She Sure is a Miracle!

What a day!  What a visit!  We are so excited about continued great news about Macy's liver!

We headed up Thursday night so we could be ready for an early morning visit on Friday.  Thank you to Mamaw for keeping McKenna for us, and to my friend Megan for taking McKenna to school and keeping her after school until we could get her!  The Ronald McDonald House didn't have a room again, so we booked our go-to hotel that is a couple of blocks from the hospital.  After working and going to school all day, and riding in the car for over 3 hours, we were all exhausted.  Morning came too early when the alarm went off at 6 a.m.  We hiked it back to the hospital parking garage and hopped in our car to head to an outpatient center in Lincoln Park.  The actual hospital couldn't get us in for her ultrasound, but the outpatient center could.  We didn't mind, we were just happy for an early 7:45 a.m. appt.

Her ultrasound went great, and I could see that the shunt was open on the screen, so I was relieved.  We ended up doing our labs there too since there was no wait.  We were back in the car and heading back to Lurie's by 9 a.m.  We headed to the cafeteria for some breakfast since Macy couldn't eat prior to her ultrasound. We then arrived early to the clinic to see Dr. Superina.  Our appt. wasn't actually scheduled until noon, but they told us to head up early if we had everything else done.

Dr. Superina's nurse, Joan, was amazed by how good Macy looked, and couldn't believe that Macy went to school for a full day on Thursday.  She said that she thinks Macy holds the record now as the first patient to get back into the swing of things the fastest. We couldn't have been more proud of her this week for all of her determination!

Then Dr. Superina and his partner, Caroline, came in to see Macy.  He walked in stared at her and just kept smiling.  He was so excited about how unbelievably good everything with her went and how it is currently going.  They said, "Macy, do you even realize how famous you are?  You are pretty famous around here!"  Caroline commented that they can't ever get these kind of emotions out of Dr. Superina, and she couldn't believe how happy he was.  He told about how the doctors in Paris, France have been following what he did with Macy, and are so amazed.  Caroline told Macy she was so famous she might be on People Magazine or something, lol.  Dr. Superina said, "She sure is going in my Hall of Fame!"  He went on to say that it was proof of concept that kids who you can't grow a portal vein in can eventually be changed into a Rex patient.   He said he had proved his hypothesis!  He said he was going to be writing about her for sure.  So let's just lay it out there....she was kind of his experiment.  Aaaahhhhh.....glad I fully know this AFTER the surgery!

I've mentioned before that he has only done the Rex Shunt in Abernethy patients twice before.  One was an infant, and one was a young boy.  The difference in these kids and Macy is that they had larger "original" portal veins, as well as a more robust portal vein system inside the liver on both the right and left sides.   Macy is unique due to her abnormally-shaped liver, her thread-like "original" portal vein, and almost no internal portal system inside her liver.  Back in 2016, after he re-routed her blood to the liver, it pushed blood into the liver, but her portal vein barely grew.  It helped develop the internal portal vein system on the left side, but the left side only.  So the surgery that we thought would fix her back in 2016 put her into a very long-term portal hypertension, while slowly growing the inside veins.  Then came the question of "can he change her from an Abernethy" kid to a "Rex Shunt" kid?  Well, as we all know he did!  He had never done this with a kid who had the anatomy of Macy.

So that is why this is all so amazing to the Rex Shunt medical community.  She is not the reason the Rex Shunt was created.  Kids like Macy are only supposed to be cured with a liver transplant.  That is exactly what we were told in 2008.  Dr. Superina keeps proving them wrong!  He is AMAZING!

Her numbers were all good (platelet count, electrolytes, liver enzymes, ammonia levels, and hemaglobin).  Those are the main ones we are watching.  The biggest one right now is her INR.  It was a little low at 1.9.  So we are increasing her Coumadin dose for 2 days, going back to normal on Sunday night, and then rechecking her INR on Tuesday morning.  We can now do this at home thanks to our new Coaguchek machine.  We had our training on Wednesday night, had her first reading, and are ready to do it on our own now.  The awesome part is that the machine transfers to my phone, and my phone automatically uploads the results to the company.  The company then sends the results to Dr. Superina's office.  It's so easy!  This will definitely make life easier.

So things are looking great following surgery, and we couldn't be any happier!  Macy is feeling really good, and her stomach pains are going away.  She is getting back to her "normal" self!  Yay!  We return at 3 months and 6 months for ultrasounds, labs, and clinic visits.  Then she will have an MR at 1 year.  What a blessing!  We are praying that this sweet girl can just be a teen for a while.  We are praying that our family can just enjoy being a family without the medical worries.  We want to enjoy the lake this summer, have late night bonfires with friends, let the girls have sleepovers with friends, and take some fun family vacations.  Thanks for checking in on us!