Thursday, January 31, 2013

Potential change in surgery plan...

Dr.Bragg stopped by earier tonight and surgery is scheduled at 10-to-3 (2:30) until approx 5:00 although that isnt to say the surgery will or wont take that long. She actually had a differnet thought after talking to her partner (see below) so we may end up doing a short procedure or the longer revision depending on what we decide in the morning.
She talked about getting the cardiac anesthesiologists for the surgery (vs neurosurgery anesthesiologists as then less likely to place the lengthy arterial line and better understanding of how my cardiac issues affect the surgery. She mentioned how she can  now tell the Anesthesiologists what they need to use if they have questions and is able to describe the aitway structure   + the  intruments they need to use and describe the airway issues,

She also talked about how we would be having to do cardiac surgery in the future and she really wants to get this shunt stuff figured out  in the mean time + she didnt want to do anything to cause me problems  but be able to try and figure out how to help me without causing adverse issues.

Her partner is recommending to Dr.Bragg that we do ICP monitoring but blind the results to either Dr.Bragg or myself so Dr.Bragg could get an idea if my headaches where sporadic as far as ICP (intracranial pressure) levels or pretty consistent at one general #when I am having the headaches? Either way I guess she would then take this information after a couple days of monitoring and either find a better valve for the shunts or consult w the Neurologists in her dept and see if they had any med suggesitons. Ive talked to (via text)  my Pain Mngmt dr tonight after talking to Dr.Bragg and she is happy to talk to Dr.Bragg and share the numerous meds we've tried + what we've done so we dont re-create that wheel. It seems she may be willing to try what we originally talked about w the other valves first  but is going to stop by in the morning after her first surgery so we can talk more and go over any questions she thought up and any questions I wrote down + make our decision. I did send her an article I found on 'negative pressure hydrocephalus' which seems like it may be our issue as it talks about revising shunts, they fail, revising again sometimes even w new mechanics and fail yet again repeatedly. From what I understand in negative pressure Hydro the key is to drain below atmospheric (negative) pressure amd certain positioning.

I feel conflicted, I want to figure this shunt stuff out and on the other hand I just want to be done and go home already! Dr.Bragg talked about how they have a few other pts w similar shunt failure issues and symptoms and are constantly trying to fgure these pts out as well but so far didnt have a great answer how to help.
I sort of feel like we should stick w the original plan and try replacing the TPL valve and tapping the VP shunt to see if its still flowing or do we need to replace that valve to and see if replacing the valve w a programmable type might help since we've done this much already. On the other hand maybe we should dothe ICP monitor and get a good idea. I am tending towards leaning to trying the valves though first and will see what Dr.Bragg thinks in the am.

Stay tuned,


Revision Postponed, tentative plan...

Surgery was post-poned till Friday as Dr.Bragg had a case come in Weds night she didnt feel comfortable waiting till Friday to do so we essentially swapped. As far as what she will likely do she mentioned taking out the PS Medical valve in my TPL shunt and putting in a Medtronic Strata programmable valve again so we can re-program the valve and play around w the pressures/resistance as before we had to take everything out. I honestly dont remember if I asked her if we would leave the catheter tip to cont to drain in to the pleural cavity or not but she didnt say otherwise that I can remember from our talking this morning.

She also plans to tap the VP shunt in surgery and if this isnt working said she would swap out the mini-nav (flow control) device (similar to a valve) for a Codman programmable valve for more options in drainage.    I am unsure if she would/will leave the second (Miethke) 'shunt assistant' valve in or no.

If nothing else amazingly in this all my GI system has been pretty calm and no constipation which is nice and would be very nice if it cont's! For whatever reason my body just does not seem to like to absorb the CSF well or adapt to that drainage (or something) as ive never understood the connection w constipation (TMI, I know) other than pressure puts un-do well pressure on your brain signals maybe? Doesnt explain why it would be 'calm' now then though..

I, of course missed the 'Pain and Opioids' meeting Tues night in Milw but my Pain Mngmt dr texted me that evening later on to let me know it went well and they missed my voice to balance the 'conversation' . Today she sent me a couple more messages and said they are looking at hosting more of these as the turn-out was over 200 last night (wow!) + successful and alot of interest.  On the panel I would have sat on where an Addictionologist (specializing in people w addictions to drugs) as well as a few others including reporterdly another provider who was very fair and balanced in supporting opioid use in a well thought out combination approach.  I do hope they plan more of these meetings as I truly want to be involved and bring a legitimate voice to the table that "Yes, these medications actually Do help patients and most of us are following our providers instructions, living a "normal" life (whatever that normal IS!) and not selling our medications, diverting our meds or abusing our meds!

Hoping we are coming to the close end with this series of surgeries, who knows but maybe!?
Thanks for stopping by,

Wednesday, January 30, 2013

Revision # 24... It's Groundhog day...Again...

We are doing a 3rd surgery (in just the last week) tomorrow (Weds)... This will be the 7th surgery since mid-Nov. Yikes!

Today Dr.Bragg tapped the VPL shunt and the VP shunt and with the VP shunt a blood clot came out w a bit of CSF fluid making her think this was left over from Fri nights surgery in which they encountered a blood clot I think she said when either removing or revising the VP shunt. She had thought this would help the headaches and get the VP shunt flowing better. I do think the VP shunt is working but she thought about re-tapping it tomorrow morning, I am unsure if she will.

