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


Saturday, November 12, 2016

Sinus Node Ablation rescheduled. Shunt stuff is.. well it's?

Heart Procedure (Sinus Node Ablation) rescheduled:
We have scheduled the Sinus Node Ablation, to re-try this procedure for Dec., which was my choice and request to wait till then. My Heart Team was happy schedule it out though to give healing time from this recent long in-patient stay and 3 surgeries. 4 in a 4.5wk period (I'd had surgery the wk before being re-admitted, for a small shunt revision).
It's not unusual for the Sinus Node (hearts natural pacemaker) and Sinus Node Ablation to have to be repeated more than once per my Heart Rhythm dr. and from what I'd read previously (I had kind of forgotten that but we did do this procedure in the Cath Lab at Children's a few months ago which worked for a very short while).  Given the strain this overly fast rates are putting on my heart and especially the new Mitral valve we need to get the heart rate down, so we're repeating the procedure though I can't remember if Dr.Kovach said he'd plan to try a perhaps different approach.
The older mechanical aortic valve is holding up better and looked ok (mild stenosis I believe), which is good atleast given that  valve has already been replaced shortly after it was put in the 1st time 3yrs ago).

If you wonder 'why doesn't her team try meds' and have forgotten or missed previous posts re this topic? We have, oh we have and in fact I currently take a multitude of heart meds including a relatively new (approved in the past yr) 'Happy Channel Blocker' (Ivabradine), Digoxin, and Ace Inhibitor (Lisinopril), and 2 meds to get fluid off which like the Ivabradine I take twice a day. Otherwise the blood thinner and Baby Aspirin.

This re-do ablation is is scheduled for Dec. 14th with  the same Cardiac Anesthesia (airway) dr. I've had for all prior heart surgeries and procedures in the past 3yrs.

Shunt - Neurosurgery Stuff
I requested and received copies from the recent Shunt infection/3 wk stay and then the notes from the shunt surgery done the wk prior to this long admission as well. It may sound weird but it's always a little interesting to read these notes and especially from these surgeries as I remember so very little from what was done in the surgeries. It's weird every so often odd little memories or people I met come back to me from that stay as if my mind if slooowwllly filling things in.

So what I learned really is the External drains must have clotted off 2 different times requiring 2 different replacements I guess and then a brain blood clot was found in the actual surgery when the drains where removed and the shunts put back in. Between clearing that clot in the ventricle and the issues found again in my lumbar spine (Arachnoiditis aka significant scarring. I've known we had this scarring issue for many issues and had several surgeries for it but was never entirely clear if it really was Arachnoiditis) which required a lot of scar tissue removal and a laminectomy which is really just a fancy word for spine bone removal. This was/is done to create space for the LP shunt to be put back in. This last surgery was apparently pretty tedious at 7+ hours.

Overall in the 3 or 4 wks I've been out of the hospital now the LP Shunt area is still INCREDIBLY uncomfortable and virtually any movement makes the area swell and painful/uncomfortable which really seems very much to affect that shunt functioning properly.
Brain fuzziness with thinking (hard to explain) and memory are still really off. I keep mis-placing things only to find them in spots I'd normally never put that particular item. Or driving I routinely have to use GPS on my phone even though it's places i've been in some case literally for yrs. It's unnerving and silly and frustrating and the funny (not really funny) thing is it's not all the time but often enough. I firmly believe it has to do with the LP Shunt and the swelling that's occurring and that seeming to affect the shunt functioning consistently.

The extra drainage from a CSF leak in my lumbar spine has been good though slowly seems to be going away which isn't as good bc then morning are back to being more difficult (just takes more to get going and longer to feel better). I think a lot of that CSF leak has to do with how much I do so bad on one hand b/c it makes worse the irritation and swelling and secondary symptoms but then the subsequent CSF leaking helps headaches. Irony!

Perhaps most of all and I may have written about this in the last update is I think the swelling in my lumbar (low back) area where the LP Shunt sits is caused by that shunts valve (which controls how much CSF can drain) being affected and does it intermittently stop draining as swelling and irritation builds up?  I asked the Neurosurgery Nurse this question, (could the swelling/fluid leak affect the shunt function) and she said she thought so. I definitely think it does. I HATE the valve sitting where it does and would love it to be back where it was on my side! Either place the valve sits it is still all connected, before the valve just was funneled under my skin, connected to the shunt catheters but on my side so out of irritations way when I move/function.  Out of the 2 shunts the LP Shunt we rarely had issues with the way it formerly sat.

Apparently with the last of the 3 surgeries this last time, because of the excess or irritating nature of having to clean out the blood clot my Team kept me sedated and intubated for a period of time after to prevent seizures though from what my Dad told me if I remember right I must have not had my hands tied down nor been sedated or not enough as I must have ripped out the breathing tube. I remember none of this and only remember being back on the regular Neurosurg. floor but heaven only knows if that memory is actually right either? I think it is but who knows!

