What Every Pediatrician Should Know About the Ketogenic Diet

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Didn't know how many of you saw this article...

Barb Swoyer

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What Every Pediatrician Should Know About the Ketogenic Diet

M. Freeman, MDContemp Pediatr 20(5):113-127, 2003. © 2003 Medical Economics

Company, Inc.

Posted 06/03/2003

Introduction

After years of neglect, this method of managing hard-to-control seizures is

being used more often by pediatric neurologists. A working knowledge of the diet

will help you support patients and parents and avoid treatment mistakes that can

sabotage the diet's therapeutic effects.

The ketogenic diet is a high-fat, adequate-protein, very-low-carbohydrate diet

that pediatric neurologists are using increasingly to treat children with

difficult-to-control seizures. As use of the diet grows, pediatricians and

family practitioners are likely to see more patients who are on it at office

visits or in the emergency room. They should be familiar with the diet, its side

effects, and its role in seizure management. And every physician who sees a

patient on the diet should be aware of the cardinal rule of the ketogenic diet:

Give NO CARBOHYDRATES.

History of the Diet

In the early 1920s, the only effective medications available for seizures were

phenobarbital and bromides. An osteopath, Hugh Conklin, believing that seizures

originated in the intestines, " rested " the intestines of children with seizures

by having the child fast for 10 to 20 days. Fasting often resulted in remarkable

and long-lasting seizure control. The dramatic results were replicated by

others, and a high-fat, adequate-protein, limited-carbohydrate diet was devised

to simulate the effects of starvation.[1] In the absence of sufficient

carbohydrates, fats are incompletely metabolized, leaving residual ketone

bodies. This ketosis is believed to produce the anticonvulsant effects of the

ketogenic diet by mechanisms that remain unknown.[2]

The ketogenic diet was widely used during the 1930s -- until the discovery of

phenytoin in 1938. With the subsequent development of other anticonvulsant

medications, the diet was prescribed less often. Because physicians no longer

ordered the diet, dietitians were no longer trained in its use, and it was less

successful when it was used. The diet was revived in the early 1990s and is once

again recognized as an effective therapy for children whose seizures are

difficult to control.[3-6]

Nature of the Diet

The ketogenic diet is an alternative medical therapy for children with

difficult-to-control seizures. It must be undertaken only under medical

supervision and in collaboration with a well-trained dietitian who knows how to

monitor and adjust the diet.

The diet is carefully calculated to be nutritionally adequate for the individual

child. In it, 90% of the calculated calories are in the form of fat, with no

less than 1 g/kg of protein and minimal carbohydrates.[7] The standard ratio of

fat calories to carbohydrate and protein is 4:1. Younger children and

adolescents may be placed on a 3:1 ratio to allow more protein (1 to 1.5 g/kg)

for adequate growth. Caloric intake is calculated at 75% of the usual daily

requirement. Fluids are generally restricted to 80% of the usual amount. The

diet must be supplemented with vitamins and minerals. Infants and younger

children may be given more protein and, occasionally, even less carbohydrate

than older children are.

The dietitian carefully and repeatedly adjusts the diet by phone, e-mail, or

outpatient follow-up to achieve and maintain the child's ideal body weight for

height, maximize ketosis, and achieve optimal seizure control.[7] Children

typically return for follow-up at three months, six months, and one year after

starting the diet to assure adequate growth and nutrition and to identify and

correct problems.

Appropriate Use of the Diet

With rare exceptions, the ketogenic diet should not be the first treatment for a

child with seizures. At the s Hopkins Medical Institutions, we have largely

restricted the use of the diet to children who have difficult-to-control

seizures of any type -- that is, seizures that are not controlled by the first

two anticonvulsant medications tried. Most children started on the diet at s

Hopkins have more than two seizures per week (often, many each day) and have

failed at least two anticonvulsants. These restrictions are, admittedly,

arbitrary. We believe that taking pills is far easier than maintaining the diet,

and because anticonvulsants easily control most seizures, they should be tried

first. Atonic seizures, seizures associated with the Lennox-Gastaut syndrome,

and infantile spasms are often refractory to medication, and children with these

seizure types may be candidates for early use of the diet.

We are often asked to prescribe the diet " because it is more natural " than

anticonvulsant medication. The diet is not more natural than medication; it is

only an alternative medical therapy. It must be carefully calculated, adjusted

to the patient's needs, and adhered to rigorously. We have observed that eating

three or four extra macadamia nuts, for example, or even using a sunscreen

lotion containing sorbitol just once can make the difference between seizure

control and frequent seizures. The diet should be used ONLY under medical and

nutritional supervision. It should never be started or managed by the family

alone.

Does the Diet Work?

When the ketogenic diet was reintroduced in the 1990s, many were skeptical about

its usefulness at a time when an increasing number of new anticonvulsant

medications were available. Table 1 shows the results of recent studies of the

diet at s Hopkins.[4,5]

One hundred and fifty children with difficult-to-control seizures were started

on the diet. They were followed initially for one year[4] and subsequently for

three to six years.[5] The children had mixed seizure types, with different

causes, and varying levels of intellectual function. Their mean age was 5.3

years. They averaged 410 seizures a month at baseline and had failed a mean of

6.2 anticonvulsant medications before starting the diet.

Most (83%) children who started the diet stayed on it for three months.

Seventy-one percent remained on it for six months, and 55% remained on it for a

year. The diet's effects on seizure control were independent of patients'

intelligence and developmental delay. We have found that the diet is palatable

and tolerable if it is effective -- that is, if it either substantially

decreases the number of seizures or significantly reduces medication side

effects by permitting a decrease in anticonvulsants.