The TPL shunt was basically not flowing and so she opted to schedule surgery for that for Weds and will either place back in a Strata-medtronic programmable valve or put the drainage back in to the peritoneum. I asked her to consider placing it back in to the pleural cavity as I think that worked so well at first and the stomach cramps are soooooo much less as in now occuring maybe once or twice in the past month and nothing like they used to be vs they where occuring at times almost every night.. I truly, personally think the VP shunt failed bc we didnt have an optimal placement and then the TPL shunt failed bc the VP shunt provides the majority of CSF 'support' and helps keep the TPL shunt catheter from over-loading in a sense and thus occluding. I cant help but think that if both shunts are working in their respective locations then we will be in a better, longer term situation... I know constipation IS sooo much less of an issue and seems like autonomic issues calm down incredibly well when we have both working well. Even now w just the VP shunt working w the 2nd valve support it seems like the constipation is improved. Ahhh I hope this works and LASTS!!! I would like nothing more than to be out of Dr.Bragg's hair for a while; she is fantastic but I still hate having to ask her for help so often, literally so very often! Cross your fingers, say a prayer, whatever it is you believe in!

Surgery will be in the afternoon as she will stop by in the early morning and then has an outside clinic till late morning. I imagine surgery will be middle or late afternoon. I know my parents are worried about the weather so I am hoping this time the snow will stay away. It has snowed I believe everyone of the last 3 or 4 surgeries making it crap for my parents as they live about an hour away. The one night 4 surgeries ago I believe it was they came in to post-op when I was still asleep to talk to Dr.Bragg and say 'bye' so they could get home before the weather got worse - that time I think they said it took them 2 1/2 hrs to drive an otherwise 45-min or so drive home.

I will post sometime after the surgery unless there is something to update about before hand.

Thanks for stopping by,


Sunday, January 27, 2013

Those 'second guessing voices' inside my head...

I have a really hard time with really wanting to feel better and wanting to be able to tell family (especially) that for instance a shunt surgery hasnt necessarily worked as well as we might have hoped.. I have an incredibly hard time when say my parents call in the morning or my sister calls and I can tell they are truly hoping "this will have been the one" and I just want to be done and one to work longer term and not good at really expressing my feelings as evidenced I guess if even Dr.Bragg makes comments to my parents how I keep things inside.

How do you do this 'one day at a time' thing when everyone around you is thinking '3 months ahead' as far as something working or not? I know my family gets this stuff as much as anyone can if your not the one sorting it out but it's so incredibly hard to be the patient and to watch their feelings and know it's hard on them but to feel a little like I am causing heartache. I know it all bothers my Mom alot and that on fri she was apparently really upset during the surgery and crying when Dr.Bragg came out probably bc it was somewhat un-expected surgery and kind of last minute and she probably wishes she could fix it. I wish I could fix it and I wish I knew better sometimes how to relate to howmy parents feel about all of this - my Mom and I are close but I dont think we've ever really been the type to talk a whole lot about these kinds of things untill we get pissed about something anyways. :)

I am glad I dont need to rely on other people to deal with this stuff and I can just compartmentalize when it gets to be a bit much at times and I can research my questions  to get more information +  learn about what my docs are thinking of wanting to do. I am glad I can get along w the nurses well and the residents for the mot part as I learn from them to. On the other hand I wouldnt want to give up my independent "i'll figure it out first for myself  and tell you when im ready" personality but I do kind of wish I could do a better job of sharing my feelings, asking for help sometimes (vs keeping things to myself or stressing about asking for help) - I have an entirely hard time picking up the phone and asking providers for help and hate relying on my parents for things like post-surgeries. Not bc I dont love them but bc I wish I could just make them my family and not so much like they have to care for me. Yah, I know its what parents do but it doesmt mean I like having to have help from others any better! I could be better  at understanding other peoples need to ask questions or work through their feelings (my Dad) in seeming more open ways as I dont always want to talk about how I feel. I guess all of us could be better in one way or another and these are just things I cant help but think about sometimes. I doubt i'll ever really like telling another person what I feel (vs an impersonal "this is what ive experienced and what has helped me") and will probably always be a loner in the sense id rather go to my Apt at night then out with friends or pick up my nephew vs something wild and crazy. I share alot about myself  here but dont always feel that comfortable sharing some of it and  then on the other hand feel like I wished there was someone who could have told me some of the quesitons I wondered pre-and-post diagnosis (MPS). I sometimes think if my writing this can help others both w MPS and w hydro (which it seems to from the emails and messages I get) then I can write it and sometimes share more. Writing is always mroe impersonal thankfully anyways!

As far as the shunts Im not sure what the next step is ie if Dr.Bragg will discharge me and we'll wait and see or what but i'll update probably monday if there is anything great to wite about otherwise again soon.
And if you are her great partner then when I asked hm today if he had any 'magic' tricks to flash (in front of my nurse) he (I guess) jokingly but I also think rather stuck up like told me: "I did my magic trick and gave you to Dr.Bragg" - I had afew comments to that on the tip of my tongue but didnt say them and thought to myself "You didnt pass me to anyone, you told me I was making up my symptoms and essentially wrote as much in his clinic note + then it was through my reaching out to someone I knew personall at UW I found the Adult Neurosurgeon willing to take my case + he talked to Dr.Bragg and really recommended her. I dont often admit it but his comments kind of bothered me; I am bad at second guessing myself sometimes and despite things she JUST SAID TO ME SAT couldnt help but think "does she ever regret taking me as a patient of hers?" I hope not and am guessing maybe not by all the things she does but dont  know..

