Rituximab #1

Finally, after weeks of phone calls and paperwork to get insurance approval...

 

Audra was scheduled to get her first infusion of Rituximab today !!

 

Praying that there is a miracle for sweet Audra in that bag.

 

Rituximab will deplete all the B-cells in Audra's immune system.  The B-cells are where the bad antibodies are.  Those nasty antibodies have mistakenly identified her brain as "foreign cells" that need to be attacked and destroyed.  Antibodies are a good and necessary thing when they do the right thing, like attack and destroy bacteria and virus cells.  But sometimes they get confused and attack healthy, normal cells.  Audra's brain is being attacked by those nasty antibodies, and that's where all her awful symptoms come from:

Agitation, aggression, developmental regression, incontinence, hypertension, facial twitching

 

Rituximab is not a quick fix. 

After her infusion today, she will get another one in 2 weeks.  Then we wait.  She can't get anymore Rituximab for 6 months.  So, if it works we pray that it keeps her symptoms controlled until January.  And if it doesn't work, then we pray for another treatment option.  Because living like she has for the past 5 months should not be her only option. 

 

The first step was to get an IV. 

Here she is all hot packed up hoping those little veins of hers will cooperate.

 

A fabulous nurse got the IV in on the first try with just a bit of ginger coaxing.

Then she got pre-medicated with Tylenol and Benedryl to prevent a reaction to the Rituximab.

The Benedryl on top of her morning seizure and blood pressure meds knocked her right out!

 

She slept and slept...

 



 

From about 8am to noon.

 



Then she woke up to snacks.  Girl loves her doritos!

 

The infusion took about 8 hours to complete.  It was a long day.  But one that we had been looking forward to for many weeks. 
She did really well until the last 2 hours.  Which is about when all of her morning meds usually wear off and the behaviors start showing up again.
We are praying so hard that this is the answer to getting our Audra back.
I am anxious to see if we notice a difference any time soon. 
And we'll go back in 2 weeks for infusion #2.



The second half of July

The Tooth Fairy had special plans for Audra again...

 

Last November Audra had 6 teeth removed due to terrible overcrowding in her mouth.  At that point none of her baby teeth were even loose yet.  So the dentist helped her out and the tooth fairy rewarded her.

 

Now she has more teeth coming in behind some of her other baby teeth that also are not loose yet.

So it meant a trip to the OR for sweet Audra to get her pearly whites all tuned up.

She was not a happy camper,

once I get all dressed up she knows what the next step is,

she's done this way too many times.

So many that I have lost count. 

At least they were able to get all her dental work for the year done at one time:

xrays, cleaning, fillings, crowns, extractions, you name it.

 

Sweet girl slept very soundly in the recovery room.  She didn't swell up as much this time.

But her heart rate stayed very low at 42 for about 45 minutes.  It was a bit scary to watch that number on the monitor but her blood pressure was fine so I knew she would be ok.  It was just a side effect of all the anesthesia on top of the blood pressure medicine she takes daily.



Maura went to the Vacation Bible School our church hosts.

This one is much smaller and more traditional than the huge KidFest she went to earlier this month.

 

Singing, dancing, games, snacks and Bible verses...

The last weekend in July is the annual 24 hour long fundraiser soccer tournament at the kids soccer club.  Thankfully this year was not as HOT as last year.  But there was rain in the forecast.

 

Their #1 Fan, happy with her bag of chips!

Audra and I headed home just in time to miss the rain.

The games were delayed for a little bit because of the rain but then it was back to playing...

all night long!

Maura had about a 7 hour break overnight so she actually went to sleep at one of her teammates house who lives right near the fields.

Sean slept in the car with the air conditioning running.

And Aidan was supposed to sleep between games in the tent he set up.

But I'm thinking there wasn't a lot of sleep happening...

because he couldn't stay awake before his last game the next morning.

My brother and his family came up to visit from NC at the end of July.

 Aunt Brownie always has a craft idea for the kids.

This time it was "painting rocks at the park".

 

Then we went to the mall where we were met by Uncle Tony {the birthday boy} in the parking lot,

which let to an impromptu singing of Happy Birthday right there in the parking lot!

Maura loves to shop with Aunt Brownie,

those two could wander around the mall for hours,

long after the rest of us have had our fill.

 

Another summer family get together meant...

More water balloons !!

 

Big girl cousin and little girl cousin

 

Me and my niece Brianna,

we look nothing alike {wink}

Mid-July Fun

 

It was a busy, crazy, fun, exhausting week.  We sure packed a lot into just seven days!!

Aidan was off to hockey camp each day this week.  And Maura attended KidFest on Tues, Wed and Thurs mornings.  She has gone the past few years and absolutely loves it.  She starts looking forward to it each year as soon as the end of the school year is getting close.  It's basically a Vacation Bible School...but much more extreme.  Lots of kids, lots of volunteers, lots of inflatable water slides, lots of Bible lessons and lot and lots of FUN !! 

Tuesday after KidFest...Aunt Kristy and cousin Tatum came with me to pick Maura up so we could go on a little adventure.  Our first stop was Chick-fil-A for Cow Appreciation Day which meant free meals for everyone dressed in cow print.

With bellies full of Chick-fil-A we headed over the 7-11 because it was also 7-11 Day (July 11th)...which meant free Slurpees for everyone !!

Later that night we all went to the local Carnival

Why is it so hard to get two kids to look in the same direction at the same time for a picture??!!

 Maura took her little cousins on the kiddie rides,

then she and Aidan would run off to ride the big kid rides.

Until Aidan ran into his friends,

then he ditched us...

only to come back asking for money.

 

We spent plenty of money on the carnival games but all we won to take home with us was a little goldfish.

