Tuesday, October 29, 2013

Hope

We had a great semi normal weekend last weekend.  Filled with family, friends and fun.  It was nice.  I'm exhausted.  But, I'm grateful for the memories that were created. 

Last Thursday we headed here:


It's no secret that I LOVE my Trojans and Trojan Family (yes it does exist) and so ending up at Keck with Riley was relieving.  Why?  Because after months of wondering when? where? how?  we finally landed somewhere that will be able to help Riley with her bilateral Cochlear Implants.  I can't even tell you the number of times I've cried, vented, called, tried.... to just make this a reality. 

We met with Dr. Rick Friedman.  And just as his bio says he is one of  the nicest  and easiest to talk to doctors I've met in a while.  When he asked if I had any questions I let him know I had done a ton of Dr. Google research.  He then asked if he checked out ok.  He does.  He has a ton of experience and passion behind what he is doing.  Doctors like that are the ones you want to be in front of and want on your team.  My only nerves that day came from navigating the parking structure.  Really, trust me, if you'd driven around the parking lot maze like we did for 37 minutes (yes, I timed it) looking for a parking spot, you'd understand!

If all goes as planned, Riley will have her CI surgery on December 23rd.  The best Christmas present I have ever received.  Really.  Now we just need to get past the ACTH and hope that all goes well with that and we're good to go.  And..... Riley will be the 1st ever Pediatric CI at USC.  That my friends gives me chills.  The excitement I had walking out of that appointment really helped set the tone for the rest of the weekend.  We are so thankful to each and every person that is helping to make this happen.

Friday morning Riley had PT and she jumped right back in.  Her therapist had her on her knees and arms practicing lifting her head up and she did this:
Riley trying her hardest to lift her head,
Holding her head up with some support.

Friday afternoon I ran over to Logan's school to join his class for the October birthday celebrations.  There were 4 birthdays in the class in October and Logan had been waiting since September's celebration for his party!  He really is my child, making his birthday last all month long!
 

Mmmm cupcakes!

At the front of the class with his birthday balloon!

Friday night we were able to meet up with Tia T and Uncle Mike for the Zac Brown Band concert at the Hollywood Bowl.  Grammie (my mom) came over to stay with the kiddos and hold down the fort so we could get out for some grown up time.  It was a blast and a night we'll remember for a long time.  I really wish we could make these "date" nights a more regular occurrence.  And trust me we try! 

Saturday morning we headed home early so I could grab Logan and head to the USC game.  Logan was so excited about seeing Traveler and the guy on the horse (Tommy Trojan).  We met up with Grammie, Pa Doug, Uncle Brad and Molly before the game and Logan and Molly had a blast playing around together.
Best Cousins

Cheese with Grammie
Tallest to shortest with Pa Doug
 

Seriously!!!  Where did this big kid come from!

Game time with mommy

I've missed this view!  Finally at a home game!  My happy place!!!
I absolutely love that Molly and Logan just adore each other.  Logan was so worried about Molly holding his hand and staying close to us!  Our seats are a couple tunnels away from my parent's and by the end of the 1st quarter Logan was anxious to get back to watch the game with Molly.  There happened to be a few seats open around Grammie and Pa Doug (not a surprise to those following college football :() and so Logan was able to watch the rest of the game with Molly.  I'm thrilled I decided to go to the game and take Logan.  He had a blast and is turning in to quite the USC fan!

Sunday we headed to our first Halloween activity which was Trunk or Treat at Miss Mia's preschool.  Batman and Robin were adorable and although we missed being able to bring Riley (crowds are not good for her) we still had fun.
Batman and Robin!

