
Pediatric Ketogenic Diet
A ketogenic diet is a high-fat, adequate-protein, and very-low-carbohydrate diet which has been found to help many children whose seizures are not well-controlled by anti-seizure medications. In some children, the ketogenic diet is combined with standard anticonvulsant medications. While the actual mechanism of the ketogenic diet's effectiveness against seizures is unknown, many children on the diet are able to have their epilepsy medication dose lowered, decreasing unwanted medication side effects. We are consistently ranked among the top hospitals in U.S.News & World Report, distinguished for our pediatric care. Read More Because this is a medical treatment, the ketogenic diet must be supervised by a medical team, consisting of a neurologist and dietitian, who can anticipate and manage possible nutritional deficits or other medical side effects or complications. The team helps children and their families establish and maintain the diet, and learn how to incorporate the diet into daily living. Children on the ketogenic diet are seen several times throughout the year for close monitoring, while they continue to follow with their primary neurologist for potential medication changes. In addition, the team also implements the Modified Atkins Diet and Low-Glycemic Index Therapy, other dietary therapies for which there is increasing data about benefit in the treatment of difficult-to-treat epilepsy. Conditions treated by the Ketogenic Diet Team include: Epilepsy such as Myoclonic Astatic Epilepsy and other epilepsies Glucose transporter type-1 deficiency Lennox Gastaut syndrome Pyruvate dehydrogenase deficiency Dravet syndrome What is a ketogenic diet? The ketogenic diet is sometimes offered to children who continue to have seizures while on seizure medication. When medicati Continue reading >>

Ketogenic Diet Guidelines For Infants
History of the Ketogenic Diet in Infants For many decades, the idea of using the ketogenic diet for infants with epilepsy was discouraged. Concerns were raised in chapters and books about the diet that infants were highly likely to become hypoglycemic, have complications, and unlikely to achieve ketosis. Although infants often have seizures, and at times very difficult-to-control seizures, until the 1990s, we also did not have pre-made, commercially-available ketogenic diet infant formulas. Even though most anticonvulsant drugs are not FDA-approved for use in infants, this was the main treatment option for most child neurologists. What’s New Things have changed drastically in the last few years. Published research has shown that not only can infants become ketotic, but they respond very well to dietary therapy. In fact, infantile spasms is one of the established "indications" for ketogenic diet treatment. An article from last year by Dr. Anastasia Dressler from Vienna even stated, "The ketogenic diet is highly effective and well tolerated in infants with epilepsy. Seizure freedom is more often achieved and maintained in infants." Adding to this rising tide of acceptance, the European Journal of Paediatric Neurology published guidelines for the use of ketogenic diets in infancy (defined as less than 2 years of age) this month. This was a group effort from 15 neurologists and dietitians with particular expertise in using ketogenic diet in infancy, convened at a conference in London in April 2015. What does this guideline say? Much of it is information about infants that already has been established and is similar to older children. The authors comment that the ketogenic diet can be helpful for infantile spasms, epilepsy with migrating seizures, and GLUT-1 deficiency (al Continue reading >>
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Are Protein Powders Safe?
Are protein shakes good for you? You've seen those massive tubs of whey powder in the gym or your local supermarket, but are they actually necessary for the man in training? Do protein shakes work? Scott Laidler explains all. 'Protein shakes are a helpful, convenient solution...but they won't turn your body into a temple overnight What is a protein shake There are still many misconceptions regarding protein shakes. Firstly, people often mistake them for steroids – perhaps understandable, given the big promises that drive the products' marketing campaigns. But protein shakes are purely nutritional; unlike steroids, they have no direct influence on your hormones. Another source of confusion is the differentiation between a 'mass gainer' and a protein shake. Mass gainers are used as an aid to bulking up. They typically include a large amount of simple carbohydrates that get delivered straight to your muscles. These serve a purpose after a workout when the muscle's energy is depleted, but use them at the wrong time (as many do) and the end result is fat gain instead of fat burn. Protein shakes, by contrast, deliver amino acids to muscle cells, helping them to recover after strenuous workouts. Which protein shake should I chose? Whey is the most common base for the protein powder, as it contains all of the nine essential amino acids that facilitate the healing of damaged muscles. There are three main types of whey protein: isolate, hydrolysate, and concentrate. Isolate yields a high level of protein and is low on allergenics, making it a good option for the lactose intolerant. Hydrolysate is produced in a way that effectively means it has been predigested, so its rate of absorption by the body is super fast. Concentrate, meanwhile, is the cheapest option, as the effects of Continue reading >>

