Monday, March 21, 2016

Thankful to be home after today's appointment...

There are certainly many gaps between this blog entry and the last... but here is the update from today's appnt.  Thank you all for your prayers and concerns.

We are not having surgery tomorrow thanks to a UTI that has recently been causing high fevers.  I'm strangely thankful for this infection....

Shea's fluid build up and some bleeding around the outside of his brain is no better, but it's also not worse compared to 3 weeks ago.  Since he is not having acute symptoms, they will let his body heal from the UTI and try to naturally achieve equilibrium of his head/brain for 3 more weeks.  As before we will watch to make sure he doesn't have headaches, vision changes etc etc...

Then ...the same routine... repeat MRI, see what it shows, and make the decision on the surgery or not at that time.

One other odd factor is that the ventricles of his brain... have shrunk very significantly since the last visit, to almost slit ventricles.  We are all unsure why, because his shunt is set on the very lowest ventricle drainage setting.  Dr Li hopes that the brain tissue doesn't continue to follow suit and shift  inward toward the ventricles as it has in the past... but for right now the best thing to do it to continue to watch him..

For now I'm relieved to have dodged "number 10" since june.   It's a bit unsettling to be at a point that what is happening to Shea is not making much sense, not black and white, not predictable.... but God knows and I will be content.  

We also received his bone density test results, which were poor.  The treatment to address this isn't a safe option for Shea with his other issues going on right now... so that will be put on hold until down the road.

3 more weeks of some nervous anticipation, but very happy to have him home, safe and sound for Easter.

Saturday, January 16, 2016

Brain trumps cord .... latest recooperation update on Shea

Good news, and "not so good news" in Shea's rehab.   The Good news is that the "bad" news we received this week, was not so bad that it didn't allow us to come home from Buffalo on Thursday.  (and yes I do pack a extra set of clothes now when I go .... have learned my lesson)  Also, more good news is that all of the sutures are now out... and we continue to be leak free.  I check his back every morning and every night, and have been breathing a sigh of relief when I find it to be dry each time so far.

When Shea had his MRI on Thursday we were hoping to hear that his 1) ventricles had stabilized ( meaning not shrinking too fast, that can cause problems of it's own ) AND that the 2) fluid build up in the subdural spaces (outside of the brain ) had plateaued or resolved since the shunt setting adjustment one week prior.

Number 1 did happen, however number 2 did not.  Shea's fluid build up outside of his brain has actually worsened since the MRI one week prior.   He is still over draining according to what his brain can tolerate right now.  His shunt was changed from 1.5 to 2 to slow the drainage.  This is less safe for the lower back dural repair protection, but brain trumps lower spine of course - so the changes were made.

It is thought that his ventriculostomy might still be functioning as well... so when shea's shunt is programmed for a certain rate of drainage... he's likely draining more than what another child might drain, because he's got two systems working for him now. 

We return to Buffalo in 2 weeks to see how the fluid around the outside of his brain is reacting at this new setting.  He also unfortunately developed a small sore where ortho had to remove the hardware in early December bc of a stitch that was being pesky and not dissolving properly.  We are watching it carefully, and it seems to be healing and heading in the right direction.

Our request for prayer - Prolonged fluid buildup outside of the brain or any sudden changes in volume of this fluid build up can cause risk of a brain bleed.  The team is being vigilant, and checking him frequently so I'm sure this is worst case scenario... but prayers for protection are appreciated.   Also that his spine will have fusion, and that his vertebral column will be strong and protected.

also prayers for us as we prepare his diet.  Some of his labs are still not where we want them to be, requiring supplementation and coaxing him to eat more than he would like at times... but he is gaining weight quickly.  For a child who has activity restrictions , is unable to burn calories well, and who shouldn't gain weight rapidly because of mobility issues and protecting his spine...this is a problem.  I'm hoping to get more guidance from endocrinology and dieticians, but it's been hard getting them to follow up with me.

