April 26th, 2016
Our first week started out with a bang. Katie and I had come to terms with the fact that Isla most likely had Biliary Atresia, but there was still a little hope in the back of our minds that she might still be fucking with us (too bad it wasn’t April fools). Our mornings began with wake up sessions from the blood lab. Isla, the tough cookie, did not really mind the little poke on the bottom of her heel. What she did mind was the restraint caused by one of the technicians holding her leg straight as the other quickly scraped blood into a test tube, drop by drop (the girl just liked to move it, move it!).
One of us would then sneak out to the kitchen with our pre-mixed formula and disposable baby bottle, heat some water in the microwave for the formula, and quickly creep back to the room to feed our baby girl. We would then order some breakfast from “Dial-for-Dining,” shit, shower, shave, and prepare for our day. Dial for Dining, by the way, was the food service setup the IWK had where you would choose an appetizer, entree, two drinks and a desert all for the price of $10. The food was all pretty good, unlike times I have stayed in the hospital choking back a cold bagel and some Jell-O. The food was ordered via the cafeteria and hand delivered right to our room. I still dream of the mango frozen yoghurt… Besides all of the tests, our days carried out very similarly: blood test, Dial for Dining time, eat the enormous amount of food provided by friends and family (thanks mom, Tabitha’s mom Michelle, Dad, etc), colour in adult colouring books with friends, telling Vanessa how awesome she was for house-sitting (seriously, she cleaned the cat litter, ran the dishwasher, fed our three cats, and took pictures of them for us; she was their new mother), and sleep in our single hospital bed (if we didn’t hold on to each other, one of us was bound to fall off).
One day we had a short older nurse with spiked dark hair (who quickly became one of our favourite nurses) introduce us to an amazing little sugar solution called “toot-sweet.” For those of you who do not know, this clear sugar-liquid contained in a small purple tube, was named “baby crack” on the paediatric floor. You could be giving your baby a “Horse Bite” while dunking her head into the toilet for a swirly and as long as a drop of this liquid touched her lips, she would probably be giggling the whole time. Seriously though, the mornings became a lot easier with this shit. Instead of two technicians standing over our daughter grabbing and poking while we sat on our bed red eyed and glaring; it would be two technicians as well one of us squeezing one drop at a time of Toot-Sweet as soon as Isla’s eyebrow so much as twitched. Isla could be mid-screech and as soon as the drop touched her lips she would immediately stop, start slurping like a pudgy toddler with a popsicle on a scorching hot day, and moan with each suck as though to say: “it’s so good, once it hits your lips, it’s so good!”
Needless to say, this shit was da-bomb and was used several more times during our stay...
We felt as though we met with every professional at the IWK including the kitchen sink. Everyone wanted their piece of the pie with Isla and for the most part they were necessary. We met with all of the Gastrointestinal (GI) specialists including Dr. R’s two other colleagues, the awkwardly quiet resident, and a plethora of medical students. Katie and I quickly became the experts on Biliary Atresia and had to sometimes explain it to the IWK professionals since it was such a rare disease (Dr. R claimed that they only receive 1-2 cases a year in the Maritimes). There was nutritionist who setup Isla with her special formula concentration as well as vitamin regimen. There was a social worker who checked in to see how we were coping with everything. There was the genetics team who created a pedigree as well as going over Isla’s diagnostic plan for their branch of medicine (which turns out, was the bulk of her diagnostic work). This was quite the situation and Katie and I learned more than we wanted to about our families. Luckily My mom as well as Katie’s mom were both in the room and they had a better grasp of our family disease history. “Great uncle Jib fell off a ladder when he was trying to peep on his neighbour and ever since he has a permanent erection,” said my mom. Katie’s mom said, “Cousin Beedoowapdo from Zambia got herpes from his pet Orangutang, but they are still in love.” I am surprised the poor geneticist stuck around as long as she did, however pale her skin turned. The genetics team was trying to rule out a disorder called Alagille syndrome, which can cause fewer than normal bile ducts in the liver, amongst many other things. The doctors understandably wanted to be 100% sure Isla’s condition was Biliary Atresia before performing the surgery.
