Goodbye 2020!

Glad to be Catholic...it may be messy and haphazardly decorated,  but I'm so glad to still have the tree up. It's such a bright spot in such a dreary year.  59 days until we move, that's our countdown paper chain adding all the color.  

This was at 4 am when I couldn't sleep.  I would have loved to clean up,  but let's be real.  Way too pregnant for that kind of energy. 

Had a quick last OB visit.  Induction is scheduled for Monday...4 days and counting! 3 really...but 4 more nights to get some sleep. (Read: No, not staying up until midnight!) Left via the COVID testing line.  That was less than pleasant. But accomplished!

We had some family fun...Chocolate Monopoly. (Lame, don't buy.)

Followed by Family Charades. I played as a beached whale and was a permanent guesser. No more apologies, I'm done.

Throw, Throw Burrito

I'm not gonna lie...I really debated the wisdom of this game. I mean, is it a good idea to give them something that encourages them to throw things at each other?

Well, wise or not,  they love it. You may be wondering where everyone else is...they're under the table because Jonah and Bryan have to dual. And they know it's likely to get a bit...crazy. No one wants to be a innocent casualty! 

Just Some Boys and Their Dad...

Playing old school video games. In the kitchen, like all cool houses allow. All. Day. Long. 

Christmas break at it's finest. 

Love 'em or Hate 'em?

 Came home from Mass today to a surprise at the door.

 Susan had already warned me that I probably wouldn't love what they sent, being that space isn't exactly something we have excess of here. Therfore, I was pretty sure whatever the excitement was from, it was courtesy the Martins.

I mean, who doesn't need one of these babies in their kitchen?!

It's well-loved. Bryan's in nostalgia heaven and already figured out how to get more than the 300 games it comes preloaded with. I probably won't mind it once we have a basement or some other less conspicuous place to put it....

Can't doubt the thoughtfulness or the love. 

A Christmas Miracle?!

So the meeting with the NICU doc and head of surgery (I think that is who he was) at Children's was full of very promising news! 

They saw no evidence of any GI tract abnormalities on the MRI! 

(Side note- MRIs are awful. I am sorry to anyone who has to go through that regularly. Even with my head out, I felt panicky. And then knowing you can't move for an hour? Oh my. And who knew they were crazy loud?! They did give me headphones with some beach sounds - water sounds and a pregnant lady...seemed silly but thankfully I didn't have to make them stop to use the bathroom. The worst besides the claustrophobia was actually the combo of having to wear a mask and having a nice, warm blanket on me. I was miserably hot and sweating. God is always good though, and between desperate prayers, the rosary, and the sleep deprivation as of late, I was actually able to doze for much of it!)

So, back to the results. No GI tract abnormalities gives us true hope that it isn't MMIHS! 

It gets even better. They claim that there is evidence of a thickened bladder wall. Which we did see on one ultrasound before, maybe around 24 weeks. And when I read through the report, it says they saw a normal abdominal wall. So those would both be unlikely with MMIHS or Prune Belly. 

They said they are "97%" confident that Judah has Posterior Urethral Valve (PUV). 

This is truly the best we can hope for with the current state of his urinary tract! It could be as minor as a simple surgery to remove a flap of tissue and no lasting effects! That would be assuming that the kidneys, ureters, and bladder have not sustained too much damage with the extra fluid to work properly. (If he has damage to any of those, the treatment will be ongoing and adapt to what we see.)

So the current plan is super exciting. As long as they are able to get a catheter in and keep the bladder drained, he can stay at Walter Reed with me until I am discharged. Then he will be transferred to Children's where they will use dye to check for PUV. (I actually think that they will do this at Walter Reed, but we will see.) 

If there is tissue causing a blockage, they plan to do surgery within 24 hours. Hopefully, it will be super simple with a laser. They expect the worst of it to be the anesthesia recovery. (And they estimated his current weight to be 10 pounds 15 ounces. They are pleased because the bigger he is, the better he should tolerate the anesthesia. I'm not convinced he's that big. I mean, I'm miserable but it doesn't seem any worse than any of the other kids.) After that is complete, if things start draining normally, we stay for a week or so monitoring his kidney function and then we should be able to come home!! 

