Uncertainty re Shunt plan, EP (Heart) Study details (scheduled Weds, July 1st)

I hadn't heard from Dr.Bragg's office since last Monday's Appt (she tapped the shunt, took off some fluid and had asked me to let her now how it went, which I did + she was going to talk to her Secretary after this).
The actual headaches have been pretty intense at night and in the mornings until I am up a couple hrs. This morning they where slowwwllllyyy getting better but did not finally go mostly away until I had been up, driven to Milw and at Infusion so in entirety a 4-5hr period and is still there some after that ebbing and flowing throughout the day. Yuck! My Pharmacy has been trying to get a new script for ODT Zofran from my PCP but they keep calling in the tablet form so I am thinking maybe I should ask Dr.Bragg's Nurse if she would refill it. I do have a bit left but definitely one of those scripts I do NOT want to run out of!
Anyways, I figured I'd give it time re calling about the appt f/up , I already feel like a pain in the arsh patient (I don't know I just do, even if as a patient I think we as patients have to advocate for ourselves) so I finally called late this morning (Monday). Her Secretary wasn't aware of anything, hadn't I guess talked to Dr.Bragg since last wk's appt on Mon. (atleast about my situation/case, I would imagine they must have to talk about other cases, not really sure how that works, Dr.Bragg had mentioned something about she was going to talk to Anna but maybe she didn't have a chance, who knows) so Anna looked up the clinic note and said she was sending a message to Dr.Bragg to see what she was thinking.
Anna just asked that I call her back either later this afternoon or Weds (I was at ERT till 6 and kind of got distracted doing paperwork, other calls and to be honest probably just hate calling so didn't. Like I said I do feel like a pain (not that Anna ever make ya feel like it, she's very nice).
She had said if we didn't talk today to make sure and call her Weds as today and Weds are the only days she is at the clinic this wk. Weds of course is the EP Study so I am praying I get out of that in time (takes anywhere from 3-6hrs) and not to distracted I forget to call her back and see what she finds out. I'll basically be sitting still (have to lay still, hoping they'll let me sit up with my legs straight) for another 4-6hrs after so hopefully call during that time.
She (Anna) had just said if Dr.Bragg was thinking anything valve wise w the shunt she'd get it scheduled then when we talk. I think Dr.Bragg may be out the wk after next or maybe it was just a day, not entirely sure so I don't know what we'd do schedule wise if she is thinking swap the valves. I am a little nervous about this all, not that I don't trust Dr.Bragg but when feeling better is in someone hands and you don't know the plan that is hard.
Curiously, I was looking through old files on my phone/fb and came across a comment I had posted regarding when we where first trialing the 2nd external drain to see if a 2nd shunt could help. In that I talked about how Dr.Bragg and her Partner thought equalizing the pressures in brain/spine w the 2 shunts would help - pretty much what I've been wondering given the significant stiffness and secondary pain I still have in spine/neck and have been getting intermittently in my spine since the shunt surgeries and going down to just the 1 LP Shunt in May. Doesn't mean much now obviously b/c clearly I cant do anything myself but was sort of funny to come across that post now when the very issue has been on my mind so often lately!
> Electrophysiology (Heart Rhythm) Dr.
I can't help but think about over the past couple wks, working w this EP Heart Rhythm dr. has been the COMPLETE opposite from the open Heart surgeries and although I didn't dislike that Surgeon (but also didn't know him, given out of 2 OHS I met him once) the communication w this dr vs my OHS Surgeon has been night and day, thunder and lightning different!
There where things we couldn't really know at the initial EP Appt re the Heart rhythm (Electrophysiology) Study and yet he has been really great about sharing information via email, trying to coord. with the Anesthesiologist (airway dr.) that did both previous OHS and the separate TEE + b/c she likely can't be there Weds he's taken information she shared with him re airway mngmt and shared w me he plans to share this with whoever we do have for an Anesthesia dr. on my/our case. He's also shared info re where he is in the approval process re the Ivabradine (heart failure) drug and what he thinks over all.
I spoke w the Anesthesiologist I mentioned above and she like I said spoke w Dr.Kovach and his Team to give her anesthestic recommendations and he was totally good with both my asking initially if we could try to have Dr.Taylor assigned to the case and then with her talking to him.
In other words he seems to have nailed COMMUNICATION as well - I feel like between him and Dr.Earing (my Cardiologist, both in the same clinic) I have a really great heart team for managing these issues. Albeit no patient ever wants to add yet another Specialist to their care team (in my case this makes #13 I believe) it is so nice when you do need to add someone that the 1st try turns out amiable, caring and seems to really want to make a difference.
I feel like as perhaps frustrating the 2 Open Heart Surgeries where as far as communication with my Heart Surgeon 2 yrs ago (the Surgeon was very good I just was not impressed w/the lack of communication between he and I re he never once explained things to me regarding how he felt the 1st surgery went and wasn't until my Cardiologist asked him to at the very least call me while I was still in-pt after the 2nd OHS we spoke some. I never spoke w him at any point between that 1st OHS when we knew things where failing, the 5wks in-between during weekly Cardiology f/ups and Echo's and then never the morning of the 2nd OHS or in-person after. -
Although I had already known how much I appreciated Dr.Bragg and how out of the box she always has seemed re communication, explaining her thoughts and explaining what she felt/feels issues are I REALLY figured out after that just how special she was as far as her personality as a Surgeon.
That frustrating experience all said once Dr.Earing does decide we need to replace the Mitral valve I probably would opt for the same Surgeon, bc I know he knows his stuff, I just would make sure things where better shared communication wise after!

