Day 7 in NYU Hassenfeld Pediatric Intensive Care Unit
Just Relax, You're Doing Fine....
Move over sepsis and pneumonia, seizures are stealing the spotlight today. Today started out with a scare.
Josie desatted - her oxygen level dropped significantly.
I had just woken up. I actually slept from 12am - 7am - the most I’ve slept all week. Her eyes were open and I said my usual “goooood moooooooornnnning.” She smiled. I kissed her. More beeps.
There are always beeping sounds, but this was louder. Her oxygen level was 60. F*ck. It should be 100. I watch that level like a hawk. Under 90 and I drop to a running position like a sprinter ready for the gun to go off. The nurse came in. I pressed the seizure button, and seconds later she was back to 95. Was she just mouth breathing and the BiPAP couldn't sense an O2 drop? Was it a seizure? We completely took her off Depakote on Day 5. It's the medicine that wreaked havoc on her platelets and white blood cells, and that I blame for my new gray hairs. Well, let’s not give depakote all the credit, epidiolex gives depokote’s evil superpowers an injection boost. So, as of yesterday when I spoke with Neurology, she had missed three doses so far and there were no signs of seizures on her EEG. So it seemed Josie was doing ok without any depakote. I was super happy. The neurologist suggested we raise the Epidalex to give her added protection. I agreed to it, but when I spoke to Rich he had a valid point. Why raise it when we don’t know that she needs it yet? We’re in the PICU, with the best support if she should have a seizure. I agreed.
We have been dealing with over-medication for a while now; the drugs have created other issues. We started on anti-seizure meds when she was just two years old and we’ve only gone up from there. Every time she has breakthrough seizures, we raise the meds. I’m thinking of this as an opportunity to reset.
As I’ve been saying, it’s about getting that perfect medicinal cocktail. Too much triple sec can ruin a perfectly good margarita - depending on your customer (or brain). So, the neurologist agreed last night not to raise her dose without knowing that she needs it. Well, today was a different story. She needs something.
The oxygen drop was a seizure.
She also had a seizure at 6 am while we were both sleeping. The neurologist said it was “subclinical,” meaning there were no signs of it from the outside. I could have been staring at her and wouldn’t have known. This is another reason the BiPAP is our new best friend. Does this have anything to do with the fact that we didn’t go up on epidiolex like she thought we should? The neurologist said probably not. It’s better that this happened actually so we can take action now. So we just started a new drug. Josie broke up with depakote and now she's seeing Zonegran. The neuro says this is a similar candidate for success in the arsenal of anti-seizure weapons, but without those blood issues. Of course, you never know how the relationship will go until they spend some time together. Zonegran has to meet the other members of Josie's drug family and see how they all interact together. Will they be a match? Will they make the perfect margarita? Yes, I’m on at least three analogies for seizure meds now. I hope you’re still following. She’s having seizures today. I see them. We suspected this would happen. Doesn’t make it easier on mama’s heart or hair pigment. We need this new drug to kick in, step up to the plate and show us he's the one she's been waiting for. Her oxygen is periodically dropping below 90 too. I keep jumping up to stick a catheter down her throat to suction out the gunk. Add that to my mama resume. I’m also pressing the seizure button so Neurology looks at these moments on the EEG tomorrow morning.
The good news Today’s blood work showed her platelets at 100! We want them over 150, but they were 30 when this mess started. 30. They went up to 51 yesterday and 100 today. So at this rate, we should be there tomorrow. And her white blood cells are at 4.4! Within range! Can I get whoop whoop?! How did we do it.. by kicking Depakote to the curb and the antibiotics extinguishing the infections. Let’s talk about pulmonology Josie was on the smaller BiPAP last night, nose only. This is more comfortable for sure. Her platelets were high enough now for her to continue the chest vest and cough assist, which we started yesterday. She loves the vest; she doesn't love the cough assist. She was also able to take a break from the BiPAP throughout the day and just use the nasal flow. This was great. It’s like freedom. But, not today. Pulmonology wants to put her back on the BiPAP during the day as well for more support because her blood gas is over 50 (55 to be exact) and it needs to be lower. She needs some more time with more support. When I asked, "what do you need to see from her for us to go home?" She said “It’s going to be dependent on her breathing on her own during the day while she’s here. She’s showing that she is not ready to wean off the BiPAP yet.” So, day by day. This felt like a setback and I was pretty bummed today. Between this and the seizures, today didn’t feel good. And then a FOXG1 dad shared a quote with me that turned my sadness around.
“Every setback is a set up for a greater comeback.”
Thank you Kirk, Dezzi's dad. This helped me realize we needed today’s setback so Josie can be better than before.
I’ve always played this game in my head where I make wishes for people - complete strangers. It’s a happiness hack. I’ll see someone on the subway and inside say “I wish you feel so loved.” or “I wish for you to never struggle with money.” Things like that. Usually I say “I wish for you to have a good belly laugh today.” It’s a selfish practice really, because it makes me feel so good.
I’m doing it here, I walk past the kids rooms and make a wish, “I wish for you to be the valedictorian one day.” “I wish for whatever you're here for now makes you better and stronger.” I’m telling you, try it. It's fun and you will feel so good. As soon as you have a thought that doesn't make you feel good, try wishing good things on a complete stranger.
Music Inspiration The lyrics in the title of this blog have been my guidepost since my 20s. The song is "Strange Design." The band is Phish.
“I’m dripping in this strange design. None is yours and far less mine. Hold the wheel, read the signs, keep the tires off the line. Just relax, you're doing fine… swimming in this real thing I call life. Can I bring a few companions on this ride?”
The strange design now is Frankie the Fox and our FOXG1 merch, and Team Josie is dripping in it. Check out Phans for a Cure. My music community carries us through. And wait.. it gets better. The other day the beautiful music therapist, Nina Alden, who sang "Rainbow" to Josie (as seen in the previous post), asked me if there's anything she can learn for us. I replied. But, I forgot until she came in today and said "I have something for you. And here it is...
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
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