Progress on Ivabradine (new HF Med)

The f/up appt with Dr.Kovach (EP Heart dr) is Aug 6th. F/up with Dr.Earing (Cardiologist), which is the same clinic will he 1st week of Sept. I think they wanted to stagger the 2 f/ups (I guess).
Per Dr.E's Nurse he did give the ok for Dr.Bragg to do "whatever she feels she needs to do" which I kind of suspected he would ok any revision if she still does opt to go ahead with changing valves.

I see Dr.Bragg on Thurs this next wk. I am watching Zan both Thurs and Fri at his house but my Sister was able to get his other baby sitter (whom Z also loves) to watch him for part of the day while I go to Madison.
I am hoping Dr.Bragg will take fluid off the shunt and we'll go ahead and put something on the books to switch this shunt valve.. I guess will see when I see her, I can be hopeful.. it truly seems as if mornings have just gotten more difficult. As if I wake up more and more at night (have even tried not taking the 2nd dose of cardiac meds and doesnt make a difference). If only all day could be like afternoon, I guess?
Ivabradine update
In a surprising turn, I had talked to my Pharmacist at Skywalk (Pharmacy, located in Children's Hospital) Thurs night out of curiousity if he knew anything about the new Heart failure med we've been considering.
He didnt nor did his wife who is apparently also a Pharmacist, cardiac one at that! But he offered to talk to his med supplier Fri (which he did) and run test claims through my Pharmacy providers (which he also did) to see if he could get access to the Ivabradine.
He did send a message Fri morning and is able to get access to the med through his supplier and as I figured my 2 Pharmacy providers require prior auths to be submitted prior to being able to fill the med. I knew this was likely the case w Dean per my Insur. Nurse Case Manager.
The drug is around $400/mo which to me doesnt seem that expensive given it's brand new, (I realize it's also not exactly inexpensive but.. considering Aldurazyme..) given daily and you figure the enzyme I receive by IV every week is $30,000/wk (huge hospital upcharge).. around $15,000/wk without the upcharge.
In any case Dr.Kovach emailed me with essentially the same information Jake gave me regarding access to it.
Per his contacts the drug has become much more available over the past 2mo vs the 1st time he reached out to the Pharma Co. it was still very restricted and not widely available due to the new release.
Given this information Dr.Kovach didnt think we would need the IRB (research board) approval at CHW and also didnt think we would need to admit as in-pt in order to start the med. I am unsure 100% on that part but won't complain if I can just start it on my own!
In an even better twist, today, Monday Dr.Kovach and my Pharmacist at Skywalk (in Children's Hospital) have been working together - all of us exchanging emails regarding this med and Jake (Pharmacist) had let us know he and his Prior Auth Coord. could start the process of trying to get the Ivabradine approved ahead of my appt next week with Dr.K. + he would just wait to order any med till after I saw Dr.K in clinic to be sure we decide to use it.

Dr.Kovach in turn opted to send a prescription to Jake/Skywalk so the process shall begin for working out the 2 required Prior Auths (Dean's, Navitus and Cigna for my secondary insurer).
We're hopeful we could perhaps have the PA's approved by next Thurs's appt and if so and if we do opt to start my Pharmacy will order it over night.
It seems very likely we would start/try the med as Dr.K said after the studies last wk, when he stopped by in recovery that he had talked to Dr.E, my Cardiologist (who was at an outreach clinic) and Dr.E said to Dr.K when they talked on the phone after the Cardiac Cath/EP Study that he really felt we should try it, see if it could help.

I just like this saying =)

On an unrelated note, I know they tried to use more sensitive type adhesives for the many and various patches (EKG leads x2 (I guess they had to use 2 separate sets if I remember right), the 2 rectangle patches they use if they have to shock a person, the Port cover patch, and heaven only knows what else) but wow-za do I still itch!
Thankfully it is intermittent but geesh between my back and stomach especially I at times feel a bit crazy!
Hopefully this all calms down and stops realllyyy quick! =)

Link on the new med we are planning to try, newly FDA approved in April/May:
http://www.corlanorhcp.com/mechanism-of-action.html

I thought I would clarify my form of Heart failure vs what one might think of as a traditional form of heart failure.

Heart failure can be a complication of circulation-improving treatments for congenital heart disease.
In these cases, the term “heart failure” is misleading because the heart actually may be working well; it is the related heart and lung problems that cause the symptoms.

