I haven't written in awhile.  It has been a hard time.  First Casey got a little bug ~ nothing more than a fever for a few days.  He rebounded instantly.  But as we feared...Cody then got the bug.  And Cody does not rebound from anything instantly.  For our dear, poor little fragile boy...a 'little bug' lays him up for a week or more.  And it always involves at least one E.R. visit and countless Dr. appts.  It started with a high fever, then vomiting, then dehydration...and has now settled into his usual 'recovery' phase which means he sits like a zombie for days and does nothing but stare and sleep.  It always frightens me to the soul when he gets even a cold.  And that, my friends, is mitochondrial disease.  He simply does not have the energy stores to recover. 

Keeping him hydrated is impossible.  We went to Children's Hospital yesterday and I've been on the phone with the urgent care nurse every day - each day we are on the brink of bringing him back to the E.R.  Yesterday we also had our 'twice a year' appointment with his neurologist which I will get to later. 

Cody getting sick also means Don and I don't sleep for as long as the illness lasts.  Each night I've woken to give Cody motrin every 6 hours, and an ounce of pedialyte every hour in his G-tube.  Even keeping a mitochondrial child hydrated is different than most children.  

We are exhausted...sad...scared.  Seeing him so weak is always such a wake up call about this disease.  On days when he just stares and sleeps...he seems just so close to death.  I go in every 15 minutes all day just to make sure he's breathing.  I hold his little hand and stroke it ~ and he stares at me vacantly.  I'm sure he'll recover and go back to his raspberry blowing little self.  But these 'sick' days are always dark and forboding for us.

Speaking of forboding, I interviewed Cody's neurologist about just why getting sick is so devastating to Cody.  This was after Cody had just vomited twice in the clinic room...then spent the rest of the appt. staring with sunken eyes at the Dr.  Now some might ask why I'd even think of bringing Cody to an appt. when he's sick.  Well, when you only get 2 appointments a year...you don't miss one no matter WHAT!  We are lucky that Cody's neuro stays in close touch by email and phone between those appointments...we send him videos and give updates and he responds with recommendations.  But still there's nothing like seeing him eyeball to eyeball.  So anyway, I asked why these darn colds are so horrible and he said,

"Cody has his mito disease manifested because his mitochondria are dysfunctional to a point where with everyday living without stressors, he has this disease.  When you add to that [with illness or stress], you increase his mito dysfunction and over a period of time it takes it’s toll.  So the longer you’re dysfunctional, the worse your dysfunction becomes, the more likely your mitochondria are to fail and that cycle continues until you run out of mitochondria and the end point of mitochondrial disease is literally, death.  Most kids die of a respiratory tract infection." 

Yikes - that puts things into perspective.  Sobering and not fun to hear.  It just makes us so grateful for every second we do have with him, and also hypervigilant about keeping him safe and as illness-free as possible.

There was some good news at our appt.  He had some fascinating things to say about what's in the future for mito patients.  There's a new drug on the horizon for mito disease.  Children's has an 'IRB' (whatever that is) in place to be a study site.  He said, and I quote, "There’s hope in the pipeline….there is a little light at the end of the tunnel."   He said the hitch is that you have to be deemed '90 days from death' to be a candidate because the FDA really wants to see it work.  He said there have been 2 kids with Leigh's Syndrome (a form of mito disease) and their response to the drug has been remarkable.  They were both on ventilators and now...they are NOT!   He said that when the study is approved, probably 10 kids will get into the study and 7 will respond.  Then the FDA will say we can do a phase 1 trial.  But in order to get into the trial you have to have a genetic diagnosis.  And Children's just developed a chip that does gene sequencing!  Crazy progress in a short amount of time.  Of course there's many hoops before you can use the chip offically and I can't remember what he said about that. 

Of course Don asked the obvious question...'so does this mean that someday we may not just be looking a palliative care for Cody?'  'Palliative' basically means 'no cure' and just focusing on quality of life.  Could this new drug turn anything around for our little hero?  Could he maybe talk one day?  Maybe be free from seizures?

Our neuro simply said, 'I don't know.  I do know that we have 4 patients who have responded beyond our wildest dreams.'

We'll take any ounce of hope we can hang on to.  Oh to hear Cody say 1 word.  I think I'd die on the spot.  To have 1 blessed day with no seizures.  To stop wondering with every fever....'is this just A fever....or is it THE fever that will end his life?"  I can only imagine.  And imagine is what I will do until it happens.

That's it for today.  Please keep Cody in prayer.

with our love, Shawna