Chicago Visit & 3 Month Check-Up

We headed back to Chicago for a few days for some medical tests and a follow-up visit with Dr. Superina.  When we are there for surgeries we don't get to have much fun, so we decided to throw in some fun while there this time.   We've been to Chicago many times, and seem most of the touristy things, so this time we went back to a few of our favorites.

We stayed downtown in the historical Warwick Allerton Hotel.  It opened in 1924.  It is officially a historical landmark in Chicago.  As cool as that sounds, the rooms were small, and it seemed a little dated.  However, I think it's supposed to be that way, lol.  I could see this hotel out of Macy's ICU room during our last stay.  I stared for hours at the sign on the back of the hotel that said "Tip Top Tap".  I wondered and wondered about what it was like inside, and how much fun all of the guests were having.  Were they heading out to a show, were they drinking cocktails and having appetizers.  I pray for the the mother in that ICU room today, who sits and stares. 

On Monday we had some tests at the hospital and spent most of the morning there.  Afterward, we headed to The Navy Pier for some fun!  We spent the afternoon exploring the pier, and making fun memories.  We headed out to dinner at Macy's favorite restaurant, The Cheesecake Factory, and then ended our night watching The Blue Man Group.  Jeremy and I have seen them before, but the girls hadn't, and they absolutely loved the show!  

On Tuesday, we headed to the Willis Tower.  It's 103 stories tall!  We enjoyed going out on the Sky Deck to get a crazy view of the city.  I think I was the most scared.  Macy, who is normally extremely scared of heights, did great!  She was ready to get off of the Sky Deck after the pics, but otherwise did great.  McKenna had no fear!  We explored more of the city, and headed to one of our favorite parks, Millennium Park.  Seeing The Bean never gets old!

We ended our trip with a visit to Dr. Superina and his team.  Macy's tests showed that her shunt is open!!!  Yay!  Labs were pretty good overall.  We got back in October for our 6 month check-up.  At that time she will have an MR to get a better look, as well as more labs.  We will also have a scheduled IR appointment for the next day.  IR stands for Interventional Radiology.  This is the team that performs Macy's venograms.  Macy's Rex Shunt has great overall blood flow, however it is a little narrow at the top.  Think of it like an arch.  It was placed in that way due to her abnormally shaped liver.  It is a little narrow at the very top of the arch.  It has been like this since placed, so it's not like anything has gone wrong.  In order to get the best out of her shunt, it would be best to have this area a little bit bigger.  There are two ways for this to happen.  1. It grows a little wider on its own over time.  2.  The IR team goes in through her neck, like a venogram, to balloon and stretch it a little bit.  

So when we return in October, if the MR shows that the area hasn't gotten wider on it's own, they will go in the next day to make it a little bigger.  If the MR shows that the shunt has gotten wider, we will go home.  So for now, she continues on her Coumadin to keep her blood thin, which has been the plan from the beginning (for 6 months total).  The goal is to keep that shunt open and flowing!   They were very pleased with her!  They said she looks great!!!  

I hate the thought that she might need another procedure, however if it's to fine tune her shunt and make it overall better, then we understand if it needs to be done.  We are so happy that we had a good visit!    Please continue to pray for an open and flowing shunt.  Please pray for the narrow area to grow wide enough on its own.  Please pray for Macy's safety as she continues on Coumadin.  Thanks for checking in on us!

The AMAZING Dr. Superina!

Last week, the mother of another Abernethy patient interviewed Dr. Superina in an Instagram Live Q & A.  It was an hour long interview, with some wonderful questions!  I decided to summarize the interview here to highlight some of the material that I thought was interesting.

• Dr. Superina became a surgeon just by fate, it wasn't some long thought out plan since he was a child. He would've rather been in the operating room rather than making rounds.  
• As a hub in the U.S. for Abernethy patients due to his abilities, Lurie Children's Hospital in Chicago has only seen about 70 cases total due to how rare it truly is, but only operated on 17 patients.  Macy is one of those 17.  For perspective, he compared this to the billions of gallbladder surgeries that have been completed.  
• An ultrasound is the most common way to diagnose Abernethy Malformation.  In some cases, they also do CT scans and MRs.
• He believes the cause of Abernethy stems from the cardiovascular development.
• Physicians used to believe that Abernethy didn't need treated, however we know now that it does.  Before corrective surgery, the blood is not filtered by the liver due to a lack of blood traveling through the liver.  Therefore, patients have an underdeveloped liver, possible liver tumors, high pressure in the lungs which can lead to heart problems and breathing problem, failure to thrive, and high ammonia levels that impair clear thinking and the inability to process thoughts.  It is a very hard disorder to diagnose because these symptoms don't necessarily warrant a physician to think it might be Abernethy.
• The first patient that he saw had an associated liver disease that needed a transplant.  He had never heard about it or read about it before that.  There isn't much literature, but he is willing to help any physicians that need to know more.
• He would never recommend a liver transplant for Abernethy!  He said patients are still getting transplants for this.  Most patients that come to him have been told they needed a transplant, and are seeking more info themselves or a 2nd opinion.  This is our case!  We were told at age 2 that she would need a transplant, but found him by chance of a mother's blog trying to get the word out and seek info herself.  
• 25% of his Abernethy patients have some sort of heart defect.  
• Many patients also have lung problems.  
• He recommends treating it as soon as diagnosis.  Sometimes in older patients there becomes a time where the portal vein is unsalvageable.  
• He talked briefly about Macy's case of being an Abernethy Malformation patient and being transformed into an Rex Shunt patient.  He lit up talking about how they are excited about it! He said it is extremely rare. Essentially a Rex Shunt is for a patient who is born with a normal liver and normal portal vein (the vein that runs to your liver), but somehow their portal vein stops functioning.  Abernethy patients are born with very abnormal livers and very abnormal portal veins.  
• Blood thinners came up.......it is essential and should last for at least 6 months.  
• He does not believe that Abernethy Malformation is hereditary.  He has never seen it run in a family. 
• Meds and liver......we do not need to worry about meds harming the liver.  Alcohol?  There is no concerns, however no large amounts should ever be taken in any person.  
• They are starting some tests to learn more about cognitive abilities.  Many of his patients are diagnosed with ADD or ADHD.  In most patients, this is due to the Abernethy Malformation, and is not a true Attention Deficit issue.  It should get better over time after a repair.  
• He is working very closely with a hospital in Paris, France, as well as Japan to make Abernethy Malformation known world-wide.  
• Prior to corrective surgery, high ammonia levels are sometimes treated with medications. Other patients are given a low protein diet.  
• He travels the world to do this operation.  He has traveled as far as Brazil and Israel.  He said you need sophisticated equipment, which is why he is somewhat limited to where he can perform this procedure.  He has done surgeries in third world countries like Nepal to do other liver surgeries.
• They have not studied growth in Abernethy patients, however they have in Rex Shunt patients.  There is a correlation because when nothing is going to your liver to be processed correctly to feed the rest of the body. Corrective surgery usually helps growth.  
• He has trained many surgeons, but they often become general surgeons.  He has only trained 1 or 2 surgeons that are confident enough to do Abernethy and Rex Shunt surgeries.  

We head back to Chicago next week to see our Chicago team!  Macy will have an ultrasound, lab work, and clinic visit.  She told me that she is going to beg to come off of her blood thinners, but I highly doubt that will be the case.  She will most likely be on it until October, and then another blood medication for 6 months following that.  We are anxious and excited all at the same time!  She seems to be doing so well!  Please pray for great ultrasound results, good lab work, and a wonderful 3 month check up with the team!