I will summarize her diagnostics test for the first ten days of our stay in the following paragraph. The first stop of the week was x-ray. We headed down to the x-ray room where we were met with an exceptionally bubbly x-ray technician. We got to put on the bullet-proof vests and hold Isla straight out with her arms up and legs down (she clearly did not enjoy this). They wanted to have a look at her spine since Alagille can cause an abnormal curvature in the spine. Next, we headed to the ophthalmologist (eye doctor) to check her eyes. During this test the eye doctor was checking for common Alagille indicators such as iris and optic disc issues. This was the first test where the doctor essentially took a quick glance and said there was nothing wrong (one step closer to a complete diagnosis of Biliary Atresia). The next test was the sweat test. This one was an interesting one to me. It was clear the technology has not changed in decades. The nurse rubbed a cream on Isla’s leg, hooked her up to an electrode machine, and wrapped her leg (with electrode attached) in a bag. The electrode caused her to sweat, the sweat was then captured by the bag, and the sample was sent off to test whether she was at risk for Cystic Fibrosis or not. The final piece of the genetics diagnostic puzzle was to perform an echo of Isla’s heart (ultrasound of the heart). This test was the second time we received confirmation from the nurse/technician that Isla was not at risk for Alagille (It’s also when I started wondering why we had to do so many damn genetics tests including the following blood draw). Part way through the test the nurse was looking closely at Isla’s heart and simply said: “These are textbook images, I could use them to teach a class on the anatomy of the heart.” At the end of the echo, Katie and I had come to terms with the likeliness of Isla needing surgery for Biliary Atresia. It was great that she did not have some extremely rare genetic disorder, but was still a chronic disease nonetheless, which was going to change her life. No matter where we were in the hospital, be it walking through the halls, in x-ray, blood lab, ultrasound, or paediatric unit, people always went out of their way to let us know how beautiful our little lady was. They were always astonished at how well behaved she was and shocked when we told them what we were at the hospital for (she did not look like a sickly baby, maybe a little yellow, but not sickly). Of course, Katie and I already knew how awesome our bambino was and always obliged with a gracious thank you.
After the wondrous week of test after test, we met with the GI team as well as the surgery team to go over what to expect. They advised us of the good news that Isla was healthy in every way besides her liver function. They were all on the same page now and wanted to advise us of what to expect with surgery (the Kasai procedure would be performed (see last post for explanation) as well as a biopsy of liver tissue to see the extent of damage). At this time, Isla’s name was making its rounds around the hospital. Katie’s friend Tabitha works as a nurse in the operating room so the surgical team was forewarned of the awesomeness which was Isla. Even so, they were blown away by her cuteness and quickly took a liking to our little munchkin. The surgery team was made up of several nurses, a resident, an anesthesiologist, and four surgical doctors (Dr. Superstar (performed the surgery), Dr. M, Dr, D and the surgical fellow, Dr. Victoria (who was nothing short of a great person)). The surgery team assured us that we would have the A-team, but they were not sure which one of them was going to perform the surgery as it was getting closer to Christmas and the schedule was out of whack. We had one more test to do in order to finalize the need of surgery and that was the HIDA scan. Isla had been taking a crap-ton of a red coloured liquid all week in order to dilate her liver vessels for the HIDA scan (Phenobarbital). It was the end of the week and we headed down to see the nuclear medicine team for Isla’s scan. They would be performing it once today and once more a day later in order to get clear results. We brought Isla into the room and set her up on, what looked like, an MRI machine. They had to administer a radioactive dye via intravenous so that the machine could pickup the tree-like structure of her liver vessels. The whole point of this test was to see if and how well Isla’s bile was moving through her liver and into her gut. Besides the stubborn team jabbing Isla multiple times in order to administer the dye (then settling on sticking the needle in a vein of her head), the test was fairly straight forward. However, seeing my sweet little girl stabbed by a needle on each of her appendages made me so upset I had to leave the room. As we were looking at the screen, which was pretty neat, neither one of us thought that we could see drainage into her gut. This was confirmed early the next day when we met with the surgery team again and they advised us that they would be performing surgery on Isla. One more official blow to our nerves, but this time we were prepared.
