After we were discharged on August 3, the discharge orders stated that we would get a blood draw at least once a week, every Thursday to be run STAT. Well we got Norah’s levels checked on August 6 and found out that she was on the borderline of too low… again. We were relieved to find out that they didn’t order us straight to the ER but that we needed a redraw to check levels on Monday. Much to our pleasant surprise, Norah’s sodium level went up 1 point so that she was out of the danger zone for low sodium, so they just wanted to keep a close eye on it, which means we were free to make our trip out to LA for a follow-up with the transplant team.

We left on Tuesday so we could be settled in before our appointments on Wednesday. I convinced the head doctor for the living donor transplant team to meet with us prior to our appointment with the gastroenterologist. We realized we had met him before, but were able to sit and talk with him and ask our questions. We learned a lot more that we were confused or unclear about as far as our ability to allow Norah to receive a living donor transplant instead of only being eligible for a deceased donor. While she still isn’t as big as what they want children to be ideally before transplant, the doctor said something that gave me hope oddly enough, so I’ll paraphrase but basically he said, “We say 10 kg as kind of that magic number that we look for to do living donor transplants, but because Norah is having difficulty gaining weight, we will take what we can get so long as she is healthy enough to withstand a living donor transplant.” He also mentioned that while she is in no way the epitome of health, she is relatively healthy given her diagnosis and difficulty with sodium and growth. He said that he would entertain the idea of living donors for her transplant sooner than we expected, and that there were a handful of people who called in to their team interested in donating and being tested as potential donors. Because of medical privacy laws he couldn’t tell us much more, but Jason and I both felt a renewed sense of hope after that conversation! We are so thankful that some of our friends and family would volunteer for such a selfless act to help save our daughter!!!

Then at our appointment for Norah with her gastroenterologist, we had a very good conversation as well! We talked with the nurse coordinator first, then the gastroenterologist, and then the surgeon joined briefly. Jason and I both remarked afterward how nice and knowledgeable everyone is, and we feel like we are in good hands! We talked about medication adjustments, replacing the medication that Norah hates and fights the most (as in she is intentionally trying and sometimes succeeding in throwing up almost every day during and after I give her the antibiotic), decreasing the number of medications she is currently taking, and that perhaps to ensure that Norah’s sodium stays within a more healthy range, that maybe we need to switch her lipids to a TPN, but only at night so that she could still nurse during the day. That made me happy, because that is something that I have feared is that she would have to stop eating orally or would have to stop breastfeeding. The doctor said that really it should be called PPN for what we are using it for since TPN stands for “total perienteral nutrition” which according to a google search states that, “TPN may include a combination of sugar and carbohydrates (for energy), proteins (for muscle strength), lipids (fat), electrolytes, and trace elements” but we will only be getting “partial perienteral nutrition.”

Regardless of our appointments at UCLA, we have to wait to make any changes so that the UCLA team can speak with the Las Vegas team and make sure everyone is on the same page regarding her medical management so that they make the best possible decisions for her at this time and moving forward.

The other thing that was encouraging was that they actually recommended that we have Norah multiple listed on more than 1 region’s transplant list, which is something our insurance is hesitant to do for us. They provided us with very sound reasons we should pursue multiple listing and offered to help in any way that they can. Again, we feel very fortunate to be with the UCLA team, and even grateful that they are so supportive of any way we can get Norah better faster! Thank God! What a great feeling to have after leaving a doctor’s appointment!!

After Norah got the Broviac placed, we were finally discharged the next day (July 21). We got started with our home health on Wednesday, had to have a dressing change late Friday night (I’ll get to that in a bit), and then a blood draw Tuesday the 28th. When I hadn’t heard from the doctor’s office about lab results Wednesday, I felt a little uneasy. Usually when you don’t hear about blood work it means no news is good news! Right? Wrong. I finally called the doctor’s office Thursday morning and they still hadn’t gotten the results from home health, so they had to request them again.  Low and behold, Thursday afternoon at about 3:30 I got a call that Norah’s sodium was lower than it’s ever been but because the results were from 2 days ago, they wanted a redraw. Well anyone in the medical world knows that you can’t get blood work results back before the end of the business day if you do a blood draw after about 2, even if it’s marked STAT. So there we were, on the other side of town for one of my doctor’s appointments, faced with the inability of a redraw unless we went to the ER. You probably know where this is going…

