We just got back from Cody's neuro appt.  Dr. Saneto spent a whole hour with us - Don even attended which is always nice.  It's great to have a 2nd set of ears to hear what I hear.  Especially when it's medical talk! 

The last time we talked to Dr. Saneto it was at the ER 2 times in 1 week!  And he had told he was very worried about what would happen to Cody if he had even 1 more seizure that day.  That freaked me out.  Luckily Cody didn't.  But I don't like getting that close to catastrophe.  Kids can die from infantile spasms - and it can happen when seizures start to cluster on top of each other putting them into status epilepticus (a seizure that won't end even with rescue meds.)  That's what we were looking at - his seizures just wouldn't stop no matter how much diastat we pumped into him.  But right after I heard the Dr. say "I will be very worried if he has 1 more" -- they stopped.  We've been on pins and needles ever since.  But I was happy to tell the Dr. today that in the last 19 days since our "ER nightmare", Cody's only had 3 days of bad seizures.  That's quite rare given that most months he spends about half the month with bad seizure days.  We *think* it's the Topamax, but who knows?  Seizures are unpredictable and flukey.  But at some point you just have to look at what's changed - in this case we added Topamax - and go with that.  So if it is the topamax, then it's doing something right.  Altho 3 bad seizure days in 19 still ain't great.  so we came up with a plan.  That's one of the many things I like about Saneto - he's big into "plans."  Every time we leave him or I email him he says, "so we have a plan!"  I like that - makes me feel like he's partnering with us and we're all in this together with this "plan" in place.  So our "plan" is to leave the Topamax where it is and phase out the trileptol which we were in the process of doing anyway.  He said the trileptol may be blocking some of the topamax effectiveness and by clearing the trileptol out of the picture, the topamax may do even more.  and if not, he's on a very low dose so we have lots of room to go up on the topamax.  and thankfully the dopey side effects are gone.  in fact, right now, i don't really see any negative side effects of the topamax - except a little drowsiness after the morning capsule but Cody's usually able to power thru the school day anyway.  He was also on a very low dose of clobazam to bridge him out of the ER madness and we're taking that away too.  So we'll see how he does just on lamictal & topamax - with room to go up on the topamax.  And Cody is now in the queue for the VNS surgery - hopefully in August when he has no school or ABA the last week of the month.  And the same time he's under anesthesia for the VNS surgery they'll do a fresh muscle biopsy to try to discern any mitochondrial disorders. 

Now reg. the muscle biopsy - we were going to have to fly to Cleveland for that but luckily Children's in Seattle does them now.  They'll take a sizeable chunk of Cody's thigh muscle (I bristle at writing that....makes me so sad) and analyze it for a myriad of mitrochondrial abnormalities.  There are only 3 mitochondrial issues you can treat - that will eliminate seizures.  All the rest only give you a "cause" of the seizures (like a calcium transport deficiency....or a sodium channel deficiency...etc.)  But at least we'd know what is causing Cody's seizures and brain issues.  The 3 you can treat, he doesn't have because we've already explored those.  For one of them it's a simple dose of Riboflavin and seizures go away.  Didn't work.  The other is a carnitine deficiency but Cody's carnitine levels are normal.  The 3rd has never been found in a child with infantile spasms so it's a long shot - can't remember what the name of the deficiency is.  Mito issues are Saneto's specialty and that's why we wound up with him -- so he said there's a decent chance we may find out what's wrong with Cody through this biopsy.  We hadn't done it before because it required a trip to Cleveland which is the only place where they did "fresh" biopsies rather than having to freeze the muscle and send it away.  Gross!  Even if the mito is untreatable, it would give me such peace to at least KNOW what's wrong with my son.  Every day I am tortured by the question - what is causing this!?!  I pray we'll find out what it is.  If this muscle biopsy doesn't reveal anything - we are out of causes to explore.  Our only other recourse is going to the genetic dept. at the UW every 3 years to see if there are any new genetic causes that have been discovered to test Cody for.  For example, Cody may have a genetic flaw that hasn't been given a name yet - therefore he's unable to be tested for it.  But new genetic defects are discovered every year and tests are developed to determine them.  So that's our only hope after the biopsy. 

As far as the VNS goes, we learned something interesting.  We knew the stats - VNS works in 50% of patients.  And in those 50% it reduces seizures up to 50%.  So it's not the best odds, but it's better than nothing.  But here's the interesting part - it takes 3-9 months to become effective typically.  And that's because there's evidence that it actually rewires parts of the brain.  For instance, if you stop an anti seizure med, seizures come back pretty immediately.  But if you stop the VNS, seizures stay away for a few months.  So Dr's have determined there's some level of work the VNS impulses do on the brain that actually re-wire the problem areas.  Which in our case is big news.  *If* the topamax takes away all the big seizures, we will 2nd guess whether to do the VNS.  Although the small seizures occur about 20 times a day right now and still aren't great.  But it's the big ones that really are the problem.  If the Topamax keeps the big ones decreased as it seems to be doing, then we'll do the VNS surgery in hopes of the VNS and Topamax working in tandem to remove them all - or at least a good deal of them. 

The VNS is not a small deal.  They implant a little battery operated pacemaker (it's gotten smaller in size - it's about the size of a silver dollar.)  Then they run wires from it into the Vagus Nerve that goes up the neck and into the brain.  Yuck.  The pacemaker is regulated to send a charge thru those wires every 3 minutes (or however many minutes apart the neuro determines is needed.)  You fuss with the settings over time to try to get some seizure control.  If it's deemed to not be working after the 9 months, you leave it in for life.  Again, yuck. 

So that's our "plan" - stick with Topamax - schedule the VNS & muscle biopsy - and pray. 

We also discussed stem cell therapy (we banked Casey's cord blood just in case.)  But he said (for lots of medical reasons I cant' remember enough to repeat) that it wouldn't be an option.  Primarily because it's not a matter of creating new neurons for Cody's brain - it's the fact that the neurons aren't firing properly. 

And as usual I asked my stock question: what's in the future?  He said it's still the case that Cody's brain could get better or worse over time.  His seizures could get better or worse over time.  As his frontal lobe develops, though, the Dr. said there's the definite probability that Cody will be able to control his body better which means less stimming, less low tone, less constant motion.  That was encouraging. 

All in all, exhausting.  Those appts. take the life out of me.  But being in the waiting room at the neurological dept. is always sobering - and always makes me grateful.  There are so many children with neurological damage who are so much worse off than Cody.  I am so glad for the miracle that he can walk.  And that he can hug, giggle, clap. 

And as God would have it, I ran into Debbie and Hudson in the waiting room.  She's one of the moms who came to my "mommy-hero" brunch.  She lives in Gig Harbor and Hudson was diagnosed almost a year ago.  He's on the keto diet and she was waiting for a blood draw with her two other dear little children.  Talk about having your hands full!  She has a baby with i.s., and two older kids who need all kinds of attention and care.  Hudson had a messy diaper so she had to haul all of them out to the car to change him, then haul them all back in.  She did it all with a smile and a shrug - as if it were no big deal.  Debbie, if you're reading this, you are so amazing!  God sees every time you drive up to that hospital, take all 3 of those kids out of the car, find creative ways to keep them occupied during the many Dr. appts, hold back your tears as you see other babies who aren't riddled with seizures being seen for small things like ear infections, speech impediments, etc.  It was so nice to see you today....God continues to show us we are not alone.

with love,

Shawna