From the desk of "Dr" Walcutt
Comments on everything from psychology to valproic acid, in Isabel's inimitable style.
I know of three sound, safe, proven therapeutic modalities for our kids:
It is clearly stated in the Freeman/Kelly book that ketosis is sedative in effect and there is a natural depression of the appetite. Being hungry, therefore, appears to be an indication of insufficient ketosis. Handling this is not always what dieticians do best! Inducing carbohydrate jags with high glycaemic index carbohydrates is clearly upsetting to the diet. The other point is that it is unwise to give flaxseed oil (omega-3) without any balancing omega-6. This is because the two groups are essentially in competition for the same sites. If there is a deficiency in omega-6, then you' could be exacerbating that by adding flaxseed oil.
Fine-tuning the diet means, among other things, adjusting calories to accommodate growth and/or increased activity. In Roberta's case our first dietician wanted her weight reduced from 122 to 114lbs. Roberta is active, 28 years old and 5'3". We started the diet at 1500 calories. With perfect hindsight, I see that the dietician should have maintained Roberta's weight at 124lbs (I had worked so hard feeding her a high fat diet for two years while we waited for someone to accept her on the ketogenic diet! It was miraculous for unthrifty Roberta to be at 124 lbs! She had reserves to use in case of illness.) The ketogenic diet/depakote /weight loss culminated in status. I stopped the depakote after a second near-status; and I've raised calories to 2500. I've talked to the dietician about gluconeogenesis kicking in at such a high calorie level, but she said what Millie Kelly says about ketone readings: it's not the measurement that counts. The bottom line is shaping the diet until seizure control is achieved. More calories, yes! Less depakote, yes! Step by step, shaping as you go. As for increasing protein, I strongly disagree. The diet is calculated to provide adequate protein to take care of metabolic needs. More low carbohydrate veggies would contribute significantly to improved over-all nutrition. But excess protein taxes a metabolism that is already in distress, and contributes significant additional acidity.
A pattern of early morning seizures (drop or other) as well as late afternoon break-through seizures probably indicate a depletion of nutrients necessary to CNS functioning. All of the following should be considered:
If all of the above fail, an increase in calories may be required, especially in the case of very active individuals and/or fast metabolisers. Also, the composition of meals should be examined for (a) acid/alkaline balance (b) inclusion of fresh, raw foods in hopes that their enzymes and micro-nutrients might be beneficial in promoting healthy metabolic functioning. Re acid/alkaline balance, the catch 22 on the ketogenic diet is that ketones can't tell us if they are dark because fats are being burned as per plan, or if the body is under assault from excessive seizure-provoking acidosis. I know that the acid/alkaline balancing of meals is not an orthodox western dietary concept. But I do believe that acid/alkaline balance in menu composition may turn out to be particularly relevant, in the case of the ketogenic diet.
If, like Roberta, he's a fast metaboliser and active, he'll need more calories than the norm. Remember this is a diet that must be adapted to the individual, Never the other way around. And there's no reason to feed foods of inferior nutritional value like bacon. Roberta has never done well on foods and beverages that contain additives. I don't know if she'd be seizure free and normal as she is now, if I were giving her the menus that our dietician came up with.
Fats and the heart
Re concern for heart health, I recently received a message that addresses this question well. The mother works at a CVD (coronary vascular disease) prevention centre. Before putting her child on the diet, she consulted a world-leader in preventive cardiology who was not the least bit concerned from a CVD perspective. "From a primary prevention perspective, he believes that a young person's body is much more effective processing fats than an adult's. From a secondary prevention perspective (rehabilitation), there is considerable research that has been completed showing that the effects of CVD can be reversed with changes in lifestyle, in particular diet."