Sure enough we were admitted to Sunrise again after Norah’s sodium was only 122 (normal range is 134-145). Luckily we met with Dr. Baron’s associate, Dr. Elizabeth Mileti. She was awesome! We really liked her and she was totally on the same page as us as far as decreasing the lasiks and other diuretic as well as starting Norah on lipids to help her gain weight now that we had a Broviac catheter. After a couple of days we were within the range they wanted us for sodium (still not typical, but at least high 120s) and we were cleared to go home on Monday. Unfortunately the doctor that took over on Monday (since Mileti was only there for the weekend) didn’t put the orders in quickly enough and they threatened to have us stay another night until they could figure out the lipids for use at home with home health. To make a long story short, I sincerely cried to the coordinator for our home health equipment agency and said that I just wanted to be home. I told her we had spent more of July in the hospital than out and that Norah wasn’t “getting better.” I told her that Norah didn’t eat very well and was very stressed when we were in the hospital and so I cherished every day that we got to be home and would fight for any opportunity to do so that I could. Not sure whether my tears or the coordinator made things happen, but we were able to go home late Monday evening. Thank God! I cried more tears of happiness!

Ok… backtracking just a tad, we were set up with 1 home health nurse initially on July 22, when he came out that Friday to do the dressing change, let’s just say that he did not follow proper sterile procedure in doing the dressing change. Since we had read and been demonstrated to by the nursing staff in the hospital, I knew that his technique could put Norah in jeopardy of getting infection so I kept questioning him, asking if he could do this or that, and he totally blew me off. Not to mention he sort of made fun of me when he came out initially because I was up in arms about having someone here to do the flush when the doctor’s office kept calling to make sure it would be done by such a time and he was taking his time getting to our home. Anyway… I was not impressed by this particular nurse to say the least, and so we were reassigned to a different nurse. The nurse that came out after we were discharged the last time was so great! I was impressed by her patience, her understanding, her care, and her way with Norah. The nurses that did the next blood draws were great too! I guess there’s always a bad egg in every batch! Just thankful that the original nurse we were assigned will never step foot in my house or touch my daughter ever again. Even Stella didn’t act like she trusted him… and you know how intuitive dogs are about humans. So at the end of the day, we came home, stayed home, and were set to do flushes at least every 24 hours and provide Norah a lipids supplement 4 nights a week to help her gain weight. Success!!

Well we are still in the hospital, and my fears about her veins has now been addressed. This morning Norah is having surgery to get a Broviac catheter placed in hopes that we can use it for blood draws, IV fluids, TPN if her lack of weight gain continues to be an issue, and will be in place for the transplant itself. Several weeks ago the nurse at UCLA mentioned the Broviac to me because they put one in during the transplant surgery and leave it in place for her after her surgery for her home care. The major concern was infection since its like a permanent IV that goes into her chest using the subclavian vasculature.

As if any surgery or procedure isn’t scary enough for our tiny baby girl, this morning all of the doctors (Gastrenterologist, Surgeon, and Anesthesiologist) bombarded me with more potential downfalls of this whole thing. I felt completely panicked as I kissed Norah before they wheeled her into the OR and told her how much her daddy and I love her. Why does her life have to be so unfair? How does she. Deserve all of this? Why can’t anything go smoothly and easily for us? I don’t want to sound ungrateful for all we have in our life and I don’t want pity… I’m just confused and scared for Norah and this hard, hard life she has had and continues to experience. She hasn’t gotten any breaks and all of  us are so tired through all of this. Will the pain and heartache ever stop?

Just as I ask that question, I must remember to be strong and have faith. I mean, look at this beautiful smile…

Last Friday we were instructed to get Norah’s sodium levels rechecked since they have been low since the week before. I ended up not getting to the lab for her blood draw till around 3:30 so the results were not read until Monday morning. That’s when we got the call to go back to the ER due to low sodium again. When we got to the ER they started an IV in her hand (which is always brutal because like me, the veins in her hand are hard to get to) and checked her levels again. This time they were up to 127, and the normal range is 134-145. While they were low, it was an increase from Friday’s results. The GI doctor decided to try changing the dosage on her mess rather than admit us, and we were told to get more labs either late Tuesday or early Wednesday morning. We thought, “Thank God, we aren’t being admitted!”

