Beach Blast

We had a weekend full of sun, sand, and SOCCER! 

Both Aidan and Maura participated in the Beach Blast soccer tournament in Wildwood, NJ this weekend.  We stayed at a hotel with all the other families from Maura's team.  The hotel was like all the other hotels in Wildwood {which isn't spectacular...you could call it "vintage Jersey Shore living"}...but...it was just a few steps from the Boardwalk which is as good as it gets!  And it was great for the kids to be able to hang out with each other all day long...jumping around from the pool to each other's rooms. 

They have nearly 150 soccer fields set up on the beach and the games go on all day.  It's a really huge event.  You can't really tell from the picture below because the beach is basically empty, but during game time it was a sea of people and soccer.

 

 The tournament package included 3 days wristbands for the kids to do the amusement rides on the Piers.  After checking in Friday afternoon and taking quick walk on the beach we spent the rest of the evening on the Boardwalk.

Saturday morning....

the games began!



 

Friday evening we increased one of Audra's seizure meds per the doctor's schedule.

It resulted in Audra spening nearly all of Saturday like this...



 I would wheel her all around and prop her up with pillows,

and she would stay asleep!

 

A huge ice cream cone with sprinkles after your first morning game of the day is perfectly acceptable at Beach Blast

 

A few games in and still sleeping...

 

Luckily Maura's games were always on the fields closest to the boardwalk so I could find a shady spot to park Audra and still be able to watch Maura play.

 



Maura's team made it to the Championship,

so I managed to get Audra's chair down on the beach so I could watch from the sidelines.

Maura's sweet team won 1st place !!

Aidan's team also made it to the Championship but they lost after a tough game that was close.

 

You can never have enough ice cream when it's a hot day at the beach :)

 

The kids with their medals on the balcony of our hotel room.



March/April/May

With Audra's three hospital admissions in the past 3 months plus countless outpatient visits and getting situated with home nursing care this poor little blog has really been neglected.  I was finally able to sit down long enough to finish up some old posts from March, April and May.  So scroll back to read all about our spring.  Well it may be mostly pictures with few words, but forgive me...my memory isn't holding up like it used to.  Fingers crossed that I don't get this far behind again !!

March stuff:  swimming, snow and the Art show
Riley and Aidan's Confirmation on March 23rd
Easter on April 16th
April stuff:  our 4th Familyversary and Aidan's 13th birthday
Maura's First Communion on May 7th

Home...take three

This update is coming from our favorite place...

HOME.

 

After 11 days in the hospital,

Audra came home.

Again.

 

For the third time in just 3 months,

we packed up our things

hoping not to have to return too soon.

 

This admission seemed like a turning point though.

We made more progress in figuring our sweet girl out this admission

than we did in the previous two admissions.

 

She completed 5 days of IV immunoglobulin infusions

followed by 3 days of high dose steroids,

in hopes that it will calm any inflammation in her brain

that might be triggering all of these changes in her.

 

The single antibody test that was sent to the local university hospital came back negative.

 

The full antibody panel that was sent to the Mayo Clinic won't return until next week. 

That panel includes the antibody that was elevated in her blood sample.

 

So for now, we have done all that we can do

until we know for sure if Audra has autoimmune encephalitis.

 

Thursday Audra was full of smiles,

a hint of our sweet, sassy girl...

 

Thursday I had to go home for the night because Sean had plans for a little weekend road trip and he needed the minivan...which I had.
Thankfully Audra's godmother was working at the hospital that evening so she checked in on her for me and eased my heavy heart when she reported that she was sleeping soundly by 8:30pm.
 

On Friday Maura went back down to the hospital with me.

Audra sure was happy to see her :)

{I bet she is a bit sick of seeing only my face for so many days in a row!}

 

There were plenty of hugs and kisses...

 



 

And a few games of hospital bingo...

 

Quiet time with iPad videos and games...

 

And then I convinced them to let me take Audra home Friday night instead of Saturday.

They were concerned that I wouldn't have enough help without nursing for the weekend.

But I said it didn't make any sense for me to drive home Friday evening just to have to drive back the next day to pick her up.

 

So home we all went!

 

Saturday morning we jumped right back into the routine...

