The Implementation and Maintenance of the Ketogenic Diet in Children.
The Ketogenic Diet was developed in the 1920s as a treatment for intractable seizures. This high-fat, adequate-protein, low-carbohydrate diet simulates the biochemical changes that occur during starvation. The diet, like starvation, induces a state of ketosis that appears to provide seizure control. Research is ongoing. However, the exact mechanism of how or why this therapy works as an antiepileptic measure is not known. The Johns Hopkins Pediatric Epilepsy Center is currently conducting an Ketogenic Diet Crossover Study to test the efficacy of ketosis as an antiepileptic therapy.
The Hopkins Pediatric Epilepsy Center has started more than 400 children on the Ketogenic Diet. More than half of that population remained on the diet for at least one year, and most of those who remained on the diet had a better than a 50% decrease in their otherwise difficult-to-control seizures. Improved seizure control, improved cognitive status and better quality of life are the reasons given by families for continuing this treatment. We believe that our success in sustaining the treatment is related to our management practices after initiation of the diet--what we term "fine-tuning" the diet. This article shares our implementation and management practices to assist others in providing this therapy for patients with intractable seizures.
Preparation for the Ketogenic Diet
Prior to admission, the child's medical records are reviewed, and the parents are asked to write a letter about their child and their personal goals for the diet. This letter helps us to understand and evaluate the realism and rationale behind each parent's desire for the diet. If the child is accepted into the program, parents are mailed a packet that contains a detailed pathway of what is to occur each day of hospitalization, a list of what to bring for the hospital stay, instructions on how to purchase scales and other supplies that will be needed in administering the diet at home and seizure calendars giving information on seizure activity for one month prior to hospitalization. Parents are also asked about the child's medications and dietary preferences and restrictions. Parents are instructed to view the video, "Introduction to the Ketogenic Diet," by the Charlie Foundation, and to read, "The Epilepsy Diet Treatment," prior to the admission. A packet of information mailed to each child's primary physician explains this diet treatment, gives recommendations for treatment of childhood illnesses while on the ketogenic diet, discusses common complications and provides information about the importance and availability of carbohydrate-free medications.
Patients and their families begin the Ketogenic Diet Program with an outpatient clinic evaluation. At this appointment they attend a 2-hour class on the history of the diet and a review of what is to occur during hospitalization. Fasting begins after the dinner meal on that clinic day, and children are admitted to the hospital the following morning for a 4-day stay. Fasting involves consuming nothing of caloric value but does include maintenance zero-calorie fluids throughout the fast. The 4-day stay permits close observation of the child during the critical portion of the fasting and the gradual introduction of the ketogenic diet. During this period the parents attend daily classes about the diet and its medical management.
Medical management in the hospital includes baseline blood and urine screening, blood glucose monitoring every 6 hours, fluid restriction and treatment of any complications such as hypoglycemia, nausea, vomiting and dehydration.
Calculation of the Diet
Our goal is to calculate a Ketogenic Diet formula that will provide optimal seizure control and maintain adequate nutrition for growth and development. We arrive at this by assessing the child's weight, height, age and present health, activity status, medical diagnoses and present medications. Everything that affects the child's metabolic rate and ability to consume food is important to consider. Standards for Recommended Daily Allowances (RDA) and World Health Standards are used, and the child's recent laboratory values are reviewed in planning to meet adequate nutritional needs. A good diet history is essential. The diet is easier to initiate if the child's actual weight is close to the ideal weight for his or her height. This is because optimal ketosis is more easily acquired in children who are at their ideal weight for height. We calculate the formula based on the child's ideal weight if he or she is overweight and use the actual weight if the child is underweight or close to the ideal weight. Profoundly handicapped children who are inactive require fewer calories per kilogram. Optimal protein for growth is of significant concern. Our goal is to provide 0.75-2 g of protein per kilogram of body weight depending on age, health status and rapidity of growth.
Following a 48-hour fast, the children are started on 1/3 strength feedings of eggnog over 24 hours and advanced by 1/3 every 24 hours until they are taking a full-strength Ketogenic Diet. This slow progression allows the digestive system time to adjust to the high-fat content of the diet and maintains a partial fasting state. We introduce the diet as an eggnog formula because this provides a fully ketogenic drink. Therefore, if the child does not take all of it or vomits a portion, he or she will have gotten appropriate proportions of fat, carbohydrate and protein to acquire ketosis.
