long can the ketogenic diet be taken to indicate its effectiveness or not?
  Discontinuation of the diet should be considered after 3 months of initiating KD for epilepsy if it fails; if it is completely successful in controlling seizures, it can usually be considered after 2 years depending on the clinical situation.
  What indicates that the ketogenic diet is effective?
  A >50% reduction in seizures is generally considered effective.
  Is a ketogenic diet shown to be effective, a cure?
  Seizures are completely controlled in about 20-30% of children on a ketogenic diet, with about 30-50% of children having fewer seizures, and some children having no significant effect on a ketogenic diet. A reduction in seizures in children on a ketogenic diet is only an indication that the child’s seizures can be controlled with a high ketone status in the body and does not indicate a cure.
  How long should I take the ketogenic diet when it is effective?
  It should be maintained for at least 2 years. However, in children with disorders such as GLUT-l deficiency and PDHD, it is necessary to maintain the KD for a longer period of time, and the course of treatment can be precisely adjusted according to the individual response to treatment in patients with refractory epilepsy. In children with complete seizure control, EEG and clinical information should be evaluated before stopping the diet to inform parents of the risk of recurrence after stopping the diet (its overall 20%).
  What are the adverse effects in the near and distant future after the ketogenic diet? How can they be avoided and corrected?
  Initial problems: (1) mental ill health and sleepiness are common at the beginning of the fast, and the mental status gradually improves after 1 week; (2) vomiting, diarrhea and abdominal pain occur in 12% to 50% of children in the early stage due to the high fat content, which is easier to occur in young children than in older children, but the degree is not serious, and most cases can be tolerated by themselves after a few days; (3) hypoglycemia, ketosis and acidosis may occur in a small number of children during the fast. acidosis, etc., which should be detected and given timely correction.
  Long-term problems: The incidence of hypercholesterolemia in children using KD is reported to be 14%-59%, the incidence of kidney stones and urethral stones is 5%-8%, the incidence of hypocretinemia is 2%, metabolic abnormalities are relatively minor adverse effects of KD, including hyperuricemia (2%-26%), hypocalcemia (2%), hypomagnesemia (5%), decreased amino acid levels and acidosis (2%-5%), carnitine ~5%), carnitine deficiency, rash, osteochondrosis, and rare reports such as pancreatitis and cardiomyopathy. In foreign reported cases of KD, the morbidity and mortality rate is 1.5%, but there is no definite evidence of a clear relationship between death and KD.
  Prevention issues: (1) Prevent dehydration: fluids are given uniformly throughout the day, 60-65 mL/(kg?d), not more than 120 mL each time, to avoid too much fluctuation in ketosis status. (2) Management of gastrointestinal symptoms: oral scopolamine. (3) Prevention of hypokalemia and stone disease: oral potassium citrate 0.5-1.0/d in 2 or 3 doses. (4) Multivitamin and mineral supplementation: supplement physiological requirements, calcium 600-650 mg daily.(5) Pay attention to the stability of the internal environment: blood gas analysis should be checked in children who have been fasting for a long time, children who cannot complete the daily required ketogenic diet, children with persistent mental depression or and infection. If the pH is normal, continue the diet; if the pH is low, reduce the proportion of KD or take a small amount of carbohydrate orally (20-30 mL of orange juice); if there is a very low pH and deep ketosis, discontinue the fast. (6) Monitor the physical development of the child: research reports on the effect of KD treatment on physical development are inconclusive. (7) Lipid monitoring: Some studies have reported that lipid tests are mostly normal in children treated with KD. If lipids are consistently elevated and there is no other clear explainable cause, screening of parental lipids is recommended
  Why are lipids and uric acid abnormal after ketogenic diet? What can be done?
  Because the high percentage of fat in the ketogenic diet affects the metabolic balance of the three major nutrients, mild abnormalities generally do not require special treatment. If significant hyperlipidemia or hyperuricemia occurs during the course of the ketogenic diet, the dietary ratio needs to be adjusted if necessary.
  Does it matter if my child sleeps less after the ketogenic diet?
  The child’s sleep may be affected for a week or so after the ketogenic diet is first started, and will gradually improve with continued use of ketogenic. No special treatment is needed.
  Do I still have to use anti-epileptic drugs during the ketogenic diet?
  At the time of initiating the KD, it is generally required to continue the original AEDs. if effective, it is best to start tapering the medication after a few weeks, but only try to reduce 1 at a time
  Some carbohydrate-containing medications should be taken sparingly on a ketogenic diet, why? How can I identify them?
  Because the ketogenic diet needs to be designed according to a specific ketogenic protocol and requires strict adherence to the recipe. If drugs with high carbohydrate content are consumed randomly or inadvertently, the production of ketone bodies will be affected, which can seriously aggravate seizures and lead to foregoing. If you are taking ketogenic drugs, you can read the drug instructions and consult a clinical pharmacist, dietitian or neurologist if necessary, rather than taking the drugs at your own discretion.