Epilepsy as Killer

This is Oliver. He had Dravet Syndrome and died this morning, in his mother's arms.

This is Dani. She died from complications of epilepsy on March 10th, in her parents' arms.

This is Cyndimae. She died of SUDEP (Sudden Unexplained Death in Epilepsy Patients) yesterday while napping on her father's chest after a normal day.


I don't even know what to say other than to bring these beautiful children to your attention, to ask you to bow your heads and send all your loving juju to their families. I know them all through this bizarre and wonderful community of people struggling with seizure disorders, cannabis and the fight for access. I've looked at their pictures, followed their ups and downs and spoken with their mothers over the years. These children are our children. Cyndimae, Dani and Oliver are our lights, even if extinguished.

When the Moment, Much Less the Day is Everything

I watched Sonjay Gupta's third segment of Weed the other night on CNN and was struck in particular by what the wife of a soldier being treated for PTSD said about the possible long-term risks of using medical marijuana. I'm paraphrasing here, but I remember her saying she had her husband back. She said that he had his life back. She mentioned that 22 soldiers commit suicide each day in this country. She stated that even if they were to find something negative about the use in five or ten years, they would have had life NOW. 

This living in the present moment thing is part of our popular culture, expressed in mindfulness meditation and in most conventional religions. We are urged to seize the moment, take each day as it comes, one day at a time and so forth. For those of us with children with special needs, particularly older ones who are and will remain utterly dependent upon us for the rest of their or our lives, this is something piercing and gets to the root of just how we cope. It's also an imperative when your child is at risk of sudden death or even an inevitable early death. Sophie, as you know, had been seizing hundreds of times a day for most of her nineteen years when we began using cannabis oil. She is nonverbal, needs assistance walking, is fed like a baby, wears diapers, has either her father or me sleep with her every night and has to have a padded bedroom so she doesn't hurt herself. When the seizures slowed down and then stopped for the most part, our family's life was so radically changed that we really didn't talk about it for a very long time. We still don't, really. I've thought about "long term effects" of using cannabis oil, and these thoughts are really no different than the ones I've had about the 22 pharmaceuticals she's been on as well. My fallback coping skill is a grim and dogged sense of humor, and there have been moments -- and minutes and hours and days and weeks and now one and a half years -- when I've wondered if Sophie was going to grow a Bob Marley-esque tumor in her brain. Please humor me and don't gasp too loudly. Like the wife of the soldier with PTSD, though, I believe that these largely seizure-free days and weeks and months and maybe even years, when Sophie is alert and sleeping well, when she doesn't do a face plant into her dinner every night or smash her head into the floor or the dresser in her room, are worth it.

That's what living in the moment is for someone like me.

My Italian grandmother, whom I've written of here quite often, was a deeply religious woman and also deeply suspicious. I don't know the expression but think there is one for someone who doesn't like to speak of anything positive because of the chance to jinx it. We're fiddling around with Sophie's cannabis oil, trying something new, and this morning for the first time in a very long time, she didn't have a seizure when she woke up. Rather than perpetuate superstition, I'm going to seize this moment and this day and exult in it.

Anti-Suffocation Pillows

photo via trendir.com

As you know, I am prone to subverting emails that carry horrific information by opening them, reading them, digesting them and then coldly and methodically mocking them. Said horrific information includes nearly everything related to epilepsy, particularly SUDEP, that lovely acronym for Sudden Unexplained Death in Epilepsy Patients. And while I'm enough of an Italian -- and a southern one at that -- to have embedded in my genetic code a proclivity toward deep suspicion and a concomitant belief in the power of jinxes, I use sheer willpower to over-ride those impulses and get down to it.

Let's discuss.

Today's horrific email came, once again, from a wonderful website dedicated to all things epilepsy, epilepsy.com. The lead article is titled Electric Shutdown of the Brain: Is It A Cause Of SUDEP?. Now, if you've been a reader of this blog for any amount of time, you'd know that this is a subject that quite literally strikes fear into the heart of a parent of a child with uncontrolled seizures and a person who struggles with seizure control. And up until quite recently, information about SUDEP was relegated to hushed whispers -- there was very little information at all until, I believe, a group of dedicated families in England started shouting about it. It wasn't until very, very recently, in fact, that there has been a positive plethora of information about SUDEP -- not so much answers or cures or huge understanding of it -- but recognition and awareness about it, along with suggestions for prevention. In any case, I clicked and opened the article, scanned the contents in a vain attempt to find anything new and -- actually -- found something new.

