Aku Aku

ketogenic diet

Toni called to let me know that they were talking on NBC Dateline about that all-fat diet that supposedly has remarkable success for children with severe seizures like Kai (to see all posts about Kai's medical condition click the Category: Kai link above). I turned on the TV and caught the last few minutes where they showed a 10 year old child who had beaten his seizures through the diet and is now living a normal life, even avoiding the mental retardation that usually accompanies severe seizures as a baby.

I found a web version of the piece on MSNBC Dateline's web site: Diet as possible epilepsy treatment?. It tells the story of the father of the child from the TV show, most interestingly there is a brief interview with the medical doctor who tried to convince this father to NOT try the fat diet, despite the fact that its success rate has been known for 70 years.

Larson: “You had some knowledge that this diet was probably working at Johns Hopkins and yet you dissuaded Abraham’s from attempting it. How come?” Dr. Shields: “Well because I don’t think we had exhausted all of the medical approaches yet. There were actually still other medications that we hadn’t tried yet.” Larson: “Dr. Freeman tells us that 50 to 70 percent of patients that come through his doors and get put on the diet have success. can you think of any drugs in these hard cases that have 50 to 70 percent success rate?” Dr. Shields: “Probably not that comes up to that level.”

So why are modern doctors ignoring this diet? Charlie’s own doctor has a surprising answer.

Dr. Shields: “There is no big drug company behind ketogenic diet and there probably can never be unless somebody starts marketing sausage and eggs with with cream sauce on it as a drug.”
Larson: “You are saying in a sense one of the reasons the ketogenic diet is not popular at this point is because there is not a big drug company behind it selling it to the doctors?”
Dr. Shields: “I think that’s probably true. I hate to say that, but it’s probably true.”

I happen to work within the pharmaceutical industry, as a software vendor. Like most people, I suspected that the pharmaceutical and medical industries were putting profits ahead of health care well before I got into this line of work, but my 2+ years of experience within the industry has only strengthened this view. It's terribly depressing how corporate greed is affecting our world, but that's a lament for another post.

Kai is having good weeks and bad weeks with the current medicine (the third prescription they've tried). He went a month with only a handful of seizures per day, and those only at night when he was tired. The bad weeks can be very bad though, a couple of weeks ago he had to be fed via a tube through his nose for days because of re-occuring stomach problems (which they think are related to his brain condition), and the seizures increased again.

The doctors had told Eva that he will probably be mentally retarded and possibly unable to ever learn to walk or talk, but when I visited him last week I was overjoyed to see that he is starting to learn how to walk! He took about six shaky steps toward me, it was really just wonderful. He still seems to be a long way from talking, but it is apparent that the seizures produce a lot of stress on him that makes it difficult for him to develop mentally, and that when the seizures are better under control he is capable of making serious progress. When I saw that boy on the TV segment swimming, playing the piano and walking around living a normal life, I could not help but project Kai into that picture. If there is something that can help him and it is being witheld or dismissed because of fucking corporate greed, well that just makes me angry.

scanned photos

Eva had left some photos of Kai on our refrigerator and I decided to scan them in tonight. I think these were from last summer when he was 6-12 months old.

When I drove his grandmother to the INS office this morning she was saying that she was glad some people got to meet Kai before all this happened, so we could remember when he was just a happy sweet little boy with a whole world of possibility before him....

valproic acid

Talked to Eva tonight, she says the new medicine (the one that can cause liver, bone marrow and pancreas problems and is only supposed to be for older children) has had some good results. His atopic seizures are now down to 5 per day! However, the medicine makes him very agitated and aggressive. The active ingredient is valproic acid and she said the brand was depakote. When I looked up Depakote it was described as something for manic depressives. I'm not sure if it's the same thing or not though.