The ketogenic diet has been prescribed traditionally for children older than 1

year and was long thought not to be tolerated by teenagers or adults. Recent

studies, however, document that it is well tolerated and effective in children

as young as 3 months of age,[8] teenagers,[9] and, possibly, adults.[10]

Again, we don't know how the diet works. Research is ongoing.[2,11]

How Long Do Children Stay on the Diet?

We ask parents to continue the diet for a minimum of three months to allow the

child time to adjust to it, to modify recipes to meet the child's preferences,

and to allow time for us to modify the diet to achieve maximal ketosis and

optimal seizure control. During this time we often reduce or eliminate one or

more of the anticonvulsants the child is taking. After three months, parents and

child are free to continue or discontinue the diet.

As noted, 83% of children in the s Hopkins studies stayed on the diet for

the minimum three-month period and 55% stayed on it for one year. The major

reason for discontinuing the diet was that it was not sufficiently effective to

warrant the restrictions or the bother. (Children who started the studies having

failed multiple medications and having hundreds of seizures had a lower than 10%

chance of achieving seizure control.) If the diet was effective, it was well

tolerated. Note in the table that 21 children (14%) of the initial study

population had greater than 90% seizure reduction three to six years after

starting the diet. Most children discontinue the diet after two or three years.

Figure 1 shows examples of meals on the diet. Some sample ketogenic recipes are

shown below

Figure 1.

Concerns About the Diet

Reservations that have been expressed about the ketogenic diet include the

following: " That's not a healthy diet. They won't grow. They'll all get fat. "

Figure 2 shows growth on the ketogenic diet. Children's growth rate remains

within normal ranges, although younger children grow somewhat more slowly (along

the third percentile).[12] The child's weight is closely monitored and caloric

intake is adjusted to maintain ideal body weight. Calories are increased or

decreased depending on the child's activities and growth.[13,14] Normally active

children are maintained at close to the 50th percentile of weight for height.

Children with a substantial handicap are maintained at the 10th to 25th

percentile.

Figure 2.

" Everyone knows that a high-fat diet will elevate lipids and cause heart

disease. "

Figure 3 shows changes in cholesterol and other lipids while children were on

the ketogenic diet.[14,15] True, after six months on the diet plasma lipids rise

to levels that, if maintained, would raise concern about later atherosclerosis.

Few patients remain on the diet for more than two years, however.

Figure 3.

The mean cholesterol level becomes slightly elevated, but stabilizes at 220

mg/dL. Low-density-lipoproteins (LDLs) also stabilize, and triglycerides return

to baseline levels.[14,15] Because most children discontinue the ketogenic diet

within two years of starting it and return to a diet containing far less fat,

there is little reason to expect long-term adverse effects. This supposition has

not been studied, however. Parents should be made aware of potential adverse

effects of a high-fat diet so that they can weigh those risks against the risks

associated with continuing seizures and taking anticonvulsant medications.

Care of the Ill Child -- the Pediatrician's Role

Because the ketogenic diet is best started under the care of a team that

includes a pediatric neurologist and a dietitian with experience in managing

children on the diet, few pediatricians have sufficient demand or support to

develop expertise in initiating or managing the diet on their own. The

pediatrician does play an important role as part of the management team,

however. We see this role as following the child's health, growth, and

nutritional status. An additional major role, because the family often lives a

distance from the medical center where the ketogenic diet was prescribed, is

managing intercurrent illness. There is no evidence that children on the

ketogenic diet have more (or fewer) illnesses than their peers. When children

with a seizure disorder have an illness or fever, however, ketosis may decrease

and seizures return or increase.

When the parent of a child on the diet calls to tell us the child has had a

seizure or the seizures have become worse, the first questions we ask are, " Is

he ill? Does he have a fever? Is he coming down with something? " [13] Children

who are ill should be treated appropriately (see " Points to remember: Caring for

an ill child on the ketogenic diet " ). Except for using carbohydrate-free

medications, treatment is the same as for children who are not on the diet.

Vomiting, diarrhea, pneumonia, and other illnesses are rarely reasons to

discontinue the diet. If the sick child is not tolerating solid foods, ketogenic

diet eggnogs may provide nutrition (and may be diluted if necessary). Clear,

carbohydrate-free fluids may provide adequate hydration for a day or so. Diluted

oral rehydration solutions (e.g., Pedialyte diluted in half because of the

glucose content) may be used for a day to maintain electrolyte levels, if

needed. Severe vomiting and diarrhea may cause substantial acidosis (pH less

than 7.1) and dehydration. Intravenous hydration with saline solution (without

carbohydrate) is usually sufficient to return the child to normal balance.

Give No Carbohydrates

Carbohydrates -- including glucose, fructose, starches, and compounds ending

in -ol (sorbitol, mannitol) or -ose (maltose, zylose) -- are converted

completely or partially to glucose in the body, which can negate ketosis and

lead to seizures. Most pediatric medications contain carbohydrates (see " Watch

out for carbohydrates in medications " ).

When possible, vitamins and other medications taken regularly should be

carbohydrate-free. A compounding pharmacy is usually available to make such

medications when otherwise not available.

Side Effects of the Diet

The reported side effects in children on the ketogenic diet have been

described by Wheless.[16] The two most common effects are constipation and

kidney stones. Although death, dyslipidemia, cardiomyopathy, and trace mineral

deficiencies have been reported, the causal relationship of these side effects,

so to speak, to the diet remains unclear.Constipation

We have found that adding Miralax, MCT oil, or carbohydrate-free, liquid aloe

vera, which parents can usually get at a local pharmacy, is the most effective

way to manage constipation. Other classic remedies (if carbohydrate-free) are

sometimes useful. Increasing intake of fluids may help. The point is to use

whatever works for the child.[7,13]Kidney Stones

Kidney stones occur in 6% to 7% of children on the ketogenic diet. These are

usually calcium citrate or uric acid stones caused by precipitation of these

compounds in the concentrated, acidic urine.[17,18] They often are accompanied

by " grit " in the urine, abdominal discomfort, urinary tract infection, or an

otherwise unexplained increase in the frequency of the child's seizures.

