Friday, April 8, 2016

"Your body reads the 1st line in the manual and makes its own rules" - Dr.Bragg
Why that quote/title you you ask? (Which I came across in an old draft on here I'd never finished while clearing out old things I didn't need) but it's a quote I distinctly remember Dr.Bragg saying albeit she was saying it at the time to make me laugh I believe (was after an unusual shunt infection presentation and catch, per the unfinished entry where she and her 1 NP had just come by my rm and gave me the news of the unexpected infection (we all had thought it was gallbladder related, General Surgery was involved, Dr.Bragg was just managing overall picture - I'll miss her for that! Even when she didn't need to be a part she wanted to be there, to be involved, to help).

Needless she was right then and it applies today, to the Cardiology /Heart Ryhthm drs. and seemingly to today's appt!
 I guess i'll just update on what we all talked about and where things are at? I haven't updated since last wk sometime so I re-checked INR on Monday and that was abysmally low (1.1 I believe off hand) which is basically at a level one would be at if they weren't needing to be on Coumadin in the 1st place. There where several days last wk I was sick, threw up in the mornings and although it didn't occur to me at the time I suspect/am guessing the Coumadin which I take in the morning likely wasn't dissolved/absorbed yet and likely came up with that funness, (my own word?)? I don't really know. Needless to say when Cardiology called with the result Monday they where concerned b/c when there is a mitral valve and aortic valve the risk of clots when so low is apparently greatly increased. They called in Lovenox (Enoxaperin) injections to initially do for 2 days and increase Coumadin to 10mgs times 2days, recheck before today's Appt. 
Labs done before today's Cardiology and Heart Rhythm Appts. show INR was 1.4 so not an great increase. This in turn means stay on the self injections 2x per day till Mon, re-check Mon.

Cardiology / Electrophysiology (Heart Ehythm) Appts:
Today's Cardiology/Heart Rhythm Appts went ok, nothing to earth shattering (do I say that a lot?) though a med dose change and if that doesn't work possible heart procedure to consider.
Med wise although it's not a commonly used dose (the med is fairly new, having been FDA approved last Spring, about a year ago) but we are going to trial adjusting the dose from 7.5mgs to 10mgs which is the highest dose used and isn't used often. He isn't sure this will work as what little evidence there is on the med shows statistical difference in those pts. taking 7.5mgs vs those few taking 10mgs is limited (not significant). He commented something about with this particular med it seems evidence has shown at this highest dose the body seems to just break it down vs being able to process and use it effectively. He did comment though that each person responds differently and was worth our trying. He seemed to feel since it's a med I've had the least amount of (really no) side effects with and fairly decent heart rate improvement, prior to the Mitral valve replacement it is worth trying this increase. I think our average heart rate pre-MVR was around 80bpm prior the surgery after starting the Ivabradine though we also had several other meds to help overall heart muscle function and to help heart rate if I remember right, prior to Jan. That decrease was nice!
Unknown, Potential Heart Cath-Ablation
It really is an unknown if this will help but we all feel it is worth trying and Dr.Earing (my Cardiologist) when he stopped by felt the same. It is doesn't work or any problems Dr.Kovach mentiond potentially trying a different beta blocker or calcium channel blocker we've tried in the past as the nature of the heart rate/rhythm problem can change after a Open Heart surgery. We'd more than likely still keep the Ivabradine and the other meds I am on (Digoxin, Lisinopril, Ivabradine and 2 meds for fluid), at the current doses as these are used to help heart muscle function better and likely have some affect on heart rate now).
The other thing he brought up was he said he may consider and asked my thought re going back in via a heart cath (catheters threaded typically via the groin) and doing a heart rhythm study known as a 'Sinus Node Ablation' where they thread catheter(s) around the sinus node to try and ablate around the sinus node to try to get this to "chill out" (lol, that made me laugh seriously when he said that). In doing this approach we would bypass other "drug cocktails" and try this lesser used procedure which does carry the potential risk for causing to slow of heart rate though (I didn't think to ask what degree the risk might be or if that is just an unknown) and thus need for a Pacemaker. 
This in turn I also did not think to ask can a Pacer even be done when there is programmable shunts in place given the shunts have to be reset by magnets and I believe Pacemakers do to? Something i'll have to ask Dr.Bragg about out of curiosity next wk. For now we'll pray the new dose perhaps helps, never know! 
I seem to do the opposite of what's the norm so maybe this to will be a abnormal, given many seem to not have significant HR changes at the higher dose perhaps mine will again? Can't hurt to pray and hope! =)
I admittedly can't entirely remember what Dr.Earing and I talked about (I got about 3 or 4hrs of sleep last night, fell asleep around 10-11 and woke around 2, couldn't fall back asleep. Ironically I am editing this now a day later and same Thurs night and oh gee the night before that and before that... my body is exhausted and yet sleep seems elusive in those early hours  I fall asleep fairly easily but can't seem to stay awake (pressure I think ) and thus other than later afternoon /mid evening I always feel tired.
 Thankfully that part of the day, ironically where cortisol picks up I feel more awake and get normal appetite back to - the other night I went to my Parents and yum at the dinner my Mom had made, lol. I certainly did not get her love of cooking or baking!
I met w both Dr.Kovach and Dr.Earing but admittedly remember more from Dr.Kovach (I wrote down notes right away after) than I  remember from Dr.Earing who came in shortly after. 
In his case if I remember right  he just wanted to check in, question about what was going on w Coumadin (wasn't aware why it was so low/was that likely  caused it and gave instructions what to do w dosing/when to recheck + if any issues to call over the wknd or email him.)
I'll see the 2 of them either together or separately again in 3wks.
On a slide back note  1 other thing I remember Dr.Kovach pondering over was other things that could be affecting heart rate but like Dr.Earing had at the last appt. he ticked those off and essentially said based off history he doesn't believe those are causes. 
Some things he to mentioned (he always asks how the shunts are, have I had any shunt surgeries since I've last seen them and usually knows I've not) where 
1. pain but he to felt that was very unlikely.
 2. Shunts but he to said pretty much as Dr.E has that we've had numerous shunt surgeries in the period we've been dealing with more difficult/higher heart rates and yet no matter when really good or when problematic heart rate variable doesn't seem to change, so he doesn't feel that's likely affecting it.