As far as how long Dr.Kovach expects the EP Study to take he said in his experience they generally go anywhere from 3hrs to 6hrs depending on anatomy (such as any difficulty threading the catheters to the heart) and if they find an arrhythmia and if so if they can ablate it.
After the study is done, which will be done under conscious sedation then i'll have to lay still for 4-6hrs before being able to go home. Per my Cardiology Team I also am staying on the Coumadin during this so I imagine they'll be extra cautious about preventing bleeding after.
He also said he has been in contact with the company that makes/manufactures the Ivabradine re "procuring" it (lol, that made me think of dead people, not sure why I had read that!) and he hoped we'd have access to it, if we need it within a few wks after the EP Study and thought although it is very newly approved by the FDA (April) we may not need a special IRB approval from CHW but he was checking in to that. For my insurance we'll have to submit drug prior auth exceptions. Fun, fun stuff, hey? =)
Will try to update either Weds night or a little later in the wk how the procedure and anesthesia went,
Thanks for stopping by,
Erica

"I'm ok.. but i'm not ok.. and that's ok"... sometimes it's so hard; wishing for what I can't have.

This has been a really busy week, not a bad week necessarily just a busy one but admittedly there have been many moments where I've wondered more than a few times why, just why did I try this single shunt and the 1st valve we tried last month vs going back to 2.
I still know I'd have regretted it if I had not opted to try the 0 pressure valve and I guess I truly did believe if it didn't work we'd go back to the 2 shunt system which albeit imperfect atleast I felt some better and I keep on wondering, like as in wondering - my mind in a broken record what if we'd tried the former TPL Shunt in the current LP location and then also placed the VP Shunt could it/would it have been better? I think it would have been and if it where possible could kick myself, I so, so regret this and hate how it is now.
I hate the way I feel and how in many ways I feel worse then I did before. Hell I'd take the way I felt before but I really do think if we had a shunt draining in the lumbar space and then the VP Shunt we'd be in a better place. Ugh. =/

I know Dr.Bragg is really against going back to the 2 shunt system which admittedly I get (sort of) but I also so wish we could try. It amazes me, just simply amazes me how many of my other drs have said to me including my Pain Mngmt dr today, who wasn't aware of what exactly we did last month that she (and others who've seen me in the past wks) can see something is different and can see in my eyes when I don't feel good (I have no clue what it is about my eyes? Have always wondered this as more than a few Providers have said the same at times over the yrs).
Non-the-less life has continued on, both other medical stuff and other things/other plans which atleast those are fun things w friends and hanging out w the VBS kids 1 day this wk.
Life has to go on, whether I always feel like it or not is literally 1 day at a time sometimes. I have my moments of sort of pity part I guess of sorts when I am alone bc I feel so so frustrated about all of this and why can't I just feel better, why couldn't we just go back to the 2 shunt system and try it with the 1 in the LP Space but for whatever reason I guess it's not meant to be. =/ So que sera sera, right? Doesn't mean I don't want to take a drill and make a hole for CSF to leak out myself though without causing the extreme irritation to mine spine nerves that occurs w just the 1 shunt.

I know Dr.Bragg was planning to talk to her Secretary the other day re coming up w a plan so I'm not sure if she has but will call I guess about that tmrw (Fri) if her Secretary is there (I can never remember what days she is) or otherwise on Mon.
I know my Parents want my Sister and I and the kids to go camping w them for a night in a couple wks (actually I hate camping) - they have their tow behind and my Sister and I generally do tents and I sleep on a lay out chair in the tent that sits up or in the back of the truck.
Honestly sort of dreading it w these headaches which are made even worse at night but I know the cardiac cath-EP (Heart Rhythm study and ablation) is scheduled for next Weds and given I don't know a plan yet it's not like if we did do a shunt valve swap should that be what Dr.Bragg decides is going to happen next wk.
Ugh!!! I just freaking want to feel good!!! I think I could sort of like the camping thing given it's just a night if I knew I wouldn't pee 10,000x's throughout the night due to the shunt (have no idea what it's about but no doubt in my mind it's a shunt thing) and wouldn't have the headaches/symptoms!
Just cross your fingers I guess no matter Dr.Bragg was or is able to come up w an idea to try.. And either way i'll survive for the kids sake sleeping in a tent overnight for a night I guess. I am totally not an outdoorsy sleeping kind of person!
In any case, will update once I know any details from Dr.Bragg, if you would say a prayer she is able to come up w a better plan and God's wisdom may be with her as she tries to figure something out. Please don't think this update is anything against Dr.Bragg, it is not, I truly like her and appreciate her and trust her I just simply feel frustrated and being perfectly honest here wish we could turn back and go to before. Now it just feels like what we had before the 2 shunts, like 2 or 3 steps backward vs. progress.

