BASIS, C.E. Stafstrom, J. M. Rho (Editors), Humana Press 2004, Totowa, NJ

"Things I Wish They Had Told Us: A Parent's Perspective on Childhood

*Jim Abrahams
The Charlie Foundation

/"IMPRESSION: It is my impression that Charlie has a mixed seizure disorder, mostly likely a variation of Lennox-Gastaut syndrome. Parents are full aware of the ramifications of this diagnosis. Although there are many traditional combinations and permutations of drugs that could be used here, I agree with the current approach. It is my understandin that the next drug to be tried is a combination of Felbamate and Tegretol with which I have no problem. I would also consider the combination of Felbamate with Valproate with perhaps a benzodiazepine. In addition, one wonders if the Felbamate could be pushed to an eve higher dose than it is now, since we really do not know what the maximum dose of Felbamate is in young children. Another possibility is high-dose Valproate monotherapy. One other alternative therapy which I have mentioned to the family, but only reluctantly because of the high
incidence of side-effects is high-dose ACTH. The problem is that while high-dose ACTH may be effective in stopping the seizures, they almost
always recur as the dose is tapered. This makes one wonder if the risk-benefit ratio justifies the use of this somewhat dangerous mode of therapy. Finally think that if all pharmacological or theraputic modalities fail, I would seriously consider a corpus callosotomy on this child. A corpus callosotomy would not be curative of all the seizure types, but may help the most troublesome part of his seizure complex, i.e. the "drops.”

I excerpted the above from a medical report we received on our son, Charlie. It’s not really necessary to mention the name of the doctor who wrote it. Suffice it to say he was the fifth pediatric neurologist my wife and I had taken Charlie to see in the year he had been experiencing seizures -- four of whom ran pediatric neurology departments at esteemed university hospitals around the United States. They all concurred. Yes, there were some variations in drug
combinations suggested, and I’m sure that to a certain extent this report dates itself in that there are new drugs available today. But the reason I begin with this “impression” is not so much for what it says, but rather for what it doesn’t -- what it and all the doctors who examined Charlie never told us.

Today, nine years later, I think back to those days, and realize how much time was lost, how much unnecessary damage was done to Charlie’s life, and as a result, how much needless pain was, and continues to be, experienced by Charlie and all of us who love him -- all because information was not shared. I know the value of “ifs,” but sometimes I can’t help myself: If only the first time we walked into a neurologist’s office someone had handed us a pamphlet, recommended a book or taken the time to tell us just some of the following information we have learned since then...

