Echo, Cardiology Appt update, Hand surgeon info.

My PCP and I have been trying to figure out if we can go ahead w the hand surgery via a different approach or do we need to put it off altogether (waiting would present it's own issues, see below related to the valve replacement(s)) and have been working w the Hand surgeon and his ofc to sort this out. Right now it looks like if we proceed w minimal IV fluids during surgery, use oxygen but no sedation, including no twilight sedation and a nerve block (usually done in the shoulder I believe) and the Surgeon said we could do it w/my being semi-upright during the surgery. The Surgeon didnt feel there was any other viable symptom options and feels surgery needs to be done (3rd time for carpal tunnel release R hand and EMG last fall showed severe recurrence). PCP wanted to see what Cardiology says and perhaps proceed w just the block for anesthesia and oxygen if needed to lay flat during. My Ortho surgeon did say that bc he feels the surgery really should be done we could do the surgery w/just the nerve block and otherwise fully awake (no twilight sedation) and per Cardiologist fluids would have  to be managed closely. Surgeon also said that he could do the surgery while I was partially elevated which would help avoid the breathing issues and headaches. I am not totally sure what we'll do with this yet but is a little up in the air yet though would seem to make sense to get things like this done before we have to go ahead w the valve replacements down the road which could be in a few months or could be in 6 months we just dont know.

Today I saw Cardiology w/repeat Echo related to the appt several weeks ago w/my PCP who called and talked to Dr.E's partner 2 weeks ago and both thought fup should be moved up due to a change in symptoms. The appt could have been as soon as a week after my PCP and Dr.E's partner spoke but this conflicted w the fup w Dr.Bragg from the last shunt revision and as often happens I had to put one or the other off. So anyways the Echo showed that the valve issues and heart overall are essentially the same as Jan but because symptoms are worse Dr.E is concerned do we cont to watch the valve's or do we strongly consider doing the valve replacements now as he said in cases like mine where there are many underlying, complicating factors it isnt un-common to do the surgery when valve issues are moderate-severe but it also isnt a simple decision due to the issues like the on-going (but currently managed) shunt issues/revisions, restrictive lung issues, chronic steroid replacement and prospect of further, future surgeries for the many other on-going or potential issues that occur w MPS I.  Because of the issues especially w/having to sleep upright and now always sleeping on my couch propped essentially completely upright by numerous pillows he opted to double the lasix dose to 2x's a day, is keeping the bisoprolol dose at 5mgs/day and added Aldactone which is similar to the lasix and helps eliminate fluid from the body through a different action to treat over-loading or heart failure like symptoms. The lasix dose is 2x's per day, 80mgs total and in turn hopefully help breathing and cont to buy us time as Dr.E feels we likely will have to do both valves in order to avoid having to go back in, in a year or two and replace the mitral valve vs just (likely) doing both in the first surgery. He echoed the same entiment this time as last visit in Jan of "this is going to be a hard surgery on you and probably difficult but we'll get you through." My response? I strongly wish I could breathe easier all of the time but as i've said here before this surgery scares the crap out of me, even the various brain surgeries which didnt un-nerve me that much the first time seem like a piece of cake (and probably where) compared to this, I think mostly bc of their having to cut open your chest and then wire your ribcage back together. Makes me cringe just to write this. I follow up in a month and repeat today's lab work in a week (will do a week from monday since I am already at CHW for infusion)
He did say when we do the valve replacements is a little up to me, can I live w the symptoms the way they are, he feels it's a balancing act as he is concerned on one hand waiting to long but also concerned how he thinks from other experiences the surgery or more the daily coumdadin will impact day to day life vs how it is now. Yuck. He commented the impact he thinks the blood thinner will have on future surgeries and day to day life but he strongly felt it would have a impact though I am not completely certain how other than labs have to be drawn frequently (I believe) to check levels w/ his big concern was all the troubles we've had w the shunts and of course other future possible needed surgeries like the hand issues, spine issues, etc. The shunts are probably the biggest concern as we've had so many on-going issues with these, they can fail or require revision at any time and atleast one of the shunts reside in the brain which with blood thinners would be problematic to say the least. Not awesome, definetly not awesome. Although I have the restrictive lung disease I dont think the TPL shunt residing in the pleural space will post a problem for the future valve replacement surgeries but am not certain about that, Dr.E asked (to confirm) where they where today to make sure the locations hadnt changed and didnt make any comment that it would be an issue and when I asked Dr.Bragg a few months ago she didnt seem to think the shunts being where they are now would impact the valve surgeries either. Ive had a few MPS individuals comment they would be concerned if I chose to have this valve surgeries at a hospital independent of Dr.Bragg's location which is a strong possibility will happen but (and hopefully this will remain true!) she didnt seem to think I would have any issues w the shunts during the open heart surgery.. I guess when I see Dr.E in a month we'll see how the new med and the old med, new doses are affecting lab work and we'll see if symptoms are improved/improving and decide from there if we cont to watch, give it more time or whats next.

I think thats about it, stay tuned, as usual!
Thanks for stopping by,

Erica