Mason learning how to "Trick or Treat"

Smiles with Mia
Yesterday Logan had his first field trip to the Pumpkin Patch.  I was so happy that I was able to attend and watching Logan and his classmates together is hilarious!  Those kids have some energy.  The rain held off just long enough for us to take the tractor ride, visit the animals in the petting zoo and have a quick tour of the park.  The trip ended with the kids picking out small pumpkins and decorating them.  Being the daughter of the ultimate PTA mom I've always wanted to be able to do the same for my kids so,  it was nice to be able to participate and be there for Logan.
More selfies with Logan

Yeehaw

Some of Logan's K friends
Today we had an ophthalmologist appointment and all went well.  We take Riley to The Center for the Partially Sighted and everyone that works there is just wonderful.  The doctor that Riley sees lost his own sight a few years ago.  He works with an optometrist and uses his computer to notate what is going on during the exam etc.  He explained Riley's actual vision to me today which I found interesting.  I was under the impression that Riley could not see out of one eye at all.  This isn't the case.  Without glasses Riley sees everything very thinly.  The example he used is that a 32 inch door opening looks like 12 inches to Riley.  Being me I joked that Riley thinks her mom is skinny!  Her glasses help to correct this and make things look like they normally would.  Once we get the written report from them I will have to consult Aunt Kara (my SIL) to have her explain to me more what it all means.  They were throwing around terms I have never heard of.

Tomorrow Riley has Visual Therapy, PT and ST.  It will be a busy day! 
Riley is doing ok right now.  Riley's ACTH dose was cut in half on Friday.  Unfortunately, with the dosage decrease Riley has started back with her seizures.  Not as severe at all.  Much shorter.  But, they are still back.  I am anxiously awaiting a call back from her neurologist's office to see what's next?  Dosage increase?  Office visit?  I'm just not sure.

Friday we'll have a repeat EEG.  I'm hoping it goes well.  I say hoping because the last time we had a routine EEG we ended up inpatient for 6 days.  We're still not recovered from that stay and would really like to avoid being inpatient for a while!
Queen of the side eye!
After all of that I am ready for a nap!  I will admit though that it was nice this weekend to feel "normal".

We hope you all have a very memorable and Happy Halloween.  Fight On!

Love,
Dave, Megan, Logan, Mason and Courageous Riley

Tuesday, October 22, 2013

We have a winner!!!

So sorry we're late with our contest winner announcement!  I was not feeling well last night and I left the bucket of names at the office so... without further adieu here is Mr. Logan picking our winner!  (He really was excited... I promise!)

Barbara Urso Duke!  Please email me at meganmchopper@gmail.com so we can arrange shipment of your prizes!

And now back to our weekly update!

I knew this was coming.  And I knew I'd be sad but, I didn't think it would hit me so soon!  It all started Sunday when we took the boys to the local pumpkin patch.  It was crowded and hot and stinky (smashed pumpkins in a field with 85+ degrees temps stinky).  We got our 3 pumpkins, the boys picked one out for Riley, and my desire to hang out longer just wasn't there.  Neither was Dave's.  I was sad that Riley couldn't come (we need to avoid crowds of people) and I know Dave was silently cursing me because I got the dates of the pumpkin festival wrong and we both thought we were going to an uncrowded open field, not the popular Cal Poly Pumpkin festival!  We powered through though because we had promised Logan and Mason that Sunday was the day to go to the pumpkin patch and there was no turning back.  And it was fun.  And then while at home listening to the news I heard that the "Love Ride" was Sunday.  That meant that 1 year ago Sunday we did this......




And so now begins life after illness, or holidays being celebrated a "new" way.  Most of the memories that really stick with me from the 1st year of the babies' lives are those from the last holiday season.  The babies were starting to "do" more and interact more with us and really settle in to their roles in our family.

I just want our normal life back sometimes (all the time).... and now more then ever!  The memories I have imagined since finding out I was pregnant with the twins.... the holidays.... the growing up together.... Trick or Treating.... Turkey days.... Santa and Christmas.  And yes, we will all be together, which makes my heart so happy but, it won't be the same.  We need to be careful who Riley is around.  We need to limit her activities especially now.  Will we even get to make a trip to see Santa together???  I mean I've giggled at the thought of me spraying Santa down with Lysol before my kid's could sit on his lap.  Trust me it's on my list of possible ways to get Riley to the mall!  And that makes me incredibly sad.  It makes me miss our old life.