Overall I just HOPE this settles, my Neurosurgeon can help me and will keep listening... He did agree to do imaging to make sure the shunts aren't in funky sitting locations or disconnected or something. I'll also see him in 1.5wks, I've really not wanted to go back in but I cant do this, this just isn't normal and I don't foresee time making the LP Shunt area better due to that shunt valves location/irritating nature.
Will update sometime soon,
Thanks for stopping by,


PS I hope everyone reading this voted. Seems a HUGE part of our Country didn't. = /

Tuesday, November 1, 2016

Cardiology (Heart) Appt and Echo, Video from PCH (AZ) Hydro Mtng-Dr.Bragg

Saw Cardiology today for follow up. If nothing else stopped at Hobby Lobby on my way home and picked up some more Sunday School crafts. Anyone who knows me, knows I love this store!

Anyways so today's Echo before the Appt with Dr.Earing shows the Mitral valve which was replaced some months ago (last Jan.) and had mild stenosis (narrowing of the newly replaced valve) shortly after has worsened to a moderate narrowing in the new valve now.
Dr.Earing does wonder/seem to think this worsening valve issues is  related to the Heart rate issues  (heart rate at Appt today was apparently 120s-130s I think he said), I am to the point heart rate itself I don't even notice much.
 Do I notice secondary symptoms? For sure but to a certain extent have learned to ignore these to, rest when needed or is just a new abnormally normal I guess?
We've been dealing with high heart rate for quite some time, seeming to be worse since this last Open Heart Surgery so where possible I just ignore it. Definitely wears me out/makes me more tired though I'd say some of that to at the moment is related to shunt stuff (is just weird, both good and sucky).

Dr.Earing did say he thinks perhaps once we repeat the Heart Cath/Ablation Study next month (to slow down heart rate essentially by 'frying' (lack of better word comes to mind) the sinus node with the intention being this in turn slows down heart rate) this should help the heart/valve issues.
I am sure I won't get this completely right but the slower a heart beats the more time there is to get good oxygen through the heart and muscle and properly oxygenate the blood which then goes back out to the body. This also allows the heart more time to rest in a sense in between each heart beat when it is slower so at my faster rates the filling times are less adequate and less properly oxygenated blood gets through the heart, brain and body with each beat while also putting more pressure/work on the heart itself. Not sure if that makes sense but makes sense to me. =)

 The ablation is scheduled for next month (Dec., my choice to wait, needed a break) with the same Anesthesia (airway) dr. whose done all 3 Heart Surgeries and the prior Heart Rhythm Studies/Cath and the prior ablation.
 Same Heart Rhythm dr. (Dr.Kovach)  who did the prior ablation and Heart Caths/Procedures as well. He works with my Cardiologist (same clinic) and they communicate so there to everyone is on the same page and involved in making decisions,.. - Atleast this will be an easier procedures than the Open Heart Surgeries and only an overnight stay.
General Article on Ablation

Otherwise the weird symptoms from this last of the numerous surgeries/procedures during the shunt infection stay some wks ago is sllooooowwwwllllyyyyyyy improving but biggest things seem to be 1. sleeping upright as much as possible (I always sleep on my couch but I toss and turn a lot so don't always end up actually sleeping upright).
2. As much as possible which is nearly impossible not irritating low back and causing swelling there as that reallllyyyyy seems to affect the LP Shunt and how it drains or doesn't drain or more aptly how it affects vision. I completely don't get it other than the valve which controls fluid drainage sits right along the LP Shunt now vs before it sat a bit separately (connected of course then to but not sitting within my spine) - is very frustrating and actually being totally honest irritating as I want to be able to function normally....

I don't really understand this other than when area gets irritated that really seems to affect my focusing and memory (this ones harder to explain but ability to quickly/easily recall things I know well or have done repeatedly.. Stinks! This improves to as the day goes on. Needless my phone GPS has gotten some use the past 2 wks and I tend to be thinking 1 thing and say something else only to quickly realize my error. At times it's a bit funny albeit seriously so dumb!
I did get new glasses script as this changed after surgery, distance this time whereas last surgery reading (can't think of right word) vision script changed.
Keep the Eye place in business perhaps?

I did make an Appt with my Neurosurgeon (Dr.Iskandar) as I'd emailed him about the symptoms and he wasn't really sure (we all figured this stuff would settle out once I was a little bit out from all the crazy procedures done especially the brain flush to clear the hematoma(s) (unsure if just 1 or was there 2, doesn't entirely matter) and the crazy irritation that caused.
I don't know, I don't think my body really knows what to do after all these procedures, these new shunt placements (the VPL Shunt valve sits at the back of my head so I avoid laying on that to, seems to create symptoms - easier said than done at times!)  and all that's going on re post infection recovery.
Phoenix Children's / Dr.Bragg / Hydrocehalus Assoc. Mtng -
This was the Hydrocephalus meeting I'd planned to attend last month in AZ, though was unable due to the unexpected/sudden hospitalization due to shunt infections and 4wk stay. - Anyways if you listen or go to 64.47 approx. in the video where Dr.Bragg begins speaking.
She speaks about Hydrocephalus, a bit about my MPS and Hydro at several pts and overall a good talk/talks.

I did go back to Sunday School this past wk, so weird but I'd been there the 1st wk and then was gone wks 2-6 but was GREAT to be back!  It ended up due to the holiday (Halloween/Reformation we had a group class so I group taught with a friend and her class (1st/2nd, I have 3rd/4th) which actually was good as my brain was so off that morning but overall really, really nice to be back!
Will update sometime soon - Thanks for stopping by,


Saturday, October 22, 2016

4 wks in-pt, 3 more surgeries - so glad to be on this side!

