Year In Review-Part 3

This is part of a series catching up on 2013!  See the other posts here:  Part 1 and Part 2

SEPTEMBER
Cole doesn't start school on time.  He is just too unstable.  We see a Integrated Medicine doctor.  An MD, that also practices natural medicine.  She has a theory about my adrenal insufficiency.  Cole finally makes it to school.

 We LOVE his team!  We totally know what it is like to not have a support system at school and consider ourselves so incredibly lucky to have the amazing people here.  He really struggles and has to start wearing a bib in September that has to be changed constantly because of the drool.

 I even send him to school in his wheelchair when he can not hold up his head or walk.

 Has not been eating at all for a few months and has been solely tube fed.  Sometimes it is nice to have breakfast where super hero gear is mandatory.

Made a custom letterpress invitation for Gourmet Club.  Find out I need double knee replacements.  Start 6 weeks of collagen injections under my kneecaps.  I am a huge fan of Lidoderm patches, even if you run out of regular Co Flex and need the dinosaurs to keep them on ;)

 We continue to explore treatments for Cole.  He is really struggling.

 Government Shutdown starts.  Total Major Seizure Count: 273

OCTOBER
I am asked to give a class on Style for a group of young women.

 I go to New York for a conference.  We get adorable family pictures taken by my talented friend.  Celebrate Slugger's birthday with a trip to Cox Farms.

 The light goes out of Cole's eyes and he is zoned out for over a week.

 I host Eat, Drink and Be Scary!  Had so much fun with my theme this year.  Glowing green ice cubes, dry ice, all sorts of fun and fantastic food.

 Cole pulls out his g-tube.  That makes my heart race more than any seizure nowadays.  Cole is on antibiotics.  School is like a petri dish.  I break my foot, nothing they can do about it.  It's Diffendoofer Day at school.  I take Cole to the zoo.  Decide to take Cole off of all benzodiazepines.  He is a full blown drug addict.  His body constantly searches for the next "hit" and ends up having more seizures because of it.  Long story short, rehab at home is not fun.  Realization that the hospital just can not help us any more is difficult.  I start seeing a doctor about losing my hearing and Tinnitus.  I will probably write more about that later, maybe...don't hold your breath :)  We are Super Heroes for Halloween.

 Meet Captain Canine, Super Cole, Tiger Mom and Dad...who doesn't need a secret identity.  Total Major Seizure Count: 200

NOVEMBER
Fall weather is perfect.  The leaves are amazing.  Cole gets sick again.  More antibiotics.  We start dealing with thrush as a side effect of not eating and drinking by mouth.  We go to the Newseum.  Fall baking is in full force.  Cole starts having cycles of "good days" and "bad days".  6-9 days that are almost seizure free, then 3-4 bad days where he can have 60+ major tonic clonic seizures in one day.

 We are just grateful.  Even though the bad days are really bad.  The good days are good and he can recover.  Before, he was having bad days, every day and was regressing because his brain and body never had a break.  Cole stops having nocturnal seizures every night.  Only when he is in "cycle".  After 7 months of fighting with Medicaid, we get 16 hours a day of nursing.  Totally changes our lives, since we can now have night nurses.  We are getting to see glimpses of our little boy coming back.

   Total Major Seizure Count: 257

DECEMBER
We have ice storms and more snow than we know what to do with.  DC seems to panic and shut down every single time there is a storm.  I get cortisone shots in both knees so I can play tourist with Grandma and Grandpa when they come for an early Christmas.  We eat a lot.

 Don't get to go home for Thanksgiving, Christmas, New Years, birthdays, baptisms or Grandma and Grandpa's LDS mission farewell.  However, Christmas Day is fantastic for Cole and it is the first holiday ever in 6 years that he has enjoyed and was able to participate in traditions.  Best day EVER!

 Mom gets hearing aids for Christmas.  They are actually quite small and blend easily.  You can see here where I have a top knot, you can still hardly tell they are there.