During hospitalization, parents or caregivers attend 2 hours of classroom instruction each day, as well as individual teaching about implementing the Ketogenic Diet. They learn how to manage illness or medical problems that arise when a child is maintained in a state of ketosis and how to monitor and prevent complications common to the diet. Parents need to understand these potential complications to assist in preventing serious problems.
Problems During Hospitalization
During the initiation phase children are closely monitored to prevent or provide treatment for complications such as hypoglycemia, vomiting, dehydration and medication toxicity. Hypoglycemia can occur during the fasting phase and is one important reason for initiating the diet in the hospital. Blood glucose is monitored every 6 hours. If the blood glucose level drops below 40 mg/dl, the child is assessed for symptoms of hypoglycemia. If these symptoms are not present, observation continues and the glucose is rechecked hourly until the level registers above 40 mg/dl. If the glucose level is below 40 mg/dl and the child demonstrates symptoms of hypoglycemia, 30 cc of orange juice is given orally or by nasogastric tube. If indicated, intravenous (IV) fluids are started, and glucose, 0.5 g/kg IV, may be given as a bolus. The blood glucose is then repeated in 30-60 minutes as needed until the child is stable. Every 6 hours monitoring then resumes and continues until the 2/3 strength ketogenic formula is initiated.
As the Ketogenic Diet is introduced, children may experience nausea and vomiting. This may be due to hypovolemia or dehydration secondary to inadequate fluid intake. Ketosis suppresses appetite and thirst, and during initiation of the diet lack of thirst or of appetite may become excessive. This may lead to dehydration and acidosis. It may become necessary to use IV or nasogastric tube fluids to maintain adequate hydration and permit continuation of diet introduction.
If vomiting occurs only after ingestion of the ketogenic shake, it may be due to an inability to digest the high-fat content of the formula. Diluting the shake or offering it in smaller portions more frequently may help. The administration of vitamins and calcium do not begin until the child is discharged from the hospital because they may potentiate nausea and vomiting during the introduction phase.
Antiepileptic drug levels may increase as a child becomes ketotic. If a child displays signs and symptoms of drug toxicity, slight medication decreases are made during the initiation phase of the diet. However, unless there is some symptomatology suggesting that the child is overmedicated, we try not to reduce medications for at least the first 2-3 months after initiation. Caregivers are instructed to monitor the child for signs and symptoms of toxicity. We try not to begin weaning any antiepileptic medications until we are satisfied that we have fine-tuned the diet.
Emotional support from the Ketogenic Diet Team is essential during hospitalization. Fasting is a difficult time for the patients and their families. The patients may feel they are being punished. Parents may feel they are depriving their children and may even attempt to fast with the child to show their support. If a child begins to experience complications, the parent may feel guilty for allowing this treatment to occur. The patients need age-appropriate information about what is happening to them and reassurance that they are doing a good job in a difficult situation. Parents are routinely counseled about these issues. They are encouraged not to skip meals so that they can be alert to attend classes. Parents need to provide continued support and care to their child. We try to reframe this experience as a gift they are providing their child. They are giving the child a chance for control of his or her intractable seizure disorder.
Fine-tuning the Diet
Once the children are taking full ketogenic meals, they are discharged and the work of diet maintenance and fine-tuning begins. Fine-tuning is the manipulation of calories and ketogenic ratio to acquire optimal tolerability and seizure control. For some children our initial estimate of calories and ratio is accurate and seizures are completely controlled. For most, while seizures have decreased, they continue. We attempt to give each child the best chance at seizure control by fine-tuning the diet to gain high ketosis. We aim for a urine dipstick measurement of large ketones, 80-160 mg/dl. The measurement of urinary ketones is a gross measurement. At present, however, there is no more accurate measurement for ketones available for parents to check daily within their home. Therefore, if a child continues to have seizures in spite of large urinary ketones, we attempt further dietary manipulation to improve seizure control.
If a child is experiencing continued seizures 2-3 weeks after discharge, one of the first things to consider is the use of free foods. Free foods are anything not calculated into the daily keto plans. Our free food list includes 25 g lettuce, 3 ripe olives, a brazil nut, walnut, macadamia nut, pecan or 3 filbert nuts daily. These added foods, however small, can make a difference in seizure control for some children.
