Medical Update

This past April we went to Denver to see Eden's medical team. Before heading up, we were prepping for the high possibility that this was going to be the decanulation visit, and we would come home without a trach. Prior to the visit, they arranged for a respiratory therapist here to do a positive pressure airway check. This test would give us an idea of her airway size and some breathing capability. Going into that test, I knew her level needed to be a 10 or lower in order to be in a position where her trach would come out. Her results were a 10..... so this made me a little worried, but she was still within the range they gave us.

The week before going up to Denver, Eden also began wearing a cap over her trach so her breathing was completely bypassing the trach and she was doing really well with it. All except for one little incident. She was really mad about something and began crying hard and stormed off to the other room. From around the corner I could hear her coughing and coughing in between her cries and it just sounded really strained. So I called for her to come back and as she rounded the corner her face was totally purple like she was really struggling for oxygen. So I pulled that cap off and everything was back to normal quickly. Other than that one time, wearing the cap all day every day seemed like it wasn't complicating her breathing at all. But that one incident left me a little worried.

Despite these little worries though we were beginning to get more and more excited that the time was really here. We know from experience though that letting ourselves "go there" and imagine such good possibilities had set us up for some pretty hard emotional realities in the past when things didn't pan out the way we had hoped. So with guarded optimism, we went to Denver Children's hospital for a bronchoscopy with Dr. Prager (ENT) and Dr. Deterding (Pulmonologist).

As soon as the team of doctors sat down to consult with us after the procedure, I knew it wasn't good news. Dr. Deterding spoke first and let us know she was highly concerned about a couple things with Eden's respiratory situation. First, her lungs looked really bad. From the images she had, she could see that there was a lot of mucus which lead her to be concerned about possible bronchiectasis. (Bronchiectasis is a condition in which damage to the airways causes them to widen and become flabby and scarred. Bronchiectasis often is caused by an infection or other condition that injures the walls of the airways or prevents the airways from clearing mucus. In bronchiectasis, your airways slowly lose their ability to clear out mucus. The mucus builds up, and bacteria begin to grow. This leads to repeated, serious lung infections and each infection causes more damage to the airways.)

Secondly, she was concerned about Eden's tracheomalacia. Instead of being rigid, Eden's lower trachea is soft and floppy. While observing Eden with her scope, she could see that as Eden coughed, this part of her lower airway would collapse. (This is the reason she turned purple that time while wearing the trach cap) So Dr. Deterding felt this tracheomalacia was part of the reason Eden's lungs looked so congested. She was having a hard time clearing that mucus from her lungs because her airway there was closing and not allowing as much mucus to pass through.

Then Dr. Prager began to fill us in on Eden's upper airway. In 2011 he reconstructed her upper airway which is above the trach right now. Unfortunately, it hasn't grown. The reconstruction site and grafts that he put in are stiff and constricted and aren't progressing at all. So her airway is bigger than it was before we started all of this, but it isn't big enough to sustain her long term. This explains why she was doing so well with the capped trach, but the problem is that as her body grows, her airway won't grow and without further intervention, her airway would quickly become too small again. So the fix is to reconstruct again.

Brad and I were left a little stunned, and the team of doctors were also very surprised and disappointed with the great turn of events. We had lots of questions but they didn't have lots of answers at the time. We set a plan to return in 6 weeks, and in the meantime we would begin antibiotics (for bacteria in the lungs), vest treatments and some nebulizers on Eden daily to see if that would improve her lungs. Dr. Prager also said he would consult with other ENTs to determine a plan for how the reconstruction surgery would go.

And that's basically how we left it. So at this point, I'm sure one can assume that we were pretty devastated. What kept going through my mind was how horrible the recovery was from that first reconstruction. But even more than that, I just had so much confusion and fear over the realization that we are nowhere near being done with this trach and that the process of getting it out was going to be much longer and much more complicated that I thought it would be. I naively assumed that the hard things were all behind us and that the trach would peacefully be removed and we would move on with some horrible memories from previous years that got us to that point. However, we aren't done with the hard stuff, and it's going to get a lot worse before it is going to get better.

