Ivabradine / Heart Rythm F/up Appt, Shunt stuff

Zio Patch (24hr Heart rate monitor)

I followed up with my Heart Rhythm dr. today at CHW. Mostly it was just to do an EKG 1wk after starting the Ivabradine for Heart rate and make sure there where so far no changes in QT interval (a potentially problematic issue).
I don't think he suspects this will be an issue but no-less is something that can become problematic with Ivabradine in some pts. (I have no history of abnormal arrhythmias minus the to fast of heart rate and on occasion Ectopic Atrial Arrhythmia which he suspects based off the EP Study/Cardiac Cath was more or less a non-problem.

As far as heart rate he said he had been hopeful we'd see some results by today but he also stated typically in pts. in my situation/type issues (Heart Failure, High Heart rate) based off studies in Europe they generally seen improvements/results there if there are going to be at the 1mo. time frame.
My heart rate today was 84-90ish which may not seem high to many but is concerning to them given I am on max dose Digoxin, I am on the 2 max dose meds to get fluid off and I now take this HF/Heart rate med which should conceivably work to lower heart rate but have not on their own. Additionally these rates consistently which I am or higher puts a strain on an already strained heart.
Should heart rate not change significantly by the 1mo mark he commented he would adjust the dose to double the current to take 10mgs 2x's a day. This he said is not often used but I am handling it well and with close f/up we're willing to try it if necessary if it improved heart symptoms AND bought us time to a 3rd OHS (Heart surgery). 10mgs times 2x's a day is the highest he said that is used in a small subset of patients and he would expect would help us. Hopefully we won't have to go there! =)

In order to get an idea what my heart rate is doing over a 24hr period he has me wearing a newer type of Holter monitor, this one has no wires (whohoo!) and is completely on the skin/nothing to attach elsewhere and try not to dislodge. It works a little differently than a Holter as far as what it records apparently.
I am hopeful this one won't cause the extreme skin irritation (but everything does so this to probably will) though in that case I guess just look at the flip side it's only 24hrs?
Because of my Port-A-Cath being on the L side the Tech did have to secure it mid-chest, directly over my OHS scar which admittedly wasn't all that comfortable when placing BUT I've certainly had worse. Only 24hrs, approx. 22 more to go.. =)

Zio Patch (Heart rate monitor)

Heart-Shunt
He did ask about the shunt stuff, how it went, what we ended up doing, etc. Simple answer to that I just said I think the shunt drains better BUT less consistently so more or less a wash, not a lot of improvement? I jokingly said one of these times i'll come back, you guys will ask about the shunt stuff and i'll say 'IT'S FANTASTIC!' =)
I do have faith, as Dr.Bragg says, "we'll get there", right? I am grateful he and Dr.Earing my Cardiologist never question this area though and seem to trust her, to trust she's got my best interest in mind and they both appreciate she keeps the heart stuff in mind.
He asked me if I thought we would do anything shunt wise in the next month (I just said I knew she was considering a few options and I admittedly hoped we could put the 2nd shunt back while explaining why I hoped this, that I didn't know if she would/if she did what her timing might be. Because I think it does sound (probably odd to say to someone, even if they are your Provider your (basically) hoping for something I said I know it sounds odd to basically wish for surgery (which I am NOT, I just want to feel better)!
He commented to keep him updated on this (I just said to him I thought perhaps if she did anything more indepth she would likely reach out to them though I wasn't certain) to which he said if we did opt for a more involved shunt surgery, that might cause more pain initially (to tissue/body) to let him know. He isn't opposed to us doing something he just said if we did end up doing a surgery and it occurred before the 1mo f/up he'd want to wait to asses how heart rate was reacting to the Ivabradine and any possible med dose adjustment.
^^ I know it sounds ridiculous and I said as much to him but even given as many things as I have going on in the next mo. I'd drop it all in a heart beat to put in a 2nd shunt or something else that had a good probability of helping the headache/pressure symptoms! Nuts I know.

Pic of Children's Hospital


Running for Rare Diseases (my Team MPS) Shirt

I wonder what I wonder about shunt valves; Many appt f/up's next 2 wks.

I have been thinking about this on and off the past few days, not sure anyone would know but wondering if a valve (controls how much spinal fluid goes through) can be to small to function at it's actual intended '#'? Can it if to small affect how much CSF gets through?
Have been feeling pretty yuck the past however many days w nausea, little appetite and sleeping really bad. On average I'd wake up (no lie, wish it was!) 5-8 times a night, last night despite even taking a med specific for bladder I woke up 12 times. TWELVE freaking times having to go to the bathroom and then to add insult to fricken irritated it's not even like I really have to pee much! It's definitely not a UTI not UTI like symptoms.
This bladder irritation at night has been the case since the 1st spine surgery a little over 4yrs ago but has been particularly bad this time around.
For whatever reason the LP Shunt seems to realllyyyy irritate spine nerves that go to bladder? It's the only thing I can figure out? Being quite honest by last night after dealing w the severe stomach spasms and chills/sweating for several hours it made me want to scream. I am tired of feeling bitchy (short tempered ) from so little good sleep!
I had spent yesterday after Church with my Sister and Z in FDL which was fine, tiring but fun. Not sure I was the best company (?) but was fun. I got home late afternoon and I bet wasn't home 45mins (enough time to get wash going) and severe, really, really severe stomach spasms, fever and chills/sweating + aweful nausea started. Needless I finally took a 3 med combo (was trying just about anything) finally of Ativan (which I only ever use if symptoms are really, really bad), a bladder med and MSIR which finally helped calm that down.
I literally was curled up on the couch it hurt so bad. All I could think being perfectly honest (even though I know i shouldn't question God) was what have I done so wrong that I have to feel this bad and that we just can't get a better answer?
Am I that bad of a person that God is punishing me? I,know it's the devil playing w my mind but he was doing a really damn good job last night.
I am thinking Of asking my Primary Dr for a referral back to the Spine-nerve Urologist I saw once or twice at Dean, I really don't want to see another doctor but maybe see if she could help any way this bladder/waking up so much at night. I am miserable at night and hate it. I hate how crabby/short tempered this often makes me feel during the day about stupid things I'd otherwise just ignore mostly!
Thankfully I am doing PT right now bc the shunt seems to also cause muscle discomfort albeit only on the R side when I am walking. It's always something, something to make me feel crazy!
I have taken to depressing the shunt reservoir to try and get more CSF drained. Although this valve drains at a '5' and the Codman (30 setting) drained at approx 10mmhg I feel like this valve does a worse job. I felt good initially after surgery or atleast better but I am pretty sure that was due to CSF that drains off during surgery. I do think (maybe?) it drains a tad better but not as consistent?
Oddly enough I had similar in the past when I had the Medtronic Strata, given that drained relatively low to you'd have thought I'd have had good results with that to but I never did, always was that 'doesn't seem like it drains enough' symptoms to.
Just makes me wonder if smaller valves regardless of setting can only drain so much whereas a bigger valve can allow more CSF through? For patients such as myself who have high volume CSF would that affect it?
This valve is super tiny, I cant remember exactly what Dr.Bragg compared it to but something that was pretty small.. it's length is also a lot smaller I think though that I am not as positive of, just gauging from the feel of it (still a bit puffy).
In any case I see her next Thurs (after a bunch of other appt. shuffling/rescheduling) for surgery f/up. I am just hopeful she'll agree to placing the 2nd shunt back. I can hope, right?
I don't know why exactly but I feel more positive about this working than anything in a long while.. Maybe w/ 2 shunts, 1 programmable, such as the Codman Hakim and the LP Shunt as a fixed valve would finally be good or much closer to better? I feel like the Codman Hakim is the best valve we've tried relief wise.. I guess maybe I feel hopeful (if we can try the 2 shunt system) mostly b/c of knowing the LP Shunt drains so well and consistent compared to the former TPL Shunt and it just seems like we need just a bit more. I am sure praying! Not sure if that's selfish but I am!

