Going Ahead with the ETV

We've mentioned many times in regards to Nathan's hydrocephalus and the consistent shunt failures that we were running out of good options. 

To better illustrate that point and to help everyone get a better picture of the position we were in, Dr. Riva-Cambrin informed us that the likelihood of the ETV working was only about 20%.  That meant that there was an 80% chance that it wouldn't work. 

Can you imagine that?  The best option you have in front of you only has a 20% chance that it's going to fix the problem.

As is custom, they scheduled Nathan for surgery the next day, since it is better to have him on the schedule and not need it than to not have him on there and try to fit him in.  Then we went home to discuss it and see if this is what we wanted to do.

That night, we talked a lot and prayed a lot and decided that this really was the best option available to us.

So the next day, they took Nathan for an MRI to get a good look inside his head and to make sure that there was no anatomical reason to not do the ETV.  With the MRI results looking promising, they took Nathan into surgery.

The surgery went really well and Dr. Riva-Cambrin was really pleased.  With the ETV, there was no need for the shunt.  So that was removed.  But unfortunately, none of us had any control over how Nathan's body would respond to the ETV.  A CT the next day showed that the ETV was working, so it was very promising. 

But as with all of the other attempts to fix it, only time was going to tell.

The Third Ventriculostomy

Inside your brain, there are 4 ventricles.  They consist of two lateral ventricles, the third ventricle, and the fourth ventricle.  The ventricles are filled with cerebrospinal fluid (CSF), which bathes and cushions and helps to protect the brain inside the skull.

The two lateral ventricles are relatively large and C-shaped.  There is one in each hemisphere of the brain.  They are connected to the third ventricle by the foramina of Monro.  The third ventricle is in the midline of the brain and is connected to the fourth ventricle by the aqueduct of Sylvius.  The aqueduct of Sylvius is very small and can be easily blocked, and thus is one of the primary causes of hydrocephalus.  In fact it is the location of the blockage that caused Nathan's hydrocephalus.

The fourth ventricle is also continuous with the central canal of the upper end of the spinal cord.  It provides CSF to the spinal column to help bathe and cushion the inside surface of the spinal cord.  The CSF in the spinal cord goes all the way down to the lumbar cistern near the bottom of the spinal cord.

A third ventriculostomy, sometimes referred to as an endoscopic third ventriculostomy (or ETV), is a surgical procedure that creates a bypass for the CSF around the blockage in the aqueduct of Sylvius and thus eliminates the need for a shunt.

Endoscopic means that the surgery is performed with the use of an endoscope, a thin tube that has a strong light, a powerful magnifying lens, and a passage through which tiny instruments can be passed.  Third ventriculostomy refers to the area where the bypass is made, in other words, through the thin membrane in the bottom of the third ventricle.

The CSF may then flow through the new opening to the normal fluid chambers below the base of the brain.  From there it can then be absorbed into the bloodstream like normal.

The New Plan

Once Memorial Day had passed, we met with Dr. Riva-Cambrin again to discuss our options.

Following another heart echo, Nathan's cardiologist once again stressed that his little heart was just not ready to handle a VA shunt.  So that one was still out.

Dr. Riva-Cambin mentioned doing another VPL shunt, but on the right side of the chest this time.  Considering how scary and near-deadly the last VPL shunt was, we quickly shot down that idea as adamantly as we could.

I brought up my idea for the "branching shunt" and asked if anything like that had ever been attempted before.  Dr. Riva-Cambrin said that to his knowledge it hadn't, but that he would consider it and if he used it he would write it up in a medical journal and name it after us!

At the end of our meeting, he let us know that the next day in the morning he would be meeting with all of his colleagues in the neurosurgery department to discuss Nathan's situation and to see if any of them had any ideas as well.  Then he would let us know the direction he felt we should take.

So the following day, we received a message from Dr. Riva-Cambrin letting us know one of his colleagues had suggested doing a procedure called a Third Venticulostomy and that he would be in later to explain it to us.

Trying to Come Up With a New Plan...Again

The weekend Nathan was admitted to the hospital happened to be the Friday of Memorial Day Weekend.  So we had a really, really long time to think about Nathan's shunt problems.  Which was both good and bad.

