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.

Still Working on the Wean

So tomorrow will be the end of week 2 of our wean schedule. Everything seems to be going well. She has tolerated 1/2 liter for the last two weeks just fine. I am not totally convinced that a dropping down to 1/4 liter is going to go as well. The only way to find out is to try it.

We have also met our goal with the TPN schedule. When we first brought Juliana home we only had three hours off a day from the TPN pump. So she was infusing for 21 hours a day. Now just over a year later we are down to 12 hours a day. Now that she is older this is especially important. She is able to move around a bit more without having an extra pump to drag around. It also decreases the amount of times per day that I have to access her line. We hook both the TPN and the omegaven up at night before bed time and instead of disconnecting the omegaven first and then the TPN hours later, we can disconnect all at once. I try to schedule our in home labs to coincide with our disconnect time in the morning. The risk of infection increases each time the line is accessed.

Feeding therapy is coming along very slowly but we are noticing some progress. She continues to fight off anyone or anything that comes near her face. Recently she hasn't been fight as much as before. It is very small progress but definitely an improvement. She allows finger play in the mouth more than she will with spoons or the NUK brush. I have stopped focusing on the amount of food we can get into her orally. If we can get her to at least tolerate any type of mouth stimulation we know that this will help her more in the future. The stimulation will aid in her speech progression and help her to learn how to control the food. She still does a lot of gagging when it comes to actually swallow food. Part of this in my opinion is due to the NG-tube (Nasal Gastric tube runs up the nose and then down to the stomach for feeding). At this point we have no other choice. The only way to get rid of it is to have the surgery for the reanostomosis and at that point the surgeon will put in a G-tube. The G-tube is a gastric tube used for feeding that is inserted through an incision direct to the stomach.

As the days go on I get more and more anxious. I just want to have the surgery in the past. I am more than ready to start moving forward towards getting Juliana off the TPN. Whether or not she will actually get off TPN is not a guarantee, but we could at least explore this possibility. What I dream of even more is to exit the ostomy world. Everyday the challenge of caring for Juliana's ostomies is increased. She is much more active and keeping bags on her for more than a day is near impossible. Couple this with the fact that we are only allowed enough supplies for one bag change a day and we find ourselves in a very stressful situation.

So as much as I dread having to go back to living in a hospital, I know that this upcoming surgery means some very big changes in our lives and for the better. For now we just continue to work on weaning down the oxygen to get her ready for surgery. Praying for no surprises in the upcoming months.

Finally a tooth shot!!!! The first one is not the greatest but I did the best that I could. She has four on the top and another four on the bottom.

Let the Weaning Begin!

It's finally March and we have the "okay" from the pulmonologist to start weaning Juliana off the oxygen. We are hoping to convince the surgeons to go ahead with the surgery even if she is still on oxygen. I am so glad that her pulmonologist feels the same way I do. It is pretty unlikely that Juliana will be completely off by May or June but it is more likely that she will be on a very low flow at night only. She is way over due for this surgery and we both would like to see it done as soon as possible. I understand the surgeon's desire to wait till she is completely off but another year of ostomy's and the ng-tube is not ideal.

I am very excited to see what her belly is capable of when it's all back together. It's very hopeful that she will be able to get all of her nutrition enterally and with some time she could even come off the TPN. The central line (Broviac) puts her at a high risk for infection and other complications like clots and air embolisms. If she can come off the TPN we can pull the line and dramatically reduce the risks. We have been very fortunate to be infection free for the last 10 months.

This will also open the door up for more babysitter options. Currently we only have one babysitter qualified to care for Juliana without my husband or myself present. It really limits the things that we can do like working or having child free time together. Fortunately our one babysitter (my Mom) has been really great and will even spend the night when we need her.

So the plan is to turn her down to 1/2 a liter for two weeks. If all goes well we are then going to drop her down to a 1/4 liter for another two weeks and then we can try shutting it off completely for two hours at a time. Juliana typically doesn't like to follow plans and usually does things her way, so we will just have to wait and see if it works.

We have also changed her formula to Peptamen Jr. It offers more calories and she actually tolerated the trial run. The best part is that is comes ready to serve in a can which means no more mixing, no more powder and no more bottles to wash. What is nice for Juliana is that it comes in flavors. I am hoping that this will help with feeding. It tastes much better than the neocate which may be a good incentive for drinking from the cup. Of course that is if I can get the cup anywhere near her first (still a work in progress).

It so wonderful to think about where we've been and how far we've come since that sunny afternoon when my water broke and my whole life changed. Many people may think how unfair that she's had to go through so much in her young life but I say how blessed we are to have made it through.

Pictures!!!

Juliana loves to make a mess and throw her toys around....

She is just tickled with herself when she is standing....

Say cheese and she really does it.....

Juliana with her big brother...

Amazing

Well it's been way too long since I have updated everyone on Juliana. Things have been going so well I am almost afraid to write this. She is just so amazing. We have gone from an independent sitter to trying to walk very quickly. She has no interest in actually crawling. She goes up on the hands and knees and the straight to the feet like a bear crawl. She is just tickled if you stand her upright completely and let her cruise the coach. She is also waving "hi" and "bye" and at times it sounds like she is actually saying "hi".



We went to her 15 month (adjusted age) check up last week and she is almost 20lbs. I just can't believe it. She is still not on the charts yet for height and weight but is in the 50th for her head. Feeding therapy is going a little better. She will drink from a cup but only with tons of coaxing. She is also handling her baby foods and cereal alot better. We had lots of gagging before and now just a little here and there. Some days of course are better than others.



Surgery is still planned for this summer and we will start weaning her Oxygen next month. The hope is to have her off oxygen before surgery. Her surgeon probably won't even think about operating on her til she is completely off. I am looking forward to this surgery but am very worried how Juliana is going to handle being back in the hospital now that she is older and a lot more aware.



For now we are just enjoying our time at home with her and praying we can get through the last part of winter hospital free. We have done really well so far. I had a really yucky head cold for about a week but managed to keep it from spreading to the rest of the family. Lots of hand sanitizer, disinfectant spray, and masks.