SO MANY THINGS HAPPENED SO FAST!

All good things, but it’s literally hard to keep up! Norah is doing awesome and her shiny new liver is working beautifully! We are so so so so so so so thankful and happy that this transplant happened and is giving her a new chance at life!

Anyway, so last Wednesday, Oct 14, they started Norah on breastmilk through an NG (nasal) tube on a continuous drip. It was started very, very slowly at 5mL per hour just to make sure she tolerated food in her stomach vs. IV only nutrition. Then the next day increased to 10 mL per hour continuously, then on Thursday, Oct 16, they “clumped” her feeds so that she got 30 mL every 3 hours and let me breastfeed up to 3 times a day for only 5 minutes each, but I was SOOOOO happy to do even that! It had been 19 days since she had nursed and I was getting sick of pumping and longed to hold Norah against me to feed her, even if only 5 minutes twice that day! Friday they started to increase her feeds to 40 mL every 3 hours. Saturday the doctors decided to take a big leap and gradually increase her feeds to meet her goal of 75 mL, or 2.5 oz every 3 hours. Unfortunately they did this simultaneously while increasing my breastfeeding time to 10 minutes 3 times a day. It was a little too much of a good thing, because after nursing and getting a NG feed of 45 mL, she threw up pretty much everything she had just taken in. Then, her doctors decided to stretch out each of the NG feeds so that the pump provided her 55 mL over an hour and then I was allowed to feed her no more than 10 minutes 3 times a day. Saturday was also the day that we got moved, somewhat suddenly, to the regular pediatric floor. The charge nurse for the floor came over around noon and by 4pm we were unpacking all of our belongings to settle into our new room (with a bathroom and a couch that pulls out – HALLELUJAH!) on the floor.

Both Jason and I were a little shocked at the move, but also thankful, because that means we were no longer considered as “critical” now. It’s crazy how far we came in a week… from getting extubated one Saturday to being transferred to the regular peds floor the next Saturday!

I was worried that my last post was a bit premature to be celebrating her extubation and sure enough, the next morning (hours after I wrote my entry on this site) the resident doctor called me with bad news about Norah’s breathing. At 6:30am I got the call that her breathing had worsened and she had become inconsolable overnight and would most likely have to be re-intubated. My heart sunk to the floor and I immediately welled up with tears. Simultaneously, I got dressed as fast as I possibly could and literally ran back to the hospital – arriving at 6:47am. I climbed into bed next to her and cried as I pleaded with her to just cough so that she could keep breathing on the high flow oxygen we had switched her to not 24 hours earlier. I prayed, cried and begged but within minutes of my arrival, the attending doctor came in, saw her, heard her, and said, “This child needs to be re-intubated. There is no doubt. Let’s get the supplies NOW.” I felt so, so, so defeated! She had been doing so well all day and then something seemed to have happened during the early morning hours and suddenly she sounded awful, struggling with each breath. It killed me and made me feel incredibly selfish and guilty that I had chosen to go home that night and that was when the problems started.

Even now I can’t help but wonder if she would have been able to settle, would have been able to continue breathing comfortably and easily on the nasal cannula high flow oxygen had I been there to comfort her and cheer her on when she started to get upset. I know I can’t go back in time, but such a huge part of me felt responsible for her need to be re-intubated and to put her trachea and vocal cords at risk again. Anyway, to say the least, I have been staying in the hospital at night now for about a week. If it is in my power, she will not wake to only medical staff at night searching the room for her mommy or daddy and being afraid of what someone might do to her rather than awaiting a kiss on her forehead. I can’t let that happen again. She’s been through too much, come too far, fought too hard not to know that we are there for her 100%.

I digress…

So after being re-intubated on that Monday morning, the doctors became determined to prevent this event from reoccurring since both incidents had involved the left lung collapsing. I cannot even count how many x-rays she had to get. She also got several ultrasounds with and without doppler imaging, a bronchoscopy, and a CT scan over the next several days. At one point they were concerned that during surgery her diaphragm had become paralyzed as images did not show spontaneous movement and that it’s location in her abdomen was so high that it was in-fact putting pressure on her left lung. There was talk of a diaphragm plication surgery and everything. Thankfully, upon her surgeon’s request, a repeat ultrasound revealed that the diaphragm was, indeed moving. Thank God!