Not so fast…

I took Norah for a blood draw at 3:30 Tuesday since she was due for her 6 month well check at the pediatrician later on Wednesday morning. By 9:30am Wednesday we had already gotten another call to return to the ER and we would almost definitely be admitted this time. Fast forward to today, Saturday, July 18, and we are still here, at Sunrise Hospital. To make a long story short, Norah’s sodium levels keep bouncing up and down and don’t seem to want to stabilize. She has had at least 10 blood draws, and up until Saturday they were able to do so through her IV line. Unfortunately the IV clotted that morning and so they had to poke her. Not once, not twice, but 4 times in order to get enough blood. I know the primary concern is low sodium, but I’m getting worried about her hemoglobin levels and the veins themselves after so many blood draws!

July 7 was Norah’s 6 month birthday and also her first day on the liver transplant waiting list.

I got the call at approximately 4:45pm from the nurse coordinator, Greg, who notified us that Norah is officially “listed” and that she has a score of 20. They told us while we were in LA that the highest score they had ever seen was 40 and the lowest was in the negative numbers. We can’t see the list for ourselves due to protection of patient information according to HIPAA, but we know that she is somewhere “in the middle” of the list. I was told from here on out to always have my cell phone on, charged, and with me in case we get THE CALL for her gift of life. While we still have some hope that a living donor will be found, we also know it is a bit of a long shot considering how rigorous the evaluation is for living donor candidates. In the meantime, we will try to be as prepared as possible and pray that we won’t have to wait too long on the list for the perfect match for Norah to come through.

As I was thinking about ordering one of these for myself, I wondered how many of my friends/family would also be interested?

I am most likely going to order one for myself and one for Norah to wear around her ankle so that maybe we can educate others rather than have questioning stares when they see Norah!

Norah had a really hard time waking up from anesthesia they used during her MRI this morning. She wasn’t on a breathing tube and her stats were stable, but she just couldn’t seem to wake up or stay awake! Her MRI was at approximately 9:15am, she had her last feed from 3:45-4:00am, and didn’t eat at all again till nearly 1pm. She had a really hard time staying awake long enough to get much and did so at both her 1pm and 3pm attempts to feed. Naturally I was getting nervous because she wasn’t on an IV drip, so I was afraid her electrolytes we’re going to be even further out of normal range and that she would be dehydrated. They gave her IV fluids around 5:15 and by 6:30 she had eaten twice and was more alert. We rechecked her sodium levels and the doctor on the floor talked to her GI. After conferring between the doctors, the la b, and myself, we felt comfortable about discharging Norah. We finally made it home around 8pm and have been resting and nursing ever since. Thank you for all your thoughts and prayers. Hopefully she will continue to eat well and we will be able to follow up with her GI doctor later next week!

When we were at UCLA they discussed her portal vein and that they would really like an MRI of her vasculature. They wanted the MRI to tell them if the portal vein was actually occluded or if the Doppler when doing the ultrasound couldn’t visualize blood flow because of all the excess fluid and because the vein is very small in babies.

We were scheduled for an MRI Friday, July 3. However, when they checked Norah’s electrolytes in a blood draw on Wednesday, her sodium and potassium were low, and the GI knew that those numbers could cause concern for an anesthesiologist performing the MRI. After much discussion back and forth between myself, the nurse assistant to our GI, and the anesthesiologist assigned to the case, the compromise was to do a lab draw prior to the scheduled MRI to check her levels. Unfortunately that meant that if the levels were too low, Norah’s MRI would be canceled and she would be admitted to the hospital for fluid management.

Norah was all prepped and ready for her procedure when we got the news from the anesthesiologist that her sodium levels were still dropping. Jason and I looked at each other as Jason said to me, “I just wanted to catch one break.” Luckily I had hoped for the best but planned for the worst, so I packed extra clothes and supplies for both Norah and I.  What I couldn’t have anticipated was that she was being admitted to the PICU, so would be tethered by 4 different sets of cords/lines and they would have to perform blood draws every few hours as they pushed different fluids through her IV. As they continued to hook her to more and more monitoring devices, I looked at my poor little baby and felt sad that just holding her was going to be a chore. All the while though I tried to stay positive that this was necessary so we could get the MRI for UCLA and so that they would have much-needed information prior to her transplant.