I spent a good part of the approx. 6hrs at ERT (5 for actual infusion) scheduling, canceling and rescheduling, trying to coord. 5 different appts. in the next 2 wks. 3 of these are in Milw., 1 is in Madison and then local PT.
This week I see my Heart Rhythm dr., for 1 wk f/up post starting the Ivabradine. Next wk which has turned in to nothing but chaos due to so many appts ( ERT on Monday, Cardiologist on Tues (2mo f/up post the Heart Cath/Heart Rhythm study and starting the new med though we won't know actual potential benefits of it for 1-2mo. with the hope it can help buy us time to a 3rd OHS). These 2 appts are good timing I guess (as good as any appt is ever, who really wants to sit there no offense to ones drs. as mine are very nice) but given Dr.Bragg was wanting some info from them before she would potentially put back in the 2nd shunt IF that's what she decided to do it works out..
Weds is Botox w the Neurologist at Dean and Thurs is surgery f/up with Dr.Bragg. I didn't intend for so many appts to end up in 1 wk but have already rescheduled botox numerous times and ERT runs so long I can't coord Cardiology for after that. Heart Rhythm dr (same clinic as Cardiologist, CHW) I can't remember why that couldn't be coord. Not much ever seems to be able to be!
Basically a lot of it is 1. ERT runs basically all day. 2. It is probably a lot that most of these drs have clinics on different days and different cities for several of them. God grant me serenity is all I can say!?
Thanks for stopping by, stay tuned I guess.
Erica

Discharged, day 3 post-op. How sweet to be home; next step/plan..

I was released from UW today, I would imagine Dr.Bragg would have let me go anyways given we where not planning to do anything right away (this next wk) but I had asked her if I could go home this wknd in any case. No sense in being there as I've said before when I can heal and ultimately when I can feel just as bad at home but in my own environment vs. being in-pt., not feeling good and cooped up.
Per Dr.Bragg and f/up I should make that for a week from now, I guess we'll likely discuss options I am not entirely sure why not later in this upcoming wk given we're just looking at options and if she has been able to figure anything out related to the options we've discussed.
In a way I'd have rather seen her this upcoming week, later in the wk so we could maybe get a plan made and move fwd.. Perhaps though she wanted to have time to look in to the different things she is thinking about? In any case I will talk to her Secretary this coming wk and hopefully can see her on Monday, a week from now? Am praying so, not that I obviously want to have to see her nor do I like that I'm still having to ask her for help but I really hope we could figure something out and sooner than later...
If I am able to see her that Monday i'll have to switch ERT days but I already am supposed to have other Appts. later that wk, for botox (a few mo. behind) and to see my actual Cardiologist (2 different cities, botox is Madison and Cardiology in Milw) for 2mo f/up but I'd make something work if I can, I can always reschedule something.

I see Dr.Kovach (Heart Rhythm dr.) this Thurs a wk before the Cardiology Appt, this appt being for the 1wk med f/up after starting the Ivabradine this past Thurs (day after surgery) which this heart stuff and shunt stuff admittedly has made me think more than a few times about how much more complicated the shunt stuff is now that we have to balance the heart stuff to.. It has been different than when I was leading up to the AVR replacements.
I do need to find out from Dr.Bragg what info she would like from Dr.Kovach, re: the new heart med and some potential concerns she had so he can write whatever it is in his clinic note or talk to her directly.
Part of why she wanted to take a little time before we did anything if I am remembering right was to make sure the new heart med doesn't affect any surgery or anything she might do. I can understand this, doesn't make feeling pretty crappy any easier nor does it make how long it will be till we are able to do anything very easy to deal with but I do understand given the heart issues are a big issue.. I just pray ultimately Dr.Bragg will be able to come up with an idea, preferably replacing the 2nd shunt.

I know I wrote about this last update but I realllyyyy think with our having the current shunt in the lumbar space (actually right now I sort of feel like we're back at exactly where we began?) and we know this drains so well, seems to drain pretty consistent and I know has worked the best out of any type of shunt we've had/tried. This compared to the former TPL Shunt which is where we primarily had the 2nd shunt all but a few mo. of the 2.5/3 or so yrs I had 2 shunts in place.. The TPL Shunt worked ok but no where near as good as the lumbar drains it seemed..