Sorry for no update in well over a month, it has definitely been a VERY long month + and so little of it I remember. Needless since the last update and that surgery which ironically I believe was #40 I ended up back at UWHC for another 4wks with a with a shunt infection and 3 more subsequent surgeries. This = 43 surgeries I guess now for shunts with the shunts 1st having been placed in 06' There where apparently numerous procedures done though I remember very little so i'll just share what I do.

I was admitted from clinic and though I remember very little of this the Aide told me when I was back in clinic for post op f/up's the other day that she took me from clinic over to the hospital and would ask the Neurosurgery NPs over the wks I was in-pt how I was doing and if I was still in-pt. I thought that was sweet actually as I don't really know this MA all that well. I do like her though.
With the 1st surgery of this series which was to externalize the shunts if I remember right I had an Anesthesia dr who thankfully followed what was used in the past and may even have been someone I'd had before but admittedly I don't remember a lot from that 4wks. That is a bit unnerving and almost humorous the things I do remember!

The 2nd surgery if I remember right and believe me it's all a bit of a blur after the 2nd or 3rd wk but that was to re-replace the External drains as there where apparently 2 blood clots in my brain causing the External Ventricular drain to not be working right. I've to admit I totally do not remember this surgery but think this may be the point when the Brain flush was done or perhaps it was the flush for the External Drains to clear the blood clots that occurred in my brain but that seems to be atleast partially what has knocked me for such a loop after the 2nd or 3rd surgery!
Due to complications with the blood thinners and all these surgeries there where relatively long periods I was kept off the injection blood thinner. I admittedly am not sure why that couldn't have just been reversed when needed but thankfully we seem to have avoided any clots besides the 2 that seemed to have been in my EVDs. I am still a bit confused on that part based from what Dr.Iskandar was talking about at the Appt but will get the clinic and surgery notes in due time (whenever I get around to it) so I can share these with my other pertinent Providers as well.

Apparently with the last (3rd) surgery bc the infection had been resistant to the various antibiotics (there where 2 infections but the 1st cleared within 1.5 wks of the shunts being externalized if I remember right).  The 2nd infection is the 1 that took longer to clear and more likely to recur so not only was IV Vancomycin given for that (and another antibiotic for the 1st infection, the Vanco was given under a way to prep my body from prior severe reaction and then given continuous 24hr infusion and the Vanco/Antibiotic continuous infusion ended yesterday  (Fri) this wk. Something like 5 or 6wks total on it? Yay to it finishing though! Having been able to hep lock and de-access myself Fri was like a Hallelujah moment of this all! I have left over supplies all bagged and boxed up to give to send back!

In addition to those IV antibiotics a "brain flush" was done which I can't remember if that was antibiotics or if that was some kind of other irrigant (saline?) to basically (I think but here again I don't completely remember) irritate the brain and better rid of the infection.
NOT AWESOME! That was/AWEFUL and my brain literally is still recovering from this.  Seems  getting a bit better each day and head clearer a little quicker each day but vision seriously driving me crazy in the mornings the past few wks!
 I even sleep mostly upright and yet my head (or vision really, mostly) is like on crack! Irritated so I am glad that is finally seeming to resolve and in actuality seems related to the Lumbar (low back) shunt swelling? Weird but is true.

For the last surgery which was about 1.5wks ago now the VPL Shunt was able to be placed back in the pleural space (there was some concern by the Peds General Surgeon this might not be possible due to scar tissue per Dr.Iskandar) but again per Dr.Iskandar they didn't seem to run in to to much of this (I believe this area was cleaned out by this same Peds General Surgeon that Dr.Iskandar used when Dr.Bragg put the VPL Shunt back in about a yr ago).
 The VPL Shunt again drains to pleural space though ironically this is the area that my Cardiology Team 1st suspected there was an issue when on CT Scan they saw the loculated fluid collections.  Dr.Bragg had asked Dr.Iskandar to look at this area a few months ago but he in a sense missed the issue BUT on his part I will say when in-pt unexpectedly this last time he came by and apologized and talked about how it was missed + what could have been done had he caught it earlier and what the tentative plan was now.

 I appreciated he was honest and we're all human so is what it was.

The proximal (tip, part that goes in brain) starts at the back of my head of the VPL Shunt and now runs down my back where it is tunneled further down in to my pleural (lung) space to drain. This setting is set at the bottom, lowest setting (30, drains the most).

 I admittedly hate the new VPL Shunt location as it makes laying on that part difficult/uncomfortable and seems to cause swelling when I do but maybe, hopefully with time this will go away/stop?! I am hoping as I  just want to be done with shunt stuff for awhile!

The LP Shunt placement which was done in the same surgery was also not the best, we've had to do this multiple times before with Dr.Bragg due to my anatomy/scar tissue and my underlying MPS but a laminectomy (basically removal of bone from the spine I guess is easiest way to describe it) had to be done in order to get the LP Shunt in place. This to, OUCH!
The swelling this causes, irritation and the LP Shunt valve seems to sit right in this same area of my low back vs where it used to be on my side which has seemed to very much add to the irritation and discomfort.
 I really wonder if the swelling that's going on is affecting the overall LP Shunt function or by that  I mean some of the odd symptoms as headaches themselves are fairly ok. It's the secondary symptoms..