Thanks for stopping by the rare and true confessionals of my inner feelings,

Thanks for stopping by,


Saturday, January 26, 2013

Revision #23 VP shunt, day one post-op

Yesterday Dr.Bragg did the shunt tap and opted to just tap the VP shunt for now which did show the shunt had re-ccluded within a couple days (tues to probably thurs) which those who know much about shunts know this actually isnt all that uncommon and occurs quite frequently especially for some people.  The failure rate in the first 6 months is 60% and especially high rate of failure in the first days or weeks.

She opted to book an OR last night after she finished another un-planned surgery in another of her pts who had surgery earlier in the week presumably (but I dont know) for a shunt to.  I had a different, new Anesthesiology team last night and they opted to not do a arterial 'A' line  to monitor cardiac issues as the dr was specifically a Cardiology Anesthesiologist and felt we would be fine with just careful BP monitoring and less invasive. Nice and score - take the blessed small things with the unexpected bad occurences in this case being the surgery.  As far as the revision Dr.Bragg wasnt sure up till we where in the OR if she would leave the 2nd (new) catheter in place or remove it as we didnt really know if it was helping or hurting given so quick an occlusion. She ended up leaving it in place.
She moved the catheter from the back of my head (2nd ventricle) and created a new tunnel (the former frontal shunt tunnel from Dec had closed off already; likely secondary to the MPS I think Dr.Bragg said as that shouldnt occur typically so quickly; almost makes yah understand why the shunts are so hard to figure out if that same storage is occuring in the CSF and shunts as the shunt catheters are smaller than a straw in width and already as prevously written very prone to malfunctions.

During the surgery the catheter and former valve where removed and catheter moved + then placed  in the new shunt tract line and re-connected to the new (but same style) aesculap mini-nav in-line valve. She had to use 2 different pieces of equipment to get the catheter located well and straight within the ventricle as the initial photos where grainy and they had trouble getting a clear view of where she was trying to place but also make sure the catheter wasnt occluded.  She ran in to some troublesome looking CSF as she placed the catheter at the top of my head (so they opted to do additional antibiotics) and enlarge the incision they where going within as she didnt think it would close or heal well if they left the catheter underneath it and stapled. Apparently the incision is now about double the size but hopefully still coverable by hair. (so vain I know)

I cant think of what I might be forgetting but we just wait and see now if the shunt will stay working; hope so! It seems like even though it was short lived at just a few weeks (at most) the shunt in Dec was brilliant in how I felt compared to this! Part of it is the 2nd valve at my collarbone is sore and these numerous surgeries take a toll and in reality the first couple days are the hardest. One day at a time..

Apparently Dr.Bragg in talking to my parents last night said to them something about how I hold alot inside and she thinks there's alot going on I dont talk about (or something like that.) Sweet that she cares and although I dont want her to she takes my ups and downs so personally. She wouldnt go out of her way like she has if she didnt care.  She wanted them/I to know we can always talk to her (I know and apprecate this I just am notoriously quiet when it comes to wanting to ask for help or really talking about how I feel physically much less emotionally as I dont want to whine and already have to ask for so much help from people like Dr.Bragg. Id rather not complain until something is really bad and I need to ask for help which maybe isnt always the best for my providers trying to figure me out.

Stay tuned, we'll figure this stuff out and hopefully soon!


Friday, January 25, 2013

Day 2: Floating ideas, brainstorming.

Let me start this blog by saying I am not absolutely clear I got all of this info sorted correctly but am writing this the best I understood what Dr.Bragg, Emily (Neuro-NP) and I talked about. Alot of info and I had also been reading several longer articles trying to learn more about valves.

We definitely talked about many things today as we usually do when dealing with these shunt issues, she is amazing. I dont know how she doesnt get burned out having to deal with the same issues over and over and doing challenging work and then having to basically fix it again and again (through no fault of hers). I cant imagine how challenging it must be to try and figure out a disorder like MPS and how it affects the mechanics of the spine and brain. Then do it day after day with many disorders- simply amazing! I guess it is why she and others are good at what they do.

