Monday, August 26, 2013

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 :)

Sunday, August 11, 2013

Hope and Fear

Just wanted to do a quick update on things around here. Most of you family, friends, and those who I am friends on Facebook with already know our big announcement. But we are expecting another little girl in January 2014. We put a lot of prayer, thought and research into our decision to have another child. We met with the genetics Doctors at Texas and a genetics counselor here at home and were told the chances of Elizabeth's condition reoccurring were 1-3%. Due to Elizabeth's genetic mutation being X-linked, and me not having any of the genetic abnormalities they think the mutation Elizabeth has was sporadic. We realize that life with Elizabeth can sometimes be difficult with the care she needs but we also realize that life must go on for us and Elizabeth. We can not live in fear of the unknown (easier said than done!) But have faith that God will give us no more than we can handle. And that is how I am handling this pregnancy and the fear I feel daily. I am excited to be a mommy of 2, and even more excited for Elizabeth to have the chance to be a big sister, and for this baby to meet, and love Elizabeth just as we all do. So with all of that, we went into this pregnancy more educated than when I was pregnant with Elizabeth. In an effort to be as prepared as possible, I decided to have an amniocentesis done with this pregnancy, I had one with Elizabeth and was assured the results were normal. But now we know what genetic mutation to look for specifically. So last Monday I was seen by the high risk doctor here in Chattanooga. They did an anatomy scan, and the amnio on the same day. Based on the ultrasound this baby appears normal and healthy, there are NO abnormalities visible like the ones we saw with Elizabeth. The results from the amnio will take a few weeks to come back, but we are hopeful for only good news. We chose to do these tests so we could be best prepared to care for our little girl, regardless of the outcome we will love her just as much.

As for Miss Elizabeth, she is doing so amazing! She has continued to make progress with saying more words. And has started trying to walk when you hold onto her hands. She really is making the most of those angel lungs :) She started her 2nd year of preschool last week in a new classroom. Thankfully most of the same kids are in the class, just new teachers. Every milestone she meets truly another blessing for us, there were many days I worried she would never make it this far. We head to Houston this week for testing and possible airway surgery. Matt and I both are so unsure of what to do, what decision will be best for Elizabeth. We are hoping with more information from the tests and the consultation with the surgeon, we will have more information to make this decision. We want what is best for her, sometimes I really do hate having to make these decisions that affect her life so much. I know thats part of being a parent, but sometimes it is a lot to take in - having to make choices that could mean life or death for your child or choices that could totally affect their quality of life. But with all the other decisions we have had to make, I try to remind myself that not matter what WE decide, someone bigger already knows the outcome.

I will update more as we learn more during our trip to Texas, and as we get the results on Baby #2. Thank you for your continued prayers and support! We will definitely need them as we embark on this new stage of life with Elizabeth!