Sunday, January 31, 2016

Procedure week...

We spent last week in Denver at Children's Hospital Colorado. It was a ridiculously long week, we met so many amazing people, and are completely exhausted.

We spent all day Monday in appointments or meetings. I had a fetal echocardiogram done first - baby's heart looks perfect.

Then they sent me down to MRI. That was an incredibly miserable, uncomfortable 90 minutes, on a hard table, not being able to move at. all. They needed to get better imaging of the baby.

After the MRI, we went back upstairs to have an ultrasound done. Baby looked awesome, except for the fact that the bladder was severely enlarged, which we already expected. Other than that, all of his measurements were perfect.

We met briefly with a social worker, who just wanted to make sure we had everything figured out financially, and had a good support system and a good understanding of what we were potentially facing.

The last meeting of the day was the family meeting, with the entire surgical staff, everyone that would be dealing with the baby before and after birth (if we were to deliver at that hospital). It gave us a pretty good idea of what we were looking at. The fetal surgeon was there (Crombleholme), as well as the Maternal Fetal Medicine dr (Galan), the Nephrologist (Gobel), the Neonataologist (Reynolds), Urologist (Wilcox), a pediatric resident, and the patient coordinator (Jenna). They went around the table, each giving a brief explanation of their view of the situation, and gave us information on what we were looking at. They gave us the options we had for treatment (full-speed treatment, doing everything we can now and after baby's born; doing nothing and taking a wait-and-see approach, and just comfort care after baby's born; a combination of the two, whatever we were comfortable with; or ending the pregnancy), along with the potential risks and benefits for each. We learned that the procedure we were wanting to do was still so experimental that it had never been done on a human before - only in animal trials with sheep, where it had been successful. If we chose to do that procedure, we'd be the first ever to have it done.

We went back to the hotel with a lot of information, and a lot of thinking to do. They wanted us to meet with more people the next day, more one-on-one, to get more in-depth information on what we'd be looking at, both with the procedure, the rest of the pregnancy, and in the long-term, after the baby's born. We wanted to see the worst-case scenarios, so we'd be prepared for everything.

The next morning, we went back in. We met with the Nephrologist (Kidney doctor), who answered more questions about dialysis, and then we went into the kidney center and met a family who was training to do at home peritoneal dialysis with their three-year-old daughter. They showed us how that worked, the medication list, her dialysis port, and told us a little about their story. After that, we went into the NICU and met with another family whose 4-month old little girl had been on dialysis since she was born, and had never produced urine, even in utero. She also told us about the difficulties of having a baby on dialysis, as well as being in the hospital long-term. It was an eye opening experience, seeing these sweet little girls hooked up to machines.

Later in the day, we met with the Neonatalogist, who basically went over the procedures there. It was a short meeting, since we've been through the NICU before, albeit with a healthy baby.

At the end of the day, we met with the MFM and surgeon, and went over more pros and cons of stent (the experimental procedure) vs. shunt (a procedure that's been done for over 30 years), vs. doing nothing. We had decided to do the stent procedure. We signed the consent papers, and left.

At 5:30am on Wednesday, we were back at the hospital ready for the pre-op admission. They did an IV, did vitals, had me change into compression stockings, as well as the compression cuffs they hook up to a machine. They did another ultrasound, gave me some medicine to settle my stomach, and sent me to the OR. In there, they did a spinal block, and set the catheter for an epidural, in case the spinal didn't work. They gave me pain and anxiety medications in my IV to put me to sleep, rather than using a general anesthesia. I don't remember much... I do remember snoring (hah!), and waking up a few times, telling them I was awake and asking for more medicine to go back to sleep. I think I asked someone to scratch my forehead, since my arms were strapped down. I also remember, at the very end, the doctors talking, and pushing really hard on my belly. I had no idea what was going on.

The procedure went quickly - I was out less than an hour after they started. The meds were still making me super loopy and tired, and it took several hours for the spinal to wear off. I was on strict bed rest for the next 24 hours - cathed and everything, couldn't get out of bed for any reason, even to pee.

The doctor came in later and told me how the surgery had gone. They went in first and added 900mL of fluid, since my fluid was low to begin with. After that, they inserted a camera and a laser into the baby's bladder, and they were able to laser the valves partially open. They were able to see him passing urine, which is incredible. After that. they placed the stent. When they did this, he was really close to the uterus, and the stent got stuck in the uterine wall. When they pushed him away (which is what I felt), it stayed in the uterus and pulled out of his bladder. After that, they went in with a needle and drained his bladder.