Starting the diet
Don't you think the Mayo Clinic protocol makes sense? And it is the ‘home’ of the ketogenic diet, where it was developed by Dr Wilder in the 1920s. Dr Freeman ‘believes’ there should be an initial fast. If this is to screen for metabolic disease, it makes sense. Otherwise, what's the point? Isn't it more logical to gradually adjust the body to progressively higher ratios - isn't that what fine tuning is all about? Since a significant number of children develop kidney stones because of water restriction, is it not more prudent to initiate with water ad lib and then restrict only if necessary.
If ketones are dark, (a) you're seeing residue from ketones that have burned incompletely (b) you're seeing a marker for acidosis, which can cause seizures. Therefore dark ketones can be either a good sign or a bad sign. They tell nothing about what's going on in the blood and in the brain - which is where the meaningful action takes place. If ketones are light then (a) someone's cheating / seriously miscalculating (b) the diet is not ketogenic enough, so you modify ratio / calories (c) the body is incapable of making sufficient ketones - ie the body needs more carnitine to transport fat into the brain cells where ketones can be used to fuel it - these are the ketones that matter. As far as I'm concerned, scrutinising keto-stix is, for the most part, a waste of time.
Roberta (28 years) used to have the problems you describe: constipation, lack of sleep, not eating (to which I would add not drinking, which is even more serious). Before I joined this list, I wasn't even particularly aware of these problems, let alone focused on clearing them up. I now believe it is critical to address these problems with clear and resolute focus. Before the drug era, neurologists widely recognised constipation as a problem related to seizures. Many, many factors have contributed to Roberta's success, and clearing up her chronic constipation has been one of them. To do this:
Lighten up, nurse Cratchit! It's time to celebrate Christopher's birth month. Delegate everything to an army of accomplices. No more laundry for Mom! No more lonely nights for Dad! Feed Christopher eggnog, the most nutritious, most digestible ketogenic meal. I would put it in a baby bottle and cradle him and dance with him. I would feed it with an eyedropper. I would allow the process to take all morning if necessary. Explain to him what you're doing with every fibre of your body and soul. Fall in love with him, and start that process by courting your husband like mad - it is truly a magic circle. Be a vibrant part of Christopher's every waking moment, and never rouse him from sleep. No TV glaring and blaring until he's eating proper meals. That should rally the troops to your side! Create your own eggnog (frozen as ice cream, if you like), worthy of Julia Child. Lightly poach the white and leave the yolk raw. All the great ethnic food cultures recognised the exceptional properties of raw egg yolk. But get rid of negative thoughts. If salmonella is truly lurking in your kitchen, by all means poach the yolk as well. Simply blend it with the cream. No sweetener- cream is naturally sweet. Communicate to Christopher that the egg is good; that a happy hen laid it just for Christopher; that you sent your helpers off to find the best organic egg from the most dedicated purveyors of quality food. The cream we get from the Happy Cow in Vermont is pale yellow, from an organically certified herd, and bears little resemblance to super market cream. If Roberta were in Christopher's straits, I would locate a farm and move heaven and earth to obtain raw cream straight from the cow, or the goat
I think it's a good idea to keep the environment as pure as possible - no strong toiletries, no harsh household chemicals etc. But a child seizing when picked up by mom is most likely to be a question of sheer excitement. When I look back on Roberta's 27 years of neurological problems, I remember how hopeless I felt when everything in the world seemed to bring on a seizure. But I can see in retrospect that her seizures were always better or worse according to changes in diet.
Since it's possible to have seizures that don't show up on an EEG; since some people have ‘abnormal’ EEG's with normal CNS function etc. I invite you to join Roberta and me in our contempt for tracings that have contributed nothing to our understanding or our well-being. (Not to mention that four years ago we were incarcerated together for a whole week of monitoring that yielded absolutely nothing as far as we were concerned. Oh, the neurologists loved it. Dr Balaban-Gil at Montefiore, for one, was paid $7,000 just for reading the ##$@!%^! thing).
