One minor thing that had been on the table since Norah was 24 weeks gestation was a couple of holes in her heart. Now most babies in utero have them, and when she was born the ultrasound showed one of them had closed and the other one was a PFO (Patent Foramen Ovale) which was very minor and we shouldn’t be too concerned. We had seen a pediatric cardiologist in the hospital when she was born and then during another follow-up in March. The doctor said that everything continued to look better but if she needed a transplant that she would need an echocardiogram for a more in-depth picture of her heart. Well needless to say, I called them and scheduled the echo shortly after the mention of UCLA.

After more than 5 weeks of going back and forth between Norah’s Gastroenterologist here in Vegas, the insurance company, and UCLA, we finally got an appointment for June 17 in LA.

Now skip ahead to June 11. That morning I was on my way with Norah in the car to her cardiologist appointment when a lady from the living donor team at UCLA called me and told me that I was not a candidate to be Norah’s liver donor. She said that because I have the MTHFR mutation and therefore could develop a blood clot more easily during surgery, the risk of fatality (or complications at a minimum) was greater than anyone at UCLA was comfortable with. While I’m glad to know that the doctors have both donor and recipient patients in mind for transplant, I was devastated to learn I couldn’t save my own daughter’s life if given the chance (double bonus is that Jason is a type 1 diabetic and so was already out of the running for a donor as well).

So anyway, I took Norah to her cardiology appointment and was relieved that A) the echo was fast B) the doctor allowed Norah to stay cozy wrapped in one of her favorite blankets (except over the chest) while she happily sat in her car seat, and C) we finally had good news that from what they could tell the PFO had closed!

The next day we were scheduled for yet another set of chest X-rays (2nd set in about a month) and abdominal ultrasound (the 4th in her short 5 month life). These scans were simply in preparation for our evaluation at UCLA but I would come to find out why later that day. Now Norah is a strong little girl, no doubt, but HATES being “messed with” very much. It’s why bath time and diaper changes aren’t much fun, why it takes 3 people to hold her during blood draws, and why ultrasounds take so long to complete – she screams and cries almost the whole time, every time. Anyway, it took about 15 minutes until the tech stopped and asked the doctor reading the scans if I could feed Norah to calm her down, even though she was supposed to be fasting for 3 hrs prior to the ultrasound & x-rays. After some convincing and more screaming, I was allowed to nurse her and she calmed down almost immediately.

Later that day, we had been home from the radiologist appointment a couple of hours when I got a call from Norah’s Gastroenterologist. The assistant informed me that they were concerned that the scan wasn’t done properly and that they could not visualize blood flow through the portal vein. Keep in mind that the portal vein conveys blood to the liver from the spleen, stomach, pancreas, and intestines – so kind of major. We were instructed to go directly to the Sunrise ER and they had been informed that we were on our way. I wasn’t sure what to think… part of me believed that the scan earlier had been wrong and that the doctors were just covering themselves by doing another scan to determine the blood flow through the portal vein. Another part of me felt sick, knowing that a hospital admit was not out of the question, and wondering how this would affect her ability to have a successful liver transplant.

At Sunrise they told me I was not allowed to feed her until we had gotten the “ok” from doctors following the ultrasound. This means that Norah had to be fasting for a 2nd time that day – going more than 3 hours between feedings for an exclusively breastfed baby is not much fun, let along doing it a couple of times a day! They completed the ultrasound and after a little while the ER doctor came in and told us we needed to do a second one with doppler because he had not put in the order correctly. Though he was very apologetic, that didn’t help my now screaming baby feel any better. Luckily one of the nurses recognized us from when Norah had been in the ER at only a few weeks old and instantly remembered how effective “sweeties” were in calming her. Thankfully they allowed me to use these sugar-water drops with a pacifier to keep her more comfortable during the next ultrasound/doppler scan. Afterward, they determined that indeed, the portal vein appeared occluded. With tears in my eyes and with a confused look on the doctor’s face, he said, “There’s not much we can do so your Gastroenterologist cleared you for discharge.” I was filled with emotion and immediately got on the phone with the pediatric Gastroenterologist follow at UCLA and after some discussion, the doctor explained that while there would be follow-up actions, right now there wasn’t much to be done.