However, in between these moments of sadness and self pity (and really they are just moments. there just isn't time to dwell on things too long...which is good) we are thrilled that Riley's body is still reacting well to the ACTH.  She is super agitated at night.  To the point that we've been using her extra Valium dose more nights then not.  We've tried everything and nothing is making her happy!!!  And on the flip side, with the extra Valium dose, Riley is very sleepy the next day.  Really can't win in this situation and it becomes a balance of what will help her relax and what do we need to hold off on so she can participant in therapies etc.  Not sure if we're managing it as I had hoped but, we're doing everything we can to make sure Riley is comfortable.

Last night however she was calm and so we let her rest and didn't disturb her to get in our nightly snuggles.  We really are living in the moment and taking things one day at a time.

We had our appointment with the Aero Digestive Clinic yesterday.  It's a once a month "clinic" in the ENT's office where an ENT doctor, GI doctor and Pulmonologist are all in one place at one time.  Theoretically, it makes sense but, waiting around to see all 3 doctors and having Riley poked and prodded 3 different times was a bit stressful and time consuming.

We left the appointment with plans for a bronchoscopy, endoscopy and PH probe.  The scheduling will be a bit interesting but, we're hoping to pair these tests up with Riley's inpatient continuous monitoring that will start on 11/13.  The continuous monitoring will be the 24-48 hour EEG that will show us if the ACTH is truly working.

The one good thing that came from the appointment was us getting to meet a new ENT.  Our normal ENT was also in the office yesterday and after a chat with him about certain things we're doing for Riley I feel we're not on the same page.... at all.  In fact, I was pretty taken aback  and upset over his dismissive comments.  But, the other ENT that we met happens to be a gentleman that had a g-tube and a trach when he was a baby.  In fact he had both since birth and they were removed at 4 and 8 years old respectively.  Remember that feeling of hope.  He reminded me that I needed to keep moving forward for Riley.  And it couldn't have come at a better time.

When I asked him if that is why he decided to become an ENT he said "yes, to help children just like Riley".  We clicked, he agreed with my plans for different surgeries and procedures and I could feel how much he cared about his patients in just our 15-20 minutes together.

My job as Riley's mom and advocate is to put people on her "team" that believe in her as much as we do (or can at least act like it).  I CAN NOT have someone discount our feelings or beliefs when it comes to Riley's care.  Or when it comes to decisions we are making.  Number one we are Riley's parents and there are some decisions that are made by us solely.  I don't stand around the OR barking orders while you're in surgery so please don't try and do my job.

Will everyone agree with me 100% of the time?  No.  Will I agree 100% of the time?  No.  But, to dismiss Riley's progress and potential will not at any time be tolerated by any professional dealing with Riley.  Period.  Especially, when all you know about Riley's case is what you saw in January.  Months ago....

So although I left the office near tears I am happy that we were able to meet a doctor that will be a wonderful addition to Riley's care team.  And now to break up with the other doctor.  It's like I am plotting my middle school break up with the boyfriend I was barely going steady with.  I'm sure this particular doctor won't care but, we will still be in his office every once and a while to see his associate so the chances of running in to him are high.  Just a bit awkward! 

Today we followed up with neurology.  As soon as I uttered "seizure free for 48+ hours" I knew I was in trouble.  The appointment went well and we'll have a quick EEG next week to make sure all is well.  Riley's doses of ACTH will start being titrated on Thursday.  I asked the doctor for another clarification of what the medicine was doing and he drew me a picture.  :)

I really like this neurologist so it was nice he could explain it all to me again!  As soon as we got home Riley had a startle seizure that was +5 minutes.  She was aggravated and hungry but, it still was unsettling.  I know this treatment is working so these momentary setbacks are frustrating.

Tomorrow we meet the CI surgeon from USC and Friday Riley gets her second round of botox.  As long as Riley tolerates it we aren't slowing down.  Therapies are back up and running and we'll settle back in to this routine until the next planned hospital visit in November.

