Just wanted to give you all some updates!
As of yesterday, she is breathing on her own without the ventilator. So far she
is doing great without it, she still has a tube with 40% oxygen and some humid air flowing just outside the trach opening to give her some support. The humid air helps keep her secretions moist so that they don't get hard and clog her airway (gross, I know...sorry 'bout that). It's a little scary when she starts to gag and cough, and you see all this nasty gunk come
out of her trach, but all the nurses and RTs (respiratory therapists) were all excited about it. "That's great! It means she is able to cough it out on her own and needs less suctioning from us, which can irritate her" So, at least it's normal, but still a little freaky right now. I assume we'll get used to it as she has it longer.
They started giving her Riboflavin yesterday on recommendation by Ne
urology, because of the fatty tissue they found in her muscles, which can sometimes be related to low riboflavin. She didn't seem to be tolerating it very well, because she started vomiting, and would do it 2 hours after she'd get her dose of it. At first the doctors dismissed it, thinking that the increased secretions were getting to her stomach and upsetting her tummy. But I definitely noticed a relationship between her getting the vitamin and her throwing up. She thre
w up yesterday during the day 3 times, and then again at night. Then this morning at 11 am, 2 hours after her dose, she did it again, and this time it was yellow which was the color of the
riboflavin. So, then I told the fellow in the PICU that it had to be related and she agreed. She told the attending doctor and he took her off of it. So, she's been off it for most of the day, since noon, and has had no vomiting episodes.
She also had a bit of a fever this morning at around 3 am, it was a low grade fever of 38 degrees C (100.4 F). They did some labs and her white blood cell count had gone up, which indicated that she maybe had some sort of infection. They think it could be from the trach getting some bacteria or something. They started her on antibiotics and her fever is gone, it was only one dose of it. She should be on the antibiotics for 7 days. Her temperatur
e has been fine since then. The only issue is that when she spikes a fever, her eye movements get much worse so she was doing a lot of eye rolling and blinking today. She's much more calm
I'm so happy because we've been able to lie next to her in the bed and snuggle with her. It really helps her fall asleep when we're next to her. Which is another improvement, since she used to require sedatives to sleep thru the night, now she is falling asleep on her own and is not as agitated as she was just a week ago. Even some of the nurses who are seeing her now, and hadn't seen her in a week or two could tell the difference. She still is not 100% back to "normal", like she was before she got sick; but she's not as bad as a month ago. It could be the IVIG, who knows, but to us it's a good sign. For now, we're just hoping she continues to improve.
Neurology and Genetics had some updates for us. They found an issue with one of her
chromosomes (I think it's Q1?). There seems to be a deletion within the chromosome, and they don't necessarily know what that means. It could be related to her issue, but they are going to do some more studies to find out what that deletion could represent. They are also getting us to get our blood tested to see if either of us have the same genetic mutation. The neurology attending doctor told me that the muscle biopsy results so far, according to him, don't really correlate to her movement disorder. He thinks that maybe the extra fat in her muscle could be the reason why she has a low muscle tone, but he doesn't think it has to do with the movements. They are still doing more tests on the muscle biopsy though, just to rule out if it's a mitochondrial issue or not. He also doesn't seem to think the chromosome issue is related either, but they are still looking into it. He says the most relevant clue so far is the low
neurotransmitter issue. They can correlate that with a movement disorder, which is why they
want to try the Carbo-dopa, Leva-dopa; a medication they give people with Parkinson's.
For now, the plan is to wean her off her sedation (she is completely off Fentanyl). She is still on a low dose of Precedex, they are weaning it little by little each day. As well, as her doses of Ativan, which will start to be every 6 hours instead of every 4. The neuro team wants to see what she looks like without the sedation in her system, that way they can evaluate what she looks like before the Dopa treatment and after. It would help them see if the medication is helping or n
Tomorrow she'll be getting her new trach. They say it should be a pretty quick simple procedure, hopefully nothing too exciting happens with that. They are going to start teaching us how to care for it, cleaning and suctioning; even how to change it out ourselves(!). It's a bit scary but the nurses assure us that they won't let us go home until we feel comfortable with it. They
have this freaky looking doll from the 50's I think, that they use to teach about the trach collar. I don't understand why they can't get an upgrade on the doll, it the freakin' MATTELL children's hospital! Hello? Anyway, I'm rambling because I'm tired and it's 11 pm.
Just wanted to say a HUGE thank you to all the people who have spread the word around about Leah and what we are all going through as a family. Everyone who reads this, you're going through it with us too, and it means so much to us to have support from our friends, our family and even people who don't even know us. The donation page and Star Wars charity raffle, is incredible how much people are willing to give to help us out in this time of need. And once this is all over and I have more time to do it, I'm sending everyone who has donated a personal message to say thanks.
We actually stopped by our place today and picked up some of her clothes that she could wear while she's in the hospital, mostly socks, leg warmers, headbands, pants and some skirts. We brought her Star Wars tees just to show our thanks to all the SW community that's been helping out.
I figured you'd all want to see more pictures of the little princess so I'll add some below.
Enjoy, good night and keep Princess Leah in your prayers!