Cincinnati, little Levi is headed your way!

I have spent the last 48 hours on the phone and in meetings with doctors…let me do my best to catch you up on everything we have learned. Let’s start at the beginning….some of this information may be a repeat but it is important that you get the whole picture.

Levi was born at 34 weeks gestation and in the delivery room they noticed the had a stridor (noisy breathing) indicating something was blocking his upper airway. On the first bronchoscope to check out his throat it was just thought that there was some irritation from the oxygen tube going in, then out, then back in again in short amount of time. The doctors said he just needed some time to heal so we tried steroids and rest. But Levi’s stridor stayed strong so after a few weeks we did a second bronchoscope.

With all the swelling gone from his airway the pulmonologist was able to really see what was going on. He noted that Levi had paradoxical vocal cord movement. Basically, when Levi breathes in his vocal cords shut tight instead of opening. He has been diagnosed with bilateral vocal cord paralysis (BVCP).

Here is a visual but Levi’s never really open all the way; in fact his close tightly like the bottom picture when he is upset and tries to breathe in:

There are two groups of BVCP- for the first group there is a known cause for the issue, like a specific brain malformation. We were able to rule out the brain malformation through an MRI telling us that Levi’s BVCP falls into the second category: congenital (from birth) idiopathic (unexplained).

BVCP is a very tricky diagnosis. So for kids in Levi’s group, if you do absolutely nothing 50% of them spontaneously get better (the nerve “wakes up”) within two to four years and 50% never get better and have to have a trach for life. But for Levi we don’t have the option to do nothing and wait and see, as he has a “critical airway”- he is a danger to himself because when he gets upset and cries his airway closes tighter and he can’t catch his breath.

The only option we have in Chattanooga is to make his “critical airway” safe with a tracheostomy and hope that he is part of the 50% of kids whose nerve wakes up after a few years. It would also not be safe to try eating so we would have a trach, a g-tube, and would simply be waiting, hoping, and going in every few months to see if he is ready to have the devices removed. But no guarantee that that day would ever come.

Last week I called around to some nationally known hospitals that deal specifically with airway issues. I spoke with Children’s Hospital of Philadelphia (CHOP) and learned about a new surgery they had learned from Cincinnati. They have been in communication with me all week and have been VERY eager to get their eyes on Levi’s bronchoscopy videos. They even called yesterday and gave me the head surgeons HOME ADDRESS for me to send the video to!! That is wild!

I presented this new procedure to the team here in Chattanooga and they had never heard of it, but were very intrigued by it. In fact, they are so interested in it they have ordered the equipment needed to start trying to complete it here (after more training and research of course). One of the pulmonologists said he knew the head airway surgeon at Cincinnati Children’s Hospital and wanted to reach out to him before following up with CHOP. We were thrilled by that as Cincinnati has more experience with this procedure so we gave him the green light to send the video to Cincinnati.

A whole week went by and nothing was sent. Finally last night the video was sent to the Cincinnati surgeon and he called me this morning. He said that based on the video it seemed that Levi was a perfect candidate for this procedure. Let me summarize our conversation…

The new procedure is called an Anterior Posterior Cricoid Split (APCS) and has only been done on a handful of babies. This particular surgeon has done 12 over the last 5 years, and between him and his colleagues they have done 20. He said their success rate is now at 80-90%, and we learned from CHOP that they had completed 3 over the last year with 2 successes. “Success” in this situation is being able to avoid the trach.

Now here is where things get tricky….

This is considered an “experimental procedure” so we don’t have any long term studies on the outcome. There is one other experimental procedure with a shorter recovery time that can also be tried but he has only done 3 of those, so we are leaning towards the APCS.

The surgery is done endoscopically  and in really basic terms, they would surgically put a tube into his airway on the inside that would hold open the incisions they make in the cricoid. It would require him to be intubated (on a ventilator) and relatively sedated for 10-12 days while the throat heals around the tube and when they remove the tube the hope is that the airway gap that was created will allow him to breathe freely. This procedure has to be done in the first 9 months of life as the airway is still pliable at that young an age.