Initial menus for the diet are usually calculated using generic categories for fruits and vegetables. A sample diet is found in Table 1. This approach allows more flexibility because parents can pick from a list of varied fruits and vegetables. However, the foods within these lists vary in carbohydrate content, and that variation can affect optimal ketosis for some patients. It is sometimes beneficial to calculate individual diets with specific foods. We choose a limited amount of meal plans to test this idea. If specific plans are not improving seizures within 2-3 weeks, then we resume the regular plans.
Table 1 A Sample Ketogenic Diet for a 3-Year-Old Boy With No Medical Problems Other Than Intractable Epilepsy
Wt 15.9 kg 1035 calories 19 grams protein daily daily/4:1 ketogenic ratio Ht 42.5 in. 3 meals daily: 7 grams carbohydrate 305 calories per meal daily 65 cal/kg 1 snack daily: 103.35 grams fat daily 120 calories Breakfast 22 grams egg 10 grams applesauce 18 grams butter 30 grams of 36% cream 5 grams bacon Lunch 18 grams American cheese 28 grams cucumber 15 grams butter 35 grams 35% cream Snack Peanut butter cup: 7 grams creamy Peanut butter 10 grams butter (mix together, roll into a ball and chill) Dinner 14 grams chicken 15 grams green beans 22 grams butter 35 grams 36% cream
The use of processed foods such as hotdogs and deli meats may cause a drop in urine ketones and result in an increase of seizures. The labeling of the content of processed foods is not exact, and they are usually high in carbohydrates and sodium and low in protein. This makes them not only poor choices for optimizing ketones but for supplying adequate protein as well. When finetuning a diet for a child with continued seizure activity, we ask parents to withhold processed foods for one month to determine whether it has an effect. Occasionally parents have reported to us that seizures improve after several days of withholding aspartame. We ask that they test this by withholding it for two weeks and then reintroducing it and observing the effect. If seizure frequency rises again, the aspartame is stopped. Most carbohydrate-free drinks are made with aspartame. Parents can make drinks using saccharine, but this does limit the flexibility of using readily available diet sodas.
Fats vary in content and composition. In finetuning the diet of a patient who is having difficulty producing sufficient ketosis, we may lessen or remove the less dense fats such as butter and mayonnaise and substitute these with oils. The oils most frequently used are canola, fiaxseed, olive and medium chain triglyceride (MCT oil). MCT oil is more efficiently metabolized because it is absorbed into the portal system, bypassing the lymphatic route. One disadvantage to MCT oil is that it can cause Gl disturbance if used in large amounts. When using MCT oil, we begin with 5 g/meal, or 15 total grams daily, increasing by 3 g/meal increments daily until the desired effects are obtained with minimal side effects.
Optimal ketosis requires a careful balance between endogenous fat to burn and exogenous fat for ketosis. Having reduced a child to his or her lean body mass, there is little reserve to provide ketosis when there is a prolonged time between meals. If parents feed the child dinner at 5:30 pm, put him or her to bed at 7:00 pm, and breakfast is at 7:30 am, this results in a 14-hour period between meals. These children may experience low morning ketones and early morning seizures. Three meals per day for a growing child may not provide the constant steady intake of food required to maintain an optimal steady state of ketosis. More consistent-ketosis can be provided by giving four or five small meals a day and a snack at bedtime or using small snacks throughout the day.
Our most common dietary adjustment is to reduce calories. We often overestimate the caloric needs of our population, because many of these children with difficult-to-control seizures have additional medical problems such as severe mental retardation and cerebral palsy, which limit their activities and affect the rate at which they burn calories.
If calories are excessive, the child will gain weight and not develop sufficient ketosis. Too few calories can also lower ketone levels. Initially, fasting burns fat stores and increases ketosis, but once the body is reduced to lean mass, there is insufficient fat to produce ketosis. This fat must come from the diet. An increase of too many calories at once may throw off ketosis and increase seizures in infants and small children. It is sometimes best to gradually increase calories by 25 kcal every 1-2 weeks until the desired caloric level has been reached.
