Diligent Supervision: Protecting from Epilepsy's Harms

This photo makes me uncomfortable.  But there's also something fascinating about it.  Somehow, in a way that I can't quite articulate, it reminds me of our life with epilepsy.

This is Awesome and her brother Jonas in September 2010, three years before Epilepsy reared its ugly head for Round-Two in Awesome's life. In this photo Awesome is just 5 years old and Jonas is 14.  And yes, they're in front of Niagara Falls.  Awesome was so afraid, when close to the falls, that she climbed into her brother's arms, wrapped her arms around his neck, and hid her face.  She refused to look up at the camera.  She didn't really like Niagara Falls.

If you've never been there, imagine the roar made by 85,000 cubic feet (about 64,000 gallons) of water plunging over the edge of the 100 foot falls every second.  The force and raw power of the falls, as you stand nearby, is awe-inspiring.  And a little overwhelming.  And maybe even, if you let yourself think about it, more than a little frightening...

There's something about this photo that illustrates so well, the constant fears and tensions we live with with epilepsy.  The vulnerable child so close to the overwhelming power and danger that could easily claim her.  But the calm, strong, solid embrace of her brother Jonas who loves and shelters her.  As long as Awesome holds tightly--and doesn't suddenly do something stupid--and Jonas stays put--and doesn't do something stupid--Awesome is safe--despite the overwhelming danger nearby.  In fact, there is actually some margin built in there.  Railing yes, but also a thin sliver of land between the railing and the destructive force of the falls.  The key for Awesome in the moment of this photo, was to stay within the protective limits of her brother's arms, and if not that, then within the protective limits of the railing, and if all else failed,  if she were to miscalculate or misstep, within the confines of the tiny sliver of land from which she could be rescued.

Epilepsy claims as many lives a year as breast cancer.  A child with epilepsy is five times more likely to die than non-epileptic peers in the general population, not just from SUDEP (Sudden Unexplained Death in Epilepsy), status epilepticus, and other complications of seizures, but also from injuries that occur during or result from seizures.

Whether it be Niagara Falls or epilepsy, the close juxtaposition of a child and serious danger seems all wrong.

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Awesome, who is now 12 years old, was supposed to have her first swim team practice last Thursday afternoon, but it didn't happen.

Shortly after lunch, after I'd watched her easily ace a page in her pre-algebra workbook, she'd asked if I'd get her a glass of ice water.  And so, as she settled down to start a second page of problems--with her laptop loudly playing her favorite song--I walked to the kitchen.  A moment later as I got the ice, I thought I heard gurgling noises coming from the schoolroom. I assumed I must be mistaken.  After all, I'd been gone for only a couple of minutes and Awesome had been fine so far that day--no signs whatsoever of impending trouble.  And anyway, how could I think I could hear anything accurately above the loud music and noisy tumbling ice?

But then I heard it a second time.  That gurgling noise.  This time, even above the loud music, I was certain I was hearing what I was hearing.  Plunking the cup of ice down on the counter and running down the hallway, I called Awesome's name several times.  She didn't answer.

Entering the schoolroom, I saw immediately that Awesome was in the midst of a seizure.  Deep in an altered state of consciousness, she leaned upright against the back of the couch-- her arms and legs in a relaxed starfish position.  Her Pre-Algebra workbook, and pencil had fallen to the floor.  Her head had fallen backward onto the tops of the back couch cushions.  Her face was turned upward toward the ceiling; her open eyes stared vacantly, without seeing and comprehending.  The gurgling noises in her throat---that I'd heard from 3 rooms away--were the noises she made as she struggled to breathe through airways clogged with excess saliva.  The only discernible motor symptoms of her seizure were the exaggerated breathing sounds from the rhythmic contraction of her diaphragm.  Her body was relaxed and still.  Not convulsing.  Not stiff.  That she wasn't entirely succeeding in oxygenating her blood, despite the exaggerated breathing, was evident from her very gray skin tones.

