Look Ma No Tubes!!!

Juliana has been off oxygen for a full 48 hours now!! Her sats remain in the very high 90's and I am still in disbelief. Unfortunately she's been very cranky with her new tooth coming in but she is enjoying the freedom if being untethered. Feeding is going very well and she is willingly accepting baby foods by mouth. Her pulmonologist and the team in Boston is going to be so excited. I have already emailed our service coordinator and our TPN nurse as I just couldn't wait to share the good news.

She's getting ready to be the cutest flower girl the world has ever seen! Keep up the prayers.

Also please pray for our friend Matisse who is receiving her long awaited transplant today. She's a very deserving little girl who has waited many years to receive this transplant and we are hoping that her body will accept the transplant and she can pull through surgery with as little complications as possible and one day eat like a regular kid.

All Is Well

The past few months have been very busy for me. Working full time has really stretched my time thin and I have neglected Juliana's blog. In this case no news is good news. She is still struggling with her weight but we have not seen any significant losses. We have made some changes to help her pack on those pounds which seem to be working. She has changed to a new formula that is one and a half times the amount of calories that she was receiving before. She is adjusting to it well with a few adjustments.



She is moving right along developmentally. She is babbling and carrying on conversations more often (baby conversations of course). She is follows directions well (for now at least) and you can really tell that she understands what is being said to her. About a month ago she received orthotics for her shoes to help her foot position. Within a couple of weeks she started walking across the room on her own. She now spends most of her days upright and walking everywhere. She is not professional just yet but is certainly thrilled with herself as she turns around in a complete circle without losing balance.

At our last clinic visit we actually discussed setting a date for surgery as her oxygen requirement is extremely low right now and she is even able to tolerate up to an hour completely off oxygen. Her sats off the oxygen are not fabulous but not low enough to set off the alarm on her monitor. We are hoping to get surgery done early next year so that she has enough time to recover and deal with any issues. We are excitedly anticipating her role as flower girl in my brothers wedding next year. I am sooooo excited for my brother and his new fiance.

We have made some very small but big steps in the world of feeding and mouth play. Juliana will drink from a sippy cup! Of course it's only on her time but at any rate it's an improvement. She will only drink water as of yet but we keep trying. She loves to chew on washcloths and is tolerating her toothbrush with toothpaste. Toothpaste is the only thing with flavor that she will allow in her mouth so far.

We have a couple of new therapists this month and Juliana seems to be adjusting to them well. She actually tolerated playing with play doh and wasn't as aggressive in fighting back during mouth play. We loved our old therapists but we all felt that after a year of the same faces that maybe it was time to try something new with a fresh pair of eyes.

I will post some new pictures within the next week. She has grown so much over the last few months and not just in size. She is maturing right before my eyes. It's all so hard to believe how far she's come, but I have seen it with my own eyes and she is truly a blessing.

Losing Weight

Juliana has been losing weight over the last couple of months. In April we had such a huge jump in weight and we were all very excited. We dropped down to 6 nights of PN and one night of hydration. Went back to clinic in June and she had lost 500 grams which is about a pound. So the plan going forward was to adjust her PN for increased calories and still continue with 6 nights per week.



We were due for a weight check and labs at the end of the week but unfortunatley needed a line repair so we ended up in the ER. They weighed her and much to my surprise she was down another 300 grams (about 10oz). I called in to the CAIR clinic to let them know and see if they wanted to add on the 7th night of PN and they don't. I am not sure what the reason is but they want to wait and see how she looks on Friday which is probably not going to be any better. She missed a whole night of PN and Omegaven due to the line break and was looking very dehdrated as a result. We put her back on as soon as we could but at half rate because of the repair. Until the line is completely dry we can't infuse too fast. We infused over 24 hours instead of 12 to ensure the she got the full amount of her PN. Tonight she will be back on her regular schedule but I don't think her weight is going to be back up and I am not liking the "wait and see" idea at all. I do trust that they will make the right decision for Juliana but I am on the edge of my seat for now.



I understand that these are the ups and downs of having short gut but having never dealt with a weight loss issue I am at a real loss. I cannot give her anymore than she is already getting enterally (through the feeding tube) because of her short gut but maybe we could find some way to increase her calories. I am hoping that we can turn this around real soon and get back on the growth charts.

Bath Time!

We are back from Oley and had a wonderful time in Saratoga Springs. The best part was being able to connect with so many families. Even though we may not share the exact same diagnosis alot of what we deal with day to day is the same. It was also nice to see our care team in Boston in attendance as well. I did miss most of the main sessions but they were recorded so I can catch up on what I missed. The breakout afternoon sessions were the most valuable to me.



Overall the most exciting thing we discovered is that Juliana's bags will hold up in the bath. We were always told not to get them wet or they will fall right off. For the last two years Juliana has been sponged bathed on a waterproof pad on the floor with multiple containers of water for washing and rinsing. It's not the easiest way to clean a baby but it worked for us. She would occassionally get a bath when the bags had leaked and she was due for a dressing change. That has probably happened only a few times and she would get so upset it seemed not worth it. Now that we know we will be living in ostomy world for at least another year, it seems appropriate for Juliana to experience bath time on a regular basis. After talking with a fellow ostomate we found that the bags could get wet without the risk of losing a perfectly good bag. I still expected to need to change her bags following the bath so I had all the materials ready to go. Well the bags didn't leak for two days which is a record for us.



We bought her some new bath toys in celebration and are now on a schedule that revolves around dressing and cap changes rather than dressing, cap and ostomy bag changes. Much easier to plan this way and is wonderful for Juliana's development. She is at the age where bath time is fun and she does really enjoy it.

"Ma Ma"

Juliana said Ma Ma. Maybe not to me but she did say it and will repeat it as long as you don't ask. She is babbling so much more now and is definitely working on her two year old behaviors. She enjoys pulling all the pots and pans out of the cabinet and then stacking and unstacking them over and over again. She is crawling up the stairs to go to bed now. She can make it all the way up the full flight and down the hall to her room without help (just a little coaching).

Unfortunately our clinic visit to Boston didn't go so well. Juliana lost weight for the first time since she's been home. It was rather disappointing but considering we dropped a night a TPN and she is extremely active during the day it's not completely without reason. Fortunately the docs at CHB are so awesome they are letting us keep our night off and adjusting the calories on her TPN for the other 6 nights to make up for it. I was really surprised that they would actually ask me how I liked having the night off. It is real nice to have a break especially if I am running late getting home from the grocery store or where ever. I can come in and just hook up a bag of hydration fluid. This is why we tend to leave hydration night for Friday or Saturday when I know it's more likely for us to get home late.

We are also getting ready for Oley. I can't wait to meet other families and learn more about managing Juliana's care. Our pharmacy was so helpful in making sure that we had more than enough supplies to get us through. We have an stationary oxygen tank being delivered to the hotel and a couple of extra bags of TPN were sent with our order today in case we have an issue with one while we are away. It's nice to have a pharmacy that thinks of what you need before you even need to ask. There are some people in the company who will give us a hard time but for the most part they have been good to us.

We have another quite month ahead of us filled with only our regular therapies and a couple of doctor visits. It's nice not to have to do all the crazy running around.