Sobering

I have been in the throes of medical records again.  When you see a new doctor, they always want to know what has been done in the past, what meds, what tests, etc. etc.  Digging through the last 6 years has been sobering.  What Cole's body has been through, the meds that he has tried, the hospital admissions...it really is too much for anyone that hasn't even entered Kindergarten.  However, I look back and see how strong we are.  We should have had at least two nervous breakdowns by now!!! :)

So, for the future and to be able to access it quick via the nice, old friend the Internet-I present a history of the treatments tried for my son:

Anti-Epileptic Medications (including atypical treatments)
Phenobarbitol
Versed-rescue (Buccal and Intranasal)
Ativan-rescue
Propofol-rescue
Keppra
Diastat-rescue (rectal suppository and tablet)
Ketogenic Diet
Klonopin-rescue and daily
Depakote
Topamax
Low Glycemic Index Diet
Potassium Bromide
Rocephin
N-Acetyl Cyestine
Clobazam
Verapamil
Prednisone
Diazepam tablet-daily
Phenobarbitol-rescue and daily
Zonegran
Ativan
Stiripentol
*Note:  Some medications are listed twice.  This is due to a reintroduction after many years of discontinued use.  Rescue meds, used as needed, are also listed.

Adjunct Medications
Acetaminophen
IB Proufen
Amoxicillin
Potassium Phosphate
Carnatine
Magnesium
Calcium
Centrum Multi-vitamin
Nano VM Multi-vitamin
Vitamin D
Mirilax
Cefdinir
Sennakot
Lactulose
Metamucil
Multivitamin Supplement (Carnation Instant Breakfast)
Magnesium Citrate

Treatments and Equipment
Oxygen
Pulse Oximeter
FL-41 Lenses
g-tube
Feeding Pump-IV pole, Feeding Bags, Syringes
SPIO-Stabilizing Pressure Input Orthosis
Therapy Swing
Helmet
Cooling vest
Wheelchair
Enemas
Seizure Alert Service Dog (he really is listed as Durable Medical Equipment in the law)
Suction Machine
iPad
All equipment required to administer medication-pill crusher, syringes, g tube extension, etc.

Additional Therapies and Treatments
Homeopathy
Neurofeedback
Speech Therapy (also including feeding therapy)
Occupational Therapy
Physical Therapy
Aquatic Therapy
Anat Baniel Method Therapy
Craniosacral Therapy
Sensory Integration Therapy
Behavioral Therapy

Specialists
Neurology-6 different ones
Epileptology
Genetics
Dietician
Opthamology
Gastroenterology
Anesthesiology
Surgery
Cardiology
Orthopaedist
Podiatry
Orthotics
Neuropsychology
Nephrology
Endocrinology
Developmental Pediatrician
G-Tube Nurse Practicioner
Bone Health Specialist (Ortho)
Pulmonology
Pediatric Dentist
Behaviorist

Hospitals
PCMC
IMC
CNMC
MCH
IFX
CCHMC
SCH

I was getting my hair done the other day and I spent the entire time I was "processing" on the phone making doctor's appointments.  My friend asked me if I felt like we lived at the doctor.  I had to be honest and say, "Yes".  Looking at it all listed out is a little overwhelming.  I am positive that I have missed something (probably more than just one something).  No wonder we are tired!  All I know is Cole is worth it.  We will keep fighting.

Dropped Off

Hey Friends-
Sorry it seems like we have dropped off of planet earth.

Our little world has been chaotic, to say the least, these last few months.

The extreme Reader's Digest version:
* Make A Wish was awesome.  Cole struggled.  We had fun in between the seizures and took over 400 pictures
* Cole has been in ICU 3 times and was almost taken via air ambulance to Miami
* I can't count how many times we have changed and tweaked meds
* Normal tonic clonic count right now is around 15 to 20 daily.  Mostly at night.  Never thought that could be normal
*We drove to Florida and stayed inpatient for 6 days.  Had to detox Cole and have some tough decisions ahead
* I don't have lupus!  YAY!  I do have fibromyalgia, and possibly osteo arthritis.  I've tried a few things and think that maybe I found something to help with the pain
* We are taking Cole to Seattle for a week to have an in depth Mitochondrial study done
* Brian and I got to go to Hawaii for a week with my parents and siblings; something that has been planned for 6 months.  By the skin of our teeth and with the help of 7 nurses, two good friends and a pack of teenage boys (our friend's sons), Cole was taken care of.  Sometimes we had a ratio of 3 adults to 1 kid, but it happened and we are grateful!  I have missed my Hawaii home so much
* We don't sleep.  At least for more than 2 or 3 hours at a time
* Showering has become totally optional
* Our finances are more than a little strapped
* Slugger is still awesome
* If you are our friends "In Real Life" we are so sorry that we are neglecting you
* We are glad that we didn't switch jobs last month.  Brian's co-workers have been awesome about our crazy schedule
* I have over 300 e-mails in my inbox
* We got "fired" from therapy because Cole has not been attending over 80% of his sessions (due to seizures, mind you).  I am not sure I have the strength to pull out Tiger Mama and fight this one
* I don't watch TV any more
* I gained 7 pounds in Hawaii and it was totally worth it!
* ESY Summer school started today and I am really pumped about his IEP and the goals for the next year
* It is very hot and humid around here.  Or it is pouring rain
* Cole has a new kiddie accordion and it is hilarious to watch him play it
* Had a Neuropysch eval and found that Cole is still severely delayed in a lot of areas, but has made awesome progress in others
* We are grateful for insurance