Sometimes, a change in the ketogenic ratio is needed to acquire improved seizure control. Raising the ratio increases the amount of fats consumed and, it is hoped, increases ketosis, thereby resulting in better seizure control. Occasionally we decrease the ratio if a child becomes anorectic and will not eat, remains too acidotic, is experiencing frequent illnesses or is having persistent digestive difficulties on the diet. Most children are maintained on a 4:1 ratio (4 g of fat to 1 g of carbohydrate and protein) throughout their first year on the diet. After that year we attempt to slowly taper the ratio. A 3:1 ratio provides better nutrition and is more palatable. However, our main goal is to preserve seizure control. If, as we lower the ratio, control is not optimal or seizures increase, we may choose to continue the 4:1 ratio.
Overweight children are an exception. We frequently place them on a calorie-restricted diet in 3:1 ratio to facilitate weight loss. As these children loose weight, they burn their own body fat and this produces high ketosis. As they reach ideal weight, they have less of their own body fat to burn. At this point we increase the ratio to maintain the same high level of ketones. We seldom use a 5:1 ratio and we will not remain at this high ratio for more than 6 months because it is very hard to provide adequate nutrition at this ratio.
To achieve adequate ketosis, we often decrease carbohydrates to below 5 total grams daily. This allows us to increase the ketogenic ratio without compromising protein and enables us increase protein intake for growing children. This has been especially effective in infant formulas because infants are used to using ketones as their energy source in utero. New ketogenic diet patients may experience some mild symptoms of hypoglycemia if carbohydrates are too restricted at first. However, once children's metabolism adjusts to burning fats for fuel, they tolerate the low carbohydrates well.
Medications can be a source of substantial carbohydrates. Wherever possible, liquid and chewable pediatric medications should be avoided. Many tablets contain carbohydrates as fillers. Most medications can be formulated in carbohydratefree forms by using a compounding pharmacy.
If a child continues to experience persistent seizures and ketones are not measuring at a high level, we assess the need to calculate the carbohydrate content of any medicines the child is routinely using. We can do this is two ways. We calculate a small amount of fat, usually butter, to take with each medication dose. When this is done, we subtract the calories in the fat from the total daily calories and adjust meals as needed to compensate. The other way is to calculate these carbohydrates into the meals and have medications taken with meals. Topical medications, lotions, or hygiene products may contain sorbitol or other sugar-based ingredient and have the potential to affect ketosis. Parents are instructed to be aware of this and to read labels. If unavailable commercially, these products can be made carbohydrate-free by a compounding pharmacy.
Monitoring the Diet
Optimal fine-tuning and maintenance of the diet can be accomplished only with diligent monitoring and close contact between the parent and the Ketogenic Diet Team. Telephone support of parents during the first (6-8 weeks is crucial to success on the diet. At each phone contact, we assess the child's seizure frequency, medications, activity level and overall well-being. We instruct parents to be ready to provide us information regarding ketone levels, daily calories, ketogenic ratio, changes in weight and height and any problems in tolerating or accepting the diet. Unless there is a significant adjustment problem, we don't make any dietary changes for at least 2 weeks to a month. Children need this time to adjust to the changes imposed upon their metabolism as a result of ketosis. When we do make adjustments, we only make one change at a time and then allow time to assess the effect of the change. If we change the ketogenic diet, medications remain constant. If we change the medications, no changes are made to the diet. If a child is continuing to experience seizures, the wait is often very difficult for parents. They are eager for improvements. We tell parents that if we make adjustments to the diet too quickly, we may inadvertently pass over the best plan for their child.
Once a child's diet has been fine-tuned, parents are encouraged to use the Johns Hopkins Ketogenic Diet Computer Program to calculate individual meal plans. This allows more family freedom in meal choices. It also helps parents feel more involved in their child's epilepsy treatment. Parents are able to provide their child a greater variety of foods he or she specifically likes to eat within the restrictions of the ketogenic diet and nutritional needs. This program can be purchased through the Epilepsy Foundation of the Chesapeake Region, Towson, MD. A physician prescription is required.
