Can't Complain

Juliana has been great. I am afraid to even say it but it's been 3 weeks off oxygen and we are cold and virus free in our house. I actually brought Juliana out to play in the snow on Monday which she wasn't too impressed by. It was still nice to be able to bundle her up and take her out without any strings attached.

The kids were very pleased with their gifts this christmas. At first rough and tough Juliana wasn't into her new baby dolls but gradually she has been playing with them more and more. She likes to carry them by their leg and toss them into the shopping cart head first. She still prefers playing with her brothers cars, dinosaurs and footballs but Omar is okay with as long as she shares her dolls with him. A baby doll just happened to be on his wish list this year anyway.

Juliana is getting the last of her teeth in which seem to be the worst of them all. She can be very cranky at times which is usually healed by a little motrin. She is still eating well by mouth 3-5 times a day now without much resistance. I have been really focused on trying to get everyone to treat her as a regular kid including her therapists. Although her gut doesn't function the way a regular childs gut would she still behaves the way I would expect any other 2 year old to and refusing to eat is not very uncommon. So now that we know she is physically able to tolerate milk and baby food by mouth without aspirating or gagging there is no excuse for her not to eat. Usually if you let her know your not going to back down she will comply and at times has found that she actually enjoyed the taste of what she was given.

Lately her form of control has been the feeding tube. She likes to pull this out a few times a night (bedtime usually). We believe this is her protesting against bedtime. We have caught her trying to put it back in herself and on one occasion I supervised her as she threaded the tube up her nose and she almost got it half way down. Her fine motor control is not quite perfected yet so I had to step in and finish the job. I think this is when you know that you've had an NG-tube for way to long when the 2 year old can put it in on her own.

We are very hopeful that Juliana will be able to start school in the fall without her backpack. It's hard to imagine my little peanut going to school but really it's the other kids I worry about. Juliana is very tough and doesn't mind pushing people around if they get in her way (just ask lil Omar). If all goes well with surgery I anticipate that she should also be able to come off TPN and IV fluids within the year. Of course only time will tell.

We look forward to taking on 2011 and all of it's scheduled and unscheduled events. It's going to be a pretty busy year with Juliana having surgery this spring, preparing for the start of preschool in the fall and a vacation wedding to attend. I am still hoping to squeeze in a trip to see my Gramma. It's been a long time since I have been out to see her and I would love for her to meet her great grandchildren in person.

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.

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.

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.