Get That Thing Outta Here!

Who needs a trach? Not this guy!

Our little Superman, prepped for his big day

Decannulation was a huge success! Zeke rocked it like he has everything else, which means he turned it up to eleven. When you need that extra boost, you gotta take it to eleven. The day wasn't quite as full of pomp and circumstance as I expected, but I will take it. After first scoping his airway to make sure there were no obstructions, we got the go ahead to "make it so." Jenny did the honors and pulled the trach out with expert care. Were there fireworks? Were there the cheers of adoring crowds? No, there was not. Zeke coughed and then went back to Mickey Mouse, seeming not to notice.

Our first family shot sans trach

Zeke had to spend the night in the hospital for monitoring, but did amazingly well every step of the way. He still needs a little oxygen at night and during naps, but that will hopefully disappear over time.

The success of decannulation also means that it is kidney time! The transplant team wasted little time in picking a date. Are you ready? On July 28th, Ezekiel will get a new kidney. That's right, they will take a kidney out of his Aunt Michelle and slap it into his abdominal cavity. This still seems like black magic to my primitive caveman brain.

"Do ya mean it, Pa? I can have a kidney, for true?"

Transplant will be such an amazing step for his health, I really don't know what else to say. It feels like this is the end of his journey. After all he's been through, I wish that were true. But let's not forget that his path from here is much brighter, and less covered in puke. I dread waiting the 5 hours for him to emerge from surgery, but I wiggle with excitement at the thought of seeing him on the other side. Something tells me you do too.

A sincere "Thank you" to all of you for your continued support. It means the world to Zeke and his Mom... and his Dad.

Caleb

Tomorrow is Only a Day Away

Hark, who goes there!?

As always seems to be the case, much has transpired since last we spoke. It would seem as though I should update you all more often. Although, keeping you in suspense (or "suspenders" as Goofy would say) is so much more exciting.

"Oh hey, didn't see you there."

Last time we talked Zeke was nearing decannulation (the removal of his trach). Sadly, just days before that process was supposed to be scheduled, he got sick. We were able to manage his symptoms at home for the first day or so, but come that Sunday afternoon, we could no longer keep his oxygen numbers high enough and had to take him in to the ER. He was admitted to the hospital with a respiratory infection called RSV. I paid close attention when those results came back so I can tell you that RSV stands for Respiratory Somethingsomething Virus. S-M-R-T! What that meant is that he had some inflammation in his lungs, and without the adequate reserve lung function to make up the difference, he needed support again. He ended up back on the ventilator and quite a lot of oxygen. There wasn't much we could do other than let the virus run its course. After 12 days in the hospital, his oxygen needs were finally low enough that would take him home.

Hospital oxygen just tastes sweeter

While in the hospital, we had a care conference to talk about what this illness meant for his timeline. It was obvious that, at the very least, decannulation would need to be pushed back a couple of months. It was possible that he would need even longer to recover, which could potentially push everything back to next year, transplant included.

While we were distracted with all of that, Zeke was plotting. As part of his prime directive, he sent us into a worry spiral. While we were busy fretting, he was busy destroying the viral invaders. After a couple weeks at home, he was able to be weaned off the vent and has now been completely off for several weeks. He's doing so well that I think we're going to pull his trach out tomorrow. He doesn't need it anymore and Jenny and I have decided it's time.

Bubbles can be enjoyed with or without a trach

Ok, fine, we didn't decide it was time, his doctor did! On Tuesday the 21st, Zeke is scheduled for decannulation! They'll have to scope his airway first, but as long as that all checks out, that trach comes out. This is such a huge landmark for him and his health... what else can I say, the dude is a rockstar!

This is all excellent news for transplant as well. With his bladder fixes on hold until after transplant, only the trach was standing in his way. If all goes well on Tuesday, he could get his new kidney in late July or early August! Where is he going to get a kidney from, you ask? Oh, haven't you heard?

"Hey, Aunt Michelle, look into my eyes. You are getting very sleepy. You will wake up in a tub of ice, missing a kidney. Do not be alarmed."

I want to take this opportunity to thank everyone that got tested and those who offered to get tested. It means more than you know. Most of all, though, we have to thank Michelle. I've said it before and I'll definitely say it again: Getting a kidney will transform this boy's life. The constant nausea, the puking, the lack of energy, the discomfort, the dialysis: gone. How amazing is that? This truly is the greatest gift she could ever give to our son. In return, we'll give her... uhm, I don't, uh... Well, I guess we can make Zeke write her a card every year on her birthday, or maybe just a case of beer. Yeah, we got this.

