The Door is in Sight

Well as her pulmonologist put it today, we can see the front door and are just knocking down all the obstacles in between. She is so close and we are all shooting for Monday. We moved to the floor last Saturday and I must say that I was very impressed by the whole staff on 7-3. The nurses were very attentive and took the time to learn about Juliana and her history. The doctors came into the room and were very interested in hearing my take on the whole situation which is not usually done. In most instances the docs come in say this is what we are going to do and that is that. This usually causes anxiety for me because not everyone understands the big picture with Juliana and more importantly their plan doesn't include all of the details that I need to hear. I am often told that not every parent wants to hear all the fine details. Someone must have given them the heads up. They came in and said let us know what you want to do and it was done.



While in the PICU we met a wonderful intern who has simply amazed me. When she gets the ball she runs with it. She took the time to call Boston and coordinate labs and TPN orders. We are fortunate that she moved to 7-3 on Tuesday. She knows Juliana very well at this point and is very efficient at everything. She doesn't miss a beat and communicates very well with me which is most important.

Today we stopped the ativan and decreased the dose on the morphine. She is tolerating the weans very well and has no signs of withdrawal. We did however have an incident with one of the stitches being pulled from her line. This caused a small superficial wound that began oozing a few days ago. This invites Surgery and Infectious Disease back into the picture. The big concern is that we are running out of access options for Juliana so this line is to be protected at all costs. It's not just a central line, it's our life line. ID wants to pull it and Surgery says no way (as do I). Blood cultures were drawn from both the line and her body. So far all results are negative, but the site is still oozing. Dressing changes are ordered daily till this clears up and she is back on antibiotics. The attendings don't feel like this will prevent her from going home. We can continue to give her antibiotics at home and monitor the situation. The ultrasound and all the lab results lead us to believe that this is not a line infection nor an abcess.

We are now in the process of getting her TPN order into the pharmacy for Monday delivery. We are narrowing down the home meds and readjusting according to Juliana's needs. Also on the agenda is the hearing screening which we have attempted twice unsuccessfully. The hope is to get her hearing tested and her seen by pediatric opthamology before heading home so we won't have to do it out patient. We are all so ready to get her back home again.

On our way....

Juliana is fabulous. She is very happy and back to herself. It's hard to believe that she was just on ECMO a couple of weeks ago. She was extubated a couple of days ago and should be on nasal cannula by tomorrow. She is currently on high flow at 3 liters and if all goes well we will be moved to the floor in the next couple of days. Of course I argue that if she is well enough to go the floor then she is well enough to go home. Once she is on nasal cannula there isn't anything that we don't do or can't do at home.

So my goal for the next few days is to break Juliana out of the hospital. I have had enough of the hospital and it's not that I don't appreciate them saving Juliana's life again. It's just that she has spent more of her life in the hospital than at home. I also have big trust issues. It really takes time for me to feel comfortable with the staff at any hospital. Moving her to the floor would just mean more staff that I have to train. I think when you are the parent of a very sick kid you have a different view of the world. I am the biggest advocate for my daughter and I will protect her at all costs. Sometimes it means typing out instructions for her care and posting them over her bed, talking with the nurse managers about what I expect from the nurses and badgering the doctors to answer questions about why, what, when and what if. I just can't sit back and let them handle everything. I have to be involved in everything from the smallest change to the biggest and if something slips by me like a change in medication my anxiety goes way up. I just might go crazy if we don't get out of here soon.

While inpatient at the hospital we were able to get a few things done. She was due for her eye exam while she was on ECMO, so I wasn't quite able to bring her in for her check up. I finally was able to arrange for the retina specialist to come see her while in the hospital. So she had her exam yesterday and the traction on the right retina is gone. We are scheduling a visit with a pediatric opthamologist because she will most likely be nearsighted and will need some vision correction. The retina specialist also recommended that Juliana be fitted for a special contact that will trick the orbital bones around the left eye to continue to grow. Since she has no vision in the left eye, the eye most likely will not grow and therefore the bones around the eye will not grow. Although this is only a cosmetic issue I think if it's something we can fix we might as well. Today she should be seen by audiology again. Now that she is extubated they will be able to test her more accurately. Initially they had found that there was some high frequency hearing loss on the left but the test was inconclusive because there was not enough data to compare. I am hopeful that the initial thoughts were inaccurate. Despite everything she is happy and smiling and charming the pants off everyone who sees her. Mama is so proud of her little girl.