It was finally official, after the gruelling tens days of diagnosis, that Isla certainly had Biliary Atresia (of course not 110% sure until she was opened up, but 99.9% repeating). We found comfort in the amazing surgery team and Tabitha assured us, now that we knew who was performing the surgery, that Dr. Superstar was the best person to do it. Dr. Superstar had spoken with us about how Biliary Atresia, however un-ideal it was, was the best of the worst things for Isla to have. Firstly, babies are very resilient and the liver is especially so. The liver is one of the only organs that regenerates (thankfully for all of you alcohol abusers out there) and only needs to have a blood type match in order to donate a live piece of liver. Secondly, The Kasai procedure is not a permanent fix for 95% of all patients, but the goal is to prolong the time needed before the child needs a transplant (gives the child time to grow before partaking in a major surgery). Thirdly, Dr, Superstar was the only doctor on the surgical team who had performed the surgery before, so he was the right man for the job. These facts shed some light on Katie and my concerns and I am pretty sure we both decided, right then and there, that we would both be volunteering to be her liver donor. This would mean that we would have to slightly tweak our lifestyle: drastically cutting back on alcohol consumption, lowering our BMI’s, and continuing to stay focused on a healthy living/eating plan. We already actively pursued these goals, but raising Isla under these conditions so that she can make healthy choices herself, was the final kick in the pants for Katie and I. The surgery was booked for the 22nd of December and other than being at home, we learned that the IWK was an amazing place to be for Christmas.
The day had arrived, and saying that Katie and I were on edge was an understatement. Luckily Katie’s good friend, Tabitha, volunteered to be with us when we handed Isla over to the surgery team. Since we were headed to Tabitha’s place of work, we had no problem navigating the hospital to where we needed to go. Of course, when we arrived at the waiting room all of Tabitha’s co-workers needed to see the famous Isla and it was nice to see how great the staff was in whom we were entrusting our babies’ wellbeing to. I will never forget the final moments before the team took her into surgery. The anesthesiologist had come out to explain the procedure, but I did not hear a word he said, I was just nodding and going through the motions. I had my sweet baby girl in my arms, Tabitha, Katie and I had all kissed her “good-luck.” Then the younger nurse, must not have seen me kiss Isla, and said: “daddy has to kiss his little girl.” I kissed her, longer this time, said I loved her, looked up and saw the whole nursing team was crying as well, and that was it for me. I don’t think I have ever cried so much in my life, the cliche tough guy in me could no longer hold back. It was official, Isla had broken down my tough exterior and I would forever have a soft spot in my heart for her. The only other thought I had besides Isla was: “this poor innocent lady who just so happened to hit the button in the elevator to the surgery floor, only to have the doors open up to Katie, Tabitha, and I holding each other in this state.” We gathered our composure as we headed up the elevator back to the 7th floor where both of our families were waiting. We quickly gathered our things and moved to the surgical unit, which is where Isla would be heading after her surgery.
That night everyone convinced me to finally leave the hospital, since I could not be by Isla’s side in surgery, and we stayed at the Snillis’ house. Watching some movies, having a beer and sleeping in their comfortable bed (a straw bed would be desirable after two weeks in a single and cot) was much needed. Besides Katie and I jumping and staring at each other intently each time a phone or microwave beeped, it was a pretty straightforward evening. Dr. Superstar had promised to have his staff inform us periodically of surgery updates, which were all: “progressing well.” Finally the final update came from Dr. Superstar himself who called to let us know the surgery went well and that he was ready to meet with us to go over post surgery expectations as well as to see how we were doing. The post surgery recovery stretched through Isla’s first Christmas, but I will talk about this in more detail in my next post. I promise, it will all be good news and then we can get back to the crazy-silly day-to-day shenanigans of Isla and her family!