 Again, I will probably, maybe write about the emotions connected to this.  Total Major Seizure Count: 204

All in all, we had an absolute whirlwind year.  This is the major Reader's Digest compact version, with a lot of holes.  Sometimes it is amazing to take a step back and realize how much can change in twelve months.  All that matters at the end of the day is we are trying our best.  I still get to snuggle this little guy who is not so little any more!  He is a true warrior and makes me want to keep fighting, even when it seems like I don't have the strength.

Year In Review Part 2

This is part of a series covering 2013!  See Part One here

MAY
Went on the Embassy Tours.  One of the coolest things I have ever done!

Cole is super sick.  Attended two Derby parties, hooray for hats!  End up in the PICU.

Find out that one of our Dravet friends that we know personally earned her wings.

Did a lot of Zumba with friends. Got a chunk surgically taken out of my scalp.

End up in the PICU, again and then again.  We were basically in the hospital for three weeks in a row with only a few days break in between admits.  Total Major Seizure Count: 54

JUNE
Went downtown with some great friends.  Started a major obsession with the Library of Congress.  Found out I don't have Lupus, I have Fibromyalgia and Osteoarthritis.  Say good-bye to some of our dear friends when they move.  Why does everyone around here move constantly?!  Seizure control is null and void.  Having up to 16 to 20 TC's a day.

 Get pretty desperate and almost Life Flight Cole to Miami.  Instead, we take a risk, drug him up, pack our bags and drive to Florida.

 Stay inpatient for a week and do a major wean of Phenobarbitol while we are there.

 Total Major Seizure Count: 95

JULY
By divine intervention, Brian and Niki get to make it to Hawaii on a trip that has been planned for 6 months with her family.

There is a ratio of 3 adults to 1 Cole while we are gone.  7 Nurses switching off around the clock and help from friends who are salt of the earth.  It was Brian's first trip and Niki's return to "Home" where she used to live while going to school.  We jump off a 45 foot waterfall and try to have a good time even though we are thoroughly burned out.  Niki got a baseline EEG since it had been 8 years.

Slugger goes to 4 Paws for Ability for some refresher training.  We ended up in the PICU, again, while he is gone.  Had a 2 hour status seizure.  We start importing Stiripentol from France since we have tried just about every thing else under the sun.  See the list.  Total Major Seizure Count: 244

AUGUST
We take Cole to Seattle Children's Hospital.

 He has 6 TC's before we leave, 1 at the gate, 10 in the air, 2 at the airport after we deplane and multiple after we left baggage claim.  One of the most stressful days of my life.  He has muscle biopsy surgery to determine he has a Mitochondrial disorder on top of Dravet Syndrome.

 Our summer vacation was flying across the country, having 9 Dr. appointments and 1 surgery in 4 different locations in 5 days.  We even have sleep studies in two different facilities on the same night.

Cole is so drugged from the Stiripentol that he is Zombie-like and he doesn't have a nocturnal seizure, like we had been experiencing every night for almost a year.  It always happens that way :)  My sleep study shows I am sleep deprived.  I paid money for that?!  LOL

 Have a pistachio macaron at Pikes Market that I will remember forever.  Get to see some of our great Dravet friends.  Cole is having 30 to 40 big tc's every day.  Went on a night tour of the monuments.  It was perfect, highly suggest it.  Had an overwhelming display of love from family with "Cole Is My Super Hero" as the theme for our annual reunion.  Total Major Seizure Count: 176

Year In Review-Part 1

I decided to just do a little catch up post and it turned into a series :)  That can happen when you don't write for months at a time.  Here's a little about what we have been up to.

For a little bit of history right before 2013, look here

JANUARY
We start off the year in Utah.  After a series of Rocephin shots and a major miracle, we get to go home for Christmas.  Cole enjoyed sledding in Great Grandma's yard in between seizures.

Had our traditional fondue party.  Got to have lunch with some of my Tiger Moms.  We went bowling for Cole's birthday at least 3 separate times.