Parent Support and Keto Coaches
Maintenance of a ketogenic diet sounds like a full-time job for parents with already overextended lifestyles. Many of our parents are coping with the demands of raising their families, caring for a child with special needs, as well as managing employment outside the home. Grocery shopping time alone can be doubled the first month or so after starting the Ketogenic Diet. Labels on each food and medication must be read, and new eating habits formed. Schools, day care centers, pediatricians, pharmacists, neighbors and grandparents must be educated about the maintenance of the diet. Remarkably, our parents do very well. They tell us that what at first seems to consume all their time does become routine and they gradually incorporate the Ketogenic Diet into their lifestyle.
During this time other parents whose children have been on the Ketogenic Diet can provide valuable support and advice. Upon discharge of the child, all families are given the name and phone number of a parent "keto coach" in their area. These keto coaches do not fine-tune diets, make meals or give medical advice. They offer suggestions and support regarding the implementation and maintenance of the diet and ways to organize the routines of a ketogenic household that make life easier.
Weaning of lntiepileptic Medications
Once fine-tuning has been achieved, the patient appears adjusted to the diet and seizures are stabilized, weaning of antiepileptic medications can be initiated. The appropriate time to begin to taper medications is determined on an individual basis for each patient. A general guideline, however, is at approximately 6 months into the diet. A slow, steady, successful weaning is much preferable to a rapid one that results in uncontrollable seizures and the reintroduction of medications. During hospitalization we instruct the parents about medication weaning. We teach the signs and symptoms of drug withdrawal, what they can expect and what action to take if necessary. The amount of drug decreased at each interval and the length of the weaning depends on the drug. Parents are taught to expect some seizure breakthrough with each decrease. This usually occurs 24-72 hours after a decrease and may last up to a week. They are instructed not to increase the medication dose at the onset of seizure activity. They are to maintain the weaning and wait out this withdrawal period. If seizure activity becomes excessive, lorazepam or diazepam can be used to assist them through the weaning. When weaning phenobarbital or clonazepam, we have reduced as slowly as 0.1 mg every two weeks to minimize withdrawal. We do this by having a compounding pharmacy make up a 1 mg/ml oral solution and use a 1-ml syringe that measures in 0.1-mg increments. We wean only one medication at a time, and no diet changes are made if a drag decrease is made. If a diet adjustment is necessary, the medication wean is held for at least two weeks to allow the diet to be adjusted.
Problems to Anticipate
Caring for a sick child on the Ketogenic Diet can be a challenge. Dehydration and severe acidosis are common problems during childhood illnesses. Children on the diet are susceptible to these complications because they are maintained on a constant fluid restriction and in a mild state of acidosis. Parents are taught how to manage common illnesses. Hydration is of primary concern. If a child has fever, vomiting or diarrhea, fluid restriction is immediately removed. Parents must be instructed to offer carbohydrate-free fluids frequently and as tolerated. The fluid restriction is not reintroduced until the child has been symptom-free for several days. To further reduce the incidence of dehydration, carbohydrate-free fever-reducers are to be used; acetaminophen suppositories are recommended. Once vomiting and or diarrhea have stopped, the diet is reintroduced at 1/3 strength and advanced as tolerated to prevent symptoms from reoccurring.
Parents are taught the signs and symptoms of potential complications, what to do to prevent them and how to treat them if necessary. As part of this instruction, they receive a sick day handout to assist them and their primary physicians in treating the most common symptoms: vomiting, fever, diarrhea, sore throat, cough and discomforts. These problems rarely require discontinuation of the Ketogenic Diet. Adequate oral or IV hydration, bicarbonate replacement and occasionally conservative administration of carbohydrates usually correct the underlying acute metabolic disturbance without compromising ketosis and seizure control. However, when medically indicated, it is occasionally necessary to stop the diet temporarily to treat an illness. When this occurs, we gradually resume the diet once the patient is medically stable.
When seizures are a problem during illnesses, the short-term administration of additional carbohydrate-free preparations of antiepileptic medications may be indicated. Carbohydrate-free medications for fever, vomiting and diarrhea are available and should be used when necessary. Commonly prescribed medications can be formulated carbohydrate free through a compounding pharmacy. Parents should research their communities for a compounding pharmacy.
Parents are instructed that they can expect their child's previously normal pattern of bowel movements to change on the diet since the bulk of the meals is smaller. This change does not necessarily indicate constipation. However, if the child is uncomfortable, is straining, is complaining of abdominal cramping and has hard stool, then the child needs treatment for constipation. If these symptoms occur, we try the following:
* Use group A vegetables, like lettuce, because these will provide double the bulk of green beans, a Group B vegetable.
