Neurosurgery Appt; nothing really. OHS-Heart Surgeon Appt next wk.. Sometimes it's just hard..

Had Appt with Dr.Bragg today, this was rescheduled from yesterday due to the weather and not her clinic day so it ended up I got done in Rardiology about an hour before the Appt was scheduled for.
I just went over to the Children's Hospital and figured I'd wait, turns out she was in a surgery so she sent 1 of her Residents down (1 whom I think will be a really good Neurosurgeon but not really familiar w/my not very normal presentation) and anyways wasn't exactly a forthcoming appt. I suck at explaining my symptoms and especially what I have been experiencing lately so wasn't to optimistic when the Resident came in that anything would be figured out.
After we talked he went back and talked to Dr.Bragg in the OR and they called back down to 1 of her NPs who came and talked to me again. I figured this would be the case b/c I don't present very normally, I suck at explaining how I feel/what's different and yet Dr.Bragg is pretty good at figuring me out while most others are not generally. Anyways Dr.Bragg I guess opted to wait and see (fine) and I should call them back next week with an update or if anything has changed. Given this has been going on for a week or more and the memory/feeling very off has been since the 1st surgery (hard to explain this one) but had improved also a good deal when we turned the shunts down to drain the most (lowest setting on each shunt) I am not all that optimistic it's just suddenly going to improve. Can hope right?
I have no idea how to explain this and if I did I think it would help me explain it to Dr.Bragg who in turn then might have a better idea. I do 110% think (know) it is shunt related just don't know what or why or how to explain symptoms.
I have been wondering if the VPL Shunt is either sitting against some weird spot in the brain or in a spot it's never been before that can cause irriation and thus changes in the areas I've been dealing with? Again I honestly just don't know how to explain this. I do think this shunt (VPL) is working as when it seems to drain several times a day the irritation is very present albeit even if annoying I can live w the irritation b/c it typically means i'll finally feel some better. - Honestly I feel like I am talking in circles trying to explain this!?..

Even the vision issues, though I've not received my new glasses/lenses yet I have to admit I am not very optimistic this is going to help the overall symptoms either as it is the same visual presentation I get anytime there are shunt issues. Am still hoping a little though.
I do wonder and wondered even before we put this 2nd (VPL) Shunt in if the LP Shunt is not working well or not working very well for a couple reasons.
1. Prior to our placing the 2nd shunt in early Nov. when we'd tap the LP Shunt we weren't able to get very much fluid off and was very uncomfortable during the taps. I have had on/off leg pain which I also had last yr when the TPL Shunt wasn't working but we where not aware and the leg stuff just gradually built up to worse and worse over months but our being unaware it was caused by the non-working/not working well TPL Shunt made it an issue we just tried to then manage. The leg symptoms completely went away when that shunt was fixed (or maybe that's when it was removed cant remember exactly).
It' quite possible it is not the same issue now but seems similar; who knows?
In any case I don't know and wish it would just improve on it's own. I obviously like and appreciate Dr.Bragg but so wish I didn't need to ask for her help and so wish this symptoms would just go away on their own.
Admittedly (and I rarely share how I feel, today being an exception on fb and here) I worry a bit about if I end up going in to the upcoming OHS like symptoms are now seems a little daunting. =/ I also wonder from talking to a friend whose daughter also has complicated hydrocephalus (but only 1 shunt) but has multiple valves in place.. Her Mom said this was so her daughter wasn't going in every other wk for revisions.. We did try something similar back when I'd had just 1 shunt and didn't work at all b/c way to little CSF drainage..
I wonder would adding a 2nd valve to just 1 of my current shunts maybe help better now? Obviously I'd still have 2 separate shunts and would trying a 2nd valve in just 1 of the 2 shunts to slow CSF drainage a small amount? I don't know and not sure what Dr.Bragg would think but worth asking her about I guess? This way 1 of the 2 shunts would still drain free flow? I cant say I feel entirely optimistic we'll ever figure a perfect answer out but worth asking I guess? I do trust Dr.Bragg, atleast she hasn't given up so far..

Heart Surgeon Appt
I meet w my Heart Surgeon next week, not sure if she is/was able to arrange her schedule to be there but if so the Anesthesia dr. whose done all prior heart surgeries and procedures in the last 2.5yrs was going to try and arrange her schedule to be at either this appt or at the pre-op appt as she wants to be able to have in place an immediate post op plan for how long they'll keep sedated/intubated after the heart surgery and better management plan this go round. I trust her and know she has been thinking about this for some time so am glad this dr. has been considering this re post op management.
I have some questions for the Heart Surgeon and my Team including
1. What is new Coumadin/surgery blood thinner dosing when we do the 3rd OHS/2nd implanted heart valve?
2. Is the Port going to be ok in this surgery? - An important question to me to know/have a plan b/c of the difficult peripheral IV placements and weekly ERT Infusion.
3. What valve (mechanical I know) is he planning to use and straightfwd OHS (vs aortic valve we had to do aortic route enlargement in order to use an adult size valve) or does he feel he has to do something else?
4. On the off chance they do do have to place a chest tube on the R side what is their plan w the VPL Shunt catheter (ie what will they do with it, place via connector with my LP Shunt, externalize, etc)?
5. What is immediate post op plan (this goes more to my Anesthesia dr.).
Just some questions I have and better to ask them ahead of time since I am meeting with him next week, a few weeks ahead of the heart surgery.
Stay tuned, if you believe in it please say a prayer I an able to sort these headache/weird symptoms out and 2. things settle. I do not like having to ask my Providers for help and feel bad when it's less than clear.
Thanks for stopping by,
Eria

Eye Appt, seeing Dr.Bragg.. Christmas was still good.

Last wks Eye Appt went ok, turns out there where changes in several areas though I am not sure I'll remember it all correctly.. Vision wise the astigmatism is worse, far sighted vision has worsened and he added to my eye glases script for near sighted issues as well which he said had worsened since last yr. I can't remember what the 1 other issue was but mustn't have been to big. I did figure out if when they put in the dilating drops you don't keep your eyes closed the entire time they say your vision will be blurried but certainly not as out of focus as otherwise! This was nice and good as I had somewhere I wanted to be a few hrs after!
This yr the Eye dr. highly recommended (emphasized repeatedly) that I add the bifocal option to my new far sighted lens script. In addition last yr this Dr had recommended bifocals but the script was mild enough I could get away with out it (mostly just didn't want to adapt, lol) and honestly when the shunts where really good vision for reading was much improved so those periods where shunt(s) weren't as good I just haven't read much.
Thankfully things like this I can enlarge as I type and edit (though clearly anyone who reads this knows I still seem to likely miss enough). =)

The vision issues have admittedly stunk some as I love picking up a good book to read so perhaps it will be a good thing if I can get used to having the bifocals on the new frames as I can hopefully read more easily again?
Hopefully these things will help overall though at this pt (not yet having the new script or frames) I admittedly am thinking it's pretty unlikely the worsened pressure/vision is going to improve just by having the new/stronger glasses (lenses). In the past almost anytime vision was like it is now it would improve when we got the shunt/shunts working better again so I admittedly just don't know. Who knows?
I should get the new frames w/lenses (very similar in style to my current) sometime later this wk.
Because the pressure/headache type symptoms just seem to be getting worse over the past few weeks and not at all improving + the vision and in general so many areas just off I talked to Dr.Bragg and am going in to see her tmrw (Tues). She would have seen me today (Monday) but the weather here in WI is pretty miserable so she offered to see me Tues. which I am grateful for. I think she may be on vacation or not in the office the rest of this wk, I am not entirely sure but whatever the case very grateful she made time to see me.
I was really, really, realllyyyy trying to avoid having to see her (not b/c I don't like her, she is more than kind and has done so much for my care) but just want to figure this out, this time has been weiirrddd in many ways compared to many shunt issues.. I am always amazed honestly she doesn't give up and doesn't seem to get tired of trying to help me!?! I am definitely grateful though.
Like I said I really wish I wouldn't need to ask for her help, again not b/c she's not in reality amazing but I also know these symptoms are so off, worse than sometimes (but not vomiting off, just off in a weird, hard to explain way) so Dr.Bragg's Secretary called this morning and they've scheduled a CT Scan + Appt with Dr.Bragg Tues afternoon.
Hopefully, maybe some simple answer? I meet with the Heart Surgeon next wk Tues and this wk due to the weather today am missing ERT. It is a LITTLE frustrating that given FMLH's infusion clinic is open 7days a wk they can't manage to fit in the ERT Infusion a different day this wk but it's always that way "b/c your infusion runs over such a long period". When at CHW and my Infusion Nurses did ERT 1 day a week then had regular jobs at CHW they still managed most of the time to be able to reschedule infusion for a different day during the wk if I was sick or bad weather.. FMLH is CRAP in this regard!
Ironically, unrelated (and I don't at all regret not taking the earlier date) but had I opted for the 1st OHS date offered 1.5wks ago i'd be done with the 3rd OHS and on to recovery. Like I said though I don't regret not taking that 1st offered date - I am glad to have been able to spend Christmas outside the hospital! We had a good few days, even if I wasn't feeling the greatest at many moments I am still very, very grateful I was outside the hospital and with family!
Will update any info from tomorrow's Appt with Dr.Bragg in the next day or two.
Thanks for stopping by,
Erica

Nothing big, little updates... things on my mind..

