Friday, December 11, 2015

Crash Course in ETV... aka at least we were home for a day and a half.

Disclaimer! - if you are only looking for today's update skip to the last few paragraphs.

Shea was discharged from Strong and our ongoing spine saga ( see last blog ) on monday.  He was able to spend a lovely tuesday at home.  I even made it back to work in the office rather than working from the hospital for a day, thanks to nurse Renee coming early.

Wednesday we had a planned follow up appointment with Dr Li in Buffalo.  This was an appointment I had scheduled  due to the few days of shunt related symptoms in November that sent us to the emergency room right before surgery number 5 and 6 on the spine.  ( His ventricles were enlarged )  I had asked Rochester neuro about follow up imaging of his brain before discharge, and they responded with a "no" because his shunt type symptoms had cleared.   As some of you know, I've had a less than ideal track record at home with neurosurg.   I asked Dr Li to see Shea so i could rest easier, and although he had no openings... he made a spot for us.

Upon exam, from asking some questions, and checking the pressure in the back of his eyes, Dr Li didn't want us to return home without a rapid MRI first.  His gut was correct.  Not only had the ventricles not returned to baseline, but they were actually significantly larger than they had been during our ED visit a few weeks ago.  We were admitted right away... and the plan was made for surgery friday, after blood work and cultures finalized.  Shea's shunt tap showed complete blockage of the proximal shunt catheter.

We were given the option of doing a full shunt replacement or if a more complete presurg MRI showed proper anatomy ( enough space to comfortably avoid the basilar artery ), we could opt for an EVT.  EVT is endoscopic third ventriculostomy.  I had never heard of this procedure.  I'm not even sure they do them in Rochester.   As a mom who is always really well educated on my kids conditions and treatments, this was a shocker for me.  I am very pleased they let me weigh in so much on the final decision.... but having to make that decision and learn as much as possible about it in 24 hours has been a load on my shoulders.  To over simplify it, instead of the shunt tubing dumping the excess cerebrospinal fluid into the abdomen,  no shunt is used and a hole is created in the base of the third ventricle so that the fluid can empty into the natural cisterns and passageways of the brain mimicking normal drainage.

The pros of EVT are that it is done with the scope, doesn't require any hardware, and would not have tubing that needed to travel through the neck and into the abdomen.  Other pros are that if you successfully get through the first few years without a failure, chances are very good that you won't need an EVT revision or shunt for the rest of your life.  Also a somewhat lower chance of infection, and there is no "hardware" that can malfunction.  Also EVT can only be done during times when the ventricles are enlarged.  If we didn't try it now, there may not be a good time to try it in the future if needed.

It is not a magic fix to be sure, especially in the population of spina bifida.  The early failure rate , meaning in the first few months specifically and in some cases the first few years is just as high if not higher than shunt failure in sb population.(50%)  The hospital stay is longer, and the follow ups after surgery are more frequent.  Also some risk of change in short term memory or hormonal regulation are a rare side effect.

You can see why this decision was tough... EVT would mean more upfront work and risk, for a hopeful better long term outcome and permanant solution.  Opting for the shunt may give us better initial success...but in the long run just as likely to fail or more likely to have infection.

I sense that God orchestrated catching this complete failure and dangerous ventricle size while we were in Buffalo with Dr Li and still stable...  not while in an emergent failure scenario with likely seizures, loss of consciousness etc etc, and in a position to not be stable enought to get to Dr Li for intervention.   Dr Li seemed to be leaning toward the EVT, even though he was clear it was our decision.  I did stress over the options, but in the end decided to go with what it seemed the doctor was suggesting because he has earned my trust and shown repeatedly that he is vested in shea's outcome and well being.  ( i've never seen a surgeon walk the stretcher from pre op to surgery himself... i took notice once again )

So, where do we stand now?   The EVT was a success and done with technical ease.  His non functioning shunt ( brain and neck portion ) was removed, the distal catheter ( from his neck to his abdomen ) will stay in place because it is harder / more risky to remove this than to leave it alone.  He has a resivour in place that will stay permenantly.  This is so that if he does have failure symptoms they can still do a "tap" to get information or to drain excess fluid quickly.  He has a pressure monitor to tell us what his intraventricular pressure is.  It needs to stay below 20 or CSF will have to be drained.  Shea's pressures were beautiful, < 10 until 8pm.  We've had a couple of spikes above 20 that were short lived.  When he lays on his R side he's in the high teens, but it's better when he's on his L side...staying in the low teens.  Shea's also showing rapid heart beat, 170 at times while asleep and on morphine.  The plan was to let sleep and morphine lower the pulse, but it hasn't been successful yet.  They are discussing whether or not to do an ekg tonight.  For now they are giving an additional bolus of IV hydration.  We will stay in ICU until Shea tolerates activity and position changes with good pressure and the csf pressure monitor is relieved.    He also of course has a headache, but that is to be expected after having people poke around in your brain all day right?!

We are "cautiously" optimistic... a phrase we've heard just a few times in the last few months.  I don't know if he will recover brilliantly and we'll be on our way home early in the week.... or if the EVT won't take and then we end up shunting as well.  Passing of time will bring that to light.

Up until wednesday my mind was so consumed with his lumbar bone healing and the outcome of his hardware fixating and fusion succeeding that his shunt hadn't even been on my radar.  Now... it almost seems that the spine is getting pushed out of the scene.... as we focus on this now.  We were supposed to have lumbar incision stitches removed on tuesday along with our first weekly xray to check the integrity of the remaining right sided lumbar hardware.... so we shall see.  Plan B may have to be made.... looks like a temp has just started to emerge as well.  Expected after such a day.  ( yes he's on lots of preventative antibiotic again )

If you've read this far, you are a trooper and should earn a prize.  Thanks to everyone again and again for the helps, prayers and encouragement.... we shall see what the next day or two brings.


  1. Thanks (as always) for sharing and keeping us posted. Praying for all of you!!

  2. Know that Lee and I are both reading your post and praying for Shea and you. I ask the Lord to give you His peace in the midst of this storm. Blessings on you both.

  3. You're an amazing mother, Liz...I pray for your strength, endurance, patience and love.... God bless your sweet Shea....he's a tough little brave.