We ask parents to test the child's urine weekly for blood using test strips

When urine is positive for blood on two consecutive tests, the pediatrician

performs a urinalysis to look for red blood cells. A positive microscopic

urinalysis is followed by a consultation with a urologist and ultrasonography of

the kidney. A spot urine specimen taken every three months is also tested for

the calcium/creatinine ratio, which should be less than 0.2. An elevated ratio,

or the presence of a stone, is treated by increasing fluid intake to 100% to

120% of the usual daily requirement and alkalinizing urine to pH 6.5 with

polycitrate solution (a solution containing sodium and potassium citrate, which

is readily available at pharmacies). Most stones dissolve, but persistent stones

may need ultrasound treatment or, rarely, surgery. Once a stone has been

treated, patients have remained successfully on the ketogenic diet with the

addition of liberalized fluids and polycitrate.

How Do You Know if the Diet Is Helping?

If the seizures are gone, and the medication side effects are reduced, the

diet is helping. If seizures continue to occur, although less often, and the

child is still taking medication, parents may ask, " Is the diet helping? " The

only way to determine whether the diet is helping is to test it by discontinuing

it to see if the frequency of seizures remains the same or increases. The

classic way to discontinue the diet is to taper the ratio of fat calories to

protein and carbohydrate very slowly, progressing every three to six months from

a 4:1 ratio to 3.5:1, then to 3:1, to 2.5:1, and to 2:1 -- and then

discontinuing the diet.

A more rapid way, which we often use but have not assessed carefully, is to

maintain the same meal plans but substitute regular milk for the cream in the

diet. This reduces the ratio from 4:1 to approximately 2.5:1. After one week, if

the frequency of the child's seizures remains unchanged, we switch from regular

milk to skimmed milk, which reduces the ratio to about 1.5:1 If the frequency

still remains unchanged, we discontinue the diet. The advantage of this approach

is that it maintains the restrictions of the diet during the test period -- the

child is not allowed to eat foods such as candy or potato chips -- so that it is

not difficult for the child to resume the regular ketogenic diet if necessary.

A third way to test the diet is to allow the child to cheat -- a little. If

nothing adverse happens, gradually introduce some carbohydrates into the child's

meals until he or she is no longer in ketosis.

Worth a Try

The ketogenic diet is used more and more in children whose seizures are

difficult to control.[19] When it is effective, it is well-tolerated by children

and parents. Pediatricians need to be aware of the diet -- especially its

carbohydrate limitations -- and be prepared to support the child and his (or

her) parents during intercurrent childhood illnesses as they struggle with

difficult seizure problems.

Successful use of the diet often permits a decrease in anticonvulsant

medications and, therefore, their side effects. When a child with

difficult-to-control seizures becomes seizure-free or virtually so, and is able

to decrease or eliminate the side effects of multiple medications, the diet is

effective. If, after two years, the child can discontinue the diet, has no

seizures, and requires no anticonvulsant medication, that is a very significant

achievement. Even when the diet does not prove effective, most parents are

grateful to have given it a chance.

Tables

Table 1. Outcomes of the ketogenic diet at s Hopkins Medical Institutions

Time from start of the ketogenic diet*Seizure control

Seizure-free N (N/150)>90% reduction N (N/150)50%-90% reduction N (N/150)<50%

reduction N (N/150)Patients maintained on the diet N (N/150)

3 mo4 (3%)46 (31%)39 (26%)36 (24%)125 (83%)

6 mo5 (3%)43 (29%)29 (19%)29 (19%)106 (71%)

12 mo11 (7%)30 (20%)34 (23%)8 (5%)83 (55%)

3-6 yr20 (13%)21 (14%)24 (17%)18 (12%)15 (10%)

*150 patients started the diet.

References

Swink TD, Vining EPG, Freeman JM: The ketogenic diet 1996. Adv Pediatr

1997;44:297

Schwartzkroin PA: Mechanisms underlying the anti-epileptic efficacy of the

ketogenic diet. Epilepsy Res 1999;37:171

LeFever F, Aronson N: Ketogenic diet for the treatment of refractory epilepsy

in children: A systematic review of efficacy. Pediatrics 2000;105(4)URL:

http://www.pediatrics.org/cgi/content/full/105/4/e46 .

Freeman JM, Vining EPG, Pillas DJ, et al: The efficacy of the ketogenic diet

-- 1998: A prospective evaluation of intervention in 150 children. Pediatrics

1998;102:1358

Hemingway C, Freeman JM, Pillas DJ, et al: The ketogenic diet: A 3-6-year

follow-up of 150 children prospectively enrolled. Pediatrics 2001;108:898

Freeman JM, Vining EPG: Seizures rapidly decrease after fasting: Preliminary

studies of the ketogenic diet. Arch Pediatr Adolesc Med 1999;153:946

Freeman JM, Freeman JB, MT: The Ketogenic Diet: A Treatment of Epilepsy,

ed 3. New York, Demos Press, 2001

Kossoff EH, Pyzik PL, McGrogan JR, et al: Efficacy of the ketogenic diet for

infantile spasms. Pediatrics 2002;109(5):780

Mady MA, Kossoff EH, McGregor AL, et al: The ketogenic diet: Adolescents can

do it too. Epilepsia (in press)