I called for Awesome's eldest brother Jacob who'd been sitting at the kitchen table.  He came running quickly, and together we eased Awesome over onto her side to allow gravity to move the saliva out of her throat and airways, to make breathing easier.  As we moved Awesome, we observed transient motor activity in her right leg; it resolved almost as soon as it started.

Awesome's altered consciousness continued for a minute more as I fumbled to pull the case full of emergency seizure medication syringes out of the bag I wear on my person at all times, unzip it, find the correct oral syringe, and discharge the emergency CBD into the outer pocket of Awesome's mouth.  Though Awesome's seizure was almost over already--signaled by her eyes which were now closed--the CBD would help keep other seizures at bay, speed recovery time, and ease post-ictal (after-seizure) impairments.

Almost immediately after I gave her the CBD, Awesome fell deeply asleep.

While she slept, Jacob and I put a clean, dry washcloth under her mouth to collect the excess saliva and a pulse oximeter on her finger to monitor pulse and oxygenation.  Oxygenation was in the low 80's at first, but within a few minutes, it bounced back up into the 90's.

As I sat there on the floor next to the couch, watching the pink return to Awesome's skin and watching the reassuring rhythm of her breathing as she slept, I wondered about the seizure she'd just had.  Had her seizure included a short period of tonic-clonic (convulsive) activity before I'd gotten into the room?  This was important to me because the truth is this: Awesome's never had a complex partial that hasn't either instantaneously or slowly generalized into a tonic-clonic (a convulsive) seizure.  Her big seizures have always gone there--like a one-way ticket.  And so the idea that this seizure might not have was a radical one.

Being an epilepsy parent is very often about using clues to solve--or at least propose various solutions to (often we don't settle on one but instead suspend judgment, holding a collection of possible solutions in our minds to see what pans out in the longer term)-- various mysteries--large and small.  Put another way, epilepsy parents spend a lot of time noticing things, collecting data, analyzing data for possible explanations, and then forming hypotheses about what might be going on. You can use the private eye/sleuth analogy or the scientist analogy, but either way, epilepsy parents are often left puzzling things out to form theories about their child's epilepsy: what's going on and why.  Theories are important because they both offer explanations for past events and suggest subsequent actions that might have the power to change future events.  Me trying to figure out what had happened during this seizure was just another instance of this.  I found myself looking for clues and using those clues to puzzle out what was most likely true.  In this case, given that Awesome was sitting upright when we arrived, not twisted to the right and fallen over which usually happens if she's seated; given that she'd not fallen to the ground like she normally eventually does with convulsive seizures; given that her body was more limp than stiff; and given that whatever way the seizure had started, it hadn't had time to go on for very long, the evidence pointed to the likelihood that she'd NOT had a tonic-clonic as part of this seizure, but rather, it might have been a complex partial--from start to finish.

For an epilepsy parent, the post-ictal period when your child sleeps and you sit beside them monitoring their vital signs, and waiting for them to wake, is an emotional time and also a time of reflection and contemplation.  Probably for a lot of us, the intensity is also heightened by the leftover adrenaline pulsing through our bodies and brains.  Mixed in with my sadness and disappointment that Awesome had had another big seizure--this one the 75th in her lifetime--was a little thrill of hope.  What if this seizure hadn't involved any motor activity at all, save her rhythmic diaphragm contractions and the tiny bit of activity we'd seen in her right leg?!  If that was indeed the case, it would be nothing short of a very encouraging break-through.  CBD and especially 15:1 CBD (a CBD with a higher THC content) has been steadily shortening Awesome's big seizures and blunting their severity.  In fact, the last seizure before this one had involved only about 30 seconds of tonic-clonic activity.  What if this one had involved none at all?

