Texas Trip.. Learing about our Donor Family and Surgery Options

Our trip to Texas was definitely filled with emotions this time. We are back home in TN now, as we decided to wait and think about the surgery more before making a decision.

On our 1st day of appointments, Elizabeth had her monthly bloodwork done and we met with the Transplant Team.  Back in April, I had written a letter to our Donor family expressing our gratitude for their decision to donate and saving Elizabeth's life. I realized when I wrote there was a chance we may not hear back from them. But I really wanted them to know how important they are to us, and what a difference this gift is making in all of our lives. During our appt with transplant, our coordinator gave me a letter from our Donor mom. I was so nervous to open it, but so happy to hear from them and know that they had received our letter. So as we sat in the waiting room waiting for Elizabeth's CT scan, Matt and I read the letter together. As we were sitting there worried about Elizabeth's pending procedure, this letter was a reminder of hope, a reminder of what has gotten her this far.  Our donor mother, Samantha and I have been on contact via Facebook and I asked her permission to share some information about Elizabeth's angel with you all. Elizabeth's angel is a 4 month old little boy named Elijah, he was born Dec 23, 2010 just days after we were transferred to Texas to start Elizabeth's wait for the "perfect lungs".  I have seen pictures of this precious boy, and it still amazes me that his family was able to make such a huge decision during their time of loss. We are and will forever be grateful to them. We truly hope that learning about Elizabeth gives them some comfort in knowing that Elijah continues to make an impact on others, even from heaven.


As for the remainder of Elizabeth's trip/appointments:

After the Bronchoscopy

CT Scan - We learned that she now apparently has a reaction to contrast. She broke out into a red rash all over, but thankfully it went away. But now she will need to predosed with medication prior to future testing. The CT scan showed that her aorta and innomiate artery are both enlarged and that is what is causing the major compression of her airway. Enlarged vessels/arteries are one of the complications/concerns with the Filamin A mutation she has. Scan also showed there is some slight compression where her trach used to be. Her bronchoscopy showed the same issues and gave a different view of the compression from inside her airway. We were able to look at all these images as the doctors explained them. We knew of these problems from all the previous testing at Vanderbilt but just never had such "good" images to really show us how bad the compression was.

After her CT Scan

So we were given 4 options with regards to treatment/surgery and the risks/complications:

p1~ Do nothing, continue with supplemental oxygen during sleep and hope that she doesn't get worse or run into any complications. The doctors all agreed that this is not the option they prefer. They feel something needs to be done to correct the problem. The biggest concern with her airway collapsing is that it can cause air trapping in her lungs, basically killing parts of her lungs and decreasing her lung function. With Elizabeth we need to preserve these angel lungs as long as possible! The survival rate at 5 years is less than 50% - which they reminded us of during this chat. So we need to be sure we do everything we can to not cause damage to the lungs.
2~ Replace the trach, it will keep her airway open and provide peep to keep her lungs open. The downside- due to the innomianate artery being dilated the trach could rub on it causing it to erode over time, leading to internal bleeding. And the major risk of infection having a trach causes could potentially damage her lungs more. This option was also voted "not the best option."
3~ The option that the Doctors recommended most - Basically relieve the compression on the airway by surgically replacing part of the innominate artery with a graft and moving the position some so it was no longer on the airway. The graft would be smaller and possibly out of the way. Sounds easy enough right. The downside, since during transplant the Dr lifted her aorta to attach it to her sternum in hopes of relieving the compression that now makes it difficult to open her chest up. Basically he said the hardest part of this procedure would be ensuring he does not cut her aorta when he opens her sternum. Replacing part of the innominate artery would mean affecting the bloodflow to the right side of her brain/arm and potential for stroke. The possibility that these dilated arteries will not "hold" a graft and start leaking. The fact that she is immune suppressed and healing could be difficult for her. And the potential for needing to go on heart/lung bypass during the procedure causes other risks. Add to all of that, that this may not fix the compression. So basically this was our "best option" with some pretty serious risks. THEN, once she recovers from this major surgery, possibly have another surgery to fix the floppy area where the trach was by removing a piece of her rib and placing it in the area to strengthen and hold it open.
4~ Our last option which is definitely the last resort, would be to completely remove the floppy/compressed parts of her trachea and to try sew the remaining sections together.  And I wont even go into the risks of this one, since we basically have no plans to consider unless we have no other options left.

So, if you've made it this far in this post, you realize that this decision is NOT an easy one for us to make. The risks are very big with such a large procedure and the chances of something going wrong are pretty significant. Matt and I just felt, now was not to the time to decide and the Dr told us it was not emergant that we decide during this visit, that we could take time to think and discuss it. And of course, since this mutation she has is pretty rare, there aren't any cases to really look at and see how well this procedure has worked in the past. So we wanted to come home, spend more time with Elizabeth, talk to our families about it and hopefully find some guidance. Elizabeth also has an appointment coming up with Cardiology, and I really feel we need to check the status of her heart and artery dilatation with him to be sure there aren't any other issues going on that may need to be resolved/checked first.  And then we will follow up again in Texas in October with transplant for her lung check up. So, all that to say, please pray for guidance on this decision for us, and continued stability for Elizabeth's airway. And of course that her angel lungs continue to do well. Thank you to everyone who called/texted/messaged us during this trip. It was definitely the most stressful and worrisome since transplant for us, and I think Matt an I both are really concerned about making the best decision for Elizabeth.

Excited to be home with her toys/books~ I let her destroy the room :)