Anti-suffocation pillows.***

I don't think you need me to explain what anti-suffocation pillows are, but I did go onto the UK website and will be ordering a couple very soon. When I do these things -- open emails about sudden unexplained death in epilepsy patients (SUDEP, again!), note my own daughter's high risk of SUDEP (override the Italian peasant jinx mentality in seeking information!), scan for information that I don't already know (why, why, why don't they know more about these things?), skip over to foreign websites about anti-suffocation pillows (!!!!!!!), use a Coin Converter to see how much said pillows cost in U.S. Dollars (cool tool!), wonder if the material they use for the cover is gross, imagining some kind of thin, motel paper like consistency (feel sheepish that aesthetics trump survival for an instant!), wait to order because the pillows are so expensive and I have to do some financial juggling, first (what if she dies before I order the pillows!) -- well, I am struck by the absurdity of this life I lead (and others like me) and filled with gratitude that I have a sense of humor that often trumps panic and paralysis.

Or should I say suffocates panic and paralysis?





***Anti-suffocation pillows available to order HERE.

A Lesson

Sudden Unexpected Death in Epilepsy (SUDEP) is now recognized as the leading cause of mortality associated with epilepsy, outside of status epilepticus and underlying etiologies. The general level of awareness of SUDEP has risen considerably in the last several years, but the general public is still largely unaware of just how devastating a diagnosis of epilepsy can be. In fact, not even professionals devoted much attention or research to it until the early 2000s.

Risk factors for SUDEP include the early onset of seizures, frequent generalized tonic-clonic seizures (those previously known as "grand mal"), young adult age, poor compliance with an anti-epileptic drug (AED) regimen and multiple AEDs used. Patients with epilepsy have a significantly higher rate of mortality than the general population, and SUDEP accounts for 8-17% of the deaths of people with epilepsy.

I know of seven children and young adults whose death was determined to be from SUDEP.

We can all manage our fear (and trust me, I do fear) by educating ourselves and advocating for increased research about SUDEP. In late June, Partners Against Mortality in Epilepsy is holding a conference about SUDEP in Chicago. You can read more about it here.

Now I'm going to have a swig of vodka.

The largest eye

Ostriches evidently have the largest eyes of any land animal, and it's a myth, apparently, that they stick their heads in the sand to avoid things.

When I received the email in my inbox yesterday from Medscape, I didn't read anything I didn't already know. I sadly know several children who have died from SUDEP. Either The Husband or I have been sleeping with Sophie all of her life because of that risk.

What struck me about the article was the definitive study that was done, proving that children with uncontrolled seizures are at dramatically greater risk of early death than those whose seizures are controlled. SUDEP has other risk factors as well, and the important point to be made is that the medical community has not been as forthright as they might have been about SUDEP for a very long time. The reasons for this are many, as I said in the other post. In fact, it was in England that a group of parents who had lost children to SUDEP formed a group to explore why they had no idea that death was a risk in their children with epilepsy. It has only been a decade or so since this issue has received the attention it so deserves. A fascinating history of the disorder and the appalling lack of research surrounding it, including the story of Queen Elizabeth's uncle, Prince John, who died of the disorder, can be read HERE.

What does it mean for people like me? Other than the shock value, the ominous foreboding, the fear and anxiety that the article provokes in me personally, it actually forces me to pull my head out of the proverbial sand, talk about this issue, educate others about this issue and work harder to increase understanding and awareness around any issues having to do with epilepsy. We need to know more about this disorder. We need more money to study this disorder -- for prevention -- for management -- we need to end seizures.

So, scratch the ostrich in the sand concept. Who am I kidding? I'm the person who ran to Barnes and Noble and looked up "seizures" when the New Neurologist diagnosed Sophie and told me don't read anything about infantile spasms because it's all depressing.  I read EVERYTHING, always have and always will.

I'm going to use my HUGE eyes, read these articles and hold my head up. I know I can't control whether this fate might befall my child, but I can sure as hell learn about it and work to end it.

(but I will put my head in the sand rather than read about the increasing violence in Libya, including our country's actions, the continued grotesquery of Newt Gingrich and the hypocrisy of the Repubs in general)

What would you do?

