Thursday, December 29, 2016

8:45 / 10:45 Shunt surgery / Post Heart Ablation Appt.

πŸ˜ƒIt seems sort of hard to believe another Christmas is over and another year will soon draw to a near!? I suspect i'll be writing this post over the next week (this was in fact true, I've edited and changed a few things so it makes sense!) but have had so many things on my mind the past few wks. From feeling guilty in a way for my oft to opinionated self (in trying to help people, not that I think I am better than others, if there's 1 thing I know, I most definitely know I am in no way superior to anyone and in fact often feel guilty for all who help me/support me and for what I feel like is so little support I can give in turn).
 I guess what I mean is truly being able to offer useful advice, being able to help people more  and at times recognizing when to just shush. I'll be the 1st to admit I'm not always good at this!

Going in to this new year with a surgery the day before the New Year (tmrw) and having had a virus or something since Christmas Day but no major symptoms other than hoarse voice since and a sore throat that 1st day I am hoping I get cleared for surgery tmrw.
I've talked to Neurosurgery several times over the past few days, they want to go ahead with surgery given it's primarily just been the hoarse voice but ultimately they are leaving it up to the Anesthesia Team i'll have so I won't truly know till I am checked in at UW. I am praying! Really it's just that the catheter seems to be irritating more and further areas so while headaches have been pretty darn good (i'll miss that!) the rest is enough to drive me to want to drink, except I don't drink 
    The abnormal (coiled) catheter. 2nd pic (next to it, right after the LP   Shunt placed) what it should look like.

I saw my Heart Rhythm dr. earlier this wk; a f/up Appt. from the repeat ablation we did a few wks ago. While heart rate is back in the low 100s again, it seems from the EKG done today, right before seeing Dr.Kovach that while heart rate is back to being to high it is still different than before we did the ablation.
 From what he feels based off that EKG and depending upon what he sees when gets and reviews the Zio (Heart) monitor I did over 48hrs he had a few thoughts.
 If heart rate is in fact coming from a different area in the Sinus node this is better from what he said though i'll admit I can't remember why 1 area is better than another but he was/will be pleased if that is the case.
 Overall if heart rate stays high though once I am over this virus then he just said we'd likely revisit restarting the Ivabradine which unlike some of the other heart meds I take is more specific to try and get heart rate down and works on a different area of the heart than do most classes of meds including the others I take.

He also said if the area where this fast of rhythm is coming from stays where it is/shows on the post ablation clinic EKG he would hesitate to go back in and "hammer away at it more" (lol, I didn't take any notes but distinctly remember that comment) as he has fear that we'd very likely end up with need for a pacemaker then.
  It is basically a careful balance of managing symptoms/controlling secondary affects this added work from the heart beating overly fast has on my heart muscle and the mechanical heart valves and not doing to much that we cause an issue in the opposite direction (heart not able to beat enough on it's own).
I'm not sure why as it doesn't seem a common issue in MPS but we had issues almost from the start after we replaced the aortic valve and ended up completely re-doing that 6wks later and this new (Mitral) valve which we replaced a year ago this Jan is showing moderate stenosis (narrowing) in the new valve already. Not ideal!? I do know that's partially why the 'whole exome' testing was done by my BCH Genetics Team as they and my Cardiologist where interested if there was perhaps something besides just MPS going on, I guess.

The truer test re heart rate may be what the 48hr monitor I wore a few days ago shows, re does that show heart rate primarily coming from the same area the 30sec EKG in clinic showed? If it is primarily coming from this new area that is at least, possibly more promising.. It'd be fantastic if heart rate itself where down as that seems to really affect my breathing and SOB feeling (is intermittent, not constant and more of a 'trying to get a deep enough, easy enough breath of air' vs 'I've just run a distance and short of breath/trying to catch my breath now' feeling) but any improvement is an improvement, right?

 Since I f/up with my Cardiologist a couple wks from now Dr.Kovach just said he'd try to stop in when I see Dr.Earing (Cardiology) in a couple wks (14th), see how various symptoms and heart rate are. He was concerned with those symptoms and tmrws surgery and was that the cause of the increased heart rate (I tend to think it wasn't as heart rate trended back up within a day or so of the ablation) but wants to make sure the symptoms aren't related to the virus I seem to be battling now (is sort of a weird one, kind of wants to be a virus but knock on wood hasn't been to bad - am praying stays this way and goes away completely)!
 We'll decide perhaps at next Appt (but not sure) if we'll re-add any meds (the only 1 I'm not on right now is Ivabradine, which ironically of all the heart meds I take is also the gentlest/least-to no side effects!). Otherwise I take Digoxin, Lisinopril for heart muscle/help heart overall primarily (I think I got what they are for right) and then baby Aspirin, Lovenox injections and Lasix + Spironolactone for fluid/help heart overall.
 Ah irony, you kill me sometimes!