As I sat there, it occurred to me that there are two ways to affect seizure frequency.  One is to simply eliminate seizures--something that the pharmaceutical anti-epileptic drugs (AED's) are supposed to do, but failed to do for Awesome.  The other way is to make seizures shorter and shorter and milder and milder until they disappear altogether--which for Awesome also hadn't happened with AED's--but might it be happening with CBD.  Could this last seizure have been the seizure in which the tonic-clonic portion had been eliminated entirely?  I got a little thrill from that thought.  It would have been exciting to follow this line of what-if's to a happy conclusion, but as I sat there watching my child, I realized that it was too risky.  Too much disappointment might result from grasping onto this hope and considering its possibility too strongly and too prematurely.  I would simply have to wait and see.  Entertaining hope beyond what was really warranted has always proven unwise.

This much was certain: there was no one in the room with Awesome when her seizure started, and there were no video cameras (I didn't turn on our Arlo video cameras because I was planning to be gone just a few minutes)--and Awesome herself doesn't know what happened.  We'll never know what kind of seizure Awesome had last Thursday--whether it did or didn't include a tonic-clonic.

As I sat there stroking her head and thinking about how much I loved her, I thought about how disturbing it is to leave a room for just a few minutes, and to return to find your child not just seizing, but struggling to breathe--and having turned gray from lack of oxygen.

What might have happened had I not walked into the room just then?  What if I hadn't heard the gurgling sound and also had gotten distracted?  What if I'd decided to stir the soup, adjust the spices, put away the few stray things on the counter, let the dog out, wipe the counter off, or whatever.  What if, while Awesome lay stretched out with her head back, struggling to breathe through a saliva clogged airway, I had dawdled unaware in the kitchen believing that Awesome was working on her page of pre-algebra problems?  I'd felt no huge hurry to get back there with her glass of ice water.

We already have a large collection of "what if we hadn't been there or hadn't walked in when we did?" scenarios.  They're disturbing to contemplate.  What would have happened had I not walked into the bathroom when I had that evening when I opened the door to find Awesome wedged into an alcove between wall and toilet, her head bent unnaturally?  What would have happened had I not been beside her the morning that she woke in a seizure, her face pressed into the mattress, producing large amounts of saliva, and struggling to get air?  What would have happened if her father hadn't been standing across the counter from her when a seizure struck so suddenly that one second she was standing there talking and the next second she was falling so that he had only enough time to reach across the counter and grab at a handful of her clothing--to slow down her fall?  What if she had fallen on that large shard of broken glass--the large jagged bottom of the drinking glass sitting on the ground like a dagger--instead of falling a foot away from it that Christmas morning when she seized, knocking the glass off the nightstand and then falling off the bed onto the glass shards?  And perhaps most terrifying because of their sheer number, what would have happened had any of the 50 status epilepticus seizures she's had in her lifetime have gone unnoticed for minutes, half an hour, an hour, several hours or even overnight?

Every epilepsy parent worries about injuries and deaths directly related to epilepsy.  But when your child has a very strong history of status epilepticus--seizures that simply do not end on their own without drug intervention--any seizure that goes undetected for long could kill your child.  And while CBD has largely eliminated that status epilepticus threat for Awesome since last November (2016), it is not an elimination that I count on.  I can't.  Why not?  Because the consequence--the price--of being wrong about it--if I was wrong--is too great.

Status seizures are ipso facto, medical emergencies.  According to  ILAE (the International League Against Epilepsy) guidelines, the defining time for a status seizure keeps shortening.  It used to be 30 minutes.  Then 10.  And now it's 5.  As I understand it, it's not that a 5 minute seizure has gotten or is now considered more dangerous, nor is it even necessarily damaging, but it's that some portion of 5 minute seizures will turn out to be seizures that aren't going to stop on their own without medical intervention.  And it's these seizures that they worry about.  It takes time to implement emergency medical help.  It doesn't happen quickly.  And yet every minute that goes by with a status seizure makes the situation more serious.  Every minute that goes by makes it statistically less likely that a seizure is going to stop on its own without medical intervention.

