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.

No Surgery!!!

Well it's been a while but not much has changed. Juliana is on 1/2 a liter of oxygen and from the looks of it she's not going anywhere soon. We had a chest x-ray done today and although it doesn't look terrible her lungs are just not ready. Her lungs are going to need a more time to grow due to her prematurity. Since she had to start using her lungs far before they were actually ready, they way her lungs have developed is very different from a full term or near full term baby. Months on ventilator has caused damage to the lungs that only time can heal.

So it's starting to look more and more like we won't be having surgery this year. I am glad that we have an x-ray that shows that her lungs just aren't ready to handle the massive amounts of fluid that will be required for the surgery. Her pulmonologists now feels that without a huge growth spurt the chances of Juliana coming off oxygen this summer are very slim. I really wanted to believe that it was possible but deep down I started to realize that it wasn't going to happen this year.

So off to Oley we go for the first time. The Oley Foundation is a non profit organization dedicated to educating and providing resources to IV and tube fed adults, children, their caretakers and healthcare professionals. Each year they host a conference where consumers are able to attend different informational sessions. They also have social events throughout the week. I am very excited to be able to attend this year. Not only will it be a much needed reprieve from everyday life but I look forward to learning more about how to manage Juliana's care in many ways. It will also be nice for Omar to see other children with issues that are similar to his sister's.

In other news Juliana is well on her way to walking. She is cruising on all the furniture and is getting into everything. She can make it down one step from the kitchen to the back porch and has learned how to safely slide off the couch. She is also becoming more and more vocal. She is stringing sounds together and is also able to understand more. She has become my little shadow following me around the house now which is very cute but sometimes limits what I can get done. We are very pleased with Juliana's progress.

Oral feeds with Juliana have improved. She is more tolerant of oral stimulation and the gagging has decreased. She lets me clean her mouth twice a day without too much of a fight. We have been letting her taste different table foods but she is not really interested yet. She is very good at finding the little spec of food I put in her mouth and taking it out. I am going to talk to our nutritionist at our next visit to see if there are other fluids that we can try with her. She may not drink very much but the goal is to train her to like foods and eating so that when we can give her larger volumes she will enjoy it.

She is doing very well with her hearing testing. She still shows a mild to moderate hearing loss. We are now going to move on to the next phase of testing which will involve her placing an object into a box when she hears a sound. Over the next month we will be training her how to test. Once she has learned how to test then we can bring her in and test different sounds to further evaluate her hearing. As she gets older we can more accurately test her hearing and adjust her hearing aids as needed.

Now that surgery is off the table we are looking forward to enjoying the summer with the kids. I just hope it's not too hot this summer so we can spend some time outdoors without risking dehydration.

April Clinic Visit

Yesterday we took a trip up to Boston for Juliana's clinic visit. She gained 500 grams since our last visit and is now up to 21 lbs 2oz. and in the 11th percentile for her weight according to her adjusted age. We are continuing to increase her feeds according to her stool outputs and are now going to eliminate one night of TPN. So she will receive her TPN 6 nights a week and on the 7th night we will give her a bag of hydration fluid. Some other changes to the make up of the TPN may be made depending on the result of her labs.

Her surgeon was actually at clinic this week and we were able to discuss the plans for surgery. He is very adamant that Juliana be completely off oxygen before we can even set a date for surgery. I must say while I am not completely happy with this I am very grateful that he has a sincere concern for Juliana's health. Knowing the toll this type of surgery can take on kids with normal functioning lungs, he is very weary to attempt this until she is at her very best. With ECMO being our only fail safe should she have trouble handling the massive amounts of fluids required for the surgery, I feel that going in we should all be confident that Juliana is ready. I don't think Juliana would be able to tolerate another run of ECMO. So it may be that we have to wait it out another year. Juliana may also surprise all of us and come off oxygen before the summer is out and we could get her in before the next viral season begins. I am preparing myself mentally to have this go both ways, but only time will tell.

Juliana continues to move closer and closer towards walking. We have gotten her to wear her backpack during the day now. She can do everything in it but roll which she hasn't been doing too much of anyway. She is crawling and can pull her self to stand almost anywhere. She cruises the furniture and walks around the crib with ease. I am so amazed at the progress she is making.

I had to throw in a pic of Juliana's big brother Omar playing in the sand table.

Back on Track

So we are back on track for weaning. Juliana is back to her base line for oxygen and within the next week we will try to wean her again. It's nice to put the suction machine back in the closet. She is still getting mucinex twice a day to help keep things under control.

In other news..... Juliana is officially a crawler. She is actually up on both her hands and knees. Now I have to pull the gates out of the closet. I was in the kitchen the other day washing up the dishes when I hear a little voice behind me saying "hi, hi, hi" (this is Juliana's way of getting your attention). She had crawled into the kitchen and was sitting there on the floor with the cheesiest smile ever. She is really proud of her self when she knows she has accomplished something. She is also pulling one of her brothers old tricks of pulling out all of the books and movies from the shelves and throwing them into a big pile on the floor. I forgot what toddler life was like, but it's so refreshing to see Juliana doing normal toddler things.


It's been great to see her relationship with her brother blossom. Omar is generally very loving to his sister and often likes to "help" her out. Her response is to push him away, pull his hair, or grab his face (typical little sister stuff). What's even better is that Omar just lets it happen. Occasionally he comes crying to me "Juli hit me" and then he's fine. She is still his biggest fan though and he's her best entertainment. No one can make Juliana laugh the way Omar does. They definitely have the love-hate sibling relationship mastered already.

We have a clinic visit in Boston next week and hopefully our surgeon will be there. I hope to come back with a set plan for surgery this summer. I also am curious to see how much weight she has gained on the new formula.

Weaning on Hold

So what I was afraid of has come true. After having a fabulous virus free winter we have ended it with a cold. It's quite disappointing because we have waited all winter to start weaning her oxygen and now we have to put things on hold. On the bright side she is not sick enough to require a hospital stay or make her uncomfortable. She has some extra congestion and an occasional cough.

Just hoping this doesn't delay things too much. I am still hoping that we can get her in for surgery this summer. Back to the good old wait and see game.