Setbacks

The number one thing I am learning about this special needs journey is that it's completely UNPREDICTABLE.

There are so many ups and downs, and emotionally that is exhausting.  I allowed myself to get too excited about how well Jayson was doing in February through April.  I got so caught up in the beauty of it that I allowed myself to think that might be our new norm!!!  Seizures were controlled, illness was minimal, neurologically he was stable, and there were no surgeries planned.  Life was uniquely perfect!  For the first time in over FOUR YEARS my husband and I began talking about the future!  Things we might do, where we might travel, how we might grow our family...  But then we stumbled.  Big time.  It shook us pretty badly.  We thought Jayson was stable for the long haul.  We thought he had gone through enough and that maybe he would be able to move forward for a while without many setbacks.  We were wrong.

Through March and April, Jayson seizures started escalating again.  We just noticed them making a comeback, but weren't super worried.  Could be an illness brewing.  Could be seasonal allergies.  Could be a new food we introduced.  Could be a neuro flare up.  Could be all of the spring thunderstorms.  It was hard to know.  But by the end of April, they had reached a level that we couldn't tolerate.  They interfered with Jayson's quality of life, upset him, kept him from sleeping, and clustered all day and night long.  I sort of panicked.  The keto diet had been working!!  What was happening?  We made a lot of calls to doctors.  We took videos of seizure clusters and behaviors before and after seizures.  I edited the videos to make short clips to send to doctors.  We emailed our key players.  We saw the pediatrician to rule out any infections or illnesses.  Nothing.  No explanations.  No response from our team members.  Crickets.  I was getting frustrated.

Just then, the vomiting started.

Either this could be an illness explaining the neuro flare up
Or
This was a concerning sign of intracranial pressure

We tried to first treat this like an illness.  We endured over 24 hours of heavy vomiting.  We endured our child seizing uncontrollably.  We endured him losing consciousness after violently vomiting a couple of times.  We endured the endless diarrhea.

But after 24 hours of all of these things, we knew we had reached our limit of what we could endure at home.  We wondered if it was time to get Jayson checked.  He was completely out of it, lethargic and listless at home.  After a particularly concerning period of non-responsiveness, I decided to check his blood sugars.  48.  They were 48.  It was time to go.

We packed him up and I packed a hospital bag and we headed to Primary's.  The doctor exam showed only mild dehydration and he had stopped vomiting.  They considered giving him some fluids and sending him home, until they got the lab work back.  Labs were clear.  He was in crisis.  He was much more dehydrated than he appeared.  He was in severe ketoacidosis.  It was an admit for sure.

I had two goals of this admit-- get Jayson stable and well, and figure out how to handle treatment of stomach viruses at home on the ketogenic diet.  Here's the problem.  When typical or other special kids get a stomach virus, the suggested treatment is clear fluids such as pedialyte, Powerade or apple juice.  Well all of those things have sugar.  They are not an appropriate treatment for kids on the ketogenic diet.  When a child with epilepsy gets sick, seizures already increase.  We don't want to bring on more by bringing them out of ketosis with a sugar drink.  So we give Powerade Zero, which is sugar free and carb free.  Again, this presents a problem for children with prolonged illness.  NO ONE can survive without any sugars or carbs for very long.  After 24-48 hours of only Powerade Zero, Jayson gets too acidotic which propagates more seizures and vomiting.  Ketoacidosis is a life threatening crisis.  So what we've done in the past is after a couple of days of only tolerating clear liquids, we are forced to switch from Powerade Zero to Pedialyte, even though it has sugars.  Jayson's body goes into acidosis, so we have to bring it out with some sugars and carbs.  After another couple of days of only Pedialyte, Jayson's body starts to get out of ketosis.  This triggers more seizures and sometimes vomiting.  When Jayson catches a stomach virus, his GI system shuts down.  It stops digesting and moving things through, even clear liquids.  It refuses anything but clear liquids for about a week.  Then it takes another 2-3 weeks to tolerate his typical feeding regimen.  Going in and out of ketosis can also be life threatening.  He isn't supposed to do that out of the hospital, yet every time he gets a stomach virus he experiences ketoacidosis, then gets out of ketosis, then we have to bring him back into ketosis... all at home.  It's dangerous.  It's uncomfortable.  It's scary.  It's something I don't feel comfortable with.  So we hoped this hospital stay would help us figure out a way of treating Jayson when he catches a stomach virus so we would know how to safely manage it at home.