Another hurdle. Awesome.

The first month after surgery felt amazing! While it was like bringing home a newborn all over again (we had to relearn her hunger cues, how to take care of her incision, how to give her medications, working on breastfeeding, doing super quick diaper changes because every time she laid down she screamed in fear), we were just so happy that our little girl was doing so well. All her doctors marveled at her progress in such a short amount of time and her bilirubin got down to 1.3 after surgery… lower than it had ever been!

Grandma Jacki came to visit again right after we got home to help with the adjustment and to let Mommy & Daddy get some sleep after such an exhausting 2 weeks in the hospital. Seemed like everyone was smiley about everything!

We also had visits from Papa Fred, Grandma GG, and Jason’s brother and his family over the next few weeks.

It was about 6 weeks after surgery and I was continuing to notice swelling around the incision and all around Norah’s right side of her abdomen. I was assured over and over that it was fine, and even when her bilirubin levels were checked after my insistence due to concerns about lighter stools, the doctors kept telling us that it was too soon after surgery to be concerned that something was going wrong. She didn’t have fevers, didn’t appear to be sensitive to the touch anywhere on her stomach, wasn’t vomiting or having diarrhea, and kept having plenty of wet and dirty diapers so none of her doctors were concerned. However, later in April I again pushed for blood work as Norah appeared to be getting more and more yellow to me and I wasn’t satisfied with just phone calls to the doctors’ assistants telling me not to worry. I knew better…

Norah’s bilirubin suddenly jumped up again, and this time it was pretty major. We had a couple more blood draws, doctors appointments, ultrasounds and chest x-rays but the bottom line is that our gastroenterologist’s office called me late on a Friday afternoon and his assistant relayed a message along the lines of, “A HIDA scan would not be helpful diagnostically at this point because even if there was drainage her bilirubin levels are too high for appropriate readings/scans. She is being referred to UCLA to their liver transplant evaluation team and your insurance has already been checked as UCLA is a preferred provider and they will contact you for an appointment.” I’m sure there were other things said and that these weren’t the exact words, but the gist of it was that the doctor felt that she had taken a pretty drastic turn for the worse. We were devastated to no end. Absolutely. Utterly. Devastated. Jason came home from work early that night so we could all just be together as a family as we tried to comprehend what was ahead of us. How could things have been going so, SO well and then go so horribly wrong in such a short amount of time? We still don’t know…

Norah was on the regular pediatric floor for the next week. She slept most of the first few days after the surgery but when she was awake she looked right at Daddy and I. We didn’t get to hold her that much for those first few days but by Tuesday, she gave me her first smile since Friday morning before surgery. I was so happy I couldn’t help but bawl my eyes out and kiss her over and over again. Grandma Tamara also got to hold her a couple of times, and we all were so excited to see her skin get less and less yellow and for her to finally get some pink in her cheeks. Her bilirubin dropped significantly over that week and a half and everyone, doctors, nurses, and our family were hopeful that the surgery worked and that she would be well for a long time. The surgeon said to us that we were “lucky” because only about a third of Kasai procedures work this well. We were so happy! Not to mention they finally let me give her some breast milk by bottle Wednesday and Thursday, 1.2-1oz at a time, but by Friday they allowed me to nurse again. Both Norah and I were so anxious and ready to get to resume this special bond and for her to get nourishment in her preferred way, skin-to-skin with me.

Norah didn’t make it home quite in time to celebrate her second month birthday, so we did a mini photo shoot in the hospital. She was pretty quick to pull on her tubes and lines so we had to keep socks on her hands to prevent her from hurting herself. Nevertheless I got to put clothes back on her and it felt like we had our baby back, better than new!

