Face of hope

Face of hope
Courtesy: TIffany Kay Photography

Friday, October 25, 2013

Shunt Revision #1 - well, sort of......

After much back and forth and weighing options as to where to turn next, Dr. R ended up taking Meagan back for exploratory surgery today.  We butted heads a little bit on the plan as he is very conservative surgically.  Don't get me wrong, I'm completely ok with that - the last thing a hydro parent needs is a "surgery happy" neurosurgeon. However, with a kid like Meagan who is pretty complex, when she presents with repeated symptoms and all other logical causes have been dutifully ruled out, my feeling is surgery to explore her main issue (brain and shunt) cannot be ruled out.  Dr. R and I differed on this a bit and I could definitely feel tension in the room before he took her back.  I stood my ground, however, because I truly felt in my gut we needed to look into her head - I felt this is where the true root of her recent symptoms stemmed from and warranted surgery.  Initially this was not my idea - he had said a few days back if all her other tests came back clean, the next step was to explore the shunt.  My guess is that his hesitation today was simply because Meagan was not in acute danger - she wasn't crashing or in an immediately threatening situation so he hesitated.  I understand, but ultimately, Meagan is my child.  She has been hurting. Throwing up. And now this week losing oxygen at night and having new strange tremors (which happened AGAIN last night by the way).  It's my job to push for what could possibly provide insight into relieving these things, and unfortunately this time, it seemed the answer was a shunt surgery.
Another night of desats and tremors

Another puke and pain episode

Dr R's plan was to possibly revise her shunt if hers proved to be not working correctly and also to place a second catheter in the back of her head, as that area has a lot more fluid than the front.  In a simple visual, there is a lot of brain in the front of her head, and a lot of fluid in the back of her head.  The brain in front has very little fluid - which is actually not good. We do need a certain amount of fluid in our brains to keep our brain tissue moist and things flowing properly.  So Meagan went to surgery around 1530 today.  About an hour later I got a call she was still being operated on but things were going well.  I was anxious to hear the results of what Dr. R found.

Snuggling Daddy before surgery

Dr. R came into Meagan's hospital room to chat with me.  But in Meagan, her insides have done essentially the opposite of what most Hydro kids who are shunted do - her ventricles have shrunk so much that they have almost collapsed.  Therefore, when the catheter sucks fluid from her ventricles, after a certain amount of time there is so little fluid there that instead of CSF, the vacuum from the shunt simply pulls the ventricle walls in on each other and they collapse.  This causes extreme discomfort, pain, and can definitely account for Meagan's behavior before she vomits.  Once she vomits, the forceful nature of it pops those ventricles open temporarily, thereby relieving the immediate pain, also explaining why she was happy after a vomiting spell.  Dr. R also noticed that at the back there was so much fluid it was really imbalanced with how the front of the brain needed to drain.  It's like the front has too little fluid, and the back has too much.  Essentially, to make an easy visual, imagine Meagan's head like a big circle.  The back half of the circle is filled with fluid and the front half with brain tissue.  The top of that circle is open (from where she had her cranial surgery last year)... these spaces provide pressure relief, but in Meagan's case, it's almost like her brain tissue is "running" for those vaults - craving that space, thereby leaving a big vacuum in the back.  On top of that, add her collapsing slit like ventricles, and the fact that she has very little fluid to drain, and we have a whole big mess.  People may initially think too little fluid is good - as her whole problem at birth was too much. But it isn't so.  Just as too much fluid can cause symptoms and brain damage, so can too little.  It all has to be in balance, like most things in our bodies.  So it presents a whole list of new problems and complications as to how to "fix" this.  

The clinical term for some of what Meagan is experiencing is called Slit Ventricle Syndrome.(This is also an excellent article).  And for lack of a better word, this is an absolute beast to deal with.  Because of all the push/pull, back and forth, too little/too much fluid, it is a nightmare to manage.  Many times, kids are left to "band aid" solutions - medicines for the pain episodes, and no way to really "pop" those ventricles back open. Sometimes there are ways but it cannot be done at the time, or cannot be done immediately, and sometimes no matter what, symptoms continue to recur.  However, this is where my thankfulness for Dr. R comes into play.  He refuses to let Meagan just sit and be a victim of SVS.  He, instead, wants to fix it.  That said, it is not an easy fix. And certainly not a fast one, nor a guaranteed one.  But at least he wants to be proactive.  