Tomorrow morning at 8:20AM I pick up Frau Jettmar at the CalTrain station so she can get her
visa extension from the INS offices downtown.

lennox-gastaut

I was spelling the name of Kai's condition wrong, which explains why I found only a smattering of information in a google search. It's actually spelled Lennox-Gastaut and there is much information about it available on the web. Here's a good overview of the syndrome: NINDS Lennox-Gastaut Syndrome Information Page.

It gives names to the different types of seizures:

• tonic (stiffening of the body, upward deviation of the eyes, dilation of the pupils, and altered respiratory patterns)
  
• atonic (brief loss of muscle tone and consciousness, causing abrupt falls)
  
• atypical absence (staring spells)
  
• myoclonic (sudden muscle jerks)

Actually it doesn't mention the tiny ones only the EEG picks up. But it looks like Kai's first seizure was the tonic variety, and that atonic seizures are the ones that have so far been uncontrollable. I think he had a myoclonic seizure when I was holding him the other day, but its hard for me to be sure.

lennox gasteaux

I just got off the phone with Eva. She is in the hospital with Kai again. Over the past day or two they were required to increase his dosage of medicine (it's part of the schedule to increase it over time) and his condition got much worse. He was vomiting, not eating/drinking and lethargic all of the time.

They are now switching him to a new medicine, but it is normally only used for older children so it is dangerous. Side effects of this medicine are bone marrow and liver damage.

Eva and her mother are Austrian citizens. Eva is here on a student visa as she finishes her PhD at Stanford. Frau Jettmar's visa runs out next week so she would have to go back to Austria for at least a week leaving Eva by herself. I had planned on going down as often as possible while her mom was away to help, because caring for Kai is a difficult task even for two people, but her mom has been granted an emergency visa extension by the INS. On Tuesday I will have to pick her up at the CalTrain station and take her to the San Francisco INS office so she can receive the new visa.

Eva tell me they have now decided which label to give Kai's condition. It is called Lennox Gasteaux, and this is apparently the label they give when they don't know what else to call it. I've tried searching for information on the Internet but so far haven't turned up much (1,2). One article I found paints a bleak picture:

About 20 miles from Auburn's campus a freckled, six-year-old boy named Michael Barlett cannot speak, communicate or even feed himself.

Supervising Michael has become a 24 hours-a-day job for Sally and her husband since they received custody of him four years ago. "You feed him. You do everything for him," Sally said. "The only thing he does is walk and hold his own sippy cup. He is not like a regular kid where you can just open the door up and let him run outside and play in the front yard. He doesn't comprehend 'I can't go out into the road.' He just wanders."

But I do find it encouraging that at least that child learned to walk.

I'm concerned about Kai's ability to walk. Obviously avoiding a life strapped to a wheelchair would be a good thing, so earlier today I posted a request on craigslist asking for any old unused baby walkers:

I'm hoping to try to design a safe walker for him, where he could wear a torso harness over his clothing which is suspended (perhaps via bungee cords?) from a support structure attached to the walker. I'm hoping a couple of kind people could donate an old walker for me to experiment with.

I'm surprised at the dearth of information on Lennox Gasteaux on the Internet. I will certainly start posting anything I find out about this disease here. I also have copied one article which only exists in the google cache to my website (here), this one talks about a high fat diet which has met with some success in controlling seizures in otherwise untreatable children.

kai

There has been something besides work weighing heavily on me for the past few weeks. Kai Jettmar is 16 months old. About a month ago he had his first seizure, a massive one, which nearly killed him.

Kai is the son of my ex-girlfriend Eva and she has been raising him without the benefit of Kai's father's assistance (other than some monetary assistance after the Child Support Services tracked him down in Seattle). I always felt sorry for Kai, as he would have to grow up without a father. I've looked forward to helping fill that void in some small way by being "Uncle Dav".

