The List

In the last few weeks Cole has had a downward spiral.  For those who have been asking and have Dravet kiddos of their own, here is the list of things that we have tried and the rundown on what has been happening.  This isn’t even the half of it, but for record keeping purposes I will hit the highlights.

October-Cole starts seizing more often.  At this point we have practically stopped using benzos and rescue meds in general.  He has once again become immune to them and we fear withdrawal symptoms.  Also, in prep for starting Onfi.  Only used rescue meds 3 times all month.  We go to Camp For Courageous Kids.  I still need to write about that.  It was wonderful!  Had our first 3 Tonic Clonics in one day.  We stopped Potassium Bromide in late August and the month of getting it out of his system has ended.  Start Onfi.  Largest break in between major seizures was 4 days.  Every seizure looks different.  Different types, different times, different lengths.  There is no regularity or ability to predict what is coming next.  This has kind of been the theme of Dravet, but this year has just been mind blowing.  It makes Sluggers job extremely hard and training new nurses difficult. 17 major seizures recorded.

We prepare for Superstorm Sandy.  End up evacuating anyway.  We were gone for 5 days.  Drive 14 hours home.  The next day, Daddy leaves for NYC to help with the aftermath.  He is gone for 2 weeks. 

November-Get a small break when we increase Onfi.  Have 7 days with no major seizures.  Then things get hairy.  Daddy is still gone.  Have a nurse quit and so only have coverage 3 days a week.  Start Anat Baniel Method Therapy.  It is a very slow process with Cole and his sensory issues.  He doesn't like strangers to touch him.

Cole starts having at least 2 tonic clonics every day.  Nothing is stopping them.  Onfi, double Keppra, and rescue meds are hardly working when we use them.  Still using all rescue meds and double Keppra sparingly.  Cole starts having major regressions.  Reverts back to oral phase, acts a lot like an infant and starts sticking every thing in his mouth.  Has some potty accidents.  Loses some of the songs he knew before.

Brian comes home and the next day we leave for Miami.  Read about our Florida adventure HERE.   24 major seizures recorded, not counting nocturnal.

December-We drop the rest of the Onfi.  Cole finishes his amoxicillin on the 1^st.  We get ready to start Verapamil.  Want to go back to school and get settled into routine before we start something new after all of the travel.  Silly us.  The 2^nd reintroduces daytime TCs.  It is so weird how he has a honeymoon every time he is on high dose antibiotics.  We start a love/hate relationship with 911.  We call them at least once a week all month.  If you know us at all, you know that it is totally abnormal for us to get to our breaking point so often.  Cole continues to live his legacy and stops seizing once we call, or once the paramedics show up.  Whether we wait for 29 minutes or 8 minutes, the story is the same.  It does not work if we pretend.  He knows J We have to be serious and dial for real.  We start the Verapamil.  Cole catches a cold the first day he goes back to school and shares the love with Mom and his main nurse.  We stop the Verapamil, after one whole day!  Still not sure if it works for him, but we couldn’t know while he was sick.  Also didn’t want to take the chance of a major drop in blood pressure when he was ill.  We take him to the doc, strep negative, flu negative and throat culture is negative. He loses his voice and looks horrible, but still has some stamina.  The entire month the longest break he has from tonic clonics is 2 days, and that only happened once.  The myo’s abound.  The TCs ramp up from 2 a day to 3 a day.  Every single day.  Cole regresses further and does strange infant-like behavior like chewing on his feet and licking the floor.  We try double doses of Keppra, since that helped on the airplane in Florida, kind of.  It helps maybe 1 out of three tries.  Mom gets super sick and wants to curl up in a ball and hide.  We don’t have nurses, so this makes things interesting.  Cole, per his M.O. continues to be sick, for a long time.  At this point, Cole has been de-sating to the 30s and 40s during major seizures and will hover for minutes even with 5 Liters of oxygen.  He is congested and occasionally coughs, but it is just a virus, right?  We throw around the idea of possible pneumonia.  I am talking to the pediatrician daily. 

