Fever!!!

After a pretty uneventful summer, Juliana decided to end it with a fever. I went in to check on Juliana and complete the 5 am diaper change duties, I noticed that she was a little warm. I took her temperature and the thermometer read 101.8. I have never been so worried about a baby with a fever as I am with Juliana. Within 20 minutes bags were packed and loaded into the car and we were off to the hospital. I drove straight past Waterbury Hospital and headed to Yale. If we needed to be admitted for any reason that's where we would end up anyway.

I arrive at Yale and go through the list of medical issues and medications. The nurse takes us right in to a room to get us settled and five minutes later our favorite resident walks in. This doctor went the extra mile for us while we were in the ICU in April and May. She took the time to contact Children's in Boston to cooridinate Juliana's nutritional needs with the protocols set by the CAIR clinic. She was able to get permission for us to give Juliana her Omegaven while inpatient even though it is still investigational and not approved by the FDA. She is very genuine and really knows Juliana's story. She even remembered how many centimeters of bowel Juliana had removed. I was so glad that I did not have to explain all of Juliana's medical history and the fact that she knows us really saved us from an admission.

We had blood culures and nasal swabs sent down to the lab. We also had some regular blood tests done to check elctrolytes and a CBC which is a "complete blood count". The CBC checks white cell counts, hemoglobin, hematocrit and others. I am always concerned about her electrolytes. Her sodium levels have been known to get dangerously high without any other real signs of dehyrdation. Fortunately all of these test came back normal. Her pulomonogist was contacted and she felt that we could ride this one out at home so once the nasal swabs came back negative for any major respiratory virsuses or flus we were able to head home. She was also given a 24 hour dose of iv antibiotics. If the cultures had started to grow anything we would have had to come back to the hospital for more antibiotics and cultures. I waited by my phone for the rest of the weekend hoping it wouldn't ring and it didn't. It's now been more than 48 hours so it's safe to say that Juliana does not have a line infection.

I have come to the realization that I will never get over the emotional trauma from having to put Juliana on ECMO. As I have expressed many times before I am very scared of what this winter will bring. So I have decided that the best way to deal with this is to be completely proactive. This may mean that we have Thanksgiving dinner at home with no visitors or that we will have to save our Christmas celebrations till the spring. It also may mean that any visitors we do have will be sprayed down with disinfectant at the door and asked to wear gloves, gowns and masks. It may not come to those extremes but we will definitely be making all of the necessary extra precautions to make sure we can keep our family as healthy as possible for Juliana's sake.

We have already begun working on getting our Synagis shots approved for the winter and both Juliana and Omar Jr. will get their flu shots in couple of weeks. Omar and I have plans to get ours shots next week. Since the onset of "swine flu" the schools have become very diligent about disinfecting common areas which makes me happy. Worrying about what will or can happen doesn't really help any, but being prepared for what will or can happen will help in a big way.

Through all of this Juliana still remains joyful and playful. She doesn't seem to know that she is sick and I am not going to tell her. Even with a second tooth pushing up she continues to laugh and play and light up my world.

Break Through

Juliana finally broke skin this weekend. She is surprisingly in good spirits and not very cranky at all. We are also scheduled to get our new hearing aids next week. I can't wait to see the look on Juliana's face when she can actually hear sounds like before. Unfortunately when we went to see th ENT for medical clearance we found that she had fluid in her ears again. Nothing to really worry about just yet but if this becomes a chronic issue and/or the fluid doesn't clear up we may have a discussion about tubes.

It's been very nice to not have any real news to report. Hopefully we can keep it this way. I'll try to get some pics up of the new tooth.

The Waiting Game

We are back from our trip to Boston with no distressing phone calls on the drive home. The visit was pretty uneventful and probably one of the shortest times spent in clinic. Now begins the waiting game. As the respiratory virus season approaches, we know for sure that Juliana will not be having surgery. Since she is still on oxygen her surgeon has decided against reanostomosis till at least after the winter. I already knew that plans for surgery were heading in this direction. Our next clinic visit is in two months which is the longest we will have stayed away from a hospital since Juliana's birth.

Our goals still remain the same for now, stay out of the hospital, continue to grow and avoid infection at all costs.

Pictures!!!