Sirven J, Whedon B, Caplan D, et al: The ketogenic diet for intractable

epilepsy in adults: Preliminary results. Epilepsia 1999;40(12):1721

Stafstrom CE, Spencer S: The ketogenic diet: A therapy in search of an

explanation. Neurology 2000;54:282

Vining EPG, Pyzik P, McGrogan J, et al: Growth of children on the ketogenic

diet. Dev Med Child Neurol 2002;44:796

Casey JC, McGrogan J, Pillas D, et al: The implementation and maintenance of

the ketogenic diet in children. J Neurosci Nurs 1999;31:294

Vining EPG, Kwiterovitch P, Freeman JM, et al: The effect of the ketogenic

diet on plasma cholesterol. Presented at the Annual Meeting of the American

Epilepsy Society, San Francisco, December 1996. Epilepsia 1996;37(Suppl 5):107

Kwiterovitch P, Vining EPG, Goodman S, et al: Changing lipid profiles after

institution of the ketogenic diet. (in preparation)

Wheless JW: The ketogenic diet: An effective medical therapy with

side-effects. J Child Neurol 2001;16:633

Furth SL, Casey JC, Pyzik PL, et al: Risk factors for urolithiasis in children

on the ketogenic diet. Pediatr Nephrol 2000;15:126

Kossoff EH, Pyzik PL, Furth SL, et al: Kidney stones, carbonic anhydrase

inhibitors, and the ketogenic diet. Epilepsia 2002;43(10):1168

Hemingway C, Pyzik PL, Freeman JM: Changing physician attitudes toward the

ketogenic diet: A parent-centered approach to physician education about a

medication alternative. Epilepsy and Behavior 2001;2:574

Sidebar: Points to Remember: Caring for an Ill Child on the Ketogenic Diet

If antibiotics are required, they must be carbohydrate-free. Oral or

intravenous fluids also must be carbohydrate-free.

Children on the diet usually have mild acidosis (pH, 7.15 to 7.3). The serum

bicarbonate level is usually approximately 15 mEq/L. Such acidosis is not a

concern. Corrective measures are needed only if the pH is less than 7, or the

bicarbonate is less than 10 mEq/L.

Blood glucose usually runs in the 60s (mg/dL), and that level is well

tolerated. Ketones in the urine should be 3+ or 4+.

If the child is critically ill and has previously benefited from the ketogenic

diet, the diet may, if absolutely necessary, be broken with glucose until the

illness abates. The diet can then be reinstituted, starting with one third of

the diet and progressing with daily increases to the previous full diet -- that

is, if the child is getting 1,000 cal in three meals (330 cal/meal), reinstitute

the diet at 110 cal/meal, then progress to 220 cal/meal and finally 330

cal/meal.

Sidebar: Ketogenic Recipes

Peanut Butter " Sandwich "

27 g egg white

13 g cottage cheese (4% fat)

26 g butter

4 g Skippy peanut butter

1/4 tsp cream of tartar

Sweetener

Whip egg white until peaks form. Add cottage cheese, cream of tartar, and a

couple of drops of sweetener. Place two piles of the egg white mixture onto a

cookie sheet sprayed with Pam. Bake at 350° for 20 to 25 minutes (until lightly

browned). When the baked egg whites have cooled, mix butter and peanut butter

together and spread on the egg whites to make a " sandwich. " To make bagels, make

holes in the egg white piles on the cookie sheet before baking.

The sandwich plus 30 g of cream as a drink makes a 345-calorie meal with a 4:1

ratio of fat to protein and carbohydrate.Keto Cookies

2 egg whites

1 tsp cream of tartar

1 small package of sugar-free Jello

Beat egg whites until stiff. Add cream of tartar and Jello. Place on nonstick

baking sheet and bake at 325° for 6 to 8 min or until brown. Makes 20 cookies.

One serving = 2 cookies.

Sidebar: Watch Out for Carbohydrates in Medications

Do not give IV fluids containing glucose. Give 50% normal saline or normal

saline instead.

Most liquid children's medications are sweetened with a carbohydrate

component. Such medications may negate ketosis and lead to seizures.

Even sunscreen lotions, other skin lotions, and shampoos often contain

sorbitol, which may be absorbed through the skin and negate ketosis.

Funding Information

M. Freeman, MD has nothing to disclose in regard to affiliations with, or

financial interests in, any organization that may have an interest in any part

of this article.

M. Freeman, MD, Lederer Professor of Pediatric Epilepsy, s Hopkins

Medical Institutions, Baltimore, Md.

[Guest guest]

Can you copy and paste the article in a post? It says we have to log in to read

it.

Thanks,

[Guest guest]

Wow, very interesting Barb! Thanks for sending this in! I think I should

get my neurologists email address so I can send her this as well as her

nurse! LOL! Well I gave them the information had sent me so

HOPEFULLY they comply. I'll keep you posted )

Halina

What Every Pediatrician Should Know About the Ketogenic

Diet

> Didn't know how many of you saw this article...

> Barb Swoyer

>

> What Every Pediatrician Should Know About the Ketogenic Diet

>

> M. Freeman, MDContemp Pediatr 20(5):113-127, 2003. © 2003 Medical

Economics Company, Inc.

>

> Posted 06/03/2003

> Introduction

>

> After years of neglect, this method of managing hard-to-control seizures

is being used more often by pediatric neurologists. A working knowledge of

the diet will help you support patients and parents and avoid treatment

mistakes that can sabotage the diet's therapeutic effects.