In any case if there is anything new or interesting to update after this weeks appts (Pain Mngmt and Dr.Bragg, Weds and Thurs) i'll do so.
Otherwise I watch Zan some of Thurs and on Fri to as I mentioned above. I am sure he and I will find some adventures, likely at the Marsh if he has any say! =)
Thanks for stopping by,
Erica

Cardiac Cath, EP Study - what's next

Pre-Op Consent (not a great pic, cut off 1/2 the wording)

What was done today:
Cardiac Catheterization
Electrophysiology Study
Consented but ended up not needing Ablation
Transeptal puncture

The Cardiac Cath was to check the pressures in both R and L sides of heart. The L side is very elevated but R side looked good.
I can't remember exact wording but something to the effect of 'your L side is significantly elevated but not the worst I've seen and thankfully your body is handling (secondary from Mitral valve) this pretty well with the meds you take (meds to get fluid off).

The flip side of my issues if not well managed would be as he said 'your not in and out of the hospital every few wks thankfully'. <-- This is my wording btw NOT Dr.Kovach's; is my trying to paraphrase.
He commented (which I do know from talking to him and Dr.Earing) the faster heart rates probably make dealing with the Mitral valve harder due to less time between each beat for the heart to relax and properly fill with oxygen.
Our hope is we can "kick the can down the road a little longer"; try to lower heart rate, the question is will it help (trying this new heart failure med)? Per Dr.Kovach Dr.E is leaning towards the med, he commented "your not a low risk candidate for MVR, especially given the amount of issues you had with the Aortic valve replacement(s)".
Again along the same lines he commented, if there's a way we can help you feel better with the least risk, whereas OHS is really risk/benefit equation, they'd prefer to take the least risk approach with the best benefit. Some things they can do to help feel better including help get heart rate down which may help me to feel better, to deal with the mitral valve issue.
The actual procedure was done at Children's Hospital (WI) in Milw.

As far as the EP Study, the Ablation was not needed (good in that it wasn't needed, bad in a way as it could have been an easier potential fix than getting access to and trying the recently, newly FDA approved Heart failure med (Ivabradine).
The transeptal puncture would have been using a catheter, using (I believe if I remember right) a catheter tip that freezes an abnormal area after they literally would have punctured a small hole between the L and R sides of the heart. The cold-freeze cath would had it been needed been used to freeze an abnormal signal.
Thankfully this was not needed as it sounded fairly intense and involved w not insignificant risks (though small) that could require patching the newly created hole and/or OHS to repair the dysfunctioned (my own word?) sinus node. Again this part I may not have gotten completely right but is the jist of what is done when needed.

For Anesthesia I did have Dr.Taylor who as I've written about before did both my prior OHS's and the Transthoracic Echo that was done in-between the 2 heart surgeries.
It sort of amazed me (in a good way!) this morning but not only did she and Dr.Kovach have the stress dose steroid (for surgery, cortisol replacement) in place and set but as I think I wrote about last post she had talked to the pre-op Nurses re my Port and accessing it + she remembered the little things from several yrs. ago (I thought your 1st OHS surgery date was coming up pretty soon').
She remembered the joint issues, headache/positioning issues, the meds I took then vs now and somehow we got to talking about those actual heart surgeries + her being happy she was there today 'in an easier capacity' (again my words but essentially what she said) vs. our having to deal with and get access on the airway issues (typically includes glidescope or fiberoptic intubation) with smaller sized airway breathing tube.
I don't think my airway is really anyone's (probable) favorite airway to try and secure pre-surgeries!
For the actual Cardiac Cath and EP Study due to the type of arrhythmia Dr.Kovach was looking for she opted to use very light sedation where I could still talk with them and answer questions they asked (not that I remember! I told her when she mentioned this ahead of time that I was not taking claim for anything I said while under sedation) =) I know there have been times in the past I've had conversations w Dr.Bragg and others while being put under, Eeks!
The EP Study and Cardiac Cath ended up only taking about 2 hrs (they gave a 2-6hr window it could potentially fall in) which was nice, I guess? I know Dr.Taylor took great pains to try and have me as comfortable as possible during the test while on the table and gave extra anti-inflammatory med to help which 'what I suspect will be significant joint pain in the next few days'.
It was nice she and the Team would care and try to prevent this as much as possible.
Due to all that was potentially to be done they type and crossed for blood products, especially I suppose b/c I was kept on the Coumadin and Baby Aspirin leading up to today's procedure and blood clot risk is higher in Individuals w Mech. valves having Cardiac Cath/EP Studies done.

Ivabradine / Shunt valve revision
As far as the Ivabradine
Dr.Kovach seemed ok with my getting the shunt revision, I had to leave a VM for Dr.E's Nurse today about f/up ( a couple weeks with Dr.Kovach to asses the Cath entry sites and more importantly to further discuss where we are at w getting access to the new med.

