Spinal Fluid leak cont's, F/up with Team. Speaking at SF, CA 26th

I've had a pretty big CSF leak now for about 2 or 3 wks after this last shunt revision though the leak is slowly decreasing in size but initially it was really good  which started about a wk after surgery which in and of itself was a little unusual (the late onsent of the leak) but I sure wasn't complaining! I really started to feel good and that is always such a huge blessing!!! I think the leak is slowly getting smaller which makes me sad (very sad!) but for now this extra fluid sure is a night and day difference in how I feel! I feel like a actual human being! For the most part I don't have to think much about things that usually these days make my brain feel all fuzzy and mornings are just like normal and I just don't generally feel as tired!! SOOOO nice!!! I know I say this every time I have a spinal fluid leak in my spine but I wish so bad this would last or there where a permanent way to recreate! I can wish, right!?

With the CSF Leak, the Shunts may be working but my body still wants to drain more CSF; and I guess will find any way it can which it did in this case about a wk after the last shunt revision as mentioned above which really the surgery  wasn't a major surgery (the lumbar shunt catheter was pulled down from thoracic (mid) spine to sit back in lumbar area. Perhaps b/c of all the scar tissue and because my body wants to drain excess CSF it doesn't care and will find a way when there's even a slight breach (of sorts) in the skin/dura (I think that's the area but I am not sure, where they go through for surgery) in spine that finds a way to create a temporary Cerebral Spinal Fluid (CSF) Leak.
I was talking to Dr.Bragg some about this all, asking her questions to better understand why the Spinal fluid leaks and External drains work so much better than do just the shunts alone and she explained it that with just the shunts they go directly in to the ventricle in the brain and can only drain what spinal fluid is in the ventricles (which in my case are very small so not a lot of CSF available to drain).
 With CSF leaks these are likely draining spinal fluid from around my brain/skull, my body finds alternate pathways to get fluid out and thus my to thick of a skull/not enough room in my head for my brain (essentially) causes headaches and symptoms except when the fluid finds a alternate way to drain! These in turn act a lot  like with the CSF (Spinal fluid) leaks and External drains. Weirdly cool! So wish there where a way to permanently recreate this! It does make me better understand why Dr.Bragg really advocated doing the skull-bone  thinning surgery (in this surgery they thin the bone of the skull so it can in theory expand with even slight changes in pressure like people without Hydrocephalus/Complex Hydrocephalus are able to do. That said there's no guarantee a surgery like this would work and has risks and my Nrsgn now is much more conservative so he wants more data first.

I met with  my Peds Neurosurgeon today to evaluate having the spine (low back/ lumbar) stitches removed (they left them in last wk b/c of the CSF leak, concern if the incision would break open under the pressure) and to discuss surgery/further plans.
As off now he is pleased (I think) surgery went well and we're looking at further options. The surgery did 75% help the radiating pain down my R side and that also went up my back. I get it some but no where near what it was and THAT is soooo nice!!!!
As well he is looking in to a device that's approved overseas but not yet approved here in the U.S so he is looking in to it for myself and a few other of his complex Pts to get a better (but blinded) idea of what my pressures are (ya, I know b/c we don't have a lot of data on this already).
I am not sure and I don't think there is but would be so great if there where a way to permanently recreate thee Spinal fluid leaks so the extra CSF to get out around the 2 shunts (which drain from ventricle in my brain to pleural (lung) space and from lumbar (low back) to peritoneal (abdomen essentially) to drain CSF (Spinal fluid).
If I understand It correctly with these CSF leaks the spinal fluid probably isn't even coming from my ventricles (b/c they are so small and there is only limited fluid that can drain) but instead the fluid is likely draining from around my skull/brain and I feel so good b/c this frees up space in my very tight, very narrowed space in my head and thus really good headache relief. B/c I have such a thickened and non-compliant skull ( can't adjust for even small changes in pressure like a normal person can even many people who are shunted) these CSF leaks offer  a alternate way for CSF (Spinal fluid) to leak out and create room in my head. It's AWESOME while they last!
I have to admit this extra room between skull and brain when the CSF leaks is a concept I didn't totally get up till this time and 1 of my very awesome drs took the time to really explain it to me.