Given the LP Shunt works sooo much better than the former TPL Shunt and as I may have written about before we don't have any infection in CSF right now (had adverse symptoms during the period post the LP Shunt placement to infection being found some mo. later and then LP Shunt switched to the TPL Shunt at that time.
I really feel if we replaced the VP Shunt we could have good positive results in pressure and maybe longer lasting, with both shunts backing each other up in a sense.. I don't feel good now or long parts of every day pretty much due to the 1 current LP Shunt doesn't drain enough on it's own. Not at all ideal but I pray we could try 2 shunts again. Again I know, reading this 1 might think 'she's nuts' to want something else but if you where in my body and had previously had 2 shunts that at time did offer good relief (just not long term) you'd understand I think..

Obviously I don't ideally want any more surgery much less another device (hardware) in my body BUT it just seems like the best chance to get better relief.. I'd do about anything to not deal with these headaches/symptoms at night and so much of the day!
I completely trust Dr.Bragg, admittedly I am always surprised she hasn't given up and just thrown up her hands to say 'we've tried all we can, I have nothing else' but instead she seems to go above and beyond to try and find options.
As I've said many times before I am beyond grateful to her, I can't imagine it is easy for her to not have a simple answer and to not just give up but she doesn't.. it is as i've said amazing to me and I owe her, if only a hug perhaps.

I am always relieved I have her on my side and she seems to really want to help me to feel better. This to amazes me b/c she sees/knowns I go on about my life, kind of living my life around the bad parts of every day/night and many drs. much less people would say 'well she mustn't feel that bad if she is still doing the things she does'. - the thing as anyone with a chronic diagnosis knows is if we waited for when we only felt much better I'd be waiting a long, long time and a lot of my life would be passing by..

In any case please say prayers we can come up with an option and possibly it be going back to the 2nd shunt... As I've said before I ultimately it is Dr.Bragg's decision but I can hope (knowing how weird that sounds I know) we could try this or something even better such as those things perhaps she's mentioned..

I have many things coming up in Sept including Sun School restarts, a Conference in Salt Lake mid-Sept and a few other minor soI honestly have no idea when we would do any surgery IF Dr.Bragg came up with an idea/plan..
Once I realized this earlier today (I don't know why but has now felt like it was August this month so I haven't been thinking of what's coming up I guess) it definitely made me wish we could get something done sooner than later. That is not to be the case so I just ask additionally for prayer for better days and patience and as we sort things out I can cont to deal with this and con't to live my life to a decent extent while waiting and hoping..

I know this update is a little all over the place but I am going to end on this note. As hard as it is leaving the hospital not feeling good I feel like after the 1st few days home I find a new reserve, an energy of sorts I don't entirely feel that helps me deal with waiting, not feeling great and more waiting..
.
I do have to say, and not sure why it surprises me but It is amazing to me today how many people have asked "are you ok" or "are you with us" (sometimes just spacing out I guess) or similar but "are you doing ok" from the Nurses to family, etc.
What choice do we as patients in complicated situations have but to be "ok"? Between that and the number of Nurses today on D6-4 who know me and my history that asked about the shunts and if I thought we'd ever add back the 2nd shunt. My answer? I can only hope (I know like I've said above that makes me sound crazy) but we know our bodies as patients..
In any case, sorry this is so long and long winded probably.
Thanks for stopping by,
Erica

Surgery #40 (?), unsure. Slight improvements, still headaches.. Thinking aout options..

Kind of going to do this backward, post-surgery update 1st and somethings Dr.Bragg and I talked about this a.m then the surgery update. Weird I know but it's what I do best (weird, out of normal). =)
Dr.Bragg stopped by this morning, we where talking about the surgery, how the valve she used seemed to have given us a little more drainage but still isn't enough. I mistakenly when her and I have talked about the valve she ended up using thought it was a .5 valve so just a smidge of a difference from what we used in May but that 'smidge' difference might be enough to give us a bit of flow resistance so as not to irritate the spine nerves so much but would still allow drainage close to negative #'s.
Instead what the valve was is a '5' setting meaning more drainage then the Codman 30 shunt valve I had for the past few mo (and had in both previous shunts when I had 2 in place draining.
The 30 setting equaled something like a pressure of 10 per Dr.Bragg so no real significant surprise that wasn't enough drainage on it's own. Given we know I feel best at -5 when the EVDs are in place even this '5' fixed pressure valve I guess not a huge surprise it doesn't allow enough CSF to drain out..
She was going to talk to one of her Shunt people and see if they by chance knew of any valves that allowed more drainage than this current '5' valve but a bit more resistance/less drainage irritation  then when I had the '0' pressure valve a few mo. ago..
Some other options Dr.Bragg mentioned possibly today that she either would like to try if she could figure out a way or that we're considering include:
2. Recreating somehow a CSF fluid pocked like the pseudomeningocele (an area where CSF leaked and built up under the skin and would absorb, build back up, absorb acting in a way like a shunt but under the skin). I had really good results with that and acts similarly to how how an External Drain (EVD) works. She has mentioned this before but not sure there is a way to do that..

