Blogging about life in Minnesota, raising our six kids with Down syndrome while battling Breast Cancer.

Be the kind of woman that when your feet hit the floor in the morning the devil says, "Oh shit! She's up!"

Wednesday, November 18, 2015

Playing catch up

Now that I have borrowed a computer from the hospital's family resource center (thank you Geek Squad!) that I can keep in the room during our stay, I can also update the blog. Typing on the phone is so not fun. Its probably not fun for you to read through the typos either!

So...back to last week...

On Saturday we were finally given the possible diagnosis of OTC Deficiency. This is an x-linked Urea process disorder. Of course it is very rare, but of Urea Processing disorders it is the momst common. Angela's body is missing the enzyme needed to process ammonia in the body. Isn't it crazy that our bodies produce ammonia? I knew that, but..yeah...didn't really need to know that.

It was urgent that Angela receive a rescue drug called Ammunol, which is in class of drugs called "scavenger" drugs. It does just as it sounds, runs around the blood stream eating up the protein based molecules and converting them to products that can be excreted through the urine. The problem is there were only 6 doses of the drug in the 5 state region, and of course not where we are, St. Paul Children's Hospital. The drug was flown up from Mayo.

Because this hospital has never administered this drug before, all the staff was in a tizzy. A bit excited, really. Oh boy! We get a new drug for the first time! I wasn't really feeling it, since this drug has some potentially very harmful side effects, is caustic, and CANNOT mix with anything else!

Angela got a PICC line put in, then was moved to the PICU. The drug arrived around 1:00 am. All the nurses in the PICU, along with the doctors, went over the protocol given by Mayo on how to administer the drug, the signs and side effects to watch for, etc. Everyone was hyper-focused and I just stayed out of the way. Finally the Ammunol was started at 2:00 am, and would run for 24 hours. She had 5 other drips running at the same time: lipids, D10, electrolytes, arginine and I forget what else!

Over the course of the next 24 hours her blood glucose levels taken every 30-60 minutes. If she moved over 150 she was started on an insulin drip until she came back down. In addition, her ammonia levels were checked hourly to make sure it was trending down. For the first 12 hours Angela mostly just slept. Then she started complaining of her PICC line and arm hurting her. A couple hours of that and it was full-out crying that her arm hurt. Poor baby. I couldn't do anything to make the hurting stop at all. She was just miserable. It was the longest 24 hours.

Finally at 2:00 a.m. Monday morning the dose was complete. She complained about her arm a bit more. But the worst was that her Ammonia levels jumped right back to where they had started. The doctors thought she was needing to go back on the Ammunol for another 24 hours! Then the metabolic geneticist got back to them and said no. What has happened is she has gone into a catabolic state. Meaning her body was creating its own protien by attacking her own muscle, then converting it to ammonia again. So she was started on TPN (nutrition that goes right into the veins) so her body didn't feel starved and could stop attacking itself.

Finally on Tuesday, when her ammonia levels were consistently sitting at 80, we moved her back up to a regular room.  While she was out of imminent danger at that point, there was still a lot to do.


eliz said...

Oh poor precious Angela. My heart aches for her. What a mess. Hang in there sweet Love. (((HUGS)))) praying!

Jo's Corner said...

Hi Leah, Just want you to know that Angela and you have been in my thoughts and more importantly, my prayers. Keep on fighting, Angela! You are an inspiration. - Jo