>

> The ketogenic diet is a high-fat, adequate-protein, very-low-carbohydrate

diet that pediatric neurologists are using increasingly to treat children

with difficult-to-control seizures. As use of the diet grows, pediatricians

and family practitioners are likely to see more patients who are on it at

office visits or in the emergency room. They should be familiar with the

diet, its side effects, and its role in seizure management. And every

physician who sees a patient on the diet should be aware of the cardinal

rule of the ketogenic diet: Give NO CARBOHYDRATES.

>

> History of the Diet

>

> In the early 1920s, the only effective medications available for seizures

were phenobarbital and bromides. An osteopath, Hugh Conklin, believing that

seizures originated in the intestines, " rested " the intestines of children

with seizures by having the child fast for 10 to 20 days. Fasting often

resulted in remarkable and long-lasting seizure control. The dramatic

results were replicated by others, and a high-fat, adequate-protein,

limited-carbohydrate diet was devised to simulate the effects of

starvation.[1] In the absence of sufficient carbohydrates, fats are

incompletely metabolized, leaving residual ketone bodies. This ketosis is

believed to produce the anticonvulsant effects of the ketogenic diet by

mechanisms that remain unknown.[2]

>

> The ketogenic diet was widely used during the 1930s -- until the discovery

of phenytoin in 1938. With the subsequent development of other

anticonvulsant medications, the diet was prescribed less often. Because

physicians no longer ordered the diet, dietitians were no longer trained in

its use, and it was less successful when it was used. The diet was revived

in the early 1990s and is once again recognized as an effective therapy for

children whose seizures are difficult to control.[3-6]

>

> Nature of the Diet

>

> The ketogenic diet is an alternative medical therapy for children with

difficult-to-control seizures. It must be undertaken only under medical

supervision and in collaboration with a well-trained dietitian who knows how

to monitor and adjust the diet.

>

> The diet is carefully calculated to be nutritionally adequate for the

individual child. In it, 90% of the calculated calories are in the form of

fat, with no less than 1 g/kg of protein and minimal carbohydrates.[7] The

standard ratio of fat calories to carbohydrate and protein is 4:1. Younger

children and adolescents may be placed on a 3:1 ratio to allow more protein

(1 to 1.5 g/kg) for adequate growth. Caloric intake is calculated at 75% of

the usual daily requirement. Fluids are generally restricted to 80% of the

usual amount. The diet must be supplemented with vitamins and minerals.

Infants and younger children may be given more protein and, occasionally,

even less carbohydrate than older children are.

>

> The dietitian carefully and repeatedly adjusts the diet by phone, e-mail,

or outpatient follow-up to achieve and maintain the child's ideal body

weight for height, maximize ketosis, and achieve optimal seizure control.[7]

Children typically return for follow-up at three months, six months, and one

year after starting the diet to assure adequate growth and nutrition and to

identify and correct problems.

>

> Appropriate Use of the Diet

> With rare exceptions, the ketogenic diet should not be the first treatment

for a child with seizures. At the s Hopkins Medical Institutions, we

have largely restricted the use of the diet to children who have

difficult-to-control seizures of any type -- that is, seizures that are not

controlled by the first two anticonvulsant medications tried. Most children

started on the diet at s Hopkins have more than two seizures per week

(often, many each day) and have failed at least two anticonvulsants. These

restrictions are, admittedly, arbitrary. We believe that taking pills is far

easier than maintaining the diet, and because anticonvulsants easily control

most seizures, they should be tried first. Atonic seizures, seizures

associated with the Lennox-Gastaut syndrome, and infantile spasms are often

refractory to medication, and children with these seizure types may be

candidates for early use of the diet.

>

> We are often asked to prescribe the diet " because it is more natural " than

anticonvulsant medication. The diet is not more natural than medication; it

is only an alternative medical therapy. It must be carefully calculated,

adjusted to the patient's needs, and adhered to rigorously. We have observed

that eating three or four extra macadamia nuts, for example, or even using a

sunscreen lotion containing sorbitol just once can make the difference

between seizure control and frequent seizures. The diet should be used ONLY

under medical and nutritional supervision. It should never be started or

managed by the family alone.

>

> Does the Diet Work?

>

> When the ketogenic diet was reintroduced in the 1990s, many were skeptical

about its usefulness at a time when an increasing number of new

anticonvulsant medications were available. Table 1 shows the results of

recent studies of the diet at s Hopkins.[4,5]

>

> One hundred and fifty children with difficult-to-control seizures were

started on the diet. They were followed initially for one year[4] and

subsequently for three to six years.[5] The children had mixed seizure

types, with different causes, and varying levels of intellectual function.

Their mean age was 5.3 years. They averaged 410 seizures a month at baseline

and had failed a mean of 6.2 anticonvulsant medications before starting the

diet.

>

> Most (83%) children who started the diet stayed on it for three months.

Seventy-one percent remained on it for six months, and 55% remained on it

for a year. The diet's effects on seizure control were independent of

patients' intelligence and developmental delay. We have found that the diet

is palatable and tolerable if it is effective -- that is, if it either

substantially decreases the number of seizures or significantly reduces

medication side effects by permitting a decrease in anticonvulsants.

>

> The ketogenic diet has been prescribed traditionally for children older

than 1 year and was long thought not to be tolerated by teenagers or adults.

Recent studies, however, document that it is well tolerated and effective in

children as young as 3 months of age,[8] teenagers,[9] and, possibly,

adults.[10]

>

> Again, we don't know how the diet works. Research is ongoing.[2,11]

>

> How Long Do Children Stay on the Diet?