Dan is in Chicago in freezing temps this weekend... and mom is home manning the troops on a 4 day weekend!  (yikes)  Grandma Kulp is wintering down south as of today... and didn't take me with her ... !   Shea did get through 2 half days and 2 full days of school last week and was a champ.  

Thank you all for checking in.....  Liz(zzzzzzzzzz)   xoxo

Monday, December 28, 2015

This is for our helpers... please read a letter meant for YOU....

A short update, and then a letter for all of you who read, help, pray and support...

Shea has stayed out of the hospital for 4 days now!  He is stable and not in pain.  His cares are increased right now, and that proves  a bit challenging as we try get back to our normal work schedules, keep up with Christmas day and Simon's birthday craziness, as well as have all the kids home for a long school break.  (shea has a habit of being dc'ed the night before a major holiday and long school break! ) Next week he see's ortho to check the xray of his fusion and one sided hardware integrity ... then we see neurosurg for follow up and suture removal from 6 incisions, GI and endocrinology in the next few days.  Hopefully with in another week or so we will have some news on when he will be cleared to return to he's been out since mid November!

I am overwhelmed in both the good and wonderful way and the plain old overwhelmed way ... So thankful and so blessed that he is home and healing, that we are all together, that we had christmas together, that he is recovering so well.  So thankful for Dan who has done so much to keep everything afloat.  Thankful that it was my son Simon's 11th birthday today, and that he is thriving and beautiful, and has been with us 8 years now. 

Now to get to YOU, all of YOU.  Some have prayed, some have encouraged, some have cooked or brought meals, some have given christmas cookies, some have helped with childcare, some have given christmas gifts, some have given monetary donations and gift cards to help with the extra expenses of hospitalization out of town, some have given more christmas cookies :), some have given a listening ear and mentored, some have written shea cards and letters, some have given christmas cookies, and some have helped with christmas prep and delivering items.  We have some blessings we can't even account for... some clothing donations, a pointsetta, and on and on.  Pal Mac has made videos and checked in often, rallied behind Shea....Bottom line is you guys are wonderful, we don't take any of it for granted... we appreciate you and your support for our son's rehabilitation and support of our family, and lastly for supporting my cookie addiction ( and helping me to gain at least 5 lbs or more this month... )

We hope that we can bless you all someday as well.  For right now we will continue to adjust to our new and hopefully temporary normal, try to catch up with sleep and count our blessings as often as we can when we start to feel overwhelmed.

Another big prayer request is this.  It is with a heavy heart that we will opt to not persue the imaging that would have been planned to see if mechanical compression remains in shea's mid spine ( the decompression from june, that may not have been complete, or because of so many structural changes may be recompressed. ) I think the test itself may be too risky because it could involve dye in the spinal cord.  Also even if we found he is still compressed, I don't think it would be a good choice to put him through any additional surgery to try to correct it.... just too risky for his delicate system, and too much for him to endure.  Prior to the domino effect that started in october i would have said shea had 75% of his baseline strength back in his right leg, and 50 ot 60 % in his left leg.  I hope and pray that once we recover from bedrest and atrophy, and once he is allowed to stand again... that we will get that much back once again.  I hope and pray he can get all that he had once back somehow.... but i don't think anymore intervention would be wise.   Please pray for a sense of peace and not mourning in this matter...

I think of how his life might have been if not here, with these hospitals and doctors... and i feel grateful.  Things could be much much different for him.  I think of how things might have been for him without his extended family... all of you.  We are blessed...even in trials.

Wednesday, December 23, 2015

dec 23rd... counting down to Christmas.

Shea had his programmable shunt placed on tuesday.  Everything went relatively smooth in regards to the surgery itself.  Post op drainage checks and shunt series look good.  Surgical pain is managable, and not out of the ordinary.

About 2 hours after surgery shea developed upset stomach, tachycardia and persistant headache.  When all these sypmtoms were still going strong by 8 pm it was decided he needed another rapid mri to make sure he wasn't having pooling of fluid outside of the brain bc of overdrainage, or any bleeding or problems from the surgery itself.  The MRI checked out just fine. 