You are a Jedi. Like your father before you, and his father before him.

I promise to update you all on tomorrow's events as soon as it's possible. Until then, try not to stay up all night worrying (I'm looking at you, Jenny)

Caleb

A Year in the Making

Come one, come all, a tale I shall tell. It is a tale of a young hero, surrounded by peril... and toys.

This entry says it's a year in the making because on the 23rd, Ezekiel will have been home for one whole year. As is normal for any child his age, many things have changed in a year. Behold:

Where once there was chunk...

... now there is hawk

Since the last time we talked about Big Baby Z, many things have happened. He had just started spending time off the ventilator and now he's off around the clock. Once he progressed to being off all day, he had a sleep study done at the hospital and was cleared to be off at night as well. He now sleeps with just some heat and humidity delivered via a trach mask to help keep his mucus from getting thick and blocking his airway. He still needs a little oxygen at night, but that shouldn't keep us from progressing to the next steps. What is the next step, you ask? Why, getting that trach out, of course! Assuming there are no hiccups, they're hoping to pull his trach in late April. They'll go in with a scope to check out his airway, and if that all looks good, out it comes! How awesome will that be?! This will have such a significant impact on our daily lives I'm not even sure where to start. We will finally be able to have babysitters that aren't specifically trained on how to manage a trach, or more simply stated: family. We've decided to hold our very own Hunger Games to see who gets him first. All the trach related equipment will be no more, allowing us to move about the house more freely and travel without loading the car down with emergency supplies. Oh, and the obvious upside to his overall health and well being, can't forget that one.

"Reveal your secrets to me, you blasted contraption!"

I mentioned last time that he might need some help with the bladder, and that has since been confirmed. He went in for some tests and those showed how bad his bladder really is. The excessive swelling in utero caused the bladder wall the scar and the lack of use since has caused the entire bladder to shrivel. There has been talk of a surgery that would use Ezekiel's own ureters to expand the bladder, but that specific procedure is still being debated. What we do know, is that his bladder needs some help, the exact help is still TBD.

He got some on the paper... 20% would be a generous estimate

On to the biggest, baddest piece of them all... the kidney. This drama on the kidney front has continued unabated. I have since been removed from the donor pool and Jenny is back in as a last resort. My brother Micah and cousin Adam were in, then out, and now might be back in (or out). Jenny's sister, Michelle, is currently the next one up to bat for her all day evaluation. These are just the highlights of all our kidney drama. There have been canceled evaluations, evaluations that were cut short, and someone has been playing dosey doe with our donor order. It could be any one of these people, or maybe none of them, who knows. Whatever the outcome, Jenny, Zeke and I will be eternally grateful to them for their willingness to go through so much for one so little. The generosity of this act cannot be overstated, so no matter how many kudos I give them in person or on the world wide web, it will never be enough. Getting a kidney will transform this boy's life and for that, we cannot wait.

Catch!

That's probably enough sap for now. As my main man Yoda would say, "Judge me by my size, do you?"

Caleb

Bigger, Stronger... Faster?

They say practice makes perfect, and this kid has been practicing for this moment for his whole life.

Notice anything?

For those of you that may need more of a hint than just the above picture, Zeke is now spending time off of his vent! When last we talked, he had just started his weaning process and now we've reached the third stage. This time off the vent started last week with 15 minute intervals and has increased to nearly over 3 hours a day. We will continue to increase until he is off of the vent for all waking hours and then we'll start weaning his support while sleeping. Having him off his vent is just amazing. We finally get to hold our son without any tubes or cords getting in our way. We celebrated this occasion by running around this house with Zeke held high, superman style. We usually just camp in one room for the majority of the day since moving all of his equipment is time consuming, but no more! Now the world, or perhaps just our house, is his oyster. Along with this progress comes another first:

Wait for it...

During these times off the vent, Zeke can use a speaking valve! This is a one way valve that allows him to breathe in through the trach but forces him to exhale through his mouth and/or nose, allowing him to talk. He's been making little noises past his trach for a while now, but it's a lot of work to do it that way. With the speaking valve attached, he babbles away like a crazy little man. Hearing his voice is just awesome. For now he's just making random noises, but that's where we all start. Some of us barely ever make it past incoherent blathering by adult hood, so we're not too worried about him catching up.

