Not getting any worse....

So the good news of the day is that Juliana is not getting any worse. The swelling is getting better but she still has a lot more peeing to do. She is off the high blood pressure meds and is now on Dopamine for low blood pressure. She is oozing out a lot of blood from her cannula site and there is not much to do about that. The only thing we can do is to keep replacing the lost blood with more packed red blood cells. To date we have 48 hours with no growth from the blood cultures. This doesn't necessarily mean that the bacteria is gone but it is at least surpressed by the antibiotics. Her pulomonogist came by today and heard a couple of deep breath sounds. She is still no where near ready to come off the machine but breath sounds means that we may be on our way to healing now. I just hope that she can stay on the machine long enough to heal before her brain starts to bleed.

I am happy with her progress but there are still many concerns. The blood loss really worries me but I know that she won't bleed to death because we are able to just replace whatever blood she has lost. Unfortunately she can't be moved and has to lay in the pool of blood that is forming underneath her. The nurses and I are doing everything we can to keep her clean without moving her but it is very hard.

She has a great team of doctors attending her right now but it is very difficult to keep a close eye on her when they are 7th floor and we are on the 4th floor. Hopefully her lungs will make a speedy recovery and we can get off the ECMO within the next week and back to the 7th floor. Nights have been hard without her home. I still wake up in the middle of the night to check on her and she is not there. At least when we go back to the PICU I can stay with her at the hospital.

ECMO

So here we are back at Yale in the worst condition I have ever seen Juliana. She is on ECMO (extracorporeal membrane oxygenation) which is a process of oxygenating blood outside of the body. She has swelled up quite a bit and is almost unrecognizable. Her head is very big because when they went in to cannulate her veins there was a bit of trouble. The preferable location to cannulate would be the main vein and artery on the right side of the neck. This is the same location that her Central Broviac line was placed and unfortunately the vein on that side has some stenosis or narrowing. This makes this vein not a eligible for cannulation for the ECMO. So next they went to the left side of the neck. The surgeon was successful at placing to cannulas on this side. The down side is that the two major veins that drain blood from the head and brain are now either occupied or too damaged to function. So the smaller veins in the neck will have to learn to pick up the slack so to speak. This however is going to take lots of time. There are some major risks associated with this issue. The first which is a major risk with ECMO is bleeding in the brain because of all of the heparin that is used to prevent clots in the blod running through the machine and the cannulas. The second is clotting. With a lot of blood settling in the brain because there aren't enough large exits, we face the issue of the blood that is there waiting will form clots. So our very creative surgeon decided to place a third cannula to act as a large vein to allow blood to exit from the head. This was successfully placed yesterday with no complication. Unfortunately there is already a lot of swelling in her head and this will not change that. It will however prevent this from getting any worse. Over time the swelling should go down but it will take lots of time. We anticipate that she will be on the ECMO machine for at 2-3 weeks. The goal is to give her lungs ample time to recover and then slowly wean her support from the machine.

I wont be posting any pictures. She has double in size and it's not pretty at all. Somehow I am able to look past everything that is going on with her but I know for others it's just not the same. My husband is still having a hard time seeing her this way. She is back at in the Newborn Special Care Unit at Yale so we know a lot of people there and they all remember Juliana. It's nice to be surrounded by so many people who know and love Juliana. They have seen her through the toughest moments in her life. I guess it's only fitting that we would be back in the same unit. Once she is off the machine however she will return to the PICU. Under normal circumstances she would not be allowed back in to NBSCU but because there is another baby already on another ECMO machine we have to be on the same floor. This is mostly because of logistics. ECMO is a pretty big production and requires a perfusionist to be at the bedside 24/7. She is being monitored by the doctors of the PICU but some of her old doctors are still chiming in on the situation. They all really work well together which gives me great hope. I feel like I have been the coach and the moral booster for the team. Through all the concerns and fears the doctors have I still feel in my heart the Juliana will pull through this. I know what she is capable of and that she has beaten all the odds time and time again. So when they say that there is a 50% chance that she won't come off the machine and will not survive, I see that glass half full. There is a 50% chance that she will come off the machine and survive. She is no ordinary kid and I spend a lot of time reminding people that she is as strong as strong gets. She goes down quick but always gets back up. She will get through this.

Back in the ICU

Juliana is back in the hospital. This is the sickest I have seen her in a very long time. I never thought that I would hear someone say to me that she might not make it again but here we are. She has an infection from what we can tell thus far. The infection is causing her other organs to fail. She is intubated again and back on the high frequency oscillator with nitric oxide. It is set to very high settings and if this doesn't work we may end up going on ECMO (heart lung machine). Everytime she wakes up her sats drop and her heart stops so they are keeping her sedated and paralyzed. She doesn't look god at all. I really wish I had something good to say but i don't. I will try to post updates on her condition as much as possible. Please pray for my Juliana. She had come so far to lose her now.