Driving home tonight, from Infusion I had a lot on my mind, particularly thinking about the shunts, on/off headaches (definitely improved with the 2 shunts back in place which I am very grateful for yet at the same time there have been some pretty brutal momemts (days)! There are some moments, usually at night or in the early morning when the symptoms are there - still improved with the 2nd shunt back in place but symptoms there.

Anyways in addition to that I was thinking about the upcoming 3rd Heart surgery (OHS), about my Sunday School kids (I know random, scattered thoughts) and anyways turned on the music I have downloaded on my phone app to distract my thoughts.
This in turn I started thinking about favorite hymns and bible verses - again random but at the same time thoughts that bring great peace to me, probably bc these are songs and verses I've always liked.
Bible Verses
Ecclessiastes 3: 1-?
Ecclesiastes 3 New International Version (NIV)
A Time for Everything
3 There is a time for everything,
and a season for every activity under the heavens:
2 a time to be born and a time to die,
a time to plant and a time to uproot,
3 a time to kill and a time to heal,
a time to tear down and a time to build,
4
a time to weep and a time to laugh,
a time to mourn and a time to dance,
5
a time to scatter stones and a time to gather them,
a time to embrace and a time to refrain from embracing,
6
a time to search and a time to give up,
a time to keep and a time to throw away, 7

a time to tear and a time to mend,
a time to be silent and a time to speak, 8

a time to love and a time to hate,
a time for war and a time for peace.

Psalm 23 - The Lord is My Shepherd
1. The Lord's my Shepherd, I'll not want;
He makes me down to lie

In pastures green; He leadeth me
The quiet waters by.
2. My soul He doth restore again
And me to walk doth make
Within the paths of righteousness,
E'en for His own name's sake.
3. Yea, tho' I walk in death's dark vale,
Yet will I fear no ill;
For Thou art with me, and Thy rod
And staff me comfort still.

Back on medical-OHS related stuff, my Endocrine drs office called last wk, they sent over their instructions for pre/post Heart (OHS) Surgery to my Heart and Anesthesia Team.
Given its the same Team in entirety as the past 2 OHS they likely know the drill but my Endocrine just wanted to be sure. I am grateful she is so in depth and cares to be thorough.

Headaches have been wildly up and down this past couple wks, pretty much gone by the end of the day but the mornings pretty well stink. Yay hooray but want to pound my fist through a wall in some cases?..But seriously it still is better than prior to us putting the 2nd shunt back in, weird but true! I don't want to call Dr.Bragg, the symptoms are variable and yet I don't know do I say something to her before we get closer to the OHS date (about a Month away)? Damn I don't know what is the right thing I should do and I know Dr.Bragg always says we're a "Team" (I believe her, I don't think she would just say this) but I also so don't want to ask her for help again. AGGHH!!! Honestly I think the hardest thing since this last shunt surgery even though it had greatly improved in the 1st 2 wks is the odd little things with memory, like i'll be driving and while i'll know where I'm going i'll think I'm going the wrong way (but beats the other wk where I would turn and go the wrong way, was directions I'd take say to get to my Apt but totally not where I needed to be going at that moment or I'd be at a store and have to think for a couple mins before I left which ways I needed to go to get to the next stop or home! Now I just feel off with littler things like writing, still really weird with filling out forms/worksheets/writing in general, i'll know what I need to write but literally have to think about how to write letters before I can get them right OR lots and lots of white out! My poor Christmas cards this yr, some where fine, others not so much! White out really became my friend - almost funny but also frustratingly stupid bc I would know it was wrong but had to think about before I could make it right. Ahh well, my poor brain is just got a little stupider I guess?..
This wk is Christmas, hard to believe but also beautiful and so grateful to have spent this Christmas out of the hospital!!! I know 2016 already has the heart surgery scheduled but I celebrating 2 Christmases in a row not at UW or any other hospital and my 1st year in 4yrs of teaching I was able to not only plan but then also be at our Sun School kids Christmas Party. - I thought it went well, the SS kids seemed to have fun and I especially loved the hugs I received not only from my kids, in my class but even from a kid or 2 I had in yrs past! Love all of my kids and seeing them happy while also learning.
Thanks for stopping by, will try to update again soon, if nothing else I meet with my Heart Surgeon in a couple wks, after the Holidays.
Erica
>

Odds and Ends; nothing big. Shunts; Good, Frustrating, good.

Nothing big to update; we've made adjustments to the VPL Shunt which have made my feeling so off center (thinking?) better - not perfect but sure better a and that IS nice! Vision is still off BUT Better than it was.. before it was just kind of a mess in general - I could see and do things but lol, it was not normal by any measure. Now it just seem blurry with trying to read (which is not my normal either in reality, I feel like the LP Shunt is the better primary shunt) and I feel like the 2 together does make it much better as the day goes on but damn these mornings SUCK! Atleast today (Fri) was the best morning so far though, yippee!
Anyways I've made the Eye appt with local Optometrist and still need to set up with my Neuro-Opthalm. (I don't have a regular Opthalmologist). Who knows with this crap!?
I can remember Dr.Bragg saying something along the lines last year with the 2 shunts how they work better together but we know together the 2 don't really add up to 2 shunts and I think/knew she was absolutely right then and I know she's right still as headaches get so much better as the day goes on but still no where near as good as say when we have an External Drain (EVD, an external shunt, drains spinal fluid outside the body) in place... nor as good as when we do shunt taps and drain fluid off. Still i'll take this and who knows maybe each day will con't to get a little better or maybe it is that I need stronger glasses rx and if so i'll take that to. Whatever helps, little steps! Now sure beats a week ago or almost 2 wks ago!
I asked Dr.Bragg 2 questions, both why she did she think the pressure headaches would be so much worse in the morning and then get better as the day went on, almost to a normal as the day goes on? And the other if she knew, on the off chance if a a R sided pleural chest tube would have to be placed how would this work (My Cardiologist and Heart Surgeon told Dr.Bragg they didn't feel one would be need to be but would have a plan in place in case a R sided chest tube where) and anyways with this upcoming 3rd OHS did she know what the Team would do with the shunt catheter in the mean time on the off chance a R sided chest tube is needed?
So far the consensus seems is they feel the chance of needing a chest tube on the R side is small but if a R sided chest tube is needed the VPL Shunt would have to be I think per Dr.Bragg externalized and at some point probably connected with my LP Shunt in the peritoneum. Then later Dr.Bragg could re-internalize in pleural space. Hopefully won't be needed!
Hopefully this just wont be needed but I like to know details so was curious what she thought and given so many chest tubes where needed with both prior open heart surgeries (albeit different valve replaced) I am more or less just curious what she thought/thinks.
I'll be sure and ask my Heart Surgeon and Cardiologist and Anesthesiologist their thoughts to on this prior to the Heart surgery in Jan. - from Dr.E's (my Cardiologist) note it seems he thinks similarly, that it would be externalized and down the road Dr.Bragg's Team could put it back in the pleural space.
As far as why are headaches worse in the morning and get better as the day goes on Dr.Bragg said Spinal Fluid pressure is highest in the mornings and decreases as the day goes on so this likely explains why I feel worse in the mornings and improve as the day goes on. Frustrating!!! Perhaps most frustrating of all is the mixed up/feeling like I can't comprehend things normally though this is much improved, (seriously compared to a wk ago it is hugely improved). Vision is a little improved (other than writing is still a little off), reading is crap (I can enlarge my phone thankfully) but can't really read a book which bites big time, I love reading!

I am very grateful we have the 2nd shunt back in place but have to admit this past 2 wks has been a little frustrating with trying to sort out how to best get on top of these symptoms! It was even almost like when we had the shunts turned higher that the shunts drained better but this caused the weirdness/off center/off my normal thinking feeling-like I couldn't organize my thoughts or quite think normally. Yet we have them set at the bottom which drains the most and man the difference in that 1st hour yesterday (Weds) was like HALLELUJIA but the shunts don't seem to drain as well (Or really I think it's more they seem to drain, probably vents collapse, pressure builds, drains, ventricles collapse, pressure builds-forcing ventricles open repeatedly so not continuous headache (pressure) relief. CRAZY is what these shunts are! I said to Dr.Bragg I don't know how she stays sane in her business having her work and often (atleast in my case due to the MPS complexity) fail, having to re-do it over and over and yet she's so sane and nice and down to earth! <--- Hopefully each day does con't to improve as it has =)
In any case as I've said before I am grateful she doesn't give up and she does view my care and issues as a Team effort-us working together to make it better!
On a different note, I can't remember if I wrote about it in the last post but the Anesthesia (airway) dr who has done all my prior open heart surgeries (OHS's) and both prior heart procedures in the past 2+ yrs sent me a message and she is working to try and coord. her schedule so she can be at either the appt with Dr.Mitchell (Jan 5th I believe it is off hand) or the pre-op Appt, which is the day before this 3rd OHS (Jan 20th I believe).
She like I feels we should have in place a better post-op management plan for after this 3rd heart surgery to include 1. how long they'll leave intubated/sedated (both for airway and for recovery) and for post op recovery-pain mngmt control. I am glad she is thinking about these things! I do not really remember a lot about the 1st initial days with the 1st two (especially the 1st one given I was sedated for the initial 3 days and intubated) but apparently it was rough so I think her having been there and thinking this through plus putting her voice in to this is a good thing!
Dr.Kovach, my Heart Rhythm dr, (is in the same clinic as Dr.Earing, my Cardiologist) sent a message and said he would stop by sometime after the OHS while in-pt but was curious to see if HR would slow down with this surgery and with taking stress off the heart having to work so hard. He said his stopping the Ivabradine or not would depend on how heart rate did with this surgery. So it appears he'll stay involved and atleast there is a plan there to.
Next wk is a busy week with appts and other things going on, f/up appts and things at Church so hopefully this weather stays nice - I LOVE it!
Thanks for stopping by,
Erica

3rd OHS Scheduled.. Thanks but no thanks to OHS before Christmas...