He did say he has been in touch w the company that makes the med but does have to sort out if we'll need an IRB (review board) approval at CHW to use it/him to prescribe it and that b/c the drug is so newly approved it isn't yet widely available in the U.S so he needed to reach out to the right people and give his pitch essentially about having a patient he and my Cardiologist feel would be an ideal candidate for taking it.
As far as meds I came in on taking, he was going to talk to Dr.Earing about the Cardizem, he doesn't feel it has a benefit and feels we should consider stopping it (not something I am against!) but he did feel we should keep the Digoxin on board as that works differently, by helping to strengthen the heart.

Once we started the Ivabradine I think I may have wrote about before he said it typically takes about a wk to lower heart rate and 1-2mo to see how it is affecting overall heart function and symptoms. It seems from talking to him at that point we would re-eval doing Mitral valve replacement (which no one wants to have to do).
As far as a 3rd OHS and MVR he like Dr.Earing believes it is a risk/benefit issue, ie we likely will have no choice but to do it but is a matter of when. Some in the MPS community have asked me why they are waiting so long to do it and the honest answer is 2. things.
The surgery was scary (leading up to it, not knowing) the 1st time and well not that awesome the 2nd time so we know without almost certainty it would likely be difficult at best a 3rd time when done and what issues would they run in to during?
It's quite possible there is someone out there but I can't say I've ever met another MPS Individual whose had 3 open heart surgeries..
The moral of today is while we didn't find a area to ablate and thus non-med wise hopefully help the to fast of heart rate/tachycardia and seeming related heart symptoms I did have a great Anesthesia dr. working with me (she's very down to earth and yet was so willing (interested?) to learn about what I have/deal with) when I first met her 2 yrs ago and she worked w me today to try and make the Cardiac Cath/EP Study a success as much as possible comfort wise..

Team Effort is really the over-riding thing that makes my care a success most of the time and most definitely what helped today. She (Dr.Taylor) and Dr.Kovach communicated both with me and with each other willingly leading up to today's anesthesia/sedation and Heart studies and before the test today + obviously during/after.
On a side note, I want to share this really sweet, thoughtful note I received from one of my (I consider) closer (as in really cares, go out of way, tries to stay up to date and doesn't give up) Providers prior to today's procedure:
Erica. Will be thinking of you tomorrow. You will do great! Keep strong and see you soon.
Dr.Bragg f/up
Stay tuned, I f/up with Dr.Bragg next wk, am hoping she can tap the shunt and take off some fluid. Also hoping we can sort out this current LP Shunt valve and potentially replacing it w the 'Mininav 5' which has much less resistance. I am still nervous it would really irritate spine nerves more but at the same time these headaches...
Will update again soon,
Thanks for stopping by
Erica

Dr.Taylor, Anesthestiologist

2 years ago (7/23), 1st of the 2 Open Heart Surgeries (OHS)

