Showing posts with label Vigabatrin. Show all posts
Showing posts with label Vigabatrin. Show all posts

Sunday, March 18, 2018

Parables of Neurology, The Cosmos



Star Trails
Photographer: Carl Jackson



At some point in the waning years of the last century, Sophie was on her eighth or ninth drug, a drug that wasn't yet FDA-approved but that The Neurologist At The Time thought worth trying. I received the drug from a pharmacy in London, a tiny little shop on a cobblestoned street with a 17th century sign hanging outside that swung in the English rain. You figure out which part of that sentence is fiction. I've told this story before. The drug was a white powder and came in a foil packet called a sachet, and after I carefully poured the contents out, I cut them with a razor blade to get just the right amount for the baby, about enough to fit into a 1/4 teaspoon. I dipped my finger into the powder and put it on my tongue. Powerful enough to stop seizures but bitter enough to spit out. I gave it to the baby.

Know that the word irritable is frequently used in neurology literature as a possible side effect. An Earlier Neurologist listened to my complaints about the Baby's constant fretting and said, You have to figure out what your tolerance is as far as irritability, after which I lit the fuse that connected the telephones of the last century that we were using and blew him up. That should be parenthetical.

On the third day and then night of the drug in the sachet, Sophie began screaming all night long, flailing her arms and arching her back. She screamed until she became hoarse. You can't imagine what a hoarse infant sounds like -- just air but more -- air that you can hear, and I spoke into the air as I walked with her, up and down, up and down. Enough. This is enough. No more. We will not do this. The next morning I called The Neurologist At The Time. The Neurologist At The Time was what they called cutting edge, no pun intended, knives and docs, docs and knives, and I liked him. I was going to say love but that would be fiction. I called him up and said, The Baby is beyond irritable. She is psychotic, if babies can be psychotic. She is still seizing. She is now on two non-FDA-approved drugs and is being weaned from Phenobarbital. How many babies do you know on this combination? Could the three drugs be interacting? 












(silence)













The Neurologist At The Time said, That's a very interesting idea, and the words travelled as sound through a wire connecting us, across the country (I was visiting my parents in Atlanta and The Neurologist At The Time lived in New York City) and assembled themselves into block letters that floated out of the can I held up to my ear and spun round my head.


T h a t ' s    a   v e r y   i n t e r e s t i n g   i d e a.












(silence)









I have a Bachelor's degree in English and French literature. I've also read an indeterminate number of novels, including all the classics in French. I've studied Mandarin Chinese, excelled in modernist poetry and wrote an honors thesis on Pascal's Pensees. The only science class I took in college was Zoology, and during my senior year I thought I might round out the piles of novels and poetry that lay everywhere in my room by taking Waste Management. I got a D in Calculus.  Yet, evidently, as per The Neurologist At The Time, The Cutting-Edge Neurologist, I was having interesting ideas about my nine-month old daughter's brain and its response to seizure drugs from other continents.

I never got over this, by the way.

The landscape changed in a moment, over the telephone. The tiny little mother mind™was born in that moment, and I'd describe the birth as a kind of star trail like the photo the Bird Photographer took in the middle of the California desert, a time-lapse of stars burning or dying or traveling as the world spins on its axis, but that might be a mixed metaphor, and I  don't know physics either or even photography. Black holes. No man's land.




This is not fiction.


Stephen Hawking said, The greatest enemy of knowledge is not ignorance, it is the illusion of knowledge.


Thursday, July 7, 2011

We're a weird bunch.

Humor is tragedy plus time.

Mark Twain



My friend Erika and I were talking on the phone last night, and I was sharing with her my recent visit to The Neurologist. Erika has a child with a seizure disorder as well, and even though her daughter is many years younger than my Sophie, Erika's sense of humor is sharp, achingly so, and she jumps right into the Roman gladiator pit with me.

As you might have surmised, Sophie's honeymoon from daily seizures is now officially over. The drug Vimpat has ceased working as well as it was, and now we're on the tiresome wheel of deciding what to do next. We don't really have any options that jump out at one, unless you think another ketogenic diet trial is worth it.  The second and last time we tried the keto diet was about a decade ago (the first time was in the early days -- the mid-90s, when not many people even knew about it), and while it helped Sophie a bit as far as seizure control, it also turned her into a caged tiger, ravenously, desperately hungry with impacted poop (I'm not mincing words). It traumatized me so deeply that I look on that time as being equal to the trauma of her diagnosis and the early days of high dose steroids. That the connection between mothers, children and food is a deeply primitive one is not lost on me, but acknowledging it does nothing to dull the pain of that time.

A second choice is a revisiting of the drug Vigabatrin (or Sabril, as it's also known)-- one of the "newer" approved drugs and one that we tried, also, back in the mid-90s when we ordered it from England. I've waxed philosophical about Vigabatrin in a chapter in my yet-unfinished book -- the chapter was then published on epilepsy.com's website.  The drug wasn't approved for many years in this country because of some very serious side effects involving the retinas of the eyes. It's now approved and used as a front line drug to control infantile spasms, the terrible epileptic syndrome that Sophie was diagnosed with -- a form of epilepsy that continues to stymie the best minds. In fact, very little progress has been made for babies and children with infantile spasms in the sixteen years that we've been part of that club. In order to go on the drug now, one is subjected to a strict protocol -- eye exam baselines and the signing and initialing of pages of caveats and warnings and information. This is what I did at The Neurologist appointment yesterday, and as I told Erika, I found it bizarre and not a little hilarious that I was able to casually check the little box and print my initials, EA, next  to sentences like: I understand that about 1 in 3 infants taking Sabril will have damage to their vision. I understand that if any vision loss occurs, it will not improve even if my infant stops taking Sabril. 

I loved Erika when she burst out laughing when I told her about initialing this sentence: I understand that there is no way to tell if my infant will develop vision loss.

Unless you get it, you're probably not laughing, but we were and I think if we were two women in vaudeville we might have been slapping our knees in hilarity, knocking each other over with the force of the absurdity.

What really set us to being utterly cracked was my description of our other "option" for control of seizures: the vagal nerve stimulator or VNS. You can google and read about it online, if you'd like to know more. It's been around for quite some time and really doesn't have a fabulous efficacy rate -- the rule of thirds, much like drugs (one third improve, one third stay the same, one third get worse or go off). In any case, here's a scan of the box that had the informational DVD and brochure. 


In describing the packaging, I wondered aloud to Erika about the work that went into that marketing and how hard a group worked on  just the right words, the right picture, how much to suggest, how to harness hope and propel people toward treatment. I wondered aloud to Erika whether the two women frolicking on the beach might be our daughters one day or maybe even the two of us, looking beyond. 

We just laughed and laughed and laughed.

Sanity and happiness are an impossible combination.

Mark Twain

LinkWithin

Related Posts Plugin for WordPress, Blogger...