The irritation this causes holy shiiiittteeeee!!!! Literally I can be pretty forgetful/disorganized and other things as it is but I have found ways to deal with this and use various means so for the most part I function and remain organized.. This? This holy smokes has sucked! Thankfully seeming to get better a little at a time but damn!

I have to say and I guess I am not that surprised but I AM GRATEFUL during all this infection and long 4wks in-pt, repeated surgeries, issues, etc my former Peds Neurosurgeon (Dr.Bragg) stayed in touch, talked to my now Neurosurgeon (Dr.Iskandar) repeatedly and she kept up to date on what was going on + was a support to me which means/meant a lot.
 I am beyond grateful to Dr.Bragg and her support, feedback/answering my questions has meant a lot as she really knows my history + honestly she cares. Definitely meant and means a lot given she really wouldn't have to stay part of my Team!

I followed up with Peds Neurosurgery the other day and Cardiology Nurse is working to change the. 6mo appt and Echo that was originally scheduled this past Tues (earlier this wk).
 I also talked to my Heart Rhythm dr (same ofc as Cardiology) and he was ok with our post poning the Heart Rhythm Study and Ablation (the re-do) till later in Dec. I need a break from medical procedures or surgeries so am grateful for this!

The post surgeries/procedures/infection in-pt stay f/up Neurosurgery Appt went ok. 
Stitches where removed from my back incision (the NP said there where about 20 stitches  + the stitches where removed from the EVD and former VP Shunt locations.
The rest of incisions are either disolveable sutures or other forms of closure and honestly I haven't even counted how many incisions there are but atleast 6 from 2 on my head, several on my back and abdomen and where the valve from the LP Shunt formerly on my side.
That is probably what I miss the most is the LP Shunt valve being in  a less irritable area! Crazy but I guess never surprises me after infections and what ones body can put up with! I am just glad to be 1.5-2ish wks out from surgery now!

I'm very sure there is stuff I've not remembered to write in this post from the last 4+ wks but atleast I am on this side of the shunt infections, done with the many wks of IV antibiotics and hopefully keep healing so I can get back to a more normal for me!
Thanks for stopping by,


Saturday, September 10, 2016

#40, Shunt revision.. relief at last..

Post op, #40
Sorry it's a few days our from surgery and I've not updated... I did get out after just 2 days as the surgery itself was pretty minimal (changing out part of the catheter that was abnormally scarred to an area) and I didn't have a lot of pain. If anything I've had far more discomfort since getting home but not actual incision pain.
For whatever reason despite the headaches being a lot better (not perfect, not as good as say when entire new shunt system is put  in but the headaches are better) - BUT side pain, HOLY SHIITTEEE, it's like it won't let up , hurts something fierce when I cough, or stand up or walk or try to sleep on that side (which stinks actually b/c my hips do not take well to being laid on all night on just 1 side. EEkk!!! I try to hold my side when I cough which helps a little but not enough.
My stomach is swollen.. It's like, seriously body, enough already... Couldn't we just enjoy a little time with something feeling some better!?!?!?! Is enough to drive the Pope crazy!
I've only ever experience it like this one other time and I absolutely pray this time is not like that time.. I may cry if it is!
I guess if it's still like this on Monday, basically unrelenting i'll call, though who exactly I'm not to sure. Seems likely shunt related but I really don't know given swollen stomach and headaches aren't bad... 

Why it matters to mean to someone... why it matters that Providers care, are willing to try (and listen) and work together..
I've thought a lot about this very thing this past week. I was pretttyyyyy nervous going in to Tues surgery, while I knew there was something wrong I didn't know what and I didn't know if it would be found easily. Thankfully it was or seemed to be but still with Dr.Bragg I knew what to expect and I just didn't this time.
Post op blood thinner mngmt was a bit of a mess. Despite Cardiology Team having sent over their specific recommendations that wasn't followed and the wrong (prior) recommendations where going to be implemented. After 2 days essentially of this I talked to Dr.Iskandar and asked if he would himself reach out to my Cardiology Team and talk to them himself + sort out a future surgery plan. He was agreeable to this and actually called them the same day + he sent me the recommendations typed up andasked for my opinion by Sat. morning.
After this he revised/edited a bit and was planning to send to the rest of my Core Providers and  run it past Cardiology Team before having it entered in my Med record. I am thankful he asked my opinion and listened when I asked him to reach out to the Team!  Seems positive initial steps...

Do I think we'll still have times we don't see eye to eye? Probably bc we're 2 distinctly different personalities with different views on my MPS and mngmt BUT if we both try and both listen to each other then I think this may work... I am cautiously hopeful....

Related but unrelated I will say I've always been curious with the manometer 'test' (what they use to test the shunt function in surgery)  if they are holding this in a vertical position wouldn't gravity cause the spinal fluid (CSF) to drain better even if say there where a partial issue further down? Especially if say that issue where at the distal (bottom) portion of a shunt?
I suppose another reason I often wonder this (not really related to this surgery but just in general curious question/thought) is the fall after I started seeing Dr.Bragg we where having 1 shunt issue after another so she (Dr.Bragg) had 1 of their Peds General Surgeons help her on a surgery of mine where she/they went directly to the distal (end) of the then LP Shunt and despite it looking like it flowed with the manometer test when they had a direct view they could see where the shunt catheter  was stuck/scarred to intestine or something in that area and likely having an affect on drainage without it being obvious.