A Holiday at Home and a Little Clarification

Finally Juliana will be spending a holiday away from the hospital. Every holiday since she was born has been spent in the hospital, so when we went to clinic yesterday I was almost positive that she would be admitted again. Juliana didn't sleep the whole way up to Boston so I knew she would be so cranky and she was up until the moment that her one of her doctors came down to the cafeteria to tell us that her labs came back normal and we could go home. I put her down in the stroller so that I could eat and she was just grinning from ear to ear. It was like night and day. I know she really doesn't understand what is going on but I think the stress that was lifted off my shoulders must have signalled something to her. She went to bed last night and woke up this morning with that same happy smiling face.

It will still be a couple of months till she is ready for surgery. She really needs to get over her respiratory illness and hopefully off oxygen before she will be ready for surgery. She is continuing to grow but at her own pace of course. The doctors would really like to see a boost in her weight gain. We really don't want to get her too far behind so once again we are going to increase the calories on the formula and the concentration on the TPN. I hope this works. It's really hard to tell people how old Juliana really is when she wears newborn clothes. For her actual age she should be almost twice her size in weight and for her adjusted age she is about 5 pounds shy of the average. Regardless of what the charts say though we are just happy she is growing. I am very positive that after her surgery she will start packing on the pounds.

For those of you who may not really understand, Juliana lost quite a bit of her intestines (about 22cm) 7 months ago. She had a disease where some of the tissues in her intestine had become necrotized (or dead). These tissues had to be removed and she was left with about 20-30 cm of intestines. When we eat we use the whole intestinal system to process foods and absorb the nutrients we need to live and grow. Juliana's intestine we left in three pieces. She uses a very small portion of her intestines to process her formula and the rest is unused at this time. She receives a very elemental formula which is what they call predigested. It is amino acid based so the stomach doesn't have to work as hard to break it down. Even with this predigested formula she is still only able to take a tiny amount at a time.

She has two ostomies, a jejunostomy and an ileostomy. Each ostomy is a part of the intestine where and opening was created and brought to the surface of the skin. We use an ostomy bag to collect waste from these openings. She doesn't poop from her rectum. When she has her surgery the ostomies will be reversed (reanostomsis). Everything will be put back together and she will have one continuous digestive tract. She will also get a G tube for feeding which is a tube that is placed into the stomach through an incision in the abdomen. We are very happy about that because she won't need her NG tube anymore. The NG tube is the yellow tube you see going up her nose. This tube goes up her nose and then down into her stomach. She loves to pull this tube out which means I get to fight with her to get it back in and make sure it's actually in her stomach. With the g tube we can avoid those battles and see her pretty face without all the tape.

I took some pictures so you can really see what she has going on. When she first had her surgery it was all very overwhelming but now it's pretty normal for us. I have to remember though that it's not normal to everyone else. I hope this makes things a little clearer.

We put protective barrier shields around the stomas and then stick the bags on. The one on the right is prolapsed and looks larger but the one on the left puts out the most stool. I ocassionally take pictures to monitor the conditions of her stomas and the skin around it. She has a little bit of skin breakdown on the right side of her left stoma which you can see above.

Here are some more pics just of Juli and her brother Omar hanging around the house.

Juliana with her cousins.

Another Visit to the Hospital

As much as I wanted to go back to Yale to visit all of our friends, I really didn't want to do it this way. Juliana started off with what seemed to be just a slight cold. She was a bit congested for a few days but her lungs sounded clear, her work of breathing hadn't changed and her oxygen requirement remained the same. Sunday morning she her oxygen sats began to dip into the high eighties low nineties so we turned her oxygen up a bit. It was not uncommon for Juli to require a little bit more oxygen with a cold. Well a little bit suddenly turned into a lot more. We were able to keep her saturation levels in the 90's but her oxygen level went from her normal 1/8 of a liter to 2 1/2 liters. Her work of breathing began to increase and she was just irritable. All I could think was RSV and in the hospital for weeks. It's like I have PTSD. The moment I think of going back to the hospital my eyes well up. Despite my aversion to staying in the hospital I knew that if I didn't bring her in things would only get worse.

So we brought her into to the ER and they took several chest x-rays. The first thought after looking at the x-rays was pneumonia but fortunately after a second look it was confirmed that she had a slight collapse in her right lung. A collapse is much easier to get over than pneumonia although still not fun at all. She also was positive for para flu which is a respiratory virus. We spent two nights in the hospital and we were on our way home. She is still sick but we can treat her symptoms at home and it is much more comfortable for the both of us. After this I am sure that we will not be having surgery this month or next month either. Fortunately we can wait

Everyone was surprised to see how great Juliana looked since she was still on TPN. I blame it all on the omegaven. If it weren't for omegaven we would be shopping for a liver right now with a very sick looking yellow little girl. We are so grateful for Children's Hospital Boston and everyone on Juliana's care team. They really go out of their way to look out for her best interest.