3. and what I happen to think would work best, Dr.Bragg was a bit more hesistant but didn't say no altogether.
Put a  2nd shunt back  in VP (brain) with perhaps a programmable valve and keep the fixed pressure valve that is in my LP Shunt. We know my LP Shunt drains so well/so continuous but we can't quite get it to be just enough pressure drained without the extreme spine-nerve irritation.. This way (and what I really think would be our best bet), I'd have CSF draining from 2 different locations, the VP Shunt even if it didn't work continuously could potentially give us just that small extra amount of fluid drainage we need to get to a really good place (how I feel) wise and shouldn't cause irriation to spine nerves.
With this Dr.Bragg wanted to think some on this and probably she said she'd want to make sure the new heart med wasn't going to affect the pressure/headaches at all before she would replace a 2nd shunt if that's what we opted for.
I feel such nausea and headaches and affecting vision some the way things are I am  praying we can atleast try adding a 2nd shunt back. I really feel it would be the best option, most likely to succeed now that my other shunt is back draining in lumbar area where we know it drains so much better than the former TPL Shunt did.
I do see my EP dr late next wk so if it was Cardiology she wanted the ok from again I would be seeing them and if need be I know either my EP (Heart Rhythm) or my Cardiologist would talk to her.
Weds's Surgery -
Back in the OR yesterday as everything was getting set up before I would be put to sleep I was kind of listening to the 2 Residents behind me. The 1 Resident to the other Resident as they where hooking up monitoring equipment for surgery: talking about the heart issues: "Oh that's all been treated, we don't have to worry about that now " - umm ok?! So not true and I mentioned to them while the aortic valve had been replaced the Mitral valve is now a bigger problem based off changes detected in Echo and other testing this summer. Yikes sometimes!
I did have the Anesthesia Dr I had today atleast once before and I think several times in past surgeries, I have always liked him but he didn't entirely seem totally up on the newer heart changes either (mitral valve low end of severe range, increased (elevated) pulmonary pressures (which I think this has to do with oxygen in the heart, I can't remember for sure ) and the Congestive Heart Failure due to the mixed mitral valve dis.
Non-the-less I knew this was very likely going to be a very short surgery (actual shunt part look roughly 30mins) so I just corrected the Residents BUT had it been a longer or bigger shunt issue you can bet I'd have made sure they knew ALL the issues/new changes!
While I rarely worry about the actual surgeries, if I did I'd be more nuts than I probably already am. That said I will be 1st to say overhearing those 2 Residents talking about the heart issues so non-chalantly definitely had my nerves up a tad! Thankfully (I guess, lol) the IV sedation was given w in 5mins of that after time out was done and everything hooked up.

We had quite a wait in pre-op due to being asked to arrive an hour early if we could as Dr.Bragg was trying to get the surgery moved up. Normally she tries to do all my surgeries 1st case/early but she had clinic this morning so anyways we got there at 12:00 and talked to Dr.Bragg pretty quickly + my initial Nurse came by to start everything.
I had my Port accessed already from Tues's Lab draw at CHW in Milw so that made the Nurses happy (lol, they where really happy, was funny).
My Primary Dr during the pre-op physical a wk or so ago had put in her notes about the very specific pre-surgery stress dose steroid timing and so the Nurses had already had that ordered up. We did have to clear up a tad bit of confusion regarding it's timing w my Anesthesia dr. but it was given an hour before I went back to the OR. So nice when it's smooth re this med and surgery timing! One if the Nurses I had today had also had me back in May/June for 1 of those surgeries and she said to me something along the lines "Well that went a lot more smooth then the last time/last surgery here!" =) I would have to agree w that Nurses sentiments!

My Mom and I where talking about the shunt issues, my headaches and she was talking about as babies how she'd notice differences between my twin and I between ways we developed and little issues I had that Aaron or non of my other siblings had. How it wasn't caught then but my headaches started back in 1st grade really badly but imaging didn't reveal anything and was eventually chalked up to migraines or sinus headaches.
How after a while, while I still got the headaches was less often till some yrs ago and Dr.Bragg's belief the headaches back then where really a form of Hydrocephalus, and this not being caught and in turn going un-treated Dr.Bragg has felt the cause of why we have so many issues w shunts now.
We where talking about I think how I so often get comments from friends and people on things like 'Well I hope they get it right this time" which I know people mean well and genuinely some people don't know how you learn to put on a 'face' so to speak when not feeling good but that in turn when you receive above comments ya almost feel guilty! Like I know I don't feel good but feel like I should b/c people think that I look good..
Dr.Bragg sent me a message the day before surgery and I must admit I thought it was so sweet of her. Essentially after mentioning a bit about the surgery plan she (probably to reassure me which I admit I appreciated) made the comments:
"It is worth trying and if it doesn't work we'll keep trying until we get it right. You are worth it and deserve to feel better". - She is kind for sure and thoughtful and she never gives up on me even when I am struggling at times! Although I don't need 'cheerleaders' sometimes I think that is what she tries to do, to offer encouragement?

On a side note I have to admit I am taken aback but think it is really sweet how many of my friends have made comments about how I deal with Insur., the trials they have had or do have and yet I seem to deal w these things and not have as many issues or I am able to get it sorted out. Even my Cardiologist comments from time to time how he is awed I have been able to get Dean, my primary Insurer to cover him as an in-network Provider and to cover tests there.
Apparently he has to talk directly to the Med Director for other of his pts. in order for some of them to get exceptions to see him and still others he's I guess never gotten approval for a few pts to see him. I often wish I could help other pts. with Insur. while I am under no circumstances a pro at insur. issues I have been able to learn and think Patients can be so empowered when they are better able to sort these sorts of things for themselves as much as possible.
While I am really stubborn and will keep at an issue till I get it approved or talk to the right person but I also have to give a lot of credit to my former, 1st Case Manager at Genzyme and what she taught me when she realized I was asking her a lot of questions and trying to learn. I'll always be grateful to Steph and her patience! Related to my medical care there have been a lot of "everyone needs someone like that person" in their life and I feel truly blessed to have had not 1, not just 2 but many, many people whether Friends or some Providers or people I've worked with in various settings that have helped me!
In any case, thanks for stopping by,
Erica

Feeling the feeling I don't normally feel..

How was that for a play on words? (the title) =)
I don't often get this feeling with surgeries (nervous) and honestly my being kind of nervous isn't even about the surgery it's more about is this valve going to work/is it going to cause the same extreme spine-nerve irritation and wicked stiffness I experienced back in May/June? I still get the neck/upper back stiffness to some extent on and off but it's no where near what it was when we had the '0' pressure valve in place on this LP Shunt. I sooo want this valve to work/to be enough for fluid drainage to relieve the CSF/pressure headaches but admittedly worry a little (the nerves thing) we're just going to make things worse again.