It was hard having Nathan in the hospital again.  And it was especially hard having him with an EVD again.  But most of all it was particularly hard not having a plan on what to do next to finally take care of these blasted shunt problems.

At the same time, though, it was nice to have the extra time to really, really think about things.  And there was a lot to think about.  And some of it was heavy stuff to think about, too.

Having the shunt fail again was completely devastating.  This shunt was supposed to work.  This was "the golden shunt."  Since we had previously exhausted all of our good options, we were at a complete loss of what to do next.

When we met with Dr. Walker, he mentioned a ventriculocholocystic, or gall bladder, shunt.  They are quite rare, and Dr. Walker said that he had never done one in anyone as small as Nathan before.  And when you factor in how many procedures Dr. Walker has done, that is making quite a statement.  And throw in the fact that it would likely be very, very dangerous, we were quick to put that one near the bottom of the list.  

He also mentioned a ventriculo-ureteral shunt, or placing the shunt into the ureter, the muscular tube that connects the kidneys to the bladder.  You don't have to worry about absorption with this type of shunt.  Instead, the patient just pees off the excess fluid.  But, especially in someone as young as Nathan, you would have to watch electrolyte levels due to all of the urination.  So maybe a better option, but still not a great option.

All of this weighed heavily on our minds and spirits.  We weren't really worried that Nathan was going to die, but rather the quality of life he was going to have.  Was he destined to be stuck in a hospital room draining into a bag forever?  Would we ever get to have him at home for more than just a few weeks?  These were all questions we didn't have answers to.

So in an effort to seek some type of spiritual guidance or inspiration, Bekah and I went together to our church's highest place of worship.  There, we were reminded of the bonds that we share with each other and with our son.  And that he will always be our son in this life and in the next.  We remembered the blessings and promises that the Lord has promised to the faithful.  It was nice to regain perspective, have a quiet place to think and pray, and be able to spend a little time together as away from our problems as we could get for about an hour or so.

We also decided to spend the weekend brainstorming as many ideas as we could that might be helpful to our neurosurgeon.

First and foremost, we wanted the cardiologist to perform another echo on Nathan's heart to see if there was any improvement that would permit the placement of a VA shunt.

Personally, I came up with what I considered a great idea that I called a "branching shunt" (check out the diagram below):

Our biggest issue with the shunts so far had been Nathan's inability to absorb the CSF.  Once his body had absorbed all that it could, it would just build up until the area was full and cause the shunt to back-up and stop working.  So under my idea, if the distal end of the shunt were to drain into two separate places, if one got full, it could drain into the other.  And then hopefully, before that spot got full, space would empty up in the other spot.  And it could just drain back and forth like that forever.

And those two things were pretty much all that we could come up with.  So we also started praying that the surgeon or one of his colleagues could receive inspiration on something that would help Nathan and give him the best chance to have a normal life.

To us it didn't matter who got the epiphany, the revelation, the inspiration, or whatever you want to call it.  Just as long as someone got it and that Nathan would benefit from it.  That's all that mattered.

Back at the Hospital

It didn’t take very long before we realized that we weren’t going to make it to clinic the next week.  And since it was the weekend and since we were tired of being in emergent situations, we decided to go ahead and make an appointment with the neurosurgery department so that someone could check Nathan over.

When we got there, they took a bunch of X-rays, but couldn’t see anything wrong.  So the most likely scenario was that the shunt was blocked…again.  So Nathan was readmitted to the hospital for the 5^th time.

Since Dr. Riva-Cambrin was out of town, Dr. Marion Walker was the one who would be operating on Nathan.  Dr. Walker is somewhat of a legend at Primary Children’s, having been there for over 25 years and having performed thousands upon thousands of neurosurgical procedures.  Really, there is no one you’d want operating on your child more than Dr. Walker. 

The surgery was surprisingly quicker than we had expected.  Dr. Walker re-emerged from the OR after what couldn’t have been more than a half an hour.  He told us that Nathan’s tummy was just full of fluid and that that was the reason for the blockage.  So he had inserted an EVD to drain the fluid while we figured out what to do about the shunt.