Heart Cath- Rhythm Study, 7:00 arrival/9:00 procedure Children's Hospital

Tomorrow's Heart Cath (EP Study) and potential ablation is slated to start at 9:00am with 7:00am arrival. Typically at Children's patients would have to arrive till 1.5hrs before but b/c they need a urine sample, have to access the Port (yay to my Anesthesiologist, Dr.Taylor for talking to the pre-op Nurses on my behalf about this!!) and the stress dose steroid is to be given an hour before any sort of sedation is given, no matter big or small.
In tomorrow's case we're using conscious sedation but apparently i'll be awake during the study if I understood the Nurse and my EP dr. both correctly whereas w some forms of conscious sedation I've atleast drifted off in to another land of sleep of sorts.
The reason for being awake/very midly sedated is the arrhythmia the Cardiac Team is looking for "tends to go to sleep when pts. go to sleep". Dr.Kovach, my EP dr. felt based off my recent Holter monitor this was definitely likely the case as my HR went from the abnormally high rates to down in to the low 60s very suddenly and must have stayed there as I slept.
The Stress dose steroid is ordered, which is always a relief to know I don't have to battle w the Anesthesia drs. about this and timing (some don't want to give it at all, some think it can be given after they've given some sedation but before they given fully put a patient under). Another bonus I suppose of having the same Anesthesia dr. on my case for this who did both OHS 2yrs ago and 2yrs ago in Sept. + the sedated TEE (Echo where a probe is put down a patients throat to see internal views of their heart).
INR
Per Jane, my Cardiologists Nurse she would call regarding f/up, whether it would be Dr.Earing and Dr.Kovach together or whether it would be something else such as I would imagine (but not certain) just f/up with Dr.Kovach if they do find an arrhythmia and feel reasonably good they where able to ablate and get rid of it and thus this overly fast heart rate would settle down and slow down on it's own.
If that is the case chances are we won't have to go on the new Heart failure med, Ivabradine and we may (but no one knows for certain) have more time to needing to replace the Mitral valve if symptoms improved w this study tomorrow.
If the Team does not find an arrhythmia they can ablate or if they find an arrhythmia but it is not amenable to ablation then likely f/up will be meeting w both Dr.Kovach and Dr.Earing together to discuss our next steps (likely the med) and what next.
I am a little curious how tomorrow plays out..I think out of anything related to tomorrow I am a little nervous about laying flat during the study but as I think I wrote about in my last blog update (or maybe it was on fb, can't remember) hopefully Dr.Taylor, my Anesthesiologist can help come up w something to manage any nausea if this became an issue during tomorrow.
It is an out-pt procedure, it can take according to Dr.Kovach anywhere from 2 (or maybe it was 3) hours up to 6hrs depending on any difficulties they run in to getting the catheters in place, mapping the electrical activity of the heart and if they find any arrhythmia/abnormal areas and if they do how well it responds to being hit w the RFA which is the energy source used to ablate abnormal areas.
I'll update on fb and update here either tomorrow night or later in the week how everything went.
Prayers always appreciated =)
Erica
What is RF Ablation:
Just an article on what radiofrequency ablation is, ironically i've had RFA used in my spine before to help nerve issues.
In Radiofrequency Ablation abnormal nerve signals are destroyed with high frequency energy. This often cures the individual of the problem permanently. - See more at: http://pediatricheartspecialists.com/articles/detail/atrial_tachycardia#sthash.XJ4gyZFj.dpuf a radiofrequency ablation. In this procedure, the abnormal focus of tissue is ablated or destroyed

Camping, Next weeks plan (Cardiology Procedure, Anesthesia), etc.