Thanks for hanging in there with us!  Through the ups and downs, the vents and the celebrations.  As anyone who has ever had a child with a special need (whether it be an illness or long term diagnosis) knows, the one thing you have in your court is being the parent and knowing your child the best.  I know I am rather blunt and open about our daily lives but, it took me a few months to really figure out how important it was for me to question and ask questions when it comes to Riley's care.  Often times I feel like we are taught to grin and nod (especially when it comes to our medical care) and that is not what cooperative medicine to me is all about.  And trust me, there are so many people involved in Riley's care that without cooperation nothing would get accomplished! 
And because I just love these videos I have seen recently floating around YouTube I will share Katy Perry's Roar done at CHAD.  If you've never spent any time at a Children's Hospital this will touch you and if you have it might make you cry.  These places are where miracles happen.  Everyday.  Fight On!


Love,
Dave, Megan, Logan, Mason and Courageous Riley

Thursday, October 17, 2013

Week 1 of ACTH and a Giveaway

We're home and man is it nice!  Riley's meds arrived an hour earlier then scheduled Tuesday at 9:30am.  They were sent from Tennessee so I was a bit worried about them making it in time but, UPS came through!

Tuesday morning was a bit hectic as Logan had Grandparent's day at school so we had to put a plan in place to get all the Grandparent's to school and make sure someone was home to sign for the medication.  Thankfully, everything fell in to place and the timing worked.  Grandpa and Grandma Hopper were able to bring Dave down to the hospital to meet us and we were ready to go around noon.

Riley is still doing well.  I know she is so happy to be home.  Her agitation last night wasn't as severe as it was in the hospital and I am hoping that continues.  She's starting to get a bit puffy and swollen.  Hopefully, this will only last for the first 2 weeks that she is on the higher dose of ACTH.

I was hesitant to write this (fearing I'd jinx it) but, in the last 36 hours Riley has only had one small seizure.  ONE!  Yes, I am yelling.  She's only been on the ACTH for 1 week so this is fabulous progress!  It means the ACTH is working!!!!

I thought I'd explain a bit about ACTH and what it is doing.  It is amazing to me the things that are available now that weren't available just a few years ago.  I've pulled some info from the pharmaceutical company and Infantilespasmscenter.org.  .

From Infantilespasmscenter.org:
"According to the American Academy of Neurology (AAN) and the Child Neurology Society (CNS), the goals of therapy for infantile spasms (IS) are: [3]
Many studies show that when hypsarrhythmia (chaotic brain waves) lasts for long periods of time, brain development can be damaged. However, when babies with IS are treated early in the course of the illness, they may have a better outcome.[3]

Hormonal Therapy

ACTH (Acthar)

ACTH (Acthar) is a prescription medication that may work by helping the body produce natural hormones such as cortisol and by having a direct effect on the brain. It is in gel form and is given as an injection into the muscle. The gel is designed to slowly release the medication into the body after it is injected.
ACTH (Acthar) is a prescription medicine that is used to treat infantile spasms in infants and babies under 2 years of age.[8] (Acthar Medication Guide) In one clinical study, 87% of subjects who received ACTH had no spasms and no hypsarrhythmia within 2 weeks.[7] (Acthar Prescribing Information, 2010)
The recommended dosage of ACTH (Acthar) for treating IS is 150 units per meter squared, divided into two daily injections of 75 units per meter squared each. After two weeks of treatment, the dose may be gradually lowered over a two-week period and then stopped.[7] (Acthar Prescribing Information, 2010) If treatment is successful, the spasms and hypsarrhythmia (chaotic brain waves) should disappear.


And from the pharmaceutical company:


Acthar Is Not a Steroid

Acthar is not a steroid. It contains the hormone ACTH, which stands for adrenocorticotropin ("a-DRE-no-cor-ti-co-TRO-pin"). Acthar is a highly purified preparation of ACTH in gelatin.