Off the O2 for a moment... she pulled her tube so Dad took the opportunity to get a pic with a tube/ tape free face

Juliana has to have her own spoon when she eats or we end up fighting. There's no food on this spoon but it's still great for chewing on.

Eating is hard work....

She is actually enjoying sitting in the Bumbo.

She loves her music player... also great to chew on.

Short Gut at Home

Learning to live with short gut at home has been quite an adventure. I am happy to say that after six months at home I have become pretty efficient at handling supplies and routines. It did take some time to get used to everything. Juliana is on oxygen and also receives TPN/ Omegaven through a Brovaic line. She also has an NG tube for feeding and two ostomies for pooping.

Organization is the one tool that makes all of this possible at home. We have tons of supplies for everything related to Juliana. I have a dressor set up in the living room with all of Juliana's medical supplies. Keeping everything in one place helps me to stay on top of all our supply needs.

I set up daily bags of the basic supplies we need each day to set up her TPN/ Omegaven. I have found this very helpful for get things set up in a hurry. I can get TPN/Omegaven setup and hooked up in about 15 minutes.

Set up consists of adding the vitamins to the TPN, mix well and then priming (replacing the air in the tube with TPN) the tubing attached.

We then spike the Omegaven and prime the tubing for this as well. After setting up the "Y" extension and priming with saline we connect it all together and we are now ready to hook her up.

Since Omegaven comes in a nice glass bottle not really made for travel, we put Juliana to bed first and hook everything up upstairs. Typically she stays in her crib till the Omegaven is done and then we bring her back downstairs for the day. Her feeding pump and TPN pump fit nicely into on backpack. We use the bag sent for use with the feeding pump. The rear compartment is meant to hold larger feeding bags, but we use it for TPN. Having both in one bag makes travel in and out of the house much easier.

This is the feeding pump in the front compartment and her TPN and pump in the rear.

Since Juliana has become very interested in all her tubing we have to hide everything from her. The blanket trick has been working pretty well. We keep everything under the blanket and then tucked behind another blanket rolled up along the side of the crib. By the way this is Juliana about two minutes after being put in her crib (if only it was this easy to put her big brother to bed)

She is also on oxygen 24/7. We have on big liquid O2 tank in the living room and we use a 50' extender. With the extra length she can go any where in the house including the outside porch. We have two monitors, one for upstairs and one for downstairs. The cable is long enough that I can have one on my night stand at night. The alarm will wake us up and not the kids. When traveling we have refillable portable tanks, but I also make sure to keep a back up cylinder in the car. Since we travel pretty far from home at least once a month, I like to have a security blanket with me.

Over time I have discovered that being prepared is number one when it comes to ostomy care at home and on the go. I try to always make sure that we have the supplies we need ready to go at a moments notice (sometimes all you get is a moment). Right now ostomy bag changes are a daily event and we are still trying out different bags to see which will give us the most wear time. I have been successful with minimizing the amount of time it takes to change an ostomy bag. I keep a few sets of bags and barrier rings cut and ready to stick for a quick change at all times. This has become very important now that Juliana is maturing and moving all over the place. If it takes too long to change her bag Juliana will surely make a poopy mess all over the place and with a Broviac line that is prone to infection that is the last thing I want to happen.

We do have a lot on our plate when it comes to Juliana and I have to remind myself of that sometimes. Not every day is perfect but we do the best we can to make the most of it.

Tomorrow we head up to Boston for our monthly clinic. My fingers are crossed that all will go well. Since surgery is tentatively postpone till at least next spring, our goal from now until then is to stay hospital free. So many prayers will be needed.

No News is Good News

So the past few weeks have been pretty uneventful. We went to our clinic visit in Boston yesterday and of course my anxiety level was up. It's so hard to know how those labs are going to turn out. It's usually when everything seems pretty normal that we end up with an abnormal result. We left the clinic with the understanding that if her electrolytes were out of range they would call me on my cell and we would take her to Yale.

Well no news is good news and everything was in normal range. We made it home last night without a phone call. They did however call me this morning to discuss some changes. Juliana's lung disease causes the doctors to be a little nervous about increasing the volume on her TPN. If she gets too much fluid it can overload her lungs, but if she doesn't get enough fluid she will become dehydrated. Her urine output has been on the low side lately and she has gained a bit of weight this month (yeah!), so they have decided to increase the volume again. She will have labs drawn again in two weeks to see how it all goes and of course we will be watching her very carefully as usual. Next week we will also see her pulmonologist which will be just in time with all of the changes.