Like I said I think we found the issue in this revision but makes ya wonder about that portion of shunts in a general way.

It's not about replacing..
As any rare disease family and especially those of us with ultra orphan (very) rare disorders know it's important our docs talk, are familiar with our whole history and they have the 'Team player' ability (vs I can figure this out on my own, I don't need to know what that Pts other Providers feel/think).
On fb someone commented on a post I wrote about getting discharged today and saying they felt the whole situation sounded more hopeful and where praying things do stay good between us, in working together.
I after thinking about this some commented back that while Dr.Iskandar is never going to be Dr.Bragg bc she just has a uniquely caring personality he and I will find our own normal if we keep working at it and each can adapt a little to the other (that sounds weird to say perhaps).
Really it's not even about replacing her (Dr.Bragg)   b/c she's still   a part of my Team and well some people (Providers in this case) just aren't meant to be replaced...

For him and I it's just finding what works for the 2 of us, to make our Provider/Patient relationship a good one where I trust him and he trusts me..  and we con't to make decisions together. Sharing with each other related to the issue at hand (for instance he gave me a few things to think about and ponder and decide if I was willing to try them and as I mentioned I asked of him a few things (such as talking to Cardiology) and I thanked him for con'ting to work with Dr.Bragg bc I know she knows my history inside out and she can help him and I as we look at possible options.

He did use a dressing over the incision which has been annoying and which I still wash my hair but thankfully quite a few of the staples seem to have fallen out, yay!  Otherwise I get the rest removed next Thurs. I LOVED that Dr.Bragg used no dressings the last yr or so after VP Shunt surgeries! =) Not the worse issue just more of an annoyance really. =)
   AZ Bound....
On a side note I am going out to AZ to a Hydro mtng in early Oct. - I have friends out there and Dr.Bragg is speaking at the mtng so though why not. All arragements are made so am kind of looking fwd to this. =)
Will update sometimes soon. Say a prayer this side pain either resolves on it's own or is something SIMPLE!!


                                       I chose this pic because I had prayed so often leading up to surgery that if it be God's will an answer could be found and atleast some relief  be given. I do think energy wise it may (I hope!!) help once we get the heart (rate) stuff under control but I so needed atleast some relief of these headaches to. Not perfect but better than it was!
                              Just have been thinking a lot this wk about 'Zebra docs' and how I am

                                           blessed to    have so many on my Team and a new 1 that I can wear in.

Monday, September 5, 2016

Tues (9/6) Shunt Surgery (3pm surgery)- getting things in place, new but not new Team

   Sorry I wrote this last wk but forgot to come back and publish it..
Tues surgery is scheduled for 3pm with arrival at 1pm.  If lucky surgery will be on time?
I am bummed to say the least about this - mostly just b/c the ridiculous 'no eating after midnight' no matter that actual surgery isn't until 3:00 (1:00PM arrival) which if it runs as afternoon cases most generally do at UW means 3pm will likely be several hrs later. I hope not but realistically this is more often the case. I NEVER understand why if surgery is at 7am 1 can eat till midnight but if surgery is at 3pm one is still supposed to stop eating at midningt? I'm just venting, please don't take this as my being pissed off, I really just need to vent. There is just a lot that frustrates me and writing is my outlet.
People say 'well if you get called in earlier' but the thing is I'm not in-pt so wouldn't be at UW anyways... ARGGHHH!!!! Hard candy it will be.

It's definitely been a lot of sorting and coordinating this wk for this surgery to make sure Neurosurgery had what they needed from Cardiology (Fri must have talked to/emailed with or left voice mails for my Cardiology Team and the Neurosurgery Nurses no less than 4 or so times each)! - Was crazy (!!) though I also have to say each office and the NPs I was working with where AWESOME about helping to make sure each side (Cardiology/Neurosurgery) had what they needed for Tues surgery. For that I am grateful and really appreciate both Team care and for that matter each NPs that I talked to would ask how things where going (not necessary but nice).

Definitely seemed ALOT messier this time then most other recent surgeries but by the end of today, I think (hope, lol) everything is in place.. There are a few things I wonder about but can only pray, right?... I think it will be a very, very, VERY long time till I will stop wishing Dr.Bragg where just here and am sure next wk, in pre-op, in the OR (especially as being sedated/anesthesia) and then post op in-pt i'll wish she where there bc she listened, she knew and she made things run smooth. I'm sure some think 'she's not coming back so get over it' which fair enough if they do but as any rare disease Pt who handles their own care and who has a good Team knows when we have a good Provider(s) ir is genuinely hard to lose them!
 She just knew the little things she did to make various people (Residents?) back off, or not mess with things (Cardiology meds? Stress dose steroid for surgeries?)  or make sure post op was managed well pain wise (often more about joints than is about actual incisional stuff or as much about body as it is about incisional stuff from positioning and my body just not doing to many positions to well).

I can't change it but sure doesn't mean I wont miss having her who just knew so much about making things go well AND she listened + didn't try to pretend she knew what she didn't.
Non-the-less, everything happens for a reason (I guess) and I guess I just have to trust? Is hard but trying. Sometimes easier than others.

