If you wonder what "wash, rinse, repeat" refers to, this would be in regard to today's Cardiology Appt and Echo after having just been in the same clinic last week for the Heart Rhythm (EP) dr. appt. - A friend of mine, not MPS but has her own health issues gave this analogy for dealing w what is going on and seemed quite fitting! Basically get up and go on no matter.
Today's Cardiology Appt was both a f/up from the new Cardizem dose the beginning of May as well as the 6mo Echo. - Needless to say for whatever reason it's beginning to seem like I get not the beat cardiac news after every shunt infection I deal with? Granted it was 1.5yrs ago a few short wks after I'd gotten out of the hospital from that shunt infection I had to see Cardiology for a normal f/up and Echo then to and that was when I found out the Aortic valve was then in the severe range and would need to be replaced.
Can you guess what today's Echo showed? Why yes, of course it showed the Mitral valve had worsened further and is now in the severe range to. Because we are trying to sort the arrhythmia issues and b/c it isn't a clear picture (is it ever?) Dr.Earing is unsure if we should proceed to replacing the mitral valve (would be a 2nd mechanical valve, requiring different approach to thinning blood before surgeries/procedures and the 3rd open surgery for heart). - Right now I can safely be off Coumadin for up to 3-5 days before a surgery/procedure but with a 2nd valve in place we would have to do self injections leading up to any invasive procedures/surgery or in some cases Heparin or something similar has to be given till right before I guess - I am not entirely clear on this other than Dr.Earing is really concerned and not happy this has worsened/another heart surgery is in our (likely) near future.
In the mean time bc the picture is so unclear ie should we proceed to mitral valve replacement (in other pts he would recommend it at this point but b/c my history is so complicated and he feels this surgery will be rougher than the other 2 (as far as actual surgery I guess) due to so much scar tissue from the other 2 heart surgeries in addition to my MPS issues in general and even higher risk he would like to sort things 1 by 1. I agree w this.
They'll get the EP Study scheduled which he was pretty unhappy this hadn't been done already last wk after I was seen in the other clinic but he wants it done as soon as possible. He just said he'd talk to my other dr. today and would make sure they called w arrangements.
The biggest concern right now re why they want to do the EP-Cath Study is based off an EKG done in the clinic last wk during the Appt w the Heart Rhythm dr. they felt there was strong suspicion of an ectopic atrial tachycardia which is a type of arrhythmia. Apparently if it is this and they map it during the catheter study (catheter usually goes up the groin) they can ablate it/rid the heart of this abnormal rhythm.
Should that be unsuccessful they will move to the new heart failure med - evabradine which as I wrote last wk is for a different type of heart rhythm/heart failure issue. This is a brand new med so will have to approved by my Insur., though I have no doubt between Dr.Earing, myself, the EP dr and with the EP Study results we could get it approved w my Nurse-Case Managers help. I do have to laugh though, just about every Appt Dr.E asks how I manage to get him covered/approved to see (he and 4 other drs are out of network exceptions) which on my end hasn't been to difficult.
After those 2 things, should the EP Study be negative and the med not control symptoms well enough Dr.E said he would unfortunately move next to mitral valve replacement as all heart wise points to this being a big probable source of issue. I've a feeling this would not occur till later in Fall or Winter but could be wrong. He just said he'd want a f/up appt after the EP Study and would at very minimum want an Echo in 6mo if we hadn't done anything beyond the Study and med.
Given all this, he brought up having talked to my Metabolic Genetics dr in Boston and that he agreed we could try the Elmiron though he felt we should perhaps try to sort some of these other things first. As he put it "there are a lot of things going on with you and I worry about complicating the picture even more".
He did talk about how he and Dr.Bodamer talked about my having the moderate phenotypes for MPS I (mutations, somewhat of a predictor where a person w MPS I will fall on the spectrum) but my heart issues are a lot worse than the typical MPS I pt and on the other hand I don't have some of the more typical features. Like Dr.Bodamer, Dr.E said he wondered if there could be a secondary underlying connective tissue disorder which would/could possibly explain some of my features and why I don't have some of the more classic-moderate MPS I features.