Introduction Of A Ketogenic Diet In Young Infants.
Abstract The ketogenic diet is a rational treatment for pyruvate dehydrogenase complex deficiency (McKusick 312170) and GLUT1 deficiency syndrome (McKusick 138140). An increasing number of patients are diagnosed in early infancy, but few data are available on the introduction of a ketogenic diet in this age group. GLUT1 deficiency syndrome was suspected in four infants presenting with seizures and unexplained hypoglycorrhachia. A ketogenic diet was introduced at 6-28 weeks of age. Ketosis was initiated by fasting, monitored by bedside blood glucose and 3-hydroxybutyrate determinations, and was maintained successfully using supplemented carbohydrate-free infant formula and emulgated triglycerides. All patients developed ketosis within 24 h. 3-Hydroxybutyrate concentrations available at the bedside correlated inversely with the base excess. At glucose levels < or = 40 mg/dl patients remained asymptomatic in the presence of ketones. The ketogenic formula was tolerated well, parental compliance was good, and all patients remained seizure-free on the diet. GLUT1 deficiency was confirmed in two patients; the diet was discontinued in the other two patients. In one infant, failure to thrive on medium-chain triglycerides was effectively reversed using long-chain triglycerides. Urine dipstick analyses failed to detect ketosis in another infant. Adverse effects of the diet were limited to renal stones in one patient. The ketogenic diet can be introduced and maintained successfully in young infants using long-chain fat emulsion. Monitoring 3-hydroxybutyrate at the bedside was useful for metabolic control and superior to urine dipstick analysis. Seizure control was effective and adverse effects were limited, but evaluation of the long-term effects of the ketogenic diet in this age g Continue reading >>

Epilepsy, Children, And The Ketogenic Diet
Some parents of children with epilepsy are skeptical of the ketogenic diet when they first hear about it. A diet that can control epilepsy and stop seizures without any medication? It almost sounds like a scam. But the ketogenic diet is real and legitimate. It works very well in many people. The catch is that it's extremely demanding and difficult to follow. In fact, it is so difficult to follow that most doctors recommend it only for people who haven't been able to control their seizures with medicine. The ketogenic diet is an extremely high-fat diet that requires a child to eat four times as many fat calories as calories from protein or carbohydrates. A meal might include a small portion of chicken, a little bit of fruit, and a lot of fat, typically butter or cream. Frankly, it's a difficult diet to swallow. Your child may start the diet in the hospital, so nurses and doctors can observe the first few days. Your child will probably need to go without any food for 36 to 48 hours before beginning the diet. After that, food is gradually increased over a few days. This diet does not provide all the vitamins a body needs, so your child will probably have to take sugar-free vitamin supplements. No one knows why the ketogenic diet -- which was developed in the 1920's -- works so well. But we do know something about how it affects the body. By reducing the number of carbohydrates a person eats, the body is forced to burn fat for energy, a process called ketosis. This ketosis is the same process that kicks in when someone is fasting -- on purpose or because of starvation. Fasting has been a traditional seizure treatment for centuries. How ketosis helps with epilepsy isn't known, but it does. Researchers at Johns Hopkins University studied 150 children with epilepsy in one impo Continue reading >>
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Ketogenic Diet
What is the ketogenic diet? The "classic" ketogenic diet is a special high-fat, low-carbohydrate diet that helps to control seizures in some people with epilepsy. It is prescribed by a physician and carefully monitored by a dietitian. It is usually used in children with seizures that do not respond to medications. It is stricter than the modified Atkins diet, requiring careful measurements of calories, fluids, and proteins. Foods are weighed and measured. The name ketogenic means that it produces ketones in the body. (keto = ketone; genic = producing) Ketones are formed when the body uses fat for its source of energy. Usually the body uses carbohydrates (such as sugar, bread, pasta) for its fuel. Because the ketogenic diet is very low in carbohydrates, fats become the primary fuel instead. The body can work very well on ketones (and fats). Ketones are not dangerous. They can be detected in the urine, blood, and breath. Ketones are one of the more likely mechanisms of action of the diet, with higher ketone levels often leading to improved seizure control. However, there are many other theories for why the diet will work. Who will it help? Doctors usually recommend the ketogenic diet for children whose seizures have not responded to several different seizure medicines. The classic diet is usually not recommended for adults, mostly because the restricted food choices make it hard to follow. However, the modified Atkins diet does work well. This also should be done with a good team of adult neurologists and dietitians. The ketogenic diet has been shown in many studies to be particularly helpful for some epilepsy conditions. These include infantile spasms, Rett syndrome, tuberous sclerosis complex, Dravet syndrome, Doose syndrome, and GLUT-1 deficiency. Using a formula-only Continue reading >>