Phoenix -
- On a side note, arrangements are made to fly out to Phoenix next mo (Oct) for 3 days (Fri-Sun) (fly in Fri, mtng Sat., fly home Sun afternoon). Not exactly the best timing with Sun School just starting and potentially missing the 1st day (hoping not) next Sun, 2 wks later we're supposed to go camping (not entirely my favorite thing, lol) and then this in early Oct. I do have the person who subs for me lined up for the late Sept and Oct dates. Playing it by ear for next wk and just hoping i'll be there! I have my lesson written up (worked on it at ERT last wk, to get it done ahead of time). So we'll see?

Anesthesia - Airway -
As far as anesthesia (airway) goes (and fully hoping it IS someone familiar with my airway!) i'll Print the Anesthesia information from Dr.Taylor from this past Heart procedure (Heart Cath / Heart Ablation) to give to the Anesthesia dr. assigned to my case. At UW thankfully it is usually someone assigned that has had me before but since she (Dr.Taylor) had increased difficulty this last time with airway (she sent what she found, and what equipment she specifically ended up using which i'll share with the Team next wk.)  I figure it makes sense to share that information with the Team that will have me, to try to make it perhaps easier for them and perhaps they can use the same things Dr.Taylor did to make intubation/airway easier. Hopefully it's someone that is amenable to just following another drs. suggestions! Most of the Anesthsia drs that have been on my Team at UW have been pretty good about this sort of thing (following what's worked easiest in the past).

  Cardiology - Blood Thinners / Surgery Plans
As far as pre/post surgery and what Cardiology told my Primary dr., the plan is to stop the Lovenox (Enoxperin) injections the night before surgery (tonight) and skip the morning dose then either this injection is to be restarted Tues night or the Neurosurgery Team will have to start a heparin drip until they are comfortable restarting the injections.
This is the case bc we have the mitral valve and aortic valves now so with mitral valve requires more careful monitoring and (if I remember right) due to higher blood flow blood has to be thinner so thus no window like there could be when I had just the aortic valve. Before we could be off the Coumadin for upwards of (I believe it was, right off hand) 3 days before and up to 5days after).
The Neurosurgery Team has these instructions and when the NP messaged me the other day to ask about getting INR checked the day before surgery (I'm guessing she hadn't yet seen these instructions/plan) I let her know this info as well so that part should be set. Lol, I am not a worrying type but have to admit I think I am going to worry about this blood thinner-post surgery aspect till it is set in stone the day of surgery and I am probably more stressing about Port access and what to do with this.
  Aldurazyme (ERT Infusion) In-Pt.
I talked to the Neurosurgery NP (she's kind of amazing, she's already talked to the Neurosurgery (D6-4) Pharmacy and gotten the Aldurazyme ordered for next wk + that was shipped earlier last wk per my my Case Manager at Genzyme. When the floor Pharmacists called Fri they confirmed they had the Aldurazyme at UW.

 Port Access - Access Team (Timing w/Holiday wknd)
The thing I am stressing a bit about is this same NP talked to the Access Team at UW who told her they can be paged the morning of surgery to come down and access the Port but they can't set up a time ahead of time. This simply (hopefully simple!) means as soon as I got to UW and checked in I'd have to have my pre-op Nurse page the Access Team to come down which would be fine in and of itself and then I'd just have to hope they could come down before and access it (the Port) atleast an hour before the actual surgery as the steroid has to be given in a specific time frame.

I talked to my Primary dr and she's going to put in an order with the Home Health Company so in case of future surgeries where it falls on a weird day I can just get the flush and heparin for the Port from home health but this time I am just going to have the Port accessed the day of surgery. Makes me a tad nervous but hoping it works out ok!?!

Stress dose Steroids:
 In any case these are the stress dose steroid instructions, I am sharing here as much so I have a simple place to access them (i'll print a copy to but in case I don't have that on hand) to look and comfirm what post op dosing is. I admittedly even after all these yrs still have to look and see the dosing schedule when there are questions. That's a little bit sad actually, probably given in just the past 5 yrs I've been through more than 45 surgeries and tmrw's surgery is #40 (overall) for the shunts!

In any case will update sometime this wk after Tues surgery. If you believe in prayer please pray there is an easy answer and an easy fix with the VP Shunt!
 Thanks for stopping by,


PS This is completely (sort of) unrelated but I feel like people judge a person for trying to stay busy and sharing what they are experiencing and almost especially for not whining constantly about what they are feeling. Why is this? Bc I share what I'm feeling but not every other word is a bitch about what isn't being done or whose not doing enough or how bad I'm feeling (I feel like I vent enough but I also try to apologize for doing this very thing, I don't want to be insufferable?!?) Yet I feel like at times people expect bc 1 goes about their life, trying to stay busy, being involved in things that 'she must not feel that bad' and why is that? How can we possibly judge what another person feels? Why do we care what another person shares? In reality shouldn't/isn't it about trying to help each other?                                          

Stress Dose Steroid Scheduling:
Due to patient's adrenal insufficiency, stress dose steroids will be required for her surgical procedure.