There are a lot of Grand Canyon sized gaps in there.  All in all, we are tired, we are stressed, we are still married, we are still happy and trying to find the good in every day.  We live minute to minute and that means things like dishes, blogging and changing our clothes don't happen on a regularly scheduled basis.  Our lawn looks like a jungle, our hair is unkempt, but we are trying to do what is best for our boy.  Sometimes that means just sitting back and letting go of the things that you thought were important and realizing that they really weren't that important at all.  Or maybe I am just trying to make myself feel better about not vacuuming? :)

Peace and Blessings, Friends!  One of these days I will jump back on the bandwagon.

Mixologist

It is no secret for those who know me that I love beverages.  I have an entire board on Pintrest dedicated to them.  It's probably a really good thing that I don't drink alcohol, because I would probably spend a good chunk of money on fruity drinks :)  Cocktails turn into mocktails around here, but there is still something satisfying about mixing flavors to come up with a new wonderful combination.  For example, last night I made a strawberry, pineapple, coconut, vanilla, Sprite concoction that was awesome!  I think I would make a pretty good bar tender.  I could probably get really into Mixology.

When you are mixing and matching medications, it has been termed "cocktails" just like those fruity drinks with a toothpick.

It seems like through our constant tweaking lately we have found the perfect combination to buy us a short break.  It is not a quick fix, but you don't stay drunk for days from one glass of wine.  In theory, anti-epileptic drugs work on the brain like alcohol.  It makes it sleepy, slows it down so that it can't fire off and have as many seizures.  When Cole's brain is over-active, we have found (for now) that giving him a specific cocktail will buy us at least 8 hours without a tonic clonic.  Now this is not something that we can do every day, but it has sure proven useful in the last two months.

Double dose of Keppra-an AED that he has been on for a long time.
5 mg of Diazepam (Valium)-a benzodiazepene
and 7.5 mL of IB Proufen-an anti-inflammatory

It doesn't matter if he has a fever or not.  We have tried these things separately also as a form of rescue medication.  Right now, it only works when you do all three together.  Strange, I know.  Weirder things have happened.

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

Bromide

We started Potassium Bromide a few weeks ago. This is a medication that is considered "old school". It was used for hundreds of years, but fell out of doctor's circulation when bigger and better Anti-Epileptic Drugs (AEDs) came along. It is still widely used by veterinarians, to treat dogs with epilepsy.

Since Cole is severely intractable (hard to treat) our treatment team is at a crossroads where we are ready to use out-of-the-box treatments. Bromide takes a very long time to reach therapeutic level, about three months. We are going extremely slow since Cole is so sensitive and Bromide has an incredible half life. It stays in the system for weeks, versus hours like other drugs. You can not get it at your local pharmacy. We have been using a compound pharmacy that is out of state to make it for us.

So far, it has been good. Potassium Bromide is a salt, so it dissolves well in warm water and is easy to push through Cole's g-tube. We will just have to be patient and see if it makes a difference. We are really looking to decrease myoclonics, since he is still having on average 400 a day.

One concern was that Bromide can interact with chlorine and patients are cautioned to stay away from chlorinated water. However, there is no research to back this up, just hearsay from an organic chemist who is certain that the reaction between chlorine and bromide would cause an effect similar to the bends in SCUBA diving. Our doctor gave us all of the necessary precautions and warnings. Us being us...we tried it out in lots of different environments. We poured three capsules in the pool, waiting for effervescence. We tried a cap in the hot tub, to see if increased temperature would cause the bubbles. We called vets and asked if they had ever heard of cautioning pet owners to keep their animals away from pool. Their opinions and alll of our tests turned up negative. We then decided to not totally submerge Cole and took him to our local splash pad.
He had a good time testing our hypothesis!
No problems. We showed the pictures to his doctor and he laughed. Apparently, we have proved ourselves. We always keep a close eye on Cole anyway when he is swimming. I am just glad that we didn't have to take something else that he loves away from him.