Treating kids with difficult to control seizures is 20% science and 80% art. It was not long after we received the above doctor’s report that I was watching a CNN report on childhood epilepsy and there was one of the doctors who had seen Charlie making that statement. (Actually the statistic he used was “10 % science and 90% art,” but since then I’ve put the art versus science question to many neurologists and none peg it lower than 75% art, so I’ve rounded the number down). “Hold on a second,” I thought when I heard the CNN broadcast. Those were lab coats Charlie’s doctors were wearing, not smocks to protect them from splattering paint! They were scientists! Interior decorating is an art form -- not pediatric neurology. In discussing the subject with me, a world-renowned pediatric neurologist likened treating difficult cases to fishing. Just as you return to one fishing hole time and again after you’ve had luck once, so too a neurologist will return to the same drug combination time and again after one success. To a parent, this is a big deal. Clearly it puts the onus on the parent of the sick child to ask a key question, “Based on what?” “Based on what information are you telling me this, doctor?” Similarly I believe it puts a duty on the doctor to advise a patient if he is basing a statement on anecdote, experience, or science. An example: Lennox Gasteau Syndrome features the full gamut of seizure types. Charlie suffered most of them -- including tonic-clonic seizures. We were instructed at the time by his doctors that a tonic-clonic seizure, if allowed to continue for over thirty minutes, could damage his young brain. Therefore, at thirty minutes, drug intervention with Valium or Ativan would be necessary. We lived ten minutes from a hospital. So,whenever Charlie would begin one of his many tonic-clonic seizures, the first thing we did was to make note of the time. Twenty minutes into the seizure we’d get into the car and take off for the hospital. Certainly, as we sat there trying to comfort him during his many tonic-clonic seizures, every parental instinct in us told us that these prolonged
misfirings of his brain had to be damaging and we should put a stop to them earlier if possible. But we were typical, terrified parents and never questioned from whence the magical thirty minutes came. We never asked, “Based on what?” Several years later, after Charlie’s seizures were controlled by the ketogenic diet, and when both his physical and mental delays were apparent, I attended an epilepsy conference and learned the
thirty-minute rule had been shortened to five minutes. Now tonic-clonic seizures could damage the brain in five minutes. All those hours of
holding him when we could have stopped his seizures had in fact been detrimental to Charlie. The thirty-minute rule was not scientific.
There were no blinded studies documenting the long-term effects oftonic-clonic seizures of more or less than thirty minutes. At best, the
thirty-minute rule was based on anecdote, and at worst it was based on lore. I wish that someone had told us. I wish we had known the art versus
science percentage. I wish we had asked, “Based on what?” There’s no science behind it. This was an argument selectively used by several physicians who saw Charlie. Monotherapy, then multiple drugs, and finally a surgery failed to slow his seizures. He started to lose abilities he once had and, like most parents, we became more desperate. Holistic approaches, Chinese herbs, neck re-alignment and a host of non-Western approaches were suggested. The medical argument against them was: no science. I’d have no problem with that notion were it spread evenly across the playing field. But it’s not. From the naive patient’s point of view, when that argument is used to overrule “non-traditional” therapies, but omitted from Western recommendations, there is the assumption that Western approaches are backed by science. So, for example, when Dilantin was added to Charlie’s daily Felbatol, Tegretol, and benzodiazapine, in the absence of any cautionary thought regarding “no science,” I wrongly assumed (and probably never questioned) that modern science was at work. Nancy and I dutifully coerced Charlie into swallowing all that stuff around the clock and watched the terrible side-effects. Far from our minds was the notion he might be a guinea pig. If not a “crap shoot,” the chance of this drug combo stopping even some of his seizures and leaving him with any acceptable quality of life was beyond remote. What’s more, the effects of the simultaneous use of these potent drugs on his one year-old brain was and is completely without scientific documentation. We assumed naively that if there were no medical books
on the interactions and efficacy of that combination of drugs, certainly there were articles. As a matter of fact, I came to learn, there is no hard science regarding efficacy or side- effects behind any multiple drug combinations in the treatment of pediatric epilepsy. Again, my problem is more than trying multiple drug combinations. What I wish they had told us is that the “there’s no science behind it” argument is used to dissuade us from some approaches and omitted from others at the doctor's discretion. Seventy percent of new epilepsy patients have their seizures controlled with the first anti-epileptic drug they try -- almost irrespective of which drug it is. After the first drug fails, there is a 10% to 15% chance the second drug will work and only a 20 % chance drugs will ever control a child’s seizures. In other words, after a second anti-epileptic drug fails, four out of five children will not achieve seizure control with drugs alone. I’ve been told by some that this fact, were it known by many parents, would rob them of hope. I disagree. If someone had told us, it would have robbed us of complacency.

_A Couple of Drug Facts

1) The minimum criterion for the FDA to approve a new anti-epileptic drug, assuming the drug’s side-effects are not prohibitive, is that the drug stops 50% of the seizures in at least 50% of the children (who do not drop out of the study because of intolerance, noncompliance, or other reasons). In other words, if one hundred kids test a new drug and twenty-five drop out of the study, thirty-eight must have at least a fifty percent reduction in seizures. There is no requirement for a new drug to stop seizures to be approved by the FDA. For parent of a child with difficult-to-control seizures, there is always the "buzz," the promise about the next drug -- the next "great White hope" -- to arrive on the market. Very few of us know this liberal criterion for FDA approval.

2) In 1993, a new drug, Felbatol, met those requirements and was approved by the FDA for children with difficult-to-control seizures. It was produced by a company named Carter-Wallace. I wrote to Carter-Wallace to get their annual stock holders report. From this report, it was clear that in the two years prior to the release of Felbatol, the company spent $20,000,000 -- not on research and development, but on marketing the drug. In 1993, there were about twelve hundred pediatric neurologists in the U.S. to whom their advertising campaign was aimed. I’ll let you do the math. Twenty million dollars to convince twelve hundred folks to prescribe a drug! I certainly wish someone had told us that bit of information before we allowed Charlie to spend all those sleepless nights while he was on Felbatol, without helping his seizure control.