It doesn’t matter what we do.  If we use rescue meds, the seizure doesn’t stop.  When we used to administer medication like Diastat or Versed, at least it helped to prevent future seizures that day and would give him some sort of a break.  Cole is seizing again within a few hours, even with Diastat.  Has 26 major tonic clonics (this is not including the short nocturnal ones) in 12 days.  During all of this we call Dr Miller.  He suggests that we start Bromide again for a short-term solution.  We know that he broke out in a rash about 2 ½ weeks in to it before.  So, back to dog medication we go.  Immediately we notice that Cole’s speech declines.  By the second day, he is slurring his words.  By the third day, he is drooling even without seizures. We are just looking for a short-term solution.  Something to just break the cycle of seizures.  We know that Cole can’t handle long term Potassium Bromide.  We see our son slipping away from us.  Call Dr Miller again.  Don’t want to wade into any more old medications.  We could start increased eye fluttering, maybe even those evil drop seizures could come back?!  Things are already bad here, why would we want to possibly make it worse?  So, we get into “illogical and completely bizarre” territory.  Discuss things like IVIG or prednisone.  Heaven help us, ACTH comes up.  Also high doses of antibiotics…to control seizures.  Illogical.  Remember how Cole had a honeymoon with the Amoxicillin?   As bizarre as it seems, some kids with Dravet have decreased seizures on high doses of antibiotics.  There are side effects, but it is less risk than steroids.  So-we decided to start 3 days of 1000mg Rocephin shots, that day.  Cole gets 6 huge shots in the behind.  Fun...not really.  Dr Miller wants to keep up the Bromide for a week and we talk about steroids as the next approach.  By now, we are on the fourth day of Potassium Bromide; Cole can barely speak at all.   He is drooling like an infant and does not want to eat.  His gait is slowing.  He is grabbing his face before a seizure, going tonic and then scratching himself.  He looks like he has been in a fight with a rabid raccoon.  Perfect for those family pictures next week.  Brian and I decide to take him off Potassium Bromide.  This entire time we are vacillating between deciding to go or not to go home for Christmas (cross country flight).  The next morning, Cole wakes up shaking like I have not seen him for a long time.  The tremoring and myoclonics are so intense that his whole body is involved.  Almost into tonic clonic territory.  We wonder if it is due to this, due to that.  Always having to play detective.  We give him a double dose of Keppra and 5 mg of diazepam.  Decide to continue with the antibiotic shots.  I say, "We are not going home".  Cole improves throughout the day and regains some speech.  He still sounds like a drunken sailor and cannot say the ABCs, but hey…he is talking!  We’ll take it!!!

The entire day of the 2^nd antibiotic shot, he does not have a tonic clonic.  We are still holding our breath, but so grateful for the small break for our child.  His color starts to come back; he doesn’t look like death warmed over.  Day 3 of the antibiotic (today), at the time of publishing, he has had two big seizures.  Better than 3!  So-if you really feel like nothing is working and your child just keeps clustering no matter what you do,  go out on a bizarre limb and try high doses of antibiotics.  Or maybe not.  It is not a long-term solution, but worth a shot.  I think.    31 major seizures recorded, not counting nocturnal, and we have 11 days left.

 I still don’t know if we are going to go home for Christmas.  But really, what if this is his last Christmas?  Won’t we regret staying just because we were scared?  Our life the last few years has taught us to live to the fullest.  So we will probably put him in a drug-induced stupor, board that plane and hang on for dear life.  Who needs performance enhancing drugs, bungee jumping, marathon running and rock and roll?  Just get a child with Dravet Syndrome and you can have all of the adrenaline rushes you could ever ask for! J

Our Florida Adventures

We had our 6 month visit with Dr Miller scheduled for November.  We decided to move it from the regular clinic day and combine it with our Thanksgiving break.  Two birds with one stone! 

Before we even got to the hospital, we had all sorts of drama.  Cole had been having very frequent tonic clonics at this point and we were nervous (as always) about flying.  Somehow taking care of a seizure at 30,000 feet isn't so easy.  Landing the plane, also not so easy.  We gave him a double dose of Keppra per Dr Miller and sent up a hundred prayers that he could make it on the flight.  God has a sense of humor!  Cole made the flight, but had a severe seizure at baggage claim.  We were quite the sight.  Urine streaming down my legs (Cole's), holding a seizing child, Brian trying to grab our bags off of the carousel, Slugger wondering what the heck was going on.  Hey-he didn't have one ON the plane!  The next day he was so bad that we considered calling 911 and going to the local hospital.  However, we totally knew that if they admitted us, we would not be discharged in time to make our appointment in Miami the next day.  We didn't call, Cole made it through the night.  We got up the next day and headed 3 hours south to Miami Childrens Hospital.