**So the 3rd OHS is scheduled for Jan., 21st with pre-op scheduled for Jan 20th and I asked to meet with Dr.Mitchell, (my Heart Surgeon) ahead of surgery so that is scheduled for Jan. 5th. This is all at Children's Hospital of Milw. **

What a week (or couple wks?) - Whew this last surgery has both been sooo good and yet so befuddling In how it's seemed to mess with my brain! I do still get headaches but these are not as bad as they where prior to surgery which I am grateful for! This said my vision with typing this and if I try to read (I haven't even bothered with trying to read a book which makes me a little sad!) I just feel so out of sorts and is driving me batty!
Last wk our turning down the VPL Shunt 2 notches did help with the headaches and with the foggy brain/feeling off centered but at the same time I have to say this is still so there and keeping me feeling so weird feeling. Gawd I hope we can figure this out! We do have 2 more notches to turn the shunt down which I talked to Dr.Bragg and she was more than fine with our doing (she had told me as much last wk to).
I talked to her Secretary, Dr.Bragg was going to be out of the office this wk other than today, Dr.Bragg offered to try and see me today in her clinic but I can only do Infusion certain days due to the length of it and is hard to reschedule so I am working with her Secretary to see her 1 NP and have Emily re-progam the VPL Shunt on Weds.
I think turning the VPL Shunt down last wk helped but have to admit I have just never had this type of symptoms so while I know it's pressure related I am a little baffled by this all! Vision (like trying to read or trying to type this) is nothing short of frustratingly getting on my nerves! I am way behind on seeing the Eye dr (lol, something my Primary dr mentions every time I see her in the past yr) so infinally made an Eye Appt that is in 2wks. Maybe I just need stronger glasses but a little weird, if that is the case that it occurred right after this last shunt surgery. That all said reading Dr.Bragg's and the General Surgeon's surgery notes the surgery was nothing short of tedious it seems to get the new (VPL) Shunt placed. So who knows?

Heart surgery wise, Dr.Mitchell, my Heart Surgeon drs. Secretary called today and they have talked to both my Anesthesia dr. (Dr.Taylor, airway) and to my Cardiologist (Dr.Earing) and while I do want to feel better I did laugh and politely decline their offer to do this MVR replacement already the end of next wk. I am thinking they must have thought I'd opt to take the surgery date they offered next wk as the Secretary offered it to me while also adding 'You can decline and we'll pick a new date'. I did turn the date down politely and Lori, in turn talked to my Cardiologist and Anesthesia drs Team and came back by this afternoon (Monday) with new options. Perhaps some Patients would want to feel better that badly and not mind the risk of being in-pt for the holidays but I have so many things I want to do yet before Christmas and frankly have just spent to many prior Christmas's in recent years (2 of the prior 4 or 5) in-pt so I really, really want to try to avoid this one being in a hospital! Just need the shunts to last!
While it would mean missing less of my Sunday School class had I taken the OHS late next wk I have to many things for Christmas celebration planned with my SS kids and our program in the next couple wks. and with family. So Lori (Secretary) talked to Dr.Earing and coord. a time in Jan between his schedule and Dr.Mitchel's so they are there for surgery together. They also coord this all with Dr.Taylor for a time that works for us all. Yippe, skipee (note sarcasm).

Now to just keep both shunts working!!!!!

Stay tuned, never seems to be dull on the MPS front (I would take dull, it would be alright..).

Say a prayer this shunt/oddness improves/goes away!
Thanks for stopping by,
Erica

Turned down VPL Shunt, Former Airway (Anesthesia) Dr on board (3rd OHS)

I've not yet heard from Dr.Mitchell, my CV (Heart) Surgeon ofc but I also know it takes time. I know Dr.Earing works ALOT faster than most Dr's offices!
I know scheduling wise the Team is looking at Jan or Febr and I did hear from Dr.Taylor,  the Anethesia dr. who has done both prior OHS and both prior heart procedures.

She has reviewed my Cardiologists notes and
 is going to talk to the Team and coord. timing so I am relieved she's willing to do the Anethesia for this surgery to.. It takes stress off me knowing I'll have a Dr that's familiar w the airway issues to in addition to my Heart drs. have seen my Heart before in surgery.

Perhaps it sounds odd to be more concerned about the airway issues but I do feel more relieved w most (all?) surgeries or procedures when there's an Anesthesia Dr on the Team whose done surgery on my case before.

I had a post op Appt today w Dr.Bragg in which she told me not only was there 7 (or maybe it was 8) incisions, and sone funny conversation related to this, the General Surgeon and how rough my skin is to get shunt catheters through. 

I had to laugh when we where talking about the surgery, her and the General Surgeons response during!   Our skin with MPS can be unusually tough although most often it does soften once those of us who receive ERT start it. For whatever resin in my case this seems to either never have happened or has reversed itself as Dr.Bragg talks about w lost every surgery how tough the skin is to unnel catheters  through on order to place the shunts.

It seems I was also wrong about what setting the 2nd (VPL) Shunt was at.
As it turns out (and probably explains my damned  confusion /scatterbrained/feeling like I was literally losing my mind this past 2 wks) the VPL Shunt was set 4 above the bottom (but after surgery pressure was  to low so I had asked for the shunt to be set higher temporarily and while still in-pt we did turn it down 2   settings) so in fact yesterday (Thurs) we actually where able to turn it down 2 settings and Dr.Bragg said we have 2 more settings we can go down if needed. That made me super happy!
So at today's (Thurs ) appt Dr.Bragg turned the VPL Shunt down 2 more which definitely has already made a difference in my feeling like I was just danged confused about stupid stuff! In Madison today I needed to stop at 2 stores and I literally had to turn around no less than 5 times trying to get some place even though I knew where the store was! I backtracked, would turn around, back track some more. It was almost funny albeit super frustrating bc I knew where I needed to go but couldn't figure out what exact way I needed to take - this to places I go frequently!

Dr.Bragg did say  if I felt I needed it to let her know and she'd turn the shunt down the final 2 settings. I am glad she's willing if needed!

The only down side to this shunt (draining to pleural space)  was I already was getting a semi persistent cough the past few days by mid/later in the day day and can tell it's a little worse today. THAT is going to suck if it cont's during (after) the OHS!

As far as the up coming OHS and the VPL Shunt draining to the pleural space, my Heart Team/Dr.Bragg being are w it draining to lung space.  Dr.Bragg said part of this was they felt there was only a small chance they'd need to put a chest tube in on the R side and all where happy this shunt seems to be working so well so if needed /if they did end up needing to place a chest tube in that R sided pleural space during the OHS, the shunt cathether would just be externalized at that part of the shunt temporarily. I guess Dr.Bragg would re-internalize it later.
I amniy entirely sure how that would work given she's at a different hospital /different city but will ask her. I am hopeful this won't happen BUT if it did I am glad all (Dr.Bragg, Cardiologist and Heart Surgeon) are at least on the same page and I guess would talk to each other then to sort it out..
Will update when I know a surgery date,
Thanks for stopping by,

Erica

Cardiology Appt; Moving ahead with Mitral Valve replacement (3rd OHS)..

Do you ever have a hunch about an appt before you go? I have sort of felt in the past wk especially that today's Cardiology Appt was going to end up with us moving ahead with this MVR (open heart surgery).. Oddly enough, although I had this feeling I also kept thinking to myself, well maybe b/c I feel so about this, maybe it in fact will end up the Echo (heart ultrasound, takes pictures of heart valves and structures) will show improvements? LOL, oh so naïve am I... Believe me while I kind of hoped it would show improvements (no real reason it would given Mitral valve has continued to worsen slowly but steadily over the past 1-1.5yrs) I also kept thinking, well the last time we put 1 of the shunts to drain to the pleural (lung) space, a few wks later we did a Echo (just happened with timing, wasn't on purpose either that time, 2.5 yrs ago or this time) showed the then moderate-severe aortic valve was in the severe range. Now 2.5yrs later the Mitral valve is in the severe range.
For whatever reason God must think I am pretty bad ass and strong or something (I am 1/2 joking) that he'd give me this mitral valve challenge not 2.5yrs after I went through not 1 but 2 open heart surgeries to take out and replace the Aortic valve and to enlarge the aortic root?
Non-the-less, challenge accepted and I know whatever God's intent he is with me every step of the way and I am not going to question why he does the things he does nor when. I am instead grateful that God has given me the faith I have in him, even if it is shakeable and sometimes (probably) a questionable faith on my part.. I am grateful God has given me the Team of Providers (purposely capitalized those 1st letters) he has, that he has placed these Providers in my life and that God is with me through each and every struggle, storm and good moment I have..