Now if only there where a way, even if a unconventional way to recreate this! Alas we'll try to get the device I mentioned above approved and we'll go from there.

 I guess i'll really enjoy the pressure relief while it lasts and pray that by some miracle my Neurosurgeon would really get it this time that I'm not just crazy, that I'm not making this up that  even his own Team could see the difference not to mention 3 of my own Providers last wk made comments they could see a noticeable difference b/c the pressure was off. The same comments I hear everytime the shunts are either working really well or I have these CSF leaks.

Heart Rhythm Appt - Symptoms -
 I saw my Heart Rhythm dr last week and he and my Cardiologist agreed it's reasonable to start a trial of the med Amiodarone which is a Heart Rhythm med (can have plenty of side effects) so we are doing this with some monitoring. I see my Cardiologist anyways next months so we'll trial it and monitor as we go along. Initially I start at a higher dose and then after a week I back down to just once a day.
   
                           Speaking at this Pharma Oct 26th, San Fran, CA

Sangamo Biotech/Pharma (MPS I Research Co.)

I go next week to San Francisco based Sangamo Pharma (Biotech Company) to give a talk on living with MPS I. I am currently putting a powerpoint together for this. My intended topic is 'How did I get here and where do I go from here
 w/a focus on training more drs (especially Peds/Adult drs), arming Pts w/their care (where possible) and team effort (Patients, Providers, Pharma). I am hoping to finish my powerpoint tomorrow at ERT (Infusion) but we'll see!

Knee:
This R knee issue is still going on, maybe getting a little better? Sometimes I think so then other times doesn't seem much like it. It's been since around labor day I think (or when I first went out to CA for the initial enrollment portion for the Humira study). As  a side note they are holding my spot and I am planning to go out for the last part of enrollment the end of Nov., we're figuring dates out yet.
Thanks for stopping by, will try to update if nothing else after the SF trip mid-next wk.

Erica

#45 ? - LP Shunt revised; is it good? Hard to say

I got out of UW Fri - a relatively short stay is always nice albeit even nicer when your leaving and your head feels good. Mine does not and have been super tired (I did not sleep well while in-pt although thankfully I did have a recliner to sit/sleep in which helped a little).
Dr.Iskandar (Neurosurgeon) when he came by Fri asked why I was sleeping in the recliner and do I always do that (kind of goes to show how little what we say as Patients gets through to some drs, I don't think he does it on purpose but I've told him several times I sleep in my recliner at home b/c of the headaches and shunts drain better when not laying down). Anyways it seemed to really occur to him that it might be an issue for me (imagine that, I not be lying or something?) I doubt still it will change anything though, he just doesn't get it.

Symptoms since surgery have been really frustratingly not great. The headaches (pressure) is still there, vision issues with certain things still there - that improves as the day goes on though doesn't seem to entirely go away - I am hoping with a few more days maybe this will be better. It seems today as bad as this morning was (it's bad when I have to take triple dose of pain med for actual pain (wish it touched the headaches/pressure!) - I am hoping my Physical Therapist can work some of his magic on my shoulder/back stuff (surgery related, I'm not sure it's actually from the shunt but don't really know yet).
As the shunt seems to drain it helps the headache as is good but then I get this very intermittent, very sudden wave of lightheaded feeling - super odd and hopefully goes away to! It literally makes me feel like I am sitting on shifty sand vs on a solid surface (lol, not sure if that makes sense) and then my head just feels buzzy/off for a bit till it settles again after awhile, as if the pressure drains and then adjusts. VERY, very weird!
 It stinks though how with certain shunt placements you feel fantastic from day 1, particularly when brand new systems are placed (not that I want a whole big surgery its just that its a common sentiment I hear especially in us MPS Pts. we often feel so much better with new shunt systems then we do when parts of shunt are replaced). Just another odd thing some of us have I guess.