Right now, we are expecting an induction on the 4th of January. So my goal is home by the 18. Just my personal hope, I even gave a few extra days of wiggle room.  Keeping it positive here!

So what if it isn't PUV? Then it is likely PBS or MMIHS. We actually should be able to tell if it is PBS right away, as generally the abdomen looks like a prune. MMIHS will be more of a concern if he can't eat or process food properly. 

What about his feet? Well, they said they couldn't confirm the bilateral club foot because he was squished too tightly in there. It is highly unlikely they went away, as we have had confirmation on every ultrasound since 20 weeks. We expect treatment will have to wait until after we get through the worst of the rest of this. They may not start treatment until we are in MI, since we will be moving when he is 8 weeks old. Frustrating, but so minor compared to the rest!

So the only thing, other than the feet, that doesn't match up with the PUV is the extra fluid I have. Polyhydramnios is common with MMIHS but PUV and PBS both normally have too little fluid, Oligohydramnios. When I mentioned that, they said they saw that in the doc notes but the fluid level was normal this time (AFI of 16). (I've been watching carbs and sugar because many people have had luck reducing fluid by doing so and have gone from can't eat, can't breathe, can't sleep to none of those issues. I knew it had gone down and I'm thrilled!) So...no comment, kinda like the feet. 

Bottom line? We are very cautiously optimistic. Both PBS and MMIHS are often diagnosed as Posterior Urethral Valve initially, mostly because it is far more common. Oftentimes it can take months to get the right diagnosis, despite the child having many difficulties. We really want the right diagnosis from the start, so he has the best treatment possible. Additionally, there is a chance that things could be dire, even with PUV, if his kidneys can't function properly- or at all. We are, again, optimistic because they see healthy renal tissue around both kidneys and with all the fluid we know at least one kidney is doing something! But we can't know how things will work when his body has to do all the work itself. 

SO PLEASE KEEP PRAYING!

Well, the countdown draws close to the end. We are in the single digits! Here's to a peaceful, productive week in which we can get ready to add another little guy to our life! 

Don't feel obligated to look through all the pictures, I'm mostly posting them for easy reference for myself and for the kids as they get older. But they are kinda neat looking, I've never seen fetal MRI images before.

Cute little squish, you can see his little toes in this shot. And clearly his eyes! (A little creepy, I know.) The big blob in his abdomen is his bladder. The white lines above are probably his ureters.

 I love his little fat rolls above his knee here. 

The big blob is his bladder. I don't know if all other the other white spots are his ureters, but the backwards c- shaped one looks like most of the other images I have seen. Ureters should be like little straws that aren't even visible on scans in a healthy situation. 

Balloon looking thing on the right is the bladder.

Waving hello! Forgot to mention that they did a lot of brain imaging and the report said all looked good.

I can't figure out what he looks like here. Some kind of stuffed animal or cartoon I have seen. 

Profile shot. Wonder if his lips are actually that big or if it is like Selah's nose that had us all worried for nothing on her ultrasounds!

View from the back. You can see his abdomen is pretty distended looking in this shot.

Children's National Consult

Well, they did manage to squeeze us in this week,  after all. I got a call yesterday afternoon informing me that I needed to be AT the hospital at 6 am for an MRI followed by an ultrasound,  rounded out with a telemed meeting with the surgeon,  neonatalogist, and I think another unidentified person.  

I've never had an MRI before.  I hope I never have another.  It took an hour.  It was not an open MRI, though thankfully my head was out. You can't move.  They placed something over me so they could get images of Judah. I had to wear a mask the whole time (SO over COVID!) Blessedly, I was on my side,  not back, to allow proper blood flow to Judah. However,  I was still uncomfortable.  And they put a warm blanket on me. Between the blanket,  the mask,  and the personal heater living in my abdomen...I was so hot I was sweating.  And did I mention you can't move?!  The good news was,  I've had so little sleep and having been up since 3 am, I did doze off some.  And between that and the rosary, I managed to not panic. It was hard, though.  