If you would say a prayer for next weeks Cardiac-Electrophysiology Study that my Team may have success, whether that be just mapping the heart or whether that be mapping the heart, finding an arrhythmia and being able to potentially ablate it. The study is being done under a form of anesthesia, conscious sedation and several catheters will be threaded up my legs through the groin to reach the heart to map the electrical (rhythm) system + if needed electricity can be given through to the catheters to rid the heart of abnormal signal pathways if any are found.
This humid weather is really difficult on breathing so any progress we could make towards improving breathing (and thus stalling mitral valve replacement) ;) either through the EP Study or if we have to the new med would be great!
In any case will update soon,
Thanks so much for stopping by,
Erica

Cardiology, EP (Heart) Cath Study Scheduled, Shunt Appt., etc.

I apologize I wrote part of this after last weeks appt meaning to at the very least finish it after yesterday's fup appt with Dr.Bragg and I didn't so I will try to keep it semi short (don't fall laughing). ;0) I'll update the Cardiology EP Study info and then update Dr.Bragg's stuff from Monday below the update from last Thurs.

So the EP Study Procedure (heart catheterization and ablation, a test where catheters are threaded up the legs through the groin to the heart) is scheduled for next Weds, July 1st at 7:30 with a 6:00 arrival.
The Anesthesiologist who has done all previous cardiac surgeries (2) and the separate TEE (Trans Echo, basically a probe inserted down the throat under sedation) at CHW is out of town next wk (she was sweet enough to message me after talking to my EP dr and the Nurses to give them a heads up on what she would recommend as far as an anesthesia approach).
Anyways bc she is gone i'll meet someone else which I guess isn't a bad thing BUT doesn't make it any less slight nerve wracking having someone unfamiliar with airway! Trust, just gotta trust, right?
We are opting to do this Heart catheter via conscious sedation which I am fine w for 2 reasons (my choice and the suspected Arrhythmia per my EP dr. has the best chance of being captured when a pt is at minimum semi-conscious). Apparently per a CHD fb forum I am on many of the women there think I am nuts for doing a possible ablation under this form of anesthesia but I'm not sure how it could be that bad (knock on wood)?
In any case I am unsure how long it is expected it would/could take but the "recovery" (laying still - hope they realize I aint gonna be layin me no flat!) is 4-6hrs after the procedure and then you are able to go home. The procedure normally done w 1 EP dr is scheduled w my dr and his Partner in case they do have to do an ablation, apparently it takes more hands or more involved which atleast if needed it will all be done in 1 sitting.
Following this EP Study/probable (potential) ablation Dr.Kovach and Dr.Earing feel we'll likely start the new heart failure med (Evabradine) though they expect even though they'll have the data from the EP Study (apparently required in order to use this med) they may have to get IRB approval from CHW and request it from the Drug manufacturer (perhaps b/c it is so new and sub-specialized?).. It also will have to be appealed via a prior auth to my insurance as it is not yet on their formulary but in that case my Cardiologist apparently knows the Dean Medical Director well and my Insur. Nurse Case Manager didn't think we'd have an issue. So we'll see? Perhaps we'll get lucky enough and the ablation alone would work but neither seem to be to optimistic about that (either way we have to do the EP Study to get information for the med which works to modify the sinus node and how that works I guess).
No matter any of this it seems Dr.Earing thinks we'll have to replace the mitral valve sooner than later as it is causing (adding to?) decreased oxygen levels w minimal exercise (per a 6MWT they did), increased pulmonary venous pressures and the mitral valve keeps worsening (faster than he had hoped) which he suspects all adds to our symptom issues to.