Day of surgery ---Hydrocortisone 100 mg IV Q 8 hours. The first dose should be one hour prior to the surgery.
If hemodynamically stable then:
POD #1---Hydrocortisone 50 mg IV BID or 50 mg PO BID
POD #2---Hydrocortisone 30 mg PO BID
POD #3---Hydrocortisone---20 mg PO in AM and 10 mg PO in PM
POD #4---Hydrocortisone---resume regular daily dosing of 10 mg in the AM and 5 mg in the PM.


Friday, August 26, 2016

Pre-op (surgery) update, few other things..

Pre-op Appt was the other day (lol this is ridiculously sad (really sad!) but less than 1.5 wks till the shunt surgery now and am waiting.. picturing the fluid draining off and a bowling ball like weight coming off my head - hoping this helps for awhile again)! My PCP asked if Dr.Iskandar planned to look at both shunts or just at the VPL Shunt which I am not actually sure. I know he showed me on MRI where if he finds an issue he thinks it will be but I have to admit I have wondered some about the LP Shunt to. I have little doubt it's working more just wonder is it working fully?

New Neurosurgeon -
Dr.Simpson asked about the new (apparently now at UW) Peds Neurosurgeon and if I planned to (request) to meet him which I said to her I didn't really know how  to go about this? I worry I'd be burning bridges if I asked to meet the new Nrsgn (Dr.Ahmed) given Dr.Iskandar seems like he's planning to keep my case? Honestly I would just like to the meet the other guy, see his personality. What I REALLY want out of a Nrsgn again is someone that picks up the phone and talks to (especially) my Cardiology Team and who doesn't kind of give the pretense they are super familiar with my disorder when I know they are not.. Or who I tell them something that's well known fact in the MPS Commnunity, that Dr.Bragg, the Nrsgn that referred me to her and drs in our Community all know but then the dr. goes against that in their own notes? I realize this may sound bad to say it like that it's just what any of us in a rare disease communing with a issue that's not all that common to our rare disorder want/wish for.. And when you had a Provider that was pretty phenomenal at communication well it's hard to not want that again1?!?

She (Dr.Simpson), suggested I ask to make an Appt with the new Nrsgn and just gauge his personality, get to know him a little, see if we get along well (does he take an interest in my case).  Per a former Patient of Dr.Bragg's (the Mom's little girl was the Patient) appts are starting to be scheduled with the new guy so I am hoping i'll meet him when in-pt for the shunt surgery? I feel weird about this whole situation as it was originally intended I'd go to the new guy once he got here but then shunt issues sort of got in the way.
 Shoot I even could work with Dr.Iskandar if he'd work with the rest of my Team and I felt a bit more like it was a partnership.. I do appreciate atleast that he's worked with Dr.Bragg (even if I sense it drives him a little crazy I'd kept in touch with her, not sure). I don't know? I just want  a Neurosurgeon again that it's a full partnership... Not one where they say 1 thing to me and another in notes/etc..
So I'm just not entirely sure yet!?!

Cardiology - Surgery (meds, blood thinners)
Cardiology - Lovenox (Enoxaperin technically) right now the plan is Dr.Simpson is talking to Cardiology to find out if they want this injection both the night before and the morning of or to skip the morning of. Given a mitral valve now technically I guess it's recommended the injection be given even the morning of procedures but she's going to find out and let me know and send the recommendations to Neurosurgery. This to... totally something Dr.Bragg would have double checked herself.. I guess it was just the comfort that she wanted to know herself? It's not at all I don't trust my Team b/c I do just knowing everyone communicated was HUGE.

Other meds i'll skip the morning dose of Lasix but take the longer acting Spironolactone to keep fluid off/prevent fluid overload. Per Cardiology and my PCP will do the same as I'd do with Dr.Bragg w surgeries and stop the Baby Aspirin 5 days prior to surgery.

I'll admit it bothers me a little but given the on-going heart issues and Dr.Iskandar being new to my Team/new to MPS that he hasn't said anything about reaching out to my Cardiology Team.
 I know this was a huge reason my Cardiologist liked working with Dr.Bragg (she frequently worked with them, asked their opinion or just kept them up to date on what she intended to do/did they have any concern from heart stand pt.
She didn't always talk to them but if there was a new med or issue she'd reach out and confirm. As any one in our MPS Community knows having docs that do this is HUGE. Dr.Earing appreciated this as well,  I think he just likes to know his Patients Providers are keeping the whole picture in mind and Team player?

Side Note....
Kind of on a side note, I was thinking about this earlier but I fully realize several of my Providers read this blog (not a bad thing I guess, perhaps unnerving bc this is where I go where I feel like I can share what I am feeling about some things but still good I guess for Providers to see inside their Patients worlds outside just a medical appt... Even if I primarily only write about medical stuff here I guess is still another perspective on a Patients life?.. But that said sometimes I am really honest about my frustrations, worries or fears here in ways I don't share elsewhere. I guess read at ones own discretion (if that's the right word to use, not sure)? ... I'm probably never going to stop writing here what I feel b/c this in a way is my sanity (writing).
Elmiron, Anti Inflammatory:
 Dr.Simpson and I talked about this med and possible use, about what it does (it is now believed there is an inflammatory component to MPS I, which makes sense really bc many of us w MPS I are originally mis-diagnosed with some form or multiple forms of arthritis and muscle related disorders). She is going to reach out to my Genetics dr. in Boston re dosing as he (Dr.Bodamer) and my Cardiologist (Dr.Earing) have been talking about starting the med for about a yr now to see if it could help some of my issues.
Elmiron crosses the BBB (gets to the brain) and has been shown if I remember right to affect GAG storage which is what we do not break down with MPS I due to the missing/deficient enzyme. Perhaps this could help our Hydrocehaplus/shunt and heart issues some. It does have mild blood thinning properties so that  part would have to be sorted given we already have issues stabilizing INR (currently just doing Lovenox (Enoxaperin) injections vs oral Coumadin for this very reason to provide a stable low blood thinning threshold. We'll see. I LOVE that Dr.Simpson is AWESOME about talking to and working w any of my Providers to sort any given issue out!