So on Saturday, Oct 10, after many breathing treatments, tests and many “sprints” on the ventilator, Norah was finally extubated a 3rd time. They put her on a nasal IMV so that her breathing was still being regulated by the ventilator for a day and a half, then she was switched to high flow nasal cannula on 10 liters of oxygen and slowly weaned down. In an almost ceremonious fashion, on Wednesday morning, I watched with glee as the nurse literally cut the oxygen line away from Norah’s face and allowed her to breathe completely on her own.

She remains on “room air” and is still getting breathing treatments as well as chest and back PT (basically using a rubber plunger looking device to break up any secretions in her lungs by applying moderate frequent pressure to her chest and back several times a day… aka “pounding” on her to ensure she will not develop a mucous plug). We are so incredibly proud of her despite her 2 “failed” extubations. She just had to be ready for the right time to come off of the support of machines on her own!!

THE PROGRESSION

Intubated and relying on the breathing tube and ventilator 10/10

Nasal IMV and still using the ventilator 10/11

High Flow Oxygen through a Nasal Cannula 10/12

Breathing independently! 10/13

So I left off writing the morning that we took Norah down to surgery and I feel like this week has been so emotional, so full in so many ways, I haven’t had the energy to post what I really wanted to say. A BIG thank you to Tamara and Gary (Jason’s parents) for being with us through the biggest week of all 3 of our lives!!! It was great to have the company, the hugs, and the relief that someone could stay with Norah when Jason and I needed to get away for a bit, even if it was just for lunch.

Norah’s surgery thus far seems very successful. The liver fit better than expected, and while it still protrudes out of the right side of her belly a bit, they were able to close her completely, which is a HUGE relief! Her surgery last around 6.5-7 hours and her surgeon has come in every day to check on her and do a quick evaluation, look over her labs, etc. but he seems very pleased with how things are going. Her liver enzymes continue to go down and her total bilirubin is 1.5! That’s only about 0.2 above the normal range!! She is getting more and more pink every day and her eyes are clearing up so well! Hard to say what color they will be, but regardless they are beautiful!!

She is also getting back to her old self, giving all the nurses a hard time with how “wiggly” and “grabby” she is. All of them have commented how quick and how smart she is, but they all seem to love her and appreciate her fighting spirit! Especially now that she can move more freely without feeling like she is going to choke now that they removed the breathing tube this morning (which we are hopeful will stay out this time), she is spunky and rearing to go! Thus she has to wear wrist restraints to keep her from pulling at her lines and cords to protect her from herself.

Today was not only huge in that she got taken off the ventilator, but they also needed to do 2 dressing changes, a bath, and she kicked out the IV in her foot. She slept hard after the second dressing change and bath, but was really restless at 8:30 when Jason and I came back after dinner. I filled the nurse in on some of the things Norah likes, including music, head rubs, and sleeping on her side. Very shortly after turning her to her side, she started snoring in her sleep, so it was at that point that Jason and I felt comfortable leaving her for the night.

It’s been 1 week since she received her precious gift of a second chance at life and it just feels so surreal, even still. I’m not sure when my head is truly going to be able to wrap itself around what has transpired over the last 7 days, but I’m sure the next several weeks will be full of small milestones! We are so grateful that there was a family out there that made the difficult decision to allow their child to help others when he passed. Please pray for both our family and the donor family as both have much healing to do over the coming days, weeks, and months. We feel so blessed by God’s miracles and the way he worked this all out, even if it threw Jason and I for a big loop. Hehe. Probably best that we didn’t have much time to try to process and anticipate it all!! Now we have a bright future to look ahead to!!!!!

We had just gotten off the phone with Jason’s parents Saturday night to tell them that it was looking good for us to go home Sunday when IT happened. One of the pediatric gastroenterology surgeon fellows stepped into our room and the rest feels like it was a blur.

She said that she wanted to come talk to us because Norah got an offer for a liver donor. The donor was a young preteen involved in a traumatic event that resulted in brain death. The head surgeon for the UCLA pediatric liver transplant team felt that the left central lobe would be a perfect fit for Norah once it was split. We were told that Norah was the primary offer candidate, meaning the doctors felt that this portion would best fit Norah’s needs for a new liver. There was a backup candidate but Norah and the piece of liver from this particular donor seemed to be the most idyllic match.