I obliged the hospital staff, helped give her medications, nursed her, reporting the time spent nursing and kept dirty diapers for them to weigh, but at 6am her sodium was still low. They did one final push of a hypertonic solution and the anesthesiologist finally agreed to do the MRI around 9am, 5.5hours after Norah had last eaten. I felt terrible for her.  This was the second time in 24 hours she had to be fasting. She had done this all too often. As I kissed her before being taken into the room for them to start the MRI, I couldn’t help but cry softly to myself. I so wish she didn’t have to go through all this. I so wish she didn’t have to endure all these tests, the poking and prodding, the surgeries, the medications… I just wanted her to have some “normalcy” for once in her life. In her nearly 6 months of life she has had to deal with more than some people do in their entire lifetimes. Thank God she is strong… For her, for Jason, and my sake.

If there was ever to say, “Yeah, I know,” this was the day. The day started out a little hurried as Norah decided she needed to eat every hour so that it was hard for me to get ready in the morning and get out the door. We finally made it to our usual gathering at Well Rounded Momma for the Momma’s Milk Circle, which is for breastfeeding moms to visit, hang out with other moms and babies, get support, and if needed, get help from a lactation counselor. We didn’t have much time since I had a doctor appointment that afternoon, but I at least wanted to stop by, say hello to my friends, and weigh Norah. On their scale Norah weighed about 13lb, 3oz, which was almost a pound and a half lighter than she was 1 week before (14lb, 9oz). That means the stronger lasics/diuretics we had started were working to remove fluid from her belly. Because of her skin color and her giant scar though, it’s hard for other moms to not only notice Norah, but unknowingly stare at her.

One mom in particular who has brought up concern and questions to me before sat next to me and seemed to continue where she left off last time. She asked me about Norah’s condition, about liver transplant, and then commented that even though her belly looked better than last week when she saw her, her arms and legs looked really thin, like she was malnourished. It was at that point that I decided it was time for us to leave. I felt as if she was accusing me of under-feeding her or not making sure she got enough calories or something. I tried to briefly explain that due to Biliary Atresia her body has difficulty absorbing nutrients, which makes weight gain difficult. Then came the words that absolutely put me over the edge… “Oh this must be so hard for you.” She didn’t know me, she didn’t know Norah, she knew nothing about our lives, about Biliary Atresia, about needing a new liver for Norah to survive! All of a sudden I felt a very unwelcome pity. As I quickly packed Norah up to leave, I peered at the group of women and saw many new moms of whom I had never met stare at Norah, and I immediately welled up with tears and knew that no one understood. As I drove away I realized that not only was this a place where I would continue to get looks, comments and questions about Norah, but that everywhere I went people were probably staring at us, and my heart sank.

As if that wasn’t hard enough, when I arrived to my doctor’s appointment, Norah was insistent that she needed to nurse again… While I completed my new patient paperwork, in the waiting room, without a cover. When we were finally taken into the exam room after having been there for an hour, one of the first things the nurse assistant said upon shutting the door was, “Whoa. He’s yellow!” Not only did I have to briefly educate him on biliary atresia, but after several minutes of me saying she, he still referred to Norah as a male, so I had to say, “SHE is a girl.” About a half hour later the doctor popped his head in and said that he would be a couple more minutes because he had to attend to an older lady who was sick, but before he popped back out, he took one look at Norah in her car seat and said, “I only work with adults, not babies. I’m not a pediatrician.” All I could do was reply with an unfiltered, attitude in my voice response of, “Yeah, I know.” Then when he finally came in to see me, he looked at my bloodshot eyes (I had been crying between when he had popped his head in and when he came back) and told me that my eyes were likely due to seasonal allergies. I tried to explain to him that I nurse Norah, so I still don’t get a lot of sleep, not to mention Norah’s BA causes a lot of stress and anxiety, but he didn’t have time to listen to me apparently. He continued to do a quick examination and talk at me, then handed me a lab slip and said to schedule a follow-up appointment in one week.