Article related to the arrhythmia my EP (Heart Rhythm) dr. and Cardiologist suspect and are looking to try and get rid of in the EP Study next wk:
http://atrialtachycardia.net/ectopic-atrial-tachycardia/
Editing this to add an 'ectopic' beat which is what my Cardiology Team feels the abnormal heart rhythm/heart beat likely is, is defined as:
ectopic
/ec·top·ic/ (ek-top´ik)
1. pertaining to ectopia.
2. located away from normal position.
3. arising from an abnormal site or tissue.
This past weekend was a blast, I am really not at all a camping type person, for a few reasons but had such a good time with my Sister, niece and nephew and my Parents who where already up at the Campground in Shawano a few days before Sara and I got there.
The campground includes a small store, outdoor eating restaurant that also has different types of ice cream, the beach which you can check out paddle boats, canoes and swim (water was pretty warm though I just walked in while the kids swam).
There is many different games to check out from ladder ball, horseshoes (I think that's what it is called), basketball, tennis (lol at my Sister and I and my Dad who joined us after a while, he was busy having fun taking pics initially). ;)
We took out one of the paddleboats (another site to be seen I'm sure as the water was so wavy from boats and wave runners). There is woods that was right by our site for Zan to catch butterflies in, walking paths and so many more activities for kids (and grown up kids?!?) =)
Best of all perhaps it didn't rain this year!
I have been feeling short of breathe on and off throughout almost every day, worst when I got upstairs in my Apt bldng to do wash, walk out in the heat or even often from room to room. Is just very variable. in a way it seems worse then before the AVR surgeries but I am sure the humidity and high temps don't help. My Cardiology Team advised on "high humidity days to avoid being outdoors" due to the lung and heart issues but for real, I love warm weather and being outside and if I have to walk a little slower or stop a little more to catch my breath i'd do that vs letting life pass me by over symptoms!
Thankfully although symptoms due occur fairly frequently I do also get many periods w/less or no symptoms of being short of breathe (SOB).
The fact I feel short of breathe quite often simply leaning down to say fill the cat dishes and walking 5 steps back over to set them down is a little disconcerting some days but that to, hopefully the EP Study next week will help this if my Team is able to find an arrhythmia and it is amenable to being ablated (using electrical activity through separate catheters to burn the inappropriate signal(s). If my Team doesn't find an arrhythmia then we'll likely move on to the new heart failure (HF) med and hopefully that will help slow heart rate and in turn help symptoms... Ironically this cardiac test takes place exactly 1 day short of being 2 yrs since the 1st OHS (AVR/Konno Procedure) took place in 2013. Odd how that happened! This study mapping the heart and potential ablation should be a wee bit easier (without a doubt!) than the Aortic Valve replacement, aortic root enlargement and VSD Patch where + subsequent 3days of being sedated/intubated 2 yrs ago!
I think the worst part will likely be laying flat for so long (3-6hrs for procedure + 4-6 hrs after where i'll have to keep my legs straight though I am reallllyyyy hoping my Team will let me lay with my upper body somewhat upright. I plan to talk to my Team about this, the worst they'll say is no, right?
My EP dr feels w/the EP Study if either A. they don't find an arrhythmia (abnormal heart beat) as suspected or B, they find an arrhythmia but upon trying to ablate/get rid of it the site isn't able to be ablated then he, myself and my Cardiologist will sit down and discuss the Ivabradine. The specific arrhythmia the EP dr. suspects is an Ectopic Atrial tachycardia or extra/early beats occurring from the atria I believe is what that means.
If we opt to go the new med route he feels it takes about a week to slow heart rate and they generally give 1-2 mo to see if it will help symptoms or not. After that 1-2month period we'd re-eval and either con't on it and keep monitoring or B. go fwd with the Mitral valve replacement. I had asked if once someone has Valve replacement are they able to come off the HF med and he said he could not say in my case for certain. His thought was at whatever point we opted to do the Mitral valve replacement they'd keep the med in place till after and then do a trial I believe he said going off of it to see how I did without it. It seems he and Dr.Earing (Cardiologist) feel there may be issues separate from the mitral valve affecting the heart failure and they just don't know and wont know if all symptoms would go away/if meds would be able to be stopped post any MVR until after.
I guess I see no or little point in worrying about that all till we get to that point, was just info I kind of was curious about.
The actual EP Study and ablation if they find an arrhythmia they feel may be ablatable is Weds, 22nd at Children's and as planned now will be my EP dr and one of his Partners (also an Heart Rhythm dr.) + the Anesthesiologist who did all my previous heart surgeries and procedure. Although it would have been nice had the procedure occurred a few wks ago as originally planned as we likely would be moved on to the 2nd step (if needed be and maybe even be working out the shunt valve change details).. BUT I also feel really comfortable with the approach we are taking w this procedure and anesthesia approach and more comfortable having the Anesthesia dr. there who is so familiar w my history and did all former OHS surgeries and procedures.
With this procedure/Heart Cath we are using mild conscious sedation so i'll be numbed up where the multiple catheters are inserted and given very mild sedation but also be awake and apparently aware to give the best chance of success finding the suspected 'EAT' arrhythmia (which as Dr.Kovach explained is a type of abnormal rhythm that typically "goes to sleep when pts. go to sleep" and so they have to do the catheter procedure awake. I am ok with this, a bit nervous about the headaches BUT I also know the Anesthesia dr. knows my history and if need be (i'll talk to her ahead of time) perhaps Dr.Taylor can give something to help if I got nausea from laying flat. Admittedly the other thing that makes me a tad nervous (but where better to be I guess if it is the case) is feeling short of breathe during the procedure. Will take it as it comes though and not to worried or nervous.
Talked to Dr.Bragg's Nurse:
On a separate note I talked to Dr.Bragg's Nurse earlier this wk about possibly seeing her (Dr.Bragg) to see if she might be willing to tap the shunt and take off fluid to give relief which Lisa thought would be ok. Given Dr,Bragg was/is out of the office this wk, it seemed to make sense to wait and see her till the wk after the EP Study that way I should have an idea from my Cardiology Team what their plans are post this procedure and should be able to get their ok in the mean time that Dr.Bragg could start scheduling the date to put in a different shunt valve. This way I can talk to her about all of it at that appt to?..
In any case that's about it, will update after the study next wk if nothing else,
Thanks for stopping by,
Erica

Informative message from EP dr (regarding EP Study/Med)

I don't typically make it a point to share emails/messages I receive from my Providers but on occasion there are ones that I feel the information could benefit the greater MPS Community or others I know or perhaps even other HCP's who may be reading this blog.
This is an email received from my EP (Heart Rhythm) dr. regarding the EP Study and Heart med. - Informative to me.
He has been really great at answering questions of mine that have come over over the past 3 or so wks since I first met him and he brought up doing the EP/Heart Rhythm study and ablation. As I think I may have wrote about in another recent blog post, even though I've only met him and his NP once I feel like this is another score for my 'good' Providers Team. =) Below his email see other info.