Meagan's MRI - notice a lot going on up front (your left) with little fluid and a lot of fluid towards the back surrounding the back 

 Meagan's CT scan is on the left below - notice her shunt catheter almost sticking "in to" the tissue and the lack of open ventricles in her brain (as opposed to the typical scan below where you can see the black "butterfly" area in the center which is what normal fluid filled ventricles should be.  In Meagan's scan, you can't really see her vents at all - which is what Dr. R found in surgery today - hence the term "slit like").

On the way up to the PICU after surgery, Dr. R had Meagan go to CT to obtain a 3D scan of her head post-op.  This, he said, will provide him with the "road map" he needs to start putting together a management plan for Meagan.  Sometimes it will mean watching and monitoring and sometimes it will mean possible revisions or extra catheter placements, but overall, we need a plan.  For tonight, his goal is to just see how she does following this initial surgery.  He ended up not changing Meagan's shunt - but he did notice it was not dripping evenly.  The CSF would drip, drip, drip, and then stop, and then flow, and then drip, drip drip.  This is not ideal at all.  He cleaned out her shunt, flushing it to flow more evenly and measured all the pressures in her head to bring things more to a balance.  Next, he noticed because her ventricles were so slit, the catheter was not in the right place at all.  It was too close to the ventricle wall, thereby sucking it in intermittently, and contributing to Meagan's symptoms.  He shortened the catheter and re-placed it more central to her ventricle so it was in a better spot to minimize collapse.  He was happy with this and felt now that he knew a lot more about how complicated Meagan's situation was, we could stop at this point.  If by chance she doesn't do well the next day or so, he is considering putting in an EVD (external ventricular drain) into the back of her fluid space.  He would let it drain and watch over a certain amount of time how it drained and how she handled it.  If it seemed to help, at that point he would consider putting a second permanent catheter into the other fluid space in the brain. So for now it's watch and see and if he needs to take her back to the OR tomorrow he will.

Bloodshot eye - always gets this way before an episode.

I'm sure it seems like practically a book I just wrote above - but, that's how tricky Meagan's case is.  Dr. R said in doing over 10,000 shunts in his career he's never had a case like Meagan. Go figure. :)  He said she is just so incredibly complex and tricky that the simple answer just never seems to fit with her as it always has a domino effect. It was wonderful to talk with him in detail, and really have a heart to heart about the situation.  The conversation was informative, extremely detailed, and also light hearted.  We seemed to get back on the same page and agreed to work together as a team to manage this new situation with Meagan and get her the best relief and best improvement possible.  We joked a little bit about the tension earlier in the day, both realizing we had been stressed about Meagan's situation and had been unclear with each other about what the expected outcome was.  I reiterated how grateful I was for such a good doctor looking out for Meagan and her needs, and he repeated how glad he was I was such a good advocate for Megs and stood my ground when I felt something was wrong.  I know he truly cares for her - the way he snuggles her, the way he so passionately wants to do his job, down to the way he even makes her incision crooked so her hair doesn't grow back in a straight line and never shaves her hair - every way he deals with her is with love and wisdom, and that is irreplaceable.  

Meagan's incision - such care taken to not shave any hair 
and make a crooked hairline so things grow back naturally.

So that is basically where we stand.  Your thought now may be "uh, exactly where do you stand?" LOL... it's a ton of information to digest.  Dr. R said if I read on everything going on with Meagan I would probably never stop - that's how much information is out there, and how varied and complex her situation is.  I am honestly not surprised - Meagan has never been a "by the book" kid, and so why would she start now?  I'm very hopeful we can get this under control and also very hopeful we can see her new symptoms (her desats and tremors) subside as we move forward.  It has been quite a crazy 9 days getting to this point, but I feel like we are finally at the top of the mountain.  As I look forward, there is a larger mountain in front of us for sure - but I don't' feel intimidated because we know now what is in store.  And with great family, great love, and great prayer around Meagan, I have no doubt she will be standing atop that next hill in no time.  Dr. R won't settle for anything less.  And neither will we.

 Resting post op...rocking out the Halloween pillow in the ICU


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  2. Molly and Brian,
    You have my many prayers as things move forward! You are amazing parents of one amazing little girl. I can't get past her big beautiful eyes.

  3. ♥♥♥♥♥♥♥ prayers from us! What a week!

  4. UB, AA, and CB are always nearby! Let us know what we can do. :)

    1. Will do!! So lucky to live near you all!!

  5. I'm so sorry to hear she has slit ventricles. My ventricle are also slit which is why my pressure is eith too low(most of the time) or too high. It's not an easy thing to deal with but I am happy you have a plan together. She seems like a very sweet, brave girl and she won't let anything stop her! I am praying you don't have to do anything and that it stays stable. I will pray for you are your family.