Note - we were able to experience his first reprogramming after the MRI as the magnets from this machine will alter the settings on his shunt.  He was originally set at .5.  This setting is the "lowest" it can go, meaning it provides the most amount of drainage .... to keep pressure off of the dural repair.  The magnet from the MRI set him up to 1.5 briefly until he was reprogrammed. 

(public service announcement, no cool magnetic toys should be set on my son's head :)

By Wednesday morning when his symptoms were still intense, they decided he was probably experiencing low pressure syndrome, and his shunt was reprogrammed to 1.0.  It seems that this change is keeping Shea mostly headache and nausea free when he is laying flat in bed with his head of bed less than 20 degrees.  Unfortunately trying slowly and repeatedly throughout the day Shea still can't tolerate sitting upright, in bed or his wheelchair.  The head pain becomes severe, and it even escalated to vomitting today ... poor guy.... but he is more alert, eating a little bit and able to find comfortable positions most of the time.   They have good pain medication for him to use when he needs it.

There was talk of setting his shunt to 1.5 tonight to see if we could get further improvement of symptoms.  However.... for various reasons we are holding off on this until tomorrow now.
     - 1.0 is safer for the dural repair, so they want to give his body a fair chance to acclimate to this setting and only make one change per day
     - 1.5 would be the next setting to chose, however we can not go any above 1.5 in the recent future... so if we do this we really hope that it will solve the problem
     - there are some GI factors that may be affecting the efficiency of his shunt and some of his other syptoms.  We've decided to address that more thouroughly all day today as well.

Right now he's sleeping comfortably.  He's stable, and in a safe place to be going through this.  Although we want to be home in the worst way ... we know help and problem solving is just a phone call away here.

I would ask prayers for symptoms to resolve on there own by tomorrow.  Prayers if they don't resolve naturally that the 1.5 setting would give full relief.  Prayers that this is truely a low pressure issue, and not a shunt failure.  If anyone is interested he has a medtronic strata valve.

No matter what is happening here, you can't take excitement of Christmas and Santa away from this boy for sure!  He is asking non stop,.... how many days, hours etc until it's Christmas day?  So fun to see....  To my 3 at home... i'm sorry i'm missing out on this exciting time for you this year.  Hint hint husband.... pictures and videos from home would be i don't miss so much.

We're hoping for an ideal scenario to be able to go home on Christmas Eve if things fall into place for him tomorrow.  If this isn't meant to be.... we will make the best of it and bring the kulp tribe and Christmas to Bryant ave room 106.  We already have had our lights up ..... we shall see.

Again and again thank you to all of his cheerleaders who have supported us with meals, videos, hello's, assistance with managing the homefront and more.  We are so very thankful.

Monday, December 21, 2015

Monday 21st, # 9 Tomorrow

Today was another good day.
- He continues to not have leaking of CSF through his lumbar incision
- continues to tolerate sitting up well
- still has the extraventricular drain, so moving is a challenge, each time level of head changes the unit must be clamped and reset
- no fever, cultures remain clean!
- He had his MRI today.  To the surprise of all, even though each hour the EVD is draining 5 to 20 cc's of CSF off his system, his ventricles have not decreased in size.  A factor in this could be that the profuse leakage from the lumbar region is now closed.  The leak itself was acting to decrease pressure and now that the repair has occurred he has more of a closed system that he's not used to.  Although his ventricles are large his pressures are very safe right now.  More proof that in order to keep his system safe and to protect the repaired dura in the lower spine a programmable shunt must be inserted.
- We are watching a very small lesion on one of the 4 scalp incisions.
- Shunt placement surgery and removal of EVD is scheduled for the second half of the day tomorrow.  Best case scenario, if no problems occur we may have 24 more hours after this 9th surgery... and then be discharged to home.  I have hopes.... but also am being realistic.

