Tuesday, May 3, 2011

Nightmare Of A Weekend

As a prefix to the rest of this post I would like to first inform you that Amber has been sick since Easter Sunday afternoon. Last Monday and Tuesday her fever was 102-103. By Thursday and Friday it seemed that she was getting better. 
This is her Friday afternoon playing in the sink. I put her to bed Friday night and everything seemed fine. Set up her feeding bag around 10 and checked in on her around 12:30 because she was coughing. At 2:30am she started coughing and choking so I went in to suction her. I always wrap her up tight in her blanket before suction so she can't fight me. When I pulled back her blanket she was laying in a pool of blood. I can't even describe to you how scary it was. I opened her P.J.'s and could see it was coming from the G-tube, I was a little relived because I thought she had pulled her G-tube out.

We brought her downstairs, took of her G-tube dressing and called the PACT team to help asses the situation. It took us over and hour to stop the bleeding. It was coming from the around the G-tube (aka G-tube site) but we couldn't tell what was causing it. Because she didn't continue to bleed her Dr decided she didn't need to be taken to the E.R. and as long as she didn't start bleeding again or show any signs of a more serious problem we were probably fine and should just follow up with G.I. or her pediatrician. 
Yes this is our goofy girl at 3 in the morning kicking her legs! 

The next morning she was running a fever of 100.7 which isn't a bad fever but had me worried because she had been fever free for a little over 2 days. On top of the bleeding from the night before I was worried it was a sign of a stomach infection and after consulting PACT again we decided to bring her into the E.R. to run some tests and just make sure she was ok. 

Things just got worse from here on out according to the E.R. Drs but not too bad according to the ones that know her better.
The E.R. Dr's decided the bleeding was due to granulation tissue (gross tissue overgrowth basically). This answer didn't necessarily surprise us because she's more prone to getting granulation tissues and G-tube site issue when she coughs a lot . Here is an old post the last paragraph kind of explains why this happens to her.

The G-tube trouble shooting took no time at all and we should have gone home then but they were concerned about her sickness. They called for chest x-rays and some blood work which was fine with us. They also did the blood work she should have had on Thursday, all she needed for that was whats called a venus blood gas (aka VBS) which tells you how well the lungs are removing co2. 

The x-rays showed that parts of her lungs were deflated, which can happen with bad respiratory infections but could also be the start of pneumonia. It also showed that the vertebrae in the top of her spine/neck are starting to slip, this can happen with her disease. 
A normal VBS is around 40, Amber's is usually 50 Saturday night hers came in at 68/71 (they drew it twice). 

Needless to say this was not good news. The E.R. Dr's seriously overreacted.  They wanted to rush her to ICU and intubate her, something that Luke and I have decided against. After telling them intubation was out of the question they were trying to push us to still go to ICU and put her on a CPAP or BPAP machine but couldn't promise that she would ever come off of it. They also told us that if we were to take her home that night we should be firm in our decision because she wouldn't make it through the night. Yeah, we were shocked. We know her disease is progressing but we were not expecting to hear news like this this soon.

We decided to have her admitted to the C.C.C. (complex coordinated care) floor for overnight observation and if her breathing did indeed worsen we would decided what intervention to go with. She had a normal sick night. Meaning she had coughing, choking and some oxygen saturation drops but nothing seriously life threatening. 

Like any nightmare things were better in the morning. We got to talk to the PACT team and hear from her geneticist and pulmonologist. The first thing they told us was she could definitely go home! They said that although the chest x-ray looked like the start of pneumonia it looked more like a viral or bacterial infection but would start her on a heavy duty antibiotic just to be on the safe side. They also said that the results of the VBS were not surprising, show that her disease is progressing and they feel that (like being in the 50's) it's something that her body is adapting to.

Where do we go from here?
First of all Amber got a new G-tube! Oh yeah, the whole reason we were there was for the G-tube issue! This is the old gross, icky, nasty one in the picture. G-tube are pretty interesting (to me) and therefore one of my next posts will be about them. 
Second the spinal slipping. Isn't a great thing but it's another thing that shows she has I-Cell (as if the blood work and everything else wasn't enough). At this point until she sees her orthopedist were not sure what this means for her. It could mean her car seat could change (again) but we already handle her with extreme care. Any surgical fix like always is out for her.
For the sickness and breathing difficulties we'll need to follow up with her pediatrician and see her pulmonologist again.  It's definitely frustrating that we can't really do anything more for her. I'd love to tell you she's feeling better already but it's hard to tell. Yesterday she had more energy today she didn't and her fever was back to 101.9 tonight. Her cough is definitely not getting any better and it's a little worse than it was this weekend. We're just praying the antibiotic kicks in and she can get over this!

The best part of this weekend was Amber by far. Even though she is/was so sick she was being so silly. One of the best moments was; she was laying on the bed in the E.R., looking up at the ceiling, talking and laughing. After about 20 minutes I looked up to see what she was being so friendly to and saw this red balloon! 
She's also turned into a huge flirt she gives everyone (and somethings things) kisses!

I think I'll stop here for the night! I'm sorry about the information overload!
Tomorrow Amber has an evaluation with Early Intervention, we're praying she's healthy enough to cooperate. Friday we have an interview with a nursing agency we're thinking about brining into our home. 


  1. Thank you for your updates Schuyler! We were so worried on Sunday! I'm glad you are all home, and things were not as serious as the ER doctors thought! You're in our prayers as always! <3

  2. Oh Amber you are so precious! Love these pics! Doesnt seem to matter what is going on, Amber enjoys life and constantly makes us smile!!! Thanks for the update and the beautiful pics Skye...you are a mom who is amazing beyond words! Praying the antibiotics start doing their thing and this respiratory infection will soon be gone!♥♥♥

  3. So glad she is home and back to her happy self. I know the anxiety you and Luke must have been going through is just so awful... please know we are always here if you need us. no matter the time of day or night, you can always call us to talk. For real!! :)

  4. Amber is such a little blessing! What a sweet spirit!
    We love you all.

  5. Thank you for your updates and thoughts. You are all heroes!