How Acthar May Reduce Inflammation

Acthar works in 2 ways:
  • Directly with your immune system and central nervous system and with your body to help it produce its own natural steroid hormones (cortisol, corticosterone, and aldosterone)
    • These natural hormones may help reduce inflammation causing your relapse
  • Acthar is believed to affect T cells and B cells
    • This action is believed to impact the inflammatory processes


If this doesn't work which we are praying that it does there are other treatments available.  ACTH is the first drug to try though.  So now we sit back and watch this medicine do it's magic.  It's going to be a long 2 weeks until our next EEG!

I have two exciting things to share with you all today as well.

The first is that one of the first doctors that was a part of Riley's case from day one is going to be doing a case study on Riley's missed diagnosis and treatment etc.  Why is this exciting you might ask?  For us, as parents, the one thing we've hoped to eventually do is educate either families or doctors about Febrile Seizures, signs of bacterial meningitis and ways to diagnose it early.  Those 24 hours that we lost were so precious. And can't be given back to us.  When she asked if we would consent to her using Riley's case I couldn't say YES fast enough.  If just 1 family is saved from this heart ache, if just 1 child is diagnosed properly because of what this doctor is doing we will feel like we've made a difference.  And, because we are human, hearing that a doctor is seeing things the way we have from day one was vindicating.

So we're excited to see what becomes of this case study and we'll anxiously await the presentation in February to a conference of 500+ doctors!  We've been told we can attend the presentation.

I also want to invite you all to enter a little giveaway.  I've never done this but, after all of the support we've received and after such a successful CHOC Walk, we figured it was time to give back.

So......
We'll be giving away.........

Your very own 2013 CHOC Walk Tshirt!  Riley is not included in this giveaway!!!

A 2013 CHOC Walk medallion and lanyard........

1 Disneyland Park Hopper ticket (to ship in December and valid through 11/2014)

A $25 Disney gift card!

So.... How do you enter????

2 ways.

Leave a comment here on the blog. 1 entry
Like our NEW Team Riley Facebook page 1 entry
Team Riley Roo

Of course I've waited forever to start this page and Team Riley is already taken!  So Team Riley Roo (Riley Roo being a nickname we have for our little Miss) it is!

I'll take entries until Sunday night at midnight PST.  We'll draw and announce the winner on Monday.

Thanks for continuing to pray for us, think of us and follow us on our journey.  And.... Good Luck!

P.S. Feel free to share the FB page.  The more the merrier!!!

Love,
Dave, Megan, Logan, Mason and Courageous Riley Roo!


Tuesday, October 15, 2013

CHOC Walk and ACTH

**** Meant to post this last night and then fell asleep mid-post!  I know if I don't get this up it may be awhile before I can write another one!  We're waiting on a UPS delivery of Riley's meds and then we'll be headed home!!!****

You know those days when you are so exhausted you don't even know how you are going to make it to your bed.  Yesterday was one of those days!  Heck, I think I am still tired today.  However, my heart is so full and I am so happy we were able to be a part of something truly magical. (As cheesy as that sounds)  In fact, we're already talking about next year!  And the best news from today is that as long as everything goes as planned Riley should be home tomorrow!!! 

Riley is doing really well!  The ACTH is working from what we can tell so far.  This is such a relief.  But.... Riley is starting to feel some of the side effects.  We've termed this "roid rage". I had to step out of the room tonight for a bit of a breather because it is really hard to watch.  She can't get comfortable and tones and flexes until she is red faced. I don't know how we are going to get through 8 weeks of this.  However her startle seizures (IS:Infantile Spasms) have become fewer and fewer and without jinxing anything I think... just maybe we've crossed over to a new baseline.  Riley's phenobarbital will be increased a bit as well.  Last night she had an extra large does to load her up. Please continue to join us with prayers and good thoughts and whatever you have to help keep this positivity going!  And to help get us through the next 2 months.  I just keep reminding myself that this is best for Riley in the long run.  It's the unknowns that are leaving me a bit on edge though.   But, we'll power through this just like everything else and just take each day as it comes.