We also started Prevacid, after finding that his upper GI series revealed he is having reflux, which could be the reason for his gagging all of the time. Since starting both of these medications, Cole's seizure control has been better. The Bromide is certainly not therapeutic yet, and Prevacid is certainly not an AED. Who knows?! I am just grateful that it seems like we have broken out of the cycle of needing rescue meds every day.

Plan A, B and C

I will start when we almost went to the hospital, before we went to the hospital.

We woke up early the morning after and packed into the car, heading down to see our personal rockstar, Dr Miller. Got Cole all hooked up.

All he wanted was to hold the board they strap babies on to when they put on their leads. Okay?

When we finally got settled in our room, all Cole cared about was the electronics and the magic keyboard. Are you surprised?

Since Cole had a fever, we gave him Tylenol, which curbed his small seizures (we find this is the case) and so he did not perform very well during the beginning of his Video EEG. As it wore off and he got tired of being poked, prodded and examined...the myoclonics joined the party and we had plenty of data. I have mentioned more than once that I have a disdain for EEG's. Willing your child to have seizures is such a backwards experience. You spend their whole life wishing that they will be healthy and strong, then you get them all hooked up and hope that they just seize away so doctors can capture data. You are armed with a button, ready to push at the slightest hint of activity. Turns out, I was 100% right at capturing seizures if I was looking at Cole. Only had a less than 10% error rate, which were probably attributed to blinking/drowsiness that we attributed as eye flutters. He had a great myoclonic cluster that lasted over an hour and a half. When the resident heard it had been that long, he came in and gave me a stiff lecture about how we should call him if we think Cole is seizing. Uh, do you want to take a seat? We'll be here all night.

These people could not fathom that we were not giving him heavy drugs to stop the small seizures. Eventually, I asked for something, they didn't get it fast enough and so I went to my own stash...I got in trouble again. Seriously, they wanted to give him 10mg of Diastat for myoclonics. I finally got them to compromise at 5mg. I appreciate doctors and modern medicine, but more is not more when it comes to drugs. Why use 10, when 2.5 will work? Every time we use benzo's, there are side effects. I know Cole well enough to realize that little seizures, just need little drugs. Big seizures need big drugs. Glad that I don't take it personally any more. I used to beat myself up after I would hear that we were doing things wrong, from doctors who had never met us or didn't even know our son. Now, I know that we know our son best. What works for us, is what we are going to do. Thank goodness Dr Miller is on board with this. Eventually we got an EEG that looked like he was drunk, since he had the Diastat.

They really should make pediatric hospital beds, bigger...since the majority of the time they have a parent in there with them.

We made the employees look far and wide for "the car". Cole knows that when he is in the hospital, they have cars. It is part of his in-patient routine. Miami only has 2 cars for the entire hospital and they are in the playrooms. We need to get one donated to the neuro unit. The boy freaked out for an hour after he woke up from his seizure induced sleep, because all he wanted was a car.

Slugger was a celebrity and people kept stopping by to visit him. When Brian would take him outside he would get accosted on his way there and back, by families who wanted them to visit their room. Every one was certain they were volunteers :)

SO-results are that Cole is having 200-400 small seizures a day. This is compared to 70-100 a day last year, when we thought things were bad. Oh, Dravet...why do you have to keep proving to us that things can always get worse. It truly is laughable how nervous we have been along the way and now we look back on those days and laugh. Dravet has truly taught us the power of the human will and the strength that you can find in yourself when you feel like you have absolutely nothing left to give. I remember that trip we took in 2009 and how freaking scared we were to cross the Nevada desert, that something might happen. This was before drops, before photosensitivity, before temperature issues...before a lot of things. We look back on those few days and how absolutely precious they were. We spent an entire day at the beach and nothing happened. That little trip with just the three of us will never be possible again. We will attempt something like it, sure. But this time armed with oxygen, a bag of rescue meds, a cooling vest, FL-41 lenses and a seizure alert dog...and maybe we will stay for an hour.

I like to talk to Dr Miller about Plan A, Plan B and Plan C, since we know that most things don't work out for Cole. It is not being pessimistic, it is just being realistic. I want to know what the next step is, with his help. Right now, the first step was to increase Cole's Keppra. He has been on it a long time, when he misses a dose he has a seizure, so we know it is working. It is one of the safest anti-epileptic drugs on the market, not being broken down in the liver like most of the others. So, to make up for his height and weight change for the last year, we re-calculated his dose to be almost exactly what he was on previously, but took into account the changes his body has made. We found out in FL that Cole has grown two inches since December. I knew his feet, but wow-no wonder I keep finding clothes that fit yesterday don't fit today. Plan A-change the Keppra dose, with this we also changed it to three times a day, hoping to get rid of that early evening hump we have been having trouble with. So thankful for technology, that phone of mine reminds me every day at 2:00 that it is Keppra time. I have to pack a syringe that is pre-drawn into his lunch box if he is going to school.