Drugs and the diet
We feel very strongly that anti epileptic drugs, as well as some other drugs, interfere with the diet. Our success has depended on:
Valproic acid (depakote)
I've tried to get information directly from both the technical and medical services divisions of Abbott (manufacturers of depakote), but the bottom line is they don't have any answers: they don't know how valproic acid works, let alone how it works in combination with the ketogenic diet. I was told they don't know if valproic acid generates ketones; but, as we know, keto-metabolites do wind up in the urine. Our 12 years experience with valproic acid tells me that too high doses of valproic acid culminate in acidosis/seizures. Is Clarissa's CO2 level checked regularly? If it is low, she is acidotic, needs fluids, and electrolytes should be balanced. Fluid deprivation in combination with valproic acid/ketogenic diet is a grave problem. (My own perception of acidosis is that it causes the body to seize as part of a mechanism to regain homeostasis).
As we decreased the dilantin, her depakote level got too high and she went into status. Our neurologist does not accept this explanation (he doesn't have an explanation), because he never saw a high level flagged in black and white on a piece of paper. It says in the PDR:
"The concomitant administration of valproic acid with drugs that exhibit extensive protein binding (e.g. aspirin, phenytoin (dilantin)) may result in alteration of serum concentrations."
It has been my experience with depakote (about 10 years) that it's very difficult to get a handle on the actual level. Roberta's levels taken over the years have never been particularly readable or useful. Our keto-nurse who has considerable prior experience with the diet elsewhere had told us to wean depakote first ( and I think I may have seen that advice posted here too). But the treating neurologist chose to wean first what we considered to be the least effective medication (dilantin). That is a logical approach, but I believe the keto-nurse is better informed in this instance. She says that Hopkins weans depakote first. Another thought - since Roberta's used to a cocktail of dilantin and depakote, I'm wondering whether they should be reduced together at the same time. Has anyone reduced two anti epileptic drugs in tandem?
Re depakote: anti epileptic drugs can aggravate biochemical imbalances that cause seizures. So we're all in a Catch 22 situation. How is it possible to evaluate the effects of the diet if the patient is on medication? How can this be resolved if the neurologist will reduce medication only if the seizures lessen? Personally, we found the diet and depakote to be pure hell!
My heart goes out to all those on the horns of the depakote dilemma. The worst case scenario plays out as follows: the neurologist won't lower valproic acid until the seizures diminish. But the seizures won't diminish until you stop the depakote - totally - because valproic acid, at smaller dosage levels, can interact adversely with the ketogenic diet. If the diet is being implemented with shaky nutritional and caloric support (which is the norm), it's just a question of time until the dieter is metabolically compromised and the diet declared a failure. It sounds as if there is little trust between you and your neurologists. It will take forthright communication on your part to restore necessary mutual trust. Explain how you feel about the diet; that, for you, the diet is your child's salvation.
Whereas barbiturates like phenolbarbitol have to be weaned slowly due to withdrawal effects, this is not the case for valproic acid, which is recognised to be a drug that can be weaned quickly.
Has anyone ever weaned drugs in tandem?. After all, they've been used as a ‘cocktail’. Why not wean them as such – ie, instead of reducing the ‘gin’ first until eliminated, and then the ‘vermouth’ etc, why not consistently treat the mixture as a unique substance to be reduced as a whole?
I have believed for some time that a better approach would be to wean multiple drugs in tandem. It's too late to try that approach, but I think it's clear that Elizabeth is reacting now to an altered level of neurontin. When drug ‘cocktails’ are used, withdrawal of one ingredient causes levels and/or potency of the other(s) to increase.
Drugs and learning
When you write that "it is highly unlikely that the medications would cause linguistic problems, only motor speech disorders (eg slurred speech)", perhaps a healthy shot of consciousness-raising is in order. Imagine someone taking a beginner's class in Japanese, and that you show up most days toxic from a controlled or uncontrolled substance of your choosing. In cases where ability to concentrate is diminished by a drug, learning cannot but be adversely impacted.
continue to References
return to Roberta's diet
(update 1.2: 18 July 2002)
(issue 1: 2 April 1998)