Home, again {for now}

Audra fighting the effects of doubling her Depakote dose on Tuesday

 

Where oh where is my sweet smiley girl?

Audra was discharged from the hospital on Thursday at 11:00 am and we headed right over to her 11:30 neurology appointment.  Her neurologist was surprised to see us in clinic...she said she was planning on coming to see Audra at the hospital that day.  And well...she was less than happy that Audra was discharged without a solid plan for medication adjustments and follow up.  Audra is on many new meds now and some of her old meds have had dosage changes by new doctors.  So it's beginning to get difficult to understand who's in charge of what and who to call for what.

Neurology appointment summary:

• The bloodwork that was sent to the Mayo Clinic to evaluate for autoimmune encephalitis came back with one elevated antibody titer.  It isn't super high, but still 7x the normal value.  Seems like they aren't sure what to make of this yet, but it probably requires some further investigation.  And the possibility of a spinal tap to test her CSF was mentioned again
• We will increase her evening dose of Depakote this coming Saturday.  So far the Depakote hasn't seemed to have an effect on her behavior, and she definitely seems to be having more trouble staying asleep overnight the past few days.  We continue to hope and pray that Depakote is the answer to helping Audra
• Audra will be re-admitted to CHOP in June (hopefully the week of the 5th when her neurologist is on service) to have another video EEG to see if the Depakote has "calmed down" her abnormal EEG.  Depending on the results they will consider adding Valium to her ever-growing list of medications

Once we finally got home Thursday afternoon a new home care nurse came over for us to meet/interview.  She ended up staying for the whole 8 hour shift (4pm-midnight) and she said she'd be back Monday.  God bless her for taking on Audra!  {I promise I'll post about the whole home care nursing process soon!}

Friday was Maura's Race for Education at school. 

 

Riley and Audra were off school because they had dentist appointments

so they went with me to cheer her on.

 

 

And Audra even walked a few laps with Maura

 



Our feisty little Audra has been quite the Houdini lately while in the car.  We can't get her to keep any kind of seatbelt on, no matter how much we extra fasten it and hide the release button.  She manages to wiggle her way out of the straps no matter what!  And with her no fear attitude recently she tries to open doors and windows or crawl in the front seat while we are driving.  Sooo...

I found a 5 point harness car seat that accommodates up to 50lbs so hopefully she can use it for a while.  Thankfully we had an uneventful ride into the city for their dentist appointments, now that Audra was securely strapped in :)

 

Riley is never happy about being at the dentist,

but at least now he tolerates it,

which says a lot about how much he has matured and learned to cope

since the early days of me holding him down for just a quick peek by the dentist.

 

Audra's dentist said it was time for another trip to Day Surgery to fix up her teeth.

So, within 24 hours of being discharged from CHOP she already has two invitations to return...

an admission for a video EEG and a trip to Day Surgery. 

 

And as if that wasn't enough medial excitement for Audra in just 24 hours, she ended up needing a trip to urgent care also...she still has drainage coming from her left ear.  She finished up her antibiotics earlier this week for the ear infection/ruptured ear drum we found 2 weeks ago.  One quick look and the doctor said her ear was still full of pus!  So time for a stronger antibiotic and ear drops. 

The rest of our weekend was packed with lots of activities and sports:

Maura's dance performance group participated in a competition

 

She was in two dances and only had about 20 minutes in between the two.

She had to change her costume quickly AND switch her hair into a French braid,

which I have no idea how to do!

 

Big dancers to the rescue...

They had some of the older girls lined up ready to do the little one's hair as soon as they had their costume changed.  Phew!

 

Before the awards ceremony started they had all the girls up on stage with huge glow sticks dancing to music with one of the judges

Riley's main event of the weekend was his last track meet of the season. 

He's not a big fan of running, but I think he's good at it, and it's good exercise for him.

He only exercises his fingers all week long playing video games so I make him exercise his body on weekends by participating in Special Olympics swimming, track, soccer, etc. 

Forget the gold medals...

hanging with these characters might have been the highlight of his day!

We also squeezed in a few soccer games, an ice hockey game and a quick rode trip to Long Island for a couple roller hockey games.  You would think we had 40 hours days this weekend with all that we managed to do!

Tomorrow is back to school again for Audra, hopefully she manages a bit better.  There are only 3 weeks until the end of the school year!

A new week

Monday morning at 1:00am sleeping beauty decided to awaken !  And 3 and 4 and 5am too.