Non-the-less I personally think the heart rate is unrelated to this 'whatever it is, whatever my body is doing' and more related to the usual heart rate issues we've been dealing with, even if originating from a different area but we shall see.  Either way is nice that the ablation seems to have had atleast some impact!
Overall otherwise  symptoms wise the shortness of breath is there some, I have felt incredibly tired and incredibly uncomfortable (this last 1 from the Lumbar (low back) shunt catheter which either seems to be shifting (?) or just in general causing irritation b/c of where it's been sitting out of place. RE the SOB and tired we're hoping is maybe just related to the virus my bodies deciding what to do with.
Whatever it is I HOPE symptoms go AWAY for good, each day has been less of a hoarse voice so am really hoping will just be good for Fri! It's not even that I'm thrilled to be doing this surgery as I'm not, I'm anything but BUT then at the same time holy crud the low back/mid-back discomfort is something I can't even explain! And when it gets to a certain point (not pain but seeming swelling) then it's affects little things (hard to explain but I've talked about it here before) that's SUPER annoying!
Will be nice when that's just working right again! I'm sure i'll miss the spinal fluid leak I've had going on as that has been reeeaallllyyy good but the rest is just annoying/uncomfortable!

In any case will try to update sometime in the next few days if surgery is a go tmrw (if Anesthesia gives their ok).
Thanks for stopping by,
                                                                 Zan and I
                              My Sister, myself and my niece (we do this every yr)
Happy New Year! I am truly blessed by so many people and things in my own life! It's been a tough year at times, with changes to my Provider Team, quite a few more surgeries  and other things and yet it's another yr. i've beat MPS!


Friday, December 16, 2016

Sinus Node (Modification) Ablation done -

Our goal with re-trying this sinus node modification (ablation but intent not to completely kill/ablate the sinus node as that's the heart natural pacemaker)  was to get Heart rate in the 70s. This worked for  about  the 1st 24+ hrs (varied a little) where heart rate had seemed to settle in to the 80s.  Had Heart rate stayed there Dr.Kovach said he would not likely repeat the procedure I think if I remember right b/c the risk of permanent damage to the sinus node rises some each time and 80s would still have been 10-20 beat drop from what we've averaged (90s-110s for the most part).

The Ablation itself went somewhat ahead of time which is always a HUGE bonus (less time to think about all the various food I want b/c I can't have it πŸ˜  ). Maybe this going ahead of time was a small pay back for the last surgery having been hours behind? =) Who knows but non-the-less is always nice!
 I have to say to I'm always a little impressed, with Dr.Taylor and her anesthesia approach, it's like 1 min we're talking or I'm aware and the next second I'm not. At UW (where shunt surgeries are) I'm not sure what's different but I fight that anesthesia stuff to the last bit, lol trying to keep my eyes open. It's a control thing, I'm sure. A bit like not taking the pre-OR sedation meds they can offer (on this Dr.Taylor often makes comment along the line 'she's cool as a cucumber', lol or 'we don't have to worry about her'. I just like knowing what's going on as long as possible. =)
 I always say and seriously wonder (but not sure I really want to know!) what I say when I'm out but not really out. I can remember a few times Dr.Bragg would talk about conversations I was having or things I'd ask (and for that matter with the last surgery - can't remember if it was the last OHS back in Jan, almost a yr ago or if it was when we where doing the last ablation but I apparently asked Dr.Taylor if she'd/they could take pictures in the OR). So weird on my part!

So anyways the ablation worked initially, like the 1st time we tried though this time seemed to last for about 24hrs (maybe it was about the same last time, I actually can't remember)  and has steadily climbed and stayed back up in the 90s to 100s since being home. I know Dr.Kovach said it's not to unusual with the sinus node to have to do repeat ablations but it's still kind of a bummer! If only the meds would work like they used to, pre-the MVR replacement (had better control of heart rate and thus heart function).