One of many things she talkd about after I asked her why she thought we would have had basically good luck w the VP shunt and LP shunt keeping each other from failing after we re-replaced the LP shunt last spring while the VP shunt was still in place and despite not having perfect results (but we where dealing w the shunt infections so it  makes sense) but now having moved the LP shunt to a TPL (thoracic pleural) shunt and having had the VP shunt occlude once already in a couple week span why did she think we where having these issues again now w the new shunt locations? (after both shunts seeming to have worked great for the first week or couple weeks which isnt a long span but it was still the best I had felt in a very long time other than the external drains) and did she think there was a reason with doing this revision on Tues it would have possibly already re-occluded? - (why are we having issues w these new shunt locations essentially when shunts before in LP and VP seemed to atleast support each other from so frequent occlusions?) -   She thought that narrowing in my thoracic spine due to the mucopolysaccharidoses and less CSF in the particular area of the shunt catheter (more space above/below it) and pressures in the chest if I understood Dr.Bragg correctly are lower than pressures in the stomach so this could affect how the Thoracic-Pleural shunt seemed to be working but not working well last week when we tapped it. (is why we also did the chest Xray)  There is less available CSF fluid to drain or pull from? (this is a part I am unsure if I got totally correct). On the other hand pressures in the stomach are higher than pressures in the chest and that could be posing a absorption problem or problems with both of the shunts draining but in different ways or amounts?  I asked Dr.Bragg today if she thought the cont'd non-resolution of the headaches with this revisions could be related to a problem with CSF not 'liking' draining in to the peritoneum (area of the stomach) despite our having one of the shunts draining elsewhere since Dec (before this revision) and she did say they had been talking about/thinking about this as well and was something she was considering depending if we do end up doing the shunt taps in the morning and depending on those results. I dont know if that is the issue or part of the issue where she would consider moving the VP shunt tip as the heart is out and I am unsure if 2 shunts can drain in to a lung space or would the other shunt be drained to the other lung space? If we're lucky I wont have to find out that answer. =? At the same time will worry about it when/if we have to cross that bridge. No sense spending to much time mullting what I cant change one way or another right now.

She mentioned she would like to if I was okay with it re-tap both shunts in the morning because of last weeks  not great TPL shunt tap result (amount) and the collapsed ventricle where the VP shunt is + the catheter tip not being in best ventricle now.  I am fine with if we need to since it will help us get answers one way or another about these issues. (ie is the TPL shunt working and has the VP shunt re-occluded already or is one working or partially working and/or should we move the end of the VP shunt from the stomach.) As usual I hope we dont have to and these shunts would just work but is as always just one day at a time....

A few other things she talked about was should we take out this new 2nd valve as it could be ( causing not enough CSF to drain (which we knew all along could happen) when I am up-right now as the '0' setting when I lay down is pretty much what we need. I dont lay down much bc of the heart/breathing) but it still had made a difference before the shunt occluded and stopped working early last week and after the shunts where replaced in Dec. At that point if I did wake up w a H/A it pretty much went away w/in 10-15mins and was soooo mild so a matter of finding a middle point of little or no headache but keeping the ventricle open and the shunt un-occluded it seems. On the other hand the pressure setting when I am up-right that ICP (intracranial pressure/pressure inside the skull) has to hit is pretty high (for me) at 15  and may be to high before it will drain any CSF so thus could be why I am having the headaches still, I am unsure why the ventricles would have collapsed though. It is all a lot to learn and always something new and this new set up is even more complicated seeming atleast to me (in how it is supposed to work).  Dr.Bragg always explains it well and uses these funny analogies to explain all this shunt and drainage/headache stuff in easy to understand terms but I dont always remember it anywhere near as well as she tells it. =)

At the very least atleast I am not throwing up and just headaches, some nausea and vision affected - the throwing up is the roughest part so is nice to have a reprieve from it as that is typically the hardest to control. The rest is no fun but atleast made somewhat better typically by being upright.

I'll update after I talk to Dr.Bragg tomorrow if there is something to update on ie if we do tap the shunts or if feeling better or whatever.
Thanks for stopping by,


Wednesday, January 23, 2013

Day 1 Post-Op , Revision 22

For all my fellow fb'ers you've maybe see this already (not alot of new, news so just re-posting what I wrote there) and for those not on fb this will be new to you! =)
Thanks for stopping by,
Just spoke to Dr.Bragg and the tip of the catheter is actually not in the ventricle they wanted (2nd instead of 3rd) despite using the
'GPS' guidance system but her partner thinks it is ok so we watch
and see for a few days. There are 4 ventricles and from what I understand 2 are more often used due to location but doesnt mean it's an automatic out.

I am so tired from not sleeping overnight I di...dnt think to even ask many questions other than this catheter placemennt she mentioned w/ the revision. Sad that it already seems like eons ago and was just yesterday. ... We wait and watch for next couple days.. CT scan this a.m showed ventricle had collapsed
whereas it was enlarged before surgery (due to failure) so a sort of no win scenario but it may correct itself when pressure builds up from inside outward causing ventricle walls to re-open but hopefully not occlude. (Thinkn of a straw in jello I guess vs a straw in water one is much easier to suck through/drain through).

I know many of you wonder why "we cant get it right" but its not a simple answer - MPS causes the brain to be less compliant, the
ventricles to be stiffer and less able to respond to pressure changes (thus partial cause of occlusions) and shunts in general in a non-MPS, more typical Hydrocephalus pt have a 60% failure rate regardless of surgeon in the first few months which tells you something... I hate the surgeries to but admittedly the second guessing is kind of hard bc I know its so much more complicated than I could ever all share here and especially for non-MPS people it's just not straight fwd. And even most MPS kids (I dont actually know to many adults w MPS w shunts likely bc there are so few of us) dont seem to have anywhere near the issues ive had or seeming complicating picture. I truly appreciate advice and support but is hard when people suggest the Neurosurgeons are doing something wrong as they really arent and are working w their medical engineers and Neurologists ("mathmeticians") to figure these issues out and truly thinking outside the box as a team to find solution one step at a time.

MPS is a complicated disorder and Hydro/shunts are a far from perfect science so taken together it can be very discouraging seeming but you have to pur your faith in the one person who knows your outcome "God) and your trust in the specialists you entrust your care to be able to go fwd as simply as one step at a time some days and one day at a time many weeks.