So the surgery didn't go as planned... And while we knew that was a risk, it was still a huge letdown. We're hoping he's able to continue passing urine on his own, that the valves stay open, and that it's enough to relieve the pressure on his kidneys.

They gave us the option of staying over the weekend and attempting the shunt procedure on Monday, but we decided to wait. The amount of pain I was in from that procedure, in addition to my likeliness of going into preterm labor on my own, and the increased risk of preterm labor, and amniotic rupture from multiple procedures, made us decide to wait and see what our Dr here in Florida said.

The larger incision in my abdomen was (and four days later, still is) incredibly painful. It feels like it's pulling from the inside, any time I stand up, and it hurts for anything to touch it. After this, I also stand firm in my stance that I will NEVER again willingly have an epidural done. My back hurt so bad after, and is still very tender. It hurt to move, at all.

They had me on the contraction monitor, because any irritation to the uterus can cause contractions. I had several throughout the day, but they weren't really that painful. Later in the evening, around 8:00pm, the contractions got really really bad. They were coming every 2-4 minutes, and were so incredibly painful. I cried through every one of them.

Thursday morning they came in to do another ultrasound to make sure he was doing well. The contractions the night before hadn't caused any dilation, which was amazing.  Amniotic fluid levels were good, and his bladder was bigger than the day before, but it was a more normal size. (Normal is relative... It was still larger than it should be in a healthy baby, but since his bladder was so distended before, it makes sense for it to still be stretched out.) I was beyond happy when they removed the catheter and said that I could get up, and they were going to be discharging me.

That afternoon, as we were walking across the hospital lobby, headed back to the hotel, I felt a small gush of fluid. I went to the bathroom, hoping it was just bladder irritation from having a catheter for over 26 hours. After that, I felt an even bigger gush. We went straight back upstairs. They did testing on the fluid, and confirmed that it was amniotic fluid. During the procedure, the amniotic membrane fully separated from the chorion, another membrane between the amniotic sac and the uterine wall. Because of the separation, fluid was able to leak into the space between the membranes. We were hoping that the leakage was just the fluid from in between, and that I wouldn't lose more of the fluid around the baby. I went back in on Friday morning, before our flight home, to have another ultrasound. Cervix was still long and closed, which is good, but the amniotic fluid around the baby was extremely low. They gave me orders to stay down as much as possible, and follow up with my doctor asap.

I have an appointment with him tomorrow (Monday) morning. I've stopped leaking now, and I'm not sure if that's because there's not fluid left, or if it's because the membranes have shifted, and the holes from the surgery aren't lined up anymore (which is what the doctors in Denver said might happen, and were hopeful would happen). We're praying for his bladder to be draining, that his urine output is able to add more amniotic fluid, and that his lungs will be able to develop.

I do have to say... Just about every single person we came into contact with at Children's Hospital Colorado was amazing. They all took the time to explain things to us, to make sure we understood, and helped ease the anxiety and fear just a little bit. It was also really nice that my parents were able to take some time off work and be there with us.

Sorry if this is all scattered and hard to understand... It's hard to remember everything from such a hectic week.

Friday, January 22, 2016

Finally, answers, but still waiting...

I went back in on Tuesday, 1/19/16, for another ultrasound. The fluid levels were better, but the baby's bladder was really large again. After talking, we decided that the best option at this point was to go to Denver for the stent procedure. Because the fluid is still at normal levels (and seems to be getting better), they're not really concerned about lung development at this point. Right now, we're trying to alleviate the pressure on baby's kidneys to try to prevent more damage.

Since then, life has been a whirlwind of stress, phone calls, more phone calls, trying to plan, talking to four different doctor's offices, in addition to several different departments at Tricare, relaying information to family, planning for someone to come stay with my older kids, and literally, just a LOT of stress.

After the referral was sent from Perinatal to OB (on base), they had to send it to my PCM (off base) to be sent in and approved, because Tricare won't pay travel expenses unless the referral comes from the PCM... So I had to wait until Wednesday morning to get in to her office for her to send the referral in, and waited, and waited, and waited, for it to go through. I was on the phone with everyone I could have been, trying to speed up the process. Finally, late Thursday afternoon, everything went through and was approved. This has probably been some of the most stressful few days of my life.