Update on Monday's and Thurs Appts, Neurosurgery : Today's Appt (Thurs last wk) with Dr.Bragg went well, I won't lie I was really nervous and really was not feeling great on and off throughout the day so I just was hoping it would go well, which it seemed to.
Dr.Bragg opted to turn down the Codman valve setting to the final lowest level (30 or what equates to a 3 in CSF numbers I guess) - I am glad she did though also admittedly was nervous would it further irritate the spine nerves/stiffness that has been so prevalent since a few wks ago? So far I can tell a small difference w the drainage (I can always tell within a 1/2hr-to an hr period generally) though I think if we had that drainage + some it would be much better.
As far as what she is thinking she brought back up potentially replacing the current Codman Hakim valve with the .5 mininav valve which has a little more resistance (to drainage) than the 0 pressure valve we had but still would she feels allow more CSF to drain then the current valve we have. A bonus to that valve I suppose it is not programmable so would be no issue with imaging, etc. I am obviously a little concerned it would further exacerbate the really badly irritated spine nerves but at the same time I feel like we need to do something.. I really wish we had the 2 shunts draining from 2 locations and can't help but think since there appears to be better drainage from the lumbar space than was the thoracic space it together w a VP Shunt would just work overall better. =/?
We talked about the cardiac stuff, the upcoming EP Study which Dr.Kovach's Team is scheduling (likely for July) but he 1st wanted a chance to review the Holter (24hr continuous EKG) monitor and see if there was any clear evidence on it for an arrhythmia - he suspects based off a quick EKG done at his Appt last wk that there may be an ectopic atrial tachycardia but isn't for certain. Should he find clear evidence on the Holter he said he would be scheduling the EP Study for a longer than is typical period so he and a 2nd pair of hands could ablate the arrhythmia (problem area) while they where doing the catheter study. Otherwise I am uncertain offhand how long a typical EP (heart rhythm) catheter study takes. I had one 11 or so yrs ago but details are pretty fuzzy!
Anyways Dr.Bragg just asked that I f/up with her next wk apologizing for making me come back to see her again. While not ideal to have to drive there again I was atleast on a slightly positive spin able to re-arrange my weekly ERT Infusion and will do that on Tues now + have another Appt on Thurs. I can't remember for sure but I think she wanted this so we could eval how the change in setting went which it did help and albeit far from perfect atleast I wasn't throwing up at night and that is/was an improvement I was happy to take!
She did tap the LP Shunt at Monday's Appt and take off if I remembering around 9ml of fluid which helped a great deal for the most part of Monday. I had hoped it would help me get through 1 night of not waking up a zillion times having to go to the bathroom but that was not to be the case. Instead of (no lie) 6-7-8 times I think I only got up around 4-5 times Monday night. I don't know why pressure at night seems affect need to get up and pee so much but others I know w MPS have experienced the same when having issues. It seems to be a autonomic thing.
As far as a plan she is going to figure a plan out and work with her Secretary to make it happen. Stay tuned on that.. I really, really hope whatever we decide to do helps without causing the often severe spine-nerve irritation I get both when we drain more and at times like yesterday when we did tap the shunt and drew off a semi-larger amount.
Dean Insurance Out of Network (Provider) In-Network exceptions: On a good side note all but 1 of the 2015-16 DHP (Insur.) out of network, in-network prior auths for July 1 are back and approved including 1 or 2 unexpected ones (that I otherwise use my secondary insur. for). - There are 2 my Case Manager called me about today and was going to go back to the Med Director and have amended to include further wording/testing but otherwise it is good! Always nice when this is done albeit Marge and my PMD do the majority of the effort and I just weight in. =)
In any case i'll update again when there's something to update about,
Thanks for stopping by
Erica

"Wash, rinse, repeat" - Cardiology Appt; alot going on, Mitral valve changes

If you wonder what "wash, rinse, repeat" refers to, this would be in regard to today's Cardiology Appt and Echo after having just been in the same clinic last week for the Heart Rhythm (EP) dr. appt. - A friend of mine, not MPS but has her own health issues gave this analogy for dealing w what is going on and seemed quite fitting! Basically get up and go on no matter.

Today's Cardiology Appt was both a f/up from the new Cardizem dose the beginning of May as well as the 6mo Echo. - Needless to say for whatever reason it's beginning to seem like I get not the beat cardiac news after every shunt infection I deal with? Granted it was 1.5yrs ago a few short wks after I'd gotten out of the hospital from that shunt infection I had to see Cardiology for a normal f/up and Echo then to and that was when I found out the Aortic valve was then in the severe range and would need to be replaced.
Can you guess what today's Echo showed? Why yes, of course it showed the Mitral valve had worsened further and is now in the severe range to. Because we are trying to sort the arrhythmia issues and b/c it isn't a clear picture (is it ever?) Dr.Earing is unsure if we should proceed to replacing the mitral valve (would be a 2nd mechanical valve, requiring different approach to thinning blood before surgeries/procedures and the 3rd open surgery for heart). - Right now I can safely be off Coumadin for up to 3-5 days before a surgery/procedure but with a 2nd valve in place we would have to do self injections leading up to any invasive procedures/surgery or in some cases Heparin or something similar has to be given till right before I guess - I am not entirely clear on this other than Dr.Earing is really concerned and not happy this has worsened/another heart surgery is in our (likely) near future.