* Make sure that the child is receiving the total minimum fluid allowance and, if needed, increase daily fluids by 100-150 cc to improve regularity.
* Calculate small amounts of MCT Oil into each meal.
* Carbohydrate-free stool softeners may be used daily. We have found that a daily dose of epsom salts (1-2 tsp/daily) is an effective stool softener.
If a child is already constipated, he or she needs treatment for this before any of the preventive treatments will be effective. Unflavored milk of magnesia, unflavored Fleets Phosphosoda or enemas may be used effectively. If the stool is soft but not easily expelled, a glycerine suppository may be helpful.
The fluid restriction necessary to maintain high ketosis and the chronic acidosis accompanying the diet predisposes these children to kidney stones, which may be either uric acid or calcium oxalate stones. Parents are instructed to check urine once a week for blood using hematest sticks. If the urine tests positive for blood, they are instructed to stop their fluid restriction and force fluids for 48 hours and then increase the daily fluid restriction by approximately 200 cc. If blood in the urine persists following the 48-hour flush, parents are instructed to send urine for testing that includes reagent strip analysis, microanalysis, calcium, creatinine and uric acid. If the calcium creatinine ratio is above 0.2 mg and the urine remains positive for blood, a renal sonogram is obtained and the child is started on bicitra or polycitra K powder 2 meq/kg to alkalinize the urine. Most stones are small and appear only as "sand" in the urine. Occasionally small stones are found in the calyx of the kidney and may even be passed. Symptoms of kidney stones include not only the classic severe pain and blood in the urine, but may include nonspecific illness with fever, malaise, poor appetite or an otherwise unexplained increase in seizures. With alkalinization, even established stones may disappear. Lithotripsy or operative removal has rarely been necessary.
On the diet, fluid is usually restricted to approximately 1 cc/calorie daily. The rationale for this has not been tested. We believe that over time an equilibrium is achieved in which with minimal hydration, ketones are better retained. Children who are more active or who live in hot climates may need more fluid to ensure that optimal hydration is maintained and to prevent stone formation.
We teach parents that each food within the child's ketogenic meal is specifically calculated to maintain adequate ketosis and therefore no portion of any meal may be omitted or withheld to feed at a later time. Parents often become concerned if the child refuses to eat all the ketogenic meal because this is the treatment for the child's seizures. Eating may become an issue of control, leading to stress on the family. This may predispose a child to develop dysfunctional eating habits. To induce the child to eat, caretakers may engage in games, participate in cajoling or force feeding; this leads to prolonged unpleasant meals. It is not difficult to recognize that the child is in control, using these times to gain attention, even perverse attention. Parents must be warned that these problems may occur. We have seen these behaviors become apparent as early as during the diet initiation, especially if the child has a prior history of being a poor eater. When these problems are recognized, modification must begin immediately. We tell parents that the child has 20 minutes to complete the meals. Parents should place the food on the table or begin to feed the child if they are not a self-feeder. No television or other entertaining diversion is allowed during mealtime. They can request, only once, that the child eat. If the meal is not eaten within 20 minutes, the mealtime is over. Parents are to calmly take the food away and not give anything else to eat until the next mealtime. This plan is to be followed at each mealtime. Parents need to be careful not to give any negative responses or behave negatively toward the child following a meal that is not eaten. When feeding problems have become well established, it is sometimes helpful for the primary feeder to allow someone else to feed the child for a period of time to assist in breaking the pattern.
Our culture places significant importance on food. It is an essential tool for socialization. We celebrate with food, reward with food and communicate love with food. When a child has serious medical problems that prevent participation in the usual childhood activities, food becomes even more important. Eating may be the only enjoyable social activity in which a disabled child can participate. Parents and extended family and friends, all too aware of how much this child is missing, typically compensate for these losses with food.
When families choose Ketogenic Diet Therapy, these issues need to be addressed prior to diet initiation. The family needs to reframe perceptions they may have in depriving their child through the use of the Ketogenic Diet. This treatment is an opportunity to take control of their child's intractable seizures. If a family is unable to overcome the feeling that they are depriving their child of emotionally satisfying foods, they will have a very difficult time maintaining the Ketogenic Diet over the recommended 2-year period. Parents, extended family and all those involved in this child's life need to find alternative activities for socialization and positive reinforcement. This task is not always easy, but our families do remarkably well.