Since we had already dealt with an EVD once, we weren’t really excited about doing it again.  But when we finally got to see Nathan again after surgery, we discovered one of the reasons why Dr. Walker is a legend.

Instead of inserting the EVD into Nathan’s head, he put it directly into Nathan’s stomach.  I thought it was just pure genius.  He limited the amount of surgery that Nathan would have to go through at that time, but produced the same results.  We were very, very impressed to say the least.

But now we back to the same old question: what are we going to do with this shunt?  And unfortunately, all of the good answers were already taken.

Seeking a Spiritual Uplift

The news of Nathan's most recent shunt failure was completely devastating to us.  Especially since the previous 2 weeks had been absolutely wonderful.  And with the uncertainty of the shunt situation breathing down our necks, it could probably go without saying that our spirits were really, really low.

Not knowing what else to do, we scheduled a time to visit with the leader of our local church congregation, hoping to get some type of guidance, or a boost to our faith, or at least to "rally our spirits" a little.

Unfortunately, we left the meeting feeling no better off than when we had gotten there.  Maybe we didn't explain Nathan's situation well enough, or maybe we didn't explain our despair well enough.  Maybe our expectations of the meeting were too high.  I don't know.  I won't go into details, but I felt like he just didn't get it....or....something.

Ugh.

Here We Go Again...

Only a couple of weeks after being discharged, we were at our weekly appointment with the pediatrician when he noticed a squishy bulge behind Nathan's right ear, right where the new shunt passed.  Dr. Cornish suspected that the shunt was again malfunctioning.  So he made a call up to the neurosurgery department to confer and consult. 

They recommended that we schedule an appointment for the following Wednesday when Dr. Riva-Cambrin would be in clinic, but to call and schedule an appointment with another neurosurgeon if we weren't going to be able to make it that long.

I don't know that it's possible to put into words the devastation and disappointment we felt at ANOTHER shunt failure.  We had so much hope that this one was going to work.  This one was SUPPOSED to work.  All the other options available to us were bad, worse, worser, and worst. 

And Nathan had been doing so well developmentally.  Probably the best he'd ever done out of the hospital.

So what were we going to do now? 

Nathan's First Time at Church

Since Nathan was doing so well and since the minimum of the 3-6 month moratorium of not taking Nathan to public places had passed, we decided to go ahead and take him to church for the first time.  However, we decided to only keep him there for part of the services and to keep him away from as many people as possible -- just in case.


Just for fun, I got Nathan and I matching pants and ties for church:

A Time of Really Good Progress

After three long weeks in the hospital, we were happy to all be home again.  Most of all, we were happy to have a working shunt again.  And Nathan seemed happy to be home and to have a working shunt as well.

When healthy, Nathan was always a very happy child.  But following this shunt surgery, his happiness seemed to multiply tenfold.  He seemed to always be smiling and giggling.  And, for the first time, he started to move around!  They weren't big moves, but we were soooo excited.  Mostly, he would just roll over on to his side, but it would usually be in an effort to try and reach something that he knew was around but that he couldn't get to just by feeling around.

Now we realize that most parents probably don't celebrate things like that, but when your child is over 8 months old, things like that were HUGE milestones to us and very worth of celebrating.

In addition, we were able to condense his feedings down to over 30 minutes, instead of the usual hour or so that it normally took.  Which was also wonderful!

So all in all, it was a time a really good progress.  We were super excited that Nathan was finally in a position to be allowed to learn and grow and progress without being stuck on his back all the time in a hospital bed.  So we were looking forward to a wonderful summer.

Okay, Let's Try This Again...

As soon as Nathan's infection had cleared, Dr. Riva-Cambrin was ready to reinsert the shunt.  The plan was to reinsert it in the same spot at last time.  However, since the last shunt had been infected, it is policy to move the catheter end of the shunt to the opposite side of the head.  So since all of Nathan's shunts to this point had been on the left side, this time the shunt catheter would be on the right side.