Yesterday was THE CHOC Walk.  It's been a crazy couple of days but, the memories that we made are priceless.  Simply wonderful.  And, because we decided with Tia T and Unlce Mike last minute to get rooms at the Hilton near Disneyland Saturday night the memories will continue for years to come.  It was, hands down, one of the best times we've had with the boys in long time and add in Miss Mia, Tia T and Uncle Mike and we now know we need to do something similar again.  Logan was SO excited to stay in a hotel and both boys loved playing around and exploring every nook and cranny in the room. 

Grandpa Hopper was able to come down to CHOC and stay with Riley so that this could all become a reality.  We always know when Grandpa is at the hospital that Riley will be taken care of.  It makes it so much easier to be away although I will admit that there were many moments Saturday night/Sunday morning that I felt sad.  We were supposed to be doing things with Mia and the twins and Logan all together.  Like we did last year when we all went to the pumpkin patch.


 

Those are the times that are hard for me.   When we're apart or doing things normal families do  my heart is definitely torn.  We were definitely missing a huge part of our family. 

We hit up Downtown Disney, without restaurant reservations Saturday night, yes I know all of you Disneyland lovers/planners are cringing.  Right around the time when I was ready to throw in the towel and just grab Mcd's we had a text from HOB that they had a table ready.  Thank goodness.  Mason without a nap is not fun!  Trust me!

After dinner we went back to the hotel and hung out before the fireworks at Disneyland started.  We had an amazing view from Tia T and Uncle Mike's room!  It was the perfect ending to a wonderful night! 

Yesterday I was up at 4am so that I could blow up the balloons we had for the walk.  Apparently Mason thought it was a good time to get up as well!  He's was in bed early last night.  4am is not good for anyone!

We had a bit of an issue with the valet at our hotel in the morning and I honestly didn't think we'd ever make it over to Disneyland.  Add in the fact that a zillion people were trying to get in to the parking lot at the same time and my anxiety was in full effect!  We made it though and met up with our amazing team of walkers.  The rest of the moring was wonderful, perfect.  Better then we could have ever imagined.

The entire event still leaves me choked up.  The support we had/have was/is amazing.  We've raised: $15,080 so far!  And I know we still have donations coming in.  How amazing is that!  For our first go round and deciding to walk pretty last minute I am proud of what our little team did!

Thank you to:

Alegria
Andy, Aimee & Sam Swanson
Anne Fisher
Annonymous
Annonymous
Augie flores
Aunt Kathryn
Aunt Kathy & Uncle John
Aunt Linda
Beverly Marksbury
Charles McCluer
Community Bank
Craig Schmidt
Craig Stewart
Deb Blanchard
Diana Cravotta
Doug & Jan McEachern
 
Escarciga family

Eva Margueriette
Gatt Family
Gina Parrille & Family
Grammie & Pa Doug
Grandma
Jan & Doug McEachern
Jeff Jaramillo
Jimmy Fahey
Kari McWhirter
Kathleen Malone
Katie Figueroa
Keri Sikich
Kristen Hill
Lindsay Jenkins
Lindy Evans
Lisa Mitrovich
Marie McDuffie
Mark & Joanna Crawford
Mark Miskinis
Mary McGinnis
 
Mike & Katie Shilling

Patti Hegarty
Penny Grund
Roberto Gutierrez
 
Rudy and Beverly Hopper
Sondra Stokes
Sue Montgomery
The Eggleston Family
The Giordano's
The Hopper Family
The Kneiers
The Leininger Family
The McEachern Family
The Puertas Family
The Schmidts
Uncle Ed, Aunt Toni, & Bailey
Yolanda Agajanian and Family
Chris Gallo
Maria Sacco
Steve Miller
Felicitia Colon
Naseem Waheed
Ewa and Richard Shilling
Jan McEachern
Selena Miller
Marty Salazar
Matt and Dana Stanman
Sue Seeker
Leane Stephens
Pat Seeker

Looking around and seeing all of the teams and hearing about their stories made the day even more special. I also think it was humbling. To know we have Riley and we're still fighting is a good feeling. Some families don't have that option.  As we walked out of Main Street and past the infamous Castle we heard "was in a wheelchair last year and is walking down Main Street this year". It was hard to not be teary. So many inspirational kids/families all around us.   We also ran in to many of the special people that have been a part of Team Riley while caring for her at CHOC and Healthbridge.  Some of the most caring, special people we have met along this road.  I just wish we'd had Miss Riley with us to say hello!
 