>

> We ask parents to continue the diet for a minimum of three months to allow

the child time to adjust to it, to modify recipes to meet the child's

preferences, and to allow time for us to modify the diet to achieve maximal

ketosis and optimal seizure control. During this time we often reduce or

eliminate one or more of the anticonvulsants the child is taking. After

three months, parents and child are free to continue or discontinue the

diet.

>

> As noted, 83% of children in the s Hopkins studies stayed on the diet

for the minimum three-month period and 55% stayed on it for one year. The

major reason for discontinuing the diet was that it was not sufficiently

effective to warrant the restrictions or the bother. (Children who started

the studies having failed multiple medications and having hundreds of

seizures had a lower than 10% chance of achieving seizure control.) If the

diet was effective, it was well tolerated. Note in the table that 21

children (14%) of the initial study population had greater than 90% seizure

reduction three to six years after starting the diet. Most children

discontinue the diet after two or three years. Figure 1 shows examples of

meals on the diet. Some sample ketogenic recipes are shown below

>

>

>

> Figure 1.

> Concerns About the Diet

>

> Reservations that have been expressed about the ketogenic diet include

the following: " That's not a healthy diet. They won't grow. They'll all get

fat. "

> Figure 2 shows growth on the ketogenic diet. Children's growth rate

remains within normal ranges, although younger children grow somewhat more

slowly (along the third percentile).[12] The child's weight is closely

monitored and caloric intake is adjusted to maintain ideal body weight.

Calories are increased or decreased depending on the child's activities and

growth.[13,14] Normally active children are maintained at close to the 50th

percentile of weight for height. Children with a substantial handicap are

maintained at the 10th to 25th percentile.

>

>

>

>

> Figure 2.

> " Everyone knows that a high-fat diet will elevate lipids and cause heart

disease. "

> Figure 3 shows changes in cholesterol and other lipids while children were

on the ketogenic diet.[14,15] True, after six months on the diet plasma

lipids rise to levels that, if maintained, would raise concern about later

atherosclerosis. Few patients remain on the diet for more than two years,

however.

>

>

>

>

>

>

>

>

>

>

> Figure 3.

>

> The mean cholesterol level becomes slightly elevated, but stabilizes at

220 mg/dL. Low-density-lipoproteins (LDLs) also stabilize, and triglycerides

return to baseline levels.[14,15] Because most children discontinue the

ketogenic diet within two years of starting it and return to a diet

containing far less fat, there is little reason to expect long-term adverse

effects. This supposition has not been studied, however. Parents should be

made aware of potential adverse effects of a high-fat diet so that they can

weigh those risks against the risks associated with continuing seizures and

taking anticonvulsant medications.

>

> Care of the Ill Child -- the Pediatrician's Role

>

> Because the ketogenic diet is best started under the care of a team that

includes a pediatric neurologist and a dietitian with experience in managing

children on the diet, few pediatricians have sufficient demand or support to

develop expertise in initiating or managing the diet on their own. The

pediatrician does play an important role as part of the management team,

however. We see this role as following the child's health, growth, and

nutritional status. An additional major role, because the family often lives

a distance from the medical center where the ketogenic diet was prescribed,

is managing intercurrent illness. There is no evidence that children on the

ketogenic diet have more (or fewer) illnesses than their peers. When

children with a seizure disorder have an illness or fever, however, ketosis

may decrease and seizures return or increase.

> When the parent of a child on the diet calls to tell us the child has

had a seizure or the seizures have become worse, the first questions we ask

are, " Is he ill? Does he have a fever? Is he coming down with

something? " [13] Children who are ill should be treated appropriately (see

" Points to remember: Caring for an ill child on the ketogenic diet " ). Except

for using carbohydrate-free medications, treatment is the same as for

children who are not on the diet.

> Vomiting, diarrhea, pneumonia, and other illnesses are rarely reasons to

discontinue the diet. If the sick child is not tolerating solid foods,

ketogenic diet eggnogs may provide nutrition (and may be diluted if

necessary). Clear, carbohydrate-free fluids may provide adequate hydration

for a day or so. Diluted oral rehydration solutions (e.g., Pedialyte diluted

in half because of the glucose content) may be used for a day to maintain

electrolyte levels, if needed. Severe vomiting and diarrhea may cause

substantial acidosis (pH less than 7.1) and dehydration. Intravenous

hydration with saline solution (without carbohydrate) is usually sufficient

to return the child to normal balance.

>

> Give No Carbohydrates

>

> Carbohydrates -- including glucose, fructose, starches, and compounds

ending in -ol (sorbitol, mannitol) or -ose (maltose, zylose) -- are

converted completely or partially to glucose in the body, which can negate

ketosis and lead to seizures. Most pediatric medications contain

carbohydrates (see " Watch out for carbohydrates in medications " ).

> When possible, vitamins and other medications taken regularly should be

carbohydrate-free. A compounding pharmacy is usually available to make such

medications when otherwise not available.

>

> Side Effects of the Diet

>

> The reported side effects in children on the ketogenic diet have been

described by Wheless.[16] The two most common effects are constipation and

kidney stones. Although death, dyslipidemia, cardiomyopathy, and trace

mineral deficiencies have been reported, the causal relationship of these

side effects, so to speak, to the diet remains unclear.Constipation

> We have found that adding Miralax, MCT oil, or carbohydrate-free, liquid

aloe vera, which parents can usually get at a local pharmacy, is the most

effective way to manage constipation. Other classic remedies (if

carbohydrate-free) are sometimes useful. Increasing intake of fluids may

help. The point is to use whatever works for the child.[7,13]Kidney Stones

> Kidney stones occur in 6% to 7% of children on the ketogenic diet. These

are usually calcium citrate or uric acid stones caused by precipitation of

these compounds in the concentrated, acidic urine.[17,18] They often are

accompanied by " grit " in the urine, abdominal discomfort, urinary tract

infection, or an otherwise unexplained increase in the frequency of the

child's seizures.