Per today's Echo the Mitral valve had again worsened, which this is the 3rd time within a year the gradients have changed. Dr.Earing, my Cardiologist commented they could not get a reading (or good reading?) of the Tricuspid valve measurements and I think there was 1 other area though that I cant remember for sure. The Pulmonary Pressures where already high/increased back in summer so none of it is a good sign nor good for me in general.
He commented, if it where any other Patient of his, he'd have recommended a open heart surgery-valve replacement already a year ago but given I have so many other complicating factors (lung issues/breathing, joint issues/arthritis type issues, the autonomic issues and endocrine issues + the headaches he wasn't in a hurry to go to surgery as 1.We don't know what of your symptoms this will help, though I do think we need to do this'. and 2. 'I know you would tell me when you got to a point you thought we needed to do it and symptoms where to bad, I trust you'. (my paraphrasing). Heart rate today was unusually high compared to how it has been though no real good answer why that is. I am hopeful once this MVR is done and things stabilize, given they keep heart meds for awhile if they in the end would decide to keep anything that they would keep the Ivabradine over some of the other heart meds but we'll figure that out as time goes on. Heart rates in general have been improved on this med, minus today and some days. It hasn't really helped overall breathing/heart symptoms but I have to say in general having a even slightly slower heart rate has been nice!
Timing wise, Dr.Earing has already talked to my Heart Surgeon after I left today, the same dr. that did both prior OHS (with this one I plan to ask more questions and have a better plan in place, have asked if I can meet with Dr.Earing, Dr.Mitchell and with Dr.Taylor the Anesthesia dr. if she is able to do my surgery before the OHS day so we have a better plan in place for after). - I know last summer when Dr.Taylor did the anesthesia for my Heart Rhythm Study and Heart Cath she said to me she wondered if we did end up having to do a 3rd OHS if we should follow a longer intubation/sedation after any surgery and would that make recovery in the initial days easier than it was after the 2nd OHS (1st OHS initial days where easier than was the 2nd OHS though I admittedly don't remember it well). After the 1st OHS they kept me sedated and intubated for 3 days and after the 2nd one they extubated after 24hrs. + was a much more difficult initial recovery.
I am not sure but want to make sure these sort of things are ironed out ahead of time and a plan in place.. I'd also like my Heart Surgeon to talk to me atleast once after the surgery, I feel like you get the best information regarding a surgery a Patient has from the Surgeon who does it..
As far as the actual surgery goes we have time to plan so are likely not looking at doing the MVR replacement till Jan or Febr. after the holidays. This in order to give everyone time to plan and get the best plan in place. Per Dr.Bragg who I got a message from late this afternoon, Dr.Earing had already called her (I knew he was trying, Dr.E's Nurse Jane had called me re what to do with my Coumadin dosing for this wk as the level was pretty low despite being back on it for over a wk. and I'd forgotten to ask them what to do a the Appt.) Jane in turn after telling me what dose to take for this wk and when to re-check again asked for Dr.Bragg's #/best number to call (I hadn't thought to give it to them but gave her Dr.Bragg's Secretary who is amazing) and he must have called her right away and reached her.
They in turn must have talked and Dr.Bragg said Dr.Earing had already talked to my Heart Surgeon who felt we wouldn't need to move the VPL Shunt for this OHS. I admittedly am surprised by this but also happy! I am going to talk to Dr.Bragg more about this on Thurs at my Post-op f/up but if she's comfortable that is great and takes 1 complicating thing out of planning the OHS/getting it scheduled as we'd otherwise need to schedule the VPS move, wait a month after that then do the OHS.
Dr.Bragg also said in her message we could turn down the VPL Shunt at the f/up appt which I think will be good, my scatter brained-ness I am hoping is just bc pressure is a little to high! Today for instance I would be driving and kept thinking I was going the wrong way or in fact 1 time did follow a car the wrong way which not a huge deal, just meant back tracking but seriously! This on a hwy and route I have taken hundreds of times in the past 11-12 yrs! This or like figuring out what time I need to leave to get some place, I literally, no lie but have looked at my phone and alarm and reset it atleast 1/2 a doz. times last night and then still looked at it 2-3 times this morning figuring out what time I needed to get ready and leave. I mean seriously, how stupid but also almost funny bc it IS so stupid! My brain is like up and left the building 1/2 the time!
Anyways so regarding the OHS for the mitral valve I should hear tmrw (Weds) I guess or this wk and can decide when I want to do it. Definitely not before the holidays and most likely Jan or Febr. This means having to get someone to teach my Sun School class for a few wks but i'll sort this once I have a date picked out.. Apparently Dr.Earing wasn't aware I still teach SS as he said to me something along the line 'I didn't know you worked with kids, I'm not telling you not to do it especially if you enjoy it but the germs especially in your case aren't insignificant', lol. LOL =)
Dr.Earing when talking about the continuing, fairly fast progressing changes on the Echo and how unusual my valve changes/appearance are commented how he still wonders if some of my heart/heart valve issues are an Ehlers Danlos/Connective tissue dx in addition to the MPS and commented while he wonders this he has never seen my particular valve issues in any other of his (many) EDS/Connective tissue pts. but noR has he seen my particular valve issues in any other MPS pts. He was glad we're doing the gene testing although cautioned (which I already know) how much time this can take to get answers give it is a research study. To that I just said if it eventually gives another answer, great and if not we've not really lost anything. What matters is we just do our best to treat the issues as we can..

Otheriwse I am not sure what this MVR replacement will mean Coumadin wise. I think if I am not mistaken it means having to do Lovenox/Heparin injections anytime we'd have to go off Coumadin or anytime INR (blood thinner level) is low but I am not entirely sure and forgot to ask. I have to recheck INR on Monday so will ask Dr.E's Nurse, Jane then when they call with the new level. I know in some cases pts. have to bridge before surgeries or do other measures so I am a little curious.
Will update when I know anything further,
Thanks for stopping by,
Erica

VPL Shunt put back in, hard recovery, so happy!

**It appears there may be 5 incisions not the 4 I was thinking from last wks surgery, have to ask Dr.Bragg about this. But.. it seems like there are 2 under breast area, 2 along neck/area and 1 at the back of my head, where the proximal (tip) of the shunt goes in. Odd but wow and if so crazy (but grateful they do what's needed to get a good placement, as weird as that probably sounds)...

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Sorry for no update after last weeks surgery - Dr.Bragg worked w the Peds General Surgeon and Anesthesia was able to (apparently if I am remembering right) do the whole surgery with just an LMA to secure airway. That admittedly floored me and made me (at the same time) a little nervous heading back to surgery. I know she was going to use some fixation device during surgery to get a good catheter placement in the ventricle at the back of my skull so maybe that helped and is why they where able to use LMA despite having to (I would think) reposition during.
All seemed to get well in surgery, though this one definitely kicked my butt as far as recovery time after! I have a bit of a difficult time as it is recovering from some of these surgeries but this one was something altogether! Per Dr.Bragg she was super happy with the placement of the catheter in the ventricle and had (If I am remembering right) really good CSF flow this time which I can believe given the noticeable difference in symptoms. =) Initially we reset the 1 shunt to drain at a high #/less CSF and apparently from what Dr.Bragg said today the new shunt must also have been set higher (drain less) than we normally go. Initially this was definitely needed and as this past wk wore on my shunt setting on the new shunt was set back down to the lowest (drains the most). The LP Shunt is apparently still got room to go down on it so that is actually a good thing (that we have room to adjust) to!
In total there are 4 incisions, 2 on my neck, 1 on my upper chest and 1 at the back of my skull where they tunnel the shunt catheter in to the ventricle. Typically we've been able to get a shunt placed with just 3 incisions but Dr.Bragg said my skin was so tough and hard to get the shunt catheter through so they needed to do more indepth access while tunneling the catheter.
Discomfort wise is actually still quite a bit, it is much improved from a week ago, when surgery was but definitely still a degree of soreness I'm not quite used to! I'd say the worst area(s) are where the shunt was tunneled in to pleural space (below R breast) and otherwise the other 3 are improved but variable, ie more discomfort than I would say is my normal (saying a lot). This all said I'd do it again in a heartbear, not even over this surgery pain but if needed this seems to be the best working shunt we've had in a long, long time! I don't feel 100% and have virtually no appetite (not sure I ate any full meals this wk and even fairly minimal snacking) but I do feel better - not 100% but sure is better than leading up to this surgery! We've had 2 shunts before in place and working but this appears to be the best! Yay!!
I actually don't know how long surgery took but however long it was, was totally worth it and even with the discomfort I am sooo, soooo glad we put the 2nd one back in AND that it seems to be working so well!
This surgery definitely played with my mind a bit, what a week (lol) for odd things or instances I would fine myself in but to feel probably 70% better all I can say is ALELUIJAH! (sorry sp)! I told my Mom today if this shunt lasted like this for 6mo I'd celebrate (and I rarely ever (if ever) do the 'maybe this will be the last time we see you/maybe this time is it' thing!
I see Dr.Bragg Thurs next wk so we'll decide then if we're going to adjust the setting on the other shunt then.