I'm not to sure what next step will be, but I know further ablations are on the table as 1 option. It's not even like they hurt or are terrible, really the worst is the 4hr period I have to lay flat after (I thought this time might be a bit better but it seems even when the shunts are good or in current case 1 is good and 1 is leaking causing it to be a bad/good my body just doesn't like to be flat and the shunts just don't drain particularly well laying flat.

On a side note, Dr.Taylor (anesthesia, my airway dr. for all these heart procedures and open heart surgeries) mentioned while we where waiting in pre-op that she had a Fellow she asked to come in on the case with her. She wasn't yet sure if he was going to and I forgot to ask her when she stopped by Thurs morning, the day after if this Fellow had been in on the case with her or not. (usually she  just stops by to go over what she used, her findings, see how things are).
She has been interested in doing a small group presentation or write up (honestly thinking about it I can't remember which it was!)  on our history together, her airway findings and I think changes she's dealt with through the various (three) surgeries and 3 procedures.
In all that I just think it's funny though definitely nice to have had her for it all b/c she's become so familiar with my case, with MPS that she keeps a running tally of how many surgeries and procedures we've done together and knows when the 1st and subsequent ones where.

They did not do measurements of the various heart functions with this Cath/Ablation so I have to find out from Dr.Earing if we in fact have to do a repeat Echo before I see him (beginning of Jan., after the shunt surgery) or if we are good there. He had mentioned something about this at the last Appt but I can't remember what exactly he said and it's not listed in his clinic note. Either way not a huge deal.
I didn't think of it at the time Dr.Kovach mentioned he was going to place cathether in both sides of my groin (each side of body) as well as 1 in my neck (but that 1 that was placed in my neck is where my VPL Shunt used to be until that was removed last month and instead tunneled down the back of my neck and my back to pleural (lung) space. I am curious if getting the Heart Cath in was easy or any issues. If I remember (lol, questionable) i'll ask Dr.Kovach this at the f/up appt. in a couple wks.

Airway wise Dr.Taylor used LMA which in my case is much easier placement (does not require complete airway access with breathing tube). For whatever reason whether related to that tube placement or related to the Cath down my neck but part of my tongue is numb. It's a very weird feeling (lack of feeling)!
 Otherwise they where checking various labs yesterday which the wrong Lovenox test was put in (I didn't even know they could check that, who knew!) I know my Peds Neurosurgery Team has asked about that a few times.
The other one that came back low (significantly low I guess per the ACHD Fellow) was iron. We've battled this level on and off since the 3rd OHS back in Jan. so once the shunt surgery is done i'll go back on it again. Yay to another pill although atleast I found a brand at the health store that doesn't have the side effects that prescription or retail store bought iron typically has.

I joked to Dr.Kovach and the 1 NP (separately) yesterday, someday we'll get off more of these heart meds!?!? LOL, I can keep telling myself that anyways. =D

 I think this coming wk I just have botox injections, which I'd rescheduled from last month. I have to double check but wouldn't mind a  fairly quiet wk appts wise (ERT Infusion Monday, Botox whichever day it is, may be Tues.) but have to check that all.

                                                           Sunday School

Tomorrow (Sunday) is our Sunday School Christmas party - I'd done these or something similar with my 3rd/4th grade kids a few yrs ago but we're making candy cane ornaments with R + Wh. beads, fuzzy sticks (can't think of what the actual name of these are), ribbon and bells.  I found a story related to Christianity and what the Candy Cane means so printed that out and made small enough copies to tie 1 to each kiddos ornament with the bell and ribbon. The other ornaments in the above pic are ones my kids did last wk, our story was 'The Birth of John'.

In any case, thanks for stopping by,


Sunday, December 11, 2016

Heart Procedure/Ablation - Weds at CHW

This should be short (don't laugh to hard!) - just  a quick update with the Heart Procedure/Sinus Node Ablation procedure info.

                           Sunday School - 'Birth of John' 12/11/16 - 'Joy', 'Peace', 'Noel' (wood) ornaments

The Heart Ablation is set to take place Weds, at Children's Hospital (Milw) and planned (like the last time) as a short, 1 night stay.
Typically ablations are done as an out-patient procedure but b/c of my (significant) past history with the 3 Open Heart Surgeries and several other heart procedures (Caths, prior ablation) Dr.Kovach requested this-like the last time be a 1 night stay for monitoring after.