Well, we struggled to meet my two goals.  Jayson's illness persisted.  Although he didn't vomit, his diarrhea was impressive, his stomach cramping painful, and his labs and symptomology concerning.  His neuro system struggled.  It triggered a pretty bad dysautonomia flare up.  His hands and feet swelled, turned purple, were ice cold and impossible to warm.  His heart rate was all over the place and erratic.  His blood pressures were very high one minute and very low the next.  His body temperatures were impressively low.  I had to reassure our nurses that this is all within Jayson's "normal" limits during a dysautonomia flare up.  One night his heart rate was getting pretty low and sounded the alarms.  I reassured the nurse that this happens at home, and just asked her to make note of it in Jayson's chart.  What I didn't expect, however, was for it to keep going down... down... down... 30's... 20's... teens... 5... ZERO.

Yes, my son's heart rate hit zero.

At this point, the crazy alarms started sounding.  I checked his placement of his sensors assuming one of them had come off or was not reading.  No... they were all reading.  The nurse rushed back in and we watched Jayson as he continued to breathe in and out, likely thanks to his bipap breathing for him, yet his heart rate would go back up to the 30's, then 60's, then drop down again... to zero...

I was scared, but tried to stay calm.  The nurse mentioned it's a good sign that he's still breathing.  She checked the sensor placement as well, then grabbed a stethescope to listen to his heart.  She looked confused and I asked her, "Is it jumping all over and having long pauses between beats?"  She nodded.  "Yes, it will have very long pauses, then beat a couple of times, then another long pause."  I breathed a sigh of relief knowing this is what I had witnessed at home as I have listened to his heart during these bradycardic events.  I assumed this was just another event caused by his dysautonomia.  I explained to the nurse that he does this sometimes at home.  She asked what I usually do, and I said we usually stir him a little and cause him to wake.  That's what she and I did, and his heart returned to a normal pattern and beats per minute.  My anxiety and blood pressure had to have been through the roof by this point  and it took me hours to calm down enough to sleep, but everything was fine.  Jayson was fine.  But what a scare.

I requested a consult with our keto specialist.  I had low expectations, but boy, she still blew me out of the water.  Our conversation went something like this:

Keto Specialist/Neurologist:  "Hi, I heard you had some questions about the keto diet?"

Me: "Yes, I'm wondering if we can come up with a treatment plan for Jayson since he keeps getting stomach viruses and he has GI and motility issues which make it so he's on clear liquids for a week after vomiting.  Being on Powerade Zero that long makes him acidotic and giving him Pedialyte brings him out of ketosis.  I'm wondering what we can do to balance this?"

Keto: "Yeah, Pedialyte will do that.  Don't give him Pedialyte."

Me:  "But Powerade Zero after 24-48 hours makes him dangerously acidotic."

Keto:  "Um, what is that drink you mentioned?"

Me:  "Powerade Zero.  Grape flavor."

Keto:  "Why in the world would you give him that?  What is that?"

Me:  "Um... you told us in our training that is what we should give him in place of Pedialyte.  And that's what it says in our books and manuals you gave us."

Keto:  "Oh, that's what it says huh?  Okay then.  Well I think it might be best if you try to not let him get sick."

Me:  "Um... we take every precaution, I assure you."

Keto:  "Yeah, getting a stomach virus should be rare.  So it might be best if you could just keep him from getting sick."

Me:  "He goes to school at a special needs school in the most severe classroom.  It's as safe as possible, but he puts everything in his mouth so he's going to get sick."

Keto:  "Maybe you could keep things out of his mouth."

Me:  "Really?  We certainly try!  Why do you think my 4 year old son still takes a pacifier?  To keep his hands and things out of his mouth.  He craves oral stim.  We need a plan."

Keto:  "What do you suggest?"

Me:  "Well another doctor wondered if it might work and be safe to mix half Pedialyte and half Powerade Zero so that he's getting some sugars and carbs, but not a lot.  What do you think?"

Keto:  "Well I think you will only know if you try it."

Me:  "Um... okay thanks."

And that was that.  Helpful, huh?

So clearly, she was no help... as usual.

After 4 days in patient we decided we could manage things at home and work towards Jayson's recovery on our own.  It gets sooooo hard in the hospital to make any changes in treatment.  You have to get orders for everything and get permission to do the smallest things.  We knew in order to get Jayson better we needed to be free from the hospital so we can make adjustments and any decisions regarding his feeds and bowel treatments necessary to help him.

