I guess I'll just try to update from where I last left off. A LOT has happened since then though, and for the sake of all of our time I'll try to be brief.
But given I do my best to be honest and straight forward on this blog, you should know up front...I'm crap at being brief.
Anyway, last weekend that I worked I got an update saying none of Avelyn's labs were resulting out. We were told that it could be any number of things, from a clotted PICC line, to laboratory errors, to high triglyceride levels. We were told not to worry, it's all likely work out. The chances of something being drastically wrong were slim. I guess no one remembers what child they are speaking of when we get this kind of news.
If something is exceedingly rare, or even better, only documented but once in the literature, it's likely to happen to Avelyn. Yea, because we have that kid.
So turns out Avelyn's triglyceride levels were 23x normal. We had doctors tell us they had never seen such a thing before in 20+ years of practice. Her triglyceride levels being so high kept causing her labs to hemolyze and not result out properly. It also puts her at an extremely high risk pancreatitis, which could very likely be fatal.
The thought still makes me sick, even though they have since come down to 10x normal (which is still insane).
For days various specialists were in and out but none were able to provide any clear answers. The hepatic specialists told us the lipidplogists would have answers for us. The lipidplogists told us, it was likely due to hepatic dysfunction. So around in circles we went with no real answers.
The team here on the unit thinks the cause is multifactorial and likely that she has been getting way too many carbohydrates, via fortified feeds and through her peritoneal dialysis and that she couldn't keep up with them. I can't help but agree with this theory.
Sean and I worried for months that she was getting too much sugar/carbs in her fortified feeds (the main ingredient of the fortifier is corn syrup) and in her high glucose concentrated dialysate. We brought it up often. Actually a couple weeks ago I brought it up again with renewed vigor when a nurse practitioner joked about how chunky Avelyn looked, and a protein supplement was added after her growth (length) was noted to be stagnant.
Anyway, to combat the insanely high triglyceride levels and elevated liver labs- she was started on fish oil and a medication intended to help with bile sludge (actigall). She was also put on a zero fat diet. She was given straight skimmed breastmilk with only the protein fortifier for three days.
Throughout all of this time she was also started on a new blood pressure medication due to hypertension. She got her first dose of the propranolol Thursday afternoon and it seemed to really be helping by the evening.
Then at 3 am she woke upset and got mottled. I asked the nurse to turn on the light and we agreed her purfusion didn't look great but she pinked up very quickly-within a minute or two.
I let it go. You'd think I would know better by now. I am still kicking myself for not making a stink of it. I know we live and learn as parents, but the stakes are so much higher with Avelyn.
The next morning I left for work. When I was a ways past Cookeville, around two hours out, I got a call from Sean saying Avelyn had basically just gone into respiratory arrest.
He said he walked in am from grabbing a coffee and she was purple and graying by the second.The nurse was right behind him and luckily the doctors were next door.
It all happened very quickly.
One minute she was fine, the next her airway spasmed closed and she couldn't move any air in or out of her lungs. Her airway was completely spasmed closed.
She got emergency rescue meds, including epi and albuterol to open her airway (while she was prepped for intubation) and luckily it worked, she was then placed on high flow respiratory support-and later continuous albuterol.
Meanwhile, I am still on the road, driving in shock (yea, stupid, I know). I made it another 15 minutes down the interstate before I was in hysterics and had to turn around and head back. I don't know why I thought I still needed to go to work other than being in a state of shock and disbelief. It didn't take long to click.
After all we've been through, Avelyn's track record isn't the best. I felt I needed to be there, even though Sean had done his best to reassure that she was stable- I knew in my heart I NEEDED to be there. Thank god for a mother's instinct (that shines even when the rest of your brain shuts down) because, she didn't stay stable for long.
After the initial crash, where she was very very nearly intubated, she did alright through the rest of the day and night- not great but alright. The next morning she went down hard and fast again. After another bolus or epi and more continuous albuterol she stabilized but then at noon she again began to struggle for each breath. This continued well into the evening. It was awful. She was seriously in respiratory distress ALL DAY.
The providers said that intubating patients with bronchial spasms often makes the situation worse, and that the plan was to try to support her through this acute reaction as best as they could.
At first it was believed that this was all due to the propranolol but as time went on and she continued to get worse everyone was perplexed. Sean and I raised suspicions for other or additional causes. The only changes in her treatment were the propranolol, the biliary sludge med (actigall) and fish oil. Sean and all requested to speak with one of the doctors on over the weekend (who was/is amazing-as all of them here are) and we requested for all of the recent meds to be discontinued. Doubt was raised as to the likelihood of an anaphylactic response to fish oil or actigall (which we agreed with) but we requested them to be stopped anyway (Avelyn has been the one in a million baby too many times...).
So she got her last dose of both medications at noon on Saturday, just before we talked with the doc. The unfortunate thing about these meds is both have an extraordinarily long half life (up to 89 hours), meaning they stay in your system a long time.
Avelyn continues to have anaphylactic symptoms. She's four days out, is on a continuous epi infusion, is getting IV and nebulized steroids, IV benedryl and is still having bronchial constriction, swelling of her eyes, mouth, tongue and upper airway. She is still requiring continuous high flow respiratory support and sedation as she has bronchial constriction/spasms if she gets worked up. It's awful and there's no precedent for how to treat such a reaction in children, let alone one as fragile as Avelyn.
Only one other example of a fish oil allergy exists in the literature and that person had anaphylactic symptoms for a week. That's crazy.
I feel terrible for Avelyn. She has been struggling so much through all of this. There's not a whole lot more that can be done to get her through. She's had labs sent off to see if she has antibodies against fish, those likely won't be back until next week-not that it would change her treatment course.
So basically she just has to get through this week, maybe longer. All three medications are listed as allergies for now. Maybe eventually we'll be able to tease out the problem, maybe not.
In the mean time, we've been snuggling a lot and just trying to keep her calm. There's not a lot else to be done for our air hungry baby girl.
I'll update again soon.
We love you all and thank you for checking in on our sweet baby girl. She continues to be quite the wonder.
The pictures below are in chronological order, you can see how swollen she is the last photo though at the time I took this photo to show how much her swelling had gone down. I don't usually take photos of her at her worst. It's just too sad. :(