I'll leave you with a little picture story of our weekend and wonderful day.  We're looking forward to making this an annual tradition and hope to add many more walkers to our "Team" next year!  Fight On!
 



Our reason for walking!  My hero Miss Riley Roo!!!  Riley got a CHOC Walk medallion and daddy got to practice giving her shot.
 
Before heading to the hotel we stopped by to see sissy!  The boys had the 5th floor playroom to themselves!  I'm amazed we were able to leave!


 





 Downtown Disney fun!
 
 
Playing around in our jammies!  And... The Disneyland fireworks show!
 
Good morning Anaheim!!!  We're ready to walk!
 
 
Shoulder rides and walk fun!  Logan & Molly had a blast together! #memories
 
 
Checking out Disneyland!  Storm Troopers and Princesses cheering us on.
 
 
Grammie pushing the stroller!  Stroller dads!  One happy big brother!
 
Mia & Daddy (And some of our most favorite PICU nurses in the background!) Baby Luke walked too!  My love & rock!  More shoulder rides!
 
 
We made it to the finish line!!!!
 
Tired brothers!  The CHOC Walk is hard work!
 
Our amazing walkers!  The kindest, nicest group there was!  XOXOXO
 
Love,
Dave, Megan, Logan, Mason and Courageous Riley
 


 

 























 





 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 







Thursday, October 10, 2013

Infantile Spasms and ACTH

Well we're settling in for a few days possibly week here at CHOC.  Hopefully I can explain this all in the most educated way.  However, please please remember I am not a doctor.  Just a mom trying to get a grasp on everything going on and relay the information to all of you!

We had a scheduled EEG yesterday afternoon.  I had called on Tuesday to check on how long it would be and was told we'd be at the hospital for 2 hours including check in/prep/EEG.  Honestly, Dave and I were concerned this wasn't enough time to really see what's been going on with Miss Riley.

We made it through the 45 mins or so of the EEG and Riley was very good.  Calm, relaxed and did not have any of her startle seizures, as we've been calling them.  So.... I asked the tech if I could try and provoke her.  There are certain times when Riley does these seizures and I was pretty certain I could get her going.  I did not want to leave without having the EEG tech get the seizure on tape.  It would have been a wasted trip really and we know Riley needs some assistance to control these seizures.

Well, sure enough we turned the lights on, I played a video from my phone and Riley started.  The tech started recording again and then promptly left the room....  Ummm okay.  I texted Dave right away and said we're staying.  I just knew.  Again. 

She came back 10-15 mins later maybe it was longer, maybe it was shorter.  Really it's a blur.  She had more electrodes with her and let us know that neurology had reviewed the test and wanted us to head to the ER.  They'd admit us and we'd have continuous monitoring.  At this point we thought we'd be here 24 hours or so and then get to go home.  Well we were wrong.

Things didn't really become clear until this morning.  What Riley is presenting with is two different types of seizures.  One is managed pretty well with phenobarbital.  The other isn't  being managed.  Her startle seizure started a few months ago, here and there.  If you recall last month we were in the ER because her seizure activity had increased to multiple episodes a day lasting longer then we'd like.  We had an overnight stay a med adjustment and went home.  Worked okay for a few days and then the seizures started increasing again.  I really wish we'd had the continuous monitoring last month while we were here but, not much we can do now.  When I tried to joke about the what ifs and what could shoulda woulda in regards to everything (we're tlaking back to the day after Christmas) today the very dry, zero personality neurologist said I was going to give myself a stress ulcer.   I was trying to make a joke btw not being serious.  He had also never heard the term "Dr. Google" which humored me beyond belief.  Sometimes it's the silly ridiculous things that get us through the days.