I completely, 110% trust Dr.Bragg, even if it seems like at times perhaps I don't, my posts and uncertainty have nothing to do with her, b/c I've learned she is really persistent, she cares and even when she doesn't quite know what the best answer is she'll ask around, research and try to come up with a new possibly better plan to help vs just abandoning me b/c it's gotten to complicated. She also works with my other drs. and coordinating is a feat in and of itself on some things!
I actually really feel for her, it has to be hard I would think to have a patient like me who it isn't simple, we get good times but then it's as if my body changes and we have to find a new 'better, good normal' (I know that's probably an odd way to put it) in how to manage this. It is why I think she is amazing, when so many others would give up she has not.
In any case I just pray this might be the answer, I pray it goes well, that Anesthesia goes well and whoever we have assigned to mY Team (usually 2 drs.) is someone familiar with my care that is good about the necessary pre-surgery med dosing and with the airway issues/cardiac issues.
I pray if under the really frustrating circumstances this valve wouldn't work Dr.Bragg is able to come back with a different idea; honestly ultimately I pray if it came down to it we could try a valve like what we're going to do (if it didn't work or caused to much irritation but was enough or close to enough drainage) we could do 1 fixed pressure valve in maybe a bit higher of a setting and 1 (maybe?) programmable valve in a 2nd shunt again which I honestly do just feel is what could be best and could work better now that there's no infection (as there was the last time I had a LP Shunt and a VP Shunt together)...
Obviously it's not up to me but I really feel like with how much better LP Shunts work than do the TPL Shunts we could get a good valve in that shunt and then maybe a programmable valve in a VP Shunt I feel like it could be high potential to be much closer to really good.
Anyways, I guess we'll see, right? It is all in God's hands and in God's time re when things will be good and he is with Dr.Bragg (really any of my Providers, all of us) I know in everything we do.
I have ERT on Monday. I do plan to call Dr.Bragg's Secretary I think to make sure everything we need beside INR level is in place on top of many other un-related phone calls I have. Tues i'll get INR drawn back at CHW (I spend a ridiculous amount of time driving back and forth to either Milw. or back and forth to Madison during a wk!) and have Port accessed for Weds.
Exciting times!?
Thanks for stopping by, will update after Weds's surgery, am so praying it is a success!
Erica

Ivabradine approved. Shunt revision next Weds, start med after?

We have the med approved! I picked it up Weds, my normal Infusion (ERT) was a different day due to our having gone to Bay Beach on Mon. Given my Pharmacy, Skywalk is right next door I was able to stop there before going to ERT.

I had talked to my Heart Rhythm Dr (email) yesterday morning, he had emailed to let me know iue planned to work on the Pharmacy benefit appeals yesterday. Late yesterday afternoon he emailed again and had talked to my secondary drug benefit provider (Cigna) who overturned their denial and gave us a approval for the Ivabradine (Corlanor)!
I talked to the Skywalk Prior Auth Coord. I often work with at the Pharmacy Tues afternoon after reading Dr.Kovach's email to let her know atleast my secondary insurer had overturned their appeal and to see if approval would go through. She stated they had the med in-house, which they actually had just ordered it the day before "we knew with your persistence you'd get it covered", (each month like with my Butrans Patch b/c I am their only patient/customer on it they will order this Ivabradine when I give Collette the heads up and then they get it overnighted and I am able to pick up typically within 24hrs. I usually would call in the Butrans a few days/week before I was going to pick it up so will coord. that and this Ivabradine to be ordered around the same time and pick up the same time.
My Primary Dr at the pre-op Appt last wk had suggested waiting till after next wks surgery to start the med (have to do a 5 day "holiday" between stopping the Diltiazem, a Calcium Channel blocker and stopping the Ivabradine (Corlanor) anyways so it is fine.
Per Dr. Kovach's (EP/Heart Rhythm) dr. NP she agreed about waiting to start the med till after surgery next wk. Dr.Kovach had wanted the 1st med f/up to be 1 wk after starting Ivabradine for a EKG/Clinic f/up so that is set for late the following wk. (2 wks from tmrw) unless of course something changes w/starting the med, but his NP commented if needed to let them know and we'd adjust the f/up as needed if need be.
As it is I see my Cardiologist then a wk after this (1st wk of Sept) so as is now it all works well with starting this med. His NP did say should we have any issues with surgery just to let them know and they'd schedule the f/up around surgery/being out.
Most of my Specialists work pretty well together and communicate well when needed so if needed I am sure that will happen post med start. I am unsure if Dr.Bragg has talked to the team or is planning to rgarding Anesthesia but my Cardiology Team (both Kovach and Earing, Heart Rhythm and Cardiologist) are well aware of this upcoming shunt revision.
I am thinking just to make sure all 'T's are crossed and 'I's' dotted I may call Dr.Bragg' Secretary on Monday to make sure they have everything they need (minus INR which i'll do on Tues) like the official 'ok' from my Cardiologist, all the lab work and pre-op from my PCP (She is great about getting this stuff in) and/or anything else they may have needed just to be sure we don't get to Tues. and are trying to scramble... I always wonder if I am annoying by checking on this sort of thing but at the same time the alternate is getting to the last minute and their not having something they need... I am at FMLH and CHW both Mon. (ERT Infusion) and Tues (INR and Port access for Tues surgery) so if something where needed I can fairly easily get ahold of my Team that is in Milw. and get it..

