There's Always Been a Rainbow..

"When it rains, it pours, but you didn't even notice... it ain't rainin' anymore.

It's hard to breathe when all you know is the struggle of stayin' above the risin' water line."

I'm going to write updates here, in Josie's OG blog. It will be so much easier for me to keep our incredible Team Josie updated, and I'm always happy to return here to Josie's Journey.

A trip to the ER turned into the PICU - Night One

We drove Josie to the NYU Emergency Room on Friday. She was sleeping for just way too long. She does this from time to time and it's always a guessing game as to why. Could it be her seizure medication? Is she on too much? We just raised one, it must be that! Is it COVID? Is she just choosing to sleep all day like her Grandma Helen? Is she just over this cold dark winter? I am. Is she having seizures in her sleep that we are missing and she's postictal (the fancy word for being knocked out by the seizure monster).

We shoot darts; we guess. She can't tell us.

She was sleeping all day - for almost 2 days and by 4pm she was just limp and she was cold. We could not wake her up. It became scary. We took her temperature and her O2 and both were normal. I played "Rainbow" and I got a smile, so I knew she was there, but this wasn't right. So I packed a bag and we drove on in.

When we got to the ER, I made a bit of a stink and said we can't be in the same room with everyone, she might have severely low blood counts (which was true), and they got us right in the pediatric ER. I will say this a lot, the team at NYU was fantastic and saved our little girl.

The first nurse came in and took her oxygen, which was at 60! Within moments what seemed like every doctor, nurse, you name it, was in our room. Josie was frighteningly cold. They put the bear hugger blanket on her to warm her up. Extreme cold temperatures are equivalent to high temperatures, so I've learned. They sprung into action. It was so hard for them to get blood and an IV in, because Josie's veins are always hard to find and being so cold didn't help. They got it. They started her on an antibiotic. They got urine to culture.

Josie couldn't breathe, I heard someone yell "get the box." I still don't know what that meant, but I was thinking to rescue her heart? WTF. My knees felt weak. Rich and I felt moments of complete and utter fear. We thought we might lose her. They intubated her and got us up to the PICU around 10 pm.

Fast Forward to Night 5

Without going into the play-by-play of the past few days, I will say it wasn't until Day 4 that I could exhale a bit and felt that she was going to be ok. They were able to lower her sedation and remove the vent in place for a nasal flow.

Today she took a step back from the nasal flow and is on the BiPAP.

She's very sick. It's been an TV-worthy journey to discover what the hell happened.

This much we know..

Josie was so tired because she had a UTI that caused sepsis.

A bacteria we all have in our stomachs called Klebsiella created an infection that wreaked havoc. At the same time Josie has an infection in her lungs called Pseudomonas. She has aspiration bacterial pneumonia. This could have been going on for a while, but perhaps while she was sleeping and so lethargic, her drool was going in rather than out and she was aspirating her saliva. She's a gold medalist drooler and that's an easy way to aspirate.

A doctor said today - "kids are resilient and they very often seem fine and teeter on the edge until they push over and then they get very sick very fast." Josie has seemed pretty good - outside seizures - for quite some time. I even told someone recently, "she doesn't really get sick too often." Eat. My. Words.

Please give me a moment to dump all my guilt here and leave it here for good.. Ok here it goes:

I should have gone to the ER earlier. I should have taken her to a pulmonologist along the way just as part of her routine.We shouldn't have tried to give her tastes of food by mouth at all. She needed another swallow study first. I should have done a damn swallow study just because. I should've done another sleep study already. I should have gotten more opinions on how to manage her drool better when the solution we explored didn't work for her. Ok I'm done.

So what else is at play here.

Her platelets are super low. So is her white blood cell count. This could be from the sepsis, for sure, but it's also been an ongoing problem from one her seizure medications called Depokote, or valproic acid. We have a love hate relationship with this medicine. It's been a key ingredient in the medicinal anti-seizure cocktail, but like most cocktail ingredients, there's a downside. I think I just compared Depokote to tequila.