I am scheduled for a 12:15procedure with 10:45 arrive time. This I admittedly regret a bit not taking the early procedure time (when the Cath Lab scheduler called she offered this time given I'd be staying overnight anyways but if I wanted she would/could schedule it as a 1st case).
I NEVER thought I'd see the day I prefer the earrlllyyy, 1st case surgeries or procedures but I do! Simply b/c with 1st case schedule you get in on time, the surgery/Cath Labs aren't running behind yet and frankly not eating for that long kind of bites (how's that for a bit whiny!?! =) )
 I will certainly survive though, I can't imagine even if it ran behind the wait could be anywhere near as bad as the last surgery at UW which I was 'nothing to eat' after midnight but surgery didn't end up starting till if I remember right like 7 or 8 that night .- The surgery was originally supposed to be (again if I am remembering right) a 1:00-ish surgery time.

On a good note, I had it worked out with the same Anesthesia (airway) dr. i've used for all prior heart surgeries and procedures for her to do this 1 as well;  I emailed her to ask if the late procedure start time would affect her being able to be on my case. She emailed back earlier and said this would not so that atleast is a relief!
At UW I/Peds Neurosurgery uses a wider net of Anesthesia (airway) drs. but that came to be bc I knew Dr.Bragg knew atleast an idea what was required with airway/access/intubation so I didn't really have to worry much. She (Dr.Bragg) if needed would tell me she could make sure the right equipment was used and now there's a pool of those Anesthesia drs. that are virtually always  assigned to my case.
I am sure if at some point Dr.Taylor where to retire and if where needed i'll have to start over at CHW but for now she's available so I use her. =)

                                           Ornaments pre-Sunday School kids, decorating

In any case, will update sometime later in the wk after the procedure.
Stay warm (unless your a lucky duck and live somewhere warm, then I am jealous, lol!),


Sunday, December 4, 2016

Lumbar Shunt Catheter out of place; A revision (surgery) scheduled. Heart Procedure as well.

                                 - See below (the pics) for update -
The LP (Low back) Shunt catheter out of place/wound up and  a mess - see 2nd pic for what it should look like.
                                        Xray done right after the last surgery, shunt replaced.

    Neurosurgery Appt - F/up
I followed up with my Neurosurgeon a few days ago; I had emailed him last wk to ask if we could do a Xray of the LP Shunt mainly; I just wanted to make sure it was ok as I've suspected/wondered since I was released from UW a little over a month ago if there wasn't a problem somewhere along the shunt catheter.
I mentioned this in the email and to Dr.Iskandar at the Appt yesterday that  i'd wondered if maybe where we had the valve this time was intermittently affecting the shunt? He in turn said he did not think so but was pretty sure he did know where/what the problem was and pulled up the Xray images on the exam room computer screen. Needless the above is not normal as the catheter has backed out of it's normal placement / is  wound up (see 3rd pic where the catheter is a little wiggly but clearly in place vs the 1st pic where the catheter is looped multiple times and out of place.)

    Surgery schedule
He left it up to me when to do the shunt revision but we'll have to go back in and re-position this catheter just a matter of when. Given I have the Heart Ablation procedure in 2wks and headaches are pretty well under control (thanks to the spinal fluid leak in my low back) i've opted to wait until after the Holidays (or after Christmas anyways, the surgery is scheduled for Dec 30th). He feels the fluid (CSF) leak is being caused by the out of place catheter and there having been such a wider area that was opened in my spine in this last surgery.
I guess if nothing else in all this that extra, intermittent CSF drainage has been pretty good for headaches and helped appetite some!
 The leak and catheter issue likely occurred due to the bone removal (laminectomy) that was done and is likely  a big reason behind the catheter being worked it's way out of place (due to a much larger area is created/opened up in the spine) in the same location the shunt cathether then gets tunneled in and sits (in the above Xray if you look closely you can see the catheter originally was tunneled a fair ways up my spine within the Cerebral Spinal Fluid, likely originally sitting in the upper lumbar space or thoracic (mid-spine) space   but I think Dr.Iskandar said he didn't anchor it or maybe it was a single suture used to anchor the catheter in place I can't remember for sure but whatever he did I don't think is unusual. For whatever reason in MPS Pts we tend to have issues with devices moving and coming out of place so that + the larger opening in the bone = the catheter was able to back it's way out of the proper location and is now a curled up mess of sorts (a catheter shouldn't  have loops much less so many loops to it when within the spine apparently).