Otherwise I had 3mo botox today, which at the last Appt we had added an area more central so do shoulders, neck and mid/mid-low back area and while that newer area isn't particularly comfortable getting injected it all works so well! I really do need to go back to PT sooner than later to help overall leg/hip and the muscles that control posture but the botox does work well!

Hydro Association (1 day) Conf. - Phoenix, AZ
Lastly I both know an MPS Family that goes to the hospital Dr.Bragg is now at (they are seeing her next wk) and a few months ago met on fb another shunt Mom who was moving to AZ (not an MPS Family) and I follow the PCH/Barrow fb page (actually kind of an interesting page, they do a good job updating and interesting Hydro pieces here and there).  Anyways it turns out PCH is collaborating with the Hydrocephalus Assoc to host a 1  day Hydro Conf.

I'm not yet sure if i'll go or not but the Foundation I work for has offered to send me if I do chose to. This is in Oct so not the greatest timing what with Sunday School starts back up in a few wks (Sun after Labor Day), the shunt surgery is the 6th (not a huge recovery) but then we're also doing the Heart Ablation procedure in-pt at CHW again so likely another Sun. I'd miss of SS topped by there's another Sun. in late Sept. i'll miss.

This all said other than is EXHAUSTING traveling (but maybe i'll be in the (atleast from shunt aspect) feeling better mode so that would help? Would be fun to see friends out there and Dr.Bragg's new hospital.  I'll likely decide sometime mid-Sept so we'll see?

In any case, thanks for stopping by,



Thursday, August 18, 2016

Appt, Heart Rhythm Team.. Take 2 Heart Procedure (sinus node. heart rate)

This update shouuulllddddd be shorter (but then again I say that and usually end up not being)!

Heart Rhythm Appt. Take 2 (t-minus shunt surgery 1st)
Anyways saw Heart Rhythm doc today and (atleast I was somewhat surprised) he is recommending once we get through this upcoming shunt surgery to re-try the Heart Ablation study (with same idea to try and kill/deaden abnormal (scar?) tissue creating abnormally fast rhythms from the sinus node.

The Sinus Node is the hearts natural pacemaker so is same risks as last time (biggest being the ablation causes to slow of a heart rate which in turn require a Pacemaker) but Dr.Kovach and Dr.Earing (my Cardiologist) feel the benefits out-weight the risks and especially since (as Dr.Kovach put it) they did a similar, less intensive approach last month and it did slow heart rate for about 8hrs.

Why the initial (Ablation)  response?
I can't remember if he said why he thought we would have gotten that initial benefit (besides I jokingly said to him our heart issues are a bit like the shunt issues - we get good results then back slides again to not being effective/not working well enough, lol my body just likes to stay it's status quo I think.. Change? Why would we want to give this girl's body permanent, effective change?! Is how I imagine my body, lol if it could have conversations with itself).
He just commented, as 1 of several comments (but the only I remember) that this area they ablated last month "woke up" after about 8hrs. His hope is if he goes in this time I think he said using 3 cathethers (but not absolute on that part) and he plans to insert one of the catheters in my neck via an artery to get a different vantage point/possible treating point that might give them more efficacy. I cant say I got all of that absolutely right other than I remember the "woke up after 8hrs" (I remember lol) and his mentioning approaching this via both groin and neck this next time.

Shunt surgery 1st... Heart Ablation to follow..
He asked that once the shunt surgery is done and I am released/feel up to it I let him know and contact his Nurses so they could initiate getting the next EP (Heart Rhythm) study set and scheduled.  He asked right away about the shunt stuff and was happy it was being done though he to understands this like the other shunt surgeries likely won't be a long term fix either to our on-going shunt problems. It more he'd like to wait to do this procedure till after the shunt surgery "so you can lay more comfortably flat and isn't as miserable after for you for the procedure and post-recovery" (indirect quote).
I am game with that as last time my Anesthesia dr (as I've mentioned before is the same Anesthesia (airway) dr whose done all 3 of the Cardiac procedures and the 3 Open Heart Surgeries in the past 3yrs) opted to do sedation for the 4hr period in order I would be able to lay flat (I still am amazed by that,  I totally would have said I was awake after). Is so weird the things we don't realize!

So in any case i'll let him and his Nurses know once the shunt surgery is over and they'll work on scheduling/coordinating it. Kind of craptastic this all ends up falling just as the Sunday School year is about to start gearing back up! In any case will figure it out as necessary and if both the shunt and the heart surgery and studies help improve symptoms then bring it on I guess.

Pre-op is mid-next wk for the shunt surgery, will update sometime soon. Thanks for stopping by,