Milo is creepin'

Now, on to the kidneys... or in this case, the lack thereof. We have now taken a few steps towards transplant. Zeke had his first transplant evaluation about a month ago, which was an all day appointment that was largely informational. We met with lots of people to talk over the specifics of how this will all go down. Spoiler alert: It's going to be terrifying. The other thing that happened that day is Jenny and I were tested to see if we were a match with Ezekiel for kidney donation. Jenny and I both passed the first step, but sadly Ezekiel has some antibodies to some of Jenny's blood, which means she won't be able to donate this time around. That's alright though, she's already done so much for this kid and she doesn't really need another scar. Just a couple weeks ago I went on to the next step in transplant evaluation, a full day of tests. The thrust of this day was to give me a total work over to make sure that I'm healthy enough to give up a kidney and go through a major surgery. We also got another round of information, this time geared towards the donor instead of the recipient. We're still waiting on some of the results from that day, so we'll hopefully know more soon. If I pass this round, then there will just be one more round of tests as transplant gets closer to make sure everything is still on the up and up. If it all checks out, I've been told they'll scoop the kidney out with a giant ice cream scoop and duck tape it to Zeke's side... pretty sure that's how it works. As you can see, I was paying close attention during both transplant appointments. Top of the class!

Kidneys are not required for standing.

Another thing that has come back into consideration is Ezekiel's bladder issues. His bladder was badly damaged at birth from the stress of being overloaded in utero. This is important because it doesn't make a ton of sense to plug a new kidney into a bladder that's incapable of doing its job. To that end they did a bladder ultrasound recently and discovered, though being no surprise to anyone, his bladder still looks to be in rough shape. They'll have to go in with a scope before they can be certain of the extent of the damage, and the results of that scope will determine what sort of treatment he will need. Apparently they can go as far as building him a new bladder using some of his small intestine! I've decided that the only way this could be possible would be with the use of black magic. On an unrelated note, I've started collecting goats. If you have any to sell I'll take them off your hands. I promise they'll be treated well... super well.

"I already did 50 push ups this morning, 51 is a running total."

Other than those silly medical things, Zeke is doing awesome. His physical development seems to have kicked into high gear in the last few weeks. His newfound freedom from the vent has definitely helped. Without his vent circuit in his way, he'll roll across the living room and cruise through the kitchen in his walker. Before long, we won't be able to keep track of this little monster.

Movin' and a groovin'

That's it for now! As they say in the biz, may the force be with you.

Caleb

We've Missed You

Many moons have passed since last we spoke, one of them was even red! What's our young hero up to?

Enjoying his first beer, that's what

It's been a good long while since our last update, for which I apologize. I wanted to make sure I had something more interesting than new teeth and nights full of baby vomit to share with you, and now I have just that. Since Ezekiel's respiratory status is driving this ship, let's start there.

A few weeks ago, we started the weaning process. We were waiting for him to be able to sit up on his own, and now he finally can. At first, this entails simply changing the respiratory rate on his ventilator. His vent does two things for him currently: it provides breaths from start to finish needing no input from him whatsoever, and it also supports any breaths he takes on his own. The first step of the weaning process is to slowly reduce the number of breaths that the vent gives him, forcing him to initiate more breaths on his own. That number has dropped a couple times and will continue to drop as he tolerates it. So far, he has handled the wean pretty well. Once we get to spot where Zeke is initiating all of his breaths without issue, we can start trials off of the ventilator. This is exactly what it sounds like, we'll actually detach him from the vent for very short periods of time. This is a landmark step in his development. Don't forget, this boy has never breathed without support in his life (save for the quick periods when he's detached for trach changes and what not). If he does well, the time he spends off the vent every day will slowly increase until he is free of the vent all together. Zeke's primary lung doctor feels that if all goes well, he could be free of his trach and vent as soon as next summer! This would obviously be best case, but it's very exciting to be working toward this goal.

The "Little Chief" trying on his Great Grandfather's Fireman's Helmet

This timeline also affects the timing of his transplant. I mentioned this before, but just as a quick reminder, he needs to be trach free before he can get his kidney transplant. So, if his trach comes out in the aforementioned timeline, he could potentially get his kidney next summer/fall! The transplant process is rather lengthy so it's kicking off soon, we have our very first appointment at the transplant clinic later this month. This is a long (4-6 hour) informational appointment where we will get the low down on all the steps to come.

