Let me start by giving you some back story. Parker's disease, ARPKD, comes with a liver disease known as CHF- Congenital Hepatic Fibrosis. If you have ARPKD, you automatically have CHF, because they come hand in hand. Most children don't show signs of CHF until later in life. Until now we have done routine tests and check ups with his GI doctor, but everything always seemed to be "fine". When the results of the CT scan came back, though, they weren't. Parker's liver isn't draining right. When your liver doesn't drain right, it backs up and doesn't allow blood to flow through it. Parker's body, as amazing as it is, has been fighting to create a "natural shunt". He has created new veins and arteries by himself to bring blood around the liver and back to the heart. This is not good. It's called portal hypertension.
The doctors here have met as a team, researched everything they could, and consulted other hospitals and doctors. This situation isn't completely uncommon in patients with this disease. Parker is not the first child this has happened to, but his team has decided that this is out of their league. They don't have the experience to handle his transplant case. They spoke with several doctors and gave us several options as to who we should see. As of right now, we are waiting for a doctor from CHOP, Children's Hospital of Philadelphia to call us. They are the top children's hospital in the nation and have tons of experience in kidney/liver involvement. They are paired with the ARPKD/CHF Alliance and have a researcher specifically for CHF.
We don't know exactly what will happen. We don't know if he will need a liver transplant, or if they can use a renal-spleenal shunt to control things. We don't know how long we will be there, but have been told to expect to be there for months after transplant. I don't know if we are going there first for tests and then coming home or if we are going there and staying there.
All that I do know, is that we have the most amazing donor. She has said that no matter what we are in this together and that she is willing to come with us to Philadelphia- over 1,000 miles.
When I hear from the doctors at CHOP, I will update more.
I should add that we have so much respect for our team. Their honesty with us is amazing. I applaud them for admitting that this is too much for them, instead of being egotistic.
As you can imagine, we are having a hard time wrapping our heads around this. We know that our big guy is a fighter, but weren't expecting this obstacle for a while. Please keep us in your prayers.
Kristen, so sorry to read this, but in the words I see a lot of hope.
ReplyDeleteSo grateful for your team that is willing to do whatever it takes. I know a few kiddos who have gone through shunt procedures, so please let me know if I can connect you!