Jason and I looked at each other and I’m pretty sure neither of us could breathe for several seconds as the doctor continued to give us information. I began to cry almost hysterically and my immediate reaction was to turn the offer down. We were almost out of the clear from this hospital admission, Norah was doing very well, and we were going to get to go home. Then this news fell into our laps. While I didn’t want to be ungrateful, I also didn’t want to believe that she needed THIS liver and that transplant might be NOW OR NEVER! I kept thinking, how is this liver not going to someone much, much sicker than Norah? Surely this can’t be our time. As quickly as these thoughts came and went, so too did the guilt that I was not more happy about this life-saving gift offer or considering the donor family who I know was having an infinitely worse time dealing with the news that their child would never wake up.

Why does this stuff have to exist? Why should children have to die in order to save other children who also don’t deserve to have to NEED transplant in order to survive?

While I kept saying out loud that we could get an offer for a new liver any day, I guess I didn’t actually believe it, so I was shocked when the doctor said those words, “there’s been an offer.” Especially because from what I have heard, read, and talked to others about being listed, usually offers don’t come through until the candidate’s PELD score is in the 30s; Norah’s just dropped to 20 a week and a half ago. It just wasn’t making sense. Not to mention we had no place lined up to stay, we had run out of most of our own medications and clean clothes, and all our preparation for this day was sitting at home in Vegas, waiting for us. This was not the scenario we had pictures in our minds.

Sometime around 2am, an anesthesiologist came to our room to talk to us about the risks and plan as far as her anesthesia. He sounded quite confident that the surgery would happen for Norah, and had us sign all the consents so that we would be ready as soon as we heard the official word from the surgical team.

While in situations like this patient privacy is incredibly important, we were told that the donor was close by, somewhere in North California, and that the family wanted the organs procured that night. The doctor felt pretty confident about Norah and the liver being the right match in size, blood type, health, etc. However, they said that until the surgeon from UCLA actually saw it, we wouldn’t know if it would still be our liver or not, which wouldn’t be before 3am. Jason and I might have gotten a combined total of 3 hours of sleep as we anxiously waited for news one way or the other. It wasn’t until after 5:30am that the nurse came in and told us that we got the green light and she would be taken down for surgery sometime after 6am with a surgery scheduled time around 7.

It was around 7:30am when the gentleman from transport came to bring Norah down to the OR. He could see how little she was and how much she and I were clinging to one another, so he let me carry her down while he pushed her bed and Jason walked along side us with the IV pole. We briefly met the anesthesiologist and surgeon who would be assigned to her surgery as well as the nurse relaying between the doctors in the OR and us during the surgery. After a rather quick goodbye between Jason, Norah and I, with tear-filled eyes we handed our baby to the anesthesiologist and within seconds she was gone from our sight. As we walked away I was still in disbelief that this was actually happening. Was it actually possible that my laughing, smiling, cuddling baby girl would soon go under the knife and have an entire organ removed and replaced? How is it that while her eyes and skin were so yellow, she was in such a happy mood in the moments leading up to this surgery and that soon she would be put under and we would not be able to hold her for days or even weeks? It was just all so completely surreal. My head was swimming and my stomach felt sick.

Well, it was bound to happen sooner or later. Yesterday morning Norah was admitted the hospital after having a fever and being incredibly irritable and unable to sleep for over 24 hours. We had a regular scheduled appointment on Wednesday with our transplant team but didn’t make it since we were instructed to go straight to the ER. Normally in babies there isn’t a major concern with fevers unless it’s consistently over 102 for her age group, but because she is at risk for several kinds of infections (cholangitis from her Kasai procedure and sepsis or a blood line infection from her Broviac site) they are very cautious about any sign of trouble and fever of 100.4 or higher.