The Classic Ketogenic Diet: Evidence, Diet Calculation And Case Reports
Lindsey Thompson, MS, RD, CSP, LDN Zahava Turner, RD, CSP, LDN About our Keto Ambassadors Disclosures Lindsey Thompson - Consultant - Nutricia North America as Keto Ambassador Zahava Turner - Consultant - Nutricia North America as Keto Ambassador Objectives • Review research on the classical ketogenic diet • Describe the basic components of a classical ketogenic diet and ratio • Calculate a classical ketogenic diet including calories, protein, fat and carbohydrate goals • Review the classical ketogenic diet induction process • Recognize a sample meal plan or formula components on the classical ketogenic diet Ketogenic Diet Basics • High fat, adequate protein and low carbohydrate • Mimics the metabolic state of fasting • Produces ketones Ketogenic Diet 4:1 Fat Protein Carbohydrate American Diet Fat Protein Carbohydrate Who Gets Placed On The Diet? • Patients who fail the traditional anti-convulsant therapy • Utilized by a wide variety of ages • All seizure types o Doose epilepsy o Infantile spasms • Minimum time on the diet: 3 months • Average time on the diet: 1-2 years Ketogenic Ratio • Grams of fat: protein and carbohydrate combined • Example: 4:1 ratio is 4 grams of fat to 1 gram of protein and carbohydrate combined • Higher the ratio the lower the amount of allowed protein and carbohydrates Vitamins and Supplements • Supplementation almost always needed for the diet • Typically supplement: • Complete Pediatric multivitamin/multimineral supplement • Calcium • Vitamin D • May need supplements of: • Bicarbonate • Carnitine • Selenium • Salt • Supplements will be discussed later in the workshop Seizure Tracking Continue reading >>

Ketogenic Diet As Treatment Option For Infants With Intractable Epileptic Syndromes
Abstract A special diet for those suffering from epilepsy such as the ketogenic diet (KD) is a successful alternative treatment option in young children when treatment with different combinations of anti epileptic drugs (AEDs) fails. Notes Infantile spasms (IS): or West syndrome A form of severe epilepsy presenting between 3 and 10 months of age characterized by a brisk flexion or extension of the extremities, sometimes followed by a brief tonic posture, appearing in clusters and often occurring after sleep transition and accompanied by developmental regression. Characteristically interictal EEGs reveal hypsarrhytmia, high-amplitude, chaotic background pattern, and asynchronous slow waves intermixed with multifocal spikes. Intractable epileptic syndrome General term covering epileptic syndromes (like Ohtarara syndrome and West syndrome) that do not respond to initial treatment with (multiple) anti epileptic drug regimes. Ketogenic diet (KD) High-fat, low-carbohydrate diet with adequate amount of protein that mimics the metabolic state of fasting during an anabolic situation. Due to the lack of carbohydrates energy metabolism shifts from carbohydrate to fat burning for energy, which induces production of ketone bodies (aceto acetate, β (beta)-hydroxybutyrate and acetone) that can be measured in urine and blood. An adequate level of ketosis is defined as 3–4 + (8–16 mmol/L) ketones in urine or 2–5 mmol/L ketones in blood. Ketogenic ratio The ratio of ketone-producing foods in the diet (i.e. dietary fat). The most used ratio’s are 3:1 or 4:1. This means either 3 or 4 g of ketone-producing fat vs. 1 g non-ketone-producing protein and carbohydrates. Continue reading >>