In the mean time bc the picture is so unclear ie should we proceed to mitral valve replacement (in other pts he would recommend it at this point but b/c my history is so complicated and he feels this surgery will be rougher than the other 2 (as far as actual surgery I guess) due to so much scar tissue from the other 2 heart surgeries in addition to my MPS issues in general and even higher risk he would like to sort things 1 by 1. I agree w this.
They'll get the EP Study scheduled which he was pretty unhappy this hadn't been done already last wk after I was seen in the other clinic but he wants it done as soon as possible. He just said he'd talk to my other dr. today and would make sure they called w arrangements.
The biggest concern right now re why they want to do the EP-Cath Study is based off an EKG done in the clinic last wk during the Appt w the Heart Rhythm dr. they felt there was strong suspicion of an ectopic atrial tachycardia which is a type of arrhythmia. Apparently if it is this and they map it during the catheter study (catheter usually goes up the groin) they can ablate it/rid the heart of this abnormal rhythm.
Should that be unsuccessful they will move to the new heart failure med - evabradine which as I wrote last wk is for a different type of heart rhythm/heart failure issue. This is a brand new med so will have to approved by my Insur., though I have no doubt between Dr.Earing, myself, the EP dr and with the EP Study results we could get it approved w my Nurse-Case Managers help. I do have to laugh though, just about every Appt Dr.E asks how I manage to get him covered/approved to see (he and 4 other drs are out of network exceptions) which on my end hasn't been to difficult.
After those 2 things, should the EP Study be negative and the med not control symptoms well enough Dr.E said he would unfortunately move next to mitral valve replacement as all heart wise points to this being a big probable source of issue. I've a feeling this would not occur till later in Fall or Winter but could be wrong. He just said he'd want a f/up appt after the EP Study and would at very minimum want an Echo in 6mo if we hadn't done anything beyond the Study and med.
Given all this, he brought up having talked to my Metabolic Genetics dr in Boston and that he agreed we could try the Elmiron though he felt we should perhaps try to sort some of these other things first. As he put it "there are a lot of things going on with you and I worry about complicating the picture even more".
He did talk about how he and Dr.Bodamer talked about my having the moderate phenotypes for MPS I (mutations, somewhat of a predictor where a person w MPS I will fall on the spectrum) but my heart issues are a lot worse than the typical MPS I pt and on the other hand I don't have some of the more typical features. Like Dr.Bodamer, Dr.E said he wondered if there could be a secondary underlying connective tissue disorder which would/could possibly explain some of my features and why I don't have some of the more classic-moderate MPS I features.
Dr.Bodamer is moving ahead w a study to map some MPS I pts genotype to see if there are other underlying issues and had asked me if I would be willing to do this. It requires just blood work and no cost to myself or my insur. I guess I see no reason why not to do it, especially if it could potentially help us long term. That is all being set up and Dr.Bodamer's Genetic Counselor is calling me to get this squared away and sorted out.
Related to the mitral valve concern and the gradients worsening Dr.Earing had done a relatively simple 6mwt today and he said this not only showed lung functioning worsening a bit further - enough to be even more of a concern to him given the heart issues I guess. He also said during the actual walk test my oxygen saturations where dropping which was a concern to him although likely explains my symptoms at times when I am moving about. Similar SOB symptoms do occur even when doing basically nothing though so who knows!
Although the overall appt wasn't great given these new changes to the mitral valve, the very likely need for a mitral valve replacement (MVR) in the not as distant future and and the fact that MPS just keeps marching on I did have to laugh a few times.
The ACHD Fellow came in before "The boss" (Dr.E) and as he was finishing up he says to me "I hear your famous" to which I taken slightly aback said "Lol, why do you say that?"
He says to me "I hear you do all kinds of advocacy and awareness and are famous, maybe I should get your signature!?".
Hahah, made for a good laugh, I am definitely not famous though happy my Cardiac Team was apparently filling him on MPS and the enzyme drug I receive. =)
Dr.Earing, the Fellow and I where talking about this latest shunt surgeries as Dr.E asked if I had any upcoming procedures or surgeries he should worry about? I don't and just said I think we have some figuring out to do w this current shunt given symptoms are worse now then prior to these recent shunt surgeries; how when I am laying down and get somewhat better the longer I am up but at the same time my nerves in my back still seem so overly irritated.
Anyways as we where talking about this he brought up how we'd have to manage the Coumadin before the upcoming EP Study but "we're old hats at this now so it's just a matter of figuring out exact timing.". - He then asked about the actual recent shunt surgeries as I had said once Dr.Bragg talked to him/his Team the Coumadin and injections schedule went really well and we didn't seem to have any issues.
Somehow we got to talking about all of the recent surgeries and issues and he said something like 'Neurosurgeons have weird personalities, they're not known for being patient friendly' (or something like that) but then he made the comment he likes Dr.Bragg and thinks she's one of the few that is Patient friendly (down to the earth) and easy to work with. For Dr.E to say he likes anyone else his pts see is a pretty high compliment - he's pretty skeptical of most other drs. including some that I consider very good on my team.

