And She's Off!!

Last week was kind of a drag. After being advised by her cardiologist we've decided to not go ahead with heart surgery for Amber. It was a tough decision to make and I'm having a hard time with the fact that there is nothing we can do for her.

For good we got the ok to start giving her baby food again! She was kind of skeptical of it the first day but now she's loving it again. We'll also be changing up her feeding schedule again. In the next couple of weeks she'll be moving on to big girl formula during the day and pedialight at night. We've discovered that when she has pedialight at night she's much more active during the day and looks a lot better. With this new schedule she'll be getting more food by mouth and it will be a higher calorie so it will even out any extra calories burned by mouth eating.

The best news I have for you which totally makes up for such a crappy week last week is; Amber's therapist dropped off a baby walker on Friday! She tried it out on Friday and loved just being able to stand! The therapist told us it would probably take a while before she started moving in it. We didn't get a chance to put her in it all weekend but today we put her and guess what.....she walked across the kitchen floor! We were totally shocked! I could not be more proud of her!

Sorry it's sideways I was so excited I didn't even think that I wouldn't be able to flip it. Hopefully the tech guy can work his magic and fix it for me.

I could watch this over and over again! She went all the way back twice before she got really tired out but her face is priceless! She is so happy to be able to move around. Pretty soon she'll be all over the house.

Speaking nutrition, G-tube 101

We’ll start with nutrition. Nutritionists have their own language; a mix of medspeak, mathspeak and if you’re lucky momspeak too.  They give us goals for daily calorie and liquid intake and then we hash out the reality of what she will really tolerate.  It’s a lot of give and take until we’re all happy with an outcome.

Two weeks ago we changed her feeding routine. We were doing 16 hours continuous through the pump at a rate of 24 ml/hr (an ounce is 30 mls) with as many 2 or 3 ounces bottles as we could during the time off of the pump (I think the goal was 8 ounces daily).  Now we’re supposed to be doing 4 oz bottles every 3 hours and bolus feeding her whatever she doesn’t take by mouth. Bolus means the rate is the ounces she needs multiplied by an hour so if she needs just an ounce the rate is 30ml/hr. Our goal was to get her to take the bolus feeds in half hour time. Needless to say this didn’t work at all. She got way too full and starting having a difficult time breathing and a higher heart rate. Nothing too serious but still not cool.

Last week our schedule was: 9am 4 ounce bottle, 12 pm 2 ounce bottle, 1pm 4 ounces bolus feed over two hours, 6 pm 2 ounce bottle, 8pm 8 ounces continuous at 30ml/hr. This worked pretty well for a few days but then she started to have really bad reflux from it. We’re still trying to recover from the reflux and get her to tolerate a bolus feeding during the day.

Needless to say I’ve unofficially given up on bolus feedings. It’s unofficial because I don’t have the nutritionist blessing but so far (it’s been a week) she’s still gaining weight so I’m not worried.  Now we’re doing the 8 ounce night bit and trying to get her to take around 10 ounces during the day by mouth. She’ll tolerate the bottle feedings because she can control how fast she fills up and can stop when she feels too full, an option she doesn’t have with pump feeding.

She’s still battling minor infections and granulation tissue around her g-tube site. We just use an ever growing concoction of topical antibiotics, antifungal and other things to keep it somewhat in check. Her issue is the usual all because of her odd body placements (sorry that’s the best I can explain it in a sentence). In other words her chest shape makes her lung expand where her stomach should be. When they placed her g-tube they moved her stomach over to the correct spot. Now her lungs hit her stomach and “rock” the g-tube from the inside in turn irritating the site (also the reason she has breathing issues when she gets too full). It’s a pain because there is nothing we can do about it. She can get “rocking” irritation from the outside too (just from movement) so we have it all secured to help prevent some irritation. The bad news of this is she’s allergic to tape so she constantly has hives on her stomach.  To combat the hives we us hydrocortisone cream, but prolonged use burns the skin. The whole situation rivals the lady who swallowed the fly! We’re just praying she does some more length growth so she gets some space between the lung and stomach.

Yikes, I didn’t intend for this to be a long post! I am writing these posts on lack of sleep with a very full brain so please feel free to ask me questions if something doesn’t make sense!

I'll try and give you a picture reward for reading all these long updates! :)

Getting to normal

On Friday we went to Boston...again. This time we saw our nutritionist, who we love! She is the sweetest lady. She is also a mom. So she completely understands all of our concerns with Amber suddenly not getting the oral feedings she used to have and the stress of being on the g-tube for almost a whole day (and the fact that we know our baby best!).  She gave us some awesome news!! Amber is gaining 3 times as much as they expected her to! She is now a whopping 10 lbs 14 ounces! She is looking rather chubby! The worrisome part is she is still 23 inches long, she has not gained any inches in almost 5 months. Back to good news, because Amber is doing so well she now needs to be cut back on her feeding. Everything she gets is still 26 calories. She is still getting 24 ml per hour but now it is only for 16 hours! We now have 8 hours off!! She is now getting 3 ounces by mouth every 3-4 hours as well. We're hoping she does well on this so we can move her to being on the g-tube for 12 hours and take 4 ounces every 3-4 hours.