Plan B-Add Bromide. Cole has failed Phenobarbitol, Depakote, Topamax and Clonazepam. He has 7 seizure types and is progressively getting worse. The doctors definitely classify him as complicated and intractable. So, Bromide is an "End of the road" drug that we are willing to give a shot. The only one really left is Stiripentol and we are not ready for that yet. We don't have the money.

Plan C-Look at something that we have tried in the past, with a different dose or combination. We really don't want to get to Plan C.

Here's to hoping that Plan A and B work out!

Oh, Murphy and Your Law.....

I escaped to the Fortress of Solitude (aka the movie theater) last night, alone, just because I needed a serious break from reality. Thanks again to my friend Ruth for suggesting that I take a time-out there.

As I was waiting for the previews, I thought of Mr Murphy and his law-anything that can go wrong, will...and then I laughed to myself.

I feel like I should be walking billboard for this adage, lately. Especially in the last 72 hours. If I were Jewish, there would be a lot of 'Oy' scattered throughout this post.

I mentioned that we are in the middle of an intense med wean. We have now had to come to a standstill. The road just does not fork presently in order for us to take another direction, at least until after we get home from Ohio.

Cole is not doing so hot right now, there are still a lot of seizures and 4 out of 5 days at school last week he needed rescue meds and oxygen. His sleep has been wack-a-doo, his appetite so-so. His behavior...still bad. Until further notice, being outside is now off limits. It has been a definite trigger.

We can not go any lower on the Keppra without adding something else, because that will cause him to have more big seizures. Less behavioral issues, but the larger ones and overstimulation problems have definitely increased since the initial wean of this med.

We can't start the Clobazam because 1. It will change his body chemistry and therefore his scent...not good for the dog right now. 2. We always have at least a 2 week adjusting period when starting a new med. Sometimes it's a honeymoon, sometimes it is really bad. Either way, we will not be able to tell what is what when we add a new environment and being really overstimulated at training into this equation. Is it the meds? Is it the temp? Is it? Is it? 3. Some people see a decrease in seizures when they first get their dogs. Not seizure freedom, but a small decrease. We need to know exactly what is going on with his baseline so we can see if he changes. 4. It just plain might not work. It's a lot like two other drugs that we have tried that actually made things worse. So, we just have to hold on to our hats and pack a lot of rescue meds around. Dog-who-is-yet-to-be-identified...we're going to give you lots of practice.

All the while I have had the lovely experience of shuttling back and forth to the dentist and being on antibiotics. I have an infection somewhere in my mouth, hopefully not IN my tooth. I probably need a root canal and a crown, and get this...our insurance is not active until Jan. 1 (that's a fun story)! I have an appointment with an endodontist on Wed to see just how much work needs to be done. Y'all know that mouth pain is no bueno and in turn I'm averaging around 4 to 5 hours combined sleep at night. I might actually be flying to Utah over the weekend so I can get my procedures done first thing on Mon. Turns out, without the help of insurance...it is WAY cheaper out west. Even with the plane ticket. Plus, I trust our guy out there. Here, is uncharted territory. Who knows, maybe I'll be able to throw in a lunch with my besties? Now, I am also having pain on the other side...say what?! More info on that once I see the endo on Wed. Maybe he will know what the heck is going on in there.

I sent a 4 page letter to the Superintendent, Principal and some other important people on Thurs about the dog and the law. Have not heard a word back. We had to call a plumber. The car needed a bunch of work done in order to pass inspection. We had to steam clean our couch. Still working on the oxygen fiasco. Getting closer to a solution. Six months later, but still, progress! :)

Sunday, Cole had a small seizure as he was going down the stairs and it threw off his balance. He tripped and fell down the last two or three. He kept saying his foot hurts, there is a bump on it and he was still limping Monday. Plus, an increase in seizures which means he is either in pain or getting sick. Nothing else had changed. So, we took him in for an x-ray, which was clean. Does this sound familiar? Last July, we let it go and came back 4 days later to find there was a stress fracture, (this is the other foot). With his history of high pain tolerance and a previous break, we were not going to take our chances. SO-Cole is in a splint and has to be non-weight bearing until we can see the pediatric orthopedist on Friday. Cole. Non-weight bearing. This is going to take some creativity.

On a positive note, I have been cooking a lot lately. Unfortunately, it tends to happen when I am really happy or really stressed. Been cranking out some good stuff. I watched a Doris Day movie in my bed. Cole giggled. None of us are IN the hospital. Our couch is clean! I think we have a couple of nurses with good potential (now if we can just fill all of the shifts).