My intake appointment with the Fetal Care Center in Denver (really, Aurora), is scheduled for Monday. Three days from now. I have appointments and meetings starting at 8:00am, spaced every couple of hours throughout the day, with the last one scheduled at 3:45pm. We will literally be there all. day. long. They'll be doing a fetal echo-cardiogram, to make sure the baby's heart is developing properly. Then I'll have an MRI of the baby, so they can get more clear imaging. Then I'll go in for an in-depth ultrasound for more measurements, etc... After that, we'll meet with a social worker, and at the end of the day, we'll have a meeting with the surgical team to decide on the course of action.

Then, on Tuesday, we'll have the stent procedure done. From what I've seen in research, and the perinatologist here understands, it's a relatively non-invasive procedure. They insert a (pretty wide) hollow needle through my abdomen, into the baby's bladder. They'll push a tube (surgical mesh, I believe) through the needle, and that will go from the baby's bladder, through his abdomen, to drain the urine into the amniotic sac. It's done under only local anesthesia, and apparently doesn't take very long. It's technically an outpatient procedure, but they'll admit me and keep me overnight for observation. On Wednesday, they'll do more imaging to make sure everything is ok, and I'll be released to go home.

My sister in law is flying out from Nevada to stay with the kids while we're gone, and my parents are going to drive down to Denver to be with us.

We're praying that everything goes smoothly, for us, here at home for our sister in law and the kids, with travel, with everything.

I feel a lot more calm than I expected I would. I finally broke down yesterday, when it seemed like even after several days of trying, nothing was going to work out, but it all did, and we're pretty much set to go. Other than that, the few days after we found out about the baby's condition, and an occasional moment here and there, I've been a lot more level-headed and collected than I expected to be.

There is still a chance that things may not work out in our favor. I have to acknowledge that. I do know that it's not my fault that this is happening, and that I have no control over any of this, and I'm doing my best to let go of the guilt and the thought that my body is failing me... I know that whatever happens, is what is supposed to happen, regardless of what that is.

Wednesday, January 13, 2016

Bad(dish) news... With a plan attached

At my appointment on Monday, they said that the amniotic fluid level looked better. Still low, but within normal range, which is awesome. They still drained about 30ml of fluid from his bladder. There was some fluid in his abdomen, which, the dr said, likely leaked out through the puncture made the last time they drained the bladder, but it wasn't dangerous because the fluid is sterile. They tried to drain that as well, but couldn't get the needle to go through the abdominal wall.

They sent that fluid to be tested for kidney function (electrolytes, proteins, etc...) and said they'd call me when they got the results back. He also said that he'd call the surgeon in Denver first thing Tuesday morning and discuss the case with him. I was expecting a call today, or just to have the conversation when I went in for my appt this afternoon, but the dr called yesterday afternoon.

The kidneys aren't functioning well... The results from the test were not good, showing moderate kidney damage. There's really no way to to fix or reverse that at this point - it's something that's going to have to be dealt with after he's born.

The goal now is to preserve lung development and respiratory function, and deal with the kidneys later. The only way to really make sure the lungs can develop normally is to keep enough amniotic fluid around the baby. They're not going to do any more bladder taps, because it's irrelevant. They will, however, monitor the amount of amniotic fluid weekly. If it gets low enough (I'm not sure how low that is...), they're going to send me to Denver to have the stent placed. This will allow the bladder to drain, hopefully increasing the amount of amniotic fluid enough to sustain the baby long-term.

Once they determine that the fluid is low enough to do the operation, it will move fairly quickly. I spoke with the patient care coordinator in Denver this morning. She explained some of the process to me. They will be able to get me in immediately, how soon is dependent on us being able to arrange travel to get there. They will want to do the operation within a week, or a week and a half, at the most. Once I get to Denver, the first step will be doing some more intensive imaging - an MRI to get a better picture of what they're dealing with. A more detailed ultrasound, and an echo cardiogram on the baby's heart. The next day will be the operation. They usually keep you in the hospital for about 24 hours for observation, and want to follow up 24-48 hours later. We'll likely be in Denver for 5-7 days.

Monday, January 11, 2016

Trust Me

At my 16 week OB appointment on Tuesday, January 5, the doctor wanted to do an ultrasound to check cervical length. This is something they've planned to monitor since the beginning. The ultrasound was taking longer than expected, so I was starting to worry. She asked me when my last ultrasound was (4 weeks prior), and if they had mentioned anything about the baby's anatomy. I told her no, it was with high risk, and they said everything looked perfect. She turned the ultrasound machine around and showed me what she was seeing.