He spent nearly two weeks at the Lucile Packard Children's Hospital at Stanford, which is about the best place on the planet for him to be. I am told they have one of the two most respected neurology departments in the world (the other one being Harvard). So he is getting the best care possible. Unfortunately his condition is one of the worst, and so far nothing has really worked. Kai has three types of seizures now. The major ones, like the first one which left him unconscious and not breathing (his mother had to give him mouth to mouth) and the tiny ones that are the most frequent but are only detectable by EEG, have been brought under control through a medicinal regimen. However the side effects of the medicine cause him to be so drowsy that he is rarely full cognizant.

While in the hospital he was so out of it he was unable to eat or drink, so he had to be fed intravenously. The first two times I went to visit him he only woke up once, and that was to scream out in pain when the nurse had to insert a new IV. It nearly shattered me. I don’t know if I’ve ever felt so helpless.

The third type of seizure is the one that has so far been untreatable. About 30 times a day Kai without warning passes out instantly and fully. It only lasts for a second, but the consequences are severe. When this seizure occurs he goes completely limp, like someone has pushed the reset button. Because of this someone must always be holding him, especially his head, unless he is lying down. There is a good chance he will have to wear a helmet his whole life. Eva tells me that children with conditions such as these often have dentures at the age of three because they knock all their teeth out.

Last week he was allowed to go home after nearly two weeks in the hospital, hooked up to lots of wires and tubes and having tests run on him each day. I brought Eva’s old futon down to Mountain View so he could have a safe area to play in, where his collapses might not cause as much damage. It was the first time to I saw him awake in a couple of months because I had not been down to visit since they moved to Mountain View from San Francisco. It was so nice. I think he remembered me, he was smiling and pulling on my beard (it always fascinated him). About 5 minutes after I got there as I sat on the edge of the futon he crawled towards me. When he was only an inch away he suddenly collapsed face first. It was only an instant, probably less than a second, and when he came to he started crying and basically only cried and made unhappy gestures for the next 30 minutes until his grandmother rocked him to sleep. It was the first time I had actually witnessed a seizure, and it was truly heartbreaking. Eva says he has these 30 times a day.

The most difficult thing is that because Kai is so young he really just doesn’t understand what’s going on, why all of a sudden life has become so awful. If only he could have been older when his condition made its appearance things would have been easier to deal with.

As it was, Kai’s mother and grandmother had begun to suspect that he was developmentally slow. At 15 months he was neither walking nor talking at a normal level. I didn’t see much harm in that, but apparently it was the first sign that something was wrong with his brain. His development will be even more difficult with his brain being reset continuously and addled with the medicines necessary to control the more life threatening seizures. There is a concern that he may never learn to speak because he is at the age of the prime opportunity for the brain’s language skills development, although I hope that is not true.

Eva says there are three standard medicinal regimens they try. The first has already failed, and he was on the second last week. The doctors do not have much hope that the second or third will work actually, because his case seems to be very severe. It might be beyond the capabilities of current medicine.

In some forms of epilepsy they can do a procedure where they insert an electrode into the brain at the point where they suspect the problem lies. By sending electrical current they turn off that region of the brain. This has to be done while the patient is awake so they can gauge the effect of the loss of that portion of the brain. If the effect seems reasonable, they permanently shut down the region. Unfortunately this only works in the outer layers of the brain, and scans so far have been unable to pinpoint Kai’s problem area. They suspect it is too deep to be treated with this procedure.

A more desperate surgery is one where they sever the two hemispheres of the brain. This surprisingly tends to not be as psychologically devastating as you would think. We don’t really know what it does because the workings of the brain are still so murky to modern science, but sometimes it makes life better for severe epileptic cases. The seizures don’t stop, but they may not cause unconsciousness, allowing the sufferer to sit down rather than collapse.

I’d like to apologize to anyone who has actually read this far. This is probably both my longest and saddest post ever, but it’s something I’ve been dealing with for few weeks now and I guess I needed to get it off my chest. The whole affair has already brought tears to my eyes and heaviness to my heart, and it doesn't seem to be improving. I got a quick email this evening from Eva. She says Kai has been brought back to the hospital. Tomorrow I will try to go down and see what his current condition is.