 -  Surgery date - Dec 30th
He asked and I was ok with waiting to do this LP Shunt revision till Jan (or as it turns out the surgery will indeed occur after Christmas but the day before New Years Eve. (Surgery is scheduled for Dec 30th) By doing this  Dr.Iskandar will be in town for awhile after the surgery is done in case any issues where to occur (which they better not, lol!) but was his preference

I asked if we could move the reservoir and valve which is programmable (simply means there are multiple settings on the shunt valve (a Codman Hakim valve) and these can be adjusted to allow for more or less CSF drainage. In my case we turn both valves down to the very lowest setting ='ing max amount of CSF (spinal fluid) can be drained.
It would unfortunately have been an easier surgery had I opted to keep the valve/reservoir sitting where it is now, sitting on my back within the shunt line but honestly it is SOOOOO much easier to access (again think of like a Port device, which many of us have for our IV Infusions) when the reservoir and valve sit within the shunt but on my side. vs where it is now on my low back.

I know this means they have to change positioning on the table and re-drape but having thought about it more on my drive back from the Appt., I just feel like if we're already having to do this surgery due to the catheter being out of place I want to maximize the best potential for things to go well/not be anything that could cause irritation! - As far as if or should anything worsen between now and the end of the month i'll just let Dr.Iskandar and his Team know as instructed and we'd make decisions from there. God willing nothing will though!

Heart Ablation Procedure - Dec 14th
Otherwise I have the Cardiac (Heart) Procedure already scheduled for Mid-Dec. (Dec 14th), this is just a 1 night stay though and same Anesthesia dr. i've had for all 3 Open Heart surgeries and the other 3 or 4 Heart procedures I've had. Same Heart Rhythm dr doing the procedure (Dr.Kovach so it should go smooth.

The biggest thing is really b/c it's the sinus node we're trying to slow down Dr.Kovach said this area of the heart is pretty notoriously hard to ablate or for an ablation to last long term. It's quite possible we could have a successful procedure (as we did the last time this was done, back in summer, where it lasted for about 12 hrs) but then have to repeat the procedure intermittently down the road.
My Heart Team's concern though is the mitral valve changes and is the overly fast heart rate causing these changes so we need to do something as meds aren't working (to slow rate) despite being on multiple different medication classes.  Any risks are over shadowed by increased damage to the new valve so we do what we have to.

Thankfully it is the same Anesthesia (for airway) dr. which helps. Both I and my Heart Rhythm dr. asked her if she'd be willing to do the procedure and so scheduling was coordinated with what worked for her as well. Be grateful for the small things, right?

Pre-op Appt (for shunt surgery)
Pre-Op for the Shunt revision is scheduled next Weds with my Primary Dr., in Madison. She's great about working with Cardiology where needed or with Neurosurgery if needed so should go fairly smooth.
 Blood thinner wise unless she/Cardiology suggest otherwise i'll follow the plan put together between Dr.Iskandar (Neurosurgery) and my Cardiology Team for pre-surgery and the Lovenox.
I've been on lovenox twice daily (vs oral Coumadin) since the 3rd  heart surgery last Jan., so atleast if nothing else INR isn't an issue i'll have to figure out. At some point per my Cardiologist we'll go back on the Coumadin but for now Lovenox works while we con't  to settle out the on-going shunt stuff and this upcoming heart procedure.

As a side note I have to say even though it's pretty painful with virtually any activity (being on my feet, going to the store, teaching SS, sitting I move positions constantly, etc) atleast my headaches are fairly good yet thanks to the spinal fluid (CSF) leak in my low back! This leak occurring most likely b/c of the catheter being out of place.

Anyways will update when there's something to update,
Thanks for stopping by,


PS: As a side note the brain fuzzy/memory/having a hard time figuring things out that I know well was in fact related to a shunt setting (drainage) issue last yr from a blog post I'd written that came up on my facebook 'memories'. In it I talked about the very same symptoms I have been experiencing intermittently and which where so much worse initially after the last 4 surgeries. I have to think it is a combination of at 2st the brain flush/procedure done to clear the blood clot (hemorrhage I guess)  in my brain and from the LP Shunt doing it's own thing the past few weeks/month or so while it would seem to have been working it's way out of place. Odd but hey atleast same symptoms this time where same as last yr with trying to find things/places, memory and other little things. Hard to explain but those around me even semi often would know what I was experiencing as many saw it, especially in the 1st couple wks post the last of the 4 surgeries