If all of these things go well, he could free of all of his tubes as soon as fall of next year! That's obviously a little hard for us to picture, but man will that be awesome. With a new kidney, he'll finally have more normal energy levels and be free of the constant puking. It'll be like meeting him for the first time all over again.

"Hug me while I drive."

Now that we have the big things out of the way, I'll throw down some more details if you care to continue to read about our offspring. The little monster has a few teeth now and likes to show them off. He still gets weekly physical and speech therapy. His physical development is coming along slowly, but they're pretty pleased with his progress. He's definitely behind, which can be a little tough to deal with, but the fact that he's making progress is the more important part. His speech development has hit more a snag, sadly. He was eating orally pretty well for a while, but a few weeks ago he stopped taking anything by mouth. Nobody is really sure exactly why this happened, but apparently this isn't terribly uncommon with kids who have had an oral aversion.

3-0, baby!

Lastly, I have an exciting story that relates to Ezekiel's prime directive: my ulcer. He normally gives us plenty of reasons to overreact and obsess about relatively minor issues, but about a month ago he decided to step up his game. The story starts in the middle of the night, as all good stories do. Zeke was having a rough night. Rather frequent retching and puking are a nightly occurrence for the little fella, but this night was especially bad. Jenny and I take turns sleeping in his room on the nights we do not have nursing and we had just made our 4AM switcheroo. Neither Jenny nor Ezekiel had slept for the first half of the night due to Zeke's obvious discomfort. Minutes after I took over, Zeke began retching again and what he produced advanced his prime directive by a few weeks: blood. The poor little guy had a bloody puke, followed shortly by another, much larger, one. I woke Jenny since she had been sleeping for all of 5 minutes (lazy!), and we rushed to the ER. He had another bloody puke in the car, but once we got the ER, it had stopped. They quickly ruled out some rather serious possibilities that can cause bloody vomit, but wanted to admit him given his complicated medical history. He spent a full 24 hours in the hospital and in the end, they assumed his feeding tube had caused a laceration in his stomach that had since closed. He apparently just wanted to spend a day with his old friends back at the Children's Hospital. All in all, he got a couple new meds to help prevent it from happening again and they sent us on our merry way the next morning.

Puking blood ain't no thang!

Today Ezekiel is 11 month's old, so I leave you with a picture from his mother's birthday a few weeks ago.

"I got you this card, and these magical flaming cupcakes!"

Until next time!
Caleb

Sum Sum Summertime

Welcome, welcome. Please, come on in, pull up a chair. I have a story to tell.

Everything the light touches, is our kingdom

Amidst all the exciting current events, our young hero continues to kick some booty. We've had more appointments than I can count since last we talked, but things still move much more slowly once you're at home. One thing that Ezekiel does continue to make progress on is what I like to call his prime directive: giving me an ulcer. We've had a few scares, an emergency appointment, and other sorts of excitement, but nothing has turned into a terribly big issue. My growing ulcer notwithstanding.

One of the bigger incidents was a set of lab results that were way out of whack. The main culprit was his BUN. This is a value that measures waste product in his blood and was what they used as a marker for when he needed to start dialysis. It also tells them how effectively his dialysis is working. Someone with normal kidney function will have a BUN of less than 10. Ezekiel, and other people in kidney failure, will have an elevated BUN. He usually hovers in the 30-60 range. Two weeks ago him BUN was 130. That's 22 points higher than when he started dialysis for the very first time back in the NICU. A BUN that high will make you feel sick and that was definitely the case for Ezekiel. Thankfully, after some changes to his dialysis prescription, his BUN did eventually drop back within acceptable ranges, but it took a couple weeks. This is what I mean when I talk about my ulcer. It's stuff like this that gets sorted out eventually and doesn't even really make that great of a story, but it's gotta be there, taunting me. There are a few other stories like that, but they all follow this formula: (X being everyone's favorite variable) We noticed X. We got really worried about X. We called the appropriate specialty about X. They had us come in so they could look at X. They didn't think X was bad enough to do anything specific. They asked us to keep and eye on X for a few days and let them know if anything else happened. A few days later, X was gone/stopped happening. Great story, eh!?

I can finally roll over!

There's still not much to update you on the lung front. He will still go weeks without needing oxygen, but so far he hasn't made it longer than that. We have an appointment with Zeke's primary lung doctor this coming week, so we hope to have more to report after that.