Ivabradine (Corlanor)
As far as the new heart med goes, I may have written about this previously, I honestly can't remember but I think the biggest questions for my Cardiology Team is 1. B/c the med is so new, Dr.Kovach has used it in just 1 other pt. he said their biggest question was/is not knowing if the med will help/or will it help but could it make any of my other issues worse? (an unknown, "We don't know if there will be side effects for you that aren't seen in other people b/c of all your other issues". He had made the comment, which is our goal and has been our goal with all the other heart med classes I've been tried on (but failed to adequately lower heart rate), they hope to get heart rate down and this in turn "helps you to feel better".
One question I had asked b/c normally with increased/rapid heart rate pts. more typically have high blood pressures I guess. In my case more often then not I have very low blood pressures so I was curious if this med would cause even lower BP's? I do not have adverse symptoms from the low blood pressures I have thankfully though it tends to make many of the Residents nervous often when in-pt.
Some of them will try to stop the heart meds which is ridiculous and especially given I have never experienced side effects from the low blood pressures but the meds are needed for heart issues. Anyways so Dr.K could not say if it would further decrease BP but asked that if I would experience adverse side effects to be sure and let him/his office know. This I am definitely not to worried about.
Overall as far as how long it takes to typically see a benefit the expected time is about a month and they typically give it 2months to judge whether the med is doing what it is hoped to do (bring down heart rate, in turn help heart failure symptoms) and hopefully slow mitral valve from worsening even further or perhaps quite as fast as it has been..
To directly quote Dr.Kovach (this one I wrote down verbatim, I love it!):
"This is very much us trying to pull something out of our pocket to see if it will work for you" - meaning my Cardiology Team is really trying to think a little outside the box to try and avoid a even more risky 3rd open heart surgery (OHS) for as long as we potentially can.
Ivabradine is approved overseas for Congestive Heart failure as well as to fast of heart rate (both overseas and here in the U.S) and there has been a small study on it's use in Mitral valve disease - all 3 my issues (Tachycardia, CHF, Mixed Mitral valve disease w/increased Pulmonary pressures). So in my case we are using it "off label" but technically the approved uses are issues we are dealing with, with my heart.
Stay tuned, will update again sometime soon. - On a small side note I have to add some of my family and I went to Bay Beach (Green Bay) Mon to the Amusement Park and was not only mostly beautiful outside but being a Monday most of the ride lines where pretty short. As always the day was great though goes to fast! Tues I was needless to say a little sore (but kind of worth it, my low back being the worst from 2 of the rides)! =) I started back to PT so good timing (he does hands on myofascial/lymphatic work). =)
Anyone who is on fb may have seen pics there. Next yr there will be a new train line layout (the new train was running when we where there just on the old, shorter tracks) as well as 3 new rides are being added this fall and next spring when the Park closes. It's a place absolutely perfect for kids and for adults!
Thanks for stopping by,
Erica

Pre-op Appt, Med appeals

Well what a day? Not bad but a bit frustrating for sure, definitely spent enough time on the phone between both my insurance pharmacy benefit providers re the Ivabradine. I had kind of forgotten about it but b/c the Ivabradine (Corlanor to insur.) is so newly approved it is not yet on my primary insurers formulary (approved drugs list) so instead of a Prior Auth having needed to be done apparently the right step would have been for my EP (Heart Rhythm) dr. to call and have what is called a 'Peer to Peer' discussion directly with the Medical Director re why the med is recommended/needed and the MD will then decide if the med is approved.
Ironically my secondary insurer also denied the med "b/c you need to have tried other things first", lol? I almost choked laughing when the Rep said that, I saw the prior auth form that was submitted, did they? She asked me what we've tried and I listed every single med they would want pts. to try. Seriously so WT-Heck is the problem, then? I did call and start the over the phone appeal process w the secondary insurer so they in turn call my Heart Rhythm doc and confirm the information with them and see if there is any additional info they want to add. The Customer Service Rep at Cigna said the whole process takes about 72hrs for an expedited review though that is a little confusing given it is the wknd and she said that timeframe still includes the wknd. I am not sure.. I guess it doesn't entirely matter as my Primary dr. would prefer we wait to start the new med, for safety reasons at minimum till after the shunt surgery is done and perhaps while I am still in-pt at UW with Dr.Bragg.
I know in talking to Dr.Bragg yesterday she expressed she would probably want to talk to my Cardiology Team though more b/c she didn't want Anesthesia to get upset about any recent cardiac changes (both the actual heart changes and med changes I assume) which I commented to her I had written about the very same thing, expecting if anything Anesthesia would be the one to raise concerns or some issue with this surgery and the recent heart problems. Ding, ding, guess I was likely spot on.

Regarding today's Pre-op Appt, ironically my PMD (who does an amazing job keeping up on the various, on-going and often varied but sometimes endless issues) said to me that she and the Dean Primary drs. had just received a memo on the new Heart Failure med my Team is working to get approved and she didn't think we'd have a problem getting it approved. Super ironic, hey? Apparently Dean recommends it just hasn't put it on their own insur. plan recommended drug formulary yet!?! She made the comment "Your on the cutting edge of medicine, when I got the memo I actually knew what the drug was b/c of you!" Keeping in mind this med is newly approved back in May, most docs have never used it,- I am my EP drs. 2nd pt. on it and my PMD's 1st patient I guess she may be right. Non-the-less whatever I am I just hope it helps and hope we can avoid a 3rd OHS for a good long(er) while.. I have to admit I am a little taken aback how many times I've heard 'your case is extremely complex' or 'yours is the most challenging case I have' from my Providers this wk!?!..

Actual recommendations leading up to surgery from my PMD are:
-Wait to change to new heart drug until after surgery - either in-patient or once done with surgery.
-Hold Coumadin 5 days prior to surgery
-Hold aspirin 6 days prior to surgery
-Restart Coumadin and Aspirin day after surgery (per Dr.Bragg)
-Morning of surgery take Cardizem and Digoxin (the Cardizem is the drug we're stopping 5 days prior to starting Ivabradine, so have to check that w Dr.Kovach and Dr.Bragg I guess).
-Hold Lasix and Spironolactone morning of (these take fluid off)
-Check INR day prior to surgery (i'll also have Port accessed at the same time)

That's all for now, say a prayer for the Ivabradine approval w the 2 appeals (peer to peer approval and the Cigna appeal)!
Tmrw (Monday) we (family) are headed up to Green Bay, to the Amusement Park owned by the city. There's a new, bigger replacement train with a much bigger area it drives along (around the amusement park) as well as 3 new rides that are being added. Last yr a new ride was added. The park is AWESOME! I am not so sure i'll be able to make it up the giant slide (100ft tall) but sure going to try or atleast once, it is awesome! Should be a fun day!
In any case will try to add some pics in one of the next updates. Hoping to hear something about the med this week to,
Erica

Shunt revision (surgery) scheduled, Starting the new HF med (EP dr.)

Hi All, This kind of covers alot, has been a busy wk and last wk's appt w/Dr.Bragg as well.
I opted to schedule the shunt revision with Dr.Bragg for 2wks from Weds, I could have had it done next Tues, the 11th but have a Sunday School Teacher's mtng that night and honestly even though I really, really, reaaallllyyy want to feel better SS is important to me and I know we have things to sort out for the upcoming SS yr. Believe me I wish that mtng where this wk so I could have had the surgery the 11th! Is what it is though atleast if nothing else my favorite nurse will be there.
I guess if nothing else this will give myself and my Cardiology Team the time to sort out the new HF med and get it started perhaps?