It was so nice to just sit in the dark, with hardly anyone there, and eat my sandwich and cupcake in peace last night. FYI, eating a cupcake in the dark is dangerous. I had crumbs in interesting places :) There was no one to take care of. I was forced to turn off my phone, no waiting for matches, e-mails, calls, or interruptions. Just me, turning my brain off and becoming engrossed with the story. It has been so long since I did something like that. Superman goes to the Fortress of Solitude when he needs to be alone. Me, I'm going to the movies. Murphy can't bug me there, even if it is only for 2 hours.

*I'm so frazzled that I was speaking about yesterday, today, etc...when some of this stuff happened over three days ago. Mercy! I have fixed it.*

Rageaholic

I will mention that even though it occasionally feels like I am being kicked while I am down, I am in fact grateful in a sick and twisted way that I have had problems with nursing and school. It means that Cole is actually getting the opportunity to have both. I just wish that someone had come before me to give me the 411 on everything. I guess that I am paving the way. I am still whole heartedly pushing for a Dravet community here :) I'm not regretting the move, not at all. Just think, anyone else who comes, I will know EXACTLY what to do to get what we want! :)

Moving on....

Update on the med situation.

Oh, did I mention that we are changing medication? 6 weeks of torture...coming up! We have already been at this for 5 weeks (not including when I took Cole off of the NanoVM and Carnitor), there are 6 MORE weeks to go! It is hard to just add or get rid of medications. If you just switch things around and up and down, it gets confusing as to what drug is doing what. So, we decided to take it slow...and I mean slow.

We started with a SLOW wean of Depakote and Topamax. We will be adding Clobazam (the med from Canada), but only after the Keppra wean. We are to the stage where we have eliminated the Depakote and Topamax. I was not half as scared about them as I am about touching the Keppra. 72 hours off of Topamax, Wednesday, it kicked in. The Rage is back. You may recall just how awful Keppra rage is. Let me remind you here or here, if you have not read about how each time we touch this drug bad things happen.This cutie? He has disappeared.
This smiling, mostly sweet boy? He has been replaced by a hitting, tantrum throwing, kicking, screaming version of himself. Seriously, the devil inside has been awakened. We have not even touched the Keppra yet. It is just being metabolized differently now that the other two (well, actually 4) drugs are gone and the strength is intensified. We have to go slow, since we will not be completely taking him off of this one, just lowering the dose.

I'm not sure I'm going to have any hair left by the end of this. There is a promising little box from Canada sitting on my counter and I just have to be patient. I can't touch it yet. Especially since there is now a shortage, you have to import it, and people are running out! Mercy.

I can't wait until HE comes back.

Dr Mommy, MD*

Ever the scientist, I have decided to conduct an experiment while our doctor is on vacation. Keep the AEDs (anti-epileptic drugs) stable, and get rid of the multi-vitamin powder and Carnitor. hypothesis: Maybe these two "non-critical" drugs are actually impacting seizure control.

Cole has been on Carnitor since the introduction of the ketogenic diet in June 2010. We were told that it would help protect his liver and it would give his metabolism a boost with all of the fat in his diet.

I asked if it had any contraindications, (just like I do with any drug we go on). They said no and the literature would suggest that. It is not contraindicated with any other drug. They also said: It is practically impossible to overdose on and might cause diarrhea. Okay, diarrhea is the least of our problems, and we dismissed this "safe" drug and focused on the life-changing implementation of the diet. We had much bigger things on our plate. Time passed (almost a year) and we slowly dropped the diet. However, we chose to remain on Carnitor because it couldn't hurt to give his liver extra protection if he was on Depakote, right? The doctor totally agreed. So we stayed on it. Had blood tests, things were peachy.

Here is where I messed up.

I asked questions, like I should, and took the answers given by a medical professional to be the complete answers. Remember, as awesome as they are, doctors practice medicine. I think I scanned the prescription leaflet once when we first got it, but did not see anything that stuck out to me that would be worrisome.

This is what the fine print says on the Carnitor website (which I didn't look at until recently).

Before using this medication, tell your doctor or pharmacist your medical history, especially of: kidney disease, seizures. Um, yes. Dilated kidneys in utero, the diabetes insipidous! It may be the Carnitor that is putting pressure on his kidneys? What?! NO one thought that it could be the Carnitor and we have seen 4 different doctors regarding this issue.

Seizures have been reported to occur in patients with or without pre-existing seizure activity receiving either oral or intravenous levocarnitine. In patients with pre-existing seizure activity, an increase in seizure frequency and/or severity has been reported. NO one ever mentioned that Carnitor could increase seizure activity. Even the people in Miami. I don't remember it being in the info packet from the pharmacy, but there it is...in black and white.

Fast forward to the beginning of the week.

I am constantly trying to re-asses things and see if I can make them better. I don't really make any serious moves without Dr's approval, but I am usually the one to suggest a new route of medicating. I am the instigator. Not the norm, but I have a relationship with the doctors that is trusting and when I suggest something, they will tell me if they feel it is a good idea or not. Neither the neurologist or pediatrician has the ego where they believe that only they know what is right for my child. I'm lucky. They understand that I have dedicated my life to making a better quality of life for my son and I usually have done plenty of research before I make a suggestion.