Once we discharged, problems persisted.  His motility was still slow and it took much longer than usual for him to tolerate feeds.  His dysautonomia was still flaring badly.  Seizures were still escalating and we were having to use rescue meds a couple of times a week.  We treated his allergies with a big increase in Zyrtec yet the seizures persisted.  Neither Mike nor myself got sick, so we started to question whether or not this was really a stomach virus.  We tried again to contact our team and neurologist.  After days of no response, I started to get pesky.  I went to the office and insisted on getting Jayson in to be seen.  They made some calls, got us an appointment for a week and an half later, and I finally got a return phone call from a neurology nurse the following Monday.  She spoke with our doctor and called us back to let us know it was a good idea to see ophthalmology again to get the pressure behind Jayson's eyes checked and to get a repeat brain MRI.  We got both of those scheduled.  In the meantime, Jayson started to improve some.  He had a couple of days where his personality came back in between seizures and neuro flares.  We got his feeds nearly back to baseline.  His eye pressure looked great, we saw comprehensive care and our doctor thought he was improving, and then we saw neurology and did an increase in keppra for seizures.  Seizures basically disappeared...
Just as the vomiting started back up again.

I was a mess.  This was bad.  This reaffirmed in my mind that Jayson did not have a stomach virus before.  This was neurological.  He was having frequent headaches and migraines with sensitivity to light.  He was often wanting to lay down with a cold cloth over his head or behind his head.  I got a pit in my stomach and prepared for bad news to come from the MRI.

We got the MRI, and I was more than relieved to see that things looked the same.  Yes, still the same concerning things on the last MRI.  Jayson still has a pocket of cerebral spinal fluid in his brain that we have no idea how in the hell it got there, but no biggie.  It wasn't growing.  Other things were stable as well.  So there was no evidence of increased cranial pressure.  What now??

We were back to days of vomiting, endless diarrhea, seizures, holding urine for over 24 hours, intense stomach pain, motility issues, nearly TWO WEEKS of clear liquids, lethargy, no sleep and misery.  I mean MISERY.  We had Jayson in the ER on three different occasions for concerning looking stool that looked bloody, severe abdominal and rectal pain, concern for blockage and dehydration.  His labs looked worse during one of the ER visits than our last hospital admission, but we were at a different hospital so we got them to discharge us and we continued to watch him at home.

We took him to his pediatrician and he was stumped as to what was causing Jayson to scream in extreme pain unlike anything we had ever heard before.  Then we got some test results....

ANOTHER VIRUS.
A bad one.  Adenovirus 40/41

Our pediatrician told us he hardly ever sees this one.  It's extremely rare and really nasty.

So we had answers.  And considering all the possibilities, this was probably the best possible explanation.  It wasn't intracranial pressure.  It wasn't a progressive condition.  It wasn't a new diagnosis of Periodic Vomiting Syndrome or escalating dysautonomia.  Those are all chronic with very few treatment options.  This was just another stupid virus.  How???  Who the hell knows.  But it would eventually get better.  Eventually.  I hoped.

Well we are finally coming out the other side.  Jayson has basically been very sick and in crisis since mid-April until mid-June.  It has been exhausting and heartbreaking.  I've cried a river of tears and have spent so much times on my knees asking for Jayson to be rid of his pain and discomfort.  He is still not back to baseline with his feeds, but he is getting all formula feeds.  He is still having irregular bowel movements, but we have days of normal.  He is still having unexplained pain, but it's manageable.  But most importantly, my sweet boy is getting back to his sweet, silly, curious self.  That's all I need to comfort my worried Mommy heart.  He's going to be okay.

Little J never ceases to amaze me.  His body has been through quite a lot in the past two months.  His brain, senses, motility, stomach, digestive tract, muscle tone, energy levels, and sleep patterns have all been greatly affected the past few weeks.  Yet, he is bouncing back.  Yesterday he was tackling stairs like a boss.  Two days ago he self fed himself some Kix for the first time in 6 months.  In the last week he has started making new sounds we have never heard before.  Yesterday he went back to school for the first time in months.  He is amazing.  He is relentless.  He is unbreakable.

He may have setbacks, but he always makes a comeback.  Every time.

The past couple of months reminded me that we are never out of the woods.  We never will be.  This is our life, and it won't likely be any different.  It's safe to say that this is as good as it's going to get for us.  And you know what?  I'm okay with that.  We can do this.  We can have a few good months and another few in crisis, as long as I know we will come out the other side.  As long as I know Jayson will keep fighting and will keep conquering, we can live this crazy unpredictable life.  And we will try to live it with grace, with happiness in our hearts and with faith that everything will be okay.  We may live in an undiagnosed world, and we may not have a road map, but God is our tour guide and He is teaching me to be patient, to trust, and to have faith.  With God on our side and Jayson's determination to beat the odds, I think we're going to live a pretty beautiful life.  I'd have to say, it's Uniquely Perfect.