Dr.Bodamer is moving ahead w a study to map some MPS I pts genotype to see if there are other underlying issues and had asked me if I would be willing to do this. It requires just blood work and no cost to myself or my insur. I guess I see no reason why not to do it, especially if it could potentially help us long term. That is all being set up and Dr.Bodamer's Genetic Counselor is calling me to get this squared away and sorted out.
Related to the mitral valve concern and the gradients worsening Dr.Earing had done a relatively simple 6mwt today and he said this not only showed lung functioning worsening a bit further - enough to be even more of a concern to him given the heart issues I guess. He also said during the actual walk test my oxygen saturations where dropping which was a concern to him although likely explains my symptoms at times when I am moving about. Similar SOB symptoms do occur even when doing basically nothing though so who knows!
Although the overall appt wasn't great given these new changes to the mitral valve, the very likely need for a mitral valve replacement (MVR) in the not as distant future and and the fact that MPS just keeps marching on I did have to laugh a few times.
The ACHD Fellow came in before "The boss" (Dr.E) and as he was finishing up he says to me "I hear your famous" to which I taken slightly aback said "Lol, why do you say that?"
He says to me "I hear you do all kinds of advocacy and awareness and are famous, maybe I should get your signature!?".
Hahah, made for a good laugh, I am definitely not famous though happy my Cardiac Team was apparently filling him on MPS and the enzyme drug I receive. =)
Dr.Earing, the Fellow and I where talking about this latest shunt surgeries as Dr.E asked if I had any upcoming procedures or surgeries he should worry about? I don't and just said I think we have some figuring out to do w this current shunt given symptoms are worse now then prior to these recent shunt surgeries; how when I am laying down and get somewhat better the longer I am up but at the same time my nerves in my back still seem so overly irritated.
Anyways as we where talking about this he brought up how we'd have to manage the Coumadin before the upcoming EP Study but "we're old hats at this now so it's just a matter of figuring out exact timing.". - He then asked about the actual recent shunt surgeries as I had said once Dr.Bragg talked to him/his Team the Coumadin and injections schedule went really well and we didn't seem to have any issues.
Somehow we got to talking about all of the recent surgeries and issues and he said something like 'Neurosurgeons have weird personalities, they're not known for being patient friendly' (or something like that) but then he made the comment he likes Dr.Bragg and thinks she's one of the few that is Patient friendly (down to the earth) and easy to work with. For Dr.E to say he likes anyone else his pts see is a pretty high compliment - he's pretty skeptical of most other drs. including some that I consider very good on my team.
I guess at this point it's wait for the EP Study date and then we'll go from there w all of this. I have to admit it worries me now that these issues are worse that i'll be stuck w these headaches and Dr.Bragg won't know what to do. I so badly feel we need that 2nd shunt and so many around me have commented but how do you relay that to someone you see for 10 or 15mins and when you likely look normal? =/ Agh if only prior decisions could be changed! It makes me realize although imperfect it sure was a whole helluva lot better prior to this recent infection and shunt surgeries.
I see Dr.Bragg Thurs and am praying so hard we can sort something out. Having not been sleeping great, throwing up some nights (but that was also the case in-pt, I just took valium fairly often at night) and just feeling not that great I pray we can sort something out and she'll see what others do. I trust her but that doesn't mean I'm not nervous about the appt.
Ironically I was thinking about the last time we removed an EVD (external drain) and this time and how symptoms after each where exactly mirrored (extreme nausea, throwing up and severe headache though the difference this time I recognized it soon after it began after the drain removal and the Nurses had given me pretty high doses of valium to control the symptoms and I slept for essentially the next 12-24hs sleeping off the most extreme.
The last time this same thing occurred 1.5yrs ago I had went home and was sick there for hrs before finally going to the local ER where they started valium as I was transferred back to UW. I feel like the drs witnessing the symptoms that time vs this time I was so drugged up and to it was Dr.Bragg the 1st time and not this time.
I just don't know but I do wish I felt better! Prayers for help and guidance always appreciated,
Thanks for stopping by,
Erica
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