Once we were up for the day I noticed that her clonidine patch was laying in her bed.  I have no idea how long it was off for and what that would mean for her behavior and blood pressure for the day, but we got a new one on as quickly as we could.

Turns out she awoke from her 3 day slumber in a very happy mood.  The behavior analyst attempted to do a Functional Analysis on her but of course sweet girl cooperated beautifully and didn't give in to their attempts to contrive situations that would bring out her behavioral issues.  She really is so stubborn!

She continued to have hardly any appetite on Monday.  I was hoping that she hadn't eating much since Thursday because she was so sleepy, but despite being wide awake Monday she still pretty much refused every bite I offered her.  And this girl loves her food, so it's not like her at all.  What's interesting is that her weight was up again on Monday to 18.3kg.  It's definitely not coming from nutrition, just not sure if it's from fluid retention or a med side effect. 

Monday night they started weaning her Risperdal dose.  She had started that med the night before her previous admission and we never really saw any effect from it.  Then about 2 weeks ago at a clinic visit they increased it but it clearly still didn't help because we ended up back here in the hospital anyway.  So it makes sense to wean her off it.  We continue to pray that the new med, Depakote, is the answer to solving Audra's troubles (abnormal EEG, sleep disturbance and behavior concerns).

There isn't much to report from Tuesday other than thankfully the blood work to check for a Pheochromocytoma (adrenal tumor) came back negative. 

After rounds with the doctor today the plan is:
1. increase her dose of Depakote today
2. finalize a plan for weaning off the Risperdal
3. discharge her tomorrow morning in time for her to make her scheduled neurology appointment--we are still waiting for the results of the blood work that was sent to Mayo Clinic when she saw neurology on May 4th

Now I need to get on the phone with her nursing agency to see if they can hopefully find her a new home care nurse soon.  The one she had quit the company the day after Audra was admitted.  I'm not sure that I ever posted about Audra getting home nursing care services, so I guess that's a post for another day. 

Next post from home hopefully {fingers crossed}

The ones who make me a Momma

Today I am celebrated as a mother because of 4 special ones that call me Mom !!

 

My quick trip home was refreshing and relaxing. 

But also included some necessary food shopping, laundry and bill paying.

I got to spend a little time with each of these 3 who I hadn't seen since Tuesday morning when I got them off to school. 

At the time I had no idea that I would be bringing Audra back to the hospital just a few short hours later.  These 3 are so resilient, tolerant and patient when it comes to all the extra time and attention that Audra requires.

 

This morning we had a yummy brunch at my sister's house with her family and our parents.   Then Sean, the kids and I all headed down to CHOP to visit Audra {with lots of tasty leftovers packed up for me to enjoy instead of the usual hospital food}.

We walked in to find her like this...

exactly how I left her 24 hours prior!

Me with all 4 of my little loves...

 

Audra did seem happy to see her brothers and sissy but she was still so very tired and had trouble keeping her eyes open for longer than 20 minutes.  Maura took her on a few laps around the unit in her favorite car ride push cart.

 

Other than that she napped...

and napped...

and napped !!

 

Sounds like the plan for tomorrow is to cut one of her meds in half to see if that helps with her sleepiness.  She is also due for her weekly immunoglobulin infusion.  Other than that we continue to wait for the results of the blood tests that were sent last week and the week before...

Sleeping Beauty

Is it the new medication?

or...

Is it Audra?

 

Right now it's lunch time on Saturday and Audra has basically been asleep since going to bed Thursday night.  And that was after a day full of naps Thursday.  So...is it the new medication making Audra so sleepy...or is this just one of her typical zombie-like phases but more extreme...or is it something else completely.  I've managed to get her up and keep her awake for 30 minutes a few times to offer her food, give her a shower or take her on walk around the unit...but then she falls right back to sleep!  She really didn't eat anything yesterday or so far today.  So glad she has that G-tube right now. 

Can't even keep her eyes open for her beloved pizza and French fries !

Yesterday she got a special delivery from Aunt Kristy...

 Yes...that's an 8 foot stuffed snake!!

 

Everyone here thinks it is quite the unusual gift, but there's an inside joke associated with it. Audra loves long stringy things...ribbon, shoe laces, tape, etc.  One day last fall while we were outside doing yard work Sean found a baby gardener snake and Audra just loved it!  She kept grabbing it and picking it up.  She must have like the way it squirmed around similar to ribbon when she shakes it.  Now we call her the snake whisperer.   

 

The only plan from the doctors today was to wait out this over-sleepiness that Audra is having.  They said they don't plan on making any adjustments to her medications until Monday.  They would like to see if she cycles through this similarly to how she does at home or if it persists, in which case she will need a medication adjustment. 