We need her platelets higher, way higher. We can't do the all the pulmonary therapies that will help her lungs improve while she runs the risk of internal bleeding. We also can't do an endoscopy to gain more insight into what's really happening in there. So how do we get her platelets up?

1. We take her off of Depokote. Oy. Even lowering a little has caused seizures to increase in the past. Although, we've lowered it by 6 ml for the past 2 days and so far so good. So that seems to be the plan for tonight - to completely take her off of depokote that has bee a major crutch. My hope is that she never needs it again and we can successfully swap it for another ingredient. Gin maybe?

2. Blood transfusion. No thanks. Praying we don't have to do this. Say it with me, "she will not need a blood transfusion." At one point, Day 2, they wanted to do a spinal tap to rule our meningitis but her platelet count was too low so they said they'd have to do a transfusion first. Once the cultures came back to show exactly what bacteria caused this f*#k'r, they were able to "comfortably" rule out meningitis anyway.

3. Her platelets should improve as her infection subsides. Yes, but we still have the Depakote issue so it's not enough alone.

From head to toe there is something going on that we're tackling here. 

The doctor last night said "we've got a hand in every bucket that we have to work on to make her better." I'm definitely repeating that sentence wrong. Let's go through these buckets:

- The UTI bacterial infection - Infectious Disease is on this.

- The Aspiration Pneumonia - Pulmonology and Infectious Disease.

- Her low low lung capacity (collapsed actually) - Pulmonology team - This warrants it's own post.

- Seizures - getting to the right cocktail that doesn't hurt her blood - Neurology team (our long-standing team, which is why we are at NYU.) - This warrants it's own book.

- Platletes and white blood cells - The PICU team is on this, and possibly bringing in Hematology.

- Allergic reactions - Oh this was cute. She had a full allergic reaction to one of the antibiotics and then another one to the next.

- I feel like I'm leaving something out.

The Plan

So tonight Josie will have no Depokote and an uncomfortable BiPAP Darth Vader mask on her face.We pray she just doesn't need the Depokote anymore and - no seizures! We hope the mask does it job to help expand her lung capacity and break up the mucous, while the antibiotics continue to get rid of the infections.

I'm going to stop writing now. But first...

The Music Inspiration

As always in Josie's Journey I share where the title came from. Kacey Musgraves "Rainbow" is so special to us. We play it all the time. In the morning when she first wakes up, and just any time. She always always smiles. Like I said, it was how I knew she was at all responsive. 

Once we were in the hospital, it happened again, she opened her eyes when we played it. I posted that to IG/FB and I wrote,  "I hope I can take Josie to see Kacey sing it live one day." Well, today a woman with a guitar came in for music therapy and she played and sang it for Josie. Her voice was beautiful and Josie loved it - and so did I.

My friends made me such a touching video last night set to "Rainbow" with them all wearing my FOXG1 Research Foundation's courageous mascot, Frankie the FOX. I bawled in the best way. 

 

If you could see what I see, you'd be blinded by the colors

Yellow, red, and orange, and green, and at least a million others

So tie up the bow, take off your coat, and take a look around

'Cause the sky has finally opened

The rain and wind stopped blowin'

But you're stuck out in the same ol' storm again

You hold tight to your umbrella

Well, darlin', I'm just tryin' to tell ya

That there's always been a rainbow hangin' over your head

To Team Josie...

Thank you is not strong enough to express how much the love and prayers have helped us. She opened her eyes only 20 minutes after I posted and shared with everyone what's been going on. We feel so loved and supported. I've cried so many different tears here.. fear, desperation, sadness, but also tears of such incredible gratitude for the people in our net.

The net's unbreakable.

Team Josie Strong!

For more information about the work to find a cure for every child in the world with FOXG1 syndrome, please visit our website www.FOXG1Research.org 

And to help support us, please go to www.foxg1research.org/donate