Thank you for prayers that cover him.  I've felt blessed and encouraged by many of you.  Dan and I are exhausted on the home front and here in Buffalo Children's.  Fear definitely creeps in as well, and lack of sleep doesn't mix well.... but the encouragers  and supporters have been greatly needed.

I feel my son's little system, although he's a strong strong fighter, is a bit like a set of dominoes.  Fixing one issue, often opens pandora's box.  Changing anything to fix or stablize a problem, somehow seems to start another reaction.  I'm praying for this to be the last domino... no more ripple effects.... no more snowballing. 

Looking forward to being home, .... waiting anxiously.  It will be tricky managing his acute needs in the first few weeks, school break, follow up appnts in Buffalo... but one day at a time.

Right now looking forward to Christmas, however it ends up looking.  

Today our PICU room had christmas music, christmas lights, wrapping and card writing.... we will bring Christmas here one way or another.  Will update you all after surgery tomorrow....

Friday, December 18, 2015

stable and sleeping ( 8 surgeries down this year, hopefully only one left )

Shea made it through the surgery well... he's stable and sleeping heavily in PICU.

good news- because there was so much fluid leaking they actually found the defective dural site quickly and it was in the area in the lumbar spine they could access fairly easily.  Huge sigh of relief.
bad news - it was easily found because the area that was not intact was about the size of a "female thumb." Also it was at the site of the nov 13th tear and repair, but wasn't a rip or tear now... it was a few small holes that they stitched and in general the dura had become dysfunctionally stretched and the cord was "sweating or weeping" fluid out of it.   To repair this the best they could, a few sutures were used, and then 2 layers of a synthetic substance to help reinforce the dural sheath of the spinal cord.  ( to take shea's brain out of danger when his shunt was in failure and relieve the pressure in his ventricles, the ETV was pressure was better in the ventricles... and instead the pressure was more evenly dispersed into the dura of the spinal cord... the old dural tear repair site and the fragile nature of his dura from spina bifida and repeated surgeries could not take this new pressure, even though it was "normal pressure" )

Good news - the extra ventricular drain went in well through the same tract that the ETV scope had new pathways made through grey matter.  He's talking well, has good memory, has good neuro checks and no decline in leg function.

Bad news - the soft tissue in the lumbar spine under the 2 sutures that had closed the leak last sunday was a "gaping hole" according to the surgeon.  basically the skin and 2 sutures were protecting the spinal cord from the outside world and all of it's germs.  The docs are very worried about infection, but so far he is not showing any signs, is getting loads of cultures and blood work and is on antibiotics.

Good news - the drain is taking lots of csf off of him to keep the pressure near the repaired dura as safe as possible.  The manometer is set so that if any pressure rises above 5 it drains automatically, he is not clamped.  ( before he was allowed to go up to 20, and if over 20 for 5 min they would manually drain ) now they are keeping pressures extra low to protect the repair.

He is required to lay flat for the next 48 hours, no raising the head of the bed.  We are using a folded towel as a pillow, and he can log roll left to right.  They are watching the dressing closely to see if he's leaking any csf.  Late on Sunday if the leaking is absent, they will start to let him slowly sit up.

I'm thankful that this low pressure is not causing him headaches or nausea since it's a sudden change in the dynamics of his system... we hope that when he is allowed to sit up, this will continue to be the case.

If no infection and no leakage from the lumbar skin of csf, then on monday or tuesday they will implant the programmable shunt to keep his pressures extra low as they are now.

another piece of good news is that they could mostly visualize the orthopedic hardware that is left in the left lumbar spine and it's not lose or displaced.  He will continue to wear his brace at all times if out of bed for the next few months.

we have goals,... and a plan... let's hope it's complication free.  Thank you a million times over for all the prayers, encouragement, helps....  very humbling....and vitally needed.

in surgery now, what is the plan, how did we get here?