The biggest problem with Nathan having a right-sided shunt was that the surgeon would have to work the tubing through the ECMO scar tissue in Nathan's neck / collar bone area, which was why the original shunt was put on the left side.  It wasn't a major issue, just a minor inconvenience for the surgeon.

So as had become somewhat of an unfortunate ritual, we watched them roll Nathan away to the OR as we made our way to the waiting room.

It never becomes any easier to watch them roll your child away to surgery.  And we were somewhere around the 10th surgery by this point.

After another long wait, Dr. Riva-Cambin emerged from the OR to let us know that the surgery had been successful.  Again he was optimistic that the large space in which they had placed the shunt would serve as the ideal spot for the CSF to deposit.  We were also optimistic, but we seemed to be getting less optimistic with each shunt failure.

Complaining

This is probably going to be one of the hardest posts we've done.  Both to write and to read.

When we decided to start this blog, we agreed that it was important to not only accurately describe the things that Nathan has gone through, but to also accurately describe the things that we have gone through as well.  We figured the best way to help people who may be going through the same or similar things that we have gone through was to put as much "realness" as possible into this.  So that's what we've tried to do.  We've tried really hard to explain the medical things that Nathan was going through, some of which we really didn't understand all that well at the time he was dealing with them.  We've also tried really hard explain what life was like dealing with everything that was going on too.

But the hardest things to really get into and explain from this time are our feelings.  It's really not easy to express your feelings, especially when some of those feelings are really tender.  But in order for us to be as honest as possible, we have to talk about things that were going on, how we tried to deal with them, how we coped, etc, even when some of those things are unpleasant to talk about.  If we don't do that, then we are short-changing not only ourselves, but also all those who really need to know what we did to overcome the adversities of life's situations.

As a word of warning, some of our emotions (at times) will be really raw and hard to write, and thus probably hard to read, too, especially for those of you who know us now, and even harder for some of you who knew us back then.  This is likely to be one of those times.

So grab a Kleenex (or two) and keep reading...

This marked the 4th time that Nathan had been admitted to the hospital, including when he was admitted to the NICU at birth.  And, for us, it was by far the hardest admission since the NICU.

We were trying so, so hard to be optimistic and hopeful that Nathan would get better.  With each shunt problem, we really, really wanted to believe that it was going to be our last hospitalization.  But it was so hard to be optimistic, only to turn around and feel like you've been slapped in the face.  Throw in the ambulance rides, the long, late night ER visits, and all of the traveling between home and the hospital, and we were just plain exhausted -- both physically and emotionally.

At this point, I began counting the days Nathan had been in the hospital against the days he'd been at home: 140 days in, 79 days out.  The in was still 2 months more than the out.  And at the rate we were going, there was no telling when the out would catch up.

In addition, we felt that Nathan wasn't having the opportunity to progress and develop because he was always stuck on his back in a hospital bed. 

Additionally, it was so frustrating to have to call the hospital every night to get a report on how Nathan was doing instead of just walking down the hallway to check on him.

Was this ever going to end?  Were we ever going to just keep Nathan at home?  Were these blasted shunt problems ever going to just go away?  Were we ever going to be able to not worry excessively about Nathan's health...and more importantly, his life?

Really, it's hard to say what weighed more on our minds and hearts: the frustration or the disappointment.  

Also, it was really hard trying to figure out how to continue to be strong.   After being emotionally beaten up so much, it becomes harder and harder to bounce back with the same enthusiasm that you had before. 

For that reason, I was really worried about Bekah.  Truth be told, she was fighting depression.  And sometimes the depression would win.  There were times when she would just break down and cry.  And I didn't know really how to help her.  And she didn't really know how to ask for help either.   Honestly, I thought she needed some type of counseling.  But when I brought it up, she only replied that she needed to have more faith.  Which made it that much harder to know how to help her.

I, on the other hand, when I wasn't worrying myself sick over Bekah and Nathan, was fighting against anger and loneliness. 

We were very, very grateful and appreciative for the members of our local congregation who would bring us dinners every once in a while.  On some days, especially those long, hard days, it was really a lifesaver.  It was one less thing to worry about.  And some people were really nice and would ask how Nathan was doing almost every Sunday at church.