They then sent me to ultrasound where I had a very long scan that I couldn't watch.  So ... again... no cute baby pics. And I have no idea how anything looked today.  That's always frustrating.  But Judah's heart rate was 140, and that was good! That's all the info I got. 

Now waiting for that 1200 Zoom meeting that has to be done on site.  Why? Because we live in VA and the NICU doc is only licensed in DC. So I guess that is an issue for insurance purposes? 

Even more frustrating than sitting here for 2 hours is the fact that Bryan can't participate.  He's literally been allowed at nothing but the telemed appointment we had with the nephrologist back at 22 weeks. Technically,  he could be here today but he couldn't come to my appointments beforehand so he would be waiting here for literally 6 hours to meet with the docs. Leaving all the kids home alone to get ready for Christmas Eve Mass themselves. We couldn't drive separately because the battery is dead in the truck and we can't get that replaced until Monday.  Yes,  he likely could have gotten someone to jump him, but that leaves the risk of not being able to get home.  Plus, again, we need to leave at 3 for Mass because you have to sign up for Mass slots (Again,  thanks COVID) and we signed up for the 4 o'clock before we knew we would be meeting with docs today.

Overall,  I'm glad they got us in.  And I'm glad that they put me they all this misery this am because I feel like they are doing a good job of truly trying to make an informed plan. That was my biggest complaint at Walter Reed, the "we will see what we are dealing with once he is born" attitude. I mean,  for the most part,  that's all we can do.  But having the specialists review things now and make a plan based on what they see is a relief. Assuming they obtained any new info today that is useful.  We may actually know nothing but what we knew yesterday. Will update when I can following the appointment.  

36 Weeks- Getting Close.

This week's disappointment started with no pictures, and actually not even a glimpse of Judah's face during the ultrasound. Can't deny that is the best part! He wasn't positioned well for face viewing, it seems. He's growing well,  estimated to be 9 pounds 2 oz, currently.  (Again,  his abdomen is excessively large and that is figured into the calculation so probably anot quite as big as they think. Plus, they're notoriously wrong in those estimates.) 

Things improved with the discussion with the doc (not my normal one).  She said it looked about like it did last month.  As such, it was decided to go with a 39 week induction to facilitate having a team of specialists ready. I have no interest in fighting that, especially given that it'll be January and the hospital is 45 min away.  We want the best care possible and no road-side deliveries.  

She was to consult with the pediatric nephrologist, and surgeon to make sure they were comfortable with the plan, and then get back with me.  

I didn't hear from her yesterday,  but the weather was pretty bad the last 2 days so I wasn't surprised. However,  last night I checked to see if I could see the doctor's notes online yet.  They always have more info than I'm given,  even though I feel the normal doctor does great showing and explaining things.  

Much to my surprise and devastation, her notes had some new info that she hadn't shared with me. In addition to the hydronephrosis, megaureters, and large bladder,  she was unable to identify a number of spots in the abdomen and the stomach looked abnormal. This is leading to more suspicion that it isn't Prune Belly, but instead Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS).

This was mentioned at 20 weeks, and again at 32 when they noticed the excessively large bladder.  But now with potential GI issues being seen via ultrasound,  it seems more likely.  It also explains the polyhydramnios I have (too much amniotic fluid) which is common with MMIHS but with Prune Belly too little fluid is common.  That leaves the club foot as the oddity, but that can certainly be just a separate thing. 

The short story of MMIHS is that the urinary text doesn't function right,  same with PBS. they generally need catheters to empty the bladder.  Additionally, the stomach,  intestines, and colon affected and they are generally unable to consume food by mouth. Some can for a short period of time, but eventually they need a feeding tube or (most common) a central line. 

It is very similar to PBS, often with the same genetic component.  Both are the result of smooth muscle tissue abnormalities.  The unfortunate thing is that the fatality rate is even higher.  The statistics we could find were from 2012, so we hope they are outdated. However,  they report an 88 percent fatality rate in the first year,  with the average lifespan of 3.5 months. 