Otherwise I see Cardiology for 3mo f/up, 6mo Echo on Tues so hopefully that goes well! Thanks for stopping by,
Erica >

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Surgery Mon., (Last) Tues Shunt tap, Thurs Heart Rhythm (med) f/up, Surgery .... heartache and loss.

Heart Rhythm Appt - Ivabradine f/up
I wrote below (the other day) about the appt with Dr.Bragg to take fluid off; today (Thurs) I saw Dr.Kovach, my Heart Rhythm dr. for Ivabradine f/up re the 1/mo (essentially) med f/up.
He is opting to keep the med dose the same as on the last Holter monitor the average heart rate was down to the 80s from a average heart rate of low 100s (100-110s) and he felt even if we where to lower HR further he wasn't so sure it would help symptoms more as we've not really made a big difference in symptoms I deal with heart related.
After thinking some about the appt given heart rates at virtually all appts I have been at and the few times I've read it on the phone app I have it's still been high 80s to 100s I wonder about upping the Ivabradine to 10mgs maybe during the day and keeping the 7.5mgs at the 2nd (later in the day) dose but will ask this when I f/up with Dr.Earing in 2wks (1st of Dec., Echo and Appt).
Otherwise Dr.Kovach and I talked again about do we add back in say a beta blocker, see if that helps symptoms? Do we give this Ivabradine more time? Or do we just be done with it and say we're going to do take the risk and do a 3rd open heart (OHS) surgery despite the unknowns and risks?
It's never been done 3x's in an MPS pt. but at a certain point your sort of just treading water, getting no where in a sense and so we're trying to weight when is a good time (in a there's never going to be a good time) with my body and the MPS risks?
He planned to talk to Dr.Earing (my Cardiologist) later this afternoon (I saw him late Thurs morning) and then they or we would discuss again after the Appt on the 1st what would be our next step...
I struggle a little bit with this all, I know I appear ok enough to them and that some of my symptoms though they would make more sense to Dr.Earing b/c he's known me for so many years I don't think they are always clear cut to Dr.K.
I am hoping (even though I really do trust Dr.Kovach) that when I see Dr.Earing he will be able to shed a little better light on some of the symptoms I have been having as I think I may have had Dr.Kovach perplexed a little, which I understand I think.
I don't always do a good job painting a picture of just how bad I feel often on top of I look good. This all said I don't think it's that Dr.Kovach in any way doesn't believe me I think he's just being cautious which I am ok with, he kind of wants to see how this shunt surgery goes and he wants to talk to Dr.Earing.
In any case we'll see how the Appt with Dr.Earing and the Echo goes 12/1 and then f/up with Dr.Kovach I guess will be determined based off that appt.
Talking to Dr Bragg today (Tues), both before and while she tapped the LP Shunt (to remove CSF) she asked/commented was surgery rescheduled to Monday for sure now? I think she jsut wanted to make sure it was on the schedule and we have it planned. I know she can see it on her schedule but on her part it's more about verifying we're on the same page I think. In talking about it she said something about finally we'll get you feeling better and I commented something to the effect "less than a wk to surgery; isn't it crazy to actually be looking fwd to this surgery /wanting it to occur?"
She laughed and said something about understanding and my not being the only Patient to ever say as much. Lol, I am pretty sure I said the essential same thing to Dr.Kovach today which he knows this has been in the works for some time. Lol, I think he got it to why I'd be kind of looking fwd to it being done without actually looking fwd to the surgery itself. Still it IS crazy and I know it!!!
Dr.Kovach's Nurse asked me at the Appt today (Thurs) "Do you like being there?" (in the hospital) - I am pretty sure I tried to not bust out laughing but said something to the effect if it weren't for the cleaning/not being able to clean myself (floors especially, broom anyone?) I could perhaps stand it a little better as I do get along w the majority of the Nurses and Staff fine and a few of the Nurses who traditionally ask to be on my 'Care Team' go out of their way to make it all more bearable which does indeed help. Still there's nothing like being at home! I can tolerate it but I am not a fan of hospitals.
I didn't ask Dr.Bragg, honestly, that would seem nosy to me but non-the-less Dr.Bragg said the reason she'd had to cancel and reschedule Thurs's surgery was her Husband's Mom had gotten sick unexpectedly and Weds was the 1st day she and her Husband where able to get flights to CA (where he is from, Dr.Bragg is originally from Chicago suburb).
No matter why she would have to reschedule and as much as I want surgery to just be done I could never be upset at her for needing to unexpectedly go out of town AND is sweet on her part she cared enough to send me a msg last wk + explain today.
For that matter for her to see me today for the shunt tap on her non-clinic day when she wouldn't have had to was very nice.
Just a few more days, God willing to (hopefully) feeling better.. The plan at this point is to place a VPL (Ventriculopleural) Shunt w Peds General Surgery assisting her for the portion of surgery where she'll place the shunt catheter in to the area around the lung space (due to so many previous surgeries and drain tubes) in this area causing scar tissue.
The head (brain/ventricle) portion of the shunt placement Dr Bragg said previously she planned to use a system where she can place my head in a static immovable position (I think it may be similar to what is used during C-spine/neck surgeries) as she said it's what they use in brain tumor surgeries to prevent any movement of the head.
They use that to try and get the the best placement of the shunt catheter in to the ventricle (unsure if it's the lateral or the 4th ventricle she's placing the proximal shunt catheter in) but placement will be at the back of my head.
She uses a computer and neuro-endoscope to get a good, direct view inside of the ventricle for best placement of the shunt, trying to avoid problems we've had in the past w this particular ventricle/placement.

I'll be saying my prayers! =)

Seprately on a really sad, not very great news note and has made this surgery harder still, my Grandma who has been sick and in a nursing home for several years now passed away Thurs. night. For her sake it's actually probably better, she's free of earthly pain and suffering and in a place, so, sooo very much better than earth. She's reunited with her husband my beloved Grandpa and above all in the glorious realm of God our heavenly father.


I know God has a purpose for everything but that doesn't make it easier to understand. I trust in God and I believe he has a purpose but it doesn't make my tears of loss any less. I worry about next wks surgery and not being there for the funeral. I don't think I need to be there to remember and to have memories of my Grandma but it does kind of suck. And I worry most about not being there for my Mom. She doesn't say much about it and doesn't share her emotions a lot (we share this trait in our family, hiding how we feel) but my Sister and I have talked several times about Monday's surgery and the funeral arrangements and we both worry about Mom and can tell she's upset. Why wouldn't she be though, that is her Mother and even if Grandma hasn't been the same for a long while that is Mom's Mom.
Anyways sorry to make this kind of a downer end and all, if you could keep my Mom and family in your prayers. I've prayed about it, talked to my Mom about it, talked to my Dad some and especially talked to my Sister about it and have decided to just go ahead and go forth with Monday's surgery. This means missing my Grandma's funeral but I know her spirit isn't there anyways. It is my Mom I really worry about with it all.
Ultimately even in In all things we may not understand God truly is good, and atleast we know in him a better place awaits for those we love and lose and who have had a faith in God.
Erica ***On a add in note, INR today per Cardiology was down to 1.41 after being off for just a day (last dose was Weds) so Dr.Bragg's NP said I did not have to re-check another level before Monday's surgery. Arrival time for surgery is 10:00 with surgery at 12:00, obviously this could vary a little given it is after Dr.Bragg's clinic and not a surgery day for her.
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VP Shunt rescheduled again. New date set... Life is frustrating..

j wrote the note below last evening (Thurs night) so this 1st part is an addendum of sorts... Forgive me if it perhaps gets a little confusing reading this and then reading below, I just don't think it's a bad thing sometimes to share my emotions when dealing w the very real frustrations that come w living w/MPS.
So the plan s written below, surgery for the 12th was cancelled and will now be Nov. 16th, a Monday. 

This is a non-surgery day for Dr.Bragg and is 1 week from this coming Monday. The plan is she'll still work w/Peds General Surgery to place the distal (far end) portion of the VP Shunt.
Because Monday is my normal ERT (Infusion) day this will be ordered for in-pt by the D6-4 Pharmacists and once mixed my Nurses run it for me. As of now the surgery time is slated for 12, which generally means a 10:00 arrival time. I am sure some of whether it will start on time will depend on Dr.Bragg's clinic and if it goes on time or not.
Because I normally get INR checked the day before a surgery it is a little more complicated this time but we tentatively have it planned i'll stop Coumadin this coming Weds, (standard 5 days prior) check INR Fri at CHW through Cardiology who will let me know what the # is and send the result to Dr.Bragg's Team. Chances are likely this # won't be low enough so I asked her NP to put in a 'STAT INR' order through UW and if needed a friend said UW's East Towne Lab is open till 5 on Sunday's. My Access Team Nurses will access my Port on Fri and over the wknd i'll flush/hep lock it each day + I already have the dressings needed to re-dress it after I shower.
Otherwise Dr.Bragg was willing and is going to tap the LP Shunt this wk on 1 of the few days she is back in town (scheduled for Tues) and will draw off more fluid to give temporary relief again. This only helps the headaches for a short few-ish hours but does help appetite and other things longer so in all honesty the relief is much welcomed!
The Appt with Dr.Kovach which was originally scheduled for the 19th was able to be moved up to this coming Thurs the 12th, my original surgery date. A LOT of phone calls, messages and emails ths morning but seems to be pretty well squared away/things in place for the 16th now.