Maura had a soccer tournament.

I gave Audra all her morning meds right before we left

and by the time we got to the fields she was sound asleep!

It was a pretty rainy day but I was prepared with ponchos and umbrellas.

Audra slept like this in her stroller for over two hours!

 

And then she slept some more on my lap.

 

We had cousins Charlotte and Whitney over Saturday night for a slumber party.

All the sweet girls together again!





 

Audra had a really good day Saturday.  She was calm and happy, didn't have any accidents in her pull up and even managed to sit with her sister and cousins and watch them play.  That is something I haven't seen in a long, long time.  These are all little glimmers of the "old Audra".  We are praying so hard that the treatments she received while in the hospital were the start of helping Audra.  I don't want to get my hopes up yet.  I know there is no quick fix for this.

 

Maura had her final soccer game of the season today.

And she scored her first goal !!!

 

Aidan has been in Canada with his soccer team this weekend.

They are training and competing with a local youth soccer team in Canada from Friday-Monday.

Aidan is in the front row, 4th white shirt from the right.

#6 if you can read the numbers on the blue shorts

 

Sean wasn't a chaperone for the trip but he and a few other dads decided to make a weekend trip out of it.  They left Friday afternoon and will get back late tonight.



 

The next step

Audra finished her 5 day course of IV immunoglobulin treatment last night.  She tolerated all five infusions very well. 

She has had some good days and some bad days, but most days are on the line of manageable.  Even if we are on track and treating the underlying cause of all this troubling behavioral/neurological stuff she still has a long road ahead.  These current treatments aren't curative immediately, but rather over time.  That is why it is so important to have a good medication plan to manage the symptoms.  With that in mind her neurologist has made a few medication changes:

Her Clonidine patch was removed and she was started on oral clonidine twice a day instead.  This med is used not only to help her behavior but also to improve her sleep and manage her high blood pressure.

Because the Depakote has been so effective in improving her abnormal EEG her neurologist doesn't want to make any changes to it.  But she did put her on another seizure medication, Lamictal, which is known to potentiate the effect of the Depakote in helping to manage her behavior.  But Lamictal takes months to get a therapeutic (effective) level, so we are starting low and can make changes over time.

Zyprexa is a medication that is specifically helpful in managing the behaviors associated with autoimmune encephalitis.  After giving her a trial dose last week the decided to add it to her daily medication routine. 

After finally getting the approval of Audra's immunologist she was started on a 3 day course of high dose IV steroids today.  It's a super high dose!  Audra was already seeing the effects of it tonight- she was a happy, hyper, hungry girl....and it was actually kind of hysterical !! She really hasn't eaten much of anything by mouth since being admitted, {and in fact she won't even swallow all the pills she used to so we have had to start giving her meds through her G-tube} so her big appetite tonight was a welcome change. 

For now we are holding steady, waiting for the antibody testing results from her CSF to return.  We are hoping the partial panel that was sent to a local university hospital will be back tomorrow.  But the full panel that was sent to the Mayo Clinic probably won't be back for another week.  There are 1-2 other treatment options for autoimmune encephalitis but for now her neurologist wants to wait it out until the results come in. 

This past weekend I went home for a night.  I needed to be home Saturday because Maura had THREE dance recitals.  It was an all day affair.  Each year she makes so much progress and has become such a beautiful, graceful dancer.  Makes my momma-dancer heart so proud!

And it was cousin Tatum's first dance recital.

She was so adorably cute up on that big stage dancing her little heart out!

Just love that my girl is learning to dance with the world's best dance teacher...

my very dear friend who I danced with all through high school.

There was a twinge of bittersweetness to the day...

 It brought back all the memories of my other sweet girl "dancing"

up on that very same stage last year. 

Sure my teeny tiny 8 year old was up there with the 3 year olds...

but she was UP there.

Last year she was ON the stage...

but this year we would not have even been able to bring her into the building to watch.

Things are that different.

 

I need to keep my focus on the goal...

getting Audra back to the sweet, loving, smiley girl that she used to be.