Thursday after KidFest I took Maura to the pool with Aunt Kristy, Tatum and Teagan. 

Thursday evening was the KidFest Family Finale.

Unfortunately it was raining, we still made the best of it, but it would have been so much more fun without the rain.

The girls enjoyed the water slides...

when you're yet why not get more wet.

Maura with cousins Charlotte and Whitney

There was a traveling petting zoo under tents so we spent some time there trying to keep dry.

 

 Cousins Tatum and Teagan came along too

 

There were food trucks and vendors everywhere and the main event, a bike stunt show, was supposed to take place in the parking lot.  But, because of the rain they moved it inside to the auditorium ??!!
Yes, they rode motorcycles up and down the aisles doing all kinds of fancy starts, stops and wheelies.

While we waiting for the show to start there was a slideshow with pictures from the whole week.

Maura and Charlotte made it to the big screen !!



 

On Friday my sister and I took the girls bowling

 Boy was that a lot of work to keep 4 little ones happy while having to wait their turn,

and try to make sure they didn't pinch their fingers or drop a ball on their foot.

Phew!!

 

Friday night was a sleepover,

with plenty of popcorn and ice cream cones.

 

Saturday we had cousin Daniel's Graduation Party at our house

 

All the decorations Aunt Dawn set up were so cute.

Already has me thinking for Riley's graduation in just two short years.

 

 

 

A diagnosis...and a plan ?!

Yesterday was a turning point in this whole mess of a journey we have been taking with Audra for the past 6 months. 

It has been a long, busy, exhausting 6 months filled with many meetings, appointments, new doctors, hospitalizations, new medications, new therapists, etc....not to mention way too many sleepless nights for sweet little Audra and ourselves.  A few times we did actually think we were making progress towards helping her...but never more so than yesterday.

First, she had an appointment with her Immunologist for her regular 6 month check up related to her immunodeficiency.  The last time we saw him was in January...right as this nightmare was beginning.  And I remember mentioning at that visit the new, concerning symptoms and behaviors that Audra was just starting to have.  At the time neither of us thought too much about it and agreed we'd just keep an eye on her for symptoms of a new infection, etc. that could be the cause.  After all, she checked out just fine and didn't have any physical symptoms of an illness. 

Shortly after that visit in January things really started spiraling out of control.  Many of Audra's doctors started weighing in, tests were run, but no answers were found.  In late March the revolving door of admissions and discharges from the hospital started. 

At that point, after all the routine tests were unrevealing in figuring Audra out, most of the doctors chalked it up to behavioral issues...or Autism (which she didn't have the day before!) or progression of her newly identified {and very rare} genetic syndrome (something they had probably never even heard of before reading it in Audra's chart).  Some even questioned if she was being mistreated or abused.  Others suggested we find alternative living arrangements for her.  Yes....it was that bad!

But my mama heart remained steadfast in my certainty that SOMETHING was WRONG with my sweet girl.  Something was causing all of this, and that something was medical...infectious, inflammatory, autoimmune...I didn't know exactly what but I knew there had to be a trigger. 

Sure, Audra had significant developmental issues before all this happened.  But why would it all change so suddenly...

developmental regression
agitation/aggression
self-injurious behavior
loss of potty training
loss of verbal skills
facial twitching
periods of nearly no sleep in 24 hours
then
sleeping for nearly 48 hours straight

If any other typical 9 year old began behaving like this nearly overnight there would be major concern.  It was so unfair to Audra to just blame it all on one of her other medical/developmental issues on her laundry list of diagnoses.  And I refused to stop until she received a complete medical work up. 

Then yesterday, 6 months into this journey, her immunologist said...

"Yes, it actually makes complete sense that Audra could have Autoimmune Encephalitis.  AE is an auto-antibody mediated disease and 1 in 4 kids with CVID {the immunodeficiency Audra has} develops auto-antibody mediated disease." 

My response:  "Well, I don't think her Neurologist realizes that"

I told him about the quick but short lived improvement we saw with Audra after the 3 days of high dose steroids she received last month.  He is concerned that giving additional steroids could be dangerous for Audra because of her CVID but then he said...

"Rituximab is one of the safest drugs to give kids with CVID"

My response:  "Her neurologist definitely doesn't know that.  She is worried that it's not safe for Audra because of her immunodeficiency."

He explained that Rituximab targets only the B-cells, where the auto-antibodies are, and destroys them....but leaves the remaining cells of the immune system untouched.  And because Audra already gets weekly immunoglobulin replacement infusions for her CVID, she will be protected while her body produces new B-cells. 

WHAT ?!?!  How had we not figured this out before now.

Her Immunologist assured me that he would email her neurologist immediately with his thoughts.
And off we went to Audra's next appointment...with her Neurologist !!

I love Audra's neurologist.  She is the only doctor so far that has really listened to me and entertained my thoughts/mother's gut feeling that there is a medical issue causing all of this.  She admitted Audra to the hospital last month for the spinal tap and began treatment for Autoimmune Encephalitis.  As soon as she came into the exam room yesterday for Audra's appointment she said...

"What did her immunologist tell you?"

I said..."everything he told you in the email he sent you"

And with that she said...

"Audra needs Rituximab asap"
"Maybe we will do a bridge of steroids until the Rituximab starts to work"
"There are other treatment options to consider as well"

A diagnosis??
A treatment plan??
A cure??

Oh sweet music to my ears...and heart!!

On the 4th of July we met up with my parents and my sister's family to watch fireworks.

 

What looks like a hint of a smile on Audra's face is actually just a split second of the right side of her mouth twitching.  And what limited time she spends outside of the house these days is usually spent in our arms or in the wheelchair/medical stroller.

 

But now we know...

and now we have a reason to keep fighting.

 

I promise I won't stop until you are all better baby girl !!