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Thurs post - prior to new dates/plan -

 I just need to say 1st, I am not venting about Dr.Bragg or her Team, in fact I am just venting about how sucky life is sometimes.
That said Oh.My.Gosh, I feel like this stupid VP Shunt surgery is NEVER GOING TO HAPPEN! I'll know the new date tmrw, but Dr.Bragg's Secretary called late this afternoon, something came up and Dr.Bragg has to go out of town again late next wk. 

She is out of town now,  but she'll be back in town Monday till I assume Weds. I asked if she did have any clinics or time or if she thought we could maybe do intermittent shunt taps as we have been? Sure the taps don't last long headache wise but the somewhat longer improvement in appetite and for that matter from a headache standpt being able to sleep a bit better at night is a little better than nothing?! 

Believe me if I had the choice I'd have surgery still be next Thurs, it sucks, actually it sucks ALOT. That suckiness said that is life and frankly I just seem to have this happen in general more than I'd prefer (happened several times last yr, has happened with other non-shunt surgeries, etc). I can't even explain how frustrated this makes me or how disappointed I felt at this surgery being rescheduled again (was 1st originally scheduled to be this Tues, the 10th). I JUST WANT TO FEEL BETTER!?!?! Life will go on though.

I'll be frustrated about it now, feel angry (at MPS, at my body, at how little seeming control I have despite all that I do to try and have control) and then I guess i'll move on. I don't think I can say i'll accept the change b/c Thurs is going to come next wk and I am going to be frustrated all over again but at the same time I am already trying to re-arrange my schedule to make appts that where the following week to hopefully be next wk. I am trying to just do what I can to make this a little more bearable in the mean time. I have to find a little light or positive or something in this or otherwise honestly i'll probably blow a lid at my frustration at my body and MPS lately. This freaking sucks!
Dr.Bragg did say she thought she'd be able to find space to tap the shunt and take off fluid between now and surgery. I don't know how long she is out for so not sure if it would just be once or what. Any little bit would help I guess and I am glad she gave me that info. I know she isn't doing this on purpose so I don't blame her it just still doesn't make surgery being even further away yet again any easier. It's like the surgery that never will happen it feels like sometimes. Literally we've been planning for this shunt in a sense for months and I've been essentially waiting since last May, since the shunt infection and our removing the 2 shunts and only replacing 1 back in to go back to having 2 working shunts.

 
When talking to Anna, Dr.Bragg's Secretary, I asked when she thought surgery might be rescheduled to (I am more than sure I sounded less than happy, not on purpose and it isn't Anna or anyone's fault I just well it's hard although I know I didn't take my frustration out on Anna, I was upset but not rude). Anyways, when I asked Anna about a new date she thought Thurs, the 19th which of course (why not, right?) is when I am scheduled to f/up with Dr.Kovach (heart rhythm dr.). 

Anna was going to message Dr.Bragg to see if Dr.Bragg would let surgery be done as something other than a 1st case surgery in which case she (Anna) felt surgery could be atleast a little bit sooner. This to sucks frankly, actually, I absolutely hate surgeries that are not 1st case as they tend to run behind schedule. I am then hungry and it just gets to be a really, really long day. I just wish surgery could be next wk, which it can't be obviously.
Since it cant be the 12th (which go figure my favorite Nurse would have been there that Thurs-Sun and then if I was still there she would have been there Tues-Weds to) it would be nice if surgery could be that Monday, the 16th but there it would be an afternoon case I am sure. 

I know to Dr.Bragg has to work with the Peds General Surgeon due to the Pleural space placement of the distal (end of) the VP Shunt catheter so that to makes rescheduling the surgery a bit more difficult.
I am to call Anna Fri morning if I've not heard from her by 10:30 to get the new surgery date so I am not sure what other date she might be considering. As I wrote above maybe that Monday after Dr.Bragg's clinic or that Weds but I am guessing it will be that Thurs..? I know Tues is her normal surgery day to but would assume she already has cases that day, I don't honestly know. What I do know is this shit can't come soon enough <-- sorry for language but is true (which is sad bc seriously who really wants surgery?)! =/
I did call Dr.Kovach's EP Nurse line and sent him an email as well to see if the Appt on the 19th could potentially be changed to next wk, the 12th, the original surgery date. I know Dr.Kovach was wanting to see if our putting in the VP Shunt, and thus reducing ICP (pressure on the brain) would help bring heart rates down. I hadn't originally thought to much about this before but we dealt w the same high heart rates back prior to May, before we took out the 2nd shunt so I personally don't think our putting back in a 2nd shunt again now will help bring heart rates down so we'll see but said as much to Dr.K...
This all said atleast on a little bit of a good note, although it hasn't really helped how tired I feel or the intermittent breathing (suffocating-ish/short of breathe) feeling the Ivabradine has helped HRs some. Based off this last Holter monitor, done 2wks after we increased the Ivabradine to 7.5mgs, 2x's a day the average Heart rates have went from ~100s to average high 80s. That is atleast some good news, if only it would help symptoms! I know my Team has said they can increase the new med (Ivabradine) to 10mgs, 2x/a day so I am unsure if that will happen. Dr.Kovach had also said something about he could add back a prior heart med or we could talk about replacing the Mitral valve.
Sometimes admittedly lately I feel like risks or not just doing the MVR might be worth it. Argh at decisions and sometimes difficult ones! I guess we'll see what he says at the f/up and what the next Echo shows in Dec., + talk to Dr.Earing (my Cardiologist) then to.

Anyways, remember the last 1/2 of this was written before I knew the new surgery date.

Thanks for stopping by, just over a wk now..

Erica

VP Shunt plan; 2wks to surgery. Trying an old approach..

I saw Dr.Bragg today (how many posts start with/like that?) which was fine, I learned some more info about the upcoming VP Shunt surgery-install.
I have been wondering if the LP Shunt is working optimally, I don't think it's not working just more that based off how i've felt I wonder if the LP Shunt is just not working as well as it should. I am not entirely sure but based off the CT Scan which Dr.Bragg pulled up today the ventricle size is normal (which is a good thing) and should help the VP Shunt placement be more smooth/precise placement but at the same time my ventricles are never not small except when there's a shunt issue? I definitely don't think it's that the LP Shunt isn't working b/c clearly I'd be a lot sicker and my headaches wouldn't just be intermittent but I think the ventricle size could explain why it seems like my headaches and symptoms are a lot worse? I am not entirely sure, just my feeling..

As far as surgery, my Primary dr. messaged earlier today via MyChart and had not heard back from either of my Cardiology drs. (Cardiologist and Heart Rhythm dr.) yet so she planned to call them again if she'd still not heard anything by the end of today. I honestly am not sure what Dr.Bragg would do if they said either no to surgery or wanted some extra precaution? I pray she'd talk to them to make it a go! Hopefully there just won't be an issue.
In the mean time at today's Appt while looking at the CT Scan results Dr.Bragg talked about what her plan was with this VP Shunt re-replacement. Last time we really talked in depth about this surgery and placement of the distal (draining) end we had agreed b/c of the likely potential an OHS is going to happen to put the 2nd shunt to also drain in to the peritoneum (where the LP Shunt drains).
Today she asked my opinion instead of tunneling this VP Shunt when she places it to drain to the pleural (area around lung) space. She commented if/when we do end up doing the Open heart surgery she realized we'd likely have to go back in and move the shunt distal end but she strongly felt we should maximize the best potential for the most success we could for now and have the 2 shunts drain to separate locations. I was and am happy she thought this and wants to place it in pleural space for now! For all we know a 3rd OHS could be a ways down the road, it seems unlikely given the way my 2 Heart drs talk at each appt. but I would rather have to go back in later and move the distal end temporarily and have better chance of success now with better chance for relief now.
B/c of all the previous pleural space surgeries/procedures and scar tissue she'll get General Surgery involved to do that part of the VP Shunt surgery.. I didn't think to ask her but I wonder how that works, does the shunt get tunneled from the skull, behind the ear, down the chest and then kind of around the side to the pleural space or is there a way they can tunnel it to pleural space from the front? I'll have to ask her at Monday's appt. I am kind of curious.

As far as what ventricle she is going to try to place the distal (top portion of the cathether) in in the brain she said b/c ventricle size is bigger than normal (normal size but bigger than my normal I guess?) she was going to try to place it at the back of my head in the lateral ventricle (I don't know if that's what that ventricle is actually called) as she felt she'd have better success this time then we've had in the past. She also said in prior surgeries they've used a magnetic guidance type system, it's something where there's some sort of sticker (or something) on the forehead and that with the computer I guess helps guide their placement of the shunt catheter?
Anyways instead this time she is going to use a system they use when removing brain tumors, it must be something like they use in c-spine surgeries where your head is held in place by a fixture so it gives her the best chance of best catheter placement within the ventricle (more precise placement, better chance of best outcome I think) while also minimizing/preventing any movement of head during surgery which can throw their computer guided system off.

In any case as long as no hold ups of which I'm still praying every day for there not to be surgery is 2 wks from today. I have to admit this sometimes seems like the surgery that's been a long time coming, a long wait though I am glad we have it scheduled and I know the plan. Dr.Bragg is so awesome about it all even about placing the shunt catheter in to the pleural space for now, knowing it may mean another surgery for that down the road.