Note from Dr.K.
Good evening, Erica:
These were all things I was planning on discussing with you when you came in for the EP study, but we can go ahead and start discussing them now.
If we do identify an arrhythmia that is amenable to ablation and are successfully able to get rid of it, then this will hopefully improve your symptoms. Granted your mitral valve is still a problem, so I cannot say that you will suddenly feel perfect. However this may help some of the shortness of breath and other symptoms that you experience.
If we don't identify an arrhythmia during the study, then I would say your own intrinsic pacemaker is running fast as a response to the extra work it does related to your mitral valve. Then we have to discuss what makes sense between you, me, and Dr. Earing. Ivabradine has been used primarily for treatment of heart failure in people who have bad heart function. The largest study was called the SHIFT trial, but that study specifically excluded patients who had valvular abnormalities. There have not been any big studies in patients with your particular mitral valve issues, though some smaller studies have suggested there may be some benefit. The medication does not help the valve per se, it just lowers your heart rate, which might in turn help your heart better tolerate the extra work it has to do because of the valve, and therefore help you feel better.
Once we have access to the medication, then it typically takes a week to get the heart rate down, then it's a matter of waiting for a month or two to see what effect that may have on your symptoms. If this is the route we go, and the medication does help, I would anticipate that you would likely have to be on it as long as your mitral valve is not working well. Once your valve is replaced, then we can try to come off the medication and see how you do.
I believe I hit all of your questions. Please let me know if there is something I missed, or you would like me to expand upon.
Take care,
-JK
About the EP Study - Ablation (uses Radiofrequency)
Transcatheter Radiofrequency ablation via Cardiac Cath - Electrophysiology Study to map out potential arrhythmia and suspected ectopic (early/extra) atrial rhythms or abnormal extra beats that occur in/start in certain area of the heart.
This approach is being done due to failed drug therapy (several beta blockers, current high dose calcium channel blocker as well as current high dose Digoxin which have been unsuccessful in controlling the to fast heart rhythm.
Due to this we've been unable to bring down heart rate with these meds which puts an un-due strain on the heart when there is already a strain from the severe Mitral valve leaking and narrowing. In addition to the plain to fast rhythm (tachycardia) there was/is the suspected 'EATs' rhythm on both the initial EKG at Dr.Kovach's appt and abnormal features on the 24hr Holter monitor done at that appt and read by him after, prior to his scheduling this study (he had waited so he could have a better idea if he needed to have "a 2nd pair of hands for the EP Study and Ablation" or otherwise it would have been scheduled with just him doing it.
As is he scheduled it with 1 of the other EP drs.
Because of the Holter monitor results and suspicion of the ectopic/early atrial arrhythmia the EP Heart rhythm study will be done under conscious sedation (basically awake but comfortable) and the areas where they insert the 2 or 3 catheters to gain access to thread up to the heart will be numbed to.
Apparently many people have EP Studies when there is strong suspicion of an ablation being done under general anesthetic but in all honestly even though we have an Anesthesiologist who is familiar with my airway (as mentioned in prior posts she did both the prior OHS's 2yrs ago, 22monthts ago and the TEE after the 1st OHS when it was realized there was a problem w the new valve and newly reconstructed area.) I am glad we are just doing mild sedation, makes it all easier in a way albeit i'll admit I am a little nervous about laying flat so long for this study and hope I an out just enough I am not aware enough to be aware of any headache/symptoms!
Given i'll also have to lay still for 4-6hrs after I am hoping for as I probably also wrote about before that they will let me sit atleast semi-upright if I keep my legs straight..
I know w the one other EP Study I had done 11 or so years ago, actually it was just prior to switching to start following care w Dr.E at CHW I'd had an severe allergic reaction to an IV Antibiotic that was given prior to the study (I can't remember why it was even given) and so I was so snowed from the Benadryl that even though they used conscious sedation w that I had 1st had to try and do a tilt table test which I couldn't even stand up straight for due to being so zonked from the Benadryl and then once on the EP table I remember pretty much nothing between the Benadryl and sedation.
In fact after that EP Study a relatively new continuous read implantable EKG monitor was placed and I don't even remember them telling me about that after the EP though I am sure the dr. did. If lucky I wont really remember this study either though I know the plan is to use very light sedation to have the best chance of locating the ectopic atrial arrhythmia (which apparently "goes to sleep" as the body sleeps as was evidenced according to Dr.Kovach by my 24hr Holter recording.
Stress dose steroid wise for this procedure and sedation the same steroid dosing is used as when giving general anesthetics where airway intubation is done and the Anesthesiologist is familiar w all that.
I am just patiently, impatiently waiting for this to be done so I can get clearance to move ahead to changing the shunt valve.. Granted as I've also written about w that the stiffness in mid-shoulders/neck is still there intermittently and it seems now the sensation I got when Dr.Bragg was draining the glorious CSF a few wks ago (glorious in that I got relief but the nerve almost pulling sensation that occurred during is occurring now intermittently but actually really frequently).. perhaps as the shunt is trying to drain?
I am not sure but it doesn't discriminate if I am walking or sitting when it occurs but is a twinging, nerve like pulling that is just odd.
Kind of takes my breathe away in surprise initially as it occurs.
Actually my R hip has been bothering me, albeit this is the same 'hip pain' I was getting w my previous LP Shunt the yr. leading up to seeing Dr.Bragg and then when she did her 1st revision and did that decompression surgery, what I had always thought was hip pain (b/c there is hip joint/bone issues on imaging that could correlate) turned out completely went away. Well anyways that is now recurring but on the R side along w the very (very) low back pulling/nerve stuff.
Ahh heck, I might as well face it, my body hates me - a love, mostly hate relationship ie I try to love it and it reciprocates by hating me? =) <-- have to find humor somewhere, right?
In any case hopefully it comes soon enough we can do something w the shunt.. I don't really want to wish this time away b/c I LOOVVEEE the warmth (minus the fact the humid air is making breathing feel even worse, literally like I am trying to breathe from underwater!) but I do so want to feel a wee bit better both cardiac wise, either w the Cath study or hopefully, if it helps the new med and to on the headaches front.. hopefully that different valve would help atleast some better and not make this nerve/weird pain even worse!
In any case, thanks for stopping by,
Erica
PS: I am writing this at 5 in the morning b/c I can't sleep and my obnoxious neighbor above me seems to be having a good ole' time tonight doing god only knows what. Sleep is for the birds?