I guess at this point it's wait for the EP Study date and then we'll go from there w all of this. I have to admit it worries me now that these issues are worse that i'll be stuck w these headaches and Dr.Bragg won't know what to do. I so badly feel we need that 2nd shunt and so many around me have commented but how do you relay that to someone you see for 10 or 15mins and when you likely look normal? =/ Agh if only prior decisions could be changed! It makes me realize although imperfect it sure was a whole helluva lot better prior to this recent infection and shunt surgeries.
I see Dr.Bragg Thurs and am praying so hard we can sort something out. Having not been sleeping great, throwing up some nights (but that was also the case in-pt, I just took valium fairly often at night) and just feeling not that great I pray we can sort something out and she'll see what others do. I trust her but that doesn't mean I'm not nervous about the appt.
Ironically I was thinking about the last time we removed an EVD (external drain) and this time and how symptoms after each where exactly mirrored (extreme nausea, throwing up and severe headache though the difference this time I recognized it soon after it began after the drain removal and the Nurses had given me pretty high doses of valium to control the symptoms and I slept for essentially the next 12-24hs sleeping off the most extreme.
The last time this same thing occurred 1.5yrs ago I had went home and was sick there for hrs before finally going to the local ER where they started valium as I was transferred back to UW. I feel like the drs witnessing the symptoms that time vs this time I was so drugged up and to it was Dr.Bragg the 1st time and not this time.
I just don't know but I do wish I felt better! Prayers for help and guidance always appreciated,
Thanks for stopping by,
Erica

New Heart Rhythm (EP) dr., Appt and Plan..

Today was the Appt with the EP(Heart rhythm) dr. at CHW which is also where my Cardiologist is. Seemed to go well, his NP was very nice and this dr seemed well versed on my background history, everything from the prior heart surgeries, underlying MPS and the shunt issues, etc.
Before I get to the nitty gritty I have to share this funny comment from his NP, we where talking about the various meds and especially the Lasix and Spironolactone (to get excess fluid off). She says to me after we discussed the rather large doses "How do you get any sleep at night?" Lol, I have friends who tease me about sleeping late but in all honestly much of it is b/c of the meds make me get up often 4-5-6 times a night (the other night I counted no less than 7 times I woke up) and is even worse when having shunt issues for whatever reason.
So as far as the EP dr he had talked to my Cardiologist who filled him in on my prior history, 2 OHS and our issues getting on top of the overly fast heart rate despite the many, various classes of cardiac meds I take and we have formerly tried including specifically at this moment a high dose calcium channel blocker and digoxin which works on the heart muscle itself.
He mentioned in the EKG just done there was an area that made him slightly suspicious the fast heart rate may be coming from an ectopic atrial tachycardic (early beats from the atrium of the heart). Otherwise he and my Cardiologist feel it is caused secondary to the 2 prior open heart surgeries and is an inappropriate sinus tachycardia "this name b/c we aren't very creative with names and it comes from the sinus node of the heart".
In any case he laid out a multi-step plan to figure this out and try to get on top of it. He ordered a 24hr holter monitor which is essentially for anyone who doesn't know a 1 day long continuous EKG monitor so he can see if he can find where the error is occurring from.
Although this will be done Fri afternoon i'll just return it when there Tues since I f/up with my Cardiologist Tues for an Echo and 1mo med dose change. They'll download the results and review.
He also is scheduling an EP Study which is essentially a study where he goes in via a catheter in the leg (groin) and threads this up to the heart where they can then map the various heart rhythms to see if they can find any abnormal areas such as ectopic atrial tachycardias which I believe he said could be ablated (basically gotten rid of w electricity I think via the catheter).. They are planning to coord this study w the Anesthesia (airway) dr. who did both my OHS (heart surgeries) and he gave me the option to do it w conscious sedation or to be completely asleep (full airway support). I've opted for the conscious sedation.
If the EP Study comes back normal he said he planned to begin working on off label approval right away (this wk apparently) for the new heart failure med 'Evabradine', a very newly FDA approved drug for innapropriate sinus tachycardia/heart failure. In my case he said I both do and I don't meet the qualifications for it's market approval given I have clear heart failure but he feels he'll have an easier time getting it approved via a special IRB at CHW w this approval from the drug people. I don't quite understand that but if he does that's what counts.. I know he also asked about my insur. and said it can be costly to which I commented I had checked w my Insur. Nurse Case Man. to see if it was on formulary and she said they'd just have to write a special prior auth for approval. I also commented that DHP pays for the weekly enzyme drug to which his eyes got a little bigger and he said "oh we shouldn't have any problem than!". LOL. I know my Cardiologist apparently knows the Medical Director at DHP so he'll get approved what he needs/they want and we've not had medication problems before.
As far as everything getting completed and starting the med if we end up w that Dr.Kovach felt this could be completed in a months time and he wouldn't plan to start it till after any EP Study anyways which he said he planned to do "in the next few weeks". He mentioned July was pretty wide open for this type of study at CHW. They did have the option to do the study at either CHW or next door at the Adult hospital but he prefers it be at CHW which I guess is ok given the Anesthesia dr is there.
He said to me "hopefully you can get the shunt issues sorted out in the next few wks, I am not sure what made him make that comment other than the NP was taken aback I'd just gotten out of the hospital Sun, had 3 surgeries and was still at the Appt. (my philosophy is I certaintly didn't really feel like going BUT I also could chose to either sit at home and feel miserable or I can go on with life, try to distract myself and try to not think of how bad I feel much of that time. Distraction works better I think...
As far as this new med if we started it I asked if helping the heart to beat better and thus oxygenate better could help the mitral valve, slow progression of damage and he said it likely wouldnt have a lot of affect on valve disease progression. "The mitral valve's gonna do what the mitral valves gonna do". It's possible it could help slow progression but he didn't seem optimistic about that..