 He commented something along the line 'could it be some unusual problem in you not traditionally described for MPS' but there to he couldn't say and wasn't sure any one could. Dr.E and my Genetics Dr in Boston (who I see while there next wk) have wondered if there is some other underlying factor affecting/causing the heart issues to be more atypical but that they are just waiting to see what the whole genome sequencing shows.

I don't think we'll ever really know the answer to this question really, other than if the whole genome sequencing shows something. Sooner or later we'll sort it out.

Otherwise really that is about it, i'll f/up with both him and Dr.Earing, my Cardiologist in 3wks and we'll see how the new dosing is helping or if it is and make decisions from there.
In the interim I see Dr.Bragg next wk and fly to Boston that next (Thurs) morning. I am hoping I have a little more energy by then or at the very least I am hoping I get enough relief to feel decent for my flight on Thurs morning! I fly out of Milw., an airport I don't use quite as much (but am familiar with) and have a non stop flight to Boston. I'll see my Genetics dr. late the next day.             ---
On a side note, I signed up/was interview' for a speakers bureau position with Genzyme, something Patients have to do a simple interview for and then that gets reviewed and if accepted by the Genzyme folks you go though a training process with your local Patient Liaison. 
I'll do the actual training part in a few wks. In the interim unrelated to that i'll see a bunch of friends and my Insur. Case Manager from Genzyme while I am out there next wk which is always nice! To top that all of i'll get to finally meet my RfRD Runner, Sarah and her family! 

It is a exhausting, a little daunting (due to not feeling the best) but really looking fwd to it all! Thankfully my flight back Monday of Marathon day is that evening so less stress this yr. trying to see my runner and still get to the airport on time! I also have a non stop flight on the way home (yippee!!). =) 

Will try to update soon, have a few questions for Dr.Bragg next wk so will update about those perhaps after I get back from the Marathon and Boston - I am super excited to see the many friends I have out there including the friends I am staying Fri-Monday with (Thurs i'll stay overnight in a hotel, close to BCH where Dr.Bodamer is). =) 
Thanks for stopping by,

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