Pre-op Appt
Pre-op Appt for the shunt revision is scheduled for this Fri with my Primary dr. Given I know Dr.Bragg's team appreciates her thoroughness AND Dr.Simpson is well versed on my issues, and when needed reaches out to the appropriate providers it is nice to just have it done with her.
I am a little confused if Dr.Bragg still really needs the 'official' ok from my Cardiology Team (I fwd'd my Cardiologists Nurses note to her) but none-the-less I am fairly certain Dr.Simpson will reach out to Dr.Earing for some reason or another be it the Coumadin dosing/stop date, the new HF med, etc so i'll let Dr.Simpson know so she can make sure Dr.E's Team gives her their blessing to. I am fairly certain this formality is more for the Anesthesia folks as much as it is for Dr.Bragg as they can be fairly particular and I suppose would want to know if they need to take any addt'l special precautions given the new heart changes.
Usually the things my PCP sorts out, makes sure are well taken care of are the pre-surgery steroid dosing, which although Dr.Bragg always makes sure it is ordered some of the Anesthesia people at UW can be more than a little ridiculous about giving it at the approapriate timing. I did see my Endocrine dr incidentally this wk (Weds) so did ask her yet again about the timing and yet again she verified what I know (about solu cortef having to be given at the 1hr prior to surgery point) so I gave Dr.Simpson a heads up and she is going to stress this point in her steroid dosing recommendations she sends w/the pre-op note.
Given I should likely have the this Thurs's EP Study report by the surgery i'll fwd that to Dr.Bragg to ahead of surgery.
On an unrelated note, it is probably the humidity but breathing has felt kind of bad this past few days, it's not as if it would likely be noticeable to other people, it's not as if the symptoms are like i've just run a race short of breath but non the less I feel short breath b/c it is difficult to get a adequate breathe of air, worse as the day goes on (later in the day) which is not an uncommon phenoma in CHF pts. it seems.

EP (Heart Rhythm) Cardiology F/up
I saw Dr.Kovach today at CHW (it has been a really busy wk filled with ALOT of Appts., with 1 more tmrw.) but anyways given the EP Study was pretty much normal and the Cardiac Cath showed increased pulmonary pressures and the severe mitral valve mixed disease he is opting to start the new Heart Failure med (Ivabradine) i've been talking about on and off for awhile.
We have the Prior Auth's submitted to both my Pharmacy benefit providers so is just a wait and see now. Per my Pharmacist who has helped us a great deal with this all Jake expects we would/should typically hear in 24/48hrs if it is approved. That most likely at minimum would put us at Monday. Once we know it is definitely approved, Jake (Skywalk Pharmacy in CHW, AWESOME PHarmacy!) will order the med overnight and i'll go on a 5 day washout of the Diltiazem before starting the Ivabradine.I will stay on the other 4 cardiac meds including Digoxin as the Dig., Spironolactone, Lasix and Coumadin/Baby Aspirin have different mechanism of action on the heart / heart function
Per Dr.Kovach i'll be his 2nd pt to start on the med, his other was started apparently while the med was still pre-approval but was a patient post-transplant who also experienced abnormally high/significant heart rates putting stress on that pts heart to. In his case he did have an issue but Dr.K said he felt it was more likely related to the pts. transplant not the med athough he commented they would never truly know. He also commented it has a pretty mild side effect profile but he couldnt say in my case with my many other issues if I would experience otherwise un-seen issues on it. He doesnt expect so but can't say and we just have to see. He/my Cardiologist feel any risk we could experience are out-weighted by potential benefit we hope to see.
As far as starting it I will not have to be admitted though curiously Dr.K seemed a bit hesistant about this, he did say initially i'd have to f/up at the 1wk mark post-starting it for an EKG, to discuss if any issues on it and then he'd decide as far as how often for f/up's after that. He also commented should I experience any side effects I needed to be sure and let them know so he can evaluate and adjust if needed.
I see my Cardiologist the beginning of Sept. so I suspect (but not certain, perhaps it depends how the med goes) I may not have to see Dr.K. between the 1wk post starting it and the Appt. f/up with Dr.Earing (they work together and communicate closely). I'd imagine a 2nd post-med start f/up with Dr.K would occur at some pt after the regular f/up with Dr.E depending how I was doing on it, I do know he wants to do frequent EKGs initialy on it.
Endocrine Appt
On a un-related, funny but nice note, I saw Endrocrine Weds which is a Provider I really like (but of my Core Providers I can't say there is any 1 I don't like) and anyways have to share this funny encounter:
Resident (Endocrine Appt): "I never thought i'd see someone w your really rare disorder"
Me: Laughing, "Glad you know what to look for now"
Endocrine dr: "I've actually sent some pts up there (UW) to Neurosurg. b/c you're case is probably the most complicated i've seen and I know you've gotten outstanding care there". (given I know she sees alot of really complicated cases I was a little taken aback by that comment)
Funny encounters, another new dr aware of MPS; kudos to Dr.Bragg (I dont know any of my drs who don't talk about her care)!
— at Froedtert Hospital.
I guess b/c they've seen the great care i've gotten from Dr.Bragg and her persistence in trying to help me when in many complicated shunt/hydrocephalus cases (as was my case prior to Dr.Bragg) Neurosurgeons are known to frequently give up or basically abandon complex pts., telling the pt. there is nothing more they can do and/or 'it isnt my problem'. I don't think most people would believe how often i've heard that one from other pts. and including my Endocrine dr has said she's had multiple pts the Neurosurgoens at FMLH have basically abandoned/refused to see as was my case there to. Dr.M works directly with that Dept. (she is Neuro Endocrine specialized) and yet multiple other of her pts she shares w those drs have experienced the same I did as well.... In any case she is one of my Provider that really, really likes Dr.Bragg and recognises how unusually amazing she is at her job!
On a side note to this Dr.Maas and I where talking about the recent Cardiac Cath/Heart Rhythm study and the Shunt issues in May as well as this upcoming shunt revision and she made the comment "UW really seems to be on top of making sure you get the cortisol replacement". I made the comment to her that this is true to an extent, Dr.Bragg when needed really helps advocate for it as some Anesthesia drs are pretty stubborn how they think it should/can be given and Dr.Maas has always been very particular how she wants it given.
At CHW recently they gave it no question how she (Dr.Maas) wrote for it and at UW there are some Anesthesia docs that I have that give it the hour ahead of time no question but 1 or 2 recently have argued saying something along the lines 'we give it right after sedation before your put to sleep (? um arent you basically stressing the endocrine system already at this pt?) and intubated'.. Needless I am pretty stubborn and know what works so have went to toe to toe a time or two (or 3 or 4..) refusing to go back to the OR until the solu cortef was given (which takes all of about 5mins to give through the IV). It is much more ideal when already on the floor at UW and the Neuro Nurses just give it b/c they know me and give it before I ever even go down to pre-op.
In any case, that is all for now. Will update sometime soon. Perhaps if anything worthy after tmrw's Pre-op Appt (but probably include it in a future post).
Our family goes to Bay Beach which will be exhausting but am excited for!
Will update again soon, Thanks for stopping by,
Erica