Why do I share this? Because while we how to beat those odds and have Judah for a true lifetime,  we want to be open and honest about the reality of the situation. Obviously, we are so hopeful for a miracle. And we will never quit praying for one. But we have to be realistic that he may never come home. And if he does,  it will likely result in serious lifestyle changes for our family.  Again,  we likely won't have a real prognosis until he is born and we know the true extent of what we are dealing with.

So for now we wait for a consult with Children's National in downtown DC. Looking at the positives, we are in an area with truly phenomenal pediatric care options. The best in the country. We also have John Hopkins up in Baltimore, should Children's not be equipped to deal with these issues.  And in terms of delivery,  Walter Reed is as good as it gets, at least in terms of military Healthcare. Added bonus, TRICARE will cover this all. 

If you want more info on MMIHS, I suggest starting with MMIHS.org. It's very simply and concisely explained, and even has personal stories that offer so much hope.  

Fall Family Photos

Ideal was not the conditions nor the locations. But we managed to call it good enough on our second try. The single photos of the kids were from the first try out at the National Forest nearby.

We went out to Manassas Battlefield the second time. It is so pretty there, and peaceful. Humbling to know how many lives were lost in that very place. There wasn't much color left in the trees, but it does encapsulate the area we live this year! 

Here's a few randoms to make up for the lack of posts lately. 

Sums up what it is like to have to live with all those brothers.

But she loves little Judah immensely, even if she would have picked a baby sister had she been asked.

 

Happy St Nicholas Day!

New shoes and candy for all! I even found white chocolate coins for Micah this year, as he doesn't like chocolate. (Selah set out her doll's shoes and put a doll cupcake inside. She will be a great mom.)

No Fireplace here. So we set up the tree we had just bought. It's slimmer than we realized, but I guess that is good as we were able to put it in the living room rather than the foyer. I hate to spend money on things we don't need/may not use after this year...but we didn't have a slim tree so we will find a place for it in the new house, I suppose. 

 It does look awfully sad with no decor. We decided to try to make popcorn, dried oranges, and cranberry garland. Maybe some ribbon. It certainly needs some cheer. 

Baby Judah Update

32 weeks....8 months...getting closer! 

We may need to actually start preparing. 

No good pictures today, but here is his sweet profile. 

This may be your first post of info, so I'll give more overall details.

I wish I could say things are looking great. They're not, but we do have some positives! 

His brain looks perfect, as does his heart. They looked intensely at both again.

He is measuring in the 98th percentile overall.The size of an average 36 week baby. Weighing an estimated 6 lbs 14oz (yes, with 8 weeks to go. I take this info with a grain of salt, but all measurements were 97th percentile or higher, so he won't be a little guy if we make it to 40 weeks.) This appointment he was classified with accelerated growth, but they aren't concerned at this point. Big babies tend to do better when they have to fight for their life, so here's hoping it helps him out! 

Now the bad news. (Specific details and measurements are mostly for my ability to easily reference in the future.)

Bilateral club foot is still visible (not something we expect to go away.)

The urinary tract looks worse. Both kidneys show hydronephrosis (too much fluid in the kidney). Anything over 7mm is too much at this point. Right shows 12.2mm and left 9.7mm. The right kidney still looks otherwise healthy. The left they were unable to identify a clear perimeter and are more concerned that it may not be otherwise healthy, but that is yet unclear. 

Both ureters are still classified as megaureters and appeared worse than 4 weeks ago. The right now looks like one big lake. The left seemed like it may still have some curve to it and was more like a long oval than a big circle. 

The bladder is most concerning this time. While it has been classified as prominent in all scans, and thickening of the wall was noted at 22 weeks, it's never been OBVIOUSLY too large. This time, it is. As soon as the abdomen came up on the screen, my heart sank. There was a large, black mass taking up most of the abdomen. I asked if the giant black hole was the bladder or maybe the stomach. She replied that she was still trying to map it all out. That was not reassuring. She found the stomach, which I didn't recognize because it wasn't a nice round circle this time. As the doc said, it's turned into a pancake. The bladder is so large, it has pressed up into the upper abdominal cavity and is smashing the stomach. It may also be why it was difficult to identify the left kidney and it's surrounding structures. 