I was talking to an MPS Mom just a while ago tonight asking if she might know where I could potentially find the 'MPS I recommended assesments/testing' sheet Genzyme published some yrs ago (it is an elusive fact sheet it seems!) I've asked multiple different Medical People in the MPS Community and at Genzyme and none are entirely aware where to find it but several are helping to try to locate it, so I can send to my Primary dr.
In any case this Mom and I where talking about this sheet and how it would be good to make it available to families again as the information is probably needed - it is important information to know (once I find it/get a copy i'll post it to fb for families) and then she commented something about my having a Team that works together and talks to each other and how she wished she had that for her girls (they are each early 20's), Providers that where willing to work together and communicate as needed.
Honestly I remember just exactly how bad it was in the beginning and all the advice many families gave me. I know how many Specialists I saw and didn't really connect with so I found a different one over time in that Specialty and sometimes it took several different ones before I found one I felt like was a good fit for my care/and team. I also know although it can be a pain how much time I spend driving between where I live and Madison and where I live and Milw. b/c 1/2 my Providers are in Madison including my PCP and the other 1/2 are in Milw. including my weekly ERT Infusion but with this set up my Providers actually communicate with each other.
My PCP does an amazing job talking to other of my Providers when needed and overall my care is really pretty good and especially compared to many in the MPS Community. I get comments frequently from other families on my care Team and how they wish they had Providers like I do. I don't know what the answer to that is but I can say I am grateful for the drs I have albeit it took a lot of time and years to put together who I have on my Team now!
My care now with Providers scattered at 4 different health systems and in Boston (CHW, FMLH, UW-AFCH, UW, and Dean + Dr.Bodamer at BCH in Boston) is amazingly good and frankly much better than it was when all my Specialists where right at CHW and FMLH where each could literally much more easily reach each other. I can't complain to much and I love my Team. =)
I think beyond grateful would be appropriate words to use! God is Good and b/c of him I have great Providers!
Thanks for stopping by,
Erica

This is not my meme - contrary to some of my typing I can sp 'Operation' =)

Pre-op Appt (check), checking with Cardiology for 'ok'..

One more step checked off towards having the VP Shunt re-replaced Nov 12th. Today was the pre-op Appt with my Primary dr. She has cleared all aspects other than she wants to talk to my Cardiology Team and run a couple things by them make sure they are ok to sign off and do not want to see me before hand or have any concerns with symptoms. To use Dr.Simpson's words indirectly "I'll clear you for surgery if your Cardiology Team will but if they have any hesitations we'll have to put the shunt replacement on the backburner till these heart issues are sorted out". Yay?
I said to Dr.Simpson re this that I hope my Team will either say we can go ahead with surgery and keep the f/up appts I already have scheduled in the wks after surgery (both w/Dr.Kovach and with Dr.Earing + 6mo Echo) or if anything, if they have any concerns maybe request closer monitoring by Anesthesia but give us the go ahead for surgery. I am hoping we can get this surgery done, it seems like it's the 'surgery that's never gonna happen in some ways'!?
I swear if this surgery gets cancelled I am going to lose my mind!? I CANNOT even explain how bad I have felt lately, during nights/mornings to mid-afternoon and then I finally start to feel some better. - It is both shunt/headache pressure and heart symptoms BUT I absolutely know from a headache standpt we're doing the right thing putting a 2nd shunt back in. Mornings, nights and some parts of the day are beyond ridiculously aweful - sure I keep functioning but literally I need that 2nd shunt to take some of the pressure of the LP Shunt, my abdomen, very low back and neck are so uncomfortable (in 3 different ways). I'm gonna for real cry if this surgery where to be rescheduled!!!!
I do see Dr.Bragg on Thurs so will talk to her about all of this, it is really, really bad often lately so if you believe in it say a prayer! I want to say my Cardiology Team will clear me but who even knows, I don't know why they wouldn't b/c I think they realize it's not as if we're just doing this surgery for the sake of doing surgery but in fact to help symptoms overall.
Between the heart and shunt stuff Dr.Simpson and I where talking today, she (as do all of my Providers besides Dr.Bragg) was asking about the recent shunt surgeries we'd done, trying to sort what shunt we'd just did revisions on. Honestly the conversation was kind of funny, I think we where both laughing by the end and my teasing her (PCP) a little, she couldn't keep it all straight (teasing)? ;)
I don't fault Dr.Simpson or my Providers at all but today she was asking me about what was done in the 1st of those 2 last shunt revisions (about a month ago, a couple days apart) and what was done in the 2nd revision + what is it exactly we are doing in this next surgery.
Honestly I have to laugh a little b/c who could keep all these shunt revision/surgeries straight much less the overall issues? I am not sure there is any one of my Specialists/drs who can really keep straight what shunt I have vs what I've had in the past and what each of these surgeries are we do. =) It is a lot for sure!
My Primary dr. does a really great job managing everything and though she's still relatively new to my Team (3yrs I think) and had never heard of MPS prior to that (she told me as much) but she's learned a lot, really tried to work with my Providers and to stay on top of what is recommended for testing, even testing that others of my Team (Genetics) usually does as is the case now. She also never hesitates if she thinks it's needed to reach out to any 1 of my Providers such as Cardiology this time and last month she contacted my Genetics dr. in Boston to get better details re the gene mapping study.
She ordered a chest Xray today, is calling my Cardiology Team and is going to try to order the urine/blood test that is done for MPS. (all came back normal, we're sorting out how to go about ordering the urine/blood MPS test as it's pretty specialized and just a few labs our E. that run the testing.
We where talking about her caring for me and learning about my MPS, about how ideally a Patient with a complex diagnosis like my MPS would be followed by a Provider in a newer specialty like Med/Peds. This is what Dr.Earing / Adult Congenital Cardiologists train in before doing Cardiology residency/fellowship as these Providers train in both Pediatric Medicine and in Adult-Internal Med. so would see Patients from Pediatrics on through Adult.
In any case my Primary dr. now is Internal Medicine trained and has been really good for my Team, she may not know everything about MPS but she learns, reads and talks to my Providers and she isn't afraid to make suggestions where she thinks something might be better.
As we where talking about everything going on I made the comment to her 'wouldn't dull and boring just be good' (her MA comments pretty much every time I am there how frequent they see me for pre-ops and as she's leaving each time she'll say 'I hope this is the time we don't see you for a surgery visit for a good long time'. Funny, funny sometimes these visits. We definitely laugh a little at the insanity of it all.
After I made the 'dull and boring' comment, Dr.Simpson said to me something like 'In healthcare that is often the case but that just wouldn't be you". LOL, sadly so true?
As far as knowing if surgery is a go from Dr.Simpson and Dr.Earing/Dr.Kovach's perspective Dr.Simpson was going to reach out to them and thought she'd know by Fri. I am praying for a 'YES, She can go ahead' (with surgery)!
Dr.Simpson said something along the lines 'I know your not going to be excited if the surgery is postponed (an slight understatement, I don't want any surgery but I do want these headaches/symptoms better controlled) but technically your heart comes before anything else'. I think she also know I wouldn't be wanting this surgery or going for it though if we didn't think it would really help so I think she'll do her best to get surgery approved.
Sometimes I think it is just it sucks how tied in each issue is to 1 another and for that matter I commented to her these days, sometimes I feel like we'd be better off just to do the 3rd OHS and manage the risks, get it over with so I'd feel atleast some better on the heart front!? Ugh!! As I've written about before my Cardiology Team has tossed around doing the 3rd OHS at just about every visit so it's something we've all considered and just a matter of when is the right (best/better?) time I guess.

I think in some ways it's just hit me the past few wks how truly terrible I feel many days, it kind of stinks actually. My Mom, Sister, nephew and I went bumming this past Sat and the 1 before that which is something I'd have normally looked fwd to but instead I sort of just 1/2 dreaded it (it was fun, I just stay in the car or wait at the front of stores when done) b/c even little amounts of going from 1 store to the next or garage saling flat our wears me out which I might add stinks!.
On a fun note Zan spent the past 2 Sat nights, he's easy enough so that was fun. =)
Often 1/4-1/2 way through anything we do I end up staying in the van which Zan often then will chose to hang out w me. I don't mind just waiting but I truly look fwd to the day, perhaps post a 3rd Heart surgery as kind of daunting as that tends to seem (b/c of the unknown, knowing it's never been done before in an MPS pt. 3x's) I hope I get more energy again after!
These days some days are definitely better than others and maybe it will be a little better after we put the 2nd shunt back in.