Like I mentioned, the doctor is out. I've done stuff like this before with sugar, instigating Modified Atkins by myself, removing extra Magnesium, etc. Although I would not suggest this for anyone else. I have had 30+ years of experience with epilepsy and have worked in the medical field for a long time. I never do something like this without careful thought. Always consult with your doctor before making changes, especially with prescriptions...disclaimer over :) I thought, "Let's just hold these two and see if there is a difference". The multi-vitamin, while hypoallergenic and pricey, has to have some sort of bonding agent to keep all of those vitamins together, plus flavoring, etc. There could be something in there that doesn't agree with Cole. The Carnitor, we're off the diet, on a teeny dose of Depakote, do we really need to "protect his liver", like we have been assuming all along it was doing? I knew that we could have results, or things would stay the same. It was worth a shot.

Cole has been without his multi-vitamin and Carnitor for three days. His seizures have dramatically decreased. They are still present, but he can go occasionally go a solid 10 minutes without any type of noticeable activity. Miracle.

He has been a lot more calm and his attention span has been lengthened-CARNITOR® (levocarnitine) is a naturally occurring substance required in mammalian energy metabolism. It has been shown to facilitate long-chain fatty acid entry into cellular mitochondria, thereby delivering substrate for oxidation and subsequent energy production. Fatty acids are utilized as an energy substrate in all tissues except the brain. In skeletal and cardiac muscle, fatty acids are the main substrate for energy production. So, adding energy to a child that already has attention issues and hyperactivity. I totally see how this is another reason that it was prescribed for keto, since most children can become lethargic, taking all of the carbs out of their diet. He has been nicer and more cooperative since its removal.

Even though he is having seizure activity, most of the clustering is concentrated around falling asleep, waking up and fatigue. I even did another experiment and took him to the zoo yesterday. Without pre-loading him with drugs. We were in the sun, he got in and out of the stroller multiple times, it was incredible! Usually, once he gets out of the stroller, there is no getting back in. He paid attention to the animals and exhibits and occasionally asked simple questions. It was like I had another child! We got through 3 hours at the zoo and a long time in the car (gotta love traffic) with no extra pharmacological intervention.Awful pic, sans animals, but it was the only one I got of him actually looking. Things aren't completely revolutionized :) It was a wonderful, almost "normal" day. Yes, we had a nurse, hat, glasses, oxygen and a really heavy bag full of medication, etc., but we did it!

So here is a warning to all of you. Don't take the answers you get from medical professionals as complete information. Look up the drug on it's own website, read the entire thing. Trust the professionals, but do your research! We have had this happen to us more than a couple of times now. It has usually been in relation to some sort of AED. Most doctors will titrate to the highest dose of an AED to see if it will be effective. Usually, with Cole, the higher the dose, the worse things get. Who knows if Carnitor really did protect his liver during the diet? His lab values were always normal. I can't beat myself up about it. We have been on this drug for over a year and no one has suggested stopping it. I do know that I will be suggesting that we take him off of it, after completing my week-long experiment and having proof.

Now to figure out if that multi-vitamin is really helping. Always some other experiment/adventure to embark on. You are your child's greatest advocate. Sometimes, Mommy doctors really do know best.

*UPDATE: After polling the masses, it turns out that Nano VM has indeed been the cause of more seizures in Dravet kids. Bye-bye multi-vitamin! I LOVE my social network buddies. Ask a question, and multiple people will give you the answer. I just wish that I would have asked earlier. I need to start paying more attention to the online threads, I usually just scan them when I have the time. I could have had this important information without my trial and error experiment. Que sera sera. All of these other Mommy Doctors are invaluable to me! It is so strange how our kids are each so different, yet so the same.

Drugged

Dravet is a very complex disorder. Things are constantly changing, especially in the first 5 years or so of life. Cole is a true testament to, "Never Get Comfortable".

We had a great honeymoon with Depakote. You may recall my love letter. His long seizures were less frequent, they were shorter. He seemed to be able to handle overstimulation better and started exploring the world outside of our four walls. There were still plenty of seizures, but most of them were small. Then the small seizures increased to the point that I had to use extra medication to stop the clusters. Almost every day. So we decreased the dose and added Topamax. We changed things around, lowering the dose twice and things seemed to even out again where I was only using extra rescue meds a couple of times a week instead of every day. That lasted for a week or so and the myoclonics were back, with a vengeance. It seemed that he couldn't even go 5 full minutes without some sort of seizure activity. So we changed things again, and they got worse...not better. So we doubled one med and they got just plain awful. Cole was only having 45 seconds to a minute of respite in between clusters and he also started having a lot of myoclonic eye flutters, something he has only had a handful of times in his life so far.