EP Study info

I know I updated last wk (I think?) about the EP Study/Ablation being rescheduled due to med miscommun. and rescheduled to the 22nd.
My EP dr. emailed me the day after the Study was cancelled (so the morning the study was originally scheduled to be done) both apologizing (which was nice albeit not necessary) and gave me the med instructions re: what and when to stop the various cardiac meds that I should have been told of ahead of this past wk's scheduled EP Study and Ablation.
I also heard from the Anesthesia dr. i'd originally hoped to use (she was out of town last wk., she had messaged me and let me know as much regarding being gone but that she'd also shared her anesthetic recommendations with my Team for the sedation for last wks. originally scheduled study) and she is working the 22nd. In her messages she said she should be able to be part of the Team on my case the 22nd which is good.
In addition to Dr.Taylor it will include Dr.Kovach (my EP), a 2nd EP dr, Dr.Taylor (Anesthesia) and the others involved in the case.
1 Wk ahead of the 22nd (when the study is rescheduled for) I am to stop the Diltiazem (Calcium Channel Blocker)
5 days ahead of the 22nd i'll stop the Digoxin
The morning of i'll skip both the Lasix and the Spironolactone morning doses
I'll keep taking both the Aspirin and Coumadin doses and this last time we tested INR 2 days prior but they do not want to stop either med for the study.
In the email Dr.K also included (which I did know from my Endocrine drs. office) that they received the pre-procedure stress dose steroid dosing recommendations. The recommendations for pre-surgery stress dose steroids to include the 100mcg pre-sedation IV Solu Cortef which is to be given 1hr ahead of time (I had always thought it could be given 30mins to 1hr but according to my dr they highly recommend it be given an hour ahead of time) and then i'll wean at home.
Otherwise breathing issues in this humid weather have been pretty fierce, thankfully it is not a constant but wowza when it is an issue, it is most definitely an issue and seems so much worse lately! I check INR again Weds, at CHW prior to my ERT (enzyme) infusion so may mention the change in symptoms to Jane, Dr.E's Nurse when she calls with results. I doubt anything we'd do but worth mentioning.
I am actually a little unsure if say the EP Study they find an arrhythmia and say it could be ablated (basically gotten rid off) could that help the CHF symptoms and thus the breathing symptoms?
I know he's said it is ok but I did email this question to Dr.Kovach. I am a little curious if say they don't find an arrhythmia and then we have to start the new Heart failure med but say we ended up still having to do a mitral valve replacement at some pt. (which Dr.E seems to really feel we'll have to do sooner than later, thus a 3 OHS) are pts. able to eventually get off of the Ivabradine once say MVR is done?
In any case will update when there's anything worth updating, this week I as I mentioned above I infuse on Weds. Not a lot else going on this, wk., impatiently trying to be patient waiting to get this different shunt valve so I could HOPEFULLY sleep a little better!?!?!
Otherwise it has been a busy wknd, Fri., my Mom, Sister, Z and I went to Sun Prairie and Madison to some crafty type stores and Greenhouse. Sat we had a cookout and bonfire while getting eaten alive at my Sister's. Earlier in the day Zan and I had went out to the Horicon Marsh which is only about 5-10mins from where my Sister lives. Zan wanted to come home with me and the fireworks didn't end up starting till around 10 so we left a but earlier but did see other fireworks on our drive back to my Apt (about 20min drive), this way we beat all the crazies to.
Sun my Parents had a cookout so we all went swimming and the kids played, and good food although is always even better when one is hungrier!? =) Definitely a busy and tiring wknd, especially given sleep has not been my good friend lately!
Thanks for stopping by,
Erica

Plans change, change plans. It is hard not feeling good, Wishing I felt better.