I have to re-post sometihng my sister-in-law wrote on my fb wall - I am lucky to have such great sisters:

Please keep my dear sister-in-law Erica Thiel in your thoughts and prayers today. Battling a rare disease, she just had a 5th surgery in the last month. She is such a beautiful person inside and out. Always thinking about everyone but herself, Today is a day to think about her

One, 2.. Skip a few..22 (shunt revisions)... VP shunt revised (Yes, Again)

Really only 21 though, just wanted a rhymy sounding cheer!
Today's surgery took about 2 hours. - My pre-op facebook rallying cheer was the title of this update to make me smile/be silly as I had silly, funny nurses. =)

Revised at collarbone (not flowing) and revised at proximal (tip) to re-position in the ventricles w the GPS/endoscopes due to that also not flowing once they opened up the collarbone incision. They did end up placing a mini-nav '0' one way flow (non)-valve back in this and then at the collabone when Dr.Bragg came in this morning to pre-op she and her NP said they had been talking to their medical engineers and Neurologist who helps w their very complex shunt patients and came up w a completely. new plan. She took a valve tehy had also by Miethke (H/V Shunt Assistant) which has settings of "0" in the Horizontal (laying down-flows more" position and setting of 15cm in the vertical position (standing up, gravity) to overcome the issue we have been having of over-drainage/ventricle collapsing but needing to drain alot of fluid to be at the '0' brain pressure level. (Zero CSF which is what bathes the brain and in some case such as mine the body isnt able to absorb properly and become sick).    

We dont know if this will work and arent out of options if it doesnt and Dr.Bragg still feels confident we will find the right answer + as well she is surrounded by some very amazing talent amongst her own to. As I told her and have written her before I am just really grateful she is so persistant and wonderful!.   We both thought when she got the incision open that maybe the ventricle would be open and the shunt working ( not occluded) - it does this intermittent occlude/collapse/forced open (like trying to suck fluid through an straw to fast almost the walls collapse in) thing at times and thought maybe that was what was going on but then she instead she found both the sections she opened where not working well so we ended up fixing both parts. Overall the surgery took about 2 hours w a mix of all the equipment used (the GPS like guidance system being placed, then removed to insert the endocscope whch was then removed to re0insert the GPS like system.

Anesthesia had gotten a copy of my Cardiologists notes and because the aortic and mitral stenosis is so bad (severe range) they changed up how they administered anesthesia throughout the case, how they put me to sleep (but used same airway qeuipment due to airway) and ahd of even sedating me opted to put in an aterial line as she wanted to be able to follow every heart rhythm and blood pressure moment to moment vs waiting for the machine to go off every couple minutes and concern what if the machine paused or had a malfunction tehn having to miss multiple minutes or more of rate and blood pressure potentially missing any issue out to organs not getting perfused enough. Her biggest concer (there where 2 anesthestiolosit and the Resident Anesthesiologist) was 1. already very low BP,  2. organs not getting oxygenated blood, and 3. stress on the body from the cortisol deficiency and giving this as a additional replacement if needed during the case on top of the dose an hour ahead of time.  The arterial line was interesting but didnt hurt as bad as I thought it might when being placed and they quickly albeit slowly (think starburst contradiction, ha!) in the process,

Dr.Bragg must have asked my Dad about where I was going to have the valve replacements done (he doesnt know bc I dont know bc my Cardiologist and I are mulling that over tilll we have to decide)  and commented I guess to him that we may have to put in external drains (??) during the valve replacement surgery which if true (will tyr to rememebr to ask her tomorrow) would mean needing someone she could recommend wherever we do this OR could I fly her in privately (wouldnt I wish!) as I dont think any replacements would be done at her hospital; that hasnt been mentioned at all.  I am very curious about this and if true can only figure maybe it has to do w the one shunt drainning in to/around the lung space and that affecting surgery outcome?

I am purely exhausted so will try to update anything else or anything I am forgetting romorrow.
God bless and thank you for stopping by,


Sunday, January 20, 2013

Surgery (shunt) revision #21 (or something)

Most of the time I do find it really cathartic (calming) to write here not to mention it is a really good way to be able to go back and look at what has happened in the past and look at a detail for information. The last few days (since thurs) the most I could do was open this page and leave it open I just dont feel like writing about this week and especially the last few days; I dont know where to begin I guess? I am tired and although definitely not depressed bc its not worth being depressed about and ive had other things going on I just feel alittle (or alot) like this is the movie groundhog day replaying over and over and over...   Last week it started (teh worst of it anyways) with mondays waking up in the middle of the night throwing up left, right and center, and nausea and headaches that where just out of this world (pressure)  to the point even the ativan I took wouldnt calm the headaches down. It was bad so very bad but just didnt want to go to the ER and thus back to the hospital and actually thought it would get better. Tuesday by early afternoon I was feeling somewhat better, atleast after multiple doses of the ativan I wasnt throwing up. Weds early morning not long after going to bed (so really Tues late night) I woke up w the same thing again throwing up despite various nausea meds, ativan dose, etc. AWEFUL! Probably should have given in then but didnt and finally after hours of that and being sick most of Weds to got a little better. I finally called Dr.Bragg's ofc Weds afternoon  and got an appt for thurs morning.