In any case lots going on as usual between this and other med stuff, the shunt stuff, etc. and I see my Neurosurgeon Monday so prayers are always, always, always appreciated!
Thanks so much,
Erica

Discharged; 4.5 wks in-patient, not feeeling the best, could always be worse?

Hi All, Sorry for no update, generally fb is easier though I try to do this so other families or individuals dealing w MPS or any of the issues have a place to read about experiences and to honestly it's a way I can come back and see what happened at whatever point I am trying to remember.
I did get discharged on Sat., Dr.Bragg wasn't rounding at all that wknd and I figured I could feel just as bad at home as I could at the hospital. The Nurse I had said to me when we hugged before I got in my Dad's truck "I doubt this will be goodbye for long but take care of yourself" which was both funny and a little sad, I think she could tell just how bad I felt. Hopefully i'll start to feel better and if not I cant even begin to say how hard I have been praying my body will heal and/or Dr.Bragg will have an idea how to help when I see her at fup.
I am hopeful maybe Dr.Bragg will be able to help me sort this all; I admittedly felt a little like I did something to make her upset at me though I don't know what that would be and I really, truly hope it is not the case. My Dad and I where talking some on the way home and he said he wondered if Dr.Bragg is afraid of me/of disappointing me when I brought this concern up? I hope not, I almost cried when he said that although I know he was only being honest.
My Parents both really like Dr.Bragg and appreciate her not having given up over the years. My Mom tends to be pretty skeptical of drs in general and definitely not their biggest fan but Dr.Bragg won her over long ago, I think simply bc it has been so obvious she doesn't just do her job for the money's sake and she's often went out of her way to try and help + she's always tried to look at the bigger picture in my case. BR> My Parents have been so grateful to her for not giving up on me, for continuing to try to help when they know it must be hard for her at times and for her being in a way like more of family than some of my brothers are as far as how much she tries to help, reaches out and in that she seems to care.
I know at times I've vented about the shunt situation on my fb although never personally about her (I don't think other than maybe I was scared she would run out of ideas, never really realizing that might be like saying I didn't trust her?) My Parents both commented they wonder if this could be read as my being upset with her? I hope not but not sure how to make that transparent?
I am not sure just feel like something changed in the last 1.5wks in-pt. I hope not. =/ You may ask why would I care what a Provider of mine thinks but in this case she has done so much for me to help me over the paat 4 yrs and been one of the drs who has always seemed to trust me, to trust what I said. She's worked well with other of my Providers and she's never given up shunt wise trying to help. In all this I trust her just a little nervous if I have done something to upset her.. On a semi un-related note my Mom told me that at 1 point Dr.Bragg told her the average person makes something like a shot glass full of CSF a day and I make more like a coke can worth in day. I am not sure but hope i'll remember to ask Dr.Bragg if that is part of why we have so much difficult w shunts? I know to Dr.Bragg has mentioned about the overly thickened skull bone factoring in. That was just an interesting piece of information I thought!?
Things have been pretty difficult w the seeming spine nerve irritation, when this occurs, worst as I am sitting my neck gets so, so stiff it hurts to even look down or sideways. I am guessing this is bc of the seeming severe over drainage after we placed the 0 pressure valve and then my body wasn't to happy to be fiddled with in a 3rd surgery 1wk ago when this valve was taken out and the programmable valve was put in. I kind of can't help but wonder if we should have or maybe still should turn the current valve up to a higher setting to give the nerves time to relax. I do think I'd get pressure headaches but i'm not sure otherwise how my upper back and neck are going to heal? I don't really know. I think if the nerve irritation healed we could then slowly turn the setting back down as I think lower settings are still needed. I seem to get worst symptoms when I lay down (though I try to never lay flat) and back symptoms for whatever reason as the worst when I am sitting, better when I am standing.

Otherwise I see the Heart rhythm doc this week, he is the dr my Cardiologist wanted me to see regarding potentially starting a new med that is on the market for heart failure as his hope is it will help slow heart rate and thus help slow mitral valve worsening.
I also see a new doc for botox and hoping maybe that could help, hopefully won't make it worse though it shouldn't?
Next wk I see Dr.Earing (Cardiologist) just for 1mo fup from the upped dosage of Cardizem and 6mo Echo.
I am hoping and praying I start to feel better by then!?!?!
Thanks for stopping by,
Erica

#38 (I think) shunt revision, valve changed (4 wks in-pt)