We were finally discharged on Sunday, March 8 and got to be home as a family!

Norah had been in the operating room for about 45 minutes when a staff member of the hospital called our names in the waiting room and said that the nurse wanted to speak with us. We picked up the phone and I held it out so that Jason could hear what the nurse had to say. I don’t remember everything that she said but the phrase, “We are going ahead with the Kasai” was all I needed to know that Norah did in fact have Biliary Atresia.

The link below explains what the Kasai Procedure is and provides an illustration of what is disconnected and reconnected during this surgery for Biliary Atresia:

http://uofmchildrenshospital.org/HealthLibrary/Article/88701

Keep in mind Norah was only 7 weeks old and was having major abdominal surgery… Jason and I were a mess. In fact I vaguely remember getting light-headed and nearly falling to the floor with the phone still in my hand. Jason quickly pushed me into a consultation room as I cried hysterically, asking, “Why?” and “How can that be?” through sobs. We were soon put in a private consultation room so that we didn’t have to sit with everyone else in the waiting room for the remainder of Norah’s surgery. Once complete, the surgeon came to the room to speak with us about how the procedure went. He apologized that it was Biliary Atresia and said that overall her liver didn’t appear to be that unhealthy and they did manage to see bile flow before closing her up, so there was hope that she wouldn’t need a transplant if this procedure worked well. Through teary eyes we said thank you to him and awaited the anesthesiologist to come in once she had been taken to recovery. Both doctors commented that she did very well and there didn’t seem to be any complications during surgery, but Jason and I were still in shock and overwhelmed with what was happening to our baby.

I went to see her first while Jason stayed with the anesthesiologist in the consultation room for a few minutes to prepare himself to see her. When I went to her bedside, I was mortified to see her eyes wide open and a breathing mask over most of her face as she breathed heavily. I exclaimed, “She’s having trouble breathing! She’s not ok!” but the nurse assured me that the breathing mask was just drying out her eyes and once he readjusted it, she was able to appear more relaxed and a mist was put on her face so that her eyes didn’t get overly dried out. It was tough seeing her like that, hooked up to tons of wires, cords, with a huge scar across her belly, a drain sticking out of her lower abdomen and a central line in her neck as well as a couple of IV ports (one in her left arm and another in her left leg). When Jason came to her he nearly had the same reaction that I had a few minutes before. The nursing staff quickly realized that we were having difficulty with the situation before us and go us large reclining chairs that we could safely sit in next to Norah in case one or both of us got light-headed. It was all so surreal.

After a little while they transferred Norah to the PICU where she was in a private room with a large glass window and door looking right out to the nurse’s station. There was a small fold out chair that I would sleep on so I could stay with Norah 24/7. There was a parent lounge with a couple of bathrooms and a kitchen we could use, along with the other families on the  floor, so Jason stocked me up on snacks and drinks to keep me while I lived at the hospital. Norah would stay in the PICU until Sunday afternoon when we were moved to a room on the regular pediatric floor.

Norah’s bilirubin levels were very difficult to predict after that first hospitalization. We went for several bloodwork panels, went to the hospital a time or two and in general felt totally naive to what was happening. No one we knew had ever gone through this with their babies and as 1st time parents we were teetering the line between denial and paranoia all the time. I mean, before she was born we had no idea there were 2 kinds of bilirubin and had difficulty remembering the significance of direct vs indirect bilirubin levels.

During the first month or so of her life we had many visitors! Grandma Tam and Grandpa Gary were the first to meet Norah, then Papa Fred and Nana Linda. Grandma Jacki was able to visit for 10 days and then shortly after Aunt Cindy got to visit. Norah was quite the popular gal!!!