- Today's surgical plan:

All involved, neuro/ortho feel that a full exploritory surgery to find the leak is too risky.  There are 4 likely places the leak could be * the site that tore and was repaired in the lumbar spine on nov 13th, the site that tore and was repaired during the june surgery, * the front portion of the spinal cord where it had to be released from the vertebral column in previous surgery * the thorasic nerve root that was removed in order to place the cage in june.   Because of severe anatomical differences and scar tissue from his spina bifida and the previous surgeries they have to work from the top ( normal dura ) down and would be too invasive, make his long scar even longer and be too risky.  Of these 4 sites most likely 2 of them could be very difficult to fix.  The fear also with this method is that although his ventricular pressures are now "in the high normal limits"... that even if they found the tear and repaired it that the high normal pressure would still be to much and re tear the fix. 

The plan we have decided to move forward with is to focus first on getting his ventricular pressures to "low normal".  Also just opening the area of spine where we can actually see the leaking through the skin, and pray that it's there in the dura and obvious.  If so Dr Reynolds will repair.   ( Dr Li is not here, but has been consulted repeatedly throughout the last 24 hours and approved and tweaked our plan... Dr Reynolds comes with very good reports as well )  Thirdly the fascia, soft tissue, skin will be thouroughly reinforced from the inside out in layers, rather than just incisional stitches.

The next step to KEEP ventricular pressure low, so that if a dural repair is made it can stay intact, and if a significant leak can not be found and repaired that pressures are nice and low to slow the rate of leak.  A slow leak contained within the body is minor problem as compared to any leak that has a channel to the outside world.... they are saying we are extremely lucky that through all of this, since mid november that he has not contracted central nervous system infection.

How do we keep the pressures low?  He will again have an extraventricular drain / monitor.  We will again be in PICU.  Again, anytime he changes positions or moves the position of his head his monitor has to be zero'ed ( means a nurse comes to help with every single position change, even raising or lowering the height of his bed, or the angle of the head of his bed ) They are going to drain a significant amount of csf to decrease pressure, and watch him until monday or tuesday.  If the lumbar leak no longer can find a way out of the back with the better repair down there and the created low pressure system we will know he's headed in the right direction. 

We know that he needs lower pressure than the average kid, to protect the tears... so at this point if all has gone as planned on monday or tues a shunt will be placed.  He will have 2 mechanisms in place to keep pressure low now... the shunt and the ETV.  Today the resovoir will also be removed.  Another important reason to wait to place the shunt other than to make sure the "low pressure fix" can be accomplished and that it actually works, is so that we can have 48 hours of clean csf cultures from time of last external leak.  A shunt can be placed when there is infection or chance of infection.

This is where we are now.... below is how we got back here...

wednesday after discharge we had a nice 26 hours at home, some decorations were put on the tree, lots of hugs and cuddles were shared, and a sleep in our own beds.

- thursday afternoon we received the news that we knew we would, but we hoped we wouldn't.  The lumbar leak was infact Cereb. Spinal Fluid (CSF).

- Neuro had instructed that this type of leak was not one that could be left unaddressed, that it wouldn't heal itself without intervention.  If he was not showing symptoms or not leaking through the sutures / skin... then it could be done after Christmas, soon but not urgent.  

- However thursday afternoon the CSF again began to escape through the incision and sutures.  The risk of central nervous system infection is too great.  The original plan from both Dr Li and Dr Sanders was that the dural tear would be repaired by ortho at home....   after evaluating the complexity of shea's case, the fact that Rochester neuro doesn't deal with ETV's, the fact that some staff at Strong didn't even know if Rochester neuro would take Shea back as a pt, and that Dr Sanders wanted a neurosug to be present during the procedure it became clear that Buffalo neurosurgery would again take point on this issue.

- by 5 pm on thurs after doctors had conferred "shea's" personal resident, Dr Meyers called and said Shea needed to be admitted that night so that cultures could be started, mri, bloodwork etc...

It's now Friday the 18th, one week before Christmas, and he has just been wheeled back to surgery.  He tried to be so brave, but the tears and the screaming and calling out have come.  I will see him in a few hours, but ohhh my heart is broken for him,... and for my family at home.... what this son of mine has been through.