I have 2 more appts set up w Dr.Bragg, for her to take off fluid before the surgery the 12th as long as it goes ahead. The 1st is this Thurs, the 29th and Mon the 2nd I believe which is this Thurs and next Mon, (ERT switched to Tues). Otherwise I did the Stealth CT Scan last Tues, 20th which is what she uses to place the ventricular (brain) portion of the VP Shunt catheter.
As long as no issues come up with the surgery i'll test INR per usual a day or so before the surgery. Given surgery is now on a Thurs, I may have to make a separate trip to Milw. but have to think about this some.
Lastly I did the Holter (Zio) monitor last wk for just under 2 days which turned out to be a tad on the interesting side as I kept bleeding under the monitor on/off and making a huge mess especially at night. My Team will have the results in 1.5wks or so.
Un-related to surgery I talked to the Genetics Counselor-Research person at my Genetics drs. ofc. in Boston and they mailed out the consent form for the gene mapping study. This in turn she said can take a while to get the test results, up to 6mo once I sign and mail back the consent form and complete the actual test which they use blood or saliva.
This literally maps the entire body-looking at any and all abnormalities in all genes and chromosomes both to see if there is any other underlying, un-diagnosed issue to explain the (especially) unusual heart issues my Team has dealt with.
Per the UofMn drs and my Genetics dr., both of whom see a lot of MPS pts they have never seen the issues apparently my Team dealt w in surgery and the issues we deal with now/the unusual progression I guess.
In addition to the moderate-Hurler Scheie like mutations but very mild outer features. My Cardiology Team here and Genetics Team in Boston wonder if a separate/secondary issue could potential explain the issues we have/or help them when we do go forth w the 3rd OHS planning.
In any case will update here when I know anything,
Thanks for stopping by,
Erica Pre-op, pre-surgery check list

Part of Z's Christmas list - to funny =)

Just b/c I love him =)

Recent Heart monitor (oh what a mess I made)

New jacket, a little (Team) MPS Awareness

Shunt tap (is so good), Surgery date change. Musings as a Rare and complex pt.

"I think they fail often b/c it is a such "one size fits all" solution" for a condition that is anything but "one size fits all (hydrocephalus)".
I started writing this entry the other day, before today's Appt with Dr.Bragg, to have some CSF (spinal fluid, what the shunt drains) taken off. I saw the above quote on a page I sometimes visit and thought it was pretty appropriate (AND true)!
Anyways so I did see Dr.Bragg today; after chatting some about the dates she'll be out, about setting up a few more appts to have her take off some more CSF (intermittently, gives short term therapeutic relief) to get by till we have surgery to put back in the VP Shunt. She tapped the LP Shunt reservoir which is not painful and pulled off the amount of fluid she is able. It isn't a lot but it does help, almost like a 'parting of the clouds' temporarily.
I didn't feel terrible while seeing her but it's still a noticeable difference on my part after I get up from the table - truly like my head clears and best of all, weirdest probably of all, despite there being some nerve irritation in my low back as fluid is being pulled off (nothing that would make me respond in a way to make the pulling discomfort noticeable to anyone else I don't think) the residual back discomfort that I get (much improved nerve irritation since we went back to a Codman Hakim valve but not as good w this mini Codman version of the valve as it was w the full sized valve which perhaps that sounds weird but for whatever reason it's true).
In any case the residual discomfort that is there, worst with sitting was completely gone on my drive home and has cont'd to be good tonight! The headache relief wont last as long as the back relief but it's as if it all combined gives my body a mini break, a break i'll gladly take given the shunt taps themselves minus that nerve-pulling sensation don't hurt.
Surgery itself was switched to the 12th instead of the 10th, her Secretary called today to ask about switching the day, and explain why they needed to. Originally it was going to have to be the following wk, the 18th (or 1 of those days) as I didn't want to do the 1st wk of Nov. given Dr.Bragg will be back for a few days and then out for a few days again. This 2 day change does mean having to re-arrange a few things but being perfectly honest I feel like this is what I do constantly so what's a few more calls, right? I'll survive, I just can't wait for the shunt to be back in! 

Given Dr.Bragg offered to do a few shunt taps during that time and if I cone up w any questions about the VPS replacement I can ask those to at the shunt tap appts I am OK it being slightly delayed. I'd rather do surgery when there's the best chance Dr.Bragg will be arundel the whole time! 

I am always surprised Dr.Bragg is so kind (but most of my Dr's are, maybe it's as simple as if we as pts appreciate our Providers they treat us well?) I don't know but I am always so grateful to her for her being willing to do little things to help me! She said to me if I needed anything she could help with/had any questions for her while she is out I should let her know and she'd  get back to me. She is going to a Conference part of her initial time out but just said she'd see her msgs.

 No Provider has to make themselves accessible to a Patient so I just have to say I think she is really wonderful. I know I've said it before but I feel blessed to have her on my Team. 

I sometimes think about say 5 or 6yrs ago at this time I was still looking for help and answers and now? Now she just never gives up. I don't know how I got so lucky but God is good!

On a totally different note, I wrote this earlier in the wk, a friend had written something related to her daughters surgery and I was overly tired which is when I tend to reflect on the shittier side of all we (MPS pts., rare disease pts., chronic dx pts.) deal with. -
Honestly I don't know what made me think about this, perhaps reading a friend's post about her daughter, w MPS I facing more surgery and the uncertainty at times of what decisions to make. Thinking some lately about the heart stuff, the shunt stuff but especially about the heart/lung stuff and although I don't let it stop me how it does sort of hang over every other aspect of my medical care...
I kept thinking about that night, it's shitty we have to sign these consent forms pointing out the risks of our surgeries. Crappy we have to sit in our drs. offices and hear the risks and how high risk we are for surgeries but how these surgeries are needed for better quality of life. We consent to these surgeries and procedures, yet we know they may not always work.
We spend so much time on the phone scheduling, coordinating, dealing with insurance and many people, who have no clue assume our lives are so easy b/c they have no idea the amount of time all of these things take on top of the amount of time we spend driving to, sitting in and talking to/working with our Providers.

How despite this all many of us still try to be involved in things that have nothing to do with our care (or in my case a combination of advocacy,  my work w Gene Spotlight and outside interests in-related to MPS). How sometimes we're  so exhausted it's literally a fight to pull ourselves out of bed, in to the shower and through the task/activity/mtng at hand b/c we just want to sit down in the chair or crawl under the covers and sleep and yet we (I atleast) feel guilty if I don't go to whatever it is I have going on so I go.
How we often deal with nausea and headaches and pain while we go about the 'normal' things we do. How cracking open a med bottle, opening up a thing or 2 or more of nausea med to prevent from throwing up while trying to focus on the Church service or Sunday School class or whatever your doing with family or friends that day and yet most have no clue (and sometimes that's ok).
How many people assume your abusing that handicap parking spot "because you look so normal", "do you know that's a handicap spot " and all you want to say is 'no shit asshole' but you don't,  instead educating (lecturing sometimes) the person on not assuming, on what MPS is, etc.
We know chances are if we do opt for a surgery it may or may not work and if it does work, initially we may come out feeling worse or more discomfort on 1 hand (although if goes as planned we may also experience relief on the original problem) until our bodies heal.
Some surgeries are easier than others, the ones that seem like they should be the hardest (OHS?) albeit difficult at 1st tend, at least for me ultimately to have a quicker recovery than others.
How you feel guilty when you don't feel like your recovery is going as quick as those around you seem to think it should? Still on that pain machine? It's not my Providers who I feel guilt from about this but oh do I feel guilt sometimes when in-pt, as if I am supposed to heal according to someone else's well set plan.
I don't think people always get, while some surgeries are pretty straight fwd and small, the surgery itself wreaks havoc often on an entire MPS pts body due to positioning and other factors.. I don't think family or friends or Providers mean to make pts. feel guilty but sometimes unintentionally it happens. Believe me, as pts. we wish we could heal faster to and feel more normal faster to!

When it comes to surgeries we hope by signing these consent forms, sitting through 1 more pre-op physical, 1 more "these are the risks, this is what we're going to do" that we make the right treatment choice. We hope (pray!) we get the right Anesthesia 'Team' (if we're lucky as difficult airway Pts. it's someone who has had our case before) and who takes the time to work w the whole "Team" to make the best decisions for surgeries or procedures that bring us through safely w the least amount of issues or hopefully no issues.
We hope the entire "Team" works together, truly as a Team for our care to make surgery/procedure or even just care the best it can be.
As medically complex, complicated Pts. we just want Providers that care and when really lucky albeit no pt. (including myself!) really ever wants to need a Provider or Providers time so often they become as familiar w you as some of your own friends are, if or when this DOES occur you want the type of Providers that truly do care enough to care about you as more than just a diagnosis they have to treat. They know you as a person with outside interests and likes and want to help you succeed in life...

You want to be a person first, a diagnosis 2nd.  
Ivabradine
In reading some more about the Ivabradine (Corlanor)  I was curious about some things to do with it. I learned a little bit, some things I knew, some things I knew where my own Cardiology Teams wishes for the drug and other info I read was just interesting.
Ideal Heart rate is between 50-60bpm. On average, recording HR via an app on my phone daily the #'s have been low 80s to low 100s w an occasional mid-70-80s heart rate.
So far, at least thankfully no side effects on this new 7.5mgs dose adjustment. Thankfully also the med when I picked up the new script came as a 7.5mg pill so while I'll split my old pills to use them up in the future will be nice to not need to use the pull splitter on these!
The only others med I have to split some days is Coumadin (I have multiple different strengths of the med) but those are easily split by hand even w limited hand/thumb strength!
From reading the Ivabradine (Corlanor) website they list 7.5mgs as the maximum suggested dose though apparently my Team has seen 10mgs used in some HR resistant pts.
Lastly, last wk Dr.Kovach had said I guess to let him know if there where any issues or no changes in 2wks (would be next wk) and the website also suggests any dose adjustments be done at 2wk periods.
I love the 'super hero' shape >

Sunday School Class

Just b/c he's so cute =)

Kinked catheter from prior to 2nd LP Shunt revision 9/24 -