Day 3: Macy's Journey with the Rex Shunt

Sorry I didn't post yesterday.  Surgery was much longer than anticipated, and the evening was long than I ever imagined. So even though it is Day 4 today, I am writing about Day 3. 

We started our morning bright and early @ 4:55 a.m.  We checked in at pre-op at 6, and met with multiple people from the surgery team.  Dr. Superina obviously came to chat.  He informed us that Plan 1 was the Rex Shunt.  Plan 1?  I thought there was only that plan.  You mean there is a Plan 2?  Yes, and also a Plan 3.  Jeremy and I about fell over.  I didn't like Plan 2 because it didn't repair the liver, it basically just eliminated the chance of any more stomach and esophageal varices.  Plan 3 basically created a shunt that runs from the spleen to the inferior vena cava (bypasses the liver), which is the exact state her body was in when we when she was born. 

They took her at 8:15 a.m.  She smiled as they wheeled her off, and requested that they play Luke Bryan music when she entered the operating room.  Yep, she's a superstar like that!  Jeremy and I were a mess waiting.  Especially because we didn't know what the plan was.  They started in her abdomen because they needed to get a good look at her anatomy.  Macy's anatomy is very different from most people, and actually very different from a kid who is getting the Rex Shunt for a clotted portal vein. Here is why.......she was born with Abernethy Malformation.  Malformation, meaning her liver was underdeveloped and abnormally shaped.  This we knew.  In 2016, he routed blood to her liver by closing off the shunt her body had created.  This surgery allowed her liver to grow larger, and develop robust portal veins inside.  Yay!  However, she didn't have one definite portal vein outside of the liver carrying blood to it.  This is not ideal. 

So when he got in there, he discovered many things.  1.  Her liver is very abnormally shaped.  2.  Her veins in and around the liver at all very different too.  3.  She had a lot of scar tissue from her 2016 surgery.  He had to first figure out her anatomy to see if he could even do the Rex.  He figured out a way.  Then, he had to clear away all of the scar tissue, and anything that would compete with the Rex. 

By 12:20 p.m., he was still working in the abdomen and still trying to decide about the Rex Shunt.  Once he decided that he was going to do it, he then needed to make the incision on the neck.  This didn't even start until after 2:00 p.m.  Around 4:00, we were updated again that he was back in the abdomen working to attached the vessel correctly.  Surgery ended 9 hours after it started.  The surgery was a success!  She had the Rex Shunt put in place, and blood was flowing through it.  I might add, that he has done many Rex Shunts on a typical Rex Shunt kid, but only a handful of times on an Abernethy kid.  He was actually the first surgeon in North America to do this surgery, and teaches people about it all over the world.  He was in Paris last week.

Yesterday felt like the longest day of my life.  Little did I know, the long day was just beginning. 

They just kept pushing back our time to go see Macy in the ICU.  We didn't see her until almost 6:30 p.m. She was not settling in well.  High heart rate, and struggling after being extubated.  I know I am going to get a lot to wagging fingers at me, but Jeremy and I never ate all day, and it is Thursday afternoon now, and we still have not slept. I can't eat or sleep when I am worried about her.  I actually get nauseous.  Jeremy and I are completely drained. 

I will give more details later about what happened overnight and has been happening today.  In short, Macy is still struggling.  You would not even recognize her to be honest.  We believe she is bleeding internally from the surgery.  Usually you stop bleeding following procedures on your own, however, she is on a blood thinner to keep the shunt open.  So it benefits the shunt, but isn't allowing her body to properly heal.  They are giving some meds, blood transfusions as needed, many labs, and doing another ultrasound at 4:00 p.m. today.  The ultrasound this morning showed an open Rex Shunt.  Yay!  Then a decision needs to be made about our next step.  Can they continue to treat her with meds, blood, etc?  Or does she need to go back to the OR?  Please pray for some clear answers that do what is best for Macy.  Please pray for her healing!  Please pray for me and Jeremy, we are really struggling. 

Thank you for continuing to follow us, and giving so many words of encouragement and prayers!