Echo, Cardiology Appt.

Today's Cardiology Appt was good - the Mitral valve gradient hasn't changed from last February (is at 9, moderate leaking, moderate stenosis (narrowing) which Dr.Earing cont's to be concerned about but is good it hasn't increased.
He said something to the effect of "your valve is at the range where we could intervene but I am not in your body and can't tell how you feel so you have to be a part of making the decision when to intervene and when do we go back in a 3rd time." (apparently a 9 gradient is right at the tip of being considered severe, if the # where to be a '10' per Dr.Earing they would consider the leaking and stenosis as severe and likely intervene). As far as things he advised to watch for and would necessitate coming in sooner than our otherwise 6mo Echo/Appt f/up would be rapid weight gain within a few days or over a period of a week or 2 and any changes in feeling short of breath.
He commented when we do decide to replace the mitral valve this valve to would be a mechanical and given it would be a 3rd open heart surgery (OHS) that would (could likely) present addt'l complications in any addt'l valve replacement but there is no doubt in his mind we're going to have to do it at some point. Having 2 mech. valves would mean any other surgeries would be a bit more involved with Lovenox/blood thinner injections given anytime you are not in therapeutic range and/or leading up to any surgery + (I think) some kind of heparin has to be given right up to surgery.
For now the various cardiac meds (various med classes) I take are doing a semi-reasonable albeit imperfect (again his words) job of keeping the heart rate and thus the stress of the heart pumping functioning and stress of the blood going back through the valve better controlled. The higher the heart rate the higher the overall stress on the heart and especially when there is narrowing of a valve so with the various meds Dr.E is trying to target different aspects of the heart function to minimize to what impact we can heart valve function.
I currently take several different classes of meds which as I said above semi-reasonably but imperfectly control heart rate (his goal is heart rate below 70, mine most often sits in the high 80's to mid 110's).
The meds I take include "water pills" including a high dose of Lasix twice daily (though there are occasional days when I am going to be somewhere out and about I skip a dose), Spironolactone which is a longer acting form of a water pill (Lasix typically lasts about 4 hrs each dose whereas Spironolactone, also twice daily seems to last closer to 8-10hrs and a different overall affect on fluid).
There is also the beta blocker which combats heart rate and is a long acting med 1x daily (the least favorite of mine of any of the cardiac meds which is saying a lot). Additionally there is Digoxin a reasonably high dose which has in my opinion and it seems Dr.E seems to think as well done the best of all the meds to control heart rate. This is also the newest addition of all the meds.
Last but not least (?) I take baby aspirin and Coumadin each once per day for the mechanical heart valve. The Coumadin is why we have to do such often INR-blood tests to try and get the blood level in therapeutic range.

I guess the other thing we really talked about was an article on the drug PTC (Ataluran) I gave him which I'd copied out of a Medical Journal I have (I frequently share MPS related cardiac articles with him).
After a few questions he stated and which I (we) already know from being on ERT for the past 9+ years is that Aldurazyme was thought to help some of the cardiac issues in MPS I pts. and yet has shown little benefit. He feels (as do I) Ataluran will not reverse the damage that is already present and likely we're to close to the point of needing a 3rd OHS (open heart surgery, valve replacement) that this drug isn't going to help that issue. More than likely the same for the CNS issues (atleast the Hydrocephalus) but if the drug prevented new damage once available that would be good enough to me! =)

If you could say a prayer I have an Appt with Dr.Bragg Thurs., that the Appt. goes well.. Jane, Dr.E's Nurse said to me today "You probably get more stressed about those appts than anything?" I just replied sort of laughing that I probably worry to much about the things that are pointless (non-medical stuff) and not enough about the things I should (medical stuff) perhaps b/c I've come to realize I have a decent team of providers, I can only advocate so much and worrying if something is or isn't going to happen serves little purpose other than to stress me out. Instead I just try to stay on top of what I can and learn where I can.
That said I do hope Dr.Bragg has some idea how to help the headaches/symptoms as I feel so worn out (tired) and headaches and everything - in other words kind of miserable a lot and I am 99.9% certain it is shunt related... I was supposed to have dinner w/1 of my former ERT Nurses after my Appt and after she got off work today (she works at CHW) and I just felt so tired and yucky so instead I came home we'll shoot for next wk instead.

In an un-related but related note last wk when talking to Dr.B. at that appt. she had asked if a temporary increase in pain med. might help w the intermittent but long (in length) occurring headaches which the meds really don't.
I just said since it doesn't impact the symptom we ought to not make any changes and she commented something along the line of opioid and related type meds really don't touch increased ICP type symptoms and all the more concerning to her that what I am intermittently experiencing (more frequently) is due to shunt issue. I know she really wanted to be able to help but I also appreciate that she recognizes the differences in when I am having symptoms a med might help and when I am having symptoms that are likely shunt related.

In any case, enough from me. Thanks for stopping by,
Erica