IVIG dose #1 done

After some minor IV drama last night, Audra got her 1st dose of intravenous immunoglobulin.  This is the same immunoglobulin she receives weekly for her immunodeficiency but in a much higher dose.  And it will be given once a day for 5 days.  She was pre-medicated with tylenol and benedryl prior to the infusion to prevent any reaction, and they started it at 11pm so she slept soundly through the whole thing!

Understandably, she was agitated late last night during the whole IV checking, re-taping, and eventual re-poking.  But she woke up this morning pretty ornery too.  Sweet girl...she is just so out of sorts and not herself.  We are continuing to pray everyday that this is the solution to helping Audra get back to her usual self. 

Her neurologist is working closely with many of Audra's other specialists to be certain we are covering all the bases in regards to her medications and treatment plan.

ENT came by to check out her left ear.  The first week of May she developed a bad ear infection with a ruptured ear drum and started her first round of antibiotics.  Once she completed that course the ear drainage and discomfort returned so she started a second round of stronger antibiotics and ear drops.  Last Friday I had her to the pediatrician for a follow up and her ear was still infected.  So she started a third round of antibiotics.  What's troubling is that it's the same ear that she had a surgical tympanoplasty done on a couple years ago.  So she blew through that repair with this infection.  Her ENT doctor gave her an urgent appointment to be evaluated this past week, but she missed that because of this admission.  So ENT saw her here and finally {thank goodness} that stubborn ear is heading in the right direction!

The psychiatrist from the medical/behavioral unit came by to talk about different behavior meds that are particularly helpful with autoimmune encephalitis.  Even though she is receiving the treatment for it, the behaviors associated with encephalitis can linger for a while so we need a good medication plan.  And even though our previous attempts with a few different meds haven't been helpful there is hope that one of these specific meds might make a difference.

We are hoping to get Audra transitioned off the clonidine patch and back on clonidine pills.  Prior to all these hospitalizations she only took clonidine at night to help her sleep through the night.  During her first hospital stay they started adding daytime doses due to her behaviors and then she developed hypertension so they changed her to a patch for more consistent/steady dosing.  But the patch makes me so nervous...I'm constantly watching it like a hawk to make sure it doesn't get loose and fall off {which it does...every week!}, and then make sure she doesn't chew on it or eat it, or give it to the puppy, because that would be very harmful to either of them!  So we have a plan with her nephrologist to take the patch off and put her back on pills then adjust the dose to make sure she isn't too sleepy or too hypertensive.  Quite the balancing act!

Her neurologist has been talking with the immunology team to try to figure out the best treatment plan for Audra.  The issue is that treatment for autoimmune encephalitis includes some drugs that suppress the immune system.  And since Audra has an immunodeficiency they want to make sure anything they use to treat the encephalitis is safe for her and her already compromised immune system.  Another unique balancing act.  So the IVIG will be given daily until Monday and by then they will hopefully have a plan regarding high dose steroids and Rituximab.

Otherwise we are just hanging out, watching TV, taking walks and...

hoping and praying that this IVIG kicks whatever is causing all of this to the curb

and we get to see a glimmer of the old Audra soon!

Results and a Plan

Monday night's video EEG went well, and luckily Audra slept all night long so they were able to capture plenty of data.

Tuesday morning started early when they came in at 6am to stop the pedialyte that was running through her G-tube and place an IV in preparation for her spinal tap.  Thankfully they were able to get her bloodwork drawn with the same poke as her IV so that was like a 2 for 1 deal.

I am so thankful to be here this week when Audra's primary neurologist is on service.  She knows Audra well, understands the whole back story and really empathizes with the struggle that the past few months have been.  She is determined to help Audra. 

During rounds yesterday morning she informed the whole medical team that this would not be a quick "in and out" admission.  And that no stone should be left unturned during this admission.  Audra is complex and unique and she cautioned them not to get lost in her long list of diagnoses because it's easy to blame things on any one of her many diagnoses.  It's been a long road with Audra but she deserves a complete, thorough, extensive work up.  What a relief it was to hear those words. 

The final read on Audra's video EEG came back and the verdict was that the Depakote did in fact "quiet down" her abnormal EEG.  So they probably won't make any changes in her Depakote dosing any time soon. 

Most of the day was spent waiting for the main event...her spinal tap. 