1Hi All, Surgery was Tues to remove the '0' pressure mininav valve and to replace it with the Codman Hakim programmable valve set at '40'. I had thought we where going w a .5 mininav valve as that was what Dr.Bragg had suggested last I knew. Perhaps she changed her mind along w her Partner, not really sure.
The Anesthesiologist was very good, she gave 0 arguments about the pre-surgery steroid dosing, she agreed it should be given atleast 30mins before whereas the prior 2 Anesthesia drs all argued basically with me that it was fine to give it as they where giving other sedation. I give HUGE credit and kudos to her (Dr.Rusy, I think she may have been on my case before, not sure). The Anesthesia Assistant (Resident I think) was on my case last wk to and I liked her as well. My Neuro Endocrine dr emphasizes at every appt how important it is to make sure and be persistent no matter how argumentative some Anesthesia drs get as she has commented numerous times she has some other pts w the same cortisol issue who often have to really be pushy and advocate for themselves for the pre-surgery cortisol dosing time.
As far as surgery I think it was relatively short, they just opened up 2 incisions (side and stomach) to take out the mininav valve and replace it with a valve with more resistance. There has been quite a bit of swelling on the side incision but not really painful other than when I move wrong or try to lay on that side.
I also thankfully was transferred back to the regular Neuro fl Tues night. After the EVD was removed and I was feeling better I had been transferred to the Trauma floor, this wasn't all bad as I knew some of the Nurses who formerly worked on Neuro and had 1 of them + the other that was working stopped by several times. I was definitely glad to be back on Neuro though.
As far as the severe low pressure headaches I was getting prior to Tues surgery those symptoms where pretty much resolved by last night and overnight/this morning I was having the opposite, having higher pressure headaches. I doubt pressures where severely high but I'd be willing to bet the #'s would have been higher.
Nausea meds are definitely great during these periods! During the post-surgery last wk the Nurses used a newer orally disintegrating nausea med I've thought about asking if I could try as Zofran/Ondansetron doesn't always work well.
Dr.Bragg returns Fri so hopefully i'll get a chance to talk to her about all of this. It's kind of disappointing to know i'll likely be going home not feeling great and absolutely certain if we had done the 2nd shunt it would have been better. For whatever reason when we start from scratch with complete brand new shunt systems I tend to feel a TON better.
I am set to receive my enzyme replacement (Aldurazyme) infusion on Fri as well as I may get released so I am hoping to have some of these varying symptoms ironed out but probably not holding my breathe. Today I ended up taking a nap, I didn't think i'd fall asleep but I did for several hours after i'd been up.
Thanks for stopping by, I hope next time I update whenever that is that my body is settled out!
Erica

Surgery to replace shunts 5/26, revision 6/2 (over draining) 3.5wks in-pt.

Last weeks surgery went ok, definitely has been hard since then though w very low pressure headaches. Dr.Bragg is out this past Fri to this coming Fri unexpectedly (=/) but her Partner is managing my care (we've gotten along reasonably well, this morning he was saying some things about my care/this next surgery (to place a higher pressure valve) and questioning why I needed a shunt ("b/c people with storage disorders don't need shunts" BUT I corrected this false notion and asked that he just follow what Dr.Bragg has done in the past as she knows my care and what works reasonably well. This questioning was after he even realized I had an underlying dx, as he said to me "what caused your Hydrocephalus in the 1st place?"
Fast fwd past a really, really difficult past wk spent mostly in the Neuro-ICU due to the External Drain (EVD/ICP monitor) and then after the EVD broke further down the line to this being removed Sat., my spending the day sleeping due to being sick and transfer over to the Trauma/TLC unit till a bed opens on regular Neuro-fl (D6-4).
It looks like I should get to transfer tonight so the past 2 days have been spent w very nice Nurses here including some of my former Nurses from D6-4 who now work on this unit.
Dr.Bragg's Partner stopped by this morning and their Resident just now, he is going to go w the next highest draining shunt in tomorrow's revision surgery (.5 Mininav valve) so hopefully this still allows enough drainage BUT stops the severe neck and mid-back pain I have been getting from overdraining.
I think my head doesn't necessarily mind the over draining to much but my neck and mid-spine feel like they are in agony when I am not icing it.
Of anything about this in-pt stay, infection, etc., I admittedly regret not having went back to the 2 shunts we had before. While I likely would have regretted not trying this current 0 pressure valve to see if I could tolerate it AND it was purely my decision to try it (I could have said no) I do think when we had 2 shunts it divided up the CSF draining and I just felt better body/muscle/draining wise. I am hoping this slightly higher pressure valve works but do kind of wish there was the former 2 shunts diving up the pressure flow and thus less spine irritation.
I also admittedly worry w this new valve we're putting in tomorrow will it not be enough drainage? Given I didn't have to significant of low pressure headaches and these where very intermittent I do think my body would have adapted. I do not think I could live with this to low of pressure in my spine and thus significant nerve irritation though. Hopefully if under drainage becomes an issue Dr.Bragg will help me try to sort it. I do realize why worry about something that isn't even yet an issue BUT I am a planner, a details person and it is a very real possibility.
I know this isn't a great update but will try to do better either after surgery or when I get home - believe it or not it has been 3.5wks in-pt now! =/ That is really crazy, it's definitely been eventful at times, frustrating at others.
Erica