Oh Hi Blog!

So it's been quite a long long time since I've blogged. So long in fact that apparently blogger has changed it's layout. I've got a lot to re-learn and I'm hoping this post comes out alright. I've got a lot to fill you in on but thankfully it's pretty much all good news! I'll focus just on Amber in this one and then finally post a bunch of half posts that I've got, yes I really had no excuse for not blogging.

I left you all in June in a rather downer of a mood. Amber had been suffering from pneumonia pretty much non stop since Easter. She would be horribly sick for a week (usually less) before we could get her on an antibiotic. Then she would stay horribly sick for another week or two before finally getting over it. She would be off of her antibiotic for a week before she was sick all over again. We felt like the cycle would never end. Pneumonia is probably one of the worst things an I-cell baby could get and unfortunately usually how they go. Thankfully as of the beginning of July Amber has been pneumonia free and we're going on two months of her being off of an antibiotic! We're praying she can continue to stay healthy.

I'm also happy to say that not only is Amber staying healthy she is doing incredibly well right now. As of right now her Dr's are not seeing any significant progression in her lungs or heart. This is great news especially after all the work the pneumonia put them through. Her gum overgrowth has progressed some but it's still not affecting her day to day quality. Her cornea clouding is a little worse but not really affecting much and her eye sight has improved some!

Physically Amber is also doing really really well! She is starting to sit unsupported for very short periods of time! Last year Amber started rolling onto her tummy but not very easily or very much. She is now rolling on to stomach a lot and twice now she's rolled herself back onto her back! She has also been trying to reach far for things while on her tummy and she's starting to lift her butt up. I wouldn't be surprised if she figures out her own way of crawling soon. Her other new thing is when she's in her walker instead of pushing back with both feet at the same time she pushes back one foot at a time, like you would to walk forward but only to move backwards. I'm also excited to say that her gait trainer has been approved by insurance and has been ordered!!

Overall Amber has really been surprising us with new things and doing so well! She's even been sleeping through the night, of course since we started night nursing! We could not be happier and we're praying she can stay like this for as long as possible!

Return Of The Zombie Mom

I really did mean to blog again before the NSTAR walk!!

 Unfortunately for you this means I have a lot to catch you up on. 

 We had a wonderful stay in PA. All of the pictures in this post are from our stay in PA but these are not all our pictures!  We were able to see a lot of family and have a lot of fun! Amber and I were supposed to stay for two weeks and unfortunately had to cut our trip a few days short because Amber got sick again. 

 We brought Amber to her pediatrician almost as soon as we came home and were told that she had pneumonia in both lungs. This was really scary because we're constantly told that an un-treatable pneumonia is most likely the way she will go. Thankfully since we caught it so quickly the antibiotics were able to do their job pretty much right away.

 Amber was back to her almost her normal self quickly but her lungs have taken a very long time to clear out. She finally sounded "alright" last week and is totally back to herself this week.

 We've finally started home nursing!! We've had a nurse during the day the last two days and I've loved it! I've been able to bake Luke's birthday cake stress free, get laundry done, do some much needed re-organizing to Amber's binders and blog! 

 Once "training" is done we'll have a nurse for 2 nights and 2 days. I know I've really been fighting bringing them it but it will be nice to have a little bit of a break, be able to sleep and get some much needed things done around the house. It will especially nice to have them the next time Amber is sick.

 I think that's all other than a few nightmares with our oxygen company and a few of Amber's prescriptions. I do have to say I'm not quite a Zombie Mom at the moment. This last round of Amber being sick was really rough though I had totally lost my mind hence why I wasn't blogging and I was putting the phone in the fridge. Thankfully Amber has been sleeping well for almost a week now (of course since we're going to be bringing someone in) and I can slightly turn my brain off when the nurse is here. 

 I do have a bunch of posts "in the pipeline" and I promise the next one will be about the NSTAR let's just say it went amazingly well!! Amber is appointment free until July!! We're praying that she can stay healthy she's been off the antibiotic for a week now. Last time she only had a week off before she got sick again, she's got to stay healthy so she can be a flower girl for July 2!!

I do have to say... YAY BRUINS!!!!!( Sorry to our canadian I-Cell families.)

Giving Back

On June 12 Amber, my parents and I will be walking 7 miles for 

NSTAR's Walk for Children's Hospital Boston.

We will be walking with the "Mighty Metabolites" team!

Our team will be supporting CHB's metabolism program also know as Amber's second home!

Personally I think their top right mascot is super cute!! 

It was also very weird to see this picture of Amber all over the metabolism clinic last time we were there!

This is a video from 2009 but I love it and it tells a lot about all the reasons people walk.

Why am I walking?

Let me tell you it's not the walking itself, 7 miles yikes!

It's because of who we're walking for.

If you've been following us from the beginning you'll know we've seen a ton of specialist. 

Who each in their own way have been really great but there is just something about the Dr and nurse practitioner in the metabolism clinic that has just been amazing from day one. 

They have done so much for us in terms of testing and treating Amber. They're always there to take a call or answer an e-mail. We have such a great relationship with them they feel more like family than Dr.s! 

We are so grateful that the NSTAR walk will give us a chance to support them like they've supported us!

If you'd like to help sponsor me you can check out my page HERE. 

My goal is to raise $500 and I am so close! I would love to be a Miracle Maker and raise $750! 

It's been really cool to watch this commercial on T.V. and know that we're going to be a part of it!

I'd also like to thank everyone who has already supported me and/or shared my page!

Thank you so much!!

Changes

As Amber changes this blog seems to change with her. 

What started as what I thought would be your run of the mill mom blog has turned into something quite different.

The goal is still to capture our daily life for family and friends who can't be near us. 

A glorified (public) baby book or should I say baby brag book!

It has also become an educational tool. An easy way to explain to everyone Amber's medical needs.

Thinking back to before we had Amber's diagnosis we were constantly looking and hoping for any Dr to recognize something about Amber. I can still remember the feeling when her metabolic Dr.s told us her features weren't out of the norm for them. 

Maybe that's one of the reasons why I keep up on my blogging for the other I-Cell families and parents who searched like we did to find some kind of comfort in similarity. 

Which brings me to the current changes. 

First of all our title/name has changed. From "And Baby Makes Three" to "Amber's Adventure"

This is something I've been thinking about changing for a while now. 

Please note the URL has not changed, it is still mommaskye.blogspot.com!

Second I've added "Coming Events" to the left sidebar. 

Now you can keep up with what's going on!

Third, I've changed around the labels (also left sidebar). 

Now you can easily find more specific things about Amber like, her g-tube or previous hospital stays.

I think like any blog it's still a work in progress and I'll probably be tweaking how it looks over the next week or so. Feel free to let me know what you think of it or what you'd like to see or hear about!

Also just so you know what's going on with Amber she had another rough night and most of the day. Her fever got extremely high but once it broke she seemed a lot better than she has been. 

I'm hoping that she'll just keep getting better from here on out. 

I'm also not sure why my blog reposted the post below this one, it's from before Amber was born!!

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.