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
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
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