This Wednesday we'll be in Boston again to see Complex Care Services aka C.C.S. I'm really hoping for a lot from them and I hope it doesn't blow up in my face. I'm hoping they let Amber get back to eating solids. They can explain the mystery of her mouth (still no teeth), find someone to help her chest and just overall get us plug into more people who can really help her. I've heard great things about them but after all the struggles over feedings we've had with them I'm really skeptical. 

I'm not sure where I was going with the rest of this post so I'm going to go to bed :) 

The cat has not been shaved......yet 

What Happened On Friday

I'm sure you're all dying to know what happened at our appointments on Friday. I'm so sorry to keep you waiting but all the information has liquefied my brain, and then our PA parents were visiting and there was just no time to update....and on and on with my sad excuses :)

I'll start at the very beginning because it's a very good place to start.

Endocrinology: Found in the orthopedic department in case you were wondering. They had nothing new to tell us. They had not looked at any x-rays. They do not want to see us again until DECEMBER......hmmm sounds like some other endo dude I know. Anyways, since by the naked eye Amber's skeleton is only "messed up" on the top half they were not so excited. They did get blood work to check her vitamin D levels and said if anything interesting comes back from the blood work or the genetic testing then they'll see us sooner. Oh and they'd like us to see their orthopedic surgeon for a consult on her chest, maybe some other parts.

Pulmonary: Was a much better visit than I had expected. This is what liquefied my brain. All of Amber's breathing problems are due to her hernia and odd chest shape because of this prolonged use of oxygen is actually a bad thing. She will become dependent on the oxygen and will not take deep the breaths that are needed to help develop her lungs. So he wants to start weaning her off of the oxygen. If she is weaned off of the oxygen, due to her anatomical problems she will not get enough oxygen to help her overall development. So really its a lose lose situation. The first step is to get her hernia fixed. Then she'll have to go in for an Infant Pulmonary Function Test, this will tell them how well her lungs actually work. I'm not really ready to think about that test yet so I'll explain it when she's closer to having it done. Depending on the outcome of that test and some other blood tests they did we'll begin to discuss option to better Amber's breathing. They did do an oxygen challenge while we were in there, meaning they tried her on a lower level of oxygen every 20 minutes until she was on room air. She did a lot better than we had all expected so he told us to try her on a lower level of oxygen all weekend. It didn't really work because at home it's muggy and she still having a lot of breathing difficulties when she is in her car seat and eating. I'll be calling them tomorrow to discuss how she did, possibly the blood test results and see where we're going in the mean time. Oh she will also have a sleep and probably a swallow study after the hernia operation as well. I'm sure I'm missing other things but I'll remember and explain it better when things are happening.

Nutritionist: This was a surprise. The pulmonary specialist is also a neonatologist so he's very into the over all development of Amber and wanted us to meet with his nutritionist. They also pushed for us to see her because she has not gained a very good amount (for her) the last couple of visits. She was very nice. She still wants me to nurse as much as possible and add some formula to any bottles I pump. She also gave me "recipes" to get extra calories into the one formula bottle Amber gets and her cereal, fruits and vegetables. It makes feeding a little more stressful right now and it will definitely take some time for Amber and I to get used to. We'll see the nutritionist again in July. I'm not sure how I feel about this I don't think her bones or her muscles really need the extra weight.

Hernia: I finally got my call! It was rather awkward because I was in the same building as the surgeon and it probably would have been easier to just talk in person as I was trying to check out of pulmonary at the same time (thankfully my mom was with me!). He finally got the x-rays and agrees that her hernia definitely needs to be fixed and is the reason she is still having difficulties without the oxygen. He needs to go over her x-rays with his radiologist, discuss things with his anesthesiologist and should be calling me tomorrow to schedule either a consult or a surgery date. Amber needs to be over her respiratory infection for 2 weeks before they will do the surgery so they probably won't do it for another week or so. I'm really fine with this news. I think having the surgery now will really benefit her. When they mentioned the surgery before she did not have any complications from the hernia so I was really wary of the whole ordeal. I'm really hoping that once this is fixed she will be able to breath better, not be constipated, eat better and sleep better, so just improve things over all!

And then we finally ate and went home!

It was quite the day and we've been going non stop since!

But we had a very fun and lovely weekend, it was so nice to see our family again!

Now to bed!!