While we were there, Cole had his first sleep study.  Results show there is no major change.  He is still seizing throughout the night.  No sleep apnea though, hooray!  We discussed with Dr Miller the increased frequency in tonic clonics and myoclonics.  Cole had not been sick lately and we felt like we had a baseline established, as fluid as it was.  Bottom line was, we knew that the Onfi wasn’t working. 

Dr Miller, as great as he is, basically tells us during our appointment that we are running out of options.  Which we kind of already knew, but it is hard when one of the best doctors in the country for what your child has, tells you that he can’t help you.  Cole has tried 7 or 8 meds at this point and has failed them all except Keppra-which we really don’t think is working very well anyway.  So we decide to get off of Onfi.  Next stop, Verapamil (a blood pressure med of all things).  You know we are all about Plan A, B, and C…so after that, it is time for Stiripentol or revisiting old meds.  We discuss alternatives such as VNS, medical marijuana (which is illegal where we live), IVIG, steroids such as ACTH or prednisone.  He wants to see us in 3 months.  Okay, now things are really getting real.  On the day we visit Dr Miller, Cole has three tonic clonics.  He also seems really sensitive to light.  Much more than usual and is hiding his head under a blanket.  I will say again, seizures in the car, on the freeway are definitely ranked in the worst top 5 places to have your child seizing.

The next day all hell breaks loose.  Cole has 4 tonic clonics and was basically in NCSE (non convulsive status epilepticus) the entire day…we just didn’t know it.  He is inconsolable and we can tell that he is in terrible pain.  He is actually complaining of eye pain.  For a kid who has run around on a broken foot, twice, this is huge.  The light sensitivity is very intense and he wants his special glasses on even indoors.  We try a double dose of Keppra, Motrin, Tylenol…nothing is working.  We contact Dr Miller and tell him we suspect a migraine.  He tells us to give a combo of Benadryl and Aleeve.  By tonic clonic #3 we are getting anxious.  We give Versed, it doesn’t help.  Cole can’t hold up his head, is drooling profusely and can barely speak.  Yet, he doesn’t want to be left out.  The boy is adamant that he is a part of the holiday party and refuses to sit out in the car with me.  So I take him inside and he sits on our laps, wrapped in a blanket as we eat and looks the definition of disabled, which is rare for him.  But he is there and he breaks out a crooked smile; such the definition of resilience.  At TC #4, it is evening and we call the on-call neuro, who told us she was extremely nervous and wanted us to come in.  It is never reassuring when a neurologist admits to you that they are nervous!  We explained that we were 3 hours away and would have to be Life Flighted, which could take over 5 hours from start to finish since we had to go through the local ambulance, hospital, etc.  She agreed that it could take too long.  Getting through to a new hospital that doesn’t know us and doesn’t know Dravet takes forever.  By the time they believed us enough to call Miami and get the go-ahead, it could be hours.  Remember he isn’t actively seizing at this point so to a regular doc, he would look fine.  She suggested that we actually get in the car and drive down.  At the end of the conversation she gives us instructions to give him Diastat and a double dose of Keppra.  If he has another, we are to call AirMed and get down to Miami.  No matter how inconvenient it is.  The Diastat in combo with the other meds settles Cole’s brain to the point he can sleep, just through the night.  It also tips us (and Dr Miller) off to that his supposed “migraine” was actually bizarre seizure activity.  Since pain relievers did nothing for it and a benzo did, it was probably seizures.  Weirder things have happened with Cole.  Add it to the list of "rare".  Most regular people would sleep for a day or two with all of those medications in their system, not our Mini Hulk!  He only got a 7 hour nap.  We truly experienced a miracle though and made it through the night.  I really didn’t want our first helicopter ride to be in a different state and take a couple of hours.  Thanks divine intervention! J

Those meds carried over and he did not have a major seizure the next day.  However, we got a new surprise at night.  Cole started having major nocturnal seizures.  Tonic clonics that last from 30 seconds to a minute.  He will sit up in the middle of the night, grab his face with his shaking arms and have full body convulsions.  We can only hold him and tell him that we will be there for him.  They are too short to drop his oxygen and don’t need meds.  However, it gets my brain kicked in to overdrive and I just lie there awake, waiting for the next one.  One night he had 9.