Every few days or so, I get an email in my inbox from a search engine called Medscape. I subscribe to the search engine and have indicated that I am interested in any articles pertaining to childhood epilepsy and neurology. Generally, the articles forwarded are scientific in nature --quite dense -- and I read no further than the abstract. Lately, there's been quite a lot of buzz about SUDEP (sudden unexplained death in epilepsy patients) -- nothing like an acronym, right? Traditionally, this has not been a subject that neurologists are willing to discuss with their patients, and the reasons are diverse. However, with the advent of "patient-centered care" and more involved parents, there is a demand for information, complete information, and I've noticed that SUDEP is getting a lot more publicity.

So, today, in my mailbox I get this on the subject line:

High Rate of Death in Childhood-Onset Epilepsy

Do I go there? Do I become more informed? Or do I stick my hand in the sand and la di dah out? Those who know me can probably figure out what I do, but I'm wondering whether I should.

Vulnerability

The year is young and already there have been two news stories that hit very close to home. The first is the negligence of a caretaker for her 22-year-old charge whom she left, stranded, in a bus overnight somewhere in New York. The young man evidently had severe cerebral palsy and was in a wheelchair. She knowingly left him in the bus where he rocked for over 17 hours, trying to keep warm. He suffered hypothermia when they discovered him in the morning.

The second is the death of John Travolta's son Jett in the Bahamas this morning. The young man, aged sixteen, evidently had a seizure and hit his head on the bathtub, was found by a caretaker at the hotel, but couldn't be revived. I had long thought that Jett was autistic but learned in a newspaper article that John Travolta had always denied this and that in addition to seizures, his son had suffered from Kawasaki's disease when he was younger. After the initial rush of horror, of what I think in the moment I understand, I'm painfully aware of my own selfish need to differentiate from these families. My only thought, then, is My God, My God and then all the inane platitudes that accompany tragedy.

I stare sickly at these news stories (that many people emailed me), poring over every word, almost runnning my finger over the lines. I read them and then I reread them. Looking for clues. There seem to be only cliches to describe the feelings: my heart literally goes out to them, the parents. I get it, I think, simultaneously looking for something in the story to differentiate from my own life. Sophie can't ever bathe by herself, so that wouldn't happen I think. Or Sophie's aide would never do that and besides, I'd know that she wasn't home. Where was this young man's family when he was freezing, alone, on a parked bus?.

Some people like to say that we're all one step away from death. To savor every moment because you, too, or your spouse or your bright and happy children could be taken away with a snap, like that. And that's true and unless you're just not too bright, getting older makes you painfully aware of mortality.

The vulnerability of my own daughter, Sophie, is something that I try not to dwell upon. Try not to even write about because I think, sometimes, that I'll jinx it. That by giving it form it will be more real. Or I think the opposite: that the more I think about it, the less likely it will happen. The Husband likes to say that one would be paralyzed, literally, if one entertained the thought of Sophie's vulnerability at school. She can't talk or really do anything for herself. So when we send her off to school each day with, let's face it, an aide who we've only known for a year or so, we're taking, really, the biggest leap of faith of our lives.

And then there's the other. The fact is this: people with seizures that are not controlled by medication can die suddenly. The condition has the lovely acronym of SUDEP (and I don't even want to link to it in my blog!) and it's something that The Husband and I think about every single night.

One of us, usually him, because he is far less psycho in the night, sleeps with Sophie each night. This has become habit and we look on it as one of the concessions we've made in our lives to her disability. We do it knowing that in some ways it's irrational. Even useless. A lame, pathetic attempt to control the utterly uncontrollable. "Can't you use a monitor?" someone always asks. "No," I reply. We need to be near her. We have this idea that if she were to stop breathing, we would sense that, in the night. I have to believe that we would.

That the Travoltas' tragedy is public due to their celebrity doesn't make it any bigger than the countless, faceless tragedies of other children's deaths. From seizures, from disease, from something out of the blue. But it does put it out there.Sometimes I feel like gritting my teeth and reading these stories, that sharing them will somehow prepare me for the worst. Lamely, I'll repeat There but for the grace of God, go I. But I know that isn't really true. I really just have no idea.

I'll leave the computer, the story and go back to my nest where I'll squat, my wings spread over them, these fragile eggs.