 

Today I noticed that Audra feels heavier than usual when I pick her up, and her skinny little toes seem thicker.  Usually her toes don't touch each other and there is space in between each one.  And when I gave her a shower I noticed that her legs felt "fuller" than normal.  Now all of this is probably not noticeable to anyone else, but because Audra is so skinny it is easy for me to notice slight changes.  The nurse weighed her and she was 18kg.  Audra has been 16-16.5kg for the past year with no budging.  She has NEVER been in the 17's.  So for her to 18kg is ALOT.  That means she has gained over 3 pounds since she was admitted 4 days ago...and that's with her not eating hardly anything yesterday or the day before.  It just doesn't seem possible ?!

 

So maybe that will pass too, or maybe it's a new curveball she is throwing us.  Either way it seems like we are on stand-by until Monday as far as making any changes or progress.  With that I decided to head home for the night to see my other 3 kiddies for a bit and stock up on some more food and toiletries.  During her admission last month was the very first time I ever left her alone in the hospital overnight.  This time she is not sick and feeling terrible, so it makes it easier on my heart to go home briefly.  And since she is spending 90% of each day asleep she may not even notice that I am gone!

 

Even when she's awake she looks sooo tired



The Plan

Audra got her first dose of Depakote on Wednesday night. It is given twice a day so she got another dose when she woke up yesterday morning.  The neurologist said it takes about 3 days to see results and if she doesn't show any effect from it by the 7th day then they will increase the dose.  I went upstairs to work yesterday but I heard that she was a sleepy girl all day and took a few naps.  And she slept beautifully the past two nights (all night long...the first time in 2 weeks!).  I am hoping the Depakote has helped her sleep so well and not the fact that she was exhausted from being off the wall crazy for a few days and only slept 3 hours Tuesday night.

 

It's hard to believe that this sweet angel is needing

Clonidine, Risperdal, Ativan and Depakote

plus the occasional Benedryl to keep her calm.

She hasn't had any more vomiting since Wednesday morning.  Phew!!  I was really worried that we would fall into the same trap we did last admission with all the vomiting that required a PICC line and IV nutrition.

The kidney doctors (nephrology) came to see Audra.  They have been managing her clonidine patch for the hypertension she was found to have last admission.  Their plan is to continue to same dose of the patch and watch her blood pressures.  And we can check a few screening labs since they will be doing blood work for other reasons. 

Yesterday the dentist came by the see Audra around 5pm but she was sleeping so he said he would come back.  He came back at 9pm, just as I was settling in to go to sleep.  Turns out the timing was perfect because with her nighttime meds on board Audra didn't mind him gently looking around in her mouth.  He found that she has 2 more adult teeth coming in on the inside of her mouth without the baby teeth being loose.  In November her dentist had to extract 5 teeth for this same reason.  It seems she has a very small mouth, without enough room for her adult teeth as well as delayed dentition meaning she retains her baby teeth longer than usual and her adult teeth are coming in later than normal.  Now...the dentist doesn't think this is the root cause of Audra's recent behavioral changes (and I agree) but it is absolutely uncomfortable and annoying to her most likely.  Luckily Audra has an appointment with her dentist next week. 

After her impromptu dental exam Audra was able to fall right back to sleep only to be awoken again at 10pm to have her blood drawn.  She tolerated it very well but unfortunately they only got one of the vials filled, and it wasn't the most important one...the one to check for a pheochromocytoma.  So she will have more bloodwork drawn today. 

The little princess has already woken up and gone back to sleep this morning.  Hopefully we don't have her overmedicated now.  I'm waiting on the doctors to come in with today's plan...

The sequel...Deja vu

And just like that, we are back again.  Audra has been readmitted to the Medical/Behavioral Unit at CHOP. 

She did well for about the first 7-10 days after being discharged on April 11th.  When some of the concerning behaviors started showing up again I contacted the psychiatrist who had worked with us in the hospital and she managed to squeeze in an appointment to see Audra the same day she had her nephrology follow up visit.

And, oh the follow up visits...there have been lots:

Seating clinic:  she was evaluated and fitted for a medical stroller, now we pray the insurance company approves it so that we don't have to appeal

Urology:  continue the prophylactic antibiotic to prevent UTIs and will plan for a repeat study to assess her kidney reflux and discuss surgical options at her next visit...unless she has another UTI before then

GI:  plan to try to advance her G-tube feeds once all her tummy troubles settle down, still considering additional scopes/testing to evaluate her pancreatic function

Behavioral Health:  unfortunately the didn't feel they had any services to help Audra because she required physical hands-on care to keep her safe and their staff are not trained or certified to do that (Audra was literally jumping off chairs and trying to climb out the office window during this visit, so I'm not surprised that she scared them off!)