There was a large black circle in the baby's abdomen, and she said that it looked like it was the bladder. It definitely should not have been that large. She printed more images from the ultrasound, and sent them over to high risk, and called to see if they'd want to see me sooner than my next scheduled appointment, which was that Thursday. They said Thursday was fine, and just keep that appointment.

She explained to me what some of the issues could be, that it was likely a urinary tract blockage, and that could cause serious problems, but she wasn't qualified to make a diagnosis, so I'd need to wait until I saw high risk on Thursday.

At my appointment on Thursday, they did an ultrasound to get measurements of the baby, of the amniotic fluid, and cervical length. The baby is measuring beautifully - everything was the size it should have been. Except the bladder. They did find that it's a boy! His head was 3cm, and his bladder was measuring at 2.7cm. His right kidney was enlarged also, and the left looked like it was beginning to show some damage as well.

They diagnosed him with Posterior Urethral Valves, which is basically an extra flap of tissue in the urethra that prevents him from being able to empty his bladder. This is causing the urine to back up into the kidneys, causing damage. My amniotic fluid level was still very low.

The next step was draining the fluid from the bladder via amniocentesis. The needle is huge, and it freaking hurt, but not nearly as bad as I was expecting. The dr was expecting to get maybe 1 1/2 syringes (20cc each), but ended up with 2 1/4 - about 45cc of fluid. It broke my heart for the poor baby... I can't even imagine how much pain he had to have been in. They sent the fluid out to a lab for genetic screening.

Today (Monday) I go in to have his bladder drained again. We should have some of the results for the genetic testing back today, so hopefully we'll know if it's a genetic issue, which it occasionally is with bladder obstruction.

I'll go back again Wednesday to have his bladder drained again, and at that point, they'll use the fluid to run some lab tests to check kidney function. If his kidneys are still working, from there, we'll discuss in more detail the surgical options we have.

One option is having the dr here in Pensacola place what they call a pigtail catheter (it's curled on each end) between the bladder and the amniotic sac through the abdomen. This will help to drain the fluid from the bladder, also adding more amniotic fluid. The issue with this is that it's very small, so it can easily become clogged by skin cells floating around in the amniotic fluid. Because it's such a small opening, the fluid doesn't drain quickly enough to relieve the pressure on the kidneys. And because it sticks out, babies will often pull it out.

A second option is where they'll basically open the abdomen to the bladder via an incision. This will be repaired after he's born. This procedure can be performed by several doctors throughout the US. It's much more invasive, but more common.

The third surgical option, the one my dr is leaning towards, is sending me to a specialist in Denver. He has pioneered a procedure where a stent is placed between the bladder and the amniotic fluid through the abdomen. It's less invasive than the incision, a larger opening than the catheter, and more successful. The ONLY place the surgery is done is in Denver, so we would have to travel to have it done.

The doctor did say that in some cases, draining the bladder initially reduced the pressure in the urethra enough that the baby was able to pass the urine, and the problem basically resolved itself, and kidney damage is very minimal. This is the absolute best case scenario. We can only hope for this.

There are several other scenarios... none good.

We can go in today, or Wednesday, or really, any day, and the baby will have already passed, due to pressure, kidney issues, lack of amniotic fluid, etc...
We can go in and find that there's a genetic issue, or that the kidneys aren't functioning enough to warrant having the surgery done, and we let him pass naturally.
We can have the surgery done, and the amniotic fluid is still too low, and his lungs don't develop enough to even use a respirator after he's born, and he passes due to respiratory issues.
We have the surgery done. He is born able to breathe, but has severe kidney damage and requires dialysis and/or kidney transplant right away.
The next best scenario, the one I'm really hoping for... is that we have the surgery done, and the rest of the pregnancy goes without many issues. He is born, able to breathe, with no other major issues. they do whatever surgery is necessary to remove/repair the stent, remove the valve that's causing the issues, and he doesn't have to suffer on dialysis for the rest of his life.

We've been praying constantly for the past several days. Praying that his body heals itself, mostly, but I've also been praying that I can handle whatever scenario we're faced with. That we can make it through whatever trial we're given. That I can be ok with whatever ending we get.

The most prevalent thought that's gone through my mind since we first found out has been "Trust Me." I'm trying. I really am.