She (Dr.Brragg) did shunt taps on both the Thoracic-Pleural shunt and the VP shunt and the TPL shunt didnt have much flow I guess but had a little so she believes that is working (a chest xray was clear and fine) and the VP tap did not bring any CSF flow so she suspected right away thats where the problem was. She took out the rest of the dissolvable stitches and ordered a 'quick brain' MRI after which I went back up to the clinic and she reviewed plus came back in to see me, talk about surgery which we knew we where going to have to do just werent sure if we where going to do on Fri or wait till Tues (we are waiting till Tues). The MRI actually showed the ventricles had enlarged some from the last imaging done a few weeks ago while in-patient then which NEVER happens w shunt failures so that was about as clear an answer as we could have asked for on what was going on not that we didnt already know.  After those results and talking to Dr.Bragg she sent me back down for a CT scan to help her w /her planning for the GPS guidance/endoscope system they use in surgery and I was finally able to go home.  Exhausting!

On the way home as if that all wasnt enough I rear ended (of all things) an ambulance (no patients in it, was just stopping in rush hour traffic) downtown Madison - talk about CRAPPY day!

I now have the lovely distinction of  'having the most shunt issues by far of any of their patients and absolutely the worst luck" according to Dr.Bragg's nurse - =/ (she wasnt saying it to be mean, more she couldnt/cant believe the crap luck we have w these shunts and how often the failures/revisions are occuring). - They think it has alot to do with the underlying MPS which I would agree with.

Dr.Bragg thought she would likely try the same aesculap mini-nav 'non-valve' one-way flow control (to keep CSF from backing up from the peritoneum) on the shunt but at the next highest setting ie instead of right now we have a '0' so now pressure she is going to try the next step up so there is a little bit of a pressure control to try and keep the ventricles open as she thinks they collapsed and thus caused an occlusion in the catheter (or something like that) due to too much CSF flow but at the same time that very low intercranial pressure/high flow is exactly what I feel greatest at so we have a balance to figure out. I also personally dont think my stomach is loving the CSF flow in to the peritoneum although I dont think that is affecting this particular shunt issue.

Otherwise the 'pre-op' done when back in-patient when I was re-admitte Christmas Eve via ambulance from the local hospital was able to be used for this surgery (that was a small piece of good news!) so we didnt have to worry about getting that in before this surgery since there is s few days and monday is infusion day + a holiday for most clinics.  Labs where done at AFCH's clinic.
Cardiology due to their concerns w the heart and valve changes faxed over their most recent note to Dr.Bragg's office on Friday.

Infectious Disease NP who followed me throughout the Dec stay/4 surgeries called me on Fri but I missed her call and the VM didnt come through till later in the day but she said in her message she would call me on monday with whatever recommendations she has and has already talked to Dr.Bragg/her team.

On a different note that I am looking fwd to as it is something those who know me know I am passionate about my Pain Mngmt dr called me 2 weeks ago and then sent me a message when I missed both her calls about a meeting she is co-hosting for medical professionals (of which I am not) on Pain Management, opioid prescribing, drug diversion and protecting the prescribers practice. The meeting will feature speakers from both Pain Management backgrounds, Drug Enforcement Agent (DEA), and Pharma and she asked if I would attend this and during the discussion phase participate and represent the patient population (why these meds works/what benefit they have/how it has made me able to function despite an all-encompassing multi-systemic disorder in which ive had literally dozens of surgeries and I imagine the things ive done to protect myself ..(secure lock box anyone), dont leave meds un-attended (keep them on you at others houses/parties, make sure other providers know what you are taking and be responsible). This meeting is I think I may have previously mentioned the same night as our sunday school monthly meeting (which I actually  look fwd to) which is a bummer and I am above all praying this surgery Tues just goes SMOOTH so I am OUT! My Pain Mngmt dr messages me the other day once I let her know of the shunt issues and Dr.Bragg appt and said the same thing "she really hoped it went well and really wanted me to be there at the meeting". I love and appreciate that she trusts in me of all her patients for a meeting like this and that she is so amazing at sharing all she knows so I in turn have learned so much about the Pain world and thus how I in the first place got involved initially with APF (Pain Found) bc I found it interesting and wanted to help other pts learn.
Will update if anything new before or otherwise after Tues.,
Thanks for stopping by,


Thursday, January 10, 2013

Infectious Disease, Cardiology-valve changes, Incisions..

It's been a somewhat busy few days with being back at my Apt and back to normal routine. Last Friday I saw Infectious disease and Beth asked that I call her with  any new UTI symptoms or any new symptoms of headaches ie if I am seeing Dr.Bragg bc of symptoms she wants to know.
She was sending her note to my PCP and to my Neurosurgeon and going to flag my file so an antibiotic will/should be automatically given that is susceptible to the particular UTI bug I keep getting anytime surgery is done either at UW or elsewhere. Otherwise antibiotics are always given for the actual surgeries but a different class and the particular ones given are to prevent different issues. This particular UTI bug is susceptible to only one class of drugs and resistant to the rest of the drugs they've tested so we hope it works and at some point may have to switch to treating the UTIs via IV which is the only other way the a susceptible antibiotic works other than the oral we have currently been using for the past few times. The bug that was in hte CNS isnt as antibiotic resistant but a common bug we all carry on our skin.