> We ask parents to test the child's urine weekly for blood using test

strips When urine is positive for blood on two consecutive tests, the

pediatrician performs a urinalysis to look for red blood cells. A positive

microscopic urinalysis is followed by a consultation with a urologist and

ultrasonography of the kidney. A spot urine specimen taken every three

months is also tested for the calcium/creatinine ratio, which should be less

than 0.2. An elevated ratio, or the presence of a stone, is treated by

increasing fluid intake to 100% to 120% of the usual daily requirement and

alkalinizing urine to pH 6.5 with polycitrate solution (a solution

containing sodium and potassium citrate, which is readily available at

pharmacies). Most stones dissolve, but persistent stones may need ultrasound

treatment or, rarely, surgery. Once a stone has been treated, patients have

remained successfully on the ketogenic diet with the addition of liberalized

fluids and polycitrate.

>

> How Do You Know if the Diet Is Helping?

>

> If the seizures are gone, and the medication side effects are reduced,

the diet is helping. If seizures continue to occur, although less often, and

the child is still taking medication, parents may ask, " Is the diet

helping? " The only way to determine whether the diet is helping is to test

it by discontinuing it to see if the frequency of seizures remains the same

or increases. The classic way to discontinue the diet is to taper the ratio

of fat calories to protein and carbohydrate very slowly, progressing every

three to six months from a 4:1 ratio to 3.5:1, then to 3:1, to 2.5:1, and to

2:1 -- and then discontinuing the diet.

> A more rapid way, which we often use but have not assessed carefully, is

to maintain the same meal plans but substitute regular milk for the cream in

the diet. This reduces the ratio from 4:1 to approximately 2.5:1. After one

week, if the frequency of the child's seizures remains unchanged, we switch

from regular milk to skimmed milk, which reduces the ratio to about 1.5:1 If

the frequency still remains unchanged, we discontinue the diet. The

advantage of this approach is that it maintains the restrictions of the diet

during the test period -- the child is not allowed to eat foods such as

candy or potato chips -- so that it is not difficult for the child to resume

the regular ketogenic diet if necessary.

> A third way to test the diet is to allow the child to cheat -- a little.

If nothing adverse happens, gradually introduce some carbohydrates into the

child's meals until he or she is no longer in ketosis.

>

> Worth a Try

>

> The ketogenic diet is used more and more in children whose seizures are

difficult to control.[19] When it is effective, it is well-tolerated by

children and parents. Pediatricians need to be aware of the diet --

especially its carbohydrate limitations -- and be prepared to support the

child and his (or her) parents during intercurrent childhood illnesses as

they struggle with difficult seizure problems.

> Successful use of the diet often permits a decrease in anticonvulsant

medications and, therefore, their side effects. When a child with

difficult-to-control seizures becomes seizure-free or virtually so, and is

able to decrease or eliminate the side effects of multiple medications, the

diet is effective. If, after two years, the child can discontinue the diet,

has no seizures, and requires no anticonvulsant medication, that is a very

significant achievement. Even when the diet does not prove effective, most

parents are grateful to have given it a chance.

>

> Tables

>

> Table 1. Outcomes of the ketogenic diet at s Hopkins Medical

Institutions

>

> Time from start of the ketogenic diet*Seizure control

> Seizure-free N (N/150)>90% reduction N (N/150)50%-90% reduction N

(N/150)<50% reduction N (N/150)Patients maintained on the diet N (N/150)

> 3 mo4 (3%)46 (31%)39 (26%)36 (24%)125 (83%)

> 6 mo5 (3%)43 (29%)29 (19%)29 (19%)106 (71%)

> 12 mo11 (7%)30 (20%)34 (23%)8 (5%)83 (55%)

> 3-6 yr20 (13%)21 (14%)24 (17%)18 (12%)15 (10%)

>

> *150 patients started the diet.

>

>

> References

>

> Swink TD, Vining EPG, Freeman JM: The ketogenic diet 1996. Adv Pediatr

1997;44:297

> Schwartzkroin PA: Mechanisms underlying the anti-epileptic efficacy of

the ketogenic diet. Epilepsy Res 1999;37:171

> LeFever F, Aronson N: Ketogenic diet for the treatment of refractory

epilepsy in children: A systematic review of efficacy. Pediatrics

2000;105(4)URL: http://www.pediatrics.org/cgi/content/full/105/4/e46 .

> Freeman JM, Vining EPG, Pillas DJ, et al: The efficacy of the ketogenic

diet -- 1998: A prospective evaluation of intervention in 150 children.

Pediatrics 1998;102:1358

> Hemingway C, Freeman JM, Pillas DJ, et al: The ketogenic diet: A

3-6-year follow-up of 150 children prospectively enrolled. Pediatrics

2001;108:898

> Freeman JM, Vining EPG: Seizures rapidly decrease after fasting:

Preliminary studies of the ketogenic diet. Arch Pediatr Adolesc Med

1999;153:946

> Freeman JM, Freeman JB, MT: The Ketogenic Diet: A Treatment of

Epilepsy, ed 3. New York, Demos Press, 2001

> Kossoff EH, Pyzik PL, McGrogan JR, et al: Efficacy of the ketogenic diet

for infantile spasms. Pediatrics 2002;109(5):780

> Mady MA, Kossoff EH, McGregor AL, et al: The ketogenic diet: Adolescents

can do it too. Epilepsia (in press)