Parents may be tempted to feed a child on the Ketogenic Diet separately from family mealtime. We tell families that mealtime is important and the child should be included. Ketogenic meals can almost always be made out of what is being planned to serve for the family meal. Children on the Ketogenic Diet can go to restaurants and fast food establishments. Ketogenic meals can be made at home and placed on restaurant plates or in fast food containers. Canned diet drinks can be poured into a fast food cup.
Siblings are sometimes instructed to hide what they are eating if it can't be shared with the child on the Ketogenic Diet. We tell parents that this is not a good idea because it can lead to sibling conflicts. When others in the house are eating non-ketogenic foods, this provides an opportunity for the keto-child to practice saying, "I can't have that. It's not on my special diet." When the purpose of the Ketogenic Diet is explained to siblings, they are usually very supportive and protective of their brother or sister.
Celebrations and Rewards
Children on the Ketogenic Diet can go trick-or-treating and have birthday parties. One parents' Halloween idea was for their child to sell the candy she collected to her parents and use the money to buy something special. For birthdays a cake can be made using the ketogenic cheesecake recipe. Parents can add food coloring and shape the small cake into a heart, star, animal and sun. One mother made a regular birthday cake and placed the small ketogenic cake on the top as a decoration. That way the child could blow the candles out on a regular birthday cake and feel as if he or she were sharing a part of the same cake everyone else was enjoying. Our parents have been very helpful in sharing ideas such as this.
We suggest the use of stickers, bubbles, frozen diet sodas, videos, music, teething rings and small toys as rewards. Parents may have to do some investigating, but each child has been able to be provided with something rewarding to substitute for food. This transition is especially difficult for the disabled child who is not able to comprehend the need for the change. Parental support is necessary to help make this work.
Our adolescent population presents special considerations. Epilepsy has already placed significant strain on their need for peer acceptance and independence from overprotective parents. The Ketogenic Diet can have a further impact on these issues. It is not socially acceptable to carry a lunch box full of weird foods in little plastic containers to school. An adolescent on the ketogenic diet may be inadvertently set up for embarrassment and ridicule from peers. For instance, a 16-year-old male was experiencing unexplained weight loss. We discovered that he was throwing away his ketogenic lunches at school to avoid being teased. There are ways to pack a normal looking keto lunch. A-.bag of weighed macadamia nuts and a diet soda provides a 3.5:1 ratio ketogenic meal similar to a bag of chips and a soda. Butter can be added to raise the ratio as needed.
Intractable epilepsy requires some adolescents to wear a protective helmet during the school day. Others may experience public urinary incontinence during seizure activity. Potentially, the Ketogenic Diet can provide relief from these embarrassments by achieving seizure control. Seizure medications can often be reduced, thus minimizing adverse drug effects. The Ketogenic Diet has to be worth the trouble and investment. If the adolescent perceives that the diet is beneficial, we have little difficulty in acquiring compliance.
It is not uncommon for children to attempt food stealing. It is difficult to resist forbidden foods. When parents discover that cheating has occurred, they are instructed to remain calm so as not to promote negative reinforcement. We tell them to explain to the child that he or she will need to fast if a forbidden food is eaten. If the child is experiencing seizure breakthrough, the fast should continue for 24 hours. If the child appears stable and no seizures are evident, the child skips one meal. We don't want the child to feel ashamed or that he or she has been bad. We don't want the fast to be implemented as a punishment but rather as the medical treatment that is necessary to prevent seizures if the diet is broken.
Many times a single cheating episode does not affect the seizure control, but the fast through one meal is done regardless. This establishes a consequence to eating foods that are not on the Ketogenic Diet. Frequent cheating usually results in poor seizure control. A child who can cheat frequently without experiencing seizures usually finds it difficult to remain compliant. If occasional cheating doesn't bring on seizure activity, the ketogenic ratio may be higher than necessary for seizure control. A reduction in ratio will provide less fat and more palatable foods, so that compliance is less difficult.