We are also back to polyhydramnios (too much amniotic fluid) but it's still mild. And again, reassuring that we aren't too low on fluid. Fluid means his lungs and extremities can continue to develop properly. It also means his urinary tract is doing what it is supposed to, at least to some degree. Of course, it increases my discomfort but so far we've only had 4 random days that were unbearable. Here's hoping it stays mild and I can stay somewhat comfortable.

So what does this all mean? 

Well, the doc is more and more convinced that it is likely Prune Belly Syndrome, also known as Eagle-Barrett Syndrome. This isn't good news. In simplest terms, this condition is classified by urinary tract malformations and the partial or full absence of abdominal muscles. 

Why is this most likely? 

Well it's pretty much the only syndrome that correlates the bilateral club foot and the urinary tract malformations.  (This can be sometimes found in Trisomy 18 and 21 but all testing has shown low-risk of those.) Additionally, his abdomen has measured greater than 99th percentile from 20 weeks on.

What's the prognosis? 

Well that's the hard part.  We really don't know.  It's a rare disease, affecting approximately 1 in 40,000 births.  Statistics are difficult to come by,  as is a standard treatment plan.  The only real statistics I could find show a mortality rate of 50 percent at, or shortly after, birth.  With an additional 50 percent of those who survive not making it past 5 years.  

Ouch, right? 

The statistics are scary. But we don't know for sure that it is Prune Belly, yet. There are a few other possibilities but with the bladder so prominent this time, our hopes are dwindling. We've really been praying for a miracle. And we will continue to do so. But short of complete healing or a very mild case, we are praying that we have the best possible doctors to provide the best possible care. Treatment is virtually all done on an individual case basis, but the scariest part is that most nephrologists and urologists have never seen a case. So they're just flying by the seat of their pants, taking (possibly somewhat-educated) guesses as what would be best. 

 So, that is all for the next 4 weeks if all continues as status-quo. The 16th of December will hopefully find us with more (positive!?!) info and perhaps a plan for delivery. Bryan is due to be overseas then, so we're also praying that we won't need to deliver before he gets back. 

28 weeks!

I always dread this appointment. 

Gestational diabetes screening, Rhogam injection, and bloodwork are all on my list of, "No, thanks!"

We survived, though it was a long day. OB appts started at 830am and I was stuck until hanging out until my ultrasound at 2pm. There really wasn't that much downtime, as I had to meet with the intake nurse and then the OB. Followed by glucose and bloodwork. Then I had to take blood to the blood clinic to receive my Rhogam, and return to OB for the injection. It was after 1230 by the time I accomplished all of that. 

They have removed the food-coloring and the carbonation! But they left all of the preservatives and junk. SOOOO gross. 

Pictured, 8 tubes. Then she went to grab another from elsewhere. NINE tubes of blood. I swear this is only at a military facility. I was so proud of myself for not passing out.

The reward of the miserable morning, sweet pictures of baby Judah!

It is much easier to see what his feet look like in 3D! He was moving too much to get a clear shot of the left foot, but it looks similar. I found this nice because I didn't feel like they look as bad as some of the pictures I have seen of other people's kids!

His weight is great, estimated to be 3 pounds 12 ounces. He's overall measuring in the 89 percentile, and in line with a 31 weeker. 

Fluid was normal today! Yay! (I could tell it had decreased, as I was feeling much more comfortable over the last week.) Just slightly under the threshold, but I call that a win as it means there is plenty in there for his lung development. 

As for the urinary tract...well there was one positive! 

The right kidney looked normal with no excess fluid! The left was showing too much fluid, measuring 12 mm. However, kidney tissue and development appeared normal. We are hoping maybe next time they will both have normal fluid levels!

The bladder was classified as prominent, but wasn't crazy large. Both ureters showed severe dilation. They really seem to the be the worst of the issue. They certainly were worse than last month, but maybe that will be the only issue.

Overall, I felt like it was a good appt. There was no mention of stillbirth or early delivery this time. Maybe he just felt like that point had been made, but I'll take it either way.