It's well known valves usually worsen in my MPS if tissue valve is used so that's definitely unusual and concerning to my Cardiologist but we just monitor. - I don't remember for sure but think it was double digits when we replaced this valve 2.5yrs ago although I think there was also leaking then. Now it is at 9-10mmhg so high moderate range I guess for stenosis. Thankfully although concerned why it would keep worsening Dr.Earing also said there wasn't anything we'd do now anyways and we'd just keep monitoring. The good thing (and big reason why we can just keep monitoring) is there's no signs of recurrent CHF and PA pressures look ok.
I guess he'd monitor most closely for recurrence of the CHF and I do currently (STILL, ugh! Yup I might be whining just a bit =D ) still take medium dose Lasix (20-60mgs, 2x a day) and Spironolactone (25mgs 2x a day) and then 3 or 4 other heart meds for heart function, heart rate and to help heart muscle. CRAZY though!
If someone would have asked me before my 1st OHS if I ever thought I'd go through more than 1 Open Heart Surgery much less the 3 I've already been through and then, lol heart overall and valve still be so imperfect I'd have thought they where CrAzY! Is what it is though I guess!? God knows the plan and I walk with him (thankfully and even when I falter he's still by my side)..
I take meds to help heart muscle function (low end of normal), heart beat/rhythm (was lowest it's been in a long time at the Appt (70s-80s, Dr.Earing was pleased with this and feels the Amiodarone likely is benefiting) and lastly I also take Ivabradine for heart rhythm, Amiodarone, Lisinopril (heart muscle), and the 2 meds for keeping fluid off + the Coumadin and then Lovenox when INR is to low (to bridge till we normalize blood thinner level with Coumadin).
In his note and we talked about it yesterday Dr.Earing did raise concern about how often my INR has been out of range and apparently that can have an affect on heart function - maybe b/c it's greater (thicker? lol I honestly am not sure) blood flow when we need really thin flow through the mechanical valves to decrease risk?
I go to Boston Sun., to see my Genetics dr., I know he's working on a re-formulated supplement (Resveratrol) that the hope is it will act like a chaperone therapy, cross the BBB and get in to the heart + help clear out GAG where my weekly Aldurazyme infusions do not. The feeling is these therapies whether the Aldurazyme, the potential Resveratrol re-formulation or the Gene editing/Gene therapies studies (or all taken together) will all be complimentary therapies with no one likely to be a stand alone therapy. Whatever the case I just need something better - heck something that could calm my crazy heart rhythm down would be AWESOME! Is so darn ANNOYING!!!!
Humira
Dr.Earing also asked about the Humira and what the plan was with this. How would we know if I should stay on it. At 1st I wasn't entirely sure what he meant but explained that the biggest thing I've noticed is the spine-bladder irritation is so much improved (which was/is enough for me to want to stay on it). Dr.Earing was pleased with this and like my PCP and the Rheumatology Dr. just reiterated we'd have to con't to monitor for CHF (Heart failure, a relatively common side effect of this med I guess) and infection risk, He is okay with my continuing it though as there aren't signs of CHF at the moment.
Rheumatology messaged (MyChart) and called the other day they have started the Prior Auth process with my insur., for trying to get approval of the drug. They seem to have done their research and really listened to me/Dr.Simpson as in his note Dr.Pruhs not only lists my MPS I but then secondary arthritis (information more often needed to get the med approved per Dr.Polgreen).
The big take away really and it's not new is that versus any dramatic change on any of the bigger surgeries or meds I take I made the comment to Dr.Earing I tend to get smaller changes from a lot of the stuff we do and I take but taken together all of these surgeries and meds and therapies make a much bigger difference for my life and my care. Subtle alone but much bigger differences together if that makes any sense!
Will try to update sometime soon after I get home from Boston next wk,
Thanks for stopping by,
Erica
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