We hold a Ketogenic Diet Follow-up Clinic at least once a month. Approximately 14 patients and parents are scheduled for each clinic. Patients are usually scheduled to return with the same families they met during their hospitalization to facilitate continued family support. Patients are required to come to the Follow-up Clinic at 3 months, 6 months, 1 year and annually thereafter for monitoring and maintenance of the diet. During each visit, the child and family receive an assessment by our Ketogenic Diet Team, which includes the program coordinator, physician, dietitian, nurse and social worker. The focus of this visit is seizure control, general health status, quality of life and growth and development of our patients. We assess the effect of these issues on our patients and their families and provide intervention as needed. At each visit, blood and urine are tested to monitor for complications and to ensure optimal health. Nutritional status is closely monitored. Laboratory values are assessed to ensure adequate visceral protein. Somatic protein is monitored by physical assessment. Growth patterns are followed, by both clinical physical assessment and parent reports. Dietary adjustments are made accordingly.
We obtain fasting blood work for lipid profile, complete blood count with differential, serum chemistry and electrolytes and monitoring of antiepileptic drug levels. Urine is screened for urinalysis, microorganisms, calcium, creatinine and uric acid. If the child has been experiencing any urinary complications prior to a visit, he or she is scheduled to be seen by the Nephrology Service on clinic day. An electroencephalogram is obtained at the 6 month and yearly visit. The patient is weighed and measured. We assess the need for diet or medication adjustment to improve seizure control and to maintain optimal growth and quality of life. This intensive visit is imperative for the successful maintenance of the Ketogenic Diet.
The Ketogenic Diet is a labor-intensive, effective treatment for intractable seizures. It requires significant commitment from patients, their families and the Ketogenic Diet Team. It is not difficult to get children into ketosis by placing them on a Ketogenic Diet. The challenge is to maintain the diet as well as seizure control and optimal health over an extended period of time. To best monitor the patient, prevent complications and provide intervention as needed, the Ketogenic Diet should be initiated in the hospital. Parent or caregiver education and training are essential to the maintenance of this therapy. Once the diet has been initiated, fine-tuning is often necessary to acquire the best results. The availability of the Keto Team is imperative. Optimal maintenance of this treatment is not possible without routine communication between the family and the Keto Team. Common childhood illnesses can be effectively managed even if it becomes necessary to temporarily interrupt ketosis. Mandatory clinic follow-up is necessary for the successful maintenance of the Ketogenic Diet. Ultimately, the most important aspect to the success of this treatment is a team approach. When it works, it can provide a dramatic change in the lives of children with epilepsy and their families.
I gratefully acknowledge Ms. Milly Kelly, RD, who spent 50 years providing the Ketogenic Diet to children with intractable epilepsy. I had the privilege of having her as my mentor. She graciously shared her knowledge, expertise, intuition and wisdom. Thank you, Milly.
[1.] Freeman JM, Kelly MT, Freeman JB: The Epilespy Diet Treatment. An Introduction to the Ketogenic Diet, 2nd ed. Demos Publications,1996.
[2.] Swink TD, Vining EPG, Freeman JM: The ketogenic diet:1997. Adv Ped 1997; 44: 297-329.
Questions or comment about this article may be directed to: Jane Cassilly Casey, RN LCSWc, Pediatric Epilepsy Center, Johns Hopkins Medical Institutions, Meyer 2-147, 600 North Wolfe Street, Baltimore, Maryland 21287-7247. She is Director of the Ketogenic Diet Program.
Jane McGrogan, RD, is a dietitian, Ketogenic Diet Program, Johns Hopkins Medical Institutions.
Diana Pillas, BS, is coordinator of the Pediatric Epilepsy Center Program at Johns Hopkins Medical Institutions.
Paula Pyzik, BS, is a research associate, Pediatric Epilepsy Center Program, Johns Hopkins Medical Institutions.
John Freeman, MD, is Director of the Pediatric Epilepsy Center Program at Johns Hopkins Medical Institutions.
Eileen P.G. Vining, MD, is Associate Director of the Pediatric Epilepsy Center Program at Johns Hopkins Medical Institutions.
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|Author:||Casey, Jane Cassilly; McGrogan, Jane; Pillas, Diana; Pyzik, Paula; Freeman, John; Vining, Eileen P.G|
|Publication:||Journal of Neuroscience Nursing|
|Date:||Oct 1, 1999|
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