After I left the doctor’s office I was hurt, offended, and mad all at the same time.  All that had happened in the last few hours was too much and I broke down, crying and nursing for several hours in Norah’s nursery. That’s when it occurred to me that this was only one of the many more days like this we would have until after she gets her transplant, and it made me incredibly sad. My cute, little, smiley baby girl doesn’t deserve judging looks, comments and questions and neither do I. This hand that we have been dealt is unfair and cruel, so I just have to try my best to look for the silver lining and know that as soon as we get a new liver, life will be different. Until then, I guess I need to grow tougher skin and stand up for myself. I have always and will always continue to do everything I possibly can for Norah to be her advocate when she can’t speak for herself in hopes that she can one day lead a healthy life, and no one can take that away from me.

So we finally made it to UCLA on June 17. Going in we weren’t exactly sure what was going to happen or what we would learn, so we were just eager to get there. We knew that we were going to meet with some of the most knowledgeable doctors in this part of country regarding Biliary Atresia and it’s treatment, so we were hopeful.

The first appointment we had scheduled was with a cardiologist who wanted to do another echocardiogram to see if his stronger, better technology would pick up on any issues or see a PFO. We were relieved when he came in after the test and said everything looked fine. He said he didn’t anticipate any problems that they would find before, during, or after a liver transplant in regards to her heart. Jason and I turned to each other after the appointment and said that we had a good start to the day, and hopefully that would continue.

Next we met with the nurse who coordinates care, insurance processing, etc. for liver disease for the pediatric GI. We instantly liked his personality, professionalism while talking about BA and the transplant process, as well as caring demeanor. Not to mention he was wearing a Teenage Mutant Ninja Turtles t-shirt under his scrubs (neither of us said anything to him but Jason and I talked about it later that day as we were recapping the events of the day). Anyway, both he and the pediatric GI were great. They talked to us in-depth about the listing process, about some of the things they were concerned about with Norah, and also introduced us to our social worker who would help us throughout the process. The doctor did a physical examination of Norah, asked us some questions, got her list of medications (luckily I had remembered to put them all in my phone, including pictures of the bottles in case I made an error when inputting the information) and talked to us about transplant. It was at that point that Jason and I both realized no one was saying “if” anymore, and that it was a matter of “when” that Norah would need a transplant. Suddenly we felt defeated and rather than surprised, and we were numb in the realness of the situation.

Norah was not going to survive unless we got her a new liver.

The rest of our appointments were a bit of a blur to me as we met several surgeons, an anesthesiologist, and rejoined our nurse and social worker at our final appointment. Finally the head of the surgical department for liver and kidney transplant surgeries said the words we had been dreading all day: “She definitely needs a transplant.” I know he said other things too to the effect of her needing it relatively soon, that just by the looks of her she met criteria, and made it clear that there were no other options.

We also talked about a variety of other topics including:  what her score on the PELD (Pediatric End-stage Liver Disease) would be on the transplant list, that she needed a stronger diuretic to help with her severe abdominal distention, that neither Jason nor I were donor candidates, how living organ donations worked and that because of Norah’s petite size, how small the donated liver would have to be, discussed some of our supports and questions regarding resources, etc.

https://en.wikipedia.org/wiki/Pediatric_end-stage_liver_disease

After that last appointment there was one more stop we needed to make – to get Norah’s blood work to confirm her blood type, bilirubin levels, etc. We started the day of appointments at 10am and didn’t leave the facility until around 5pm.

To say that the day was long and overwhelming is an understatement. We walked back to our hotel without talking much and just “zoned out” for a little while before we decided we needed to eat after not having a meal since breakfast. It was hard to believe we had just been through all of that in just one day, and neither Jason nor I wanted to talk to anyone about it. The certainty of Norah’s fate regarding her need for a liver transplant was always in the back of our minds, but when it was actually said and confirmed, it still felt we had been hit by a bus. How could someone even prepare themselves for that? To look at your tiny 5 month old baby with all those thoughts and fears is something I could never have anticipated.

Even after all the specialists had examined Norah and met with us to explain how badly she needed the transplant, it was still hard for me to comprehend it all. I knew she was yellow, but I had begun to shut my brain off to it as much as possible. It was then that I concluded that even in her smiles, there was pain and a general feeling of unwellness, and that she was much, much sicker than she was letting on.