On the developmental front, our young hero continues to hone his skills. After he came home, Zeke really seemed to plateau with his physical and oral development, but we've since gotten some professional help on both fronts and he's responding very well to the therapy. He even seems to have a good time while doing it!

Zeke loves showing off at physical therapy

He's also working on growing some teeth. He's had lumps on his gums for almost 2 months now, but those little runts have refused to burst through. With Zeke's oral aversion, he doesn't take much for teething toys, so he mostly just powers through.

Delicious peas!

I think that's about it for now. Not a terribly exciting update, but an update none the less.

A Month At Home

Welcome back, so nice to see you again!

If any of you are on Facebook, I assume you're aware of the fact that Ezekiel is home and we like to post pictures of his cuteness. To those who are annoyed by people posting pictures of their "stupid" kids, I would apologize, but it wouldn't be sincere. As a peace offering, please enjoy another picture of my spawn.

Boom

Since it's been a whole month since we've had an update, let's rewind the clock to when we spoke last: the day before discharge. I'll set the scene. Our young hero is just a few days shy of being 5 months old as his hospital stay draws to a close (significantly ahead of the projected 6 - 7 month stay). Jenny and I both just finished our 24 hour care sessions with no major incidents and only small amounts of vomit and other baby fluids on our clothes. On Sunday, the day before discharge, we made a flying trip to Boscobel, where my parents live, for a baby shower with my side of the family. This may seem like pointless exposition, but I bring it up for a couple of really good reasons. Number one:

Have you ever seen a cake this awesome?

Number two: On our way back, it started to snow pretty heavily. Ezekiel's streak continues! Clearly, we should have planned for this since it was a big day for him. We made it slowly and safely back to Madison in plenty of time to complete Ezekiel's first road trip. It was a little panic inducing to take him out of the hospital for the first time, but he seemed to be quite content riding in the car. He was wide eyed, soaking everything in the entire time.

So many new things to see!

That night, it snowed again. For those who forgot, it snowed the morning Jenny went into labor, it seemed quite appropriate that it would snow the morning we finally brought the little man home 5 months later. Yay Wisconsin!

The view from Zeke's room the morning of discharge

Discharge day required a little more waiting around than I expected, there were just lots of things that needed to happen in the proper order. I thought they were just dragging their feet so they would have more time to get all the floats lined up in the proper order for Zeke's parade. Sadly, I didn't see any floats on the way out of the hospital and have since written a strongly worded email to hospital management expressing my displeasure. They have yet to respond.

We made sure to stop by the NICU to give Zeke's former subjects one last chance to honor him

Our first week at home was crazy. We had just gotten him out of the car when the home nursing people arrived with lots of paperwork to go over. The next days were filled with appointments, home visits and countless phone calls to and from clinics, equipment providers, insurance and whatever else. That has slowed down a little, but all of those things are just part of our life now.

So here we are, a whole month later. There have been plenty of firsts and awesome experiences. We may sleep a little less, but we are ecstatic to have our little boy at home. We have also completely changed our stance on night nursing and now think that it is the most wonderful thing a young lad could have. We have a night nurse for two nights a week, and we look forward to those nights like a teenage girl looks forward to prom.

Please love me!

How is Zeke doing? He's doing great! As of yesterday he's a whopping 6 months old and he is a happy, smiley, chubby boy. I know, describing him as smiley still feels weird, but he is indeed smiley. The boy with the permanent scowl is no more, they must have swapped the kids at some point without our knowledge. He has pretty frequent appointments for the different areas of his health so they're keeping pretty close tabs on him. It's hard to measure specific points of progress currently, so I can't list any landmark improvements right now. But he gets stronger every day. For now, that's enough.

I've had a lot of people ask me when he's getting his trach out. Many of you may be wondering the same thing, I know I am! There is no way of knowing when, or if, that will happen. The doctors have said they think he'll be able to grow out of the trach, but there are no guarantees. He's doing really well, but don't forget that his trach is largely responsible for why he's doing so well. Weening him off it will be a very slow process, so it is here to stay for the foreseeable future. The timing of all this will also affect when he will be eligible for a kidney transplant. 18-24 months is the youngest they will transplant someone, but they don't like to transplant kids with trachs. That means that if he's still trached when that time comes, it could push his transplant back. So there you have it, I just used a whole lot of words to say "We have no idea what's happening."

Carry on, carry on.

That's all for now, thanks for reading!
Caleb