But outside of Sundays, we felt that we didn't have many friends.  Or at least any friends that lived closed by.  All we wanted were friends.  Real friends.  Someone to come over to our apartment, sit down, talk to us, ask us how we were doing.  Someone to share our burdens and sorrows and pains with.  Someone who would care during the week and not just on Sunday.  But no one ever came.

And I became increasingly more frustrated and angry.  And Bekah worried about me.  That the anger would eat me up inside and destroy me spiritually.  And she didn't know how to help me, either.

And so we struggled.  Both separately and together.  Having only each other, but not really knowing how to help each other.  But we did try.  We loved each other.  And we loved our boy.  And sometimes those were the only two things that kept us going.

Dealing with the EVD

Within a couple of days of getting the EVD, Nathan was feeling much better.  He was smiling again, kicking happily, and grabbing at things -- including the EVD on the top of his head!  Since it would have been very, very bad had Nathan pulled it out, his nurses kept a blanket over his head as a means of an out-of-reach, out-of-mind distraction.

Bekah even brought a few of his toys from home to help make life a little more bearable for him.  So, for the most part, Nathan was back to his normal, happy self.

Unfortunately, the same could not be said for both of us.  This hospital stay was probably the hardest for us since the early days of Nathan being in the NICU.

Since the EVD is basically an open wound, it has very specific rules.  The settings on the EVD must stay at a pre-specified height at all times, in order to ensure that the correct amount of fluid is being drained and the pressure inside the head stays where it should.  Because of this, the child's activities are very limited, mostly to just laying in bed.

So because of the EVD, it was really hard to do anything with Nathan.  He just had to lay there.  If we wanted to hold him, we had to call the nurse so she could clamp the EVD.  Once the EVD was clamped, we could then move to a nearby chair, sit down, and position Nathan comfortably in our arms.  The nurse would then reposition the drainage system, and unclamp the EVD.

When we needed to move again, we had to go through the same process every time.  So it was definitely on the low, low, LOW end of the fun scale.

And we had to do this for 2 weeks while they ran antibiotics to clear Nathan of the infection before they could re-insert the shunt.  And they were two of the longest, most miserable weeks of our lives.

The External Ventricular Drain

The night was short for both of us.  We each only got about 2-3 hours of sleep that night.  Bekah called around 10am to let me know that they were getting ready to take Nathan into surgery.  So I hurried and got ready and headed up to the hospital.

By the time I got there, Nathan was already in surgery.  Since the shunt was infected, they needed to remove it.  But since Nathan currently had an infection, it was unsafe to install a new one.  So the plan was to remove Nathan's infected shunt and run antibiotics for 2 weeks to clear up the infection.  But Nathan still needed to drain excess fluid from inside his brain.  So Dr. Riva-Cambrin was going to externalize the shunt with an External Ventricular Drain (EVD) for the duration of the antibiotics.

An EVD is a small flexible tube that is surgically implanted into one of the venticles of the brain.  The CSF then drains into a collection bag at the patient's bedside.

The surgery was one of the quicker ones that we had waited through.  But quick doesn't always mean trouble-free.  After Nathan had been moved to Post-Op Recovery, his nurse noticed that the EVD was not draining.  But since it sometimes takes an EVD a little bit to start draining, it wasn't something to be overly concerned about it until about an hour had passed since the surgery was over. 

Since it still wasn't draining at that point, they tried to flush it.  But when that didn't work, they suspected that it was likely blocked.  So they took Nathan for a CT scan.  Apparently, Nathan had had a tiny little bleed in his skin and the blood had seeped into the EVD tube and clotted, thus preventing the tube from draining.

So the good doctor had to take Nathan back into the OR to replace the EVD tube.

The most interesting thing about the EVD, at least for me anyway, was how unprepared I was for what it looked like.  I knew that the shunt would be externalized, but I guess I just wasn't prepared for Nathan having a tube sticking out of the top of his head with a little bag at his bedside collecting brain fluid.  It was actually quite a shocker: