tentatively hopeful !?

Day time has again brought more stable pressures.  That is his trend, good pressure when awake, pressure rises when asleep.  He had many spikes last night.  Nursing was in and out of his room all night.  More fluid was taken off of his ventricles to relieve the stress.   It will take another few days to get the results of the clear fluid that was leaking from his lower back, to rule CSF leak in or out.  The plan since 7 am is to try the next 24 hours without draining excess CSF off his ventricles when his pressures rise incase it is a lumbar leak, and to see if he can still be asymptomatic.    ( I really wonder if we’ve had a slow leak for months that wasn’t diagnosed, or if one of the two previous dural tear repair sites was stressed now that CSF flow was improved after the ETV procedure….) It’s a bit of guessing for now until we get more tests back. 

Tonight they will watch him carefully, and if his pressures rise above parameters we will attempt not draining him to see how he responds in regards to vitals and physical stability.  On call neuro will walk the night nurses through all the decisions with Dr Li’s input.  He also had the rapid MRI again today.  The lateral ventricles are a bit reduced compartd to last week and the cisterns that the 3^rd ventricle now should drain into bc of the ETV show fluid in them.   We will see how it goes without draining him for the next day, and there is a chance the drain can be removed in the next day or two.  It’s likely we will have another rapid mri soon to recheck the ventricles after not draining for 24 to 48 hours.

Once the lumbar fluid leak results are back we can take all of this and try to make some decisions.  It sounds certain that we will be in ICU until tues night or wednesday at minimum.

If we do have a csf leak ( which we continue to hope that we don’t !!), Dr Li would help to decide if it needs to have a patch repair or not.  If so then Dr Sanders would do this in Rochester after we are dc’ed from here.

Our update today is a whole lot of unknowns,  but he’s comfortable and playing.  So many in ICU are grave and fragile compared to us…. So we are thankful. 

The barbershop quartet just sang their way through the icu halls and we’ve hung a few Christmas lights…. So we are hopeful  for an easier night with less bells and alarms, and good progress tomorrow.

evenings and nights .... our witching hours

Shea had a really good day, followed by a awful evening development. The good part of the report is that fever was very low or absent today, no pain, no headaches, and he spent a good deal of the day up in his chair. He didn't have any high pressure issues either.

Saturday morning the dressing from december 4th back surgery was due to come off of the lower spine. Saturday I commented to the nurse how sweaty he was, i needed to change his gown because of dampness on his back.

Again today another "sweaty back". I decided to take a look at his incision just to make sure. As i watched him a drop of clear fluid came out of his incision. I dabbed it away with gauze. Then another drop, then another... and this continued.

I asked the nurse to paige neuro. They came and assessed and called Dr Li. It was decided to take a sample of this fluid to send off to the lab. We are hoping above hope that it's simply serous drainage, and not CSF leaking. ( We had suspected CSF leak during our emergency room visit and admission in november, but they ruled it out. During the Nov 13th surgery ( # 4 of 7 since june ) there was a dural tear which was repaired.

This was all very painful for shea, even with morphine. The leak was from incision on his keloid...which is very sensitive tissue. He would cry out that it made his legs hurt and his pulse went through the roof. I hate hate hate seeing him struggle over and over again.

I asked them to give him versaid to calm him down and possibly make it so he wouldn't remember as much, because the next step was to put 2 stiches in to close the leak to outside air.

Prayer requests... that tonight his pressure would not spike, that temp would not spike and that the steady flow of clear mystery fluid is NOT a CSF leak. Even with none of tonight's issues considered we have to make it through at least 2 full days and nights without pressure spikes of 5 minutes before they will consider pulling the csp monitor and csf drain.

Thank you for your ongoing support and prayer.

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.

searching for the finish line

Since last writing we did in fact make it home for thanksgiving.  We have been blessed by many with help, meals, and encouragement.  My last entry was hoping for the homestretch.  It was not to be, and a whole new race has been started.  The last 6 1/2 months for Shea has been a grueling marathon.  We have all tried to refuel and refresh, but the run has been long and uphill.  Right now we do not see the tape of the finish line, and that makes it harder to keep the pace.  My boy is now up to surgery number 12 total, 6 of which have happened since June of this year.  I will be honest and say I do hold tight to the fact that he is stable from a medical standpoint, and his "general health" is good.  We are much luckier than so many others who have children battling life threatening problems.  I do give thanks for this.   I know of a sweet baby girl who we played briefly with last week on the play deck... who has gone to heaven without warning.  Our struggle can never compare to so many out there.... but we are tired.   We are angry...without anyone that is currently involved to really be angry with.  We are angry with spina bifida, we are angry with 4 years in an institution causing malnutricion and health problems that could have been avoidable that likely contribute to much of this struggle, we are angry that a boy who should be racing and jumping and wrestling and making messes in the mud is laying in a sterile white hospital bed again.  But... we are supported and blessed in many ways.  So we are humbled....  and we hope hope hope for a light at the end of this all. 

We were able to get home the evening before Thanksgiving, with a drain.  We had a difficult but much needed 7 days at home thinking and hoping we might be ending the race,... but as we pressed on, each day showed clues that the end of our run was not to be.

Drainage didn't stop as it should.  Drain site deteriorated, low grade fevers persisted, trips were made back and forth to the clinic for observation, watching, waiting.  Wednesday night the drain hole opened wide and a rod was clearly visible, and also resting in a location it didn't belong.  At the same time another wound was forming from pressure from the inside out because of one of the new lumbar screws that we now know had started to loosen and back out of it's secure spot in his vertebrae.  Shea followed all the rules, infact we gave him more rules to follow than the doctors instructed... but his body and bones wouldn't hold up once again.

Thursday was spent outpatient, appointments, planning, scheduling, booking, xrays, lab work and on....

Today the screw that loosened was removed, and the portion of the rod that moved had to be cut.  They did decide that because it doesn't seem to be infection based, that it would be wise to close the incision for now, rather than rush into the wound vac.  That will all be watched and assessed carefully over the next few days.  I am thanking God that the very long central incision did not need to be reopened today as it has 5 times in the past.  Another small blessing is that the stitches were ready to be removed on that long main incision and they were able to do that under general anasthesia today and not make Shea endure more anxiety and pain of having this done while awake. 

We have started a rather aggressive regimen help him control his anxiety and panic type attacks with 2 medicines, and a plan for counselling related to repeat medical trauma.  This prong of treatment I feel is just as important at this point.

Our next phase of the race is solving mysteries.  We need to get to the bottom of why his bone continues to reject hardware, why every tme we fix a problem it causes 2 more.  Between what the doctor has suggested and what I plan to ask for, we should be investigating nutricional iput, GI efficiency of absorption, bone density issues, allergy, continued ruling out of infection and possibly working with endocrinology.

We will watch anxiously to see how the wound repair progresses, and if having fixation on the left side of his lumbar spine only will be able to fuse and succeed.  We have been given some options of what may need to happen with larger more involved surgeries of anterior fusion through the abdomen, or pelvic fixation points if it does not maintain intact.... but I would like to not think that far ahead yet.  We will take saturday and sunday as they come.  We are on the surgical schedule for monday for either wound vac application or the surgery mentioned above... but our hope and prayer is that none of those will have to happen.

As for the rest of the family.... the kids are strong and resiliant, but could also use your prayers... especially danielle as all of this takes it's toll on them also.  Dan... my partner.  Thank you for all of your hard work, and your love for our kids. 

Thank you to all who are helping us through this is many different ways.  I have an image in my head of the family trying to get shea through this race toward the finish line, and many of you offering sips of water, clapping, cheering, manning the first aide tent, directing traffic... etc etc.

We will hit the finish line at some point... I'm just not really sure when... so until then... keep on cheering.   Thanks....

Hoping for the homestretch...

It's been a few busy days since the last update.   So far everything that has been tested is still negative for infection.  CSF leak has been ruled out.  By Friday evening the shunt type symptoms had really calmed.  The drainage began to slow, but was still too much.  Saturday was spent waiting 1) to see if the drainage would slow enough to not require intervention and 2) waiting for Dr Sanders to come home from Boston. 

By Sunday morning it was evident that the drainage was still too much and would need to be irrigated, and treated with antibiotics so that it would not turn into infection... it was also too large to resolve itself anytime soon. 

This morning I was very anxious because the headaches returned and some light sensitivity....The hope is that once the hematoma is gone and not stressing the body or causing pressure in the lower spine region that the shunt symptoms will go away.  Shea has been incredibly lucky that he's made it to 9 years old and has never needed a shunt revision.  My hope is that his shunt can clear itself and that this all will come to an end soon.  I'm going to be optimistic!

Right now it's hard to tell what the shunt will do as he is still being asked to lay flat and is also on strong pain meds.  Hopefully by tomorrow we will get more upright and be on a less rigid medication schedule so we can see what happens.

He does have 2 drains in, and they are doing their jobs.  The surgeon said that if things go ideally, we may be able to go home in a day or two, and that the drains would go home with us.  I could manage them from home.  The next 24 to 48 hours will tell us a lot... again i'm going to be optimistic and say he'll be eating turkey from home, not on the 8th floor!

Shea's admitted again, drainage problems / and csf leak or shunt issues

Shea continues to battle an on and off fever.  His drainage is much more than it should be at this point and he's been needing dressing changes multiple times per day.  It is confirmed from imaging that he has a very significant hematoma.  The question is... may he also have a csf leak from a failure of fridays dural repair during surgery. 

Originally until yesterday evening, csf leak was thought to be low on the liklihood scale.  Then last night he developed a severe headache, worsening when upright, and we watched him through the night.  By morning he had light sensitivity and eye rolls.  For a bit he even had mental status changes, ( not able to say the alphabet etc ).   So of course we were off to the ED.  Dr Sanders is in Boston, and Dr Cook has stepped in to cover in his absence.

So far the CT scan shows enlarged brain ventricles.  Now we have to figure out why.  Neuro here at Strong stated talking immediately about shunt revision surgery, but i strongly advocated and was backed by others ( in buffalo neuro and roch ortho ) that  we need to rule out other causes before we jump to that conclusion.  Right now we have lab work off to test the fluid that is draining so profusely right now.  ( they did a needle aspiration )  Once we find out if it contains CSF ( cer. spinal fluid ) we will know which direction to take with treatment. 

Thank you for prayers not only for Shea here in the ED, and for doctors to be likeminded and on the correct track... but also for Dan at home, and the trickiness of managing the family, and all that entails that we usually do as a team. 

Shea has been in ED for 12 hours now... we were officially admitted at noon and waiting for a room, and i'm told one is opening up now in the children's hospital and we will be moved soon.   I will keep you posted as we know more.... 

A pretty good day today, to get ready for tomorrow's very busy day...

I'm yearning for my pillow, so this will be short and sweet...

He was able to go off the morphine today and is doing well on an oral pain med instead.  He also spent the second half of the day off of his IV fluids bc he's drinking well (ish)

After midnight tonight he will again be nothing to eat or drink to prepare for tomorrows MRI under sedation.  He will be given something by IV to relax him before going into sedation so that he doesn't fight and panic to protect his back ( and his spirit xoxo )

Dr Sanders is allowing him to sit up in the recliner without his brace, so that has been helpful for his pain control and his anxiety.

He is still fighting off that fever.... when the tylenol wears off it goes up to 102, we catch it with tylenol and then it goes back down.  It would be great if the fever breaks by tomorrow.  Post op fever is completely normal... i'm told no real intervention as long as vitals are good and pt stable, unless fever goes past post op day 3, then maybe some blood cultures to rule out infection...but it's unlikely.

He did great with his sitting, up three times for an hour each time.  At first his legs are very painful, i think bc of dural tension....but after positioning and getting settled his pain calms down with sitting up.

His brace was adjusted today, but it's still not a good fit.  The orthotist that came thinks a new one is needed, because this one has been adjusted so many times and shea's alignment and edema / size has also changed so many times.  Not to mention the first one was made in june, and 9 yr olds tend to grow!  He should be casted for this tomorrow, with a one day turn around time. 

He enjoyed some nibbles on real food today, and especially that chocolate milkshake :)

Dressing change is also scheduled for tomorrow as well.  That's all i can think of for now...  thanks for checking in.