Legal Standard of Care Regarding Provision of Information

The legal standard of care in America today, with regard to a physician sharing information with a patient, is that the physician isrequired to tell his patient, in general, the same information his colleagues would share with their patients under similar circumstances. Practically speaking, that means that if, in a court of law, a doctor can get several other doctors to testify that they would have informed their patients similarly to the way he informed his patient, he has
conformed to the standard of care. This is not to suggest that legalities were ever a consideration regarding Charlie’s case; it is only to provide information I wish I had had, the bottom line regarding information-sharing by a physician. As his dad, especially in a case like Charlie’s where his seizures were difficult-to-control and the art versus science thing was clearly applicable, I always assumed our doctors would lay out a range of treatments and together we would decide on the correct course of action -- in essence, a doctor/patient partnership. I assumed an informed joint decision-making process. I was wrong. Certainly we didn’t pretend to have the medical expertise, but then
of course the doctors didn’t live with Charlie, and had no real knowledge of his family, its agony, and his living circumstances. And of course none of the doctors we saw had ever held their own child during a seizure. Unfortunately, informed joint decision-making turned out to be wishful thinking. Information was always provided on a need-to-know basis and then in a seemingly reluctant manner. In fact, there was almost a physical chill when more than limited probing and suggesting occurred.

The Ketogenic Diet

Throughout Charlie's epilepsy, I remember crying hardest with Nancy when we received the copy of the above report. It seemed so hopeless. And yes, we were “fully aware of the ramifications” of Charlie’s diagnosis. So, as a way to prepare Charlie and the rest of us for what seemed to be many more years of seizures drugs and “progressive retardation,” I started doing some research. To my surprise, in epilepsy texts dating back to the 1920’s (and in every decade from the 20’s through the present) from Hopkins, the Mayo Clinic and many other highly regarded institutions, there was consistent anecdotal documentation regarding the ketogenic diet and its efficacy (roughly one-third of the kids who tried it became seizure-free, one-third were
significantly improved and for one-third it was ineffective). The documentation totaled literally thousands of children with epilepsy. In addition, the diet’s side-effects were minimal compared to many of those that Charlie had experienced. I learned that there was still a dietician at Johns Hopkins who had forty plus years of experience with the diet. So, one month after receiving that gloomy report, we took Charlie to Johns Hopkins. They put him on the diet. His seizures dropped from dozens a day to zero within forty-eight hours of its initiation, and he was off all medication in another month.
I don’t want to minimize the difficulty of the diet, but it was a walk on the beach next to the drugs and continued seizure activity. More importantly, we had our son back! Today Charlie is eleven years old, off the diet, and has been seizure- and drug-free for years. I asked his doctors why they left it to us to learn about the diet they all had heard of. Their answers: 1) it was too difficult; 2) being high in fat, it may be unhealthy; and 3) there was no science behind the diet. Too difficult? What could be more difficult than watching your child than watching your child slip into retardation via drugs and
seizures? Unhealthy? Compared to what? No science? No need to deal
with that subject again. The medical destiny of each of us and our children is largely up to the patient and his/her family. To think otherwise can be damaging. There is a tendency when we walk into a doctor’s office to want to hand over our problem to the doctor and say, “Here it is, please fix it.” It’s comfortable, it’s easy, and more often than not, it works. Just as we take comfort in deferring to them, many doctors are unwilling to
confide in us that we may have stepped into one of Western medicine’s black holes. There are
many and they are deep, and kids with difficult-to-control seizures are among them. So what does that mean? It means that our medical problems and our
childrens’ medical problems are precisely that -- OURS. At first, that’s a pretty
intimidating and perhaps a seemingly foolish concept, both to us and to some physicians. After all, they went through years of education. They’ve seen countless patients in their practices. And then we walk into their offices with a disease we probably don’t even know how to spell. How presumptuous and perhaps foolish of us, the patients, to ask and then pursue the hard questions, learn the side-effects, get the second opinions, do the research, and participate in the cure -- in short, become proactive. Ironically, the "side-effect" of participating in our medical destinies may not only lead to getting better sooner. It is empowering. Though I would do almost anything to go back and have Charlie not suffer epilepsy, the experience has been empowering. Whether or not responsibility for informing ourselves confirms the information we learn from our physicians, it’s nevertheless empowering. As fate would have it, I am writing this afterword from a hospital bed where I am fighting leukemia. But the bed I am lying in, the treatment I am undergoing and the doctor who is helping me are not arbitrary. Though my diagnosis was shocking and treatment was needed immediately, there was time to find information, interview oncologists and even visit hospitals before setting sail on a course. Mercifully, I found a doctor who is bright, compassionate and believes in informed joint decision-making. We take control of so many lesser issues in our families’ lives -- meals, bedtimes, TV hours -- why not have that same attitude with the most important issue - our families’ health? In the worst case, we have learned something new; in the best case, we have improved either our lives or the lives of our children. There is no downside.


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