SO-what do we do? More meds=more seizures. Less meds=more seizures. We can't go back on the diet or he will stop eating completely. Neurofeedback didn't work. Homeopathy didn't work. SPIO and other special equipment is not enough. Is it time for stiripentol already? I'm not sure what to do since we have tried Plan A, B and C. Is it time for Plan D so soon?

To stop the cycling, today we gave him a loading dose of clonazepam, "To throw water on the fire". We've done this with other meds before, but never with a benzodiazepine. The doctor reassured me that it would make him very tired and told me to make sure he got a good nap. We've used clonazepam before as a rescue drug (0.25 mg) and I knew it worked well for Cole to stop small seizures. So, with a quadruple dose in his system (basically 4 pills equaling 1 mg), plus 3 other seizure medications, he should be knocked out cold, right? Um, no...if you recall, my son has the nickname "Mini Hulk". He can withstand incredibly high doses of medication and has a very high pain threshold. Remember when I won the Mother of The Year Award when I broke his foot during therapy? Yeah, high tolerance for pain and drugs is an understatement. I actually chuckled when I read the new protocol this morning.

Instead of sleeping, like a regular person would if they had this amount of benzos in them would, he is a total chatterbox. Right now, he is alternating between talking like a pirate, singing Old MacDonald, and having conversations with inanimate objects. You can totally tell he is drugged, especially if you know him. Yet, hardly a seizure since the big dose. Oh, it is such a balance. Seizures or being strung out? All in the day of the life of Cole.

Home Of The Brave

We have now been on the new med for over a week.

What I have noticed in the days I have been able to observe:
-The myoclonics have been reduced.
-The morning is still hard. He's struggling to wake up and has a lot of myoclonic activity.
-Cole is back to sleeping more, which he always does with the intro of a new med. He's averaging 12 hours and 2 hour naps, with waking 1 or 2 times in the night. It doesn't help that he has a summer cold (official diagnosis from the doc), which is causing him to sniff all the time and be congested.

It seems that we have kept the overstimulation tolerance with the decreased Depakote. We took him on the metro for the first time to go and see fireworks on the mall. I must admit, I have high expectations when it comes to celebrating the 4th. I come from party people and a small town that goes all out. BUT, if I have to miss the celebration in my hometown, seeing Josh Groban for free in my new hometown is quite pleasant, thank you :) It took a lot of guts because we knew that there was going to be lots of people, noise, etc. We pre-loaded Cole with Valium and he did great! The way back home was more crowded than I have ever seen the metro, it reminded me of Hong Kong when everyone is going home from work. Just tuck your elbows in and hold on for dear life! Luckily, we found a little corner and Cole just acted oblivious to the chaos. Thank you, Valium!After multiple discussions, we decided to try Topamax instead of Clobazam because they have similar properties and we could get it immediately. So far, so good I guess. We are still going to start the paperwork to get Clobazam, but it is taking 10-15 days to get it into the country. SO, we'll just have it as a back up in case the Topamax doesn't work out. This being said, I think we are going to stick to this med for at least a month...he is only on a very small dose and it seems to be making a difference.Speaking of bravery, this week has had so many "firsts". Besides going on the metro and celebrating the 4th somewhere besides ID or UT, we started our nursing shifts! This has been such a great, weird, fun, stressful, tiring, wonderful, sad thing. I know...lots of emotions; but, it's the truth! Having to find someone that is qualified and then basically give them full trust in the very beginning of a relationship and counting on them to keep your child alive is strange. It takes bravery. It's also uncomfortable at times to realize that someone who is practically a stranger is sitting, awake in the next room while you sleep. Uncomfortable, yet comforting...so ironic. So we have had our first night nurse, our first day nurse and I've tried to map out a schedule. The list of things that I want to do in the next couple of weeks don't even really include fun stuff, it's totally boring things like organizing the pantry and cleaning the storage room! We'll be sure to throw in some fun stuff too :) It has been the first time that I can be in the house, but not have my eyes on Cole at all times. Weird, but a burden lifted.

Having to put the pulse oximeter on Cole every single night and sleep in a bed with me, without Cole, has also brought all of these emotions that I mentioned out in Brian. It's just another reminder that things are different. Truly though, we have entered another season in our life. We went to the grocery store together, without Cole, last night for the first time in years. It was strange, but enjoyable. We weren't racing through trying to get things done as fast as possible, it was actually kind of pleasant! :) The whole situation is just going to take some time to get used to. All in all, the bravest one out of all of us is this guy. He's a warrior.

The Depakote DL

Cole's level came back...after 4 phone calls and a few e-mails :)

It was 95, a trough, first thing in the morning before his dose. The spectrum for therapeutic is typically 50-100, except that each kid is different, and some need to be above this range in order to have the maximum effect. All in all, it was on the high end of this range for a relatively small dose.

