Shunt take ??

Ive been apprehensive since last weeks appt with my neurosurgeon but tried to block it out as much as possible over the weekend but when feeling well I wanted to have a good weekend which I did. Thanks to taking zofran pre-emtively in the morning and right away at night I only threw up early sunday morning (awoke out of a dead sleep and thanikfully have a garbage can next to my bed). Otherwise days have been pretty good with just occassional milder headaches and nights so-so.

So anyways Dr.Bragg (my neurolsurgeon) called while I was on my way home from Children's Hospital (Milw) with some information and questions. There's not even a slight chance i'll remember everything we talked about as I think we talked for well over a 1/4 of my drive and covered many things but also kind of went over the same things multiple times trying to iron the possibilities out. The jist is that they are wanting to do ICP monitoring again with the VP shunt in place to see what pressures are and then probably tie off the VP shunt (a relatively simple knot) and watch to see what pressures are. If it goes 25-30's (intracranial pressure) then it's a matter of figuring out the shunt still but otherwise possibilities of potential other options several of which she somewhat touched on like 3rd ventriculostomy (hole created so ventricles communicate I think) this I dont quite get how would work in MPS given the storage material continously but will cross that bridge if needed. Other things she mentioned was having Opthalmology come by to see if they can weight in (I get vision affected with the headaches but papiledema (pressure showing up on the optic nerve) doesnt show up often in MPS pts. She said it's possible it is more of an obstruction problem and wondered if we had done any imaging with dye although im not sure now if she meant of the brain to see if there is a place that is causing the problem or if she meant through the shunt to see if the dye gets through.  She and her colleagues wondered if they may be able to find a non-shunt way to manage the pressure issues so we will see. She talked about 2 areas especially in MPS that present different in MPS and could they isolate a specific area that pressure/problem is coming from and find a solution to it. She didnt know but apparently dealt with this in MPS pts while she trained in Utah, I kind of figured she had to have saw atleast a couple MPS pts there (Primary Childrens Hospital). I had asked her if she thought the location of my 2nd valve could be affecting the shunt (sits at my collarbone was originally below breast) and she said when she was training they where able to demonstrate if 2 valves where put next to each other the back pressures where not regulated at all compared to when the 2 valves are spaced further apart so it may be a possibility she isnt sure.

  I dislike very much not knowing solid plans and dont feel great about this (not that I dont trust her) and hate that once again have to re-schedule and re-try to figure out everything I have going on. I wish this damned shunt would just work and I wish it would just be done with so I could do other things. I feel bad for Dr.Bragg because clearly it has to suck when you struggle to help a patient and answers arent clear but I also just pray and hope and wish we figure something out soon. Please just say a prayer this monitor shows something and we can move on with an solution quickly. In the mean time tonight ive emailed one of the drs who created the Enzyme replacement drug I receive each week as he is as familiar with MPS as anyone I know to see if he had any take on all of this. I have seen a few other MPS I pts have similar issues and suddenly they get to a place their shunts just work well so thought maybe this dr would have an idea.

Dr.Bragg's nurse is working out the details of all this so if ive forgotten anything will edit this and will update when I know more.
Thanks,

Erica

Post-Op Appt, options, ideas, insight.

So where to start? I doubt im going to get all this info correct but saw my Neurosurgeon for fup late this morning regarding post-op from last weeks shunt revision. Turns out the UTI from last week grew back a bug that is resistant to many antibiotics including what I was on (it did help some interestingly) and Dr.Bragg plus her staff had to do isolation precautions. Interesting.  So she dd a shunt tap to see how the shunt is working as although my headaches improved since last weeks surgery they arent completely gone and she is concerned about this. The shunt tap took longer than normal but eventually they where able to draw back a couple cc's of fluid although the valve appears to be sluggish again. She talked about some options we have but is going to present my case on monday at their Peds-Neurosurgery weekly meeting and bounce ideas off of her colleague. (always makes me a bit nervous as he is the one who did not believe me severeal years ago) but she has promised again she isnt going to give up and thinks we can get the headaches better under control. Some options include (and this is where I should have taken notes so will have to update after mon) either moving the shunt to a different ventrice where less tissue (choroid plexus) to get in the way of the draining, adding a 3rd valve to slow down flow (though she is concerned my ventricles want to drain more fluid but when we didnt have the second valve I occluded and now when I have the second valve am still occluding because pressures appear to fluctuate variably.)  or adding a programmable shunt which we could then adjust the pressures as needed. Other options might include optic nerve fenestrations (if it is more of a PTC phenomena) which might either help the shunt or allow us to get rid of the shunt though this seemed less on her radar but I could be wrong. Im not sure which she is leaning more towards as she talked about all fairly equally. Other option may be that we need to put the distal (end) portion of the shunt tubing in a different location such as a vein to aid in better drainage and absorption as she said it is possible my body isnt absorbing the CSF well and this is causing the spikes and then lull's in pressre.  She joked that my body is allergic to these shunts which I think I might have to agree with.

Honestly I suck at waiting and so while I dont want the weekend to be done I do want it to be monday (either Dr.Bragg or Lisa will call and let me know if they came up with any ideas or what the plan is. I am at ERT infusions every mon but typically use that 5-6 hours to schedule/cancel/rearrange any appts I have or need to make, take care of endless paperwork, deal with any insurance stuff (thankfully I dont usually,typically have alot of this as my primary insur Case Manager is very good but I do end up with some things inevtiably to take care off. Compared to other days I usually have my phone nearby  so I sjouldnt miss any call from them  on what happens next and yet with me I seem to do a great job at missing call! :). I dont know actually what I hope for answers wise - I think it all freaks me out slightly (like ive said before I suck at uncertainty) but I also know Dr.Bragg is very good at what she does and I know she cares about my best outcome which is why she isnt rushing in to anything. She had asked me if I had ever been on anything before for the headaches and I have been on diamox pre-shunt although it worked some but not great. We didnt delve in to that further after talking about the affect it had on my labs (went a little crazy so former neurologist was having to adjust the dose some) but on the way home I was thinking about this all and wondered in my usual after appt insight (I so need to be one of those quick on my feet to come up with questions kind of  people!) if diamox could help now/buy time while we do try to figure out what to do. I left a message for Lisa (her nurse and whom is fantastic) asking her this very question and apologizing for not thinking of it then - I dont think Lisa minds my questions but still its not as if I am their only patient! **Add in: Lisa did call me back and talked to Dr.Bragg who didnt want to add in the diamox just because she'd really like to have almost a clean slate (so to speak) to work off of and make a plan with her team at mondays team meeting so it doesnt get messy/confusing on what is affecting what. I love that Dr.Bragg sort of thinks aloud and shares any and every possible idea and goes over each thing but then will admit it's alot to process and like I said im not that quick of a person to come up with immediate, un-planned questions!

 Also going to try sleeping upright in the recliner with a airline pillow (those u shaped pillows)  to see if it causes less neck pain (plates and screws so not as normal of movement)  and thus less headaches in morning because headaches for me are worst at night and in the morning.
If I think of anything else ive forgotten will add it later.
Say a prayer - may be a long weekend (though good things planned even if when the headaches are there and the resulting symptoms it really does stink). I really do think one has to try to make do with the good times though when possible and speaking off off to get some zofran!
Take care, God Bless,

Erica