At this point they are diagnosing Riley with Infantile Spasms.  While I feel that these have been caught relatively early I am still pretty sick about it.  Most likely her phenobarbital helped to keep these spasms at bay for awhile.  Now they are pretty regular and need to be treated.  And now here comes the complicated part.

The doctors discussed with us ACTH as the medicine to try and stop them.  ACTH is a steroid.  Treatment is over weeks and the dose is decreased slowly until eventually being stopped.  If it works this could be FABULOUS for Riley.  As it is now her EEG is pretty chaotic.  ACTH could help to bring it back to normal range.  I am hopeful that this can be accomplished.  And I am asking for your prayers and good thoughts that this DOES happen.  If not I am not sure what we'd do next.

There are side effects, as with any medication.  Riley will become swollen over the next few weeks.  Will probably look a bit like a marshmallow.  But, that will all go away over time.  She'll need to be closely monitored for higher then normal glucose, any bleeding and we'll test her blood, urine and stools.  This medication is not something you mess with.  You can't miss a dose or mess up the dosage instructions.  We'll leave here with specific notes.

It's an inject able so we've already started practicing on an orange on how to get it just right.  Thankfully, we have 24/7 nursing that will be helping with all of this.

Now here comes the crappy part.  ACTH is expensive.  Some articles I've read list doses at as much as $20k a pop.  We have insurance so that's not the issue it's the bureaucracy of having it approved.  Before we are allowed to take Riley home the meds need to be at our home and we need to bring them to CHOC to prove we have them.  They don't mess around.  It's anyones guess when that will be.

The case manager is already all over it and trying to get things moving as quickly as possible.  We hope to be home Monday possibly Tuesday at the latest. 

The first ACTH dose will be tonight.  Riley can come off of the continuous monitoring tonight!  Yay for being able to hold her!  She just fell asleep though so I may have them wait until morning.  It's going to be a goobery mess when the probes come off!
Over the next few days Riley will be closely monitored to see how she reacts to this medicine.

Riley's immune system is going to be very vulnerable for the next few months.  Anyone coming to our house needs to be sickness free and we'll be instituting a mask policy in Riley's room.  This is going to be the hardest part of all of this especially as we are gearing up for flu season and the weather is changing.  Another thing I tried to joke about with the neurologist.  You know how kid's in school are in a germ factory.  Yeah he didn't get that one either.

Tomorrow I have to make the phone call to Riley's audiologist to let her know we need to postpone her CI surgery.  We had finally made progress and were once again ready to have a consult with the surgeon in 2 weeks and now this.  Thankfully, I know we can still have it done after this but, I'm still really frustrated!  I am happy to say that once Riley is able to have the surgery it will be done at USC!!!!  It's pretty obvious the love I have for anything and everything USC so this is one development that has had me smiling ever since all of the pieces to make it a reality fit together! 

So what's next?  Not too sure right now.  Start the med and wait.  Beyond that we're hunkered down at the hospital.  Thought we'd update all of you though and once again thank you.  I know I ask a ton and hopefully it's not too much but, your prayers and good thoughts work.  Riley could use them again.  I just pray this is the answer for Riley and the beginning of good things.

And not to be forgotten is the CHOC Walk.  Unfortunately, Riley will not be able to attend.  We however will still be walking.  In fact as of tonight we are at a little over $13,500 raised!!!!  Which means we will probably pass the $15k mark by Sunday.  So thank you to ALL of our supporters.

As I sit in room 515 watching Riley sleep it only reminds me why we are walking.  Riley is in good hands.  Actually, great hands and there is no other place we'd rather be.... except maybe home.


Fight On!

Love,
Dave, Megan, Logan , Mason and Courageous Riley