We Have a Date!

Juliana did well on our journey to Boston. This was the first in a long time that it was just the two of us. First stop was clinic. She gained a little weight but we are still increasing her PN because she needs to gain even more weight. She is not even on the charts for weight but hoping this will change after surgery. Next stop was radiology for her barium study. Juliana did very well being patient and only started to get fussy at the very end. She was really ready for a nap so I was very happy to see how cooperative she was. She did get her much need nap during the drive which was all very uneventful. In four weeks we will go back and look at the rest of her intestines and pray for a bigger weight gain.

We got a date for surgery and although it wasn't exactly the week we wanted it is in the month we had hoped for. She is scheduled to be admitted on Sunday April 24th and will have surgery on the following Monday. With our hectic lives this means that I will be going up alone for the first couple of days but my husband is not very good at waiting so it's probably for the best. The surgeon says to plan for about a 2 week stay but we will plan for more so we are not too disappointed if it ends up being a little longer.

I have been waiting for 2 and a half years to get this date. Having it though doesn't make me feel any better. It's so much easier to go into emergency surgery than to know months in advanced. Now I have two months to sit and ponder all the possible outcomes for this big day and trust me I have a wild imagination. I always find myself anticipating the worst of the worst scenarios with the hope that if reality is even slightly better I can look at the glass half full.

It will be interesting to see how Juliana handles all of this. Her last hospital stay she wasn't even crawling. Now we have a fiesty 2 year old who likes things her way so I can only imagine. She is for the most part very cooperative medically speaking. I can do dressing changes which need to be done under sterile conditions without the assistance of another adult now. She knows to keep her arms and chin up and will remain still while I sterilize the site and apply the new dressing. There are many things to keep her entertained while we are inpatient so I am not terribly concerned. The biggest plus is that they have an amazing coffee shop in the hospital and a Starbucks right across theh street!

For now we hope everything will remain status quo.

The Countdown Begins

We are less than 2 months away from surgery and everything is still running according to plan in the Delgado House. Of course with Juliana that can change. It's very strange to be getting this close to surgery after waiting for over 2 years. We are finally getting rid of the big liquid oxygen tank which has now become a coat rack for little Omar (it's the perfect height for him). I was a bit nervous about having it picked up but we are keeping a couple of cylinders in the house in case of an emergency. It's been 2 months since she has been completely off oxygen so I think its time.


Juliana is also getting ready for school which is scarier for me to think of than surgery (believe it or not). I just can't imagine Juliana with other kids that are probably going to be twice her size since she is not even on the growth charts. She only weighs a lil over 20 lbs and is still rear facing in her car seat. She's a tough girl though and I am sure her toughness will more than make up for her size! Along with getting ready for school comes the evaluation of her cognitive, social, fine and gross motor and communication skills. She is delayed in all areas except her social skills which is exactly what I had expected the test to say. She is moderately delayed in her cognitive and motor skills, but her communication is severely delayed which I believe has a lot to do with her hearing loss. She is able to communicate her needs to us in her own way, but others who may not know her as well are not able to understand her needs or wants. With time and lots of work she will certainly overcome this delay.

We did have one incident this month that landed us in the ER. Juliana thought it would be fun to twist the IV pole round and round and round til she pulled the end of her Broviac (semi permanent IV) right off. We woke up to find her giggling in her crib at what she had done. We of course did not find this funny at all. In my semi frantic state I was able to syringe some heparin into the line to prevent it from clotting up. This was a difficult task because unlike our previous line breaks she had completely removed the end which left a tiny little opening at the end that was barely big enough for us to get the needle point on the syringe into and do it as sterile as possible. We clamped it and sterlized the line and surrounding area and covered it with a large tegaderm dressing to keep the clamp in place and the line protocted. The good out of all of this was that after almost a full day spent in the ER we were able to get the chest xray we needed to check the placement of her line before going into surgery. It was on the list of things to get done but we had not anticipated having to do it this way.

Fortunately all is well and the line was repaired. As we were driving home from the hospital we did get a funny phone call from a pediatric surgeon's office letting us know that they scheduled Juliana for a pre-op appointment to replace her line. For those of you who are familar with CVL's and the limited places where these can be placed you know that pulling a line that is functioning is just not something you do without cause. Certainly having a line that has needed to be repaired a few times is not a justified reason to pull a line in a child who has very limited access and has waited over 2 years to have a major surgery due to her chronic lung disease. So we kindly objected to having Juliana intubated for an unnecessary procedure further adding to the risk of having a very necessary procedure postponed. Intubating is the process in which and tube is inserted into the lungs to assist in breathing which is often necessary in younger patients that are going under anesthesia. Intubating can also introduce unwelcome bacteria in the lungs which can cause bacterial pneumonia. We have gone through this for another procedure to repair her retina which didn't work and ultimately left us back in the ICU and extended our hospital stay by another month. After all that we have been through with Juliana we got a real good laugh at the thought of a doctor scheduling a procedure for a patient without even looking into her medical history or consulting her current doctors who are managing the care of her CVL. Any child or adult with a CVL obviously has some health issues and is should be under the care of a doctor or team of doctors.

This week is going to be exciting in that we finally are going to get a more recent picture of how Juliana's intestines are looking. She is going to get a barium study done on her upper and lower GI tract which hasn't been done in a couple of years. The upper GI is done with a Barium Swallow which consists of Juliana swallowing a barium sulfate that is visable to xrays. She will also get a Barium enema to help us see the lower GI at the end of the month. We are less than 2 months away from surgery and I couldn't be more excited, nervous, anxious, and scared! Juliana hasn't had a lengthy hospital stay in a long while so that is going to be a difficult thing to deal with now that she is a stubborn 2 year old. It's hard to believe that I may be able to feed Juliana more than just a teaspoon of baby food and 1/2 an ounce of milk after this surgery is done. It's going to be hard to be separated as a family again but well worth it if we can get rid of the ostomies and more importantly get her off TPN.

For now we just wait and pray that we can continue to keep everyone healthy until then.

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.