And while I was both anxious and eager at the same time...
Audra was just....
Sleepy !

About mid-afternoon we finally headed down to the pre-op holding area,

and waited some more!

So we passed the time taking selfies:

 

While Audra was in the OR for her quick procedure I grabbed my first meal of the day from the waiting room vending machines.  

I was so hungry I would have thought just about anything was a gourmet meal :)

 

It is always nearly impossible to sneak away to grab a bite to eat when you have a little one who is NPO for anesthesia.  Not to mention the guilt I would feel with a full belly while hers was aching for food.

 

Everything went well with the spinal tap and she was back in her room quickly.  She slept for a bit then woke up cranky and irritable.  We gave her some Tylenol in case she had a headache and after a little more sleep she woke up in a better mood.  The techs came in to remove her head wrap and EEG leads, then she feasted on snacks from the unit's pantry.

It had been a long day so we both fell asleep easily much earlier than usual.  Then at 10pm the hospital phone in our room rang.  In a bit of a daze I answered it and it was Audra's neurologist.  She was calling to let me know that the preliminary results from Audra's spinal tap showed an increased number of white blood cells, which indicates inflammation.  And as crazy as it sounds...we both were relieved.  This is the first tangible piece of information we have had in the past 6 months to say that YES something is wrong with Audra...something medical is triggering these terrible behavior changes and her developmental regression.  So with that new information the plan was to start treating Audra for autoimmune encephalitis now.  The antibody testing of her spinal fluid that was sent to the Mayo Clinic will take 2 weeks for results. 

With all that new information swirling in my head it took a while for me to fall back to sleep.  Then Audra woke up from 2-5am.  She was happy and content just watching her iPad.  She managed to get a little more sleep between 5 and 7am, but then woke up vomiting.  So into the bathtub she went which meant I could scrub all the yucky EEG glue and junk out of her hair. 

Yesterday she was too sleepy to notice the special delivery she got, but today she caught a glimpse of him right away...which isn't hard because he is soooo big...

Some bunny really does love Audra!

{Thanks Aunt Kristy}

 



This morning was a long and anxious wait for the doctors to round.  The plan is to start intravenous immunoglobulin tonight for a total of 5 days.  They will wait to see her response to that before starting high dose steroids.  The other medication that is typically used causes a significant immunosuppression particularly of the B-cells so the team would like to consult with the immunologists first to determine what this might mean for Audra with her underlying immunodeficiency. 

Audra had more bloodwork drawn this morning to get a baseline of some things before the treatment begins.  With a solid plan in place that wouldn't start until later tonight I was able to go 2 flights downstairs and put in about 6 hours of work while some of the same 1:1 observers who sat with Audra during her past two admissions came to sit with her today. 

Updated blog...just in time for more Audra updates

I have finally started finishing up a bunch of old posts from January and February that I had started but not finished before all of this medical/behavioral stuff with Audra started consuming our lives.  So, if your are interested scroll back to read all about:

Maura's 9th birthday,
Chinese New Year,
Aidan's arm,
snow, the circus and more !

Or if you are just here to check in on the Princess then read on...

Unfortunately the new med they started and increased, Depakote, has not helped Audra.  We have seen no improvement with any of the meds they have tried...Risperdal, Ativan or Depakote.  I have spoken with many of her doctors by phone over the past week or two and we have come to the decision to move forward with the interventions we discussed at her last neurology appointment. 

So, today we saw her neurologist in clinic then she was directly admitted to the neurology floor at CHOP.  The plan is to do a video EEG to see if the Depakote has helped "calm down" her abnormal EEG at all.  If not, they may start her on Valium to see if that helps.  She will also have a spinal tap done to send a CSF sample to Mayo Clinic to look for antibodies that indicate if she has an autoimmune encephalitis.  Her bloodwork showed a low level titer to one particular antibody but CSF is much more sensitive.  Her neurologist is considering beginning treatment for autoimmune encephalitis before the results come back, since it takes 2 weeks for results. 

Once we got to Audra's room she got all hooked up and tucked in for what is hopefully a good nights sleep of EEG monitoring.  Praying that with all this they actually find something they can actually do something about to help our sweet girl!