F/up Dr.Bragg; we have a plan (opting to revise shunt, try a different valve)

Thurs I saw Dr.Bragg, which was a f/up planned for after I had the Cardiac Cath and EP (Heart Rhythm) Study.
She did thankfully tap the shunt to take off some fluid which is always a relief, albeit temporary but a relief no-less! It definitely made for much better sleep Thurs night which was good (waking up maybe 3 times instead 4-5-6 times) and a early morning with Z much easier! Symptoms where back by Fri but still made Fri so much easier; I really can't explain it but is almost like a reset to my body of sorts.
She did bring up about should we talk about and go ahead with making a plan for changing out this current shunt valve, given she knew I havent been feeling good headache wise. She made the comment something like (my words) 'we really dont know if this will work, if your body will be able to tolerate this either (given the extreme nerve irritation last time) but there are other options if it wouldnt and 'we've made bigger leaps of faith than this'. <-- This is why I like her, she is persistent, she doesnt give up and she looks at my big picture while wanting to make my quality of life better.

My Dad asked me the other night if I thought she would ever come back to thinking a 2nd shunt would be better again? (I can't tell you how often I get that question or similar from my Nurses, friends, a few of my other Providers) I really don't know the answer to that but con't to hope if this shunt valve didn't work (say it also causes the extreme nerve irritation for instance or isn't enough CSF drainage on it's own, maybe we would try a different approach?
Given this new valve will be a set valve, we won't have options to adjust the setting like we do with my current valve, maybe Dr.Bragg would come up with another valve that drains really well in my lumbar spine.. The current valve we have we have many settings to chose from but it isnt go low enough. The valve doesnt drain enough CSF (the fluid that builds up and causes my headaches/symptoms) so her (and my!) hope is this .5 valve will allow better and more CSF to drain without causing the extreme nerve irritation the 0 pressure valve we tried back in May did..

We do know the shunt draining in my lumbar spine definitely drains better than when the shunt was in my thoracic spine.. I con't to hope, either this just works or if not, not that I really would want a 2nd shunt again (I don't, seriously I.do.Not) except I think the former 2 shunts complimented each other better even if imperfectly..
We know that when there where the 2 shunts and when both where working the VP Shunt gave us some drainage and at times the 2 shunts when they initially worked was really well. I am hopeful either this valve we're trying next (.5 mininav) will be ok or perhaps we can try that down the road.. I sincerely just hope this could/would work.
I often wonder given when we 1st placed the 2nd shunt I also had symptoms from the residual shunt infection that past Dec. (but we didnt realize thats what it was) and symptoms didnt really creep back up for another month or so, eventually getting worse and worse till we caught the infection later on by accident would we have had good results w that then LP Shunt and the then VP Shunt?
During that whole time after the 2nd shunt was placed I was sick on and off for months (hard to explain symptoms but it was terrible) and then when we found the infection we took out the shunts, placed the EVD and once cleared put in the TPL Shunt along w VP Shunt. The TPL Shunt never seemed to work as well as LP Shunts have for me. I've often wondered more than a few times if we had 2 shunts back now but in LP and VP would it be alot closer to better/for longer? I wish we could find out!

Dr.Bragg did make the comment Thurs (which she has made at times in the past to, perhaps as a reassurance to me which if that is the case I am grateful she does) that if this doesnt work she wasnt giving up and she sees this as a work in progress, we'll con't working at it and we'll get it figured out even if slowly as we've made some progress (very true). I am grateful for her. For her not giving up and for trying to help me, especially when I know she rarely sees me truly feeling bad.
I will just say it again, I am just grateful to her =)

EP Study F/up
This Thurs is the follow up appt with Dr.Kovach in clinic. I know he and Jake, my Pharmacist (or the Pharmacist that owns CHW'S Skywalk Pharmacy) are working on the Prior Auth approvals for the Ivabradine in hopes they can get it approved prior to Thurs's appt.
I have a few ques's for Dr.K but at the same time he has been amazing about answering questions all along!
I think i'll be able to just start the med without the original admit Dr.K thought would be needed which would be great! I am very hopeful the med will help and will help us hold off on needing to do the 3rd OHS for a longer while!
Other Appts
Otherwise I see Endocrine this wk, normal f/up. She is who manages the stress dose steroids both daily, the self injections and especially coordinating with my Providers when there are surgeries/procedures such as the Cardiac Cath/EP Study 1.5wks ago and the upcoming shunt revision.
Next wk we (my Parents, Sister, her kids, myself and my other nephew) are headed up to Green Bay area to Bay Beach, the amusement park which we do every summer, both when we where kids and now since Quinn (13) was a toddler. Tues I have our 2nd Sunday School planning mtng for the upcoming school yr.

Anyways will update sometime later this wk probably both about the surgery date if I know it, I am hoping either Dr.Bragg's Team calls tomorrow or i'll try calling them to try and schedule it. I'll also update the EP Study Appt., likely in the same post.
Thanks for stopping by,
Erica