However, when at 5 weeks old Norah’s bilirubin levels again went up to over 9 mg/dl total bilirubin we were told we needed to get a liver biopsy to determine the cause. We went in on a Monday for the procedure to Sunrise hospital and after about 40 minutes the procedure was done. By Tuesday evening we were released even though th biopsy still didn’t provide a definitive diagnosis. We were told Norah needed to start on a medication and a vitamin regardless of the correct diagnosis because it was either a form of hepatitis or Biliary Atresia. We were also told we had to meet with a surgeon the next day (Wednesday) and that she would be scheduled for a cholangiogram by the end of the week.

Friday morning we met with Norah’s doctors and got her prepped and ready for the procedure. The surgeon told us that depending on what happened during the cholangiogram, he might have to perform a very serious surgery called a Kasai procedure if they determined that she indeed had Biliary Atresia. He also said that she would be in the operating room for a minimum of an hour but up to 3 depending on what happened and that a nurse would keep us updated. So the nurse took her from my arms as I looked one last time at her cute, nearly flawless belly and prayed we would see her within an hour or two.

We took Norah to the pediatrician the first chance we got and immediately our doctor noticed that Norah was very jaundice. She did a heel stick blood draw to check the bilirubin levels. A few hours later we got a phone call from the nursing staff that we had to get a redraw because they thought the blood test was misread. After going to one lab in which the phlebotomist “fished” for her veins and still couldn’t get a good draw, Jason demanded to talk to the pediatrician face-to-face to see  what the blood draw was about. He went into the office and sat down with the doctor to try and understand why we should put our tiny baby through another blood draw. She talked about the concern about high direct bilirubin levels and mentioned Biliary Atresia to us for the first time.

So into the car and to another lab we went, this time getting a good poke on the first try! Unfortunately the results of earlier were confirmed and we were sent to the ER to have Norah admitted. After a grueling time of multiple pokes and multiple nurses trying to get the blood work and IV started for Norah, we stayed overnight with no answers until the morning.

We met several doctors but Dr. Baron would come to be our new gastroenterologist. He discussed the bloodwork that had been done and had yet to be done to try to diagnose why Norah was having such high bilirubin readings. He mentioned Biliary Atresia but said it was unlikely at that time. So because nothing certain had been determined and her condition was “stable” we were sent home with more questions than answers. It was the start to our very long, difficult journey we would soon learn.

The life of Norah actually starts roughly 3.5 years before she was born. Jason and I were married in Estes Park, Colorado on September 11, 2011. It was a beautiful wedding and reception and we decided that we would no longer “prevent” pregnancy since we wanted to share our life with a precious baby of our own whenever it might happen.

Through a series of losses, we found a wonderful doctor who suggested genetic testing for me. In doing so, they found me to have a MTHFR mutation which meant that to sustain a pregnancy I would have to give myself daily injections of a blood thinner to prevent clotting in my uterus. I did so willingly but painfully from 6 weeks pregnant till the day before Norah was born. At 18 weeks I also had to have a cerclage placed because my cervix was opening far too soon and I would have experienced another more devastating miscarriage otherwise.

Finally my due date arrived: January 6, 2015. I went in to the doctor that morning feeling pretty good but anxious to finally meet my baby. Low and behold I was dilated 6cm without even knowing it, so the nursing staff immediately got on the phone with nearby hospitals trying to get us a delivery room. We ended up going to Summerlin hospital at 4 that afternoon, meeting my Doula, Sarah, there at the hospital. I felt fine with no major contractions until the decided to have a laborist break my water at 9pm. Less than 5 minutes later I was in the full swing of labor. By 12:30am on January 7, 2015, we came face to face with our long-awaited baby!

She weighed 7lbs 15oz, was 19.5″ long and was totally perfect in our eyes. Not long after she was born we decided to name her Norah Lois Booth (Lois is after her Great Grandmother on Jason’s side since she has been an important part of Jason & my life). We got to take her home Friday afternoon and were tired but thrilled to be parents of this beautiful little girl!