A plan.... with a little more waiting

So today we met the team at Texas Children’s and let me just say... WOW! When you walk into a building and are impressed just by the waiting area and clinic organization, that speaks volumes. The entire place is efficient and organized! We spent a good bit of time talking with Dr. Himes today, and we officially have a plan of action. They asked us to stay one more night so that we can get an MRV done to take a look at the vessels in his belly to determine what type of surgical shunt he is a candidate for. This is a test that we cannot get done in New Orleans as they do not have the machines we need for this there. So tomorrow morning we will report in to the outpatient unit for 6:15 am, and he will be put under sedation to have this test done. 

Today we found out that essentially Parker’s liver itself functions just fine according to all of the tests that we have done. His portal hypertension, however, is the problem we need a solution for. Parker’s liver disease is Congenital Hepatic Fibrosis of the liver. Fibrosis basically just means hardened. His liver is SO hard that the blood cannot flow through the portal vein (which goes through the middle of the liver back to the heart). Instead of the blood flowing freely, the liver is constricting the flow, and the blood is backing up and pooling into the spleen. This is all stuff that we knew already, but to hear that his liver is functioning great otherwise and that his bile ducts are in good condition, makes us feel much better. His bile ducts are dilated but they are not misshapen, therefore the bile is flowing well and he is not at increased risk for cholongitis, which is a major infection of the bile ducts. The doctor did however confirm what we already knew. One day, Parker will need a liver transplant. Things will continue to progress to that point. The good news is that he feels that today is not that day. 

So far our plan of action is to complete the MRV tomorrow. The liver doctors, surgeons, and radiologists will the convene a meeting in the coming days once they have the results to determine which shunt is the best option for him. We will come back here after all of that to meet with the entire team, including surgeons and radiologists to discuss the plan that they choose. During the same visit, Dr Himes will perform an outpatient Endoscopy under anesthesia to get a better look at the esophageal varices that they found in the MRCP last week. He will also treat these varices in order to prevent any complications that could arise due to them. Esophageal varices are vessels in the esophagus where there is increased pressure. Basically the blood flow in these vessels is restricted due to the hardened liver, so the vessels are enlarged and full. If the pressure becomes too much, and the varices aren’t treated, those vessels could rupture causing internal bleeding. This could be very dangerous and life threatening, because sometimes the bleeding can not be stopped. 

Once the doctors have treated all varices and come up with a plan for the best shunt operation for Parker, we will essentially continue to wait. From our understanding, the shunt will not be necesssry until his platelet counts reach the 10s-20s. Right now, his platelet count is holding steady around 45. The concern, and reason we are formulating a plan now, is that Parker’s numbers drop dramatically each time. Last year at this time his platelets went from 120 to 75. Just a few months ago his platelet count went from 75-45. If he drops that dramatically this time, we are absolutely in range to need the shunt immediately. So the entire team will be on the same page so that when the time comes to execute the plan, we all know what to do next. 

So, yes we did get answers today. And yes we have a plan, which makes us feel MUCH better. However there is still a lot to do. 

Thank you to everyone who has helped at home and picked up our slack at work while we are on this journey with Parker. We truly appreciate all of it! Also thanks to everyone who has checked in for updates and sent prayers/thoughts/well wishes! It means the world to us!