Wednesday, July 17, 2013

The Next Step in Alexandra's Care

Today was a HUGE, and I mean HUGE day! We got some very exciting news. Scary but really exciting.

Today we received the results from the CT scan Alexandra had back in June. It indeed showed she does have Abernethy Malformation, which is a congenital absence of the portal vein. This changes everything.

Lately, I have been scouring the internet for any and all medical articles that pertain to this condition. My brain is overflowing with information about this extremely rare condition.

Today we also did a very simple test to see if because of this malformation, in combination with the AVMs in her lungs, she does indeed have hepatopulmonary syndrome. It turns out she does. This simple test consists of having the patient lie flat and then do a simple pulse oximetry test. Then after watching their oxygen saturation's for approx 5 minutes, you have them stand up. While lying down, Alexandra's sats were pretty good, anywhere from 89 to 91. Then once we had her stand, her sats fall into the range of 79 to 81. A significant drop. Thus proving the existence of hepatopulmonary syndrome.

Now onto the really exciting and scary part of things. We all know Alexandra's issues have stemmed from those damn AVMs in her lungs. Her heart function is great. We have successfully helped her heart like we always intended to. But now as her hepatologist said "the money isn't in the heart anymore, its in the lungs and liver." Her heart is fine. But if we don't deal with the issues in her pulmonary and hepatic systems, she still will not survive.

Because of the absence of the portal vein, a large amount of hepatic blood flow misses getting back to the lungs once it has been filtered through the liver. Also because of this there is a discrepancy in the pressure between these vessels, which has also contributed to the AVMs. The only way to "correct" this is to have what they call an Auxiliary Liver Transplant.

There is a doctor, a really fancy, very innovative doctor at Yale University who has done this successfully on a 9 year old girl with similar issues. This is our chance to continue to keep her quality of life where it is for many years to come.

Alexandra's records will be sent to Yale, via Phoenix Children's and in October when we travel to Boston, she will have a transplant evaluation at Yale-New Haven Hospital in Connecticut. If it is deemed she is able to handle the transplant, she will then be listed for transplant.

What an Auxiliary Liver Transplant is, is when a small portion of a donor liver (which still has to be from a donor who will pass on), and a portal vein from the same donor is attached to her actual liver and blood is rerouted in the intended way or correct way it was meant to flow. This will resolve the AVMs and all the work that has been done on her in the cardiac fashion will do what it always was meant to. She will have a relatively normal oxygen level. This is such a hopeful and amazing thing!!!!! This is innovative. This is medical journal, amazing life saving stuff!!!!!

Now there is many obstacles this creates, which I will get into at more length as we continue on in this process. We don't know if the transplant team at Yale will find that she can handle this. It may be that they feel her heart could not handle this. But there is no time better than now to discover if she can handle this, as she is at her most stable cardiovascular wise.

Please continue to pray for Big Girl. This changes everything. We have some very busy, exciting things ahead. Thank you all for the love and support!

1 comment:

  1. I'm ready to cry!!! So happy that there may be a way to truly help her and extend her life so much further as will as greatly improve the quality of life for her. This is such amazing (and scary) news. Hoping and praying for the best, i know she can get through this!!!

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