  Low-to-mid back pain is pretty intense though moving the catheter atleast so far has seemed to help the pain related to that down my R side  some. I truly think and probably am always going to think that the bigger issue is with the valve and reservoir placement on my back and frankly will probably always be mad at myself for backing down about this last Jan (I think that's when it was). I don't blame anyone else but I should not have let myself be talked in to this. I knew and know my body but sometimes you just feel so frustrated and worn down and that is what I felt that morning in pre-op after Dr.Iskandar and I had already agreed to move these back.
I really don't care what anyone says I know my body and for that matter there must be a reason none of the Neurosurgeons before him placed these devices like this. Frustrating to go from someone you felt like listened to someone that thinks they are listening but they really aren't.

As far as surgery they did Neuro monitoring during (someone from that Team had stopped by in pre-op Tues morning to introduce themselves and say what they would be doing). B/c of TIA (mini stroke) a few weeks ago and a heart med I take for heart muscle function (Lisinopril) they  also placed a Arterial line (An A-line gives more direct, better measurements of pressures between heart and body I guess)  which I really don't remember. Biggest thing is everything seems to have went smooth during surgery. And even if I don't feel great yet I am glad for that (it having went smooth)! I know there was a fair amount of concern in pre-op that morning between the Anesthesia doc and my Neurosurgeon although I did not feel the same concern they did.

Surgery wise Dr.Iskandar said he didn't have to do as much as he thought he might as the LP Shunt was draining and contrary to what he had thought the catheter actually was already started in (led in) to my Lumbar Spine (low back) so he just had to pull it down some from thoracic spine. He wasn't or wouldn't have been able to do anything with low back scarring otherwise which is a bummer (that it's gotten this bad) but I am glad atleast the catheter could be adjusted.  He had thought the catheter started in my thoracic spine though that to because of the incisions and work he did last Fall I was almost certain it started in lumbar (low back) area and just tunneled up to thoracic spine.

Headaches have sucked actually since surgery, they where bad enough before the surgery but now are just plain crappy. I JUST WANT TO FEEL NORMAL!!!! And to not wake up everyday and it take hours and hours to feel more normal. Like I said above today seems to be 1 of the best days so far so hopefully maybe will get better. I am sure hoping!
I when talking to Dr.Iskandar on the day of discharge once again explained why I need his help and no I don't actually want to be there repeatedly asking him to believe me and that I get it I look normal to him but he sees me for 5-10mins every so often while I have to live in my body every single day and try to function. I told him how I really didn't think it was the LP Shunt that was the cause of my headaches to begin with but was glad he atleast tried adjusting the positioning, see if that would help the leg stuff.
For whatever reason this morning and I can't really explain it but sincerely hope it's just a passing thing but my L arm is sort of numb (not like numb in I can't use it but just a painful numb) - seems to go along with as the shunt is draining. It is so freaking frustrating that these shunts can't just be good and not painful and I can't just feel good. =/ - Thankfully finishing this up later on that has passed, whether it was the extra pain meds or who knows what I'm not sure but hopefully stays gone!

I  realize I sound pesimistic or something but I hope Dr.Iskandar really takes to mind what he and I talked about and does have the conference call he talked about with Dr.Bragg and Dr.Guillaume to try and come up with an alternative idea for treatment/management.  Do I think something will change or he'd actually do something with either A. the LP Shunt valve reservoir location (these sit on my very low back, whereas they used to sit on my side connected to the LP Shunt) or the VPL Shunt without another long drawn out fight? Probably not. He just doesn't get it and doesn't get that for someone like my case, some of us with MPS we present very differently and he doesn't see it b/c again he sees me for like 5-10mins at a time. I wish it didn't have to be this frustrating though! No one should have to fight to feel better and to be believed.

Will try to update soon. Please, please say a prayer things improve each day and I can start to feel like a more human and my Neurosurgeon really consider that I'm not just making this crap up!
Thank you,

Erica