So fast forward 6 weeks which put us in Denver last week to reconvene and continue some more testing. Tuesday morning, Brad and Eden headed in to the hospital for the placement of a PH probe. Upon reconstructing, Dr. Prager wants to rule out any complications that could possibly be fixed beforehand, and reflux is one of them. So the PH probe is a small tube inserted into her nose, through her esophagus and down into the stomach. It needed to be in place for 24 hours to monitor any reflux issues. We did one a few years ago, so this wasn't new to us, but it isn't something Eden remembered so we knew she would need a little prepping before. She was really scared, but she handled it so well (I'll have to let Brad tell that story).

Then Wednesday morning we got to the hospital for the lineup of events. First they removed the PH probe, then we went in for a CT scan of Eden's lungs. She was such a champ following directions by holding her breath on and off as that big machine swirled around her. A hour or so later, Eden was in for a swallow study to make sure she wasn't aspirating any liquids into her airway. Once those things were all done, we had a couple hours before we had to be back to meet with the doctors to get all the results and find out what the plan of action was going to be.

Results:
(1) The PH probe showed no signs of reflux, everything was normal.
(2) From the swallow study, it looks like Eden is doing great controlling her liquids and they don't have any concern over aspiration.
(3) The CT scan of her lungs showed great improvement (which was very relieving news). Though the tracheomalacia is still a great concern, it looks like the antibiotics and daily treatments really helped clear up some of the mucus problem. Dr. Deterding was very happy about the improvement and recommended we keep doing this daily routine for the long run. She thinks this lung treatment is something Eden will need for years to come.

So all those things were great positive reinforcements, but the tracheomalasia is still something Dr. Deterding continues to be concerned about, especially once Eden's airway is reconstructed and the trach comes out. So this brings us to the airway update with Dr. Prager. August 29th Eden is scheduled for her airway reconstruction revision surgery. Like last time, he will remove her rib/ribs to get the cartilage to use as the grafts for her airway. She will spend anywhere from 10-14 days in the hospital for recovery. This time though, the plan is to remove the trach and sew her trachea and neck closed. What we do know is that it's great news that the trach will be out, but unfortunately there are A LOT of unknowns. For the first couple days post surgery she will have a breathing tube through her nose, down her trachea and into her lungs. Once that tube is removed, we won't know exactly what to expect from the tracheomalasia and the area that he is reconstructing. For reasons not really known, the grafts from the surgery in 2011 never really took well and didn't continue to grow with her body. We are praying that this time the grafts will do what they are supposed to do which is heal nicely into the airway and begin to grow as she grows.

So we continue to have lots of questions and concerns, but unfortunately we won't know answers until we begin the process. Whatever complications arise, we will just have to deal with them as they come. Hopefully with the trach being out, her airway and grafts will heal as they are supposed to because the trach won't be in there possibly hindering proper healing.

My ultra-organized and plan loving self struggles greatly with unknowns, especially when it comes to medical issues with Eden. And these are BIG unknowns which possibly mean lots of surgeries and hard things. What I do know is that we will be traveling to and from Denver A LOT for the next several months post surgery. Eden will be put through a lot, but unfortunately it's not just about her anymore. In 2011 it was just Eden and I so traveling back and forth every month was easier and I was with her 100% of the time. But now there are two other little babies involved that also need their momma and can't be in the hospital with us. This will be messy but I know we will get through it. I've already had so many offers to help and I'm so grateful for it. I will need help that's for sure, but I struggle with asking for it so I'm so very grateful for those who have willingly offered to do anything possible.

So for now, we will continue to enjoy our summer and put these scary thoughts aside as best as possible. Come August 1st we will start proactively preparing for surgery with more medications and nebulizers, and making plans for traveling and child care, etc.

Because of all the upcoming travels and medical procedures, we have also decided that this August isn't the time for Eden to start kindergarten. With a summer birthday she is close to the deadline anyway, and we feel confident in the decision to wait until next year for her to begin. I'm very grateful we have this option so that she won't also have to deal with the struggles of a new school and anxieties about coming and going and not feeling settled in the routine. She will have enough on her plate with the medical issues that I'm glad the remaining time can be spent at home with us for a little longer. We appreciate all the love, support and prayers on Eden's behalf. It's looking like it will be a bumpy road, but we are grateful for so many loving and supportive people on our side. :)