Tuesday when we drove to LA Norah only got a few 30 minute naps before she would wake up and cry, though typically on these trips she sleeps like a champ and makes the drive relatively easy. The good news is, she was willing to take several ounces from a bottle while in the car, but the bad news is that feeding her and changing her still didn’t make her happy and we knew she was uncomfortable in some way. That night Jason and I were losing our minds because she barely slept at all, crying hysterically each time she woke up. We did everything we could think of to get her to sleep and finally just after 3am, Jason took a shift of taking her to the car so that I could sleep for an hour or so and then at 6ish we switched at I took her while he tried to get a little rest. To say it was a miserable night is putting it mildly! Around 7am I took her temperature at it was 100.2, and so I decided nurse her, then check her an hour later to see if her temperature would change one way or the other. Around 8am her temperature had shot up to 101.3 and I started making phone calls and leaving messages for both her doctor in Vegas, Dr. Baron and also her nurse coordinator at UCLA, Greg. About 30 minutes later I got phone calls from both Greg and Dr. Baron’s assistant instructing us to go to the ER immediately.

Fast forward to today and we are still here in the hospital waiting to see if any of Norah’s blood cultures grow any bacteria within 48 hours of admission and to see if another fever occurs. She has also been given several rounds of IV of antibiotics as a precaution, and we have to stay at least 24 hours to make sure nothing funky happens off of IV antibiotics so they can feel comfortable sending us home on oral antibiotics. The doctor thought there is a good chance we could be home on Sunday, so fingers crossed!

In the meantime, I think we finally found a medication that actually works to dampen the itchy skin that is a result of Norah’s high bilirubin levels. It’s blood-red and makes her “output” turn orange, but we will take it for less itching, irritability, more smiles and more sleep! In fact, after her first dose yesterday, Norah was able to go about 6 hours between feedings and only needed her pacifier and a back rub to get back to sleep a couple of times during that stretch. Thankfully I was also able to get some sleep, which I desperately needed. Jason stayed at the hotel and was able to sleep well also, so all 3 of us felt much this morning and throughout today. We shall see how well this new medication works in the long-term, but we are happy so far with the results!

Now we are just hoping all her blood work and temperature readings continue to look good so that we don’t have to buy more clothes and camp out in the hospital past Sunday!

***Side note: The doctors, nurses, and hospital in general have all been impressive to say the least! If we have to spend a month here for Norah’s transplant it won’t be so bad!!

So we have been out of the hospital for about 1.5 weeks and Norah’s ascites has decided to behave for now! She is 1cm smaller in girth around her belly than when we left the hospital, so between the IV albumin and lasix while we were in the hospital and her oral diuretics, she seems to be maintaining well. We are very happy that her belly is not huge right now, as our big girl is very interested in sitting, and sometimes standing with support. I got out her little activity table and she stood with my help (but bearing weight through her legs) for several minutes as she played with the musical light-up table activities.

Since being on TPN she has definitely had more energy during the day, which is amazing because she is almost always happy (even more so than before) and now that her belly isn’t huge, she seems comfortable and willing to do more physically!! Nights are still hit or miss, as some nights she will be fussy or wanting to nurse or play for several hours in the early morning hours, but then other nights she will sleep for a 4 hour stretch with no issues. There doesn’t seem to be much predictability for our nights, so we are just grateful for any and all sleep we can get!!

We had a HUGE scare today with Norah’s Broviac line, and thankfully that worked itself out thanks to our nurse, Carol, who is cool and collected to make up for my over-reacting. Here’s what happened:

This morning when I did Norah’s flush for her Broviac I couldn’t get any blood to pull back into the syringe (like they showed me to do with each flush) even though I could push saline & heparin through it. I was so worried about a clot or occlusion in the line! So naturally I called her doctor’s office and they wanted us to go for a “line study” at the hospital, but then the scheduler at his office said they couldn’t get is scheduled till Fri. The only alternative to making sure the integrity of the line was ok was going to the ER and having them assess her.

In the meantime, we were supposed to have our home health nurse come today and she was running behind. I texted her what the situation was, so she said before we go to the hospital to meet her at her office up by the airport (which is conveniently sort of on the way to the hospital anyway). So I did so reluctantly because the doctor’s assistant said go to the ER. But I figured it was worth a shot if we could troubleshoot the problem first, we should to save us the trip and $350 copay for the ER. Thank God I did because the nurse just moved the line a little bit and then was able to do the blood draw easily. We also did her dressing change (on a conference room table with blankets and medical sterile padding) with minimal difficulty as Norah behaved better than she ever has during this tedious process. I was incredibly relieved and grateful to our wonderful nurse!!