As our epileptologist explained to us early on, the longer a seizure goes on, the harder it is to stop.  The harder it is to stop, the more and stronger drugs it takes to stop it.  And at some point, the drugs needed to stop a seizure are so sedating that they begin to depress breathing.  Depressed breathing leads to needing help in maintaining an open airway and to needing help in breathing. And the next thing you know you have a child who is intubated, on a respirator, and in the PICU (Pediatric Intensive Care Unit).  This has always been our epileptologist's worry with our daughter--that we won't catch a status seizure early enough to stop it easily and that Awesome will end up heavily sedated, intubated, in the PICU--in really bad shape.  So far this has not happened.  It's our watchfulness that has kept us out of the ER since 2007 and completely out of the PICU.

Status seizures are nothing to mess with.  A convulsive seizure that goes on for longer than 30 minutes can cause permanent brain damage and ensure that your child has epilepsy for the rest of her life (due to the damage that results).  A convulsive seizure that goes undetected and untreated for hours could be catastrophic;  and a convulsive seizure that goes undetected at night--if a child sleeps alone--could go on for hours and easily kill her.

Sometimes I think that the hardest, most enduring difficulty with Awesome's particular case of epilepsy--at least so far--is the special need for supervision that she requires because of her very strong history of status epilepticus.

We are very aware of what can go wrong.  And how quickly it can go wrong.  Awesome's longest seizures have lasted for over an hour and a half.   With her first seizure, I presumed she was dying--it was two or three hours later until I really understood that she wasn't.  During that hospital stay we were given and taught to use the seizure rescue drug Diastat.  It was a amazing to have a way to stop Awesome's seizures ourselves, without 911 and the ER, but Diastat, even when administered ASAP after a seizure started, for Awesome would typically take 10 minutes to work to stop her convulsive seizures.  The other rescue drugs we've tried haven't worked at all to stop her seizures.  Awesome's seizures, once they get going, are not easy to stop.  And if all this wasn't bad enough, Awesome vomits during a certain number of her big seizures.  And though it is rare, she aspirated once--and developed aspiration pneumonia--leading to an illness that lasted a more than a month, involved 18 days of hospitalization, and resulted in the loss of half a lung.  Thankfully, things haven't been worse than they've been.  Many epilepsy parents have experienced and constantly experience far worse with their kids.  But things have been bad enough.  We've seen as much of the bad side--and the crises--of epilepsy as we ever want to see.

And so we are diligent.  From our point of view diligence is one of the few things standing between us and disaster.  Diligence in supervision.

The supervision that Awesome needs to stay safe is akin to the supervision that a 1 to 2 year old needs.  She needs far closer and more diligent supervision than our granddaughter Skye, who is 3 years old.  Skye can be in a room playing by herself for 10 or 15 minutes.  Awesome can't be alone for more than 5 minutes between check-in's.  And last Thursday, a check-in of 2 minutes proved inadequate....  Awesome cannot bathe or shower alone.  She's not allowed to lock doors in our house..  We either have to monitor her continuously when she's in the bathroom, or one of us checks in with her about every 2 or 3 minutes while she's there.  In fact, the bathroom is one of Awesome's most seizure prone places.  At least half of Awesome's seizures recently have occurred while she's in the bathroom.  And falling and hitting the hard surfaces of the bathroom--whether tiled walls, tiled floors, sinks, bathtubs, or shower faucets--is dangerous.  There are few times and places when Awesome can be truly alone. And when she is, we are almost always monitoring her via video camera of one sort or another.  And very few are the people who are both able and willing to take on the responsibility of Awesome's care for even a hour or two.  She's a normal kid--absolutely--in every way, except in the special supervision she requires--close supervision and someone willing and able to provide the seizure first aid she needs when she needs it.  And that first aid is immediate.  We've learned to carry Awesome's seizure rescue meds on our persons.  Awesome goes nowhere without them.  Whoever is in charge of her in the moment wears them.  There was a time when we kept them in a kitchen cabinet, but that time has passed.  Her seizures come on so quickly now there's no time to run to grab them.  Awesome needs immediate care to be kept safe.