Pictures

Okay so I said I wasn't going to be taking any pictures but I did. I feel that it's important for Juliana to understand what she has overcome so early in life. The first picture of her looks terrible but she is much better now.

This is ECMO. It is a very big production with lots of pumps that I didn't capture in the picture.

This is Elroy. Elroy is the CVVH machine that was used to help remove fluid from her blood. All the CVVH machines are named after the Jetsons. I would have preferred Rosie so I could take her home to clean my house.

In this picture you can see the CVVH on the right and the ECMO on the left. The CVVH was connected directly to the lines of the ECMO. The oxygenator is the white tube in the middle that looks like it's about to launch and Juliana is on the warmer behind all of this.

Now here is a not so pretty picture. This is Juliana two days after going on ECMO.

These are the cannulas that were surgically placed into the jugular vein in her neck.

Now here is Juliana the day after the cannulas were removed.

and here she is a few days later smiling again. She is so amazing.

Off ECMO and lots more.....

So many things have happened since my last post. I apologize for not updating everyone much sooner but this has all been very overwhelming. She is doing much better than she was two weeks ago. While on ECMO she nearly tripled in size and had tons of fluid retention. Her kidneys were still functioning but not enough to remove all of the fluid. So we introduced a new machine to the mix called a CVVH (Continuous Veno-Venous Hemofiltration). This machine was hooked up to the ECMO circuit on the venous side (which is the blood leaving her body to be oxygenated by the ECMO machine). So before the blood would go to be oxygenated it was re-routed through the CVVH for ultra filtration (removing fluids from the blood). We were able to set how much or how little fluid was removed. In one day the machine removed 750cc of fluid. This is more than Juliana weighed at birth. It was completely amazing to see your little girl go from this huge baby that was almost unrecognizable to this shrunken baby that was even smaller than when we brought her into the hospital. Soon after all the excess fluid was removed her left lung opened up and then shortly after that her right lung began to open up as well.



Since she had lost so much fluid the endotracheal tube placed to assist in ventilation now had a huge leak. You could actually hear all the air escaping and resonating in her nose and mouth. So now we were faced with the dilemma of an inadequate tube for ventilation and Juliana was in a position that made it nearly impossible to replace the tube safely and there was no way to move her into a position that would work without the risk of the ECMO cannula becoming displaced. The only other option left was to do a bronchialoscopy to safely replace the tube. So the new larger size tube is threaded onto a long thin scope (camera) and sent down alongside of the tube already in place. This is the first time I actually saw the inside of Juliana's lungs. Overall she has a great airway and vocal chords. Her right upper lobe has been problematic and the pulmonologist was able to suction out a nice chunk of mucus. The tube was replaced and a trial run of ECMO was done later that night.



On Tuesday May 5th she was scheduled to come off ECMO and since the trial run went smoothly the night before everything went according to the plan. She had a great first night of ECMO. Her blood gases were amazing and everyone thought she would be extubated the next day. Some changes were made to her ventilator settings in an effort to wean her off for extubation. Her blood gases weren't looking so good any more and she started to require more oxygen. Another chest x-ray confirmed that there was some atelectasis (collapse) on the right side again. Yesterday she had another bronchialoscopy done to try and reopen the right upper lobe. Her pulmonolgist was able to remove some mucus from that area and it seemed pretty open but the x-ray following the bronchialoscopy still showed that the lobe had not opened up yet. The x-ray however can be deceiving because in order to clean out the lobe, saline is sent down the tube to loosen things up for suctioning. So it is still possible that the lobe opened up but we are just not able to see it yet.



She is back on her formula again after two weeks of an empty belly. We started her off very slow with only 2cc per hour and gradually increased to 6cc as of yesterday. Her belly was feeling a bit firm to me which really concerns me. I asked to the doctors to hold at 6 to see what she does before moving on to 8. She has some but not a lot of bowel sounds so I don't want to overwhelm her with too much too soon. She has gone through a lot in the last two weeks and not all of her bodily functions are up to par yet.



She is definitely starting to feel better. I was able to get a little smile out of her yesterday but it was the kind that only I can see through her eyes. She is very perceptive and extremely expressive in her eyes especially now that her sedation is being weaned. She has a little more awake time than before but still spends the bulk of her day sleeping. While on ECMO and the CVVH she was on extreme amounts of pain meds. To avoid withdrawal symptoms she is slowly being weaned to a dose that is more suitable for her weight.



I have lots more to talk about but I will save that for later. Thanks again to everyone for all the prayers and support. It's great to know how much people really love and care for Juliana.

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.