Epilepsy Center | Ketogenic Diet
Diet therapy for epilepsy Diet therapy can sometimes be a good alternative for childhood epilepsy when medications cannot control seizures or have intolerable side effects. While diet therapy is often worth trying in general, it can be especially helpful for certain epilepsy syndromes, such as myoclonic astatic epilepsy (Doose syndrome). There are now a number of different diets that can be used for epilepsy: the Classic Ketogenic Diet, the Modified Atkins Diet, the Medium Chain Triglyceride Diet and the Low Glycemic Index Treatment Diet. The choice is made after the initial consultation, and depends on the epilepsy diagnosis, the child’s age and feeding habits, and family needs and preferences. Ketogenic diets are the treatment of choice for glucose transporter deficiency (GLUT1DS) and should be considered in pyruvate dehydrogenase deficiency (PDH) and other mitochondrial disorders, even when these disorders do not cause seizures. Diet therapy takes care and dedication, but it may offer children a better chance of seizure control than trying a new anticonvulsant drug. Unfortunately, we don’t yet have good predictors of whether a child will respond to diet therapy, so we usually recommend a trial of three to four months. About the ketogenic diet Known for more than a century, the ketogenic diet has recently come back into use for epilepsy and has been shown to be effective for many children when drugs fail. It can provide control of seizures for about 30 percent of children with epilepsy. In its strictest form, the ketogenic diet provides more than 90 percent of its calories through fat (as compared to the 25 to 40 percent usually recommended for children). When we burn fat for energy, rather than glucose from carbohydrates, we produce compounds known as ketone bodi Continue reading >>

Experience With The Ketogenic Diet In Infants
Abstract Objective. To evaluate the effectiveness, tolerability, and adverse effects of the ketogenic diet in infants with refractory epilepsy. Methods. A retrospective review of 32 infants who had been treated with the ketogenic diet at a large metropolitan institution. Results. Most infants (71%) were able to maintain strong ketosis. The overall effectiveness of the diet in infants was similar to that reported in the literature for older children; 19.4% became seizure-free, and an additional 35.5% had >50% reduction in seizure frequency. The diet was particularly effective for patients with infantile spasms/myoclonic seizures. There were concomitant reductions in antiepileptic medications. The majority of parents reported improvements in seizure frequency and in their child's behavior and function, particularly with respect to attention/alertness, activity level, and socialization. The diet generally was well-tolerated, and 96.4% maintained appropriate growth parameters. Adverse events, all reversible and occurring in one patient each, included renal stone, gastritis, ulcerative colitis, alteration of mentation, and hyperlipidemia. Conclusion. The ketogenic diet should be considered safe and effective treatment for infants with intractable seizures. Continue reading >>

Babies Thrive Under A Ketogenic Metabolism
Some people, even some scientists who study ketogenic metabolism, have the idea that ketogenesis is somehow abnormal, or exceptional; an adaptation for emergencies only. We disagree. One reason we think a ketogenic metabolism is normal and desirable, is that human newborns are in ketosis. Despite the moderate sugar content of human breast milk, breastfeeding is particularly ketogenic. This period of development is crucial, and there is extensive brain growth during it. Although the composition of breast milk can be affected by diet [1], it is reasonable to assume that breast milk has always been ketogenic, and this is not an effect of modernisation. When the brain is in its period of highest growth, and when the source of food is likely to be close to what it evolved to be for that period, ketones are used to fuel that growth. If nothing else, this suggests that learning is well supported by a ketogenic metabolism. It is also consistent with the ability of ketogenic diets to treat a variety of seemingly unrelated brain disorders and brain trauma. Newborn infants are in ketosis. This is their normal state. Breastfeeding is particularly ketogenic (compared to formula feeding). Breastfeeding longer (up to a point) is associated with better health outcomes. This suggests the hypothesis that weaning onto a ketogenic diet would be healthier than weaning onto a high-carb diet. (Mark-up ours) Human babies are in ketosis Soon after birth, human babies are in ketosis, and remain so while breastfeeding [2]. They use ketones and fats for energy and for brain growth. When this has been studied, in the first couple of hours after birth, babies aren't immediately in ketosis. There is a short delay [3]. During that brief period before ketogenesis starts, lactate (confusingly not to do Continue reading >>