Anyway, tomorrow we head to LA for a quick trip to see our doctors for another appointment on Wednesday afternoon so they can assess how she is doing, allow the surgeons to see her, talk about what might happen during transplant, and set us up just in case a transplant is coming up soon. Jason and I are excited and nervous to say the least. To think that it’s possible our little girl could get a life-saving liver soon is awesome, but also terrifying because we are literally taking out a very pivotal body part to put a new one in and crossing our fingers that her body doesn’t reject it while we load her up on medications! Such a scary, but necessary prospect!! We are just hoping the doctors are happy with how she is doing and that she is growing enough and stable enough in their eyes for transplant, so that we can look forward to surgery whenever it happens!

Before IV albumin and lasix on 9/11.

So we got out of the hospital last on 9/2 and were so so happy to be home. Norah seemed to be tolerating the TPN well, and was even happier, even more energetic than usual. We felt good about the extra vitamins and nutrients that her body was getting. But as I said before, the ascites is the price we pay for proper nutrition. I noticed that her belly continued to appear more and more distended over the next week, so we called our gastroenterologist and got in for a more emergent follow-up appointment. When talking with him, he also noted how big her belly seemed… again. Then he looked at us and said, “So when do you guys want to go in to the hospital?” He suggested to do it over the weekend since he was on service till Monday.

**Side note: On Friday we found out that our PELD score went down slightly to a 20 down from 21 in August, 22 in July, and 20 when we had first went on the list on 7/7. The main reason why our score dropped was that her bilirubin levels went down to 19.5, she grew, her INR was better, but luckily her albumin level was quite low.**

So yesterday we were admitted to the hospital at 11 in the morning. The plan was to do 3 rounds of IV albumin, then IV lasix and be out by the next late morning/early afternoon after a 1 night stay. The doctor suggested that we bring our home meds and TPN so we didn’t have to wait for pharmacy and we could start everything right away and get out in less than 24 hours. Well as usual, nothing is ever easy. The pharmacy decided that they were not comfortable with us using home medications because they couldn’t be certain that they were mixed correctly since an outside pharmacy mixed or filled them. Soooooo… we did the first round of albumin and lasix right after we got there around noon (which took 2 hours to administer), but then had to wait till 9 for the next round of albumin, which the pharmacy again decided was not safe enough to administer simultaneously with her TPN, which they don’t mix until 9pm anyway. Long story short, we did albumin and lasix but didn’t get the TPN started until about 11pm.

Now Norah was scheduled for 3 total rounds of albumin and lasix, so we were scheduled for the last round at 5am. The kicker was, because they wouldn’t let us run TPN at the same time, we had to do a 2 hour taper down to avoid low blood sugar, then do the albumin over 2 hours, then IV lasix and restart the TPN. If we had done the full bag of TPN, it would have taken us until 4 or 5 in the evening just to finish the bag.

Thankfully this morning the doctor found out about the hiccups with the pharmacy dictating things, and decided that Norah would be fine if we cut her TPN early and then just restarted later tonight. So she was finally taken off TPN around 3pm, and we were discharged around 4:30 this afternoon. So it wasn’t a 24 hour stay like we had hoped, but it was only 1 night, so we are thankful to be home all together tonight, sleeping in our own beds, with our Stella (who is acting incredibly unloved and neglected). And the other good news is, Norah’s belly is 2.5cm smaller than it was on Tuesday. She lost about a 1/2 lb of fluid and seems much more comfortable. Her breathing isn’t as labored, she wants to sit up more, she wanted to stand a little today, she tolerated a little tummy time, she hasn’t thrown up since yesterday morning, and she was very happy today! So overall, for one night’s worth of a hospital stay (which she was actually very well-behaved and even slept in the crib – not having to cosleep with me) it was worth her comfort and well-being. The question is, how often are we going to have to do this type of hospital admission just to keep her ascites under control? I will be so glad when she gets her new liver and her belly will STAY small!

For now, we are just thankful to be home, to be together, and to be doing well. Thanks again for all your thoughts, concern, prayers, and support!

https://www.youcaring.com/norah-booth-409688

Finally getting some good rest after her first round of albumin and lasix. She loves side sleeping and having a blanket to hold onto or touch while she’s sleeping.