Had Snowquester.  Frontal Lobe Nocturnal Seizures are in full force.  None of us are sleeping.   Found out Cole was accepted for Make A Wish with Rush Status.  Total Major Seizure Count (this is mainly tonic clonic, drop in O2, convulsion, not nocturnal): 22

FEBRUARY
Started the second Gourmet Club season.  We start a long journey with Cole's bowels and end up in the hospital, twice for backed up stool.

Found our awesome Gastro, who in this small world went to med school with my cousin and used to live down the street from us in Utah.  Who would have thought?!  Had a Favorite Things Party, I can't help it-I love excuses to party!  Had a major friendship break up.  I don't think we talk enough about how this can impact us.  I liked this point of view.  Total Major Seizure Count: 23

MARCH
Took Cole to see the Sea World exhibit in DC in anticipation for Make A Wish.  Started a fitness competition with friends.  I start going bald.

Feeling very unwell.  Initial diagnosis is Lupus.   Went to Miami Childrens.  More tweaking of the meds.  Always tweaking.  Purple Day, we love it!  Had some of our wonderful friends come and stay with us for Easter.  Total Major Seizure Count: 33

APRIL
Went downtown to see the cherry blossoms for real.

First time we had just barely moved, Cole was in the hospital downtown and I was literally throwing up in the car as we headed home after discharge.  They were pretty through the window!  Second time, we had crazy weather and the bloom only lasted for 3 days, so by the time I made arrangements to get down there it was over.  My diagnosis has been changed to possible cancer.  Then back to Lupus.  Had a milkshake that changed my life.  Make A Wish.

 This deserves multiple posts.  It was such a bittersweet experience that we will remember for the rest of eternity.  Not sure when the documenting will actually happen.  The scrapbook that Crops Of Luv made for us is something that I will treasure forever.  I turned a year older.  Total Major Seizure Count: 31 (breaks record for most in one day with 6).

Stay tuned for exciting stuff like Hawaii, more trips to hospitals near and far and more!

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.

Glad I Listened

You know, after what seems like thousands of doctors appointments, it can get hard to absorb all of the information.

This week has been kind of crappy, pun intended.  Warning to the wise, I am totally going to write about bodily functions.

The boy has not been himself lately.  I took him to the doctor last Thursday because there had been an increase in seizures and some congestion.  Wanted to make sure that it wasn't something like this where there are no signs and symptoms of illness, but low and behold he truly is sick.  Got him checked for flu and strep, both were negative.  Ears looked good.  Lungs were clear.  The doc said he just had a virus.  So we just get to wait around for it to depart.

The weekend was okay, then things started to get rough.  Around Tuesday morning both Brian and I agreed that something else was going on.  Multiple times he would just curl into the fetal position, which is not normal for him.  Remember, this kid has a great vocabulary, but can not say, "I'm sick.  I hurt.  I feel gross.  My tummy does not feel good." etc.  Plus, he doesn't feel pain like the rest of us.  All the detective work I was doing wasn't pulling up any definitive answers.  Time to go back to the doctor.

I get there and our lovely Nurse Practitioner, who is more like a family member at this point, checks him out.  I tell her that I suspect a kidney stone or a bowel obstruction.  What would cause random expressions of pain, without any other outside symptoms?  He is eating, drinking, peeing and pooping regularly.  No fever, no cough, the boy doesn't look or act sick 98% of the time.  Just increased seizures and occasionally curling up in a ball.  I tell her about a previous visit I had about a year ago where the g-tube specialist told me that kids with g-tubes can get backed up with stool, even if they are having bowel movements.  It seems like everything is normal, but they really are constipated.  He also has a genetic pre-dipostition to kidney stones.  We agree that we are not taking any chances and we need some diagnostic tests.  She does a Urine and a CBC in the office, which came back fine, but alas there is no CT, x-ray, etc available there.