There are pros and cons to the APCS. The wider airway will allow him to breathe better but also put him at a higher risk for aspiration (liquids like milk, saliva, etc getting into his lungs). He also could end up with a raspier voice as his vocal cords won’t close as tightly after the surgery, and once you widen you can’t undo it. There are also no long term studies to look at and rule out any unknown future issues.

So basically here are our two options:

Option 1: Stay in Chattanooga and put a trach and g-tube in Levi, hoping that we would be able to remove them in a few years.

Option 2: Transfer to Cincinnati and do this experimental procedure. Once the airway is open we can tackle feeding by mouth, and if that goes well then we will go home device free. If the APCS fails, he will leave Cincinnati with a trach and a g-tube.

Jake and I both agree that option 2 is worth attempting…in our opinion it can’t necessarily hurt anything to try. Almost all of the children who are considered “successes” who have had this done are still problem free at 4-5 years old (the first APCS is 5 years old right now), with one of the 4 year olds showing signs of airway issues again when he runs around for long periods, but it is not bad enough to do anything at this point.

We are working with insurance right now to arrange the transfer. An ambulance will take Levi and I to the Chattanooga airport where we will meet an air ambulance and continue to journey by plane to Cincinnati. We are told that once we are there the surgeons would like to move quickly and within a day or two get him into the operating room. He will be intubated for 10-12 days, then weaned for a week from pain/sedation meds and they will then look at his airway. They will look again at the two week post intubation mark and if all looks good we will be discharged and sent home to Tennessee. So it looks like best case scenario is a 4 week stay.

We would obviously then make several follow up visits to Cincinnati over the next several years to keep a close watch on the airway and make sure we remain problem free.

We should hear from insurance tomorrow and make the trip in the next few days!

I have no new updates to share with you about his brain damage, and won’t really have many until he gets older and we see the extent of the damage manifest.

Here is a sweet video of Levi:

I was tested today by Levi’s hematologist for some autoimmune disorders as we are hoping to find the reason for the brain damage and we can’t test him at this age. They were able to send off 7 vials to Memphis and even though I have already tested negative for these disorders, there is a special way this particular lab can spin them again to see if there is a small factor that has been missed. We are hoping to find some answers so we can be assured that the brain damage event won’t happen again.

Levi has been so sweet the last few days. He has officially hit the 5 pound mark and is officially 4 weeks old today! He has been VERY intrigued by his aquarium these days and loves to listen to worship music with mommy.

I had a friend send me a text with a Bible verse in it, and I got chills when I read it…

Psalm 27:14 “Wait for the Lord; Be strong and let your heart take courage; Yes, wait for the Lord.”

I have been SO FRUSTRATED for the last week as we have simply sat and waited for others to follow through with their promises. We have been praying that all of this be done in God’s timing, but the earthly side of me couldn’t stand the lack of follow through as I sat by my son’s bedside each day watching him struggle to breathe! My anger yesterday was at an all time high as we were told that the video still had not been sent.

But as I read this verse it just seemed to speak directly to me. WAIT FOR THE LORD. We have asked God to open doors that are meant to be opened and close those meant to be closed. We have put this into the hands of God and asked for His wisdom to guide us in the direction we are meant to go. Now our job is to be strong, take courage, and wait on God to do what we have asked him to do.

I have SO MANY things that I want to share with you all that God has been speaking to me, but I truthfully can’t even formulate thoughts into sentences right now! My brain is absolutely fried, but expect some deeper thoughts in future posts haha!

We are still very much in need of your prayers. Obviously a transfer adds a whole new level of complications to our family situation. Jake and I are going to have to part ways for a bit and each focus on one kid, with the goal of getting all four of us to Cincinnati soon.

We also need prayers for health for all four of us, and strength as we navigate through the transfer process. And most importantly, it looks like Levi might be having his big surgery within the week and we need prayers that it is completely successful and problem free.

Thank you all for following our journey, and I hope that have some good updates for you soon.

God bless,

Leah