Awesome's supervision is a 24-7 affair.  Onerous and irritating to her.  Often wearying to us.

Although Awesome has an Empatica Embrace seizure detection watch, and it helps, it's not dependable enough to trust to safeguard Awesome's life.  Sometimes--like with the seizure last Thursday--Awesome's watch is not on her wrist, because its battery's being recharged.  Sometimes--like the seizure before last--the Embrace doesn't work because it has recently become unpaired from Awesome's smartphone, making it useless for alerting us to a seizure.  And sometimes, as has happened three times now, it doesn't work because when Awesome falls during a seizure, the arm with the watch on it becomes trapped under her body so that it can't move enough to meet the requirements of the seizure algorithm--so it doesn't trip the sensor and we don't get the phone call alerting us to her seizure.

We've tried to incorporate as many new and up to date technologies into our monitoring routine as possible--the Google Echo, Google Hangout,  Foscams, the Arlo security system, etc and a few old ones too--like walkie-talkies.  But like the Empatica Embrace, most technologies are helpful but not fully dependable enough to safeguard Awesome's life.  And before Awesome could ever sleep alone we'd need systems and back-up systems and back-up systems for the back-up systems so that we would have an almost fail-proof system.  And maybe a seizure dog to top off the whole fail-proof system.  Because an undetected night time seizure could be catastrophic.

All this is life-altering for both Awesome and for us, her parents.  But this close monitoring is the only thing standing between us and disaster.  There are times when we watch Awesome closely for weeks on end with nothing happening, when we start to feel a bit ridiculous.  Like we are overdoing things.  And being too hypervigilant.  And we start to question ourselves.  And wonder about whether Awesome really needs that level of supervision.

And then inevitably, as soon as we start to question, we get a wake-up call.  There are times, like last Thursday, when a seizure falls through the cracks, and when we really realize anew just how catastrophic even one seizure could be.  It's so ironic that as closely as we watch Awesome, that last Thursday's seizure occurred in the two minutes I'd been away.  And when you walk into a room to find your child struggling to breath, choking on her own saliva, and as gray as Awesome was with that seizure, you realize anew that the dangers are real, probable, and ever present.  That you're really not dealing with a mathematics--a calculation, a formula-- where you can afford to calculate incorrectly and fall short.  Not even once.  Falling short could claim your child's health or even her life.

After all children with epilepsy die at five times the rate of children without epilepsy.  Persons with epilepsy die everyday of SUDEP.  Of accidental injuries.  Of drowning.  Of status epilepticus.  Of aspiration pneumonia.  Epilepsy claims as many lives a year as breast cancer.  And if we start to disbelieve, being a part of online epilepsy support groups ensures that we hear of those deaths at all too regular intervals.  We all hug our children a little closer when one of our own suffers the unthinkable.

And so we watch Awesome carefully.  Always.  No matter how onerous that is for her or for us.

We want Awesome to be alive to grow up.  We want her brain intact so that she can continue to ace those pre-algebra, and then algebra, geometry, and calculus courses--and so that she can pursue her dreams.   We want her around and healthy when medical science had advanced enough to finally know how to control her seizures.  And we want her around because we love her.  More than life itself.  And so we are there for her.  Watching, watching, always watching.  We offer diligent supervision to protect her, as much as it is within our powers to do so, from epilepsy's harms.





Comments

  1. Sending love and light to you all...

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  2. Wow, what an excellently written article on living with your child's epilepsy. My youngest was diagnosed with an epileptic encephalopathic syndrome last year at the age of 6. I can relate to 100% of what your wrote. Not an easy journey for sure. Most people have no idea what it's like. Also to never be able to leave them alone. Big hugs to you! <3

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  3. As the mother of a 32 year old with epilepsy, who's been living with this for over 31 years, you nailed the anxiety that it is to live with. Thank you.

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  4. This is a harrowing but beautifully written account of what it means to be a parent with a child with Epilepsy.

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  5. I completely relate. Thank you for this great post.

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