The next day, I had my mommy instinct telling me he might have an ear infection.  Mind you, there was no complaints of pain, no major fever, no pulling of the ears, no lethargy, just a hunch.  We took him in and BAM, both ears were infected; they started him on  Amoxicillin.  The rest of our Thanksgiving break was full of nocturnal seizures, but he had a break with daytime ones. 

I was so ready to go home.  I wanted our routine back.  There were rumors flying around that Brian was going to be sent back to New York which stuck fear in my heart but I knew that I had a back up plan for support and we could really use the money.  The hurricane, him being gone, Miami, the holiday and all of these seizures were getting to me.  So, we were grateful for the break from daytime chaos.  Well, at least where seizures were concerned!  We had a great time with our cousins and other family members that we don’t get to see very often.  Plus, it was 80 degrees the day we left!  Is 32 too young to become a snowbird? J

Evacuate

Hey there.... *awkward silence*
It's been a while.  How are you?

We. are. alive.

If you live in the United States, you probably heard of Frakenstorm otherwise known as Hurricane Sandy.  At one point it was headed straight for us.  So, we readied our house.  Our emergency preparedness skills kicked in, full force.  We took everything out of the basement.  Moved all of our pictures and precious possessions to the top floor.  Pulled all of our furniture away from the walls.  Put towels in all of the windows.  Got gas for the generator, bought water and food.  Stocked up on medication.  In the end, even though we were super prepared with heaters, batteries galore, flashlights, blankets and enough food to feed a large family for weeks, we still ended up evacuating.  It was the best thing for us as a family, especially for Cole.

Our house was fine and we ended up spending the time in warmer weather.  Some would say, "Why?".  Others knew exactly what we were thinking when we packed up and left in a matter of hours.  Barometric pressure, possibilities of no power, no access to the hospital, etc.  Even if there was something as simple as a tree blocking our road, it meant that the ambulance couldn't get to our house if we needed it.  We didn't know what would happen, but the majority of our life is hanging in the balance of the "cone of uncertainty" to use a phrase from the weather man.  We didn't need any more uncertainty.  Our life is an emergency and we did not need to add to it.  So, we left for a little while.

I feel like I have kind of evacuated the blog.  Even social media.  No more Instagram, very limited looking at Facebook.  It was the best thing to do in the moment.

I really want to catch you all up on what has been going on around here.  Honestly, it is overwhelming.  Failing multiple medications, stress with work, new therapies, school stuff, nursing stuff, family stuff, and more just stuff.   Cole is having more seizures than ever...and I mean, ever.  I kind of evacuated my life too.  This little semi-reseblance to a normal existence that I have been carving out for the last year has kind of been put in the closet.  Since our nursing is extremely limited I have gone back to full time caregiver 24/7.  The boy is with me everywhere.  He has also been missing lots of school.

Hurricane Sandy also took our daddy's attention for a month.  For a week he was constantly on his phone and computer and then he was in NYC for two solid weeks.  Cole was really struggling during all of this and it could have been a really dark time.  In the end, it showed me that I am stronger than I thought.  I always knew I was good under pressure, but I really reached my breaking point.  That is when people stepped in, who owed me nothing by the way, and took a little piece of the burden.  We are not out of the woods yet.  We almost had to Life Flight Cole and my little calendar book is full of days upon days of multiple major seizures per day.  We went to Miami and hopefully have a new plan (or two), but we are kind of running out of options.

Stuff like the blog, e-mail, Facebook and Instagram have been so far in the back of my mind that it seems like another life completely.  I still miss you and want to catch up on our high highs and our low lows, if only for record keeping purposes.  In between all of the trips to the hospital and the days of not showering because you can't leave Cole for a second, we have had some funny, good times.

Stuff like Cole going to the beach.  Playing with cousins we have not seen in a long time.  Cole being obsessed with excavators and wanting to call Santa on the phone.  We have always known that the only thing we can control is our attitude.  So-we had an adjustment and are going to make this holiday season the best that it can be.  Yep, our life is still an emergency; but we will show up at the ER with antlers and tacky Christmas sweaters!

Happy Holidays,
NIK

P.S. We got nominated for an award.  Kind of fun :)