EP Study was cancelled and rescheduled from tmrw (7/1) to 7/22 due miscommunication regarding meds. As disappointed as I was about this, for various reasons I initially was thinking this was atleast only 2 wks away but nope, it is 3wks from now. 3 effing weeks from now. This means at minimum I am sure another most likely 6 wks of these damn headaches which I am beyond tired and sick of and frustrated with. We can't do anything with the shunt until the EP Study is done as Dr.Bragg would like an 'all clear' basically from my Cardiology Team that they feel shunt surgery is safe.
I sort of understand this from her perspective but not entirely given 2yrs ago leading up to those 2 OHS for the aortic valve replacement/Konno procedure we did no less than 3 shunt revisions during the period we knew we where going to have to do the valve replacement. Perhaps i'll call and talk to my Cardiology Team this wk, see what they say although I still have this doubt Dr.Bragg would schedule anything till after the EP Study was done. - I feel like I am in a never ending cycle with these headaches and heart stuff and although, yes I realize it is whining and not right of me to ask I just want to ask God why? Why me? Why can't I feel better for a while, I mean really better w no headaches/pressure, no intermittent stiffness (the newest thing w the LP Shunt) and no cardiac issues/breathing issues which can get particularly bad in this humid/stuffy weather....
I understand her wanting to be safe but it just isn't easy when 1/2 the day is pretty much miserable. =/
Most of all I guess, again understanding it's not fair of me to question but why do things always seem to have to get put off/rescheduled/cancelled or something on one end causes another thing to be delayed? =/ I feel really, really frustrated and a little angry tonight.
Regarding why the Study/procedure was cancelled My EP Team and Cardiology Team had advised to stay on the Coumadin (INR was 1.88 Monday so not to bad) and had gotten instructions from my Neuro-Endocrine dr. regarding stress dose steroid dosing but apparently lines where crossed as far as my EP dr. thought I'd been made aware to stop certain meds. Per the Pre-op Nurse today I was to have stopped the Digoxin and the Cardizem and I would not take the Lasix nor the Spironolactone (water pills) the morning of the study but I was never told not to take these and unaware till the pre-op Nurse called today. =/
When the pre-op Nurse called she was going over everything, asking when I'd last taken various doses and I could tell felt really bad about this. Then Dr.Kovach's Scheduler called back tonight to give me the new study date and I hadn't yet been told this was for sure cancelled to which she couldn't apologize enough. She said after we had things squared away and after I'd explained I really was probably most upset b/c this delay meant a further delay in Dr.Bragg being able to do a shunt valve revision due to (Dr.Bragg) wanting my Cardiology Team to give their "blessing" re a revision. =/ So frustrating!!!
Needless on the medical front it was NOT an awesome day between this shunt business and finding out I'd have to wait and then finding out I'd have to wait even longer still b/c the EP Study miscommunication and reschedule. NOT Awesome at all. =/
I really appreciate my entire Medical Team but I really wish people in the medical field understood how bad we pts. feel and what it means when things get cancelled when we, the pts. are symptomatic (and for that matter dealing with multiple issues)! - Could have pounded a fist in the wall honestly, I get it miscommunication happens but for real, does it always have to be with me??? The thought of likely 6 or more wks with these damn headaches AND THEN given we're going to the mini nav .5 fixed pressure valve likely even worse spine nerve irritation just makes me sad. Not even mad about all of this, it just makes me sad knowing it's not going to be feeling good any time soon b/c 1 thing is going to affect another is going to affect another. Why, just why God can't we have simple and why can't we go back to 2 shunts w less spine-nerve irritation?
I rarely ever am in tears and is tears of frustration when I do over medical stuff but right now I am in tears over all this b/c I just don't understand why it all always has to be so hard and can't just something be simpler and go according to plan and my body play nice?
Tomorrow is a new day, thankfully, b/c I can't do anymore of this day and perhaps sleep as shabby as that is every night will help things be a little better perspective wise.
Thanks for stopping by, sorry to be so whiny. Believe me I am still grateful for my Team and for having access to the care I do it just doesn't always make uncertainty and feeling bad and seemingly (feels like) endless changing plans any easier.
Erica