> Sirven J, Whedon B, Caplan D, et al: The ketogenic diet for intractable

epilepsy in adults: Preliminary results. Epilepsia 1999;40(12):1721

> Stafstrom CE, Spencer S: The ketogenic diet: A therapy in search of an

explanation. Neurology 2000;54:282

> Vining EPG, Pyzik P, McGrogan J, et al: Growth of children on the

ketogenic diet. Dev Med Child Neurol 2002;44:796

> Casey JC, McGrogan J, Pillas D, et al: The implementation and

maintenance of the ketogenic diet in children. J Neurosci Nurs 1999;31:294

> Vining EPG, Kwiterovitch P, Freeman JM, et al: The effect of the

ketogenic diet on plasma cholesterol. Presented at the Annual Meeting of the

American Epilepsy Society, San Francisco, December 1996. Epilepsia

1996;37(Suppl 5):107

> Kwiterovitch P, Vining EPG, Goodman S, et al: Changing lipid profiles

after institution of the ketogenic diet. (in preparation)

> Wheless JW: The ketogenic diet: An effective medical therapy with

side-effects. J Child Neurol 2001;16:633

> Furth SL, Casey JC, Pyzik PL, et al: Risk factors for urolithiasis in

children on the ketogenic diet. Pediatr Nephrol 2000;15:126

> Kossoff EH, Pyzik PL, Furth SL, et al: Kidney stones, carbonic anhydrase

inhibitors, and the ketogenic diet. Epilepsia 2002;43(10):1168

> Hemingway C, Pyzik PL, Freeman JM: Changing physician attitudes toward

the ketogenic diet: A parent-centered approach to physician education about

a medication alternative. Epilepsy and Behavior 2001;2:574

> Sidebar: Points to Remember: Caring for an Ill Child on the Ketogenic

Diet

>

> If antibiotics are required, they must be carbohydrate-free. Oral or

intravenous fluids also must be carbohydrate-free.

>

> Children on the diet usually have mild acidosis (pH, 7.15 to 7.3). The

serum bicarbonate level is usually approximately 15 mEq/L. Such acidosis is

not a concern. Corrective measures are needed only if the pH is less than 7,

or the bicarbonate is less than 10 mEq/L.

>

> Blood glucose usually runs in the 60s (mg/dL), and that level is well

tolerated. Ketones in the urine should be 3+ or 4+.

>

> If the child is critically ill and has previously benefited from the

ketogenic diet, the diet may, if absolutely necessary, be broken with

glucose until the illness abates. The diet can then be reinstituted,

starting with one third of the diet and progressing with daily increases to

the previous full diet -- that is, if the child is getting 1,000 cal in thre

e meals (330 cal/meal), reinstitute the diet at 110 cal/meal, then progress

to 220 cal/meal and finally 330 cal/meal.

>

>

>

> Sidebar: Ketogenic Recipes

>

> Peanut Butter " Sandwich "

> 27 g egg white

> 13 g cottage cheese (4% fat)

> 26 g butter

> 4 g Skippy peanut butter

> 1/4 tsp cream of tartar

> Sweetener

> Whip egg white until peaks form. Add cottage cheese, cream of tartar,

and a couple of drops of sweetener. Place two piles of the egg white mixture

onto a cookie sheet sprayed with Pam. Bake at 350° for 20 to 25 minutes

(until lightly browned). When the baked egg whites have cooled, mix butter

and peanut butter together and spread on the egg whites to make a

" sandwich. " To make bagels, make holes in the egg white piles on the cookie

sheet before baking.

> The sandwich plus 30 g of cream as a drink makes a 345-calorie meal with

a 4:1 ratio of fat to protein and carbohydrate.Keto Cookies

> 2 egg whites

> 1 tsp cream of tartar

> 1 small package of sugar-free Jello

> Beat egg whites until stiff. Add cream of tartar and Jello. Place on

nonstick baking sheet and bake at 325° for 6 to 8 min or until brown. Makes

20 cookies.

> One serving = 2 cookies.

>

> Sidebar: Watch Out for Carbohydrates in Medications

>

>

> Do not give IV fluids containing glucose. Give 50% normal saline or

normal saline instead.

>

>

> Most liquid children's medications are sweetened with a carbohydrate

component. Such medications may negate ketosis and lead to seizures.

>

>

> Even sunscreen lotions, other skin lotions, and shampoos often contain

sorbitol, which may be absorbed through the skin and negate ketosis.

>

>

>

> Funding Information

>

> M. Freeman, MD has nothing to disclose in regard to affiliations

with, or financial interests in, any organization that may have an interest

in any part of this article.

>

>

> M. Freeman, MD, Lederer Professor of Pediatric Epilepsy, s

Hopkins Medical Institutions, Baltimore, Md.

>

>

>

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>

>

[Guest guest]

, This came through the list last week.

What Every Pediatrician Should Know About the Ketogenic

Diet

Hey guys

Check out this very comprehensive new article on Medscape about the keto

diet. Its done by Freeman and is definitely one of better, maybe best, I

have seen, although it still has a few growling points with me

But there are even receipes (One I had never seen for a peanut butter

sandwich which I just might try for Jess) and pictures of the meals

(which don't look that unpalatable LOL) and under the section entitled

Sidebar: idebar: Points to Remember: Caring for an Ill Child on the

Ketogenic Diet, the degree of usual acidosis is explained.

Worth checking out.....and having around for the uneducated

What Every Pediatrician Should Know About the Ketogenic Diet

After years of neglect, this method of managing hard-to-control seizures

is being used more often by pediatric neurologists.

Contemp Pediatr 20(5) 2003

http://www.medscape.com/cmecenterdirectory/neurology/?src=webmd & dest=cme & banner=\

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