She also said she would recommend in her note and to Dr.Bragg to tap the shunt and test CSF sooner if having symptoms. If I understood her right she suspects apparently that this bug could come back due to its long(er) standing nature in my CSF this time but to try and avoid this whole mess she would recommend tapping sooner to catch any potential issue sooner. For now the blood level tests she ran where perfect and CSF was testing completely negative so I personally am not worried.

The other thing she added was that she would like  to stay involved for all future shunt issues (if sny) and be consulted on in-patient Neurosurgeries. It really wasnt to big of an appt.

I Saw Cardiology today (Tues) and a little surreal actually.. I went in not thinking to much of the appt,  just another 6 months gone by, another Echo and another Fup appt and in fact in my last blog I think  I commented I actually thought it would go well..  Apparently not, which is ironic bc on the new beta blocker dose and increased lasix dose over the past few months ive actually felt better breathing wise than I had in years.  
Apparently the pressure gradients (blood flow from the valves?) is increased again and now in the severe range for the aortic stenosis (narrowing, meaning not enough oxygenated blood gets through I think) and the I think but cannot remember for sure that he said the aortic leaking is in the moderate range (an issue he is less worried about) and the mitral stenosis is squarely in the moderate range now w leaking also moderate but that I also cannot remember. He was genuinely concerned we will be facing valve replacements soon maybe as soon as this next eval in 6 months as he said he may have recommended it sooner but with the recent CNS infection the soonest he would send me to a Cardiothoracic surgeon was once I was infection free for 6 months due to the CNS shunt infection as he wants any systemic infections cleared for atleast 6 months unless more symptoms present and then he would still want 3 clear months. The heart muscle itself is handling this valve changes pretty well he said; I think before the L side was severely enlarged but as a whole the heart is holding up well.  He said that with any shunt surgeries or other surgeries fluid would have to be managed carefully and blood pressure watched carefully (due to being so low already) and with any slight change in symptoms he wants me back in to see him  right away as he seems to think this will change again quickly symptom wise (I guess).
He was concerned about the shunt infections I just got over but didnt think those where the cause of these cardiac changes (stress on the body I suppose) and more just making sure any infection like that even though neither of the shunts go in to the heart would spread to the heart (less likely as 2 different systems ie blood stream and cerebral spinal fluid but apparently not impossible).
He was going to send his dictated note to my Neurosurgeon (Anesthesia uses these as well for their planning), PCP and Pain Mngmt dr so they are aware and know these newest changes and concerns.
As far as where would we do any valve replacement and what would we do - his hospital is nationally recognized for Congenital heart disease and has a great Adult Congenital Heart program with a surgeon that has done probably hundreds of heart valves is my guess but even Dr.Earing has yet to bring up his own hospital and broached a possible private heart hospital in Minneapolis (not Mayo which is where he trained) as well as we'd have to do some homework to see who if anyone has the most experience with valve replacements in MPS I Adults. Up to this point he has always discussed mechanical valve replacements despite needing long term medication (Coumadin) management on this as bovine (animal) valves have been shown to re-stenose and build up with GAG storage in seemingly short periods of time (years but not many?).
His big concern was between now and any surgery as well as he thinks the surgery itself and post-op would be extremely difficult due to the underlying MPS diagnosis and secondary shunt, breathing/lung issues and autonomic issues and quote "it wouldnt be pretty but we'd get you through it".   That is a surgery that scares the be-jeezus out of me due to the fact of cracking your chest;  I could barely think about where my Thoracic to Pleural shunt is knowing they moved the muscle alongside or near my ribs and feeling that valve move when id move many ways I cant imagine cardiac surgery. For now I think i'll just chose denial and chose to not tihnk about it to much!?!

My Pain Mngmt dr called my cell which I missed her call and so then emailed me an invitation to a discussion/meeting she is hosting alongside a DEA Represenative and other Pain Mngmt people about Pain Mngmt prescribing and use in today's environment. She asked if I would attend and participate in the discussion. That is Jan 29th and looks pretty interesting, it's an area I still have an interest in despite APF having closed so will probably attend. I do have my sunday school monthly meeting that same night and missed last month's due to being in the hospital + actually find these meetings really interesting but my co-teacher has said she would take notes for me.

Otherwise feeling decent; have been pretty tired yesterday and today but I think (hopefully) that is more from sleep and alot going on this week + just need to catch up. :)
I saw Dr.Bragg's NP this morning as Dr.Bragg wanted the incisions looked at and so Sue took out some of the many sutures (most are dissolvable but a few where removable) and in total there are 10 different incisions from the 4 different surgeries in Dec. - holy s**t! Thankfully discomfort/healing wise the only uncomfortable ones are where the valve sits in my mid-back and that is still far improved and my stomach I think isnt loving even just one shunt draining in to it. Could be worse!

Thanks for stopping by,


Thursday, January 3, 2013

5 days out.... Happy New Year!