Off to the ER we go!  There was some miscommunication about what we were really there for and I expected the usual rigamaroll that comes with an ER visit, but this was just insane.  We were there for.ev.er.  I am usually such a fan of this hospital.

I understand that it is hard to believe me at first glance when I say my son is sick and not acting like himself, when he is running around the room and smiling.  Sure, lady.  He looks REAL sick.  So they waste a ton of time just trying to decide if I am crazy.  Then they decide to go to the opposite end of the spectrum after talking to a doctor that we rarely see and is totally on my bad list.  They order him off of his current medication, want to change it to something that we have already tried and failed, and want to order an MRI...of his head.  Because that makes sense!  We come in for abdominal pain and they want to admit him for two days and do a long, sedated procedure for his brain?  Mercy.  I am not here for seizure management.  He hasn't smacked his head...why are you ordering this?

After a few hours and explaining myself thouroughly to multiple people, they begin to understand why I don't want an MRI.  Once again, I was glad that I had listened at one of those millions of appointments.  Truly, Cole has only had one MRI and it was when he was 7 months old.  Things have totally changed since then.  I asked our neurologist if we should get an updated one.  He asked why.  I told him that we wanted to see if/how things had changed.  He then proceeded to ask me why I wanted to know.  Then, being the awesome doc that he is, told me that in the end it wouldn't change treatment. An MRI would show white matter loss, or a brain tumor.  Cole has Dravet, we know why he has seizures.  So, no brain tumor.  As for the white matter loss, it could totally be possible.  The kid has had thousands and thousands of seizures and episodes of hypoxia.  How would it change our treatment?  It really wouldn't.  Cole changes so fast, that we treat him in the present.  So, through that explanation I was educated/reaffirmed that sometimes you don't need to do major tests just for the sake of doing tests.  I already knew this based on my work history and my insurance bills, but I never thought in a million years a neurologist of all people would say that I shouldn't have an MRI for my child, who has a rare brain issue.  Good guy.  ANYWAY....

I let the ER staff (nicely) know that we are not here for an MRI.  We are not here to be admitted for "observation".  We want to check out his abdomen.  Can we do something about that?  They suggest a CT.  okay....Why are we doing a CT that has a lot of radiation and takes forever, when we could just do an abdominal x-ray and an ultrasound?  At this point, someone has finally listened to me and called Dr Miller.  Who, thank the Lord, told the doctor to listen to me because I know what I am talking about.  So-we get the x-ray and ultrasound.  Low and behold, the child has a bunch of old, backed up stool just sitting in his colon.  Ever been constipated?  Yeah, it stinks.  Times that by 10.  We would have never known unless we took him in.  Man, this child continues to amaze/confuse me.  Absolutely nothing but mommy instinct pointed to this problem.  I am just glad that it had not turned into a full obstruction and it was easily treatable.  Plus, Slugger got to feel like a movie star.  Without fail, every nurse on staff always visits our room when we come into the ER.  They love Slugger.

So, Cole has been drinking lots of PlumSmart and getting Milk of Magnesia.  He does not feel as crappy.  We will just add this to the list of crazy phenomenons.  However, the road is still rough.

Glad I listened to the g-tube nurse, glad I listened to the neuro when he told me that it wasn't necessary to do an MRI on Cole at this point.  Unless he has had head trauma or something, but not for a bellyache.  Otherwise we could still be at the hospital, admitted for nonsense and possibly catching whatever germs are floating around the building.

Cole is still not himself.  In fact, today we wondered if we were going to make another trip to the hospital.  This time for signs of low blood pressure.  The guessing continues.

In between playing detective we have had fun this week.  Valentines was always a big deal at my house growing up.  We declared the entire week up to it, "Love Week".  So for Love Week I have been making lots of heart shaped food, we got small presents, the house is decorated (unlike Christmas) and we have been enjoying Cole's excitement.  He was thrilled with the Valentines he brought home from school and little art projects throughout the week.  When he is asked who his Valentine is, he says, "Daddy".  BIG surprise ;)

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