The Ketogenic Diet In A Bottle?
Most parents and neurologists consider the ketogenic diet as only including heavy whipping cream, mayonnaise, butter, and oils. For most children receiving this therapy, they are correct. In order to create a solid food diet in which 90% of the calories are fat, these foods make up a large portion of the child’s usual intake. However, the ketogenic diet can alternatively be easily provided as a liquid-only formulation to both infants as well as children with gastrostomy tubes. To some parents this is a surprise, but many ketogenic diet experts have been utilizing these formulas for years with good results. What are these formulas? There are several ways to provide the ketogenic diet as a formula. Perhaps the most simple is to use Nutricia KetoCal™, which is available in a powdered mix and either a 3:1 or 4:1 ratio. Water is added and calories are calculated by a dietitian. The 4:1 KetoCal has a slight vanilla flavor; the 3:1 KetoCal is unflavored. These formulas are milk-based, include corn syrup solids, and are nutritionally complete. For children in whom separate components (e.g. fat, carbohydrate, and protein) are desired, or who have a lactose intolerance, “modular” ketogenic diets can be created using Ross Carbohydrate Free™ (Abbott Nutrition), Microlipid™ (Nestle Nutrition), and Polycose™ (Abbott Nutrition). A product in South Korea is also in clinical use called Ketonia™ has also been studied and is in active use, specifically for infantile spasms. A revised formulation called Ketonia II™ is now available without MCT (medium chain triglyceride) oil included. Lastly, Accera Pharmaceuticals, Inc. has developed a powdered MCT (medium chain triglyceride) oil-based powder (40 grams) that is designed to provide oral ketones to adults with Alzheimer’ Continue reading >>

What Is A Ketogenic Diet?
What Is a Ketogenic Diet? If medicine doesn't control seizures in epilepsy, sometimes doctors prescribe a ketogenic (or keto) diet. A ketogenic diet is a strict high-fat, low-carbohydrate diet that can reduce, and sometimes stop, seizures. It's called "ketogenic" because it makes ketones in the body. Ketones are made when the body uses fat for energy. By replacing carbs with fat in the diet, the body burns more fat and makes more ketones. The ketogenic diet is prescribed by a doctor. Kids on the diet need to be followed closely by a dietitian to make sure they follow the diet and get the nutrients they need. The diet starts with fasting during an overnight hospital stay. Who Needs a Ketogenic Diet? Children with seizures that are not well-controlled by medicines (called intractable epilepsy) and severe epilepsy syndromes (such as infantile spasms or Lennox-Gastaut syndrome) might benefit from a ketogenic diet. Studies show that the ketogenic diet also may help treat other conditions, such as diabetes, obesity, and even cancer. How Does a Ketogenic Diet Work? Although the ketogenic diet for epilepsy has been around since 1920, doctors aren't exactly sure how the higher ketone levels works. Some seizure types seem to respond better than others to the ketogenic diet. In babies, the keto diet is given in formula. Young children may be fed by a tube that is place in the stomach by a surgeon. This helps the child stay on the diet. How Long Do Kids Need a Ketogenic Diet? You should know if a ketogenic diet works for your child within a few months. If it does, your doctor may recommend weaning your child off the diet after 2 years of seizure control. The weaning process is done over several months to avoid triggering seizures. Some people stay on a ketogenic diet for years. Are Continue reading >>

Ketogenic Diet For The Youngest Patients
Since the ketogenic diet’s inception in the 1920s, it’s gradually become accepted as a mainstream method for treating intractable epilepsy in children. However, say pediatric neurologist Eric Kossoff and dietitian Zahava Turner, many specialists and parents don’t realize that the high-fat, moderate-protein, low-carbohydrate diet can be used in the very youngest patients, toddlers and even infants just a few months old. Though the ketogenic diet has proven enormously successful in reducing seizures in older children, Turner explains, doctors and parents had long assumed that babies and toddlers weren’t an ideal population for imposing such a restrictive regimen. “There’s been a fear of their ability to tolerate the diet and worries about their growth and development,” she says. A lack of commercial ketogenic foods on the market made administering the diet to very young patients exceedingly difficult, Turner adds. However, Kossoff says, research in the last several years has shown that the ketogenic diet can be safely given to babies and toddlers and can be effective, successfully cutting or eliminating seizures in many of these patients. With an abundance of new ketogenic prescription formulas now available, parents can administer the diet even to very young infants long before they’re eating solid food. An increasing number of specialized centers scattered around the country, including the Johns Hopkins Ketogenic Diet Center, now use the ketogenic diet as one of the first lines of treatment for pediatric epilepsy rather than a treatment of last resort. “Between a third and half of patients starting this diet are now under age 2,” Kossoff says. “That’s a big change compared to just 10 years ago.” Because the diet can have significant side effect Continue reading >>