Letters!

I've been busy busy busy! They letters are almost done they need some ribbon added around the edges and as bows to hang from. We had origionally bought plain foam letters that matched the first set of bedding. Since we changed the bedding I decided the letters needed to change too. I covered them with some scrapbooking paper and magazine clippings and I really like how they came out! Heres an upclose of each letter.

Cuteness Alert!

These are from Friday

 Toes!

 Walking to the sink

 Who said she could grow up?!

 She loves water

She wanted to sit in it

Love that smile!

Amber did really well on her Early Intervention evaluation today!! 

I'll do a post on it when I'm not so tired. Which leads me to a much needed apology for the many errors in yesterdays post. 

I think Amber is starting to get better, she's had less episodes of coughing and choking today.

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. 

Yikes, I'm really not doing well with my blogging lately! 

Theres some changes to Amber but I've just been too busy to update! 

Amber has been pretty sick on and off since January. Some of this sickness her Dr's are thinking is the I-Cell progressing and the rest is just that her body has a hard time fighting off a simple cold. It's hard to really tell because her labs show (for about a year now) that she's fighting some "infection". From birth her body has not been great at regulating her temperature, usually she has random fevers or just a higher regular temp. Also, from birth she's had a lot of mucus/secretion/snot. So you see our predicament; it seems she's always sick so it's hard to tell when she's really sick. 

Not only has she been hit pretty hard this cold season when she's not fighting off something she's been having episodes of respiratory distress. Meaning when she's attached to her pulse-oxygen monitor her oxygen levels are dropping to bad numbers, to say it the easiest way. When this happens you can also see her get paler and working harder to breath.  Unfortunately this has been happening more frequently and it's getting harder to get her to recover. 

 We've been discussing this with all of her specialists. Unfortunately it seems that we're already doing everything that we can for her. We've added a prophylactic (meaning daily) antibiotic to her daily plethora of medications. It really made a difference she was really healthy and acted like a whole new baby! Unfortunately (word of the day) she's sick again. We've also added a nasal steroid that will hopefully reduce the size of her adenoids, they are thinking that might be the explanation of the random distress. Unfortunately (are you counting) she would need another sleep study to confirm this and like the MRI we wouldn't be able to do anything with the information from this test (it would be a surgical fix which is a no). 

The other stinky news is it seems like she's needing more oxygen to recover from times of distress and needing it for longer. Her pulmonologist believes that the oxygen might actually not be helping her anymore or not as much. It could also be a reaction to oxygen and some of the medicine she's on but we won't know more until she sees her cardiologist (May 2nd). The only fix she can have for this would be to play around or to add medicine.

Our other new news is Amber has been approved for 32 hours of nursing a week. Part of me feels like this is admitting defeat. I'm trying to convince myself that it's important (since we're seeing her get worse) to get someone in now to learn her baseline (how she is not sick) so they can help more effectively in assessing a situation when she gets really sick again or progresses more. They'll probably start within the month or so once we pick the person or company that we want to work with.

I think that's it. This Thursday Amber has a GI appointment and hopefully she'll be getting a new g-tube button. They're supposed to be changed every 3-6 months and she's had the same one for almost a year. We will not be keeping it as a memento let me tell you it's gross and starting to get stinky! 

Her cardiologist appointment is her last follow up and then we'll be on another break from Dr appointments until the fall! Of course then we start summer adventures, showers and wedding season! 

Maybe by next spring time we'll have some down time!

Tomorrow is Early Intervention not sure how it will go since Amber is sick but we'll be discussing her walker! And I think I'm officially done now!! I'm also waay to tired to re-read/fix this so sorry in advance for any grammatical errors or incoherent babbling! 

Working On Wheels

Amber has been doing really well in her walker!

She's a pro at going backwards and turning sideways now! Her favorite thing is putting herself under the table.

We are still working on getting her to come forward.

 We've been trying all forms of bribery!! 

 She's also doesn't have lolly pops down yet.

 Once she gets it though....

 she loves them!

She has taken a few steps forward. Usually how it goes is, she gets really excited to move forward. Then confused about how to move so she ends up going backwards. Which makes her really happy so she goes running backwards until she gets stuck.
Along with lolly pops and cats we've bribed her with paper she can pull of the fridge and fridge/side of oven singing farm magnets. It's so cool to watch her discover more of her surroundings. I really love the visibility this walker gives her.

This is Amber's last week with the trial walker : /  Next week we'll discus the benefits of this one vs. her regular baby walker and also evaluate how she's doing in it. If it's decided that she'll be able to get one of her own I'm not sure how long the whole order/wait process will take.

Amber's New Wheels!!

Yesterday Amber started her trial with a Rifton Mini Pacer Gait Trainer!

It's soo tiny!

 It took a lot of hands to get her all set up. 

From the left; Amber's early intervention therapist, me, my dad, the equipment vendor and Amber.

 Amber's taking a break here and we're learning about all the adjustments we can make.

 It took us almost an hour and a half to get her fitted right but once she was comfortable she loved it!

It has a ton of available adjustments which is perfect for Amber's strange shapes, you can't even tell one of her legs is shorter!

 It forces her to be in a standing position, something she's not totally used to.

Because of this it's going to be more work for her until she's used to it but she's already doing really well.

 It has a ton of "accessories" to help positioning. 

Amber is using the chest prompt, the hip positioner with pad and hand holds. Since this is the trial version it also has ankle prompts, hand loops and the guide bar attached (in previous pictures). 

 It's designed to grow with her and also change as she strengthens.

Everything can be removed so it can also be used as a regular push walker.

She really likes it and since the wheels can swivel and go sideways she's already able to move and go places she can't in her regular walker. Amber is still pretty much going backwards. The wheels have settings to change how easy/hard they roll and you can also put breaks/locks on so she can only move forward.

I took this video at the end of the day, so Amber's a little tired. She can move around with her body straight and her head upright. At this point she had just discovered that she could see the floor so she wouldn't look up at all. Sorry it's kind of long but you know how kids are as soon as I stopped videoing her she would move! I did miss her two tiny steps forward but I'm sure in no time she'll be all over the place!

We have the walker for about three weeks and then the therapist and us will have to decide how beneficial to her and her development it would be. 

The Ever Amazing Amber

Apparently Amber missed the memo that's she's not supposed to be able to do most things that "normal" babies do. In my last post I talked about how she had started sitting unassisted on our laps. I've seen other babies use a boppy to help them learn how to sit. So this morning I  thought I'd give it a try with Amber. 

Yes she's SITTING! Something we never thought she would do! This is her second try. At first she was really excited about throwing herself backwards but once she got over that she did really well! 

We also played around with sitting her on her squishy pillow with the boppy behind her.  In this position she can put some weight on her feet to help stabilize herself and hopefully reduce any pain she might have in her back (something we've had issues with before). She did really well in this setup too. 

On the floor she's forced to have her back straighter. We thought this would be more uncomfortable for her. Actually she seems to breath better this way and it seems that she can get her balance better too. She sat this way for a little over 20 minutes! The more she sat the better she was at it. She caught herself from falling back and was able to play with a few different toys. 

She loves it and did not want to take a break!!

I think this accomplishment and the fact that she's feeling/sleeping a lot better is the best way to start out our busy week:

Tuesday, we have a home visit with our PACT team (the palliative care people)

Wednesday, Amber starts her trial with her gait trainer (walker)!!! 

Friday, Amber has a 18 month Well-Baby visit/follow up to being sick

We're looking forward to a productive week!!!