If she tolerates the volume increase we will be able to cut back to 18 hours of TPN down from 19 hours. I am hoping that by the end of the year she will be on TPN for 12 hours. Now that she is moving around it would be nice to have one less thing for her to get tangled up in. If she can get off oxygen by the end of the year as well we would only have the feeding tube in the daytime. Right now she has so much I couldn't even imagine how tangled she would get if she were crawling and cruising. When her therapist comes for her exercises I try to eliminate as much as I can (like unpluging the monitor and stopping her feeds) and we still end up tangled.

She is still working on her rolling. She manages to roll on to her belly but gets really frustrated. She isn't holding her head up while on her belly. I think that once she is able to support her weight on her arms and upper body that the belly to back roll will come easily. Juliana has also taken an interest in the little stuffies she's been collecting since birth. She especially likes her Zoe that she tosses around. If you hold Zoe up and make her dance Juliana starts to laugh out loud. It's the greatest sound in the world.

All in all things are going well on the health front. We are still recovering from Omar's layoff last month but he's back at work now and we are making the best of it. I did enjoy spending weekday mornings in the office over spending late afternoons and Saturdays in the office, but we do what we have to do to make it work. We are blessed to have employment at all in the current economy so I take none of it for granted.

Hopefully life will continue to be uneventful (at least until our next planned surgery).

Happy Birthday!

We made one year and what a long journey it has been. These last few days have been bitter sweet as I remember the events of this past year and how it all started. Fortunately I can focus on the sweet part and have finished beating myself up over the why.

Juliana had a quiet birthday at home. Her brother just had his birthday party the week before and that was enough excitment for all of us. She is now rolling from back to belly but gets very upset because she can't roll belly to back yet. It's pretty funny to watch her do a full roll and then start screaming and kicking her legs up. We have been showing her how to roll back to her back but she just gets too upset. She also has a new interest in pulling on her line (not good at all). I knew this day would come so we are taking many precautions to hide the line to prevent her from pulling it out. It's just really hard to hide and keep it away from the diaper area to avoid infection. I made a sleeve that velcros around the line to keep it out of her site. Hopefull this will deter her for a while.

I think we may be getting teeth soon or at least I am hoping. She has taken a disinterest in her bottle feeds which isn't the end of the world because I have two other ways to give her nutrition (NG tube and her central line). It is however frustrating because ultimately our goal is to have her get all of her nutrition by mouth. I was always so thankful that Juliana didn't have any oral aversions from her lengthy intubations. I am hoping that the reason for this sudden disinterest is that she teething and that she will soon get back to her old bottle gulping self. She is however taking her cereal just fine. She is only taking 1/2 a tsp with formula but the belly seems to like this so we will stay here for a while.

I am not sure if I shared the news of Juliana's hearing loss. It was a bit hard for me to swallow. I am not really sure why because hearing loss isn't nearly as life threatening as chronic lung disease or short bowel syndrome. Juliana has profound hearing loss in her left ear and severe loss in her right ear. At least I can stop telling little Omar to turn the volume down when he is playing his keyboard. She is scheduled to be fitted with an amplification device that will basically turn up the volume for her. She can hear noises that are classified as extremely loud but can't hear noises that are very loud like the vacuum cleaner, alarm clock, or heavy traffic. So this also means that my family will have to become tri-lingual and learn sign language as well as spanish.

Overall things have been pretty normal for us which is more than I could ever ask for. There are some things on our list of summer activities that we have to be very careful about like going to the park or the beach or taking a walk. The weather has been warming up which makes it a little harder to bring Juliana out. She is really crabby when she's hot and we also run the risk of dehydration. I have been in search of a baby sun tent so that we may be able to spend the day outside and still keep her comfy out of the sun. Fortunately it doesn't seem like it's going to get too hot this summer.

I have some videos and pics of Juliana rolling and laughing that I will try to get up soon. It was pretty hard to get her on camera because as soon as I pull it out she stops what she is doing to look at the camera. She is turning into such a character and we are so blessed to have her in our lives.