A  New Year and I cant help but wonder what will it bring? Good things I hope  for myself and for all those around me I care about.   ... As seems to be often the case these days this update has spanned a few days.
I did get out on Sat and apologize for not updating sooner. This has been a harder recovery but I guess what can I really expect with 4 surgeries in 4 weeks and both shunts very difficult to place 1 and 2 weeks ago and both shunts being in new places which means essentially new adjustments as I basically figure out how will healing be and what will just be a long term 'new'? I dont know but always dislike this part!..    ... Thankfully each day does seem to be getting back to a little more normal and as irritating as the TPL shunt is to my back/ribcage it seems to be getting a little less worse.  
Thankfully with all these surgeries and CNS infection apparently that was the cause of the c-spine/head headaches I was getting (totally different than the shunt headaches where) as I havent had one since the shunts where taken out 3 or so weeks ago and maybe even since we had with the 1st of the 4 surgeries externalized the VP shunt at abdomen. Im not sure when they completely went away but it is AWESOME. I remember asking Dr.Bragg and the ID docs if they thought the headaches would go away with the IV antibiotics and shunt removals and they definitely thought it would clear up; so nice!! I do wonder if the symptoms I would get any time we tested the shunt reservoirs or did shunt taps will also completely have gone away and not be a problem any time we have to test either of these in the future? Who knows but I wouldnt be surprised I guess.

Several of the nurses said to me last week as they jokingly have taken to saying I should be earning 'frequent flyer miles' for being on their floor and in the OR so often in the past 1 1/2 yrs that they where trying to count how many time I had been there.. (approx 20 times)  I cant help but wonder will 2013 be better? I dont know and I wont venture to be cocky about my chances but I do know atleast I am happy to have been surrounded by family, such pleasant providers and friends who care and I hope it will be less involved shunt wise.  I'll just cont to live by 'one day at a time' as far as worrying about future medical issues or to be more specific what could or might occur. I can only control now and have to trust that God is in control, which I know he is.

I had emailed Dr.Bragg about specific characteristics of the 2 shunts (ie I knew the brands but didnt know where they fixed or programmable for instance) and she emailed back relatively quickly that the VP shunt is now in the parietal ventricle location (back of head) and the 'valve' she placed is actually more of a one-way system to prevent CSF (the fluid that is drained from around the brain) from backing up the shunt system from the peritoneal cavity and less of a valve as it has no real pressure setting.
The other valve (TPLS) is a PS Medical fixed low pressure valve which basically means both shunts are able to drain a maximum amount of CSF?
Headache wise I have done well (yah!) with only minor headaches in the past 2 mornings but the days other than getting sore around the TPL shunt are better and so many comments about how much 'brighter' my eyes seem. :) I dont have a huge appetite like I have with some previous shunt surgeries but I dont think that means to much as I dont have nausea and have taken 0 anti-nausea med since last week in-patient.

I was talking to my PT today (who I think was blown away by the amount of incisions, swelling (CSF pooling at lumbar incision and in stomach) and all that has occured but also commented how much "brighter" my eyes seemed which is a common sentiment I hear from several of my providers when we have working shunts!  I told him how the neck related headaches went away with the removal of both shunts and treatment w the IV antibiotics and yet how working w him in the months prior had made short lasting benefits in the symptoms. He works alot on lymphatic issues/swelling and commented maybe we where temporarily clearing some of the CNS infection and thats why I would get atleast moderate symptom improvement but then as the lymphatic system 'clogged' up again with infection in-between each Appt I would feel worse again. Interesting but could see it making sense?

I follow up with Infectious Disease on Friday at UW just for labs (not CSF) but have been finished w the IV antibiotics actually since Friday as they decided I had been on them long enough despite the 4th shunt surgery last Weds. Needless I was more than happy to have the Port access taken out! I had gotten ERT on friday in-patient (the Neurosurgery Pharmacist, my Neurosurgery team and Insur Case Man. really go out of their way to make sure the infusion gets done)  so it was decided I would skip this monday (NY Eve) and go back to regular schedule next monday.
Dr.Bragg's NP called me yesterday about starting another round of a one-time antibiotic for a repeat UTI infection (this antibiotic which is hard to get is the only one that can treat it/treat the particular bug and/or IV in-patient antibiotics anti-b's have to be given. We are waiting for my pharmacy to order the med. On friday they will repeat the UA and potentially do another 1 time round of the med. All things considered not the hardest issue.

I see Cardiology next week and think that Appt should be ok, I know numerous times in the hospital the nurses commented how low my BP (blood pressure) would get especially at night - at a couple of times apparently down to something like 70/30 so the Neurosurg Residents had them give bolus of fluid to bring it up but I slept through all of these episodes (amazing actually!) and low BP for me w the cardiac meds I am on seems to work well for the shortness of breath as other than I think when the shunt is trying to drain/absorb in lung now the cardiac symptoms of short of breath (like trying to breathe around an elephant) are pretty non-existant and I have no adverse affects from the low BP such as dizziness or related and feel good on that end.  An Echo is repeated every 6 mo but I cant imagine any suprises or even any med changes. 

Otherwise I have done a little shopping buying a new flat screen TV (yes I was in the dark ages w a old TV, I just never cared to much as I dont watch  a ton of TV) and looking at a new couch as well. I am looking fwd to going home this weekend (for good, I have been going back and forth) to get this stuff set up and to get my new internet (changing providers from US Cellular-wifi hotspot to Charter) early next week.  I also hope soon to be able to try and live-trap the 2 or 3 cats that are still outside my Apt building as I do have someone willing to take them on a farm, so we will see! In the mean time I take care of them and that may take some time.

Will update again sometime soon if or when there is anything to update (with next few appts maybe).
Thanks for stopping by,