Our plan of action is to decrease the depakote from 500 mg a day (250 morning and night) to just a small dose of 125mg daily to see how the myoclonics react. We decided to try this first before going straight to adding another med. One that is not FDA approved and therefore costs more probably had something to do with it too!

Today was the first dose on the new regimen, after skipping his usual 250 last night. Let's just say...I'm glad we went down instead of up! Cole has had the best day seizure-wise that he has had in weeks. The morning was rough and there was still plenty of activity, but nothing compared to the last few days of constant jerking and twitching.

Fingers crossed.

Shout Out

Thanks to all of you who called, texted and responded on facebook. This is something that I truly treasure about our community. The doctor will give me one option, for now, but I want to have multiple plans. As well all know, Dravet means constant change.

After polling the masses and see what is working for everyone else-I got the same answer over and over again and I feel like I can make an educated decision!

We will let everyone know what Cole's depakote trough was as soon as I can (probably Mon). After 5 blood draws, you think I would know by now :)

It seems like the answer to decreasing the myoclonics is to add Clobazam. Hopefully, I can get that pretty little Medicaid number in the mail in the next week. I know we're approved-I just need the card! Clobazam is covered by VA Medicaid...hooray! Patience, Niki, patience.

Thanks again. A huge pat on the back for everyone who responded. We'll keep you posted.

Dazed And Confused

This week.

Things have just been getting worse it seems. We're still gung ho on trying to get out and live a little. We even went on an adventure to IKEA and had gluten-free pizza at a restaurant! Hooray! I'm tenatively planning on taking Cole to a birthday party this weekend...I know, could be a total disaster, (good thing the birthday boy's brother has DS). It looks like we are doing fabulous on the outside and I'm not going to lie, being able to spend time together outside of our home has been incredible. Seizure-wise though, things have been rough.

Brian and I have noticed some big improvements since the start of depakote a few weeks ago. Cole's speech has been more clear, he has enhanced motor skills and his overall tolerance for being overstimulated has been increased. The latter has been the biggest pay-off. We've been out and about again, within reason! It feels like going back to being housebound would be equivalent to someone handing us a pair of prison issue striped coveralls and saying with a pitiful look, 'Good luck in there". Cole has been, on average, happier and we have too for the most part.

The thing that really sucks (pardon my French) is that his seizure activity has been through the roof! There has been such an increase in myoclonics especially. It seems like each time I look at him, he is jerking. I'm shocked that he isn't more worn out, because when he is sleeping it is like being next to a machine that is programmed to twitch. I have not even attained a good shirt for the dog this week because he has constantly smelled like a seizure and would have nothing "normal" to compare it to. Well, normal in Cole's world. The whole process of scent work depends on comparing the smell of "seizure Cole" and "normal Cole" so that the dog can tell the difference and know when to alert. I have a feeling that if we had our four legged friend this week, they would be working hard. Something like our friend Hobo did with his girl yesterday. He wouldn't even go outside for a potty break. Sweet Hobo...we love him! I have given Cole Valium or Diastat every day this week for clusters of partial-complex and myoclonics. Every day. It almost transcends from rescue med to daily meds, just at random times. It's not when he wakes up, or even when he is getting close to needing another dose. There is no peak that I think changing to giving the Depakote three times a day would fix. It is an all-day, every day occurrence.

So the question is, once again, what do we do? He IS having more seizures on this med. Strange as it may seem. Yet, there have been improvements related to controlling the sub clinical seizures, as evidenced by his speech and motor skills. Do we stay on Depakote for a little longer, upping the dose and trying to get therapeutic? Do we just back off now before it gets worse? Do we try something else? Do we go back to just Keppra and see its efficacy without the diet? We can't jump back on the diet train right now since Cole has obviously shown that he is not going to stay within its boundaries and still eat by mouth. Am I ready to have a child that is tube fed daily? I think that I know the answer on that one...nope, at least not right now when he can still take things orally. We also know that we are not ready for Stiripentol yet. The price is one thing, but learning from other parents we also have found that it can lose its efficacy over time, just like all of the other drugs. Cole is only 3, I don't want to use my Ace in the Hole yet.

So many questions, and no clear answer. I would love to hear what you think. Now I know that over 200 of you check this page out daily, sometimes more. Then again, maybe Google Analytics is lying to me and there is just some bot set up in Indonesia that visits my page every half hour or so :) Please, come out of the woodwork! I think that you have all fallen in love with Cole too and know that a lot of you have been in similar situations. I'm just at the end of my rope right now when it comes to meds. My brain has slipped back into the confused haze and it is fighting back most at night. I'm lucky if I stay asleep for more than 20 minutes before I wake up again with a racing heart. I really just want what is best for him, but there is no clear answer in sight.