Nephrology:  continue the clonidine patch for now and monitor her blood pressure at home

Psychiatry:  increased one of her behavioral meds and added a supplement that has been known to calm irritability associated with autism--something Audra hasn't officially been diagnosed with yet.  And although she does show some signs of it, I am not convinced.  But right now I am willing to try just about anything to get her back to the "old Audra"

Audiology:  at the visit it was noted that her hearing aids needed to be sent in for repair...again!

Nutrition:  By this visit Audra had lost some weight but she was just coming off of 2 days of being zoned out, sleeping and not eating much after 2 days of being hyper and agitated (and probably burning off every single calorie as soon as it went in).  Agree with GI's plan to advance G-tube feeds when able

Neurology:  Audra was super ridiculously wild at this visit and her neurologist was concerned for her and my safety.  We discussed re-admitting her but in the end decided to try giving her Ativan around the clock to see if it would calm her.  Audra also had blood work done to see if she has an autoimmune encephalitis that is causing these severe behavior swings.

With all of these visits Audra missed quite a few days of school and she was also sent home one day each week for low grade fevers, which meant she needed to stay home the following day also so that she was 24 hours fever free before returning to school.  Plus she had one day of vomiting that I kept her home from school for.  So in the past few weeks she has only gone to school a handful of days. 

This past Friday and Monday they kept her in school all day despite her very challenging, dangerous and disruptive behaviors.  We had started the Ativan on Thursday but still weren't seeing an effect from it.  On Tuesday she threw up at school so I turned around on my way to work and drove back to pick her up.  Once we got home I checked my email and there just happened to be an email from her neurologist asking how Audra was doing with the Ativan.  After a few emails back and forth, detailing each day since her appointment with the neurologist 5 days ago we agree that Audra needed to be admitted to have her medications managed/adjusted.

After a long 8 hours in the ER (and one dose of benedryl that put her to sleep for 5 hours!) we finally made it to our room in the MBU at about 10:00pm.

In fact it was the exact same room that she was discharged from with the exact same nurse that discharged her exactly 4 weeks ago!  Talk about Deja vu !!

It was a long night last night...Audra woke up once we got her to the MBU and she was still quite agitated and aggressive.  She ended up only sleeping from 2-5am, which was definitely not enough sleep for this tired mama! 

Today we met with many familiar faces, all people we knew from her last admission: doctors, nurse practitioners, psychiatrist, nurses, and 1:1 sitters.  Right away the psychiatrist said that Audra's pattern of behaviors is not consistent with a solely psychiatric cause.  So they will be evaluating her for medical issues that could be contributing to her constellation of symptoms: behavioral issues, developmental regression, hypertension and facial twitching.  The bloodwork she had drawn last week to look for autoimmune encephalitis is still pending.  They will be sending more bloodwork to look for signs of an adrenal tumor (pheochromocytoma), rare and unlikely, but they will rule it out since they considered it during her last admission also.  Neurology came by and their plan is to stop the Ativan and start her on Depakote (a seizure med).  It is used to treat conditions other than seizures, and for her they hope it will calm down her abnormal EEG spikes which might in turn improve her sleep which could help her behavior issues.  Also on the list is to have the dentist come see her and look at the new teeth that are coming in behind her baby teeth despite the baby teeth not being a bit loose.  She had the same thing happen in the fall, so the dentist extracted 5 teeth to make room for the new ones to come in. 

So that's the update for now. 

Maura's 1st Communion

Today was a beautiful day!

 

May sweet girl Maura received her

First Communion

 

My mom spent many hours cutting, sewing and stitching her dress which was a labor of love.  She used the remaining fabric from my wedding dress skirt to create this beautiful, one of a kind dress for Maura.  She even added a pleated waistband similar to the one my wedding dress had, and hand sewed tiny crystals around the neckline.

She had previously used some of my wedding dress to make Maura's baptismal gown, so now all that remains is the bodice.  I'm sure we'll think of something to use that for someday!

And Maura is wearing my exact wedding veil,  no alterations needed (except a few soft pink flowers added by grandma :)

 